Exam 2 PEDS

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Disease that care caused by inborn errors of metabolism
Phenylektonuria define
An autosomal recessive disorder that is an Inborn genetic error of metabolism that results in an absence of the enzyme phenylalanine hydroxylase. This causes the inability to metabolize the amino acid phenylalanine- essential amino acid found in proteins. Without this enzyme protein is not able to be broken down.
Dietary Management of PKU
- avoid phenylalanine food = no meats, low protein grains
- measured/limited amounts of cereal, strarches, fruits, veggies
-special breads, pasta, formula
- PA free formula
-Breast fed babies- mom must eliminate phenylalanine from her diet.
- goal is to promote nutritional needs for optimum growth and maintain phenylalanine safe range of (serum 2-6 mg/dL)
Safe range for phenylalanine?
Galactosemia defineautosomal recessive inborn error of carbohydrate metabolism where they lack an enzyme necessary to convert galactose (found in milk) into glucose.Galactosemia S/SVommiting, Weight loss, jaundice, cataracts, jaundice (hepatomeagly), leathargy, hypotonia.LT consequences of galactosemialearning disabilities, lower IQ, short attention span, behavior problems.Management of Galactosemialactose free diet, soy based formula. No breast milk. Calcium supplements, watch meds with galactose and lactose based medications (tums- antacid, penicillin, birth control).Endocrine disorders-disorder of thyroid function -disorder of pituitary function -disorders of adrenal function -disorders of pancreatic function (diabetes)Disorder of thyroid function include:Main physiologic action is regulation of basal metabolic rate -hypothyroidism -hyperthyroidism -may be related to a disturbance in secretion of TSHcongenital Juvenile Hypothyroidism definea congenital autosomal recessive disease of hypoplastic thyroid gland. Not working as it should or underproducing.Juvenile Hypothriodism two typescongenital and acquired.What is a major cause of childhood mental retardationCongenital Juvenile HypothyroidismAcquired Juvenile Hypothyroidism defineSomething happened and because of that they thyroid is not producing the amount of thyroid hormones that you should.Causes of Juvenile Hypothyroidism-Partial or complete thyroidectomy for CA or Thyrotoxicosis. -Following radiation for Hodgkin or other malignancy -rare occur from dietary insufficiency (iodine) in the US.S/s of Congenital HypothyroidismHypothermia (cold) Large Protruding Tongue Teeth that don't come in like they should Short thick neck Delayed dentition Hypotonia Poor feeding Prolonged Jaundice Constipation Missed or delayed diagnosis may lead to mental defectsMissed or delayed diagnosis of Hypothyroidism results inMental defectss/s of acquired hypothyroidism- decreased rate of growth - goiter - Myxedema (dry skin, puffiness around the eyes, sparse hair) - altered body proportions; short stature, legs proportionately short compared to trunkGoiter hypo or hyperthyroidismbothGoiter defineHypertrophy of the thyroid gland. Can be congenital or acquiredCongenital goiter Usually results frommaternal ingestion of antithyroid drugs during pregnancyAcquired goiter results ofneoplasm, tumur, inflammatory disease, dietary deficiency (but rarely in children), or increased secretion of pituitary thyrotropic hormones.How do we test for goitermake the swallowNew considerationsThyroid enlargement at birth may compromise newborn's airway (congenital). May become noticeable during periods of rapid growth. Large goiters may be obvious; smaller nodules evident only on palpation.What is the priority with a child with a congenital goiterAIRWAYTherapeutic management of hypothyroidism-oral thyroid hormone replacement (Levothyroxine) taken for life. -prompt treatment needed for brain growth infant -May administer in increasing amounts over 4 to 8 week to reach euthyroidism (start with the lowest doses and titrate up until you get it where it needs to be) - need to see them every 2-3 months to increase the dose with kids growing -compliance with medication regimen is crucialHow do you know if a child is functioning well with the dose of LevothyroxineBMR not freezing Growing No s/s of hypothyroidism Not constipated.Nursing considerations for hypothyroidism-Early recognition bring it to HCP attention -encourage compliance and periodic monitoring - Teach medication administrationWhat do you teach for med administration of Levothyroidism-comes in tablets or capsules (not liquid) -Crushed and given with formula -can not be mixed with soy-based formula and it will be excreted out of the body and not used. -avoid heat exposure (don't leave in window cill) - interferes with a lot of other medshyperthyroidism/ grave's disease-excessive secretion of thyroid hormone -autoimmune response to TSH receptors -familial association, not genetic -most common in female between 12 and 14-year-old -Increase T3/T4, and decreased TSHHyperthyroidism S/SFine straight hair Bulging eyes (exophthalmos) facial flushing Sweating Enlarged Thyroid Tachycardia Increased BP Breast enlargement Gradual Weight loss Muscle wasting Localized edema Tremors Diarrhea/Vomiting Menstrual changes Finger clubbing Irritability short attention span insomnia emotional liability increased appetite Rapid pounding pulse even during sleep widen pulse pressure systolic murmurs cardiomegaly heat intolerance enlarged thyroid gland SOB with exertionTherapeutic management Hyperthyroidism-Dont need to put under extra stress: No PE until medications are controlled, dont bring in front of classroom, no sports. - dx based on increased levels of T3/T4 withs suppressed TSH -Antithyroid drugs are first line tx -Subtotal thyroidectomy (if meds doesn't work) -Ablation with radioiodineMedication treatment teaching for hyperthyroidismPropylthiouracil (PTU) Methimazole (MTZ, Tapazole) -takes 2 weeks to see results -lack of compliance - Complete remission with med therapy in 1-2 years - Watch for toxic drug reactions (Decreased WBC, Sore throat, fever).A child comes to the school nurse and complains of fever, and sore throat. The child states they taken PTU for 4 days. What should the school nurse doMedication needs to be stopped bc he has developed agranulocytosis and may be neutropenic and he needs to be sent to HCP and put into isolation starting on an antibiotic and steroid.The pt has had a subtotal thyroidectomyNeck surgery at risk for airway At risk for HypothyroidismAblation with radioiiodineAn Injected and picked up by thyroid to kill it. So the patient will have hypothyroidism and need replacement supplements.Nursing considerations Hyperthyroidism-Identification (bracelet, necklace) -Limited activity and demand placed on the child (decrease stress- post pone physical activity, PE, gym, after school activities). -Counsel family and teachers (2wks before working and fever sore throat, call HCP) -High calorie, nutritious diet -Medication- side effectsThyrotoxicosisThyroid storm. May occur from sudden release of hormone. Unusual in children but can be life threatening, May be precipitated by infection, surgery, or discontinuation of antithyroid therapy, or undiagnosed.S/S of thyrotoxicosisVery high levels T3/T4, Fever, increased HR, diarrhea, dehydration, shock and deathTreatment of thyrotoxicosisAntithyroid drugs - if already on increase the dose Propanol to decrease the heart rate Steroid IV FluidsWhen instructing the mother of an infant with Hypothyroidism the correct procedure for medication administration the nurse should: 1. Teach her to rotate injection sites daily 2. Teach her the importance of not repeating a dose that is spit-up or vomited. 3. Teach her to crush the pull and mix it with a small amount of formula 4. Teach her to withhold the medication if the baby has decreased appetite, sleepiness, and constipation3. Teach her to crush the pull and mix it with a small amount of formula. Do not mix with soy bc protein will bind to it and get rid out of body. 4-signs of hypo so they really need this medication.If a patient vomits all of her pills what do you do nextGive the pill again unless it is digoxin (bc sign of overdose)Disorders of Pituitary functionMater gland that is responsible for regulating other glands. Two portions, anterior or posterior.Anterior Functionshormone levels, TSH, Prolactin, Growth hormone, LH, FSH, ACTH,Posterior Functionsvasopressin, oxybueton,Types of HypopituitarismGondotropin deficiency (absence of sex hormones) GH deficiency (not growing linearly, somatic growth decrease) TSH deficiency (hypothyroidism) Corticotropin deficency (adrenal insufficiency)Causes of hypopituitarismPituitary or hypothalamus tumors Encephalitis Radiation to head/neck Head trauma Congenital IdiopathicHypopititarism (Growth Hormone Deficiency) defineInhibition of somatic growth in all cells. Absence or regression of secondary sex characteristics. Normal height and weight at birth-growth patterns progressively deviate. Must be distinguished from familial short stature, constitutional growth delay, or genetic disorder (turner's syndrome). Not growing linearly or the ability the you potentially can. Look at growth chart, make sure they get the nutrients they need and refer to endocrinologistClinical manifestation for Growth Hormone Deficiency-short stature- below the third percentile -Weight: normal to heavy -Skeletal proportions normal for age -Retarded bone age (7 year old with 3 year old bones) -appear younger than chronological age (L hand and wrist xrayed compared to bone age) -Delayed eruption of permanent teeth --- delay sexual development ---abnormal intelligence ---emotional problem (part of growth hormone deficiency or bc their small)Dx of Growth Hormone deficiencyDiagnosis based on history (child and parent), physical exam, x-rays, low serum GH levels (stimulation tests)Treatment of Growth hormone deficiencyWe can give them growth hormones daily subcutaneous injections of growth hormones-done at night(in stomach or arms) bc that's when we produce growth hormones at least 5x a week. Continue until final adult height reached- calculated and given until reached. May be indicated to continue therapy as an adult due to effect of GH on bone and muscle. Other hormones replacements as needed- thyroid extract, cortisone, testosterone or estrogen and progression.Prognosis of Growth Hormone deficiencyGH replacement successful in 80% of affected children. Growth rate of 3.5 to 4 cm/year before treatment and increase to 8 to 9 cm/yr after treatment (about doubled). Response varies based on age(when you started it), length of treatment(how long can continue it), frequency of doses(how often you give it), dosage, weight, and GH receptor amount.Nursing consideration for GH replacement- Family members support needs - Child's body image (psychological issues) - Preparing child for daily injections (teach how to do them) - Injections given at bedtime for best results - treatment very expensive (20-30k) try to get insurance on board. - bullying - babied in school - dress appropriately for age - held to age expected behavior - help teacher treat them appropriately like all the other kids -Identification (growth chart) -assist wit testing (die them something to stimulate secretions: insulin. Serial blood draws q 30 minutes for 3 hours to draw their growth hormones- try to use IV so dont have to continually stick them). - emotional support (angry, low self esteem, tx with chronological age not developmental)Precocious Puberty definedSexual development before age 9 in boys and before age 8 in girls. Occurs more frequently in girls.Potential causes of Precocious PubertyDisorder of the gonads, adrenal glands, or hypothalamic- pituitary gonadal axis. No causative factor in 80% to 90% of girls and 50% boys.Types of Precocious Puberty1) True or complete precocious puberty (Central Precocious puberty) 2) Precocious Pseudopuberty -- premature thelarche --premature pubarche --premature menarcheTrue or complete precocious puberty (Central Precocious puberty)Happening early identical to normal pituitary gland is prompted to release hormones (gonadotropins) that stimulate testicules or ovaries to start making other hormones = changes little boys/girlsPrecocious pseudopubertyincomplete puberty- starts and can resolve on it own or can be stopped. Can really Impact a child's height bc loose years of normal growth- no hormones are elevated.First sign of puberty for girlsbreast developmentFirst sign of puberty for boysTesticular enlargementsPremature thelarcheearly breast development- usually resolves on own but needs to be evaluatedPremature pubarcheunderarm hair and pubic hair needs to be evaluated but usually goes away on its own.Premature menarchePeriod without other evidence of sexual development- needs to be treated to be stopped.Therapeutic management of Precocious PubertyTreatment of specific cause if known Otherwise may treat with Lupron. Monthly IM injections that stop precocious puberty and continue it until puberty is supposed to start (11/12)Slows prepubertal growth to normal rates. Treatment is discontinued at age for normal pubertal changes to resume. Psychological support for child and family (A 6 year won't jump to 13 year feelings, these children can be fertile).Congenital Adrenal Hyperplasia (CAH)A family of disorders caused by decreased enzyme activity required for cortisol production in the adrenal cortex More than 90% of cases are 21-hydroxylase deficiency, 10% of cases 11 hydroxylase deficiency Overproduction of adrenal androgens and results in virilization of female fetus Decreased production of cortisol and aldosterone (Adrenal glands not producing enough cortisol but can produce too much testosterone).How to determine gender based onInside organsClinical manifestations of CAHMales- precocious genital development Females- may be born with varying degree of ambiguous genitalia. - enlarged clitoris appears as a small phallus - fused labia produce saclike structure without testes -internal female sexual organs are intact (fallopian tubes, ovaries intact). -decreased stress response -hypoglycemia -increased inflammatory response -hypotension -compromised immune system - hyponatremic dehydration <135. (increased urine output, WL, dry mucus membranes)Diagnostic Evaluation-Ultrasonography to visualize pelvis organs (to confirm gender). - chromosome typing for positive sex determination and to rule out any other genetic anomalies.Boys with CAHGoes undiagnosed until BP problem, glucose is a problem, and s/s of dehydration (Increased urine output, Wl, dry mucus membranes)--- little girls get picked up on earlier bc genial don't look right.Therapeutic Management of CAH- Confirm dx and assign a sex to the infant according to genotype - Cortisone to suppress abnormally high secretion of ACTH and replace the cortisol they aren't able to make - Reconstructive surgery as required. - have to be on cortisol for the rest of their lifeNursing considerations of CAH- Parental anxiety - Teach parents signs and symptoms of dehydration (and how to reverse it) and salt-loosing crises and to have hydrocortisone available and know how to use it. - Role of genetic counseling - child with adrenal crisis (not producing cortisol we need and have to replace it)How long will they be taking the PA formula for an infant with PKUfor the rest of his life. They will need this to get nutrients they need.If a child who was dx with PKU has short attention span, poor st memory and poor motor coordination what should the nurse do?Check to see if they are still getting that formula.Diabetes Mellitusa chronic disorder of metabolism characterized by a partial or complete deficiency of the hormone insulin. Most common metabolic diseaseCarbohydrate metabolism in type 1 DMthey have no insulin to shit off internal sugar production. They do not have an insulin bridge made by the pancreas so sugar