5. 4. 2. Goal: control bleeding episodes when they occur.
         1. Local measures: apply direct pressure, elevate, apply ice (vasoconstriction), keep immobilized during acute bleeding episodes only. For epistaxis: child should sit up and lean slightly forward.
         2. Systemic measures: administer clotting antihemophilic factor (Factor VIII or DDVAP) via IV infusion. Note: These are blood products, so a transfusion reaction is possible.
         3. Note: cryoprecipitate (a blood product) is no longer used due to risk of transmission of hepatitis and HIV.
      3. Goal: prevent long-term disability related to joint degeneration.
         1. Keep immobilized during period of acute bleeding and for 24-48 hr afterward to allow blood to clot and to prevent dislodging the clot.
         2. Administer prescribed pain medications before physical therapy sessions.
         3. Begin prescribed exercise program, starting with passive range of motion (ROM) and gradually advancing to active ROM, then full exercise program, as tolerated, to maintain maximum joint function.
         4. Avoid: prolonged immobility, braces, splints - which can lead to permanent deformities and loss of mobility.
      4. Goal: promote independence in management of own care.
         1. Encourage child to assume responsibility for choosing safe activities.
         2. Encourage child to attend regular school as much as possible; provide support through school nurse.
         3. Advise child to wear Medic Alert bracelet.
         4. Caution parents to avoid overprotecting child.
         5. Offer child chance to self-limit activities within appropriate limits (parents can offer guidance).
         6. Assist child to cope with life-threatening disorder with no known cure.
      5. Goal: health teaching.
         1. Between 9 and 12 years of age: child can be taught to self-administer clotting factor IV (before this, family can perform).
         2. As child enters adolescence: begin to discuss issues such as realistic vocations, insurance coverage, genetic transmission.
   5. Evaluation/outcome criteria:
      1. Serious injuries are prevented; bleeding is kept to a minimum.
      2. Episodes of bleeding controlled by prompt, effective intervention.
      3. There are no long-term disabilities.
      4. Child is able to manage own care independently, with minimum supervision.

Pulmonary Disorders

1. Cystic fibrosis
   1. Introduction: Cystic fibrosis is a generalized dysfunction of the exocrine glands that produces multisystem involvement. Although the disorder is inherited as an autosomal recessive defect, the basic biochemical defect is unknown. However, its probable cause is an alteration in a protein or an enzyme (e.g., pancre- atic enzyme deficiency). The basic problem is one of thick, sticky, tenacious mucous secretions that obstruct the ducts of the exocrine glands, thus affecting their ability to function. Cystic fibrosis is found in all races and socioeconomic groups, although there is a significantly lower incidence in African Americans. It is a chronic disease with no known cure and guarded prognosis; median age at death in the United States is 27.6 years. Those born in the late 1990s can be expected to survive into their forties with new therapies.