Cystic Fibrosis

What are common signs and symptoms reported during the health history in an undiagnosed child with cystic fibrosis?

Salty taste to the child's skin, meconium ileus, abdominal pain, bulky stools, poor weight gain, chronic cough, upper or lower respiratory infections

What questions should be included in the health history for a child known to have cystic fibrosis?

Respiratory status, appetite and weight gain, activity tolerance, increased need for medications, presence of fever, presence of bone pain, any other changes in physical state or medication regimen

What is the recommended treatment for mobilizing secretions from the lungs in cystic fibrosis?

Chest physiotherapy (CPT) with postural drainage.

What medication is given daily to decrease sputum viscosity in cystic fibrosis?

Recombinant human DNase (Pulmozyme).

How many people in the US are affected by cystic fibrosis?

30,000 children and adults.

What is the median age for survival in cystic fibrosis?

39.3 years.

How can growth be maintained in a child with cystic fibrosis?

Administration of pancreatic enzyme supplements, well-balanced high-calorie high-protein diet, commercially available nutritional formulas and shakes, breastfeeding with enzyme administration, fortification of breast milk or supplementation with high-calorie formulas, vitamin supplementation, gavage feedings or total parenteral nutrition as prescribed

What type of antibiotics are often prescribed for cystic fibrosis?

Aerosolized antibiotics.

What are the clinical manifestations of cystic fibrosis in the respiratory tract?

Airway obstruction, difficulty clearing secretions, respiratory distress, impaired gas exchange, chronic cough, barrel-shaped chest, decreased pulmonary function, clubbing, recurrent pneumonia, hemoptysis, pneumothorax, chronic sinusitis, nasal polyps, and cor pulmonale.

What does the CFTR mutation cause in cystic fibrosis?

Alterations in epithelial ion transport and generalized dysfunction of exocrine glands.

Who does the American College of Obstetrics and Gynecology recommend screening for cystic fibrosis?

Any person seeking preconception or prenatal care.

How can family coping be promoted for a child with cystic fibrosis?

Assist families in developing a schedule for pulmonary hygiene, appropriate diet, and enzyme supplementation, involve the family in the child's care, provide ongoing education about the illness and its treatments, recognize and respect the family's changing needs over time

What is cystic fibrosis?

Autosomal recessive disorder affecting exocrine glands.

What interventions are common for maintaining a patent airway in a child with cystic fibrosis?

Chest physiotherapy (CPT), vest airway clearance system, flutter valve device, positive expiratory pressure therapy, breathing exercises, physical exercise, administration of Pulmozyme, bronchodilators, and anti-inflammatory agents

What is the most common debilitating disease of childhood among those of European descent?

Cystic fibrosis.

What type of testing can be used to identify the CFTR mutation?

DNA testing in prenatal and newborns.

What gene mutation is responsible for cystic fibrosis?

Deletion on chromosome 7 at CFTR.

What are the consequences of increased viscosity of secretions in cystic fibrosis?

Difficult to clear secretions in sweat glands, gastrointestinal tract, pancreas, respiratory tract, and other exocrine tissues.

What happens to chloride and water transport in cystic fibrosis?

Epithelial cells fail to conduct chloride and water transport abnormalities occur.

What might be heard during auscultation of a child with cystic fibrosis?

Fine or coarse crackles, scattered or localized wheezing, diminished breath sounds, tachycardia, presence of a gallop, adequacy of bowel sounds

What should be observed during the physical examination of a child with cystic fibrosis?

General appearance and color, respiratory rate and effort, cough characteristics, sputum production, presence of barrel chest, clubbing of nail beds, presence of rectal prolapse, size and weight for age, presence of edema or distended neck veins

What are some complications of cystic fibrosis?

Hemoptysis, pneumothorax, bacterial colonization, cor pulmonale, volvulus, intussusception, intestinal obstruction, rectal prolapse, gastroesophageal reflux disease, diabetes, portal hypertension, liver failure, gallstones, and decreased fertility.

What findings might be revealed during percussion and palpation of a child with cystic fibrosis?

Hyperresonance over lung fields, decreased diaphragmatic excursion, dullness over enlarged liver or mass, asymmetric chest excursion, decreased tactile fremitus, tenderness over liver

What type of diet is recommended for children with cystic fibrosis?

Increased-calorie, high-protein diets.

What is the last resort treatment option for some children with cystic fibrosis?

Lung transplantation.

What are the clinical manifestations of cystic fibrosis in the gastrointestinal tract?

Meconium ileus, vomiting, abdominal distention and cramping, anorexia, right lower quadrant pain, fecal impaction, rectal prolapse, bowel obstruction, intussusception, obstructive cirrhosis, gallstones, gastroesophageal reflux disease, inadequate protein absorption, altered absorption of iron and vitamins, failure to thrive, hyperglycemia, and development of diabetes.

What is the therapeutic management of cystic fibrosis aimed towards?

Minimizing pulmonary complications, maximizing lung function, preventing infection, and facilitating growth.

What is the focus of nursing management for cystic fibrosis?

Minimizing pulmonary complications, promoting growth and development, facilitating coping and adjustment by the child and family

What is prescribed to promote digestion and absorption of nutrients in cystic fibrosis?

Pancreatic enzymes and supplemental fat-soluble vitamins.

What determines the choice of antibiotic in cystic fibrosis?

Sputum culture and sensitivity results.

What are common laboratory and diagnostic tests ordered for the diagnosis and assessment of cystic fibrosis?

Sweat chloride test, pulse oximetry, chest radiograph, pulmonary function tests

How can infection be prevented in a child with cystic fibrosis?

Vigorous pulmonary hygiene, administration of aerosolized antibiotics, and intravenous antibiotics for severe respiratory exacerbations

Is cystic fibrosis included in newborn screening in all states?

Yes.