During the last decades, medical science has developed significantly. One of the most remarkable achievements is the life extension and quality of life that medical science offered to thousands of patients. Cystic fibrosis is one of the diseases whose patients now live longer and enjoy a better quality of life.
What is Cystic Fibrosis?
Cystic fibrosis belongs to the category of inherited diseases and affects about 11000 people in the UK. (1) It is the most frequent inherited disease and it is caused by a faulty gene, that both parents have. Usually, the parents of a child with cystic fibrosis do not have the disease because they carry only one faulty gene and disease needs two faulty genes to be expressed. In the rare condition where both parents carry the faulty gene, the chance of having a baby with cystic fibrosis is 25% (2)
The disease mainly affects cells in several systems such as the gastrointestinal, hepatobiliary, the respiratory, reproductive tracts and sweat glands. (2) Specifically, cystic fibrosis leads to chronic lung disease, and pancreatic insufficiency, which are the most important complications of the disease.
Symptoms of cystic fibrosis
In most cases symptoms start early in childhood. Some of the most common symptoms of cystic fibrosis are: poor development of the child with low Body Mass Index(BMI), chest infections, coughs, diarrhea, shortness of breath, diabetes, osteoporosis and others. (2)
Treatment and lifestyle changes
As far as the disease is not curable, doctors try to control symptoms, limit complications and generally offer good quality of life to their patients. Besides medicines, techniques and exercises adopted by the patients, help them clear mucus from the lungs. Diet plays a key role in the progress and management of the disease. (2)
Dietary changes that may help
The pancreatic insufficiency that characterizes cystic fibrosis, leads to malabsorption of fat, which is a main component of human diet. To overcome this insufficiency, doctors administer pancreatic enzyme replacement. The standard diet for cystic fibrosis patients, is based on high energy, low fat and high protein intake. (3)
Additionally, due to the pulmonary disease, the energy needs of cystic fibrosis patients are increased between 120-150% compared to individuals of the same age and sex. Furthermore, the protein requirements are increased (120% compared to healthy individuals) due to the increased protein losses through sputum. (3)
Supplements that help
Omega 3 fatty acids
The fat malabsorption may potentially lead to an essential fatty acid deficiency. Studies have shown that patients with cystic fibrosis have low levels of omega-3 fatty acids. (4) In a recently published article, researchers tried to evaluate the dose effect of omega-3 supplementation in cystic fibrosis patients. They concluded that dietary supplementation with omega 3 fatty acids can increase its content in plasma and platelet lipids. (5)
Patients with cystic fibrosis usually have low levels of vitamin E due to the malabsorption caused by the disease. Researchers found that supplementation of omega 3 fatty acids lead to increased vitamin E levels improving the antioxidant potential of the body which is crucial in a disease like cystic fibrosis which is characterized by inflammation. (5)
Moreover, in cases of deficiency of vitamin E, serious consequences can occur like retinal and cognitive deficits, hemolytic anemia and neuromuscular degeneration. (6)
According to a European Consensus, blood testing for Vitamin E deficiency is recommended, because usually, the newly diagnosed patients with cystic fibrosis have low vitamin E levels. Thus, Vitamin E supplementation is recommended for patients with pancreatic insufficiency. (4) Furthermore, the European Society for Clinical Nutrition and Metabolism proposes regular supplementation of vitamin E (a-tocopherol) for cystic fibrosis patients until they reach the normal serum a-tocopherol levels. (6)
Low serum vitamin A levels are usual in patients with cystic fibrosis who do not comply with doctor’s advice or who are untreated. In a published article, researchers reported that hepatic Vitamin A stores in patients with cystic fibrosis were lower than normal and specifically they decreased with age but they were not associated with the severity of their liver disease. (4)
Since Vitamin A levels are negatively associated with inflammation, it is crucial to find out whether these low levels are due to the inflammation or to pure Vitamin A intake. In case the patient complies with his Vitamin A supplementation, then zinc levels evaluation is mandatory. (7)
As mentioned previously, zinc insufficiency may be evidenced by low levels of Vitamin A. Additionally, low child or infant development may be a sign of zinc deficiency in patients with cystic fibrosis. Several other symptoms of cystic fibrosis are associated with zinc deficiency: eye problems, regular infections, delayed sexual maturation, loss of taste etc. Therefore, zinc supplementation is recommended for cystic fibrosis patients who are at risk of zinc insufficiency. (6)
According to a Scandinavian study on cystic fibrosis patients, the mean Vitamin D intake was below the recommended. This was confirmed by an American study reporting that Vitamin D deficiency was significantly increased during winter. (8) A recently published study, showed that Vitamin D supplementation increases significantly the serum levels of the Vitamin and increased doses during the winter resulted in improved Vitamin D levels to a degree comparable to the summer. (9)
According to a literature review published in 2017, probiotics may improve respiratory and gastrointestinal outcomes in patients with cystic fibrosis without any side effects. (10) Another literature review (2016) that examined the effects of probiotic supplementation in children with cystic fibrosis, concluded that probiotics improve gut maturity and function. (11)
A recently published study reported that magnesium in cystic fibrosis is largely overlooked. Cystic fibrosis patients should check yearly their serum magnesium levels and in case of deficiency, they should get magnesium supplementation to reach the desired normal levels. (12)
Cystic fibrosis is a disease that is not curable, but its progress and complications are closely related to nutrition. Patients with cystic fibrosis usually are nutrient deficient due to the malabsorption and the higher nutrient demands. Healthy eating is mandatory but is not always enough and the supplementation of fat-soluble Vitamins like Vitamin E, play a key role in the management of the disease and the adequate nutritional support of the patient.
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3) Katsilambros, N., Dimosthenopoulos, C., Kontogianni, M., Manglara, E. and Poulia, K. (2011). Clinical Nutrition in Practice. 1st ed. Somerset: Wiley.