Analysis: New research indicates that quality as well as quantity matters when it comes to what people with cystic fibrosis eat
Ireland has the highest incidence of cystic fibrosis worldwide, with approximately 1,650 people living with the condition in the country. Treatments for cystic fibrosis have rapidly progressed in recent years resulting in patients living longer, fuller lives.
What is cystic fibrosis?
Cystic fibrosis is a genetic condition which is traditionally associated with persistent lung infections and low lung function, poor absorption of nutrients, malnutrition and a resulting shorter lifespan. Over time, however, improvements in clinical practices and treatment advancements have reduced the incidences of these conditions and increased the life expectancy of people living with cystic fibrosis. Specifically, new drug therapies including Kaftrio, Kalydeco and Orkambi have significantly improved lung function and nutritional status in many people with cystic fibrosis.
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From RTÉ Radio 1's Today With Claire Byrne, Brian O'Connell reports on access to cystic fibrosis drug Kaftrio
With these medical advancements, researchers have begun exploring new issues that are becoming more prevalent for people living with cystic fibrosis as the more common and familiar symptoms are often less of a focus for many.
Diet and cystic fibrosis
Since the 1980s, people living with cystic fibrosis have been encouraged to follow a diet that is generally high in calories and total fat to meet energy targets and to optimise growth and health outcomes. While this diet was considered optimal for the growth and survival of people with cystic fibrosis for many decades, it tends to be associated with higher intakes of saturated fat, sugar, and salt, which are known risk factors for cardiovascular disease in the general population.
The most up-to-date research has indicated that the diets of adults living with cystic fibrosis are generally of poor quality. This may lead to increased rates of diet-related chronic diseases like obesity, type II diabetes and cardiovascular disease, similar to the general population.
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From RTÉ Radio 1's Miriam Meets, interview with CF campaigner Orla Tinsley
However, the nutrition landscape has drastically changed as healthcare practices and treatments have moved forward. Although reaching the ideal body weight remains a focus for many, increased rates of overweight and obesity are now being observed in adults with cystic fibrosis (40.9% overweight/obese) with the rate increasing from 0.85% per annum between 2002 (14.9% overweight/obese) to 2018 (28.5% overweight/obese), to 3.3% per annum since 2018.
For many of those with cystic fibrosis, general population dietary guidelines are now advised, focusing on whole of diet and diet quality to promote health and wellbeing.
What is meant by diet quality?
Diet quality refers to a diet that has diversity across key food groups that form the basis of dietary guidelines like fruit and vegetables, grains, dairy, protein foods, fats, spreads and oils, and discretionary foods. A diet that has higher diet quality has nutrient intakes that are more desirable, while also exhibiting a lower risk of diet related chronic disease development.
This would halt diet related chronic diseases like obesity and diabetes which have increased notably within the cystic fibrosis population in recent years
A recent review comprehensively evaluated literature on the dietary intakes of adults living with cystic fibrosis and showed that foods which are energy-dense, but nutrient-poor are used to meet required energy targets. Moreover, it revealed a reliance on the use of nutritional supplements to achieve the majority of vitamin and mineral targets. This review found that "dietary guidelines" need revision, alongside "practice change" in "cystic fibrosis nutritional therapy" to "optimise nutrition and health outcomes".
The findings are not surprising, given the diet traditionally recommended for people with cystic fibrosis promoted higher energy and fat requirements. Adults living with cystic fibrosis reported to be overconsuming total fat, saturated fat, sugar and added sugar when compared to best practice nutrition guidelines.
What can be done to change this?
The results demonstrate that such dietary approaches may need to be revised for the majority of people with cystic fibrosis. This would halt the progression of diet related chronic diseases like obesity, cardiovascular disease and type-II diabetes which have increased notably within the cystic fibrosis population in recent years.
Furthermore, the diets align to that of the Irish general population, with the Healthy Ireland Survey published in 2021 showing that Irish diets are similarly high in fat, sugar and salt, relying heavily on discretionary foods and having suboptimal intakes of fruit and vegetables. Alternative dietary approaches have yet to be assessed in the cystic fibrosis population, although diets like the Mediterranean diet have proven helpful in reducing the risk of developing diet-related chronic diseases in the general population.
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