Sickle cell disease/crisis, 282.60
II. Incidence/predisposing factors
A. In the U.S., African Americans are primarily affected. Persons of African, Mediterranean, Southeast Asian, Caribbean, South and Central American, and East Indian origin may also be affected.
B. The sickle cell trait confers a protective effect against Plasmodium falciparum malaria.
C. Presence of the trait is associated with sudden death in military recruits.
D. Sickle cell trait is accompanied by increased risk for development of pneumonia or acute chest syndrome.
E. Mean age of death
1. Hemoglobin SS (HbSS) sickle cell disease
a. Women: 46 years
b. Men: 43 years
2. Sickle cell hemoglobin C (HbSC) disease
a. Women: 63 years
b. Men: 60 years
G. Sickle cell trait, HbAS, is found in about 1 in 12 African Americans.
1. Not part of the sickle cell disease syndrome
2. Life expectancy is the same as in the general population.
H. Pain
1. One third of patients with SS rarely have pain.
2. One third are hospitalized for pain 2 to 6 times a year.
3. One third have six or more pain-related hospitalizations per year.
I. Common neurologic complications in 25% of SS patients include
1. Transient ischemic attacks (TIAs)
2. Cerebral infarction
3. Cerebral hemorrhage
4. Seizures
5. Unexplained coma
III. Subjective findings
A. Symptoms usually relate to increased cell viscosity and subsequent vaso-occlusion that result in the following:
1. Generalized pain in long bones, joints
2. Fever, fatigue, malaise
3. Abdominal pain, nausea, vomiting, decreased appetite
4. Swelling in hands, feet, joints
5. Priapism
B. Depression (particularly in adolescents–due to low self-esteem and delays in maturation)
IV. Physical examination findings
A. Chronic hemolytic anemia
B. Tachycardia
C. Fever
D. Tachypnea
E. Splenomegaly
1. Frequently seen with HbSC
2. Usually nonpalpable with HbSS
F. Hepatomegaly
G. Jaundice (sclera) with darkened urine
H. Cardiomegaly
I. Retinopathy
J. Adolescents may exhibit physical immaturity.
1. Delay in sex organ development and maturation; menarche delayed until ages 15 to 17
2. Delayed adolescent growth in both height and weight
3. Decreased physical endurance compared with peers
V. Laboratory/diagnostic findings
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A. Hemoglobin electrophoresis will confirm the following:
1. Hemoglobin genotype (cellulose acetate and citrate agar gel) for sickle cell disease
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