can not go from the blood sugar into the cells. Therefore are cells are hungry, and have no energy.Symptoms of DMpolyuria, polydipsia, polyphagiaNormal blood sugar80-100Type 1 DM in usually dx in what populationChildren and young adultsType 2 DM in usually dx in what populationAdulthood but rates are risingType 1 or 2 is insulin dependent?Type 1Body does not produce insulin in which type of DM?Type 1The body fails to produce and properly uses insulin occurs in which type?Type 2Who is overweight type 1 or 2Type 2Type 2 treatmentsWeight maintenance/loss, exercise, insulin, and oral agents, Teach them to be active and make healthy choices.S/s of Type 1 DMHyperglycemia & Acidosis -Weight loss - Polyphagia - Polydipsia -Polyuria -enuresis - irritable -unusual fatigue -abdomen painYour patient comes in with acanthuses nigrocans what should we think? and what should we suggest?Type 2 DM and suggest a fasting lab work and lipid panel.Clinical Manifestations type 2 DMoverweight Fatigue Frequent infections Acanthosis Nigrocans Baby with thrush b/c have excess sugar floating around.Acanthosis nigrocansThick velvety dark pigmented lines in the elbows, neck, knee, often in overweight and african american. Need to suggest lipid panel and fasting glucose levelsWhat population would you suspect Type 2 DMpt w Acanthosis nigrocans pt w persistent infection pt who is overweight pt w thrush in their mouthDiabetes Management overviewMedical nutrition therapy Med administration - insulin -oral agents Developmental issue Glucose/urine monitoring Hypoglycemia agent Hyperglycemia agent Sick day managementMedical nutritional therapyCarbohydrates are the main food type and increase blood sugar the most. Fat can increase blood sugar later. Develop CHO goals- - conventional consistent CHO intake (hard for kids food jags) -Intensive-Insulin to CHO ratio where you cover the carbs you eat with insulin. -Appropriate meal and snack planning -Appropriate insulin regimen and physical activity programAppropriate meal and snack planning for DM patientbalanced diet extra foods during increased exercise consistent meal times avoid high sugarDeveloping an Appropriate insulin regimen and physical activity programincrease insulin with extra food Decrease insulin need with strenuous exerciseGood Carbohydrate Sources:bread, cereal, grains, pasta, rice, pastries, milk &dairy fruits, vegetablesCarbohydrate free sourcesmeat, cheese, sugar free jello (don't need a finger stick with these foods )Oral agents of Type 2 DMMetformin Meglitinides Sulfonylureas Alphaglucosidase inhibitors ThiazolinedionesRapid acting insulinsAspart (Novolog) Lispro (Humalog)Short acting insulinsregularIntermediate ActingNPH/LenteLong actingglargine (Lantus) detemir (Levemir)Most cases kids will have a insulin regimen oflong acting at night and a short acting to replace what they eat.Rapid acting onset/peak/duration15 minutes, 1 hour, 3-4 hoursShort acting onset/peak/duration30 minutes/ 3 hours/ 6-8 hoursintermediate Acting onset/peak/duration1-2 hours/ 6-8 hours/ 12-18 hoursLong acting onset/peak/duration4-6 hours/8-20 hours/24 hoursIf its little timmy birthday can he have a cupcake?YES. Just need extra insulin to cover it.Snacks mom can send in for children's bdayString cheese Beef sticks Fruit and veggiesIf have a basketball game and gave type 1 DM?Child needs to eat more before a game because exercise uses insulin more effectively.Conventional insulin therapyBID or TID (where they eat the same amount of carbs for every meal)Intensive insulin therapyBasal pump/Bolus injection insulin dosing based on what they eatTypes of tools for intensive insulin therapyinsulin pens blood glucose meters sub q ports changed 7-10 d insulin pumpsInsulin pumpsonly gives short acting insulin it delver as stead amount and can program boluses but they can stop working and are very expensiveWhen a pt comes into the hospital with a pumpIts good to compare our results to theirs. Let them be in control of theirs while still doing ours and comparing. Can stop working. so they need to always have emergency insulin with them.Blood glucose monitoring4x a day at- before meals and bedtime. Also anytime the child feels symptoms of hypoglycemia Check before recess/ PE class bc increased insulin production and uses it better so it can drop.URine ketone monitoringAnytime blood glucose level is > 240 mg/Dl on to separate occasions. During illness Pump therapy- blood glucose of > 240 mg/dL on any occasion.Management tools for intensive insulin therapyrapid acting insulin Basal rate Bolus to replace what you've ate Correction factor : Correct bs above a desired level. pump emergency kitsYour child has insulin to CHO ratio of 1:18 and a Correction factor of 1:50> 150 mg/dL how much insulin do they need if their BS is 227 and they ate 48 CHO4.2 (4 if in syringe, 4.2 if on pump)Your child has insulin to CHO ratio of 1:5 and a Correction factor of 0.5:15> 120 mg/dL how much insulin do they need if their BS is 210 and they ate 60 CHO15 unitsDM management of a toddler (1-3)-parents must differentiate misbehavior from hypoglycemia. ----say no ---- can go a long time without eating anything. - encourage child to report "funny" feelings -expect "food jagS" - give choices regarding SBGM, injection site and food choices. --- let them do the alcohol pad --- let them pick which fingerDevelopmental management of DM for a Preschooler (3-6)Reassure the child who views DM tasks as a punishment Encourage the child to participate in simple DM task - read the number on the meter - pick the site -simple addition -gather supplies Teach the child to report lows to an adult and teach the child what to eat when low.Developmental issue with DM of a schoolager (6-12)educate school personnel about DM Encourage age appropriate independence --fingersticks, -- reading meter --math -- program the pump but all needs to be double checked. ALL ACTIVITIES MUST BE SUPERVISED encourage extra circular activities and participation in social groups. 11-12 years old able to perform an occasion injection.Developmental Management of Adolescence-more capable of performing self care activities -know which food fit into meal plan and how to adjust -more willing to perform multiple injections -Risk takers (will sleep in a and not eat, think they can skip their lantus and not have an effect). -Need continued parental involvement and support. - mensuration increases blood sugar - eating disorder impact blood sugarIf a Adolescent with DM is drinking what do we need to teach themThey need to eat, alcohol will cause hypoglycemia.Sick day management of DMgive insulin as scheduled Check blood sugar more frequently Monitor urine for ketones with every urine If they aren't eating as much then you don't need to give them insulin. (exactly the same but more frequent)Complications of blood glucose alterationsHypoglycemia Hyperglycemia ketosis Acidosis DKA (hyper, keto, acidosis)Hypoglycemia<60 needs to be treated. Develops because the body doesn't have enough glucose to burn energy. Can suddenly happen.Causes of hypoglycemia- too little food - too much insulin - vomiting - exercise - change in schedules/s of hypoglycemiaLow blood sugar Hunger Headache Confusion Shakiness dizziness SweatingHypoglycemia treatmentimmediate ingestion of 15g fast acting carbohydrates; wait 15 minutes, repeat until normal. - 1/2 up of juice, or regular soft drink, 1 cup of milk, glucose tablets, cake icing, 8 life savors, package of skittles. Followed by meal or snack (protein) - peanutbutter sandwich -Cheese and crackers Once they feel fine they can go play again. Avoid chocolate bc it won't rapidly increase blood glucose. <70 have a snack 1/2 sandwich or cheese crackers.Severe hypoglycemia treatmentIF the pt is unable to swallow use glucagon emergency kit. 1Mg dose. Inject sub-q or IM once given- place patient i recovery position (onside bc glucagon will make them vomitt), to prevent aspiration. After awake must feed them. Glucagone has an expiration date so watch. Only works once- so once blood sugar is raised feed them.Hyperglycemiadevelops when the body has too much glucose in the blood >180. Serious problem if untreated.causes of hyperglycemiatoo much food(CARBS); too little activity; too little insulin; and illness/infection/stressS/S of HyperglycemiaHigh blood sugar High levels of glucose in urine Frequent urination Increased thirst dry skin Nausea Blurred visionIf the patient mom calls and says the child has a small amount of ketones in their urine NBNAincreased fluid, no caffeine, avoid exerciseIf the patient mom calls and says the child has a moderate-large amount of ketones in their urine NBNACall and probably come to ERHyperglycemia treatmentCheck urine ketones mod-large call HCP small- increase fluid and no caffeine do not increase exerciseKetosisWhen your cells don't get the glucose they need for energy, your body begins to burn fat for energy, which produces ketones. Ketones are chemicals that the body creates when it breaks down fat to use for energy. The body does this when it doesn't have enough insulin to use glucose, the body's normal source of energy. Presence of excess ketones in the body Blood ketone concentration between 0.3 and 7.0 mmol/LKetonesAcidic substances that are made when the body breaks down fat for energyKetoacidosisWhen ketones build up in the blood, they make it more acidic. Severe form of ketosis Reflects levels of 7.0 mmol/L or higher Lowers the pH to 7.3 or lower (ph level goes down, anion gap goes up)AcidosisWhen ketones build up in the blood, they make it more acidic. Ph is below 7.35S/s of acidosisDeep, rapid breathing (known as Kussmaul's respirations) confusion or lethargy abdominal painBlood tests to diagnose metabolic acidosis may include:Arterial or venous blood gas Electrolytes: Na,KCland HCO3-, (total CO2 content), Anion Gap- can be normal, high, or low. A high anion gap indicates metabolic acidosis. This usually happens in diabetes because the bicarb is low.Pt with DKA how do we know how well they are doing?Monitor anion gap- tells us how acidic it isDKA is a complex metabolic state ofhyperglycemia >300, ketosis, acidosis <7.3, HCO3 <15)S/S of DKAdeep rapid breathing, fruity breath odor, very dry mouth, N/V, lethargy/drowsinessCauses of mortality in DKA-Failure to make diagnosis -Cerebral edema (shift fo fluid from interstitial to extracellular) - hypokalemia/Hyperkalemia -Hpoglycemia -Hypovolemias/s of DKAvomiting without diarrhea Dehydration symptoms with excessive Urine output Respiratory distress without lung pathology w/o crackles or Rhonchi KussmaulDKADKA usually occurs as a consequence of absolute or relative insulin deficiency that is accompanied by an increase in counter-regulatory hormones (ie, glucagon, cortisol, growth hormone, epinephrine). This type of hormonal imbalance enhances hepatic gluconeogenesis, glycogenolysis, and lipolysis.Electrolyte disturbances also occur in DKAPotassium loss is caused by a shift of potassium from the intracellular to the extracellular space in an exchange with hydrogen ions that accumulate extracellularly in acidosis. Much of the shifted extracellular potassium is lost in urine because of osmotic diuresis . - High serum osmolarity also drives water from intracellular to extracellular space, causing dilutional hyponatremia. Sodium also is lost in the urine during the osmotic diuresis.Therapy of DKA1. fluid replacement (priority) 2. electrolyte replacement 3. Insulin therapy 4. Careful monitoringGoals of DKA-correct dehydration -correct acidosis and reverse ketosis -restore normal blood glucose -avoid complications of therapyNursing actions for the first hour of a pt with DKAFluid resuscitation: 10 mL/kg 0.9% NS IV over 1 hour.Nursing actions for the second hour of a pt with DKARehydrate fluids 0.9% NS +20mEq/L KPHos + 20 mEq/L KCl (or KAcetate). Insulin 0.1 units/kg/hour regularWhen do we change for 0.9% NS to D5 0.45% NS +20mEq/L KPHos + 20 mEq/L KCl (or KAcetate).Once the serum glucose reaches 250-300When do we know if DKA is resolvedNormal ph Normal anion gap Normal Physical exam findings pt able to tolerate oral feedings.When do we discontinue IV fluids for a pt being treated with DKA?After they tolerate oral fluidsWhat is the goal for correction of blood glucose rate in a patient being treated for DKa?100mg/dL/hour and a rise in the pH of at least 0.03 units/hour.When to Discontinue IV insulin in a pt being treated for DKA>at the time of SQ rapid acting insulin or 30 minutes after SQ regular insulin.Goal fo the first hour of treatment with IV fluidsfluid resuscitation and confirmation of DKA by lab studiesGoal fo the second hour of treatment with IV fluids- slow correction of hyperglycemia, metabolic acidosis and ketosis - Start correcting electrolyte imbalances - continued volume replacement This usually requires several hours and meticulous attention to the patient's response to therapy = shock then get more but that a Doc callInsulin treatment is begun when treating a pt with DKAafter the initial fluid resuscitation at beginning of 2nd hourInsulin therapy for DKA during second hour- Turns off the production of ketones - Decreases blood glucose - Check glucose hourly Low-dose insulin infusion Decreases risk of hypoglycemia or hypokalemia Goal is to decrease blood glucose by 100mg/dL/hourHow long is the insulin therapy given in treating a pt with DKADo not reduce or discontinue the insulin infusion based solely upon the blood glucose. The insulin infusion should be continued until the ph >7.30 and/or the HCO3>15 mEq/L and the serum ketones have cleared.Prior to insulin administration in tx a pt with DKAassess vital signs, blood glucose level and neurological status. nsulin is a "High-Alert" medication, therefore two nurses should verify the insulin order, dose, and volume prior to administration. IV tubing should be primed with the insulin solution before administration. DO NOT GIVE INSULIN BOLUSInsulin and DKAhe dose of insulin should remain at 0.1 units/kg/hour until the acidosis resolves (pH 7.3 and/or bicarbonate >15 mEq/L) • Do not decrease rate or stop the insulin administration based solely on glucose values • Once blood glucose reaches 250-300 mg/dL, maintain insulin and begin dextrose infusion At the time of insulin infusion, make sure any indwelling insulin pump has beenPotassium Administration: General Guidelines when treating your pt with DKAConsult with your Pharmacist before administering potassium. Start replacing potassium after initial fluid resuscitation and concurrent with starting insulin therapyIn general, in patients with DKA, there is a significant potassium deficit that must be replaced • Potassium replacement should continue throughout IV fluid therapy • The maximum recommended rate of IV potassium replacement is institution specificDextrose administration in treating your pt with DKAMaintain glucose between 150 to 250 mg/dL to prevent hypoglycemia • Check glucose hourly until stable • Check electrolytes every 2- 4 hrs until stableBicarbonate is contraindicated in DKA therapydue to increased risk of cerebral edema. Consult with a Pediatric Endocrinologist and/or Intensivist prior to initiating bicarbonate !Bicarbonate therapy should only be considered in cases of: Severe acidemia Life-threatening hyperkalemia .Monitoring children with DKA treatmentHourly assessments: Vital signs q 15 min -pulse, respiratory rate, B/P, oxygen saturation, temperature, and pain level (as applicable) -Neurological status -Accurate fluid intake/output -Point-of-care-testing blood glucose level hourly -Potassium level Notify the treating physician immediately with any critical lab values or change in mental status Every 2 hours: -Urine ketones -Serum β-OH -Labs (repeated every 2- 4 hours or more frequently, as indicated in more severe cases) •serum glucose, electrolytes (q8), BUN, calcium, magnesium, phosphorus, hematocrit, and blood gases •Continuous cardiac monitoring •Amount of administered insulin All insulin rate/dose changes should be doubled checked by two nurses and documented on the flow sheet. •Administer oxygen •Insert additional peripheral intravenous catheter -for repetitive blood sampling, and/or-if insulin drip is initiated Children with altered mental status or consciousness, consider:-Secure the airway -Nasogastric suction -Bladder catheterization .High Risk Patients DKA ICU ifSevere DKA (pH < 7.1 or < 7.2 in young child) - Altered level of consciousness - Under age of 5 years - Increased risk for cerebral edema • Be prepared for intubation • Caution with meds that may alter mental statusTransition off IV insulin when treating for DKApH > 7.30 and HCO3> 15-18 •Anion gap: typically less than 12 •Patient able to eat •Subcutaneous insulin: -Give sq injection, D/C IV insulin / IV dextrose, feed child -Known diabetes patient •Previous dosing •May need additional rapid acting insulin to overcome insulin resistance after DKA -New patient •0.7 - 1.0 units/kg/ day is general guidelineFetal RBC productionliver makes all the RBC then the bone marrow gradually take overs Hemoglobin F predominant for 1st 6 months of life. Fetus receives iron through the placenta from the mother and stores iron for later use 6 months. At 4-6 months they are a little anemic because have to make their own physiological anorexia.Hemoglobin Flarger capacity to carry oxygen but doesn't last as long 1 week where as Adults last 121 days.When does baby not get the iron stored for 6 monthsIF baby born premature, mom had a placenta prevue or abruption, bleed a lot or hemorrhage, mom not taking in enough baby is born depleted.Assessment of Hematologic FunctionComplete blood count w/ diff History and assessment findings: (have they been sick, bruising, fam hx, diet hx = iron def anemia, cancer or anemia). Child's energy and activity level -(are they able to keep up with kids at the playground? do they fall asleep at school? How do they do in recess). Child illness and healing patterns (how well do they get over being sick) Growth patterns - kids only want to drink milk so when that's all they do- end up anemic.Hgbability to carry oxygen. 