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Related to pulmonary fibrosis: Idiopathic pulmonary fibrosis
Causes and symptoms
- avoid exposure to particle dust such as asbestos, coal dust, and silica
- avoid exposure to chemical fumes
- do not smoke
pulmonary fibrosisScarring of lung tissue from any cause, such as PNEUMOCONIOSIS, previous PNEUMONIA or TUBERCULOSIS or repeated attacks of PULMONARY EMBOLISM. Fibrosis interferes with the efficient transfer of oxygen from the atmosphere to the blood and causes breathlessness. There is no specific treatment, short of a lung transplant, but progress can often be slowed.
|Mean LOS:||6.9 days|
|Description:||MEDICAL: Interstitial Lung Disease With Major CC|
Pulmonary fibrosis is a restrictive lung disease in which alveolar inflation is reduced, thus impairing lung function. The alveoli are affected by fibrotic tissue, which may develop after inflammation, infection, or tissue damage. The resulting scarring and distortion of pulmonary tissue lead to serious compromise in gas exchange. Fibrosis leads to decreased lung compliance and increased elastic recoil, which increases the overall work of breathing and inefficient exchange of gases.
Idiopathic pulmonary fibrosis (IPF) is of unknown etiology and is characterized by a poor prognosis and no effective treatment. Some forms of pulmonary fibrosis can be attributed to exposure to radiation or inhalation of noxious materials such as silica, asbestos, and coal dust. Pulmonary conditions that can result in pulmonary fibrosis include pneumonia, atelectasis, alveolar cell cancer, pulmonary edema, and lung surgery or trauma. Nonpulmonary causes include neuromuscular disease such as Guillain-Barré syndrome, amyotrophic lateral sclerosis, myasthenia gravis, and muscular dystrophy. Approximately one-third of patients can trace their initial episodes of dyspnea to a viral respiratory illness. Deformities of the bones, such as ankylosing spondylitis and scoliosis, can result in pulmonary fibrosis.
Genetic contribution to pulmonary fibrosis is suggested by familial cases of the disease. Variants in the gene encoding pulmonary surfactant protein A1 (SFTPA1) have increased susceptibility to idiopathic pulmonary fibrosis in nonsmokers. Mutations in SFTPA2 can cause idiopathic pulmonary fibrosis. Mutations in the gene encoding surfactant protein C (SFTPC) have been identified in some families with idiopathic pulmonary fibrosis, but not in others.
Gender, ethnic/racial, and life span considerations
Although it is possible for pulmonary fibrosis to occur at any age, the average age of patients who are diagnosed with pulmonary fibrosis is 50. Elderly patients or those exposed to risk factors for a prolonged period of time are at the greatest risk. Pulmonary fibrosis occurs in both men and women, and there is no known racial or ethnic patterns of prevalence or incidence.
Global health considerations
Around the world, the prevalence is reported as 10 to 20 cases per 100,000 individuals. The influences of geographical region, environmental exposure by region, and regional culture such as dietary habits and differences in smoking patterns are unknown.
Establish a history of work or lifestyle that may have caused the disease. Ask if the patient has worked as a coal miner or with materials such as asbestos or silica or whether he or she has lived near industrial plants that use such materials. Determine if the patient has had respiratory complications or conditions such as pneumonia, atelectasis, alveolar cell cancer, pulmonary edema, and lung surgery or trauma. Ask if the patient has experienced pain while breathing or shortness of breath. Ask if the patient smokes cigarettes. There is some suspicion that genetic factors may determine susceptibility to the disease, so take a family history of pulmonary conditions, including pulmonary fibrosis.
Although the symptoms of pulmonary fibrosis are nonspecific, the patient may have progressive dyspnea on exertion and cough. Observe the patient’s respiratory status, noting rate, depth, rhythm, and ease of breathing. In the initial phases of the disease, the physical examination may be normal and the lungs may be clear on auscultation. As the disease progresses, individuals with pulmonary fibrosis frequently develop shallow, rapid breathing patterns in an attempt to conserve energy. Auscultate the patient’s lungs; listen for diminished or absent breath sounds or coarse crackles, particularly at the lung bases on inspiration. Note chest excursion and symmetry. Check for digital clubbing. Some patients develop weight loss, fatigue, fever, and muscle pain. Because pulmonary fibrosis in its late stages can cause cor pulmonale, check for signs of cardiac failure, such as elevated neck veins, liver distention, and swelling of the lower extremities.
Patients may experience a lowering of self-esteem with increased dependence on others and changing roles. In addition, shortness of breath and difficulty in breathing usually cause increased anxiety. If the disease has an occupational source, financial concerns may play an important role if the patient is unable to return to work.
