Healthcare professionals often dismiss pain as a debilitating symptom in patients with cystic fibrosis (CF), according to a study recently published in the Journal of Cystic Fibrosis.

This observational study included 55 people with CF who completed a questionnaire regarding symptoms based on the Supportive Care Needs Survey Short Form 34, their personal experience with accessing pain relief, forced expiratory volume in 1 second (FEV1)% values, and CF transmembrane conductance regulator (CFTR) modulator therapy.

Pain was the most frequently experienced symptom, present in 76% of cases, and it was also the symptom that caused the most distress, observed in 64% of individuals. 

“Respondents expressed a concomitant desire for pain relief while avoiding opioid dependency and being labeled by the health care team as ‘drug-seeking,’” the authors wrote.

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Notably, those not on CFTR modulator therapy reported pain as distressing at a higher frequency than those who did receive treatment. On the other hand, FEV1% values did not correlate with pain. The usual locations of pain were the joints, back, sinuses, chest, abdomen, and head, in that order.

Regardless of the rather high prevalence of pain in this population, many patients described unpleasant experiences when trying to discuss pain management therapies with their healthcare providers. 

Furthermore, participants were interested in alternative methods, such as physical and psychological approaches, while being concerned about potential adverse reactions of pharmacological analgesics. 

As expected, individuals who described pain as distressing further admitted to being worried about their quality of life and ability to maintain functioning to perform daily tasks in the future. 

“While CFTR modulator therapies have improved [quality of life] for many [people with CF], it is important to continue comprehensive pain and symptom evaluation and treatment for all [people with CF], including those who are ineligible due to genetic mutations or medical contraindications or do not have a favorable response to these therapies,” the authors explained.

Finally, there were 9 other symptoms reported by more than half of the people surveyed. In order of frequency, these included: sinus discharge, fatigue, cough, difficulty sleeping, feeling bloated, shortness of breath, diarrhea, fears about CF getting worse, and anxiety. 

As for symptoms that caused distress, the study found 2 only symptoms besides pain that affected the majority of participants: fatigue and difficulty sleeping, which were present in 55% and 51%, respectively.


Dubin E, Lowers J, Dellon EP, et al. Prevalence of unmet pain and symptom management needs in adults with cystic fibrosis. J Cyst Fibros. Published online August 14, 2022. doi:10.1016/j.jcf.2022.08.006