11-16Hct% of volume of circulating RBC. 35-41RBC4.5-4.8Platelets2-4 x 100,000. Clotting factor of blood.Platelets >100,000-nmL amountNo contact sports Protective equitment such as bike helmets when riding a bike or scooter.Platelets between 50,000 and 100,000Padding with activity. Protective equipment such as bike helmet when riding a bike.Platelets <50,000extreme caution as kids can spontaneous bleed in their head. No bike or scooter. Coffee table w/ sharp corners needed padding. Important to do near checks>20,000 PlateletsKids can return to schoolCBC with differentialWBC 5000-10000 Lymph 25-40% =virus Mono 2-8% - late to acute phase of infection/2nd line Neut 54-75%= bacteria Esop 1-4% = allergies, parasite Baso 0-1% = inflam or hypersensitivityWBC 2000, Segs of 34% and Bands of 26% what is the absolute neutrophil count1200Nursing interventions for neutropeniaMonitor V/S especially temperature; Temperature >_100 is considered a medical emergency!! Good hand washing Inspect skin for breaks & redness Inspect mouth for ulcers No flowers & plants in room Low bacteria diet Change dressings & lines using sterile technique No live-virus vaccines(MMR Varicella, inhaled flu mist) Avoid contact with persons who carry viruses or sickIf you pt is neutropenic and have a temperature of >_100.0Call HCP, come to ER, place in isolation, full work upLow bacteria dietNo peper, take skin off of fruits or vegetables, avoid deli eat, pasteurized cheese, cant eat pizza if not refrigerated immediately, meat cooked really well.AnemiaThe most common hematologic disorder of childhood. A decrease in number of RBC and or hemoglobin concentration below normal. A state in which an individual can not conduct his/her activities because the oxygen carrying capacity of their blood is low.s/s of anemiaanoxeria, pallor, skin break down, jaundice, Tachycardia & Tachypnea, Altered LOC, behavior changes, weakness or low exercise tolerance***, gum hypertrophy, smooth tongue, blood in urine or stool, infections, cold intolerance.Effects of anemia on circulatory systemHemodilution Decreased peripheral resistance Increased cardiac circulation and turbulence -May have murmur -May lead to cardiac failure Cyanosis Growth retardation clubbing of the finger (chronic)Therapeutic management of anemiaTreat the underlying cause --Transfusion after hemorrhage if needed --Nutritional intervention for deficiency anemias Supportive care --Intravenous (IV) fluids to replace intravascular volume --Oxygen --Bed restNursing considerations of Amenia Anemia-Prepare child and family for lab tests (have to get their blood) -decreased oxygen demands (scheduled activities that allow for rest-10 minutes, play chess, do half mile instead of whole) -safety (cant keep up with peers, fall) -good hand hygiene and mouth care -maintain normal body temperature -prevent complications (observe for cardiac decompensation) -support familyS/S of cardiac decompensationlow urine output, Increased heart rate, decrease BPMajor anemia's of childhoodproduction -iron deficiency anemia -aplastic anemia Hemolytic -sickle cell anemia -Beta ThalassemiaAnemia caused by decreased productionbone marrow falls to produce RBCs - leukemia or other malignancy -chronic renal disease bc erythropoietin -collagen diseases -hypothyroidism -nutritional deficienciesiron deficency anemiamost prevalent nutritional disorder in the US. Happens when the body does not have enough iron to produce Hgb. Incidence has decreased with WICS/s of iron deficiency anemiairritability, anorexia. pallor of skin and mucous membranes mild growth retardation exercise intolerance Frequent infections Cognitive delays and behavioral changesetiology of iron deficiency anemia-inadequate iron stores at birth (last 4-6 months) -deficient dietary intake --rapid growth rales --excessive milk intake (limit amount bc poor source of iron Ca/Phos binds with iron and isn't readily available). --poor general eating habits (just want to eat mac and cheese) --exclusive breast feeding after 6 months (baby needs more food) -impaired iron absorption --presence or iron inhibition (phosphorus/ca interfere with iron absorption) --malabsorption disorder (lactose intolerant, IBD, Zantac) --chronic diarrheaInfant should have no more than _ oz of milk4Toddler should have no more than ___ of milk24Therapeutic management of IDAPrevention - breast milk or commercial infant formula for first 12 months of life, - limit formula to <1 L/day - Limit milk to <24 ounces/day Nutritional supplements by age 6 months -iron fortified formula, -cereal at 6m Iron supplements - ferrous sulfate for babies who exclusively breast feed Blood transfusions for severe casesNursing implications for IDAAssessment; pay particular attention to milk and iron intake. Determine and eliminate the cause (only eating mac cheese or kid drinking 7 glasses of milk) General nursing implications for anemia Provide foods rich in iron Teach parents to administer supplements Administer parenteral iron safely (iron can be deadly/overdose on flinstones) Follow-up care (come back and have levels redrawn)Foods rich in ironrasian, peanut butter(> 1 year), tofu, green leafy vegetables, beans, red meats, eggs, whole grains.How to administer oral ironBest absorbed in an acidic environment is best - OJ as a chaser Give with straw or back in the mouth past the teeth Rinse mouth/ brush teeth after administration. Teach parents; measure accurately, increase fluids and fiber in diet bc constipating. Taste horrible. Can get chewable tablets but not for infants. Avoid antacids, coffee, tea, dairy products, eggs, or whole grains one hour before or two hours after administration.Adverse effects of Oral ironNausea Gastric irritation Constipation Diarrhea Anoxeria Staining of teeth Tarry stools Overdose is lethal (parent need to store it properly)Aplastic anemiaBone Marrow failure; All formed elements of the blood are simultaneously depressed —"pancytopenia"(decreased WBC, RBC, Platelets). Pathophysiology; red bone marrow converted to yellow, fatty marrow (doesn't produce as it should)Aplastic anemia etiologyPrimary (congential) secondary (acquired)Diagnostic evaluation for anemiabone marrow biopsyTherapeutic Management of Aplastic anemia-bone marrow transplant -stem cell transplant -immunosuppressive therapy -ATG&Cyclosporin preventing and killing t-lymphocytes from attacking bone marrow to fix the aplastic anemia. -no chemo but transfusionsNursing considerations of Aplastic anemiaLook at leukemiaAnemias Caused by Increased Destruction of Red Blood CellsHemolysis (breakdown of RBC) Decreased life span of RBC Hereditary spherocytosis (HS) Splenectomy/partial splenectomy can correct hemolysis but does not correct underlying defect Splenectomy rarely before age 5 Aplastic crisis -bone marrow and stem cell are damagedsickle cell diseasegroup S hgb. If you are a carrier then have s/s but not the disease or the crisis. Usually Asymptomatic until age 6-months. Partial or complete replacement of normal Hgb with abnormal hemoglobin S (HgbS). Hgb S has altered amino acid structure making it less soluble than Hgb A. When deoxygenated Hgb S becomes more viscous and precipitates into long crystal shapes. When RBCs are sickled, they are more rigid, fragile, & rapidly destroyed. They lose their ability to flow easily through the tiny capillary bed, and obstruct capillary blood flow. Microscopic obstructions lead to engorgement and tissue ischemia RBCs with Hgb S live less than 20 days.s/s of sickle cell anemiapain, prickly tingling and sleep = hurts, but also affects organs. Usually lose spleen by age of 5 years. Splenomegaly RBC get stuck and are risk for rupture. Problem in the abdomen, liver, brain- stroke, chronic ulcer priapism- erection that won't go down.Complications of anemiaAcute Painful Episodes (Sickle Cell Crises) Stroke Sepsis Acute chest syndrome (vas-occlusive crisis in the lungs ) Reduced visual acuity Chronic leg ulcers Delayed growth & development Delayed puberty Priapism Enuresis (not able to produce concentrated urine. Lots of dilute so not uncommon to wet the bed).Acute Painful Episodes (Sickle Cell Crises) Precipitating factors-Anything that increases the body's need for oxygen or alters transport of oxygen -Trauma -Infection, fever -Physical and emotional stress -Increased blood viscosity due to dehydration -Hypoxia = vasoculsive crisis ---From high altitude, poorly pressurized airplanes (no small aircraft), hypoventilation, vasoconstriction due to hypothermiaFirst line treatment of acute episode of sickle cell diseaseopen up the vascular with fluids so that sickle cells can get through easier. Heparin does NOT help.Hypoxia in a vasocclusive crisis from sickle cell disease is caused by-From high altitude, poorly pressurized airplanes (no small aircraft) -hypoventilation, -vasoconstriction due to hypothermiaTeaching for sickle cell disease to prevent crisis-dont need to go to disney when its 80 degrees - no colorado skiing -avoid high altitude -bball games have extra gatorade -avoid being sick or around people who are sick -hand washing -hydration -decrease stressTypes of Acute painful episodes (sickle cell disease)-Vaso-occlusive (VOC) thrombotic -Splenic sequestration -Aplastic crises-Vaso-occlusive (VOC) thrombotic in sickle cell disease-AKA painful event/ painful episode -Most common type of crisis—very painful -Stasis of blood with clumping of cells in microcirculation →ischemia → infarction Sickle cells get stuck in joints, inflammation, painful, tingling, prickly feeling, b/c RBC is stuck and below that are not getting oxygen they need. -Signs—fever, pain, tissue engorgement, inflammation in the area.Splenic sequestration in sickle cell diseaseLife-threatening—death can occur within hours Blood pools in the spleen and cant get out so the spleen just keeps getting bigger. Signs -Profound anemia -Hypovolemia -ShockSickle Cell disease have a scenic sequestration and pt got spleen taking out nursing implicationsSpleen is important for infection prevention, no spleen increased risk for being illl. They need 250 mg of penicillin for 3 years and then before invasive procedure or dentist.Aplastic crises in sickle cell disease- diminished production and increased destruction of RBCs. -Triggered by viral infection or depletion of folic acid. s/s: include profound anemia, pallor.Acute Chest Syndrome in sickle cell diseaseSimilar to pneumonia -VOC or infection results in sickling in the lungs -Chest pain, fever, cough, tachypnea, wheezing, and hypoxia -Repeated episodes may lead to pulmonary hypertension -pain management -antibiotic (ICU) -blood transfusion, ventilatorPrognosis Sickle Cell Anemia•Prognosis varies on how well it managed. •No cure (except possibly bone marrow transplants) •Frequent bacterial infections may occur due to immunocompromise •Bacterial infection is leading cause of death in young children with sickle cell disease •Strokes in 5% to 10% of children with disease -Result in neurodevelopmental delay, mental retardation - need doppler study every year. -repeated episodes can lead to pulmonary HTN.Crisis Management/Nursing Considerations SCAMaintain Hydration!!! to expand dehydration. Iv fluids 20mL/kilo bolus will help pain. Maintain Oxygenation -remove constrictive clothes -rest Pain management - around the clock better than PRN -lots of exposure so have a high tolerance but HCP are scared to overmedicate. Need to advocate for their pain- may need morphine. Support for the Child & Family - anxious -cold -skin changes/tingling -in and out a lot -may be aloneThalassemiaInherited blood disorders of hemoglobin synthesis. Body just doesn't make enough RBC. Classified by Hgb chain affected and by amount of effect Autosomal recessive with varying expressivity. Both parents must be carriers to have offspring with disease in order for it to be passed on. Alpha or beta-globulin chain in Hgb synthesis is reduced or entirely absent. -Large number of unstable globulin chains. -Causes RBCs to be rigid and easily hemolyzed -Severe hemolytic anemia leading to hypoxia Normal RBC size with decreased amounts of Hgb can lead to chronic hypoxia= severe need transfusion. There is no med for this or transplant. Detected in infancy or toddlerhood. Can have mild-severe anemia.Chronic hypoxia in Thalassemia-headache -irritable -precordial and bone pain -exercise intolerance -anoxeria -epistaxisTwo types of Thalassemiaalpha- a chains effected. beta- most common has four types. Thalassemia minor—asymptomatic silent carrier Thalassemia trait—mild microcytic anemia Thalassemia intermediate—moderate to severe anemia + splenomegaly Thalassemia major "Cooley anemia"—severe anemia requiring transfusions to survives/s beta- Thalassemia major or cooley-Anemia; severe -Bronze skin tone from hemosidorosis (RBC broken down so much a bronze color = excessive iron stores in various tissues and bc of multiple transfusion). -Growth retardation and delayed puberty -Cardiac enlargement with flow murmur leading to heart failure -Bone pain, skeletal deformities, fractures -Death usually related to heart failure or iron overload (repeated blood transfusion and blood being broken down).Medical Management: Thalassemia major or cooleyDiagnosed with Hgb Electrophoresis: •Blood transfusion to maintain normal Hgb levels •Side effect—hemosiderosis -Treat with iron-chelating drugs such as deferoxamine --Binds excess iron for excretion by kidney --IV or SQ over 8-10 hours multiple times/wk --May be given at home with IV pump per parents --New oral chelation drugs(can be given at home)—deferasirox ;Worldwide use --Also give oral vitamin C to facilitate binding of ironNursing Interventions for Beta ThalassemiaAdminister packed Red Blood Cells safely and immediately within 30 minutes. Type and cross it. VS beginning & also for 1st hour. Another nurse needs to check. General measures for anemia (rest, grouping care, safety, pale/tired, cant keep up with peers, fall risk, cold- extra layer increased room temperature). •Monitor chelation Therapy -N/V/D -Decreased appetite -Rash -Increased liver enzymes (monitor) -Neutropenia -*excreted via the kidneys so adequate hydration is vital •Measures to enhance self-esteem & body image (self imagine, confidence, face looks different). •Measures to enhance home health maintenance and oral therapyRXN to transfusionfever, rash, troubled breathing, rash. IF they experience this stop and keep IV open call HCP.how bleeding stops1) blood vessels get smaller 2) platelet plug is made: 3) clotting factors-Platelets and how they stop bleedingAdhesion- platelets sticking to wall of torn blood vessel - Activation and secretion- platelets change shape to connect with other platelets - Aggregation- platelets stick to each other and to the blood vessel ALL OF THIS IS TEMPORARY •Clotting factor and how it stop bleedingA fibrin clot made from clotting factors is needed to make a fibrin weave to make strong clot or strong seal.Alterations in Clotting Function diseases•Idiopathic Thrombocytopenic Purpura •HemophiliaIdiopathic Thrombocytopenic Purpura (ITP) defineAcquired Hemorrhagic disorder (not born with it).Autoimmune destruction of platelets in the spleen.May be acute or chronic.Cause unknown. Occurrence ; Caucasian children between 2 & 8 years old. Most children recover spontaneously in 6-12 months,Clinical Manifestations ITP•Sudden onset of easy bruising, purpura (deep bleaching bruising indicative of cancer/ITP/Meningitis), and petechiae •Epistaxis (nose bleed) •Platelet count less than 20,000 •Prolonged bleeding timeMedical Management ITP•Primarily Supportive • No specific test, R/O other diseases. Can do a bone marrow test to be helpful. Draw labs= need to hold pressure for 5-10 minutes. Anti D Antibody- Plasma derived immunoglobulin.