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Effects on the Respiratory System: Pulmonary Fibrosis and Non-Cardiogenic Pulmonary Edema

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Effects on the Respiratory System: Pulmonary Fibrosis and Non-Cardiogenic Pulmonary Edema Effects on the Respiratory System: Pulmonary Fibrosis Effects on the Respiratory System: Pulmonary Fibrosis and Non-cardiogenic Pulmonary Edema Author: Ayda G. Nambayan, DSN, RN, St. Jude Children’s Research Hospital Content Reviewed by: Cindy Burleson, RN, MSN, MS, CPON, St. Jude Children’s Research Hospital Cure4Kids Release Date: 6 June 2006 Pulmonary damage associated with treatments includes pulmonary fibrosis, hypersensitivity lung disease and non-cardiogenic pulmonary edema (NCPE) also referred to acute respiratory distress syndrome [ARDS]). Although these complications have similar presenting symptoms, they differ in terms of time-relation to cancer treatment and long-term outcomes and are associated with differing radiographic findings and types of pulmonary damage. Often, pulmonary complications present as a nonspecific cough along with progressive dyspnea and low-grade fever. The most common mechanisms by which specific drugs can -induce pulmonary complications include the following. 1. direct damage to the alveoli 2. immunologic respiratory response 3. metabolic damage by the chemotherapy metabolite such as acrolein 4. antitumor actions of the drug Pulmonary Fibrosis The most common chemotherapy-associated lung injury is drug-induced pneumonitis, associated with most pulmonotoxic antineoplastic agents such as bleomycin and nitrosureas. The major concern regarding pneumonitis is the potential of progression to irreversible pulmonary fibrosis. Pulmonary fibrosis (A – 1) is the formation of fibrous scar tissue in the lungs as a consequence of inflammation or injury or both. Subsequently, fibrosis progresses to sclerosis of pulmonary vessels and bronchi, leading to bronchiectasis. Patients at risk of pulmonary fibrosis are those who have received high doses of radiation therapy to the lungs and those who have received chemotherapy consisting of drugs such as busulfan, methotrexate, melphalan, cyclophosphamide, mitomycin, carmustine and bleomycin. Assessment In children and adolescents, the most common symptoms are restlessness, anxiety and a progressive cough. Signs and symptoms of pulmonary fibrosis are the same as those of Module 7 - Document 13 Page 1 of 7 Effects on the Respiratory System: Pulmonary Fibrosis respiratory distress: tachypnea, dyspnea, cough, compromised oxygenation levels, increased respiratory effort and chest pain. Pulmonary symptoms usually occur 7 to 10 days after administration; symptoms may also have a delayed presentation of up to 10 years. Delayed presentation is usually due to irreversible lung tissue damage. Children and adolescents with respiratory fibrosis should be monitored for signs and symptoms of congestive heart disease and respiratory distress. Physical assessment of patients with such conditions may detect rapid weight changes, peripheral edema, skin mottling, cyanosis, use of respiratory muscles to breath and easy fatigability. In severe cases, muscle wasting may be evident. Physical assessments may also reveal the use of accessory muscles of breathing, shallow respirations, decreased breath sounds, possible asymmetry of chest expansion and the presence of adventitious breath sounds. The risk of developing pulmonary damage is increased by cumulative doses of chemotherapy (especially of bleomycin); the presence of comorbidities such as respiratory syncytial virus (RSV) infections, asthma, and other factors such as age and premature birth.. Therefore, the nurse’s assessment should identify any of these factors, if present. Laboratory diagnostic procedures such as pulmonary function tests and radiologic examinations may also be done. Pulse oximetry may show decreased oxygenation levels. Planning A plan of care should result in the following. Maintaining adequate oxygenation and respiratory function An understanding by the patient and family of pulmonary fibrosis and the chronic-care requirements An understanding by the patient and family of the need for long-term follow-up Compliance with the recommended therapy Implementation The goal of care should be maintenance of adequate oxygenation levels and prevention and early recognition of cardiopulmonary complications such as congestive heart failure. The patient with pulmonary fibrosis is often given oxygen therapy and corticosteroids. In addition, the nurse can implement supportive care designed to help alleviate the work of breathing such as raising the head of the bed, providing small frequent meals and foods that are easy to chew and digest. Supportive medical care such as administration of diuretics and vasopressors is designed to relieve pulmonary congestion; artificial ventilation may be used, if