diffuse interstitial pulmonary fibrosis
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fibrosis(fi-bro'sis) [ fibro- + -sis]
cystic fibrosisAbbreviation: CF
A great variety of clinical manifestations may be present, including nasal polyposis; lung changes related to thick, tenacious secretions leading to bronchiectasis; bronchitis; pneumonia; atelectasis, emphysema and respiratory failure; gallbladder diseases; intussusception; meconium ileus; salt depletion; pancreatic exocrine deficiency causing intestinal malabsorption of fats, proteins, and, to a lesser extent, carbohydrates; pancreatitis; peptic ulcer; rectal prolapse; diabetes; nutritional deficiencies; arthritis; absent vas deferens with consequent aspermia and absence of fructose in the ejaculate; failure to thrive; and delayed puberty. The child exhibits a nonproductive, paroxysmal cough, barrel chest, cyanosis, clubbed fingers and toes, malabsorption leading to poor weight gain and growth, fat-soluble vitamin deficiency (A, D, E, K) leading to clotting abnormalities, and excretion of frequent pale stools that are bulky, foul-smelling and have a high fat content.
Therapy must be individualized, carefully monitored, and continued throughout life. Pulmonary infection is controlled with antibiotics. It is essential that secretions be cleared from the airway by intermittent aerosol therapy. A mucolytic agent may be helpful as well as postural drainage, mist inhalation, and bronchodilator therapy. Bronchoalveolar lavage has been of use in some patients. In addition, bronchial drainage may be improved by use of aerosolized recombinant human DNase (rhDNase). Use of a Flutter device for airway mucus clearance is considerably more effective in increasing sputum expectoration than traditional postural drainage and clapping the chest. Lung transplantation may also be used to treat CF. High doses of ibuprofen taken consistently for years may slow progression of the disease by limiting airway inflammation. See: bronchoalveolar lavage; Flutter device
Median cumulative survival is approximately 30 years, with males surviving much longer than females for unknown reasons.
In 2001 The American College of Obstetricians and Gynecologists and the American College of Medical Genetics recommended that all prospective parents undergo screening to see if they are carriers of cystic fibrosis genes.
Both patient and family are taught to perform pulmonary chest physiotherapy and postural drainage followed by deep breathing and coughing to help mobilize secretions. Fluid intake is encouraged to thin inspissated secretions. Humidified air, with intermittent positive-pressure breathing therapy if prescribed, is provided. Dornase alfa is also administered by nebulizer as prescribed. A DNA enzyme produced by recombinant gene therapy, the drug is used to reduce the frequency of respiratory infections, to decrease sputum thickness (viscosity), and to improve pulmonary functioning in patients with cystic fibrosis.
The patient should take precautions (e.g., annual influenza immunization and at least one pneumococcal vaccination) to prevent respiratory infections and should learn to recognize and report signs and symptoms and to initiate prescribed antibiotic prophylaxis promptly. Oral pancreatic enzymes are given with meals and snacks to replace deficiencies, and foods are well-salted or a sodium supplement prescribed to combat electrolyte losses in sweat. A well-balanced high-calorie, high-protein diet is recommended, including replacement of fat-soluble vitamins if laboratory analysis indicates any deficiencies. Aerobic exercise and physical activity within permitted limits are encouraged; breathing exercises should be performed during activity to improve ventilatory capacity and activity tolerance. The child is encouraged in age-appropriate developmental tasks, and acceptable activities are substituted for those in which the child is unable to participate.
Caregivers involve the child in care by offering valid choices and encouraging decision making. The family is encouraged to discuss their feelings and concerns. Genetic testing is explained. Realistic reassurance is offered regarding expectations after an exacerbation, and emotional support is provided to help both patient and family work through feelings of anticipatory grief. Referral is made to available local chapters of support groups such as the Cystic Fibrosis Foundation.
diffuse interstitial pulmonary fibrosisIdiopathic pulmonary fibrosis.
idiopathic pulmonary fibrosis
Dyspnea, cough, exertional fatigue, and generalized weakness are common. Signs of the illness include pulmonary crackles, finger clubbing, cyanosis, and evidence of right ventricular failure (such as lower-extremity swelling). The disease typically progresses to end-stage lung disease and death within 7 years of diagnosis.
A biopsy of the lung is needed to make the diagnosis.
Corticosteroids (such as prednisone) may be helpful in 10% to 20% of patients. Lung transplantation can be curative if a donor organ is available.