(clear the antibody that kills platelets). Causes transient anemia by clearing the antibody coated RBCs. Must gave a decent amount of RBC a normal range before you give it. Intravenous Immune Globulin (IVIG). Course of Steroids (1-2 weeks) Splenectomy and IV Gamma Globulin if chronicNursing Interventions ITP•Educate Parents •Assess for bleeding •Neuro checks - spontaneous bleed in the head. •Safety measures to prevent trauma •Avoid unnecessary procedures, needle sticks, suppositories, rectal temps. etc. •Apply pressure for 5 minutes after venipuncture. •Follow-up care in home or clinic once stablized : Soft tooth brush someone with them so they dont fall. Nothing rectally, avoid contact sports, padding. Watch for: Irritability, change in LOC, h/a, slurred speech.When discharging a child with ITP from the hospital with a platelet count of 40,000, it is most important for the parents to understand the importance of; A.Bike helmets whenever the child rides. B.Keeping follow-up appointments. C.Seeking immediate medical attention for severe headache, vomiting, or cognitive changes. D.Continuing the Anti-D antibody therapy at home.C.Seeking immediate medical attention for severe headache, vomiting, or cognitive changes.Epistasis nursing care•Seek medical attention for a nose bleed lasting longer than 30-minutes •Sit up leaning forward •Apply pressure for 10-minutes •Apply ice bag to bridge of nose. If they lean back then they will swallow blood and vommit it back up.HemophiliaA group of hereditary bleeding disorders that result from deficiencies of specific clotting factors. You dont have the clotting factor you need.Types of Hemophilia:Hemophilia A -Classic hemophilia -Deficiency of factor VIII -Accounts for 80% of cases of hemophilia -Occurrence—1 in 5000 males Hemophilia B -Also known as Christmas disease -Caused by deficiency of factor IX -Accounts for 15% of cases of hemophiliaEtiology of Hemophilia A•X-linked recessive trait •Males are affected •Females may be carriers •Degree of bleeding depends on amount of clotting factor and severity of a given injury •Up to one third of cases have no known family history -In these cases disease is caused by a new mutationLabs Hemophilia ANormal platelets Normal PT PTT time prolonged Hgb & Hct = normal to lowBleeding after procedures of Hemophilia AMinor trauma, tooth extraction, minor surgeries - Large subcutaneous and intramuscular hemorrhages may occur - Bleeding into neck, chest, mouth may compromise airway - usually catch after circumcision for boys.Hemarthrosis early signsEarly Signs: stiffness, tingling, or ache followed by decreased mobility, warmth, redness, swelling, & severe pain. Need intervention. Compromise circulation blood cant get to the extremities.Medical Management of Hemophilia A•Prevent Bleeding •Replacement of deficient factor -Factor VIII concentrate (synthetic or large plasma pull) - or DDAVP (desmopressin- synthetic intracranially increases production). •Regular program of exercise & PT (active ROM, passive will hurt). - Can be given at home: teach parent how to start IV. Cant play contact sports. They can swim, golf, play chest, pad rails and coffee table , soft carpet floor knee pads when crawling.Nursing Interventions Hemophilia•Measures to prevent bleeding -safe environment -teach the use of protective equipment -avoid aspirin -SQ rather than IM, venipuncture rather than finger stick, no suppositories, adolescents need electric razors, avoid contact sports. -Soft toothbrush, pad rails, elbow pads. •Recognize and Control Bleeding -Pressure X 15 minutes for frank bleeding -RICE ; Rest ( Immobilize), Ice, Compression, elevation -Children are aware of internal bleeding and can tell you. -Be suspicious of head ache, slurred speech, altered LOC, black tarry stools.Recognize tingling, warmth and decrease mobility.Nursing Interventions Hemophilia:•Prevent side effects of bleeding; -active ROM after the acute phase -physical therapy regular basis -nutritional counseling --increasing iron foods bc loss w bleed. Want normal weight to reduce wear and tear. •Family Support and Home Care; -Med Alert bracelet -Notify school nurse and teacher.Von Willebrand diseaseA hereditary bleeding disorder involving deficiency of von Willebrand factor (a plasma protein and the carrier for factor VIII) •von Willebrand factor needed for platelet adhesion •Transmitted as autosomal dominant trait •Occurs in both males and females •The gene for the disease is located on chromosome 12Diagnosis of Von Wilebrands disease• Diagnosis—Laboratory Findings - Decreased von Willebrand factor levels - von Willebrand antigen levels - Decreased platelet agglutination - Prolonged bleeding time - PTT may be normal or prolonged.Von Willebrand Disease ( vWD) treatment- Infusion of von Willebrand protein concentrate - DDAVP infusion before surgery, during menses, or to treat bleeding episodeNursing intervention for vWD- Avoid aspirin or NSAIDs (increase bleeding time and inhibit platelet function) - Manage bleeding episodes with prompt infusion therapy - Children with vWD have normal life expectancy if well managedTherapeutic management/Nursing of HS PurpupaMost cases resolve without treatment • Corticosteroids and anticoagulants for severe or persistent cases • Symptomatic treatment • Maintain hydration • Monitor renal function • good I/o • Monitor U/A for protein and blood • edema • bun and crVariations in Pediatric Anatomy and PhysiologyHealthy full-term infant's immune system is still immature. • The newborn exhibits decreased inflammatory response to invading organisms, increasing susceptibility to infection. • Cellular immunity is generally functional at birth; humoral immunity develops over time.Primary ImmunodeficienciesMostly hereditary or congenital • Types - Humoral deficiencies - Cellular immunity deficiencies - Combination of humoral and cellular immunity deficiencies - Phagocytic system defects - Complement deficienciesSevere Combined Immunodeficiency Disease (SCID)A defect characterized by the absence of both humeral and cell mediated immunity • Exact cause unknown: Dx made by exclusion and lab values help.SCIDs S/SEarly frequent infections • Failure to thrive • Chronic diarrhea • Persistent thrush • Adventitious sounds related to pneumonia • Lab findings of very low levels of immunoglobulin's • Graft versus host reaction to any foreign tissue- something not yours enters you body -transplant.Medical/ Nursing Management SCID•IV Immunoglobulin •Sterile environment •Bone Marrow transplant (helps a lot ) •Prevent exposure to infections - cant fo outside •Support & educate familyBig difference with HIV in kids and adults is that kids.1) haven't been exposed to as many illnesses to be protected against these illnesses already and they don't have immune response after getting HIV to protect themselves against the illnesses • 2) Mode of transmission is different in children- particularly infaEtiology of HIVTransmission from infected mother to infant; Prenatal, perinatal, through breast milk (vertical transmission) 93% • Blood transfusions (horizontal transmission) • Unprotected sexual intercourse with an infected person (horizontal transmission)Common Clinical Manifestations HIV in Children• Lymphadenopathy • Hepatosplenomegaly • Oral candidiasis thrush • Chronic or recurrent diarrhea • Failure to thrive (not growing) • Developmental delay • Parotitis (salvia gland infection) -get sicker than an adult would.AIDS s/sSerious recurrent bacterial infections • Lymphoid interstitial pneumonia • Pneumocystis carinii pneumonia (PCP); most common opportunistic infection in children 3-6 months old • Candidal esophagitis • Cytomegalovirus (CMV) • Herpes simplex disease • HIV encephalopathy • wasting syndrome • Cryptosporidiosis; Water borne parasite, diarrheaDiagnosis HIVPolymerase chain reaction (PCR)- detection of proviral DNA, 95% Infants over 1 month of age. • P24 antigen (specific for HIV) • Viral Culture • Enzyme-linked immunosorbent assay (ELISA); + for 18 mos in babies born to HIV+ mothers • Western blot; + for 18 mos in babies born to HIV+ mothers • Platelet count: above 500,000 (in severe HIV infection) • CD4 counts (low in HIV infection) Tx like they are positive for the first month they are born.Therapeutic Management HIVAntiretroviral therapy- all infants born to HIV + mothers for at least first few months. • Aggressive antibiotic therapy for infections • Modified immunization schedule • Prophylaxis against opportunistic infections- anti fungal mouth wash and antibiotic low daily doseNursing Considerations HIV•Prevention - no needles, safe sex, start condoms young, no breast feeding. •Risk for infection •Knowledge deficit - Transmission & prevention; HAART -Safety issues -Storage of medicines and equipment. •Altered Nutrition: less than body requirements -Increase protein & calories to help recover from infection. -Provide preferred foods -Nutritional supplements. - can go to school -universal precautions gloves, bleach, -Altered Growth & Development; encourage child to participate in activities with other kids - Caregiver Anxiety; psychosocial concerns? Can play soccer or swim. Disclosure, anticipatory loss, financial -Pain; manage aggressively; irritable or fussy keep an eye on pain.During teaching, the nurse should advise the family of a child newly-diagnosed with Graves' disease to: A. Encourage outdoor activities. B. Limit bathing to prevent skin irritation. D. Set the thermostat higher than normal for comfortC. Promote interaction with one friend rather than a group.A student with type I diabetes mellitus complains of feeling lightheaded. Her blood sugar is 60 mg/dL. Using the 15/15 rule, the nurse should: A. Give 15 ml of juice and repeat the dose in 15 minutes. B. Give 15 grams of carbohydrate and retest the blood sugar in 15-minutes. C. Give 15 grams of carbohydrate and 15 grams of protein. D. Give 15 ounces of juice and retest the blood sugar in 15 minutes.C. Give 15 grams of carbohydrate and 15 grams of protein.The nurse is caring for a newborn infant diagnosed with Congenital Adrenal Hyperplasia (CAH ). Which of the following nursing interventions should be given highest priority. A. Supporting the family. B. Assessing for signs of dehydration. C. Preparing the infant for chromosome testing. D. Referring the family for genetic counseling.B. Assessing for signs of dehydration.The nurse working with a teenager who has Phenylketonuria (PKU ) suspects dietary noncompliance from which of the following assessments? A. The teen lists his biggest concern is not being able to eat out with his friends. B. The teen is irritable and hyperactive. C. The teen is withdrawn and refuses to answer questions. D. The teen is sleeping more than usual.B. The teen is irritable and hyperactive.A child on conventional insulin therapy accidentally received an extra 3 units of regular insulin with the 7 am dose. What initial action should be taken by the nurse? A. Have the child eat some additional carbohydrates now. B. Check the child's serum blood glucose at 1000. C. Phone the physician and report the error. D. Reduce the afternoon insulin dose by 3 units.A. Have the child eat some additional carbohydrates now.The nurse is caring for a 12-year-old who sustained abdominal injuries in an automobile accident. This is postoperative day number 5. Upon assessment the nurse finds; a pale child with cyanotic lips and the following vital signs; 97.6, 100, 28. What would the nurse expect to find when reviewing the child's most recent diagnostic results? A. CT scan showing internal hemorrhage. B. CT scan showing cardiac damage C. CBC showing severe anemia D. CBC showing leukocytosis with left shift.C. CBC showing severe anemia????Pediatric cancers:Most often arise from primitive embryonal (mesodermal) and neuroectodermal tissues • Result in leukemias, lymphomas, sarcomas, or central nervous system (CNS) tumors • Lifestyle factors NOT cause of childhood cancers • Respond well to treatmentCardinal Symptoms of Cancer in ChildrenUnusual mass or swelling • Unexplained paleness & energy loss (leukemia) • Sudden tendency to easy bruising • Persistent, localized pain or limping (oestomelitis or cancer) • Prolonged, unexplained fever or illness • Frequent headaches, often with vomiting • Sudden eye or vision changes (CNS tumor) • Excessive, rapid weight lossDiagnostic evaluation: Childhood cancer-Complete History, review of symptoms, physical exam -Labs (send to ER or hematology) -Imaging studies (tumor some place) -Biopsy (to confirm)Modes of Therapy for cancer-Surgery (more conservative) -Chemotherapy (mainstay tx ) -Radiation therapy (more conservative bc effect brain cells) -Biologic response modifiers (BRMs) (help immune system do their job more effectively.) -Bone marrow transplantationCNS tummorsBrain tumors and neuroblastoma are derived from neural tissue • Account for approximately 20% of childhood cancers • Tumors are difficult to treat, with poor survival ratess/s of brain tumorSigns and symptoms are related to anatomic location, size, and child's age •Presenting clinical signs; headache, vomiting, clumsiness, unsteady gait, cranial nerve neuropathy (blurred or double vision, head tilt), seizures, motor abnormality (clumsiness, unsteady gait), weakness, behavioral or personality changes, FTT (dont grow), irritability, vital sign disturbancesdiagnostic of brain tumor• Neurologic evaluation • MRI, CT, EEG, LP • Histologic diagnosis via surgery (look under microscope) Try to shrink it with radiation before they take it out.Therapeutic Management of brain tumorsDepends on type of tumor • Surgery • Radiotherapy if > age two years (bc problem on the brain) Chemotherapy (intrafecal chemo, introduce in resouvier). Prognosis (depends on type of tumor)Nursing Considerations for after surgery of brain tumor•Postoperative care -Positioning (prevent