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NUR 425 Sickle Cell
Terms in this set (89)
no known cause
Idiopathic Thrombocytopenia Purpura
acute or chronic condition in which the body thinks platelets are attacking the body and form antibodies to it; causes bleeding, bruising, petechiae
abdomen, trunk of the body, face, other weird areas
where does bruising and purpura occur in children with idiopathic thrombocytopenia purpura?
bleeding disorder that is a genetic autosomal recessive disorder in which factor 8/9 is not produced leading to a lack of clotting factors; these individuals require a large number of blood transfusions throughout their lives
joints of arms and legs
where does bruising and purpura occur in children with hemophilia?
sickle cell disease
A hereditary disease that causes normal, round red blood cells to become oblong, or sickle shaped, compromising blood flow and oxygenation
-x-linked recessive (both parents have the trait to pass onto the kid who gets the disease)
-bleeding into a joint, most common type of internal bleeding
RICE (rest, ice, compression, elevation)
what is the treatment for hemophilia bleeding into a joint?
normal life but with a tendency to bleed
what is the prognosis for an individual with hemophilia?
factor 8 deficiency
what deficiency causes hemophilia A/classic form of hemophilia?
factor 9 deficiency
what deficiency causes hemophilia B/Christmas disease?
These patients give themselves the deficient factor (either factor 8 or 9) through an IV 3x/week for clotting properties and primary/secondary bleeding prophylaxis
what do patients with hemophilia need in order to decrease bleeding time?
factor 8/classic hemophilia
compromises 80% of all hemophilia cases
term to describe bleeding into a joint space, which is the most common type of internal bleeding
bony changes and crippling deformities such as flexion contractors and joint fixation
_____________ and ____________ occur after repeated hemarthrosis bleeding episodes in hemophiliacs over several years
warmth, swelling, redness, pain, loss of movement
5 Sx of hemarthrosis
________________ is periodic factor replacement for children with severe hemophilia to prevent bleeding complications, including arthropathy and spontaneous life threatening bleeding events.
an effective treatment in preventing bleeding complications baby administering periodic factor replacement; involves the infusion of factor 8 concentrate on a regular basis before the onset of joint damage
what is primary prophylaxis for hemophilia?
infusion of factor 8 concentrate on a regular basis after the child experienced his or her first joint bleed
what is secondary prophylaxis for hemophilia?
a synthetic form of vasopressin (DDVAP) increases plasma factor 8 activity and is the treatment of choice for mild hemophilia, but is not effective in the treatment of severe hemophilia A
what is vasopressin and why is it used for hemophilia?
the platelets are unable to stick together and bleeding results
what will happen if an individual does not have factor 8/has hemophilia?
acute in children
chronic in adults
Idiopathic Thrombocytopenia Purpura (ITP) is a __________ condition in children and a ____________ condition in adults.
an acquired auto immune disorder that sometimes arises after a virus or bacteria in which anti platelet antibodies causes a decrease in the life span of platelets and the spleen recognizes platelets as foreign and destroys them
describe the pathophysiology of idiopathic thrombocytopenia purpura
lupus, leukemia, lymphoma
Several tests are done to rule out other disorders with which thrombocytopenia is a manifestation. What are 3 other diseases in which thrombocytopenia may be resent besides ITP?
2. IV immune globulin (IVIG)/Anti-D antibody
Treatments for ITP is supportive as the disease is self-limiting. What 2 medications can be used for the acute form of ITP? What is the treatment for chronic ITP?
causes anemia due to the destruction of RBCs but the trade off is prolonged survival or platelets; needs only one dose and is cheaper that IVIG
what is the pro and con of using anti-D antibodies for treatment of acute ITP?
Immunoglobulin G is a type of antibody. It represents about 75% of the antibodies in our blood. It binds to pathogens, such as viruses and bacteria to protect the body from infection. So IVIG Contains IgG antibodies and is used to treat various autoimmune, infectious, and idiopathic diseases. The use of IVIG in ITP targets the auto antibodies that are destroying the platelets and helps to slow down the body's immune system which is making these autoantibodies.
Explain how IV immune globulin (IVIG) can treat ITP
potential of bleeding with activity or harm
why is activity restricted in ITP patients?
giving platelets does not help because the body destroy them
why is giving platelets to ITP patients not a treatment?
In ITP, the antibody-coated platelets are often removed from circulation by the spleen. Theoretically, if the spleen is removed, the platelets will remain in the blood stream. The spleen can also be the site of antibody production. Therefore removing the spleen may reduce the amount of anti-platelet antibodies in addition to removing the antibody-coated platelets. Since the spleen is responsible for making antibodies, filtering the blood, and removing bacteria, those without a spleen have an impaired immune system.
