Study ID’s symptoms with biggest impact on life quality for CF adults

Scientists sought to mark most common symptoms since advent of modulators

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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For adults with cystic fibrosis (CF), the symptoms with the biggest impact on quality of life include pain, fatigue, shortness of breath, and mental health challenges, leading researchers to recommend routine screening to assist CF patients who could benefit from support.

“Screening for prevalent symptoms that affect quality of life the most (lack of energy, worrying, feeling sad, feeling irritable, and shortness of breath) and a less common symptom that is also strongly associated with decreased quality of life (pain) could help identify people living with CF who are most at risk for a decreased quality of life and may benefit from additional support,” the scientists wrote in “Symptoms and quality of life in adults with cystic fibrosis: A cross-sectional analysis of the InSPIRe:CF trial,” which was published in the Journal of Cystic Fibrosis. The work was funded by the National Institutes of Health and the Cystic Fibrosis Foundation.

CF is a genetic disorder that can cause a wide range of symptoms including lung and digestive issues. Historically, both have been regarded as the most important problems that need to be addressed in CF, but CF care has been radically changed over the last decade by CFTR modulators, which can increase the function of the faulty protein that causes the disease in people with specific CF-causing mutations. The therapies have radically improved lung function in eligible patients.

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Symptoms after modulators

Here, scientists in the U.S. sought to characterize what symptoms are most common and problematic for people with CF since modulators have become available.

“Understanding which symptoms are most prevalent, most distressing, and most strongly associated with quality of life may help streamline the symptom screening process and help identify individuals who may need more intensive follow-up or those who would benefit from additional support to improve their quality of life,” wrote the researchers, who analyzed symptom severity and life quality data from 262 adults with CF participating in a clinical trial testing a palliative care intervention. Palliative care is a type of medicine that focuses on improving life quality of patients and their families who face challenges associated with life-threatening diseases.

The median age of those analyzed was 33 and more than three-quarters were on modulator therapy.

The most commonly reported CF symptoms were coughing and fatigue, both reported by most patients. Mental health challenges were also common. More than one in three patients had a diagnosis of anxiety and a similar number had depression. When the patients were asked to rate which symptoms were most distressing, the most frequently picked were pain and difficulty sleeping.

Symptoms’ effect on quality of life

In statistical analyses, the researchers looked for symptoms that showed consistent associations with poor quality of life. The most frequent ones associated with the largest decreases in quality of life scores included lack of energy, worrying, feeling irritable, shortness of breath, and difficulty sleeping. Pain also strongly correlated with a worse quality of life, though it was less common.

Patients with diagnosed anxiety or depression tended to report poorer quality of life than those who didn’t have these mental health diagnoses.

The researchers said the symptoms with the biggest impact on life quality weren’t related to lung or digestive health, though these have long been regarded as the most crucial areas of care in CF. They suggested the availability of modulators may make digestive and lung health less of a problem, resulting in other symptoms becoming more important.

“While pulmonary [lung] symptoms have improved on modulator therapy, other symptoms (pain, fatigue, irritability, worrying) may not have improved as much,” wrote the researchers, who emphasized that, while assessing individual symptoms can identify areas where care is needed, how combinations of symptoms may affect life quality should be considered too. “Future analyses are needed to explore the association between symptom clusters (two or more concurrent symptoms that are stable over time and are linked by an underlying mechanism) and quality of life,” they said.