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Care given to young dying patients with cystic fibrosis (CF) appears to differ from the care given to dying children with cancer. Analyses done on end-of-life medical records of 44 young patients with CF revealed that most patients continued receiving pain and antibiotic medications until the last hours of life. Most patients received varying amounts of opiate medications for varying lengths of time to reduce pain and labored breathing. Nearly all patients died while hospitalized under orders not to resuscitate., Objective. End-of-life clinical care in cystic fibrosis (CF) differs substantially from terminal care in childhood cancer. To examine this difference, we reviewed the medical care of a cohort of CF patients treated at Children's Hospital, Boston, to document the use of preventive, therapeutic, and palliative care in the month preceding death. Patients. We reviewed the medical records of 44 patients older than 5 years who died of CF-related respiratory failure for the years 1984 to 1993. Results. Thirty-eight patients (86%) received opiates for the treatment of severe dyspnea and pain; the duration of opiate use varied from less than 1 hour to greater than 1 month. The dose of opiates varied from less than 5 mg per hour to greater than 30 mg per hour. Thirty-three patients (75%) continued to receive intravenous antibiotics in the last 12 hours of life; 32 (72%) continued to receive preventive or therapeutic oral medications in the last 12 hours of life. All patients were designated as do not resuscitate at the time of death; 43 of the patients died in the hospital with 1 patient dying at home under hospice care. Conclusions. The model of comfort care developed in childhood cancer does not adequately describe the combination of preventive, therapeutic, and palliative care given at the end of life for CF at our institution. The majority of CF patients continued to receive intravenous antibiotics and/or oral vitamin preparations while being treated with opiates for terminal pain and dyspnea. Small doses of opiates seem to be effective in the treatment of the pain and dyspnea at the end of life in CF. Pediatrics 1997;100:205-209; cystic fibrosis palliative care, terminal care., ABBREVIATIONS. CF, cystic fibrosis; FEV[1], mean forced expiratory volume at one second; DNR, do not resuscitate; BiPAP, biphasic positive airway pressure. Cystic fibrosis (CF) is the most common lethal childhood [...]

Many older children and young adults with cystic fibrosis (CF) appear to live with long-term pain. Treatment with opiates appears to be effective and safe in managing this pain. Pain frequency and types of pain medication used were identified among 55 patients older than 5 years of age who had died with CF and 23 patients with CF currently treated for pain. Eighty-four percent of the patients had long-term pain. The most frequent types of pain reported included chest pain, headaches, abdominal pain, and back pain. Pain was intense enough to require opiates in 53% of the patients with 13% requiring this medication for several months., ABSTRACT. Objective. The objective of this study was to examine the incidence and therapy of chronic pain in a group of older patients with cystic fibrosis (CF). Patients. We identified [...]

Abstract Background The clinical course of Cystic Fibrosis (CF) is usually measured using the percent predicted FEV1 and BMI Z-score referenced against a healthy population, since achieving normality is the ultimate goal of CF care. Referencing against age and sex matched CF peers may provide valuable information for patients and for comparison between CF centers or populations. Here, we used a large database of European CF patients to compute CF specific reference equations for FEV1 and BMI, derived CF-specific percentile charts and compared these European data to their nearest international equivalents. Methods 34859 FEV1 and 40947 BMI observations were used to compute European CF specific percentiles. Quantile regression was applied to raw measurements as a function of sex, age and height. Results were compared with the North American equivalent for FEV1 and with the WHO 2007 normative values for BMI. Results FEV1 and BMI percentiles illustrated the large variability between CF patients receiving the best current care. The European CF specific percentiles for FEV1 were significantly different from those in the USA from an earlier era, with higher lung function in Europe. The CF specific percentiles for BMI declined relative to the WHO standard in older children. Lung function and BMI were similar in the two largest contributing European Countries (France and Germany). Conclusion The CF specific percentile approach applied to FEV1 and BMI allows referencing patients with respect to their peers. These data allow peer to peer and population comparisons in CF patients.

Background: The clinical course of Cystic Fibrosis (CF) is usually measured using the percent predicted FEV1 and BMI Z-score referenced against a healthy population, since achieving normality is the ultimate goal of CF care. Referencing against age and sex matched CF peers may provide valuable information for patients and for comparison between CF centers or populations. Here, we used a large database of European CF patients to compute CF specific reference equations for FEV1 and BMI, derived CF-specific percentile charts and compared these European data to their nearest international equivalents. Methods: 34859 FEV1 and 40947 BMI observations were used to compute European CF specific percentiles. Quantile regression was applied to raw measurements as a function of sex, age and height. Results were compared with the North American equivalent for FEV1 and with the WHO 2007 normative values for BMI. Results: FEV1 and BMI percentiles illustrated the large variability between CF patients receiving the best current care. The European CF specific percentiles for FEV1 were significantly different from those in the USA from an earlier era, with higher lung function in Europe. The CF specific percentiles for BMI declined relative to the WHO standard in older children. Lung function and BMI were similar in the two largest contributing European Countries (France and Germany). Conclusion: The CF specific percentile approach applied to FEV1 and BMI allows referencing patients with respect to their peers. These data allow peer to peer and population comparisons in CF patients. [ABSTRACT FROM AUTHOR]