Sickle Cell Disease

Question 1

What is Sickle Cell Trait?

Answer 1

Most common form of sickle cell disease; heterozygous; carrier, rarely has symptoms.

Question 2

What is Sickle Cell Anemia?

Answer 2

Homozygous condition; has disease. Subject to sickle cell crisis.

Question 3

What is Sickle Cell Syndromes?

Answer 3

Second most frequent scd in african americans, RBC has a C shape but is NOT sickled.

Question 4

What is the incidence of SCA?

Answer 4

Predominant in black people and hispanics, others can have it; all babies are screened at birth.

Question 5

What is Sickle Cell said to be from?

Answer 5

A genetic mutation to survive malaria and was passed down since those that had it survived.

Question 6

What do heterozygous individuals have?

Answer 6

Have the sickle cell trait.

Question 7

What to homozygous individuals have?

Answer 7

Have sickle cell anemia.

Question 8

Why are newborns with sickle cell asymptomatic at birth?

Answer 8

Because of the HbF which is in 60-80% circulation.

Question 9

How long are children protected with HbF?

Answer 9

One year- then symptoms of SCA increase with complication risks.

Question 10

What is the cause of symptoms of SCA?

Answer 10

Obstructions are caused by sickled or crescent shaped RBC’s and there is an increased RBC destruction.

Question 11

How long to sickled RBCs last compared to normal RBCs?

Answer 11

Regular- 120 days

Sickled- 60 days

Question 12

What are clinical manifestations of SCA?

Answer 12

Obstruction, micro-circulation –> casoocclusion, leads to decreased blood to tissues, local hypoxia, ischemia and infarctions and strokes.

Question 13

What are symptoms of an acute episode of sickle cell disease?

Answer 13

PAIN, pooling of blood, anemia, hyperhemolytic, CVA, chest syndrome, infection, fatigue, swelling of hands and feet, back pain, jaundice, SOB, skin rask

Question 14

What are signs and symptoms of complications from SCD and SCA?

Answer 14

Chest syndrome, CVA, splenic sequestration, priapism

Question 15

What temperature/fever is a medical emergency with SCA?

Answer 15

Over 101F

Question 16

What is a hemoglobin baseline for a SCA patient?

Answer 16

7-10 mm/dl (normal is 11-15)

Question 17

Is the pH usually up or down in SCA patients?

Answer 17

Down

Question 18

What labs are heightened for SCA patients?

Answer 18

Bilirubin, reticular count (RBC production), and erythrocyte sedimentation

Question 19

How do you diagnose SCD?

Answer 19

Newborn screening, sickle turbidity test, hemoglobin electrophoresis, CBCs, Transcranial doppler to monitor intracranial blood flow.

Question 20

What do you do to medically manage SCD?

Answer 20

Rest, hydrate, electrolyte replacement, analgesics, blood replacement, antibiotics, hemophresis.

Question 21

How are SCA symptoms treated?

Answer 21

Analgesics for pain- opiods for serious pain; oxygen, blood transfusions, splenectomy

Question 22

What medication is NOT recommended for SCA patients and why?

Answer 22

Meperidine (Demarol)- It is a CNS stimulant and can cause seizures

Question 23

What are some preventative measures for crisises?

Answer 23

Routine immunizations, influenza vaccine, pneumococcal and meningococcal vaccines, oral penicillin prophylaxis

Question 24

What are some nursing assessments for SCA patients?

Answer 24

Pain, analgesia effects, crisis with crisis interventions, protocols, neuro and vascular

Question 25

What are symptoms of chest syndrome?

Answer 25

Severe chest, back, or abdominal pain, Fever over 101.5, congestion, cough, dyspnea, tachypnea, retractions, hypoxia

Question 26

What are symptoms of CVA?

Answer 26

severe, unrelieved headache, jerking of extremities and face, seizures, strange behavior, inability to move extremity, unsteady gait, stuttering, weakness, changes of vision.

Question 27

How can you prevent a CVA?

Answer 27

Red cell transfusion to make less anemic, maintain hydration, trancranial doppler to test speed of blood flow, screening for SCD

Question 28

What is the leading cause of death in SCD?

Answer 28

Acute chest syndrome

Question 29

How do you treat acute chest syndrome?

Answer 29

transfusion, bronchodilators, mechanical ventilation, antibiotics

Question 30

What do you educate the patient and family on?

Answer 30

Support, consequences from complications, early interventions, signs and symptoms, maintain hydration, know analgesia, alternative methods, help with anxiety

Question 31

What are some home care guidelines for children with SCD?

Answer 31

Fluid intake, adequate rest periods, avoid cold, avoid infection, avoid sun exposure, maintain body temp, administer penicillin, avoid aspirin

Question 32

What are some considerations for pregnant women and SCD?

Answer 32

Keep hgb 6-8, hypotonic IV to decrease extracellular fluid load, maintain hydration and oxygen during delivery.