8.3 Sickle Cell Disease

Question 1

Sickle Cell Disease

Answer 1

• Normal hemoglobin is replaced by sickled hemoglobin (sickle shaped) which causes less surface area to carry hemoglobin and oxygen
• Abnormally shaped cells can also get stuck in vessels.

Question 2

Risk Factors

Answer 2

• African Americans most affected

Question 3

SCD Patho

Answer 3

• Sickled red blood cells can obstruct vessels and vasculature which causes inflammation and tissue ischemia. This causes pain.
• There is increased destruction of RBCs. Spleen can enlarge (from sickled RBCs)

S/S
- Results from obstruction from sickle celled, inflammation, tissue ischemia, and red cell destruction.

Question 4

Manifestations

Answer 4

• Hepatomegaly (happens by age 1)
• Pigmented gallstones due to rapid RBC destruction (cholecystectomy)
• Kidneys (hematuria, impaired urine concentration, enuresis (involuntary urination), nephrotic syndrome)

Other Manifestations
- Growth retardation
- Chronic Anemia
- Possible delayed sexual maturation
- Marked susceptibility to sepsis

Question 5

Vaso-Occlusive Crisis

Answer 5

• Pain in involved area
• Extremities
• Abdominal pain (sometimes mistaken for appendicitis)
• Stroke/Visual Disturbances
• Chest (pneumonia like symptoms due to pulmonary occlusion)
• Liver (jaundice, hepatic coma)
• Kidneys (hematuria)
• Genitals (Priapism - painful extended penile erection)

Question 6

SCD Long Term Management

Answer 6

• Heart (cardiomegaly, murmurs)
• Lungs (frequent pulmonary infections or insufficiency
• Kidneys (renal failure)
• Liver (hepatomegaly and cirrhosis)
• Spleen (sequestration of the spleen and blood pooling. More susceptible to infection due to poor spleen function)
• Eyes (Visual disturbances, blindness)
• CNS (seizures and stroke)

Question 7

Management of SCD Crisis

Answer 7

• Can be brought on by stressors (extreme cold/heat, high altitudes, infection, dehydration)
• PAIN AND SUPPORTIVE MANAGEMENT
• Bed rest to prevent further occlusion
• HYDRATION IS ONE OF THE BIGGEST TREATMENT OPTIONS (vasodilation)
• Electrolyte replacement (due to hypoxia in metabolic acidosis)
• Blood replacement to replace sickled RBCs (they might need chelation therapy to reduce excess iron instilled with frequent blood transfusions)
• Antibiotic therapy to prophylactically treat infection
• Hydroxy Urea (a type a chemotherapy that helps prevent formation of sickle shaped RBCs)
• Stem Cell Transplants to replace the bone marrow that produces abnormal RBCs
• PAIN MANAGEMENT IS KEY
• Morphine or dilaudid

Question 8

Manifestations of SCD

Answer 8

• Delayed growth/puberty
• Hand-Foot Syndrome (redness and swelling of palms and feet due to chemotherapy treatment (hydroxyurea)
• Abdominal pain
• Painful episodes
• Enuresis
• Dehydration
• Cerebral Vascular Accident
• Severe Anemia