Cystic Fibrosis Nursing Care Management: Study Guide

Cystic fibrosis is an autosomal recessive disorder of exocrine gland function. A defective CFTR protein leaves secretions thick and sticky, so they plug the lungs, pancreas, and other organs, driving chronic respiratory infection and pancreatic enzyme insufficiency. End-stage lung disease is the principal cause of death, so most of your care centers on airway clearance, infection control, and getting enough calories and enzymes into a child whose body cannot absorb them.

What is Cystic Fibrosis?

Cystic fibrosis (CF) is a disease of exocrine gland function affecting multiple organ systems, chiefly producing chronic respiratory infections, pancreatic enzyme insufficiency, and their complications in untreated patients. It is autosomal recessive, and most gene carriers are asymptomatic. Pulmonary involvement occurs in 90% of patients who survive the neonatal period.

Pathophysiology

CF is caused by defects in the CF gene, which codes for the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride channel regulated by cyclic adenosine monophosphate (cAMP). CFTR mutations disrupt cAMP-regulated chloride transport across epithelial cells on mucosal surfaces. Defective CFTR means decreased chloride secretion and increased reabsorption of sodium and water across those cells. The airway surface fluid thins and mucus loses hydration, so it becomes stickier to bacteria, which promotes infection and inflammation. Secretions in the respiratory tract, pancreas, GI tract, sweat glands, and other exocrine tissues turn viscous and hard to clear.

The lungs are normal in utero, at birth, and after birth until infection and inflammation begin (aside from possibly dilated submucosal gland ducts in the airways). Soon after birth many infants acquire a lung infection that triggers an inflammatory response, infection settles in with a distinctive bacterial flora, and a repeating cycle of infection and neutrophilic inflammation takes hold.

Statistics and Incidences

In the United States the incidence is about 1 in 3,300 in white children and 1 in 16,300 in African American children. Estimated heterozygote frequency in white people is up to 1 in 20, and each child of two heterozygote parents has a 25% chance of having CF.

US prevalence by group: whites of northern European origin have 1 case per 3,200-3,500; hispanics 1 per 9,200-9,500; african americans 1 per 15,000-17,000; and asian americans 1 per 31,000. Compared with males, females with CF show greater deterioration of pulmonary function with age and a younger mean age at death.

Causes

CF is an autosomal recessive disease caused by defects in the CFTR gene, which encodes a chloride channel that also regulates the flow of other ions across the apical surface of epithelial cells.

CFTR mutations produce abnormal cAMP-regulated chloride transport; the failure of chloride conductance and the associated water transport defects yield viscid secretions in the respiratory tract, pancreas, GI tract, sweat glands, and other exocrine tissues.

Environmental factors and modifier genes shape how the disease presents. Neonates with CF and intestinal obstruction most often have abnormalities in 2 or more CFTR modifier genes, while older children develop obstruction mostly from environmental causes, such as a stricture from introduced pancreatic enzymes.

Clinical Manifestations

Age at diagnosis, presentation, severity, and rate of progression all vary widely, and manifestations shift with the patient's age at presentation.

Meconium ileus is the presenting symptom of CF in 5% to 10% of newborns who later develop further manifestations. Depleted or absent pancreatic enzymes before birth impair digestion, so meconium turns viscid and mucilaginous. Intestinal obstruction shows up as bile-stained emesis, a distended abdomen, and absence of stool. A hard, nonproductive chronic cough may be the first sign, later progressing to frequent bronchial infections. A barrel chest and clubbing of the fingers indicate chronic lack of oxygen.

Pancreatic insufficiency brings fat-soluble vitamin deficiency and malabsorption of fats, proteins, and carbohydrates (carbohydrate malabsorption is less severe than that of fats and proteins); these patients show failure to thrive despite an adequate appetite, flatulence or foul-smelling flatus, recurrent abdominal pain, and abdominal distention. Malabsorption produces steatorrhea: frequent, poorly formed, large, bulky, foul-smelling, greasy stools that float. The tears, saliva, and sweat carry abnormally high electrolyte concentrations, and most of these children have enlarged submaxillary salivary glands. Males who reach adulthood are most likely sterile from blockage or absence of the vas deferens or other ducts, and females often have thick cervical secretions that block sperm.

Assessment and Diagnostic Findings

Diagnosis rests on typical pulmonary and GI manifestations, family history, and positive sweat test results.

Noninvasive CFTR analysis recovers DNA from cells obtained by buccal brushing and can establish parental carrier status when a fetus shows sonographic meconium ileus. When only one or neither parent has an identified CF mutation but a previous child has CF, restriction fragment length polymorphism (RFLP) analysis can predict the fetus's status, using genetic material from both parents, the affected sibling, and the fetus. Newborn screening for CF is universally required in the United States. The principal confirmatory test is the sweat chloride test using pilocarpine iontophoresis, currently the only adequately sensitive and specific sweat test. On chest radiography, early changes are hyperinflation and peribronchial thickening, with progressive air trapping and bronchiectasis appearing in the upper lobes. Genotype testing is recommended for those with a positive family history and for couples planning a pregnancy.