pressure on operative side/ place on nonoperative side -Reduce ICP • quiet environment •head midline •flat on back until near say they can move • prevent jarring, loud noises, lights, coughing, bearing down, vomiting). -Neck Flexion -NPO -Comfort Measures • H/A • cant overstate them bc neuro exam •lie to forehead •analgesic ATC -Eye Care • drops • dressing/patch Promote return to optimal functioning • hemlet •scalp removed, misshaped skull • PT/OTNeuroblastomaThe most common malignant extracranial solid tumor of childhood. Most common cancer in infancy. • Majority of tumors develop in the adrenal gland or retroperitoneal sympathetic chain (Other sites—head, neck, chest, pelvis) • Anywhere but the skull • group of nerve cells that started pregnancy- earlier we catch it better prognosisNeuroblastoma dxDiagnosis: Radiologic studies, bone marrow evaluation. IVP to evaluate renal involvement (to see if kidneys are effected)s/s neuroblastomaSigns and symptoms depend on the location and stage of the disease • Metastasis may have already occurred before diagnosis is madeTherapeutic Management of Neuroblastoma• Clinical staging to establish treatment plan • Surgery to remove tumor and obtain biopsies • Radiation (to shrink tumor for removal) if older than 2 • Chemotherapy • Bone marrow transplantation or Stem cell rescue • Prognosis depends on spread and age they got it. IF younger than 1 have a better chance of surviving.Hodgkins Disease-More prevalent in 15- to 19-year-olds - Reed Sternberg cells large and abnormal lymphocytes. Neoplastic disease originating in lymphoid system • Often metastasizes to spleen, liver, bone marrow, lungs, and other tissuesNon-Hodgkins Disease- More prevalent in children younger than 14 years - No reed sternbergPath to Hodgkins Disease• M alignant B lymphocytes grow in lymph tissue • Cells multiply, lymph nodes enlarge compressing nearby structures, destroying normal cells and invading other tissues. • Presence of Reed-Sternberg cells (giant, transformed B lymphocytes with one or two nuclei ) characteristic of Hodgkin disease. • Etiology; unknown but has been linked to Epstein Barr Virus • Staged I-IV based on degree of spread • Need Lymph node biopsy for diagnosis and stagings/s of Hodgkins diseasepainless, enlarged lymph nodes, fever, night sweetsTx of Hodgkins diseaseRadiation - Chemotherapy (alone or with radiation) - HSCT - Prognosis depends on stage, tumor bulk, classificationComplications Hodgkins diseaseliver failure, secondary cancers, delayed pubertyNursing consideration Hodgkins diseasesignificant pain in effected nodes following alcohol consumptionNon-Hodgkin Lymphoma (NHL) s/smore common: Clinical appearance - Disease usually diffuse rather than nodular - Cell type undifferentiated or poorly differentiated - Dissemination occurs early, often, and rapidly - Mediastinal involvement and invasion of meninges(in brain is more likely) typicalNHL is classified based onpattern of histologic presentation: - Lymphoblastic - Burkitt or non-Burkitt - Large cellDx: of NHLbiopsyComplications: NHL:metastasis, secondary malignancyTreatement of NHLchemotherapy in three/four stages • Prognosis 90% LT survival rate if localized and not metasis.s/s of NHLpain, lymph node swelling (abdomen), diarrhea or constipation. Cough, dyspnea, orthopnea (problem breathing lying down which means some problems,s the disease is close to airway and compromise), facial edema or venous engorgement may signal mediastinal disease which is an emergency!BONE TUMORSOsteosarcoma and Ewing sarcoma account for 85% of all primary malignant bone tumors in children • Occur more commonly in males, with highest incidence during accelerated growth rate of adolescenceDX Bone tumors- Rule out trauma or infection first - Definitive diagnosis based on radiologic studies (plain films, CT scans, bone scans) and bone biopsy - MRI to evaluate neurovascular and soft tissue extension - Labs—elevated alkaline phosphatase with some bone tumorsOsteogenic SarcomaThe most frequent malignant bone tumor type in children (56%) • Peak incidence—age 15-years • Most primary tumor sites are in the metaphysis of long bones, especially legs • More than 50% occur in distal femur. Other sites—humerus, tibia, pelvis, jaw. •s/s osteogenic sarcomaSigns/symptoms; pain. Limp, decreased ROM •Complications osteogenic sarcomaComplications-metastasis (lungs or other bones), recurrence within 3- yearsTherapeutic Management: osteogenic sarcomaTraditional approach—radical surgical resection or amputation of affected area • Limb-salvage procedures—resection of bone with prosthetic replacement of affected area • Chemotherapy accompanying surgical treatment Try to save the limb or take it all out. They would do radiation then surgery then chemo.Nursing Considerations: osteogenic sarcomaPreoperative preparation is crucial • Support during adjustment to concept of amputation, surgical resection • Body image concerns—issues of adolescents • Pain management - Phantom limb pain Have epidural/nerve block what it feels like to not have it.Ewing SarcomaSecond most common malignant bone tumor in children and adolescents; rare in age more than 30 years • Arises in the marrow, especially in - Femur, tibia, ulna, humerus - Vertebrae, pelvis, scapula, ribs, skull • Highly malignant; 25% metastasis rate (lung, bone, bone marrow)Treatment of Ewing SarcomaRadiation most common first approach • Chemotherapy as adjunct to radiation • IF radiation and chemo doesn't work: Myeloblative chemotherapy followed by stem cell rescue transplant for severe cases. • Surgical resection in some cases—usually able to preserve affected limb if not spread. • Prognosis best if no metastasis at time of diagnosis; distal lesions have best potential for cure • Avoid active play or weight bearing (pathologic fracture) • easy to break bones/fragile. • no running around • no sports, recess, PERhabdomyosarcomaHighly malignant neoplasm originating from undifferentiated mesenchymal cells in muscle, tendon, bursa, and fascia or in fibrous, connective, lymphatic, or vascular tissue • Potential Sites of Rhabdomyosarcoma- head, neck, genitourinary tract, extremitiesRhabdomyosarcoma s/sdepend on site of tumor and compression of adjacent tissues. Many symptoms vague and common to childhood maladies (e.g., earache. Hard to dx.Tumor Treatment and Prognosis of RhabdomyosarcomaHighly malignant and frequent metastasis • Complete removal of tumor if possible • Radiation therapy for most tumors • Chemotherapy to shrink tumor may precede radiation therapy • Long-term chemotherapy (1 to 2 years) • Prognosis; excellent if stage 1 poor if metastasis present at diagnosis Shrink before they take it out then chemo for years.Wilms tumor (Nephroblastoma)A malignant neoplasm of the kidney. Most often affects young children - 40% are genetic. • Three times more common in African-American children • Peak age of diagnosis is 3 years • More frequent in males • Staged from 1-5.Presentation of Wilms tumor (Nephroblastoma)Most common is a well child with an abdominal mass. Less common is abdominal pain, malaise, anorexia, fever, gross hematuria, hypertension. Could be in pain could not be.Treatment of Wilms tumor (Nephroblastoma)- Immediate nephrectomy with removal of all regional lymph nodes. - Chemotherapy with or without radiation therapy depending on stage. • Long-term Survival Take out kidney and surrounding lymph followed by chemoNursing Considerations Wilms Tumor (Nephroblastoma)- Handle & bathe carefully, monitor B/P, I&O * Abdominal palpation may rupture the tumor and spill tumor contents into the peritoneal cavity.Retinoblastoma dx- Cat's eye reflex—most common sign - Strabismus—second most common sign - Red, painful eye, often with glaucoma - Blindness—late signTherapeutic Management: RetinoblastomaTreatment based on stage of tumor at time of diagnosis Early stage unilateral - Irradiation, cryotherapy - Attempt to preserve useful vision in affected eye • Bilateral disease - Attempt to preserve useful vision in least affected eye • Advanced tumor - Enucleation - Chemotherapy—some controversy If involves nerves tissue.Retinoblastoma defineCongenital malignant tumor; arises from the embryonic retinal cellsPrognosis of Retinoblastoma• Survival rate of nearly 90% • Tumor may spontaneously regress • Concern with development of secondary tumors, especially osteogenic sarcomaComplication of Prognosis of RetinoblastomaSpread to brain and opposite eye - Metastasis to lymph nodes, bone, bone marrow, and liver - Secondary tumors, most often sarcomas, occurring in children who have been treated for retinoblastomaTesticular TumorsTumors not common, but those appearing in adolescence are generally malignant • Most common form of cancer in males from ages 15 to 44 yearsTreatment Testicular Tumorsorchiectomy, followed by chemotherapy and/or radiation depending on metastasisNursing considerations of testicular tumorsAdolsences body imagine testicular exam a monthLeukemia Pathostem cells in the bone marrow produce immature WBCs. These cells proliferate rapidly by cloning filling the bone marrow. Leukemia cells enter circulation replacing normal WBCs. The malignant WBCs rapidly fill the bone marrow, replacing stem cells that produce RBCs & platelets, resulting in anemia, thrombocytopenia, neutropenia .Consequences of LeukemiaAnemia from decreased RBCs bc WBC are proliferating and crowding everything out. • Infection from neutropenia (increased WBC but immature so cant fight anything off ) • Bleeding tendencies from decreased platelet production • Spleen, liver, and lymph glands show marked infiltration, enlargement, and fibrosisEtiology of leukemiaNot well understood. -infectious component -genetic component -radiation exposures/s of leukemiaFever • Pallor • Bleeding • Lethargy • Malaise • Anorexia • Large joint & bone pain • Anemia, neutropenia, thrombocytopenia Cardinal Signs of Bone Marrow Failure - Petechiae - Frank Bleeding - Joint/bone Pain (not walking ) - FatigueCardinal signs of bone marrow failure- Petechiae - Frank Bleeding - Joint/bone Pain - FatigueDx: LeukemiaConfirmed by bone marrow biopsyLeukemia Therapeutic ManagementTreatment for presenting symptoms - Combination Chemotherapy aggressive act on cell at different cycle times. - Cranial irradiation (in some cases) - get RBC, Platelets, neutropenic precaution, if have infection: antibiotic.Nursing Care for Child with Cancer• Child and family educated on disease and treatment • Treatment administered on schedule with appropriate drug doses • Side effects of treatment managed • Treatment complications prevented • Child and family coping skills supported • Quality of life during treatment maintained • Child and family adjusted to chronic illness • Growth and development maintained during treatmentChemotherapyRemission induction Consolidation Intensification Maintenances/s of CNS involvement• Headache • Persistent nausea or Vomiting • Irritability • Dizziness • Seizures • Behavior or personality changes • 6 th cranial nerve palsy, abductus- lateral eye movementRemission Induction ChemoRemission Induction; Rapid induction of complete remission, lasts 3-4 weeks, oral steroids & IV chemotherapy. In hospitalConsolidation Chemo• Consolidation (CNS Prophylaxis); Strengthen remission. Introduce CNS prophylaxis. Intrathecal and IV administration Given in hospital Get in across BBB-LP or intrafecal to kill off cells that went into brain.Intensification chemo-destroy remaining or resistant cells. can be given in out pt setting Lower dose 1 month to kill anything elseMaintenance; chemoPrevent relapse, treat metastasis. can be given in out pt setting Every other month lower dose to stop spread.How do you know if the cancer has spread to the CNSHeadache • Persistent nausea or Vomiting • Irritability • Dizziness • Seizures • Behavior or personality changes • 6 th cranial nerve palsy, abductus- lateral eye movement.Chemotherapy kills both normal and cancerous cells! True or falseTrueST chemo effectsImmunosuppression, infection, myelosuppression, nausea, vomiting, vomiting, oral mucositis, and alopeciaBone marrow suppressionAdminister blood products safely (RBC, platlets, anc, maybe antibiotics) • Nursing interventions for anemia (cold, tired, group care, rest periods) • Nursing interventions for thrombocytopenia (soft tooth brush, no straight razor, fall risk sharp corners padded). • Administer colony- stimulating factors • Nursing interventions for neutropenia (screen visitors, hand wash, no live plants. )AlopeciaMost kids adjust well Hats, scarves, wigs may help Hair will grow back Younger dont care, adolescent do care. Teach: color and texture may differ but will grow back 3-6 months after therapycancer N/VAdminister antiemetics like ondansetron (Zofran) before starting chemotherapy and every 3-4 hours until the drugs are cleared. • Avoid strong smells; foods, perfume, smoke, flowers, take lid off food before coming in • Small frequent meals • Cool rather than hot (popsicle, milkshake)/ Administer chemo early in the day. pre/post hydration so it doesn't effect the bladder.Stomatis prevention- Keep oral mucosa & teeth clean - Use antifungal and antibacterial mouth wash QID to prevent opportunistic infections •Stomatitis treatment- Rinse mouth with normal saline - "Magic Mouth Wash" as prescribed ( have to be able to spit) No 1/2 NS, citrus or cracker. -doing this before they ate will allow them to eat more Avoid local anesthetics (Xylocaine, lidocaine) in small children - nystatin to coat the esophagus and bump. -compliance problems -brushing teeth difficultFear & AnxietyKeep the child and family well informed • Partner with the family to create the Plan of Care • Assess level of coping • Be optimistic without giving false hope • Provide support resources • connection with other families. Diversional activities Let them payDeath & DyingEncourage families to share memories • Accept that expressions of grief can be varied and unpredictable. • Think about the siblings • Find your own support • Children's Reactions to Death & Dying - Infants and Toddlers-They don't get it - Preschoolers-They kind of get it feel punished - School age-They get it fear of losing control - Adolescents- They get it and they hate it! withdraw, singled out, suicide pact.Oncologic Emergencies:Hemorrhagic Cystitis • Septic Shock; monitor for circulatory failure. Overwhelmed infection, decreased BP, increased HR,= sepsis • Hypercalcemia from large bone destruction; hydration and phosphate supplements. • Tumor lysis syndromeHemorrhagic Cystitis-sudden onset of blood in the urine combined with bladder pain. -Encourage fluid intake (pre&post hydration to minimize effect on bladder). - void frequently - Administer Mesna (Mesnex) as prescribed to protect bladder mucosa.Tumor Lysis Syndrome (TLS)Tumor cell destruction releases high levels of uric acid, potassium, and phosphates into the blood. Hyperuricemia, Hyponatremia, hypocalcemia, hyperphosphatemia, hyperkalemia, & metaboloc acidosis result. May result in acute renal failure and death.TLS risk factorLarge tumor burden, sensitivity to chemotherapy, and high proliferative rate - High WBC at diagnosiss/s of TLSflank pain, lethargy, nausea and vomiting, oliguria, pruritus, cardiac arrhythmias, impaired renal function, tetany, neurological & mental status changes.Therapy & Nursing Implications TLS:Administration of fluids, beginning before therapy (2-4 times maintenance) • Careful Intake & Output, Daily weight • Urine specific gravity (should remain < 1.010) • Administer electrolytes and allopurinol or urate oxidase (Rasburicase) to reduce conversion of metabolic by-products to uric acid. • Urine pH (should remain 7.0 to 7.5) Send urine with every void that chemo is given • Monitor for tetany and mental status changes. • Collect laboratory specimens.ST adverse effect of Radiation TherapyFatigue, nausea, vomiting, oral mucositis, myelosuppression, and