necessary. Module 7 - Document 13 Page 2 of 7 Effects on the Respiratory System: Pulmonary Fibrosis The nursing care plan should also include monitoring fluid intake and output, measuring daily weight and teaching the patient and family alternative activities that will not compromise the cardiopulmonary status of the patient. Play and other activities may be modified to better meet the patient’s tolerance level. Evaluation The desired outcomes for the patient with pulmonary fibrosis include adaptation and successful, continued management of an added chronic condition. Patient and Family Education The patient and family education should be focused on the following. Activity levels - play that does not require a great expenditure of energy - diversional activities - activities patterned to meet the patient’s ability to tolerate respiratory exertion - activities that are directed to energy conservation Prevention of complications such as respiratory infections and burns from O2 combustion - avoidance of crowds and people with infections - hand washing and general cleanliness - oxygen safety (prevention of oxygen combustion) Monitoring of the patient’s condition and early recognition of complications - signs and symptoms of increased respiratory effort - signs and symptoms of congestive heart failure – rapid weight gain, distal edema, increased work of breathing, decreased urinary output - respiratory compromise (increased need for oxygen) – cyanosis, headache, irritability, changes in mentation - identification of the conditions in which a health care provider should be contacted Ways to manage or decrease dyspnea - teaching the patient exercises that would assist with dyspnea such as pursed-lip breathing, blowing out--candle exercises - the importance of not smoking and/or being exposed to secondhand smoke - use of medications such as low-dose opioids Module 7 - Document 13 Page 3 of 7 Effects on the Respiratory System: Pulmonary Fibrosis Non-cardiogenic pulmonary edema (NCPE) Non-cardiogenic pulmonary edema (NCPE) is a rare and less recognizable pulmonary toxicity associated with cancer treatment. NCPE is characterized by the simultaneous presence of severe hypoxemia, the collection of fluids in the lungs (bilateral alveolar infiltrates on chest x-rays) with no evidence of left atrial hypertension (congestive heart failure). NCPE is diagnosed by exclusion of other possible causes such as infections and other metabolic and cancer-related causes. A supportive rationale for a diagnosis of NCPE is sometimes provided by the proximity between the time at which NCPE appears and the time that the drugs known to cause NCPE were administered. Cancer therapeutic agents often associated with NCPE (A - 2) include the following. cytarabine gemcitabine interleukin-2 (IL-2), which causes increased capillary permeability all-trans retinoic acid (ATRA), which is used to treat acute promyelocytic leukemia Early signs of NCPE may include coughing and restlessness during sleep. Later, the patient may experience trouble breathing when awake and at night. Coughing usually produces white- or pink-tinged frothy sputum. Noisy breathing (wheezing and bubbly sounds), bluish nailbeds and lips, sweating, and a fast heartbeat are other signs. The patient may also feel very anxious. NCPE is reversible and can be successfully managed with high-dose corticosteroids, diuretics and oxygen supplementation. When lung toxicity is suspected, immediate discontinuation of chemotherapy is recommended. Module 7 - Document 13 Page 4 of 7 Effects on the Respiratory System: Pulmonary Fibrosis APPENDIX A – 1 Radiation Fibrosis Loyola University Medical Education Network - LUMEN http://www.meddean.luc.edu/lumen/meded/medicine/pulmonar/cxr/atlas/radpneumonitis.htm Go Back Module 7 - Document 13 Page 5 of 7 Effects on the Respiratory System: Pulmonary Fibrosis A – 2 Cancer Therapeutic Agents Associated with the Development of Non-cardiogenic Pulmonary Edema Incidence Comments/Characteristics Therapy or Medication Cytarabine - moderate to high doses (1-1.5 High Potentially fatal toxicity observed in patients with g/m2 given by continuous infusion, or >3 leukemia. It develops 1 to 2 weeks after chemotherapy g/m2 as 2-h IV infusion per 12 h) over 3 to (usually at the initial course). Pathophysiology: 4 days increased permeability of alveolar capillaries Recombinant IL-2 (high-doseIV) given to 3% - 20% Usually described in the context of generalized patients with renal cancer or melanoma vascular leak syndrome. Severe but reversible on discontinuation of IL-2. Pathophysiology: a damaging effect on vascular endothelial cells by cytokines Bone marrow transplantation Moderate Bone marrow transplant recipients may develop NCPE in the context of systemic capillary leak syndrome 1 to 2 months after high-dose chemotherapy. A pivotal contribution by circulating leukocytes has been suggested.
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