Question

Map a nursing care plan/clinical pathway for a patient with a specific genetic disorder (e.g., sickle cell disease, hemophilia, cystic fibrosis, Huntington disease). Prepare to discuss your care plan and rationales following a minimum of three nursing diagnosis for the disease specified.

Answer 1

Cystic fibrosis: It is an autosomal recessive disorder that is caused due to mutation in the gene.
Nursing care plan:
1. Nursing diagnosis: impaired gas exchange related to airway obstruction by nasal obstruction evidenced by cough, dysnea.
Expected outcome: client will maintain optimal gas exchange.
Nursing intervention:
Monitor respiratory and heart rate. Rationale: indicates early hypoxia.
Provide adequate rest during the day with minimal sleep interruption during night. Rationale: to avoid fatigue.
Place client in semi fowlers position. Rationale: It promotes lung expansion and decreases airway collapse.
Administer oxygen therapy if needed. Rationale: It decreases the work for breathing and calorie expenditure.
2. Nursing diagnosis: Ineffective airway clearance related to increased mucopurulent production as evidenced by an ineffective cough.
Expected outcome: Client will maintain clear, open airway.
Nursing intervention:
Assess cough for effectiveness. Rationale: gives a baseline data.
Auscultate lungs for adventitious sounds. Rationale: It signifies an ongoing infection and inflammation.
Provide opportunity for exercise and physical therapy. Rationale: It helps in loosening the secretions for effective breathing.
Encourage frequent and effective cough. Rationale: to clear the mucus.
Administer bronchodilators and mycolytics. Rationale: decrease viscosity of the mucus.
3. Nursing diagnosis: Imbalanced nutrition less than body requirements related to increased caloric needs as evidenced by weight loss.
Expected outcome: Client will maintain adequate nutritional status.
Nursing intervention:
Assess abdomen for bloating; fullness, bowel sounds, stool patterns. Rationale: It provides a baseline data for malabsorption.
Encourage liberal hydration and high fiber intake. Rationale: constipation can cause increased in mucus production and dehydration.
Encourage high protein, high calorie diet. Rationale: Client with cystic fibrosis needs increased calorie of 1.2 to 1.5 of the recommended amounts.
Administer pancreatic enzymes. Rationale: supplement to digest foods.