alterations in skin integrity at the site of irradiationLT adverse effect of Radiation TherapyAlterations in growth; hormone dysfunction; hearing and vision alterations; learning problems; cardiac dysfunction; pulmonary fibrosis; hepatic, sexual, or renal dysfunction; osteoporosis; and development of secondary cancerNursing care for radiation therapyloose fitting clothes mild soap and water steroid cream skin moisturizer that is water base/ Protect from sun dry peel bluster. Do not give to kids less than 2.Pain ManagementOral or IV dosing preferred • Appropriate dosage based on body weight • Titrated to increase analgesia and minimize side effects • Use age appropriate pain scalesPromoting Adequate Nutrition for a Child With Cancer• Determine body weight and length/height norm for age. can impact linear growth and teeth. • Determine child's food preferences and provide favorite foods as allowed, including increased-calorie shakes/puddings, milk shakes, calorie dense foods • Administer antiemetics as ordered. Zofran! • Weigh child daily or weekly and measure length/height weekly. • Offer highest-calorie meals when appetite is the greatest. • Administer vitamin and mineral supplements as prescribed. • Administer TPN and intravenous lipids as ordered.Health Promotion for Children with CancerDental care •small teeth, floss, brush teeth regular checks up Immunizations - Vaccines given 2 weeks before or during chemotherapy should be considered inactivated. Child should be revaccinated or receive live virus vaccines 3 months after chemotherapy has stopped. Altered schedule no live vaccines. IVIG temp immunity 11 mo apart from liveFamily Education about cancer• "Cancer quackery" • Communicating about feelings of depression, helplessness, and hopelessness • Home care meds come back look for forever drgs changesCessation of TherapyImportance of follow-up after completion of therapy • Genetic counseling when child reaches adulthood, if appropriate took something that effects them LT. • If possibility of sterility exists, pretreatment sperm banking may be offered to adolescent boys to allow additional adulthood options Scans follow through Long-term sequelae of treatment • Psychosocial, cognitive, emotional, and physical development may be affected by treatment as well as by the disease • Specific effects of radiation therapy (cancer) • Specific effects of chemotherapy (growth and sterility)Developmental Differences in the Genitourinary SystemNephrons are immature and less efficient. Glomerular filtration and absorption rates are low because kidneys of newborns are immature. Decreased ability to concentrate urine and reabsorb amino acids. Loop of Henle is shorter which reduces the ability to reabsorb sodium and water producing very dilute urine for the first 3 months of life (why we dont give free water until 6 mon. ) Hydrogen ion excretion is reduced; acid secretion is lower; and plasma bicarbonate levels are low= more vulnerable to acidosis Not good at diluting of concentrating urine= easy for kids to be dehydrated. Newborn bladder is an abdominal organ rather than pelvic organ until about 3-4 months of age. Infants cannot concentrate urine until about 1-3 months of age Newborn kidneys are less able to adapt to deficiencies and excesses of sodium- vulnerable to fluid volume overload with infusion of isotonic saline and sodium and sodium deficit with vomiting and diarrhea.Signs & Symptoms of Genitourinary Disease NewbornPoor feeding Respiratory distress Poor urinary stream Jaundice Seizures Dehydration Vomiting UremicencephalophySigns & Symptoms of Genitourinary Disease InfantsPoor feeding Pallor Fever Failure to gain weight Persistent diaper rash Seizures Dehydration VomitingSigns & Symptoms of Genitourinary Disease childrenFrequent urination Painful Urination Enlarged kidney or bladder Excessive thirst Foul smelling urine Poor appetite Pallor Enuresis Edema Hematuria Abdominal or back pain Hypertension Tetany Growth FailureNursing Care Management Genitourinary DiseaseAccurate measurement and recording of weight, I & O, & B/P Preparing child and parents for tests Collection of specimens any change in urination get a u/aWhat is the best way to measure urine output in a non-potty trained child: A. Count Diapers B. Weigh diapers C. Daily weight of child D. Insert a Foley catheterWeight diapersU/aAppearance - Clear Color - Yellow Sp. Gr. - 1.010-1.030 pH - 4.5-8.0 Glucose - Negative Protein - Negative Ketones - Negative RBCs- <1-2 WBCs- <1-2 Casts-Moderate clear protein Nitrite- Negative Volume- 1-2ml/kg/hrU/A has glucose positive and ketones positive =DMU/A has positive nitritesinfectionU/a has postive nitratesnormalu/a positive for proteinglymnephritis or nephrotic syndromeDiagnostic Evaluation of Genitourinary DiseaseUrine Culture & Sensitivity Intravenous Pyelography (IVP) Voiding Cystoureterography (VCUG) Renal Ultrasound Kidney, Ureters, and Bladder (KUB) Cystoscopy Renal BiopsyUrine culture and sensitivityCulture what is growing and sensitive what antibiotic will treat it.Intravenous Pyelography (IVP)dye injected iV watch then eat them urinate and look at structures.Voiding Cystoureterography (VCUG)done most insert cathartic and dye and watch where it goes under X-ray and have to be still. Looking for reflux = scarring and damageRenal Ultrasoundpainless till structure of kidneyKidney, Ureters, and Bladder (KUB)Xray of Kidney, ureters and bladder.CystoscopyCamera into urethra to view structures .Renal biopsykidney failure or function issue.Repeated UTI n/iFemale (2-3) Male (1-2) need a VCUGInfants how to collect urineI/O need a really good culture Wee bag cotton ballsGenitourinary Physical AssessmentHealth history Family history- renal disease, hypertension, other renal dysfunction Physical Assessment- ◦ Abnormal rate and depth of respiration ◦ Hypertension ◦ Fever ◦ Growth retardation ◦ Signs of circulatory congestion (which are?) Pulses ◦ Abdominal distention ◦ Early signs of uremic encephalopathy (which are?) LOC, acting normal, alert and oriented ◦ Congenital abnormalities Hypospadias or Epispadias Ear abnormalities (develop at the same time as the kidney is utero)HypospadiasUrethral opening is on the ventral surface of the penis Incidence is unknown Wide range of severity May be a sign of ambiguous genitalia increased risk for UTI (not huge) LT body imagine problem cant pee standing ups/s hypospadiasIncreased risk of urinary tract infections (not much) May interfere with procreation if not fixed Body image disturbance issues (biggest deal )Hypospadias: Therapeutic ManagementSurgical repair in stages Goals of Surgical Correction ◦ enable child to void in a standing position ◦ improve physical appearance of the genitalia ◦ Not circumcised right away ◦ preserve a functionally adequate sex organ Ideal time for surgery, 6‑18 months- old WHY? risk with anesthesia - stent in for a week double diaper. They pee standing up.Hypospadias: Nursing ConsiderationsExamine every newborn male carefully Delay circumcision if there is any question Psychological preparation of parent & childHypospadias Repair: Postoperative CarePressure dressing ◦check tip of penis frequently, do not change dressing ◦ vasukine dressing Catheter/stent care ◦ drain to secondary diaper Teach home care ◦ s/s infection ◦ no straddling (no carrying on trip, no ride on toys) ◦protect stent ◦no sandbox ◦no bubble bathCRYPTORCHIDISMA condition in which one or both testicles fail to descend into the scrotum, often associated with hypospadias.Cryptorchidism: Therapeutic ManagementUltrasongraphy or Surgical Exploration - to locate testes AAP recommends treatment prior to age 1-year Medical treatment; administration of HCG (20% success) Surgical treatment; Orchiopexy b/t 6-24 months Health teaching; increased risk of testicular cancer in 3rd & 4th decade of lifeExstrophy of the BladderA rare congenital malformation resulting from a deficiency in the development of the anterior abdominal wall, symphysis pubis, bladder, and urethra during gestation. Mshaped pelvis- trouble walking and running. Bladder on outside surgery can make resolver but difficult to make a sphinchterExstrophy of the Bladder: s/sBladder exposed and appears to be turned "inside out" Boney pelvis may also be malformed, resulting an opening in pelvic arch Difficulty walking Constant seepage of urine Progressive renal failureExstrophy of the Bladder: Therapeutic ManagementSurgical closure during 1st 3 days of life - followed by second stage repair of bladder neck at ages 3‑4 (establish content bladder control if not possible stoma and I/O cath) Alternative management - creation of a continent urinary reservoir. Increased infection and heat lossExstrophy of the Bladder: Nursing Care PreoperativelyPromote early attachment Protect bladder mucosa ‑ supine position, sterile non-adherent dressing Prevent infection ‑ cleanse with wet cloth & pat dry with soft towel Prevent skin breakdown bc urine seeping on skin Promote comfort - antispasmodics; analgesics; sedatives Promote parental competence in home care and managementExstrophy of the Bladder: Nursing Care PostoperativeFocuses is on preventing infection Prevent Pressure on operative site. Manage bladder spasms with: ◦ Oxybutynin (Ditropan) drug of choice ◦ Belladonna & Opiod (B&O) suppositories. Catheterize the stoma (teach how often) Don't forget latex allergies (increase risk when older) Promote Skin Integrity Family TeachingFood allergies that indicate latex allergyavocado, Potato, kiwiObstructive UropathyAn obstruction at any level of the upper or lower urinary tract Blockage of urine flow produces dilation of the affected kidney (Hydronephrosis) Caused by fibrous band or constipation or tumor or anything interfering with urine getting to where it needs to go cant flow through ureter to bladder and back up into the kidney.Obstructive Uropathy: Clinical ManifestationsRecurrent UTI Incontinence Fever (UTI) Flank or abdominal pain Foul-smelling urine Hematuria (blood) Dysuria Polyuria, polydipsia, urgency Anemia Failure to thrive Nocturnal enuresis ()Obstructive Uropathy: Therapeutic ManagementIF cause is constipation then clean out. If it is fibrous band or tumor Surgical correction, if needed Monitor blood pressure Preparing parents and children for procedures Close observation for post operative complications Protecting and caring for catheters Teaching home care VCUG to determine if there is an obstruction -prep for procedure -CAth -Phsychosocial age -watch after surgeryVesicoureteral Reflux (VUR)Regurgitation of urine from the bladder into the ureters and kidneys. Graded 1-5 Can lead to hypertension, renal insufficiency or failure. Primary reflux is familial and usually outgrown Primary Sphincter isn't the way it should be or or pressure in the uterus. Urine is back up bc incompetence valvular mechanism Secondary as result of an acquired condition such as UTI.If Vesicoureteral reflux is classified as 1/2/3.5 txantibiotic amoxicillinIf Vesicoureteral reflux is classified as 3.5-5 txsurgery bc causing damageAssessment Findings in VURRecurrent UTI in females (2-3) or (1-2 male) Single episode of UTI in males Family history of VURVUR: Diagnosis/TreatmentVoiding Cystourethrogram (VCUG) ◦ Goal; protect kidneys from scarring LT chronic renal failure later in life this undiagnosed. Medical: antibiotics until reflux resolves Surgical: necessary when antibiotics do not work or when reflux is severe. Procedure is reimplantation of uretersVUR: Nursing Considerations, PreopMeasures to prevent infection ◦ culture and sensitivity with follow up Encourage compliance with antibiotics ◦ Teach child to empty bladder completely ◦ Teach good hygiene Have siblings screened bc fam tendency Age appropriate preparation for proceduresVUR: Nursing Considerations, Post-opCare for catheters and stents (no bible bath, double diaper, older closed cath) Administer analgesics for incision pain and antispasmodics for bladder spasms. Teach home care; prophylactic antibiotics for 1- 2 months following surgery. Daily thing at night to hang around longer and urinary stasisHERNIASProtrusion of a portion of an organ or organs though an abnormal opening. ◦ Danger arises when: Protrusion is constricted Circulation is impaired Interference with function or development of other structures Common herniation areas in children include Diaphragmatic Abdominal wall Inguinal CanalCongenital Diaphragmatic Hernia s.sS/S- often is detected in utero S/S after birth- respiratory distress, cyanosis, scaphoid abdomen (sunken, concave), impaired cardiac output(depends on size) Needs immediate medical attention. ◦ Intubation ◦ GI decompression ◦ IV fluids ◦ Will need surgery ◦ sedated ◦ oxygenated ◦ hole fixedAfter birth you can find a congenital diaphragmaticmore and more cyanotic displaced breath sounds L DH hear near heart and on right sideCongenital Diaphragmatic Hernia nursing management: PreopMonitor respiratory and fluid status, acidosis, thermoregulation, cardiac output, sedation, gastric decompressionCongenital Diaphragmatic Hernia nursing management: PostopCont ventilation- intubated and sedated Monitor for acidosis, fluid Status, GI decompression, Thermoregulation, sedation, Pain control, cardiac output, Parental bonding TPN lipids Hard time keeping temp up.Teaching for umbilical herniaparents should be able to push it in and bigger when they cry or poop. Shouldn't feel any structures.Umbilical HerniaOccurs when there is incomplete fusion of the umbilical ring where the umbilical vessels exit the abdominal wall. Affects LBW and preemies > full-term infants Affects African-Americans > Caucasians Size- variable Usually self-resolves in 3-5 years without any medical treatment or home remedies. Surgery sometimes needed for correctionInguinal HerniaThe processis vaginalis persists allowing the abdominal contents to be pushed through the opening. 