Frequency and Self-Management of Pain, Dyspnea, and Cough in Cystic Fibrosis

Abstract

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.

Sleep and Pain in Pediatric Illness: A Conceptual Review

“…Sleep disturbance is not a direct symptom of CF, but coughing has been found to interfere with sleep . It is important to note, however, that a causal relationship between cough and sleep disturbance has not been substantiated .…”

Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings

“…Interestingly, previous studies assessing the general symptom of pain in CF indicate that its most common location was the abdomen [8–11]. Nevertheless, most of these studies did not differentiate abdominal pain e.g.…”

Pain Impacts on Quality of Life and Interferes with Treatment in Adults with Cystic Fibrosis

“…Pain in CF may be due to musculoskeletal changes (Aris et al, ; Tattersall and Walshaw, ) as well as CF‐related arthropathy (Botton et al, ; Hubbard et al, ; Koch et al, ). Previous studies found back pain and headaches reported in up to 89% of adults with stable CF, while the incidence of chest pain varies between 16% and 64% (Rose et al, ; Epker et al, ; Festini et al, ; Flume et al, ; Sermet‐Gaudelus et al, ; Stenekes et al, ). However, the influence of lung disease severity on pain locations is unknown.…”