Explain how a splenectomy can treat chronic ITP
idiopathic thrombocytopenia purpura (ITP)
the most common thrombocytopenia of childhood but occurs in adults too
3. normal functioning bone marrow, possibly increased # of immature platelets
3 manifestations of ITP
sickle cell anemia
most severe type of sickle cell disease
sickle cell anemia
autosomal recessive disorder in which there is an abnormal form of hemoglobin (HgS) within the RBCs that turn into a sickle shape, are very sticky, and clump together
abnormal hemoglobin name found in sickle cell anemia
fetal hemoglobin name
Fetal hemoglobin (HgF) is completely replaced by __-__ months of age.
sticky and thick or viscous and inflexible
What are some properties that sickling give to a normal RBC?
2. severe anemia
3 manifestations of sickle cell anemia in the first years of life
The spleen dies because sickle cells filtered through the spleen get stuck and clot, causing many little infarcts of the splenic tissue, leading to fibrosis and tissue death. This can lead to infection that causes more sickled cells and creates a vicious cycle. Multiple infarcts result after repeated sickling episodes in the spleen. After a point, the spleen cannot regenerate, so it becomes scarred and atrophied resulting in autosplenectomy.
Repeated sickling episodes of RBCs in sickle cell anemia cause autosplenectomy. Explain what this is.
40-60's because of infection
Individuals with sickle cell anemia have a lifespan into the ___-___ and the main cause of death from this condition is _________.
-RBCs sickle but have a normal lifespan
-1/12 African American women have the sickle cell trait
-Provides resistance to Malaria
Name (4) characteristics of sickle cell trait (NOT anemia)
chronic disease with acute exacerbations
Is sickle cell disease acute or chronic?
African and Mediterranean ancestry
2 ethnic groups in which sickle cell disease is prominent
both parents carry the trait and the child must get both recessive traits to have the disease
what does autosomal recessive mean?
adult hemoglobin name
fetal hemoglobin (HgF), 60-80%, adult hemoglobin (HgA), 6-12 months, <1%
__________________ is the major hemoglobin present during gestation; it constitutes approximately ___-___% of total hemoglobin in the full-term newborn. It is almost completely replaced by _____________ by approximately ___-___ months of age, and it amounts to less than ___% percent of total hemoglobin in the adult.
fetal hemoglobin (HgF), adult hemoglobin (HgA), sickle cell hemoglobin (HgS), sickle cell hemoglobin (HgS)
If an infant/child has sickle cell anemia, once the ________ is used up, the child's blood contains _________ and _________. __________ is the culprit. ___________ can sickle and unsickle under appropriate conditons.
manifestation of sickle cell anemia that is caused by an infarction of the bones in the hands and feet causing pain and swelling
Those who are resistant to malaria have the SC trait. SC mutation compromises the parasites' ability to reproduce. But with this comes a Higher incidence of SCD in those areas with malaria also.
describe relationship between sickle cell trait and malaria
normal RBC lifespan
normal sickle celled RBC lifespan
-decreased PO2 in blood (hypoxemia causes more sickling of cells, more HgS present)
-increased plasma osmolality (dehydration)
-decreased blood volume
-low temperatures (vasoconstriction)
could also sickle spontaneously
6 stressors that can cause the sickling of RBCs
HgbS reacts to deoxygenation and dehydration by solidifying and stretching the RBC into a sickle shape. The degree of deoxygenation that is need to produce sickling varies with the % of Hgb S present.
1. As sickling begins, blood flow slows which promotes hypoxemia and perpetuates sickling
2. The decrease in pH decreases the hemoglobin's affinity for oxygen so less oxygen is transported
3. So as less oxygen is taken up by the hemoglobin in the lung, PO2 drops promoting sickling further.
4. This all is a great example of positive feedback making things worse!
5. Once sickling begins, it continues until the PO2 returns to normal and then it ceases spontaneously.
6. Sickled cells regain normal shape after homeostasis is achieved.
How does HgS respond to deoxygenation and cause sickling?
no, most sickled cells regain normal shape after deoxygenation and rehydration
Is the sickling of cells permanent?
Through decreasing the stressor. The stressor could be infection, dehydration, exposure to cold temperatures, correcting the acidosis. So the stopping of the sickling occurs when the oxygen tension or the amount of oxygen in the blood returns to normal. It seems that giving oxygen would help but it doesn't. Homeostasis is regained through reoxygenation and rehydration.