80% of childhood hernias are Inguinal Hernias Is often asymptomatic and painless When child cries, strains, coughs, or stands for a long time, then the hernia is more visible Needs surgical correctionUTI: Clinical Manifestations: infants◦ Fever ◦ Weight loss ◦ FTT ◦ Vomiting ◦ Diarrhea Check ears and mouth then keep going. Viral infection, U/A, blood cultures, spinal tabUTI: Diagnostic AssessmentUrine screening + Nitrites, + RBCs, + WBCs Urine culture > 100,000 colonies of a single bacteriaUTI: Therapeutic Management: Three goals1) Cure the infection 2) Identify predisposing factors 3) Prevent recurrent infectio nsUTI: Nursing StrategiesAppropriate & careful specimen collection and handling (UA to lab) Ensure adequate administration of antibiotics (teach parent they will feel better but dont stop taking and follow up w culture ) Push Fluids (cranberry juice) Promote comfort (hurt, tyelonol, prophet, heating pad, sitz bath, warm tub bath) Adequate follow-up cultures Teach preventive measures -avoid tub bath if causing problem, not constipated, wiping, empty completely, void after sex, avoid tight jeans.Which of the following should the nurse recommend to help prevent a recurrent UTI in a 6 year old? A. Avoid drinking Colas and caffeine B. Avoid tight fitting pants. C. Avoid use of bubble bath D. Empty the bladder ASAP when the urge is felt. E. All of the aboveAll of the aboveENURESISInvoluntary passage of urine by a child > 5- years old (or past the age when voluntary control should be established) ‑ may be primary or secondary, diurnal or nocturnal ENURESIS. Wont stop to potty on playground sleeping too soundly wet the bed at nightEnuresis: Etiology PRimaryFamilial tendency, decreased bladder capacity (age in years + 2 = oz), developmental or maturational lag, sleep disorder, nocturnal polyuria theory (kidneys fail to concentrate urine during sleep due to insufficient ADH).Enuresis: Etiology SecondaryPsychological factors, abuse, UTI, DiabetesEnuresis: Diagnosis/TreatmentGood History & Physical Urine sample (U/a) Wait for maturation -most children outgrow by age 10 Retention/control exercises Dont do drug therapy unless going to friends house or camp.. Drug therapy: oxybutynin (Ditropan), Imipramine (neurologic side effects) or DDAVP (nasal spray or tablet) Moisture alarm Behavior modification - positive reinforcement is keyEnuresis: Nursing Strategiesdesk Child is not lazy, nor does he wet the bed intentionally. Do not punish child Recommend books Dry ALL Night, Waking Up Dry Limit intake of bladder irritants such as chocolate and caffeine. Limit fluid intake after dinner and make sure he voids before going to bed. Use bed pads and 2 sets of sheets. Use pull ups only on sleep over's. WHY? designed to wick away moisture and we dont want them getting used to it. Teach use of alarms Provide Support & EncouragementRENAL TRAUMACommon between10 & 20 yrs. of age. Usually nonpenetrating or "blunt" force from falls, athletic injuries, MVAs Bleeding not a reliable indicator of severityRenal trauma s/sS/S; hematuria, flank pain, vomiting, abdominal pain.... Just bc blood isn't in urine doesn't mean something isn't wrong.Renal Trauma considerationsNeed observation in the ICU ◦ Can lead to renal rupture. ◦ Urinary Urokinase; released from injured renal tissue; can lead to serious bleeding. Bed rest for 3 days after cessation of gross bleeding Watch VS, LOC, urineWhich of the following would be an important nursing intervention for a child with renal trauma? A. Monitor for signs of shock. B. Observe urine color frequently for increased signs of bleeding. C. Monitor BUN & Creatinine levels every 4 hours. D. Provide distraction by arranging for time in the playroom.A. Monitor for signs of shock.Hemolytic-Uremic Syndrome (HUSCombination of hemolytic anemia and thrombocytopenia that occurs with acute renal failure. Occurs most often in children aged 6-mos to 5-years Child has gotten toxin, bacteria, virus, chemical or drug and it has insulted the kidney- invade and lead to inflammation response and lead to cap wall damage and then occlusion of blood vessel=damage to kidney. RBC break down and occlusive int the kidney.HUS: EtiologyBacterial toxins, chemicals, & viruses Diarrhea Idiopathic Inherited Drug related Malignancies Undercooked meats Raw sushi Orchard apple sider not pasteurizedHUS: PathophysiologyMicrothrombi in the glomerular arterioles lead to occlusion of the glomerular capillary loops and glomerulosclerosis, resulting in renal failure. RBCs & Platelets are damaged as they move through partially occluded blood vessels.Leads to hemolytic anemia and thrombocytopenia. It affects GFR, and decreases urine output, and acute renal failure.HUS: Source of InfectionMost cases caused by verotoxin- producing strain of E. coli . May also be Streptococcus pneumoniae or Shigella dysenteriae Undercooked ground beef accounts for most cases. Also consuming animal feces, unpasteurized dairy and fruit products, fresh vegetables.HUS: Signs/SymptomsVomiting Marked Pallor Oliguria or anuria Edema Fatigue Elevated blood pressure Abdominal pain and tenderness Neurologic changes; irritability, altered LOC, seizures, posturing, or coma U/A positive for blood, protein, pus, WBC Watery diarrhea progresses to hemorrhagic colitis, then to hemolytic anemia and thrombocytopenia. GI bug a week ago and now barely peeing.HUS: ComplicationsChronic Renal Failure Seizures and Coma(increased uremic crystals) Pancreatitis Intussusception (dehyration and excess VIB) Rectal prolapse (diarrhea) Cardiomyopathy Congestive Heart Failure Acute respiratory distress syndromeHUS: Laboratory FindingsUrinalysis: positive for blood, protein, pus, and casts. Serum: ◦ Elevated BUN & creatinine ◦ moderate to severe anemia ◦ mild to severe thrombocytopenia ◦ leukocytosis with left shift ◦ hyponatremia ◦ hyperkalemia ◦ hyperphosphatemiaHUS: Therapeutic ManagementSymptomatic tx: Maintain fluid balance Correct hypertension, acidosis, and electrolyte abnormalities (Iv fluids) Replenish circulating red blood cells Provide dialysis if needed. Body will heal itself Antibiotics won't work bc been too long.HUS: Nursing Considerations:Contact precautions (shed in stool for 12 days) Handwashing, gloves, no hand sanitizer Close attention to fluid volume status (I/O, BP, dialysis, IV fluids ) Family Support Encourage adequate nutrition within dietary restrictions Monitor for bleeding Teach preventative strategies (hand washing before eating after bathroom. Pet goes outside wash, swim diapers, get out if someone poop, fruit and veggies washed, meat cooked well use thermometer 155-160), dont drink out of well, nothing unpasteurized.massive proteinuria(dark and frothy), sudden, rapid weight gain, pleural effusion, decreased urine output, diarrhea, anorexia, pallor, muehrcke lines, BP normal or slightly decreased, frequent infections (lead to peritonitis or junky lungs), fatigue hypoproteinemia(hypoalbumemia), hyperlipidemia, and edema. Mild hematuria.Types of Nephrotic SyndromeIdiopathic; AKA Minimal-Change. ◦80% of the cases. ◦Age of onset 1-8 years Secondary; glomerulonephritis, systemic lupus erythematosus, Henoch- Schonlein purpura, diabetes, led poisoning, cancer Congenital; Rare, Autosomal recessive, death in first year without a transplant.Nephrotic syndromeOccurs as a result of increased glomerular basement membrane permeability, which allows abnormal loss of protein in urine. Fluid shift from vascular into interstitial space = edema. Increase volume in blood vessels, hypovomelia causes decreased renal blood flow and urine output. Increased hepatic synthesis of protein and lips = hyperlipidemia.Nephrosis: DiagnosisUrinalysis (protein, maybe blood, increase lipids) Serology • Renal biopsy - (if steroids dont work for 8 weeks) provides information about the glomerular status and type of nephrotic syndromeWhich of the following serum laboratory results would be consistent with a diagnosis of nephrotic Syndrome? A. Elevated protein, decreased lipids, normal HCT B. Normal C. Decreased protein, elevated lipids, HCT mildly elevatedC. Decreased protein, elevated lipids, HCT mildly elevatedNephrosis: Goals of TherapyReducing protein excretion Reducing tissue fluid retention Preventing infections and other complications such as anemia, infection, poor growth, peritonitis(and palpation, girth measurement, rigid, cellulitis) thrombosis(ambulation, scuds, lovanox), and renal failureNephrosis: Therapeutic ManagementActivity -bed rest during edema - unrestricted during remission Diet - No Added Salt (soup, processed foods) , high protein during edema - regular during remission Drugs - Corticosteroids (6-12wks) immunosuppressant therapy, loop diuretics, salt poor, albumin Usually see repsonse in 7-21 dNephrosis: Nursing ConsiderationsFluid Volume Excess R/T accumulation of fluid in tissues and third spaces. Potential Intravascular Fluid Volume deficit R/T Protein & Fluid Loss. -diuretic and then follow with albumin to replace the fluid to keep BP up. Watch Bp. Potential for Infection: R/T decreased Resistance, Steroid Therapy, & Fluid Overload Potential for impaired Skin Integrity: R/T Edema & Lowered Body Defenses Altered Nutrition: Less than Body Requirements related To Loss of Appetite & Protein Nephrosis: Nursing Considerations Ineffective breathing pattern related to pressure of ascites Body image disturbance related to change in appearance Activity intolerance related to fatigue Altered family processes related to a child with a serious illness Knowledge deficitThe parents of a child with nephrosis ask the nurse why her child needs to receive albumin. The nurse bases his response on understanding that: A. Increases in the serum albumin level will cause fluid to shift from the subcutaneous spaces back into the bloodstream. B. Increases in the serum albumin level will cause fluid to shift from the bloodstream into the subcutaneous spaces.A. Increases in the serum albumin level will cause fluid to shift from the subcutaneous spaces back into the bloodstream.Following administration of IV albumin the nurse should be alert for signs of A. Dehydration B. N/V C. Intravascular Fluid overload D. Increased edemaC. Intravascular Fluid overloadAcute GlomerulonephritisA condition in which immune processes injure the glomeruli. The disease can range from minimal to severe. Most common type = acute post-streptococcal glomerulonephritis (APSG). Followed by strep infection. The injury result in inflammation and kidney are easy and leaks out protein which leads to scarring and damage of the glomeruli and kidney.APSG: Clinical ManifestationsFever (only if infection is active) Lethargy, fatigue , malaise, weakness Headache Anorexia or vomiting Puffy face Urine; discolored (dark and frothy)with decreased volume Edema Weight gain Pallor Flank or abdominal pain Hypertension & signs and symptoms of circulatory overload.APSG: DiagnosisUrinalysis: gross hematuria, mild proteinuria, specific gravity elevated Urine culture: negative Serology; normal electrolytes (watch Potassium), elevated ASO Titer, BUN, creatinine, & sed. rate (ESR), RBC may be lowFrom the following assessment data choose the set that is most consistent with the diagnosis APSG. A. Urine output 1 ml/kg/hr, B/P elevated, elevated serum K. B. Frothy urine, elevated serum protein, normal B/P C. Urine output .6 ml/kg/hr, B/P elevated, normal serum electrolytes. D. Dark urine, B/P below normal, rapid thready pulse.C. Urine output .6 ml/kg/hr, B/P elevated, normal serum electrolytes.APSG: Therapeutic ManagementActivity - bed rest during acute phase (which last 1 -2 weeks) Diet - No added salt , low protein (if BUN is elevated) Control hypertension Antibiotics if evidence of current Strep infection (fever) Isolation from other kids that have infection because these kids are vulnerable to infection while they have APSG Children show us they are getting better by having an increase in UOP. Managed at home BP and good urine output. Manual BP. No sports,Acute renal failureA sudden, often reversible, decline in renal function that results in the accumulation of metabolic toxins as well as fluid and electrolyte imbalance.ARF: PathophysiologySevere reduction in glomerular filtration rate elevated BUN significant reduction in renal blood flow Reversible ARF: Low-output phase high-output phase gradual return to normalARF: Clinical ManifestationsOliguria = UOP < 1cc/kg/h Nausea, vomiting, diarrhea Edema Hypertension Drowsiness, lethargy Circulatory congestion Cardiac arrhythmiasARF complicationsHyponatremia; water intoxication Hyperkalemia Hypertension Anemia Seizures Cardiac FailureARF: Therapeutic Management PreventionTreat underlying cause (stop vancomycin and dehydration replace fluids) Manage fluid & electrolyte disturbances Decrease blood pressure Provide supportive therapy (diuretic followed by albumin) Drugs: Mannitol, albumin, furosemide No dialysis just lots of fluidsARF: Nursing ConsiderationsMonitor V/S & I & 0 Regulate fluid intake eat something high in nutritional value Nutrition Monitor for Complications changes in behavior or seizures Support & Comfort for Child & Family Note; when urine output is restored diuresis may be significant!!A weak irregular pulse, muscle weakness, & abdominal cramping should alert the nurse to : a. Hypocalcemia B. Hyperkalemia C. Dehydration D. Worsening renal failureBWhen caring for a child with ARF who has begun to diures, the nurse should monitor closely for symptoms of: A. Fluid volume deficit B. Hypokalemia and hypercalcemia C. Hyperkalemia D. HyponatremiaAChronic Renal Failure (CRF)A reduction in renal function that occurs over time in response to irreversible damage to the nephrons causing metabolic, biochemical and clinical disturbances ‑ a slow gradual processCRF: PathophysiologyGlomerular death or hypertrophy Accumulation of various biochemical substances in the blood. Retention of waste products, water & sodium Hyperkalemia Metabolic Acidosis Calcium & phosphorus disturbances Anemia Growth disturbancesCRF: EtiologyGlomerular disease (40%); DM, Glomerulonephritis Developmental abnormalities (20%) Hereditary renal disease (1 5%)CRF: Clinical ManifestationEarly Signs: subtle, nonspecific As Disease Progresses: ◦ Loss of normal energy ◦ Decreased appetite ◦ Hypertension ◦ Headache, muscle cramps, nausea ◦ Weight loss or growth retardation ◦ Urine output increased or decreased ◦ Facial edema ◦ Dental defects ◦ Sallow skin ◦ Bone or joint pain ◦ Untreated Uremic Symptoms.....Treatment for CRFsupportive therapy, dialysis, transplantSupportive therapy for CRFDiet ◦ excretory demands on kidney ◦ Minimize metabolic bone disease ◦ Minimize fluid & electrolyte disturbances ◦ Maintain sufficient calories for growth ◦ Need adequate protein (but eat foods high in biologic value) Vitamins Prevention of osteodystrophy ◦ Calcium carbonate ◦ Aluminum hydroxide gel Metabolic acidosis Growth Failure Anemia Hypertension InfectionsRenal TransplantPreferred means of renal replacement therapy in children More compatible with a normal lifestyle than dialysis Living related donor or cadaver donor Drug induced immunosuppression Rejection!!CRF: Nursing ConsiderationFluid & Electrolyte Imbalance Activity unrestricted Nutrition Prevent osteodystrophy Body Image Disturbance Carefully assess for worsening uremia or metabolic acidosis Altered Family processes Fleets Enemas are contraindicated!he nurse is caring for a 5-year-old with APSGN. Which of the following assessment data requires immediate attention? A. A BP of 132/80 B. Gross hematuria C. Severe periorbital edema D. Mild crackles bilaterally in lower lung fieldsAA 3 year old is 24 hours post-op from a hypospadias repair. Which of the following results should be reported to the HCP? A urine specific gravity of 1.012 B. >5 WBC/ microscopic filed on UA C. 4 RBC/ microscopic field on UA D. Urine pH of 6.2BYou are the nurse in the newborn nursery and upon examine notice a newborn male has hypospadias. What it the priority nursing intervention? A.Place indwelling catheter to keep meatus open B. Ensure circumcision is not done C. Measure strict I and O D. Administer pain medsBThe mother of a 6-year-old boy tells you during his WCC that he has been wetting the bed. The mom is very frustrated and says she has tried "everything" including spanking, but nothing is working. What should the nurse do next? A. Call social services B. Tell mom this is normal and that he should not be punished. C. Suggest mom limit fluid intake at night D. Obtain more information about symptoms and psychosocial situationDWhich of the following symptoms can be indicative of a UTI in infants? Select all that apply A. Irritability B. Poor appetite C. Fever D. Urgency E. Crying with urination F. Foul smelling urineABCEFDuring teaching, the nurse should advise the family of a child newly- diagnosed with Graves' disease to: A. Encourage outdoor activities. B. Limit bathing to prevent skin irritation. C. Promote interaction with one friend rather than a group. D. Set the thermostat higher than normal for comfortCA student with type I diabetes mellitus complains of feeling lightheaded. Her blood sugar is 60 mg/dL. Using the 15/15 rule, the nurse should: A. Give 15 ml of juice and repeat the dose in 15 minutes. B. Give 15 grams of carbohydrate and retest the blood sugar in 15- minutes. C. Give 15 grams of carbohydrate and 15 grams of protein. D. Give 15 ounces of juice and retest the blood sugar in 15 minutes.BThe nurse is caring for a newborn infant diagnosed with Congenital Adrenal Hyperplasia (CAH ). Which of the following nursing interventions should be given highest priority. A. Supporting the family. B. Assessing for signs of dehydration. C. Preparing the infant for chromosome testing. D. Referring the family for genetic counseling.BThe nurse working with a teenager who has Phenylketonuria (PKU ) suspects dietary noncompliance from which of the following assessments? A. The