Caregivers for advanced cystic fibrosis patients want to discuss intensive treatments and symptom relief
Research Activities, August 2011, No. 372
Cystic fibrosis (CF) is a genetic disease that leads to progressive decline in lung function over time, with a median survival in the United States of 37.4 years. Two exploratory studies supported by the Agency for Healthcare Research and Quality (HS10397) examined caregiver perspectives on intensive treatment discussions and end-of-life symptom control for patients with advanced CF. They are briefly described here.
Dellon, E.P., Shores, M.D., Nelson, K.I., and others (2010, December). "Caregiver perspectives on discussions about the use of intensive treatments in cystic fibrosis." Journal of Pain and Symptom Management 40(6), pp. 821-828.
The researchers interviewed 36 caregivers of patients who had died of complications of CF (or a syndrome seen in some CF patients after lung transplant) about treatment preference discussions and how to improve them. Twenty-two (61 percent) caregivers had discussed intensive treatment preferences with a physician, and 17 of these discussions were initiated during an acute illness. Intensive treatments, such as noninvasive ventilation or mechanical ventilation, which are intended to sustain life during acute respiratory failure, are commonly used in patients with advanced CF lung disease. Most (29) caregivers favored first discussing treatment preferences during a period of medical stability.
Dellon, E.P., Shores, M.D., Nelson, K.I., and others (2010, December). "Family caregiver perspectives on symptoms and treatments for patients dying from complications of cystic fibrosis." Journal of Pain and Symptom Management 40(6), pp. 829-837.
In this study, 27 caregivers answered questions about symptoms and treatments. Caregivers reported that distressing symptoms were common during the last week of life, including shortness of breath (100 percent), fatigue (96 percent), anorexia (85 percent), anxiety (74 percent), pain (67 percent), and cough (56 percent). Most caregivers (71 percent) said that symptom control was "somewhat good" and 9 percent said it was "very good." Caregivers often believed that symptoms could not be controlled because of side effects and potential for hastening death with the use of opioids and anti-anxiety medications to relieve symptoms. Many family caregivers expressed low expectations for symptom control. The researchers suggest additional studies may identify barriers to symptom assessment and management and assist in designing design interventions to overcome those barriers. Additional studies could address how to reduce and counteract side effects and discomforts of disease-specific treatments (such as chest physiotherapy or tube feeding), which providers, caregivers, and patients feel are necessary to manage advanced lung disease related to CF, so they are better tolerated by patients.