Flashcards in Sickle Cell Disease Deck (32):
What is Sickle Cell Trait?
Most common form of sickle cell disease; heterozygous; carrier, rarely has symptoms.
What is Sickle Cell Anemia?
Homozygous condition; has disease. Subject to sickle cell crisis.
What is Sickle Cell Syndromes?
Second most frequent scd in african americans, RBC has a C shape but is NOT sickled.
What is the incidence of SCA?
Predominant in black people and hispanics, others can have it; all babies are screened at birth.
What is Sickle Cell said to be from?
A genetic mutation to survive malaria and was passed down since those that had it survived.
What do heterozygous individuals have?
Have the sickle cell trait.
What to homozygous individuals have?
Have sickle cell anemia.
Why are newborns with sickle cell asymptomatic at birth?
Because of the HbF which is in 60-80% circulation.
How long are children protected with HbF?
One year- then symptoms of SCA increase with complication risks.
What is the cause of symptoms of SCA?
Obstructions are caused by sickled or crescent shaped RBC's and there is an increased RBC destruction.
How long to sickled RBCs last compared to normal RBCs?
Regular- 120 days
Sickled- 60 days
What are clinical manifestations of SCA?
Obstruction, micro-circulation --> casoocclusion, leads to decreased blood to tissues, local hypoxia, ischemia and infarctions and strokes.
What are symptoms of an acute episode of sickle cell disease?
PAIN, pooling of blood, anemia, hyperhemolytic, CVA, chest syndrome, infection, fatigue, swelling of hands and feet, back pain, jaundice, SOB, skin rask
What are signs and symptoms of complications from SCD and SCA?
Chest syndrome, CVA, splenic sequestration, priapism
What temperature/fever is a medical emergency with SCA?
What is a hemoglobin baseline for a SCA patient?
7-10 mm/dl (normal is 11-15)
Is the pH usually up or down in SCA patients?
What labs are heightened for SCA patients?
Bilirubin, reticular count (RBC production), and erythrocyte sedimentation
How do you diagnose SCD?
Newborn screening, sickle turbidity test, hemoglobin electrophoresis, CBCs, Transcranial doppler to monitor intracranial blood flow.
What do you do to medically manage SCD?
Rest, hydrate, electrolyte replacement, analgesics, blood replacement, antibiotics, hemophresis.
How are SCA symptoms treated?
Analgesics for pain- opiods for serious pain; oxygen, blood transfusions, splenectomy
What medication is NOT recommended for SCA patients and why?
Meperidine (Demarol)- It is a CNS stimulant and can cause seizures
What are some preventative measures for crisises?
Routine immunizations, influenza vaccine, pneumococcal and meningococcal vaccines, oral penicillin prophylaxis
What are some nursing assessments for SCA patients?
Pain, analgesia effects, crisis with crisis interventions, protocols, neuro and vascular
What are symptoms of chest syndrome?
Severe chest, back, or abdominal pain, Fever over 101.5, congestion, cough, dyspnea, tachypnea, retractions, hypoxia
What are symptoms of CVA?
severe, unrelieved headache, jerking of extremities and face, seizures, strange behavior, inability to move extremity, unsteady gait, stuttering, weakness, changes of vision.
How can you prevent a CVA?
Red cell transfusion to make less anemic, maintain hydration, trancranial doppler to test speed of blood flow, screening for SCD
What is the leading cause of death in SCD?
Acute chest syndrome
How do you treat acute chest syndrome?
transfusion, bronchodilators, mechanical ventilation, antibiotics
What do you educate the patient and family on?
Support, consequences from complications, early interventions, signs and symptoms, maintain hydration, know analgesia, alternative methods, help with anxiety
What are some home care guidelines for children with SCD?
Fluid intake, adequate rest periods, avoid cold, avoid infection, avoid sun exposure, maintain body temp, administer penicillin, avoid aspirin