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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.

Models of providing care at the end of life and the most appropriate time for involving palliative care services are reviewed. The principles of management of pain and other symptoms are highlighted, including the issues related to providing sedation at the end of life. Pharmacologic guidelines are included for various aspects of pain and symptom management, as well as those specific to terminal sedation in children. A brief overview is included on anticipatory grief, bereavement follow-up, and staff support.

ing a difference in her comfort and survival. It was, and is, unbearable to me that I could not save her, could not protect her. At least, I could ensure that her medical care was carried out as she wished. My medical training made this easier for me than it might be for others. I would do just about anything to have my daughter alive. Nothing can change the relentless reality that she died. However, I am comforted, knowing that she died the death she wanted. I see that as wholly the outcome of mobilizing together to deal openly and honestly with what Liza was facing, of not leaving her alone with her dying. Not all of us will be that lucky.