Medical Management

Because CF is multisystemic and requires specialist care, treatment and followup at multidisciplinary CF centers is recommended.

Feed a normal diet with extra energy and unrestricted fat; a high-energy, high-fat diet plus supplemental vitamins (especially fat-soluble) and minerals offsets malabsorption and the increased energy demand of chronic inflammation. Regular exercise improves fitness, and upper body work such as canoe paddling can build respiratory muscle endurance. Patients are seen in the CF clinic every 2-3 months to maintain growth and development, keep lung function as near normal as possible, slow the progression of lung disease, monitor GI involvement, and catch and treat complications. Routine vaccinations are indicated, including seasonal influenza.

Pharmacologic Management

Drugs may include pancreatic enzyme supplements, multivitamins (particularly fat-soluble), mucolytics, antibiotics (inhaled, oral, or parenteral), bronchodilators, anti-inflammatory agents, and CFTR potentiators.

Pancreatic enzymes aid digestion when the pancreas fails; current preparations are porcine-derived and contain varying proportions of lipase, amylase, and protease. Vitamins A, D, E, and K are fat soluble, while biotin, folic acid, niacin, pantothenic acid, the B vitamins (B-1, B-2, B-6, B-12), and vitamin C are generally water soluble. Bronchodilators must be evaluated carefully: children with bronchiectasis can have paradoxic bronchodilation in response to beta-adrenergic agents, so test pulmonary function before and after to avoid counterproductive effects. Mucolytics such as dornase alfa, an enzyme that hydrolyzes DNA, improve airway clearance. CFTR potentiators are the first treatment to target the defective CFTR protein itself, the root cause of CF. Antibiotic therapy is linked to greater likelihood of recovery after an acute decline in FEV1.

Nursing Management

Nursing Assessment

Data collection varies with the child's age and the reason for admission. Interview the caregivers for standard information plus respiratory infections, appetite and eating habits, stools, noticeable salty perspiration, any history of bowel obstruction in infancy, and known family history of CF. Gauge the caregiver's knowledge of the condition. Assess vital signs with attention to respirations (cough, breath sounds, barrel chest) and signs of pancreatic involvement. Examine the skin around the rectum for irritation and breakdown from frequent foul stools.

Nursing Diagnosis

• Ineffective airway clearance related to thick, tenacious mucus.
• Ineffective breathing pattern related to tracheobronchial obstruction.
• Risk for infection related to the bacterial growth medium of pulmonary mucus and impaired defenses.
• Imbalanced nutrition, less than body requirements, related to impaired nutrient absorption.
• Anxiety related to hospitalization.
• Compromised family coping related to the child's chronic illness and its demands on caregivers.
• Deficient caregiver knowledge related to illness, treatment, and home care.

Nursing Care Planning and Goals

• Relieve immediate respiratory distress.
• Maintain adequate oxygenation.
• Remain free from infection.
• Improve nutritional status.
• Relieve anxiety.

Nursing Interventions

Improve airway clearance. Watch for respiratory distress, teach the child to cough effectively, examine and document the mucus produced, and push fluids.

Improve breathing. Keep the child in semi-Fowler's position, use pulse oximetry, and maintain oxygen saturation higher than 90%. Give oxygen as ordered, provide mouth care every 2 to 4 hours, perform chest physiotherapy every 2 to 4 hours as ordered, plan therapeutic and diversional activities, and teach exercise to help loosen thick mucus.

Prevent infection. Insist on good handwashing for everyone, teach other hygiene habits, check vital signs every 4 hours, keep people with infections away from the child, and give antibiotics as prescribed.

Maintain nutrition. Greatly increase caloric intake, offer high-calorie, high-protein snacks such as peanut butter and cheese, give pancreatic enzymes with all meals and snacks, encourage salty snacks, report any changes in bowel movements, and weigh and measure the child.

Reduce anxiety. Provide age-appropriate activities to ease anxiety and boredom, encourage the caregiver to stay, and let the child keep familiar toys or mementos from home.

Support the family. Give the family and child room to voice fears, respond with active listening, and provide emotional support throughout the stay.

Evaluation

Goals are met when immediate respiratory distress is relieved, oxygenation is adequate, the child stays free from infection, nutritional status improves, and anxiety eases.

Documentation and Guidelines

• Impact of the condition on personal image and lifestyle.
• Current or recent antibiotic therapy.
• Plan of care.
• Teaching plan.
• Responses to interventions, teaching, and actions performed.
• Attainment or progress toward desired outcomes.
• Modifications to the plan of care.