Cystic fibrosis (CF) has been transformed from a fatal diagnosis in infancy to a chronic disease of children and young adults. Symptom patterns and disease burden in CF may be shifting to reflect the relatively healthier, older population with the disease. Self-management of symptoms is a hallmark of chronic illness, and yet we do not have a good understanding of how CF patients monitor or manage their symptoms. Children and adults were recruited through clinics in three Canadian provinces. Questionnaires with open-ended and close-ended questions in English and French, designed to assess the frequency, severity, and self-management of pain, breathlessness, and cough, were mailed to all the eligible participants. One hundred twenty-three respondents completed the survey, for a response rate of 64%. Eighty-four percent (103 of 123) of participants reported having pain. They reported an average of 2.1 locations of pain, with headache and abdominal pain most frequently described. Sixty-four percent (76 of 123) of participants reported having breathlessness, and 83% (99 of 123) of participants reported experiencing cough. Sixty-three percent (62 of 99) of participants with cough reported that cough always or sometimes interfered with their sleep. A variety of pharmacological and nonpharmacological treatments were used to manage symptoms. Pain and dyspnea are more common than suspected and a wide variety of pharmacological and nonpharmacological measures are used to treat symptoms. Cough is difficult to assess, but disturbed sleep may be an indicator of cough severity and an important symptom to consider when evaluating the overall burden of illness in those with CF.
We found IN fentanyl, which can be administered in a variety of care settings, to be a minimally invasive means of palliating distress in dying newborns and infants. No adverse events related to its use were noted.
Perinatal palliative care is an emerging area of health care. To date, no published tools assess health-care provider's knowledge and level of comfort in providing such care. A 2-phase study was undertaken to develop and implement a survey to evaluate the self-assessed competency, attitudes, and knowledge of health-care providers working in perinatal palliative care. Phase 1 included a review of the literature and appraisal of palliative and death-related instruments to inform the initial draft of the Perinatal Palliative Care Survey (PPCS). Twenty-four Canadian pediatric palliative care specialists critiqued the PPCS, establishing its face and content validity. Phase 2 involved administering the PPCS at 4 sites across Canada, resulting in 167 responses from nurses, physicians, and midwives. The majority of participants responded that they possessed a degree of comfort in providing perinatal palliative care, particularly with assessing pain (76%), managing pain (69%), assessing other symptoms (85%), and managing other symptoms (78%). Two areas where participants level of confidence or extreme confidence was diminished included having conversations with families about the possibility of their infant dying (55%) and knowing and accessing community palliative care resources (32%). Responses in the knowledge section identified gaps related to opioid use, pharmacological interventions for breathlessness, pain behaviors, and tolerance developed to opioids and sedatives. Eighty-six percent of respondents stated that if education about palliative care was made available, they would participate with priority topics identified as communication with families (75%), managing symptoms (69%), pain management (69%), and ethical issues (66%). The PPCS provides a useful assessment to determine the educational needs of health-care providers delivering perinatal palliative care.
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