teen lists his biggest concern is not being able to eat out with his friends. B. The teen is irritable and hyperactive. C. The teen is withdrawn and refuses to answer questions. D. The teen is sleeping more than usual.BA child on conventional insulin therapy accidentally received an extra 3 units of regular insulin with the 7 am dose. What initial action should be taken by the nurse? A. Have the child eat some additional carbohydrates now. B. Check the child's serum blood glucose at 1000. C. Phone the physician and report the error. D. Reduce the afternoon insulin dose by 3 units.AA child with DM 1 has been admitted in DKA. Which lab results is most consistent with this dx? A. H A1C: 5.5% B. Fasting blood glucose: 124 C. Serum pH: 7.24 D. Potassium level: 3.9CFour sick kids, all with type 1 DM are in the hospital. Which is most at risk of developing hypoglycemia? A. The child with bacterial sepsis B. The child with intussusception C. The child with jaundice D. The child with chickenpoxAThe school nurse has multiple children at school that have DM1. Before which activity should the nurse make sure a child consumes a snack? The child who: A. Sculpts in art class B. Performs experiment in chemistry C. Plays in the band D. Plays on the soccer teamDThe nurse is caring for a child who has just been admitted with a BGS of 624. The mother asks the nurse why they don't give the child insulin first to bring down the glucose. What is the best response by the nurse? A. Insulin would drop the glucose to fast, causing possibly loss of consciousness. B. The high glucose has caused your child to have a lot of water and electrolyte loss. We have to start correcting this first so their blood pressure stays up. C. When glucose levels are this high, insulin isn't effective. D. Your child would require such a large dose of insulin, that most of it would be lost in their urine and not utilized by the body.Bchild with acute gastrointestinal bleeding is admitted to the hospital. The nurse recognizes which of the following as an early symptom of shock? A. Restlessness B. Capillary refill < 2 seconds C. Low grade fever D. Decreased blood pressure?Which of the following is an important nursing consideration when caring for a child with NFTT? A. A select small group of nurses should be assigned to the patient. B. T he routine should be varied so the nurse can get a feel for the child's rhythm. C. F ood should be offered only once so as not to upset the child. D. D evelopmentally appropriate stimulation should be provided during meal times.AWhich of the following OTC drugs might contribute to a child's constipation? Choose all that apply. A. NSAIDS B. Stool Softeners C. Decongestants D. Antacids E. CorticosteroidsABCWhich of the following foods would be a good source of fiber for a 6 year old? A. Bananas B. Captain Crunch Cereal C. Popcorn D. LatticeCWhich of the following long term problems might the nurse anticipate in a child with a cleft lip and palate? A. Faulty dentition B. Hearing loss C. Speech Delay D. All of the aboveDWhich of the following would the nurse expect to find in the history of a child with Hirschsprung's disease A. Diarrhea B. Projectile Vomiting C. Regurgitation of feedings D. Foul smelling ribbon like stoolsDWhich of the following should be given highest priority when caring for an infant with Pyloric Stenosis? A. Nutritional level B. Hydration level C. Preoperative teaching D. PainB1. A 12-year-old w Graves disease. What information should the nurse discuss? - A suggest weight loss - B Encourage attending school - C emphasize that the disease will go into remission - D. Often have big appetite and weight loss.D1. A school aged child come in with sore throat and fever. The child was recently diagnosed with Graves disease and is taking propylthiouracil. What concerns should the nurse have about this child? - A: The child must not be taking her medication -B: The child may have Leukopenia - C The child needs to start an antibiotic - D The child is not getting enough sleepB1. The parents of a child who is on growths hormone replacement therapy are not satisfied with the outcome of treatment, because the child's height is not increasing. The child is still shorter than school peers. What is the most appropriate action of the nurse? - A. Increase the dose of growth hormone to the child. - B. reassure the pt and set realistic expectation - C. Change the route of administration of growth hormone - D. Advise the parents to feed the child with high protein diet.B1. An 8-year old with type 1 DM is complaining of a h/a and dizziness and is visibly sweating. What of the following should the nurse do first? A. Administer glucagon IM B. Offer the child 8oz of milk C. Administer rapid acting insulin lispro D. Offer the child 8 oz of water or calorie free liquid.B1. What changes in the management of the child with type 1 DM should be expected as a result of more exercise? A. increased food intake B. Decreased food intake C. Decreased risk of insulin shock D. Increased risk of hyperglycemiaA1. A parent with a toddler who has ambiguous genitalia ask the nurse how long it will be before the child identifies his or her gender. Which is the best answer? A. A child does not know his or her gender until he or she Is a teen B. A child known his or her gender by the age of 18-30 months C. A child knows from birth what his or her gender is D. A child of 4-6 year is beginning to learn his or her genderB1. A 7-year-old of a child with precocious puberty gets monthly ingestion of Lupron. The child has great fear of needles and pain. What technique would be best to help reduce this child's stress and help manage his pain? A. Apply EMLA to the site 60 minutes before the injection B. Have extra help to help hold the child C. Apply cold to the area prior to the injection. D. Suggest the mom talks to the physician to get the route of medication changed.A1. What is an important nursing intervention for a child w a diagnosis of hyperthyroidism? A. Encourage an increase in physical activity B. Do Pre-operative teaching for a thyroidectomy C. Promote opportunities for periods of rest - D. Do dietary planning to increase calorie intakeC1. The nurse is assigned to a newborn who has a goiter. The nurse's primary concern is which of the following? A. Reassuring the parents that the condition is only temporary and will be treated with medication B. Maintaining a patent airway and preparing for an emergency ventilation. C. Preparing the infant for surgery and initiating pre-operative teaching with parents D. Obtaining detailed history, particularly of medications taken during the mother pregnancyB- The most appropriate nursing diagnosis for a child with Type 1 DM is which of the following? A. Risk for infection r/t reduced body defenses B. Impaired urinary elimination C. Risk for injury r/t medical treatment D. Anticipatory guidanceA1. BS 396 before breakfast 1: 15 above 120 -4g CHO: 1 unit -66g of carbs for breakfast How many units of insulin35How do you know when they are out of DKA?- Eating by mouth - Reaching a normal anion gap 6-121. A 12- year old with Type 2 DM presents with a fever and 2-day history of vomiting. The nurse observes that the child's breath has a fruity odor ad breathing is deep and rapid. What should the nurse do first? A. Offer the child 8 oz of clear non caloric fluid B. Test the child urine for ketones C. prepare for an IV infusion D. Offer 25g of CarbsC1. The school nurse is talking to a 14-year about managing her type 1 DM. Which statement indicates the students understanding of the disease? A. "It really does not matter what type of carbs I eat as long as I take the right amount of insulin." B. "I should probably have a snack right after gym class" C. "I need to cut back on my carb intake after gym class" D. "Losing weight will probably help me decrease my need for insulin"AA 10-year-old has hemophilia and is in the Er after a fall at school. What is the anticipated lab data? A. A Leukocyte count of 15,000 cells/mm B. Platelet count of 75,000 C. PTT of 90 sec (nml is 60-79) D. PT is 9 sec (nml us 11-12.5 sec)CWhat are the most common clinical manifestations of hemophilia? SATA A. Fever B. Excessive bruising C. Nausea and vomiting D. Hemorrhage from any trauma E. Prolonged bleeding from or in the bodyBDEA child is receiving a blood transfusion and begins having dyspnea, hypertension, precordial pain. What is the priority of the nurses action? A. Stop the transfusion B. Give antihypertensive medication A. Give epi once B. Inert Cath and monitor outputAA child with sickle cell in a vaso-occulsive crisis is being admitted to hospital. What is priority? A. Morphine 1 mg IV STAT B. IV D5W ¼ NS at 90mL/hr C. Oxygen at 2L/min D. ABG statBA toddler has been diagnosed with iron-deficiency anemia. Which information should the nurse educate the parents regarding medication administration? A. Add iron to his morning bottle B. Have the child drink OJ after he takes the med C. Administer the med right before meals D. Crush the tablet and mix with apple SauseBA child has been dignosed with leukemia. With which of the following s/s did the child likely present to the healthcare provider? SATA A. Leg pain B. Fever C. Excessive weight gain D. Bruising E. Enlarged lymph nodesABDEA child with hemophilia A fell and injured a knee while playing outside. The knee is swollen and painful. Which of the following measure should be taken to stop the bleed? SATA A. The extremity should be immobilized. B. The extremity should be elevated C. Warm moist compresses should be applied to decrease the pain D. Passive ROM should be administered to the extremity E. Factor VIII should be administeredABEA nurse is caring for a 5 year old with sickle cell vaso-oclusive crisis. Which of the following orders should the nurse question? SATA. A. Position the child for comfort B. Apply hot packs to painful areas C. Give Demerol 25 mg IV q4 hours as needed for pain D. Restrict oral fluids E. Apply oxygen per nasal cannula to keep oxygen sat above 94%CDWhich of the following measure should the nurse teach the parent of a child with hemophilia to do first if the child sustains an injury to a joint causing bleeding? A. Give the child a dose of Tylenol B. Immobilize the joint and elevate the extremity C. Apply heat to the area D. Administer factor per home care protocol.BThe nurse is caring for a child with stomatitis after receiving chemo. Which of the following food items would be best to provide to the child. A. OJ B. Whole grain cracker C. Apple chips D. Milk shakeDThe nurse is caring for a child with ALL whose platelet counts are 20,000/mm. Based on this lab finding, what information should the nurse provide to the child and parent? A. A soft toothbrush should be used for mouthcare B. Isolation precautions should be started immediately C. The child's VS including BP, should be monitored every 4 hours D. All visitors should be discouraged from coming to see the familyAThe parents of a child bring the child to the clinic with a distended abdomen. After examination, a diagnosis of Wilms's tumor? What should you avoid?DONT PALPATEA teen comes to the clinic for possible diagnosis of Hodgkin's disease. The nurse is A. Fever, fatigue, and pain in the joints B. Anorexia with weight loss C. Enlarged, painless and movable lymph nodes in cervical area D. Enlarged liver with JaundiceCWhich of the following should be done to protect the CNS from invasion of malignant cells in a child newly diagnoses with leukemia? A. Cranial and spinal radiation B. IV steroid therapy C. Intrathecal chemo D. High dose IV chemoCThe nurse is caring for a child with ALL. Her WBC are 5000 and segs are 5% and bands are 3%. Which analyses is most appropriate? A. ANC of 400 and the child is neutropenic B. ANC of 800 and child is neutropenic C. ANC of 4000 and child is not neutropenic D. ANC of 5800 and the child is not neutropenicAA child diagnosed with leukemia is receiving allopurinol as part of the treatment plan. The parents ask why their child is receiving this medication. What information about allopurinol. A. Its an antiemetic for N/V B. Its an analgesic and helps with pain C. Reduces serum uric acid D. Medication makes chemo work betterC - - Reduces serum uric acid. When there is lysis of cells from chemotherapy there will be an increase in serum uric acid.Prednisone is given to kids with leukemia. WHY? A. Enhances protein metabolism B. Enhances sodium excretion C. Increase absorption of chemo D. Destroys abnormal lymphocytes.DHow often do we check phenylaline blood levels?Infants need weekly check and older less frequentlyTeaching about CAH how to recognize at births/s hyponatremic dehydration = low serum Na, increased urine output, weight loss, dry mucus membranes. LATER: Recognize a crisis and how to replace the cortisol. Need extra stress dosing.Henoch-Schönlein Purpura (HS Purpura)Etiology unknown, often follows URI • Occurs in ages 6 months to 16 years • Generalized vasculitis of dermal capillaries (inflame) • Extravasation of RBCs, produces petechial skin lesions • Inflammation and hemorrhage in GI tract • May lead to ongoing nephrotic syndrome- glomeral lesions.Acute renal failure etiologyPre-renal: Poor renal perfusion from dehydration , surgical shock, burns. ◦ Intrinsic: diseases, nephrotoxic drugs ◦ Post-renal: obstruction (tumor, obstructive uropathy)Nursing Considerations: RetinoblastomaPostoperative care -sunken cavity Enucleation • Large pressure dressing • Dressing changes (saline rinse, antibiotic ointment)needed. • Family support -Strategies to protect the vision in the unaffected eye. -Siblings need eye exams Q 1-3 weeks for first year of life bc heredity strong chance the other kids could have it. -safety glasses, sunglasses.Manifestations Hemophilia ABleeding tendencies range from mild to severe- can happen spontaneously. •Symptoms may not occur until 6 months of age (teeth bleed usually when it detected). -Mobility leads to injuries from falls and accidents •Hemarthrosis -Bleeding into joint spaces of knee, ankle,elbow, leading to impaired mobility •Ecchymosis •Epistaxis •Bleeding after proceduresLab values for uric acid, cr, Bun in kidsUric Acid (2-5.5) Creatinine (0.3-0.7) BUN (5-18)S&S von willebrand's disease:Easy bruising - Epistaxis - Gingival bleeding - Excessive bleeding with lacerations or surgeries - MenorrhagiaMedical Management SCA Dx: Tx: Prevention:•Diagnosed with Hgb Electrophoresis in the lab on newborn screen. •Palliative Treatment-Erythropoietin or hydroxyuria ( increase Hgb F production), PCN prophylaxis •Transcranial Doppler annually to prevent stroke and assess risk, assess intercranial blood flow •Preventing Crisis - avoid high altitude, dehydration, cold climate, •Crisis Management •Hyper-transfusion to keep hgb >10 and getting frequent transfusion leading to iron build up in skin and may have to chelation therapy. •Bone Marrow TransplantChildren:UTI s/sDysuria ◦ Frequency, urgency, incontinence ◦ Foul smelling urine, possibly hematuria ◦ Abdominal pain ◦ Fevers/sof Ewig sarcomaIntermittent pain that progressively worsens to constant & severe. Swelling and erythema at site. More likely to experience systemic symptoms (fever, spinal cord compression, respiratory distress).Mimic growing pains can be overlooked.APSG: Nursing ConsiderationsFluid Volume Excess: Intravascular, R/T decreased Glomerular Filtration Rate. Daily weight Accurate I&O, urine specific gravity, monitor level of hematuria Monitor blood pressure Monitor for electrolyte imbalance Monitor for signs of cardiopulmonary congestion. Administer diuretics safely Infection prevention Potential For Injury: Renal failure, Encephalopathy, Seizures Assess for signs/symptoms of renal failure Careful neurological evaluation Seizure precautions Knowledge Deficit; Teach parents to: take blood pressure follow the prescribed diet monitor urine output and color