Lecture 8.1 - Hematologic Disorders Flashcards
What is the lifespan of an RBC with sickle cell anemia?
10-20 days
How is sickle cell passed down?
Autosomal recessive
SCD is usually apparent by which age? What presentations are seen?
6 months
–> Pallor/jaundice and other complications
Usually caught in NB screening
Why is SCD managed with penicillin?
Spleen is not functioning - immunocompromised patients at baseline
What medications are given to manage SCD?
Penicillin prophylaxis
Hydroxyurea
Folic Acid supplementation
Chelation + transfusions
HSCT
Why would someone with SCD be given hydroxyurea?
Stimulates bone marrow to produce HbF instead of HbS
What is the purpose of folic acid for someone with SCD?
Increase RBC production
How do we prevent iron overload in patients with hemolytic anemia receiving blood transfusions?
Chelation therapy
What is the most common reason those with SCD seek care?
Vaso-occlusive pain crisis
A sickling crises results in what?
Ischemia –> Pain
Endothelial damage, local inflammation
What is acute chest syndrome? How is it managed
Lack of perfusion to lungs - they cannot perform gas exchange, infiltration.
–> Usually managed by intubation, maybe exchange transfusion
Why would we do a CXR on a patient with SCD presenting with pain and shortness of breath?
Concern for acute chest syndrome - medical emergency
Will present with pulmonary infiltrate.
What are the psychosocial effects on the child of having SCD and other chronic conditions?
Missed school and activities - loneliness
Poorly managed chronic pain
Effects of stress on parent’s caregiving.
Is chronic pain always present with SCD?
No. Only 23% of children with SCD have chronic pain
What are the psychosocial effects of having a child with SCD and other chronic conditions on the caregiver?
Missed work - financial stress
High parenting stress - elevated risk for disorders/health issues. Poor mental and physical health outcomes - poor mental health outcomes are directly related to worse child outcomes
How does structural racism affect children with SCD’s ability to seek care?
At higher risk of medical non-adherence and mistrust in HCPs
Pain is consistently not managed - lower quality of care results in poor mental and physical health outcomes across the lifespan
Which vitamin improves Iron absorption?
Vit C
What are the three kinds of sickle cell disease?
Sickle Cell Anemia - homozygous form, production of HbS
Sickle Cell-C Disease - Heterozygous variant, production of HbS and HbC (HbC is another abnormal hemoglobin. Cells with it do not sickle, but they break down faster)
Sickle Thalassemia - combination of beta thalassemia and sickle trait.
What is a sequestration crisis?
Pooling of a large amount of blood (in the spleen and sometimes liver), resulting in shock
How is a sickling crisis managed?
Manage Pain
Rest - minimized energy and oxygen expenditure
Oxygen
Hydration + Electrolyte Replacement, correcting blood pH
Blood replacement to treat anemia and reduce viscosity of sickled blood
Antibiotics to treat any infections
What is beta thalassemia? What are nursing roles in supportive care?
Partial or complete deficiency in synthesis of beta chain of Hgb molecule - defective hemoglobin formation resulting is hemolysis
Results in overabundance of erythrocyte production
Nursing Roles:
Promote adherence to transfusion and chelation therapy
Assist with coping and adjustment to living with chronic illness
Observe for complications from multiple transfusions
What is aplastic anemia? What is nursing management?
Bone marrow failure to produce WBCs, Plts, and RBCs (pancytopenia)
Supportive care, manage anemia and immunocompromised status.
Hemophilia A and B are passed on in which way?
X-recessive
What factor is impacted in Hemophilia A?
Clotting factor VIII
Where is bleeding most common with hemophilia?
Subcutaneous, intramuscular, in the joints.
What is the primary therapy for hemophilia?
Concentrated clotting factors from pooled plasma
A synthetic vasopressin increases factor VIII activity - choice for mild hemophilia if appropriate response is observed
What medications can be used to hold a clot in place with hemophilia?
Tranexamic acid and aminocaproic acid
Why should NSAIDS be used with caution in those with hemophilia?
Platelet inhibition
What is immune thrombocytopenia? How is it managed?
Acquired hemorrhagic disorder characterized by thrombocytopenia, excessive bleeding/bruising, normal bone marrow with normal or increased number of immature platelets.
Treatment: Immune globulins or splenectomy
How is DIC managed?
Managing underlying cause
–>May need platelets of fresh frozen plasma to replace lost components.
^IV heparin to inhibit thrombin formation to patients who have not responded to these interventions.
