Primary Nursing Diagnosis

Altered growth and development related to physical disabilities secondary to poor tissue perfusion

OUTCOMES. Child development; Physical maturation; Care-giver patient relationship; Parenting; Psychosocial adjustment

INTERVENTIONS. Nutritional monitoring; Nutrition therapy; Self-responsibility facilitation; Developmental enhancement; Counseling; Caregiver support

U PLANNING AND IMPLEMENTATION Collaborative

Although sickle cell disease cannot be cured, there are many treatment alternatives to prevent exacerbations, limit complications, and manage sickle cell crises. Medical management centers on the treatment of anemia and the prevention of crisis. Families are counseled to avoid the causes of crisis (i.e., dehydration, infection, hypoxia, high altitudes, vigorous exercise, and stress). To prevent aplastic crisis, prophylactic daily doses of penicillin are given to infants beginning when they are about 4 months old.

Sequestration and aplastic crises are treated with transfusions of packed RBCs. A vaso-occlusive crisis is treated with analgesia and increased hydration. Pain levels should be assessed

Pharmacologic Highlights

Medication or Drug Class

Dosage

Description

Rationale

Hydroxyurea

Initial: 10 mg/kg

Antimetabolite

Inhibits production of Hb F,

(Hydrea)

After 6 wk: 15 mg/kg

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