|Test||Normal Result||Abnormality With Condition||Explanation|
|Chest x-ray||Clear lung fields||Identification of interstitial infiltrates or ground glass pattern||Fibrotic areas have a changed appearance|
|High-resolution computed tomography||Normal lung structures||Reticular opacities, traction bronchiectasis, honeycombing, and alveolar distortion; ground-glass pattern||Fibrotic areas have a changed appearance|
|Forced expiratory flow: Maximal flow rate attained during the middle (25%–75%) of forced vital capacity maneuver||Varies by body size||25% of the predicted value||Predicts obstruction of smaller airways|
|Residual volume (RV): Volume of air remaining in lungs at end of a maximal expiration||1.2 L||Increased up to 400% normal||Increased RV indicates obstruction|
|Rheumatoid factor, antinuclear antibodies||Negative||Rheumatoid factor > 30 IU/mL; antinuclear antibodies > 1:8||Identifies autoimmune response and connective tissue disease|
Other Tests: Hemoglobin may be elevated due to chronic hypoxemia; erythrosedimentation rate may be elevated; C-reactive protein, gallium scan, serological tests, histological analysis, high-resolution computed tomography
Primary nursing diagnosis
DiagnosisIneffective breathing pattern related to shortness of breath and difficulty breathing
OutcomesRespiratory status: Gas exchange; Respiratory status: Ventilation; Symptom control behavior; Treatment behavior: Illness or injury; Comfort level
InterventionsAirway management; Anxiety reduction; Oxygen therapy; Airway suctioning; Airway insertion and stabilization; Cough enhancement; Mechanical ventilation; Positioning; Respiratory monitoring
Planning and implementation
To relieve breathing difficulties and correct hypoxia, most physicians prescribe low-flow oxygen therapy (2 to 4 L/minute). Corticosteroid therapy is the treatment of choice. If the patient does not respond to the corticosteroid therapy, immunosuppression with cyclophosphamide (Cytoxan) is considered. Cyclophosphamide reduces the white blood cell count, causing a distinct drop in the total blood lymphocyte count. Patients who do not respond to conventional therapy and whose life expectancy is less than 18 months may be candidates for lung transplantation. If cor pulmonale develops, the patient may be placed on diuretics and digitalis. Bronchodilators may improve wheezing and airway obstruction. Infections need to be identified and treated promptly. Pneumococcal and influenza vaccines are important.
|Medication or Drug Class||Dosage||Description||Rationale|
|Prednisone||5–60 mg/day PO; dose is individualized||Corticosteroid||Decreases the inflammatory process|
|Cyclophosphamide (Cytoxan)||1–5 mg/kg per day; dose is individualized||Antineoplastic||Reduces the white blood cell count, causing a distinct drop in the total blood lymphocyte count|
Other Drugs: Immunosuppressive agents azathioprine (Imuran), antifibrotic agents (colchicine), and antiviral cytokines (interferon gamma-1b). Experimental Drug: Pirfenidone (antifibrotic agent)
Focus on relieving respiratory difficulties and caring for the patient’s emotional condition. To assist with breathing, assist the patient to attain an upright, supported position to enhance respiratory excursion. Assist the patient into a Fowler’s or semi-Fowler’s position. Provide assistance with the activities of daily living as appropriate and help the patient conserve energy by alternating rest periods with periods of activity. Plan rest time of at least 1 hour after meals before engaging in activities. Teach the patient deep-breathing and coughing exercises. Use humidified air. Provide regular oral hygiene to combat dry mouth.
Because there is no cure for pulmonary fibrosis, dealing with a chronic debilitating disease requires many psychosocial adjustments for the patient and family members. Encourage the patient to verbalize concerns and fears. Encourage the patient to identify actions and care measures that help make her or him comfortable and relaxed. As much as possible, try to include the patient and family in decisionmaking about care measures. Lifestyle changes for the patient may be necessary. A well-balanced diet with adequate fluid intake is important. If the patient smokes cigarettes, cigars, or pipes, smoking cessation is an essential intervention for the patient’s survival. A job counseling session may be helpful if the patient needs to change occupations. If the patient is having trouble coping with role changes, counseling may be helpful.
Evidence-Based Practice and Health Policy
Lamas, D.L., Kawut, S.M., Bagiella, E., Philip, N., Arcasoy, S.M., & Lederer, D.J. (2011). Delayed access and survival in idiopathic pulmonary fibrosis. American Journal of Respiratory and Critical Care Medicine, 184(7), 842–847.
- Investigators conducted prospective study among 129 patients diagnosed with pulmonary fibrosis and found that increased delays in the time from dyspnea onset to initial evaluation for pulmonary fibrosis was associated with an increased mortality rate. The median delay was 2.2 years (IQR, 1 to 3.8 years).
- Compared to patients with less than a 1-year delay in evaluation, mortality was twice as likely among patients with a 1- to 2-year delay, 2.5 times more likely among patients with a 2- to 4-year delay, and 3.4 times more likely among patients with more than a 4-year delay (p = 0.04). Thirty-one percent of patients underwent lung transplantation, 27% died without undergoing lung transplantation, and 3% died after lung transplantation.
- Patients with diabetes mellitus and gastroesophageal reflux disease experienced significantly longer delays than patients without these comorbidities (p = 0.049 and p = 0.01, respectively).
- Physical changes: Skin color, respiratory patterns, breath sounds, breathing difficulties, chest symmetry and excursion, pulse oximetry
- Reaction to diagnosis and coping strategies
- Frequency of oxygen use, noting liter flow and type of delivery device, activity tolerance
Discharge and home healthcare guidelines
Teach energy conservation methods and relaxation, breathing, and coughing techniques. Explain the importance of pacing activities, avoiding strenuous activity, and providing rest periods. Teach the patient positions that can provide relief during acute episodes of dyspnea. To prevent infection, encourage the patient to receive flu and pneumococcal vaccines and to avoid crowds and people with known respiratory infections. Be sure the patient understands all medications, including the dosage, route, action, and adverse effects. If the patient is using oxygen therapy at home, teach the patient and family appropriate safety precautions. Help the patient understand the equipment and liter flow and provide information on how to obtain all the necessary equipment. Work with social services to provide for equipment in the home.
Patient discussion about pulmonary fibrosis
Q. my uncle was diagnosed with pulmonary fibrosis. can anyone help?