How does a cell return to normal after sickling has occurred?
acute symptom of sickle cell crisis in which sickled cells clump together and are static in small capillaries and results in ischemia, infarction, fever, pain, swelling, CVA, and painful episodes
- pain management
- fluids; better to over hydrate the patient because it allows the cells to flow around with more ease vs. dehydration when they are more likely to get stuck and clump together
- use of warm heat
(4) treatment options for sickle cell vaso-occlusive crisis
acute chest syndrome
acute symptom of sickle cell crisis that is an emergency similar to pneumonia; there is a potential for pulmonary embolism and is the cause of death in 25% of those with sickle cell disease; causes respiratory distress due to sickling of pulmonary vasculature
acute chest syndrome is the cause of death in __% of those with sickle cell disease
acute symptom of sickle cell crisis in which sickled cells are hemolyzed in the spleen, causing life threatening pooling of blood in the spleen and infrequently the liver
splenomegaly, severe anemia, decreased blood volume, shock
Sx of sequestration sickle cell crisis
controversial because removing the spleen makes you more prone to infections
What is the treatment for sequestration sickle cell crisis? Why is this treatment controversial?
acute symptom of sickle cell crisis due to a virus causing a decreased production of RBCs in the bone marrow (anemia) and their short lives and decreased reticulocyte count (new immature RBC)
name for new immature RBCs
How can aplastic sickle cell crisis be prevented since it is caused by a virus?
What is the treatment for aplastic sickle cell crisis?
pallor, extreme fatigue, SOB, anemia, pale, lethargic
Sx of aplastic sickle cell crisis
Lab that indicates the presence of aplastic sickle cell crisis
acute symptom of sickle cell crisis in which there is an acceleration of RBC destruction
persistent and painful erection of the penis due to obstruction of penile veins in a vast-occlusive sickle cell crisis
chronic infusion therapy
-they are also candidates for stem cell transplant
What is the current treatment for sickle cell diseased children who have experienced a stroke?
Repeat CVAs causing progressively greater brain damage occur in approximately __% of untreated children who have experienced one stroke because of sickle cell disease.
every 3-4 weeks
how often are blood transfusions given to children with sickle cell disease in order to prevent stroke?
chelation therapy may be started
What treatment should be given to reduce iron overload from chronic transfusion therapy when treating sickle cell disease?
chest pain, fever, cough, tachypnea, wheezing, hypoxia, death
Sx of acute chest syndrome sickle cell crisis
Heparin does not help. Heparin prevents certain clotting factors from working, thus reducing blood clots. These 'clots' that form in SCA are due to abnormal hgb, not a clotting factor issue. However, you may see heparin used if the patient has acute chest syndrome.
Does Heparin help in sickle cell anemia crises? Why or why not?
acute chest syndrome
In which single sickle cell crisis can heparin ONLY be used to disintegrate clot formation?
-avoid hypoxia (avoid use of cold packs for injuries)
-hydroxyruea: increases the production of fetal HgB, reduces adhesion and improves hydration of the sickled cells
-folic acid: increases production of RBCs
tactics for preventing exacerbations of sickle cell disease
increases the production of fetal HgB, reduces adhesion and improves hydration of the sickled cells
increases production of RBCs
No! The oxygen cannot reach the sickled RBCs in the clogged vessels. Oxygen should only be used if the patient is actually hypoxic in respiratory distress. Severe hypoxia must be avoided because it causes massive systemic sickling that can be fatal. Prolonged oxygen therapy can also depress bone marrow, further aggravating anemia.
Should oxygen should be used to prevent or treat exacerbations of sickle cell disease? Why or why not?
only bone marrow transplant or stem cell transplant which permanently alters the hemoglobin genotype
What is the cure for sickle cell disease?
cold packs cause vasoconstriction and occlusion
why should cold packs not be used to treat injuries in individuals with sickle cell disease?
cold causes vasoconstriction which causes hypoxia
Why should sudden changes in altitude or extremes in weather be avoided by individuals with sickle cell disease?
If a female with SCD has a stem cell transplant, she can still give SCD to offspring due to the presence of the defective gene in her eggs. A male who has SCD and a transplant has a lesser chance of passing SCD to his offspring as the sperm are constantly being made, therefore there is more genetic variation.
Why does a female have a greater chance than males of passing the sickle cell disease gene onto their offspring after a stem cell transplant?
type and screen
blood test used to find blood type and it is stored in the computer for future use when identifying blood products for the patient
type and cross
blood test used to find blood type and it is stored in the computer for immediate use as blood is pulled and reserved for use immediately after blood type is identified
which lab test can be used to rule out aplastic sickle cell anemia crisis?
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