Cystic fibrosis (CF) is a multisystem genetic disease of the exocrine glands—those glands with ducts such as the mucous, salivary, and sweat-producing glands. Cystic fibrosis, originally called CF of the pancreas, is also associated with the glands of the respiratory system and the skin, and it has the potential for multiple organ involvement. The lungs are most frequently affected, but the gastrointestinal (GI) tract (including the small intestine and pancreatic and bile ducts) and eventually the reproductive organs are affected, as well. Cystic fibrosis leads to an increase in viscosity of bronchial and pancreatic secretions, which obstruct the glandular ducts. As thick secretions block the bronchioles and alveoli, the patient develops severe atelectasis (lung collapse) and emphysema. The GI effects of the disease lead to deficiency in the enzymes trypsin, amylase, and lipase. With enzyme deficiency, the conversion and absorption of fats and proteins are altered, and vitamins A, D, E, and K are not properly absorbed. Pancreatic changes include fibrosis, cyst formation, and the development of fatty deposits that lead to pancreatic insufficiency and decreased insulin production. Intelligence and cognitive function are typically not affected. Complications of cystic fibrosis can be life-threatening. Respiratory complications include lung collapse, pneumothorax, airway collapse, and pneumonia. GI complications include dehydration, malnutrition, gastroesophageal reflux, and rectal prolapse.
The responsible gene, the cystic fibrosis transmembrane conductance regulator (CFTR), is mapped to chromosome 7. The underlying defect of this autosomal recessive condition involves a defective protein that interferes with chloride transport, which, in turn, makes the body’s secretions very thick and tenacious. The ducts of the exocrine glands subsequently become obstructed.
Nursing care plan assessment and physical examination
Cystic fibrosis has a highly variable presentation and course, ranging from mild to severe. Parents often report that the child’s skin has a characteristic taste of salt when they kiss the child. Hence, this classic early symptom is referred to as the “kiss of salt.” In addition, during the first year or two of life, the child experiences repeated upper respiratory infections such as nasopharyngitis, croup, bronchiolitis, and pneumonia. Although the child has a voracious appetite, he or she does not gain weight and has steatorrhea (frequent foul-smelling, fatty stools). Moreover, the child may not achieve developmental milestones, particularly in the area of gross motor skills.
The newborn may have a meconium ileus; this finding occurs in about 10% to 15% of the newly diagnosed cases. The infant or child may be classified as exhibiting organic failure to thrive and may fall below the 10th percentile. Early in the disease, the lungs have many adventitious breath sounds, such as rales, rhonchi, and wheezes. The anterior posterior to lateral diameter begins to increase as the disease progresses so that the child appears barrel-chested. Clubbing of the nails is indicative of advanced progression of the disease and may be noted in a toddler or a preschooler who has a severe form of the disease.
Children or adolescents with cystic fibrosis deal with a chronic illness that makes them unique from their peers. They need to feel as if they have a degree of control in their lives; this need may be manifested in refusing to take their enzymes with their meals or their insulin if they become diabetic. Body image is especially critical because of their short stature and small body structure. Many adolescents are embarrassed and try to cover up a protuberant abdomen with baggy clothing and large shirts or to disguise the clubbing of their nails with dark nail polish. In addition, the patients often learn early to achieve a sense of competency by performing well in their academics or becoming computer “wizards,” because they are unable to compete in sports.
Nursing care plan primary nursing diagnosis: Ineffective airway clearance related to excess tenacious mucus.
Nursing care plan intervention and treatment
The major goals of treatment are to improve pulmonary, GI, and pancreatic status. These goals are achieved through a combination of medications, nutrition, and exercise regimens. If antibiotics are given to prevent and treat pneumonia, the physician and pharmacist monitor therapeutic blood levels of the antibiotics to determine the peak and trough levels. To help prevent the recurrence of pneumonia, chest physiotherapy (CPT) is performed in the home or hospital four times a day before meals to avoid emesis or after an aerosol treatment. A ThAIRapy vest, a device that provides high-frequency chest wall oscillations to loosen secretions, may also be used.
Maintain calorie counts on daily meal plans; supplement nutritional needs with high-calorie feedings. A patient may also have nasogastric feedings to which pancreatic enzymes are added to ensure the digestion and absorption of fats, protein, and carbohydrates. The physician may also prescribe total parenteral nutrition and fat-soluble vitamins (A, D, E, and K).
Regular exercise, including mobility and muscle-strengthening exercises should be encouraged on a regular basis. Exercise helps maintain physical wellness and supplements the patient’s airway clearance strategies by helping to loosen pulmonary secretions.
Some patients develop right-sided heart failure, and if this occurs, most of them die within a year. They may require the use of home portable oxygen therapy and receive digoxin and/or diuretics. As the disease progresses toward the terminal phase, hemoptysis is present and cyanosis is markedly apparent.
Educate to reinforce the importance of regular CPT and expectoration of the mucus. Encourage increased fluid intake to loosen the secretions, and provide frequent mouth care before meals. Teach the parents not to offer cough suppressants, which can lead to obstruction, lung collapse, and infection. Support the child’s or adolescent’s body image concerns; compliment the patient on her or his strengths. Encourage the child to develop in as many areas as possible. Very often, other cystic fibrosis patients become a significant support group as the child matures. The child is always dramatically affected when another peer with cystic fibrosis dies. Plan group discussions with the patients and have a psychiatric nurse clinical specialist serve as facilitator of this grief work for both patients and staff. In addition, siblings often worry that they may contract the disease or they may exhibit feelings of jealousy of the attention given to the sibling with cystic fibrosis. A referral to a social worker or the Cystic Fibrosis Foundation may be needed. Counsel couples on the risk that subsequent pregnancies may result in a child with cystic fibrosis, since there is a one in four chance with any pregnancy that a child could have cystic fibrosis if both parents are carriers. Discuss the role of amniocentesis and the difficult issues surrounding terminating a pregnancy if cystic fibrosis is confirmed prenatally.
Nursing care plan discharge and home health care guidelines
Teach the patient and family how to prevent future episodes of pneumonia through CPT, expectoration of sputum, and avoidance of peers with common colds and nasopharyngitis. Explain that medications need to be taken at the time of each meal, especially pancreatic enzymes and supplemental vitamins. Teach the parents protocols for home IV care, as needed. Teach parents when to contact the physician: when temperature is elevated over 100.5°F, sputum has color to it, or the child complains of increased lung congestion or abdominal pain. Also educate parents on the need to keep routine follow-up appointments for medication, laboratory, and general checkups. Teach the patient or parents proper insulin administration and the appropriate signs and symptoms of high and low glucose levels.