The Cystic Fibrosis Foundation has released updated clinical care guidelines for the screening, diagnosis, management and treatment of vitamin D deficiency in individuals with cystic fibrosis (CF).

The new guidelines were developed by a committee of CF experts, co-chaired by Emory Healthcare endocrinologist Vin Tangpricha, MD, PhD, and are published in the April edition of the Journal of Clinical Endocrinology and Metabolism.  

Vitamin D deficiency is common in individuals with CF due to impaired absorption of fat-soluble vitamins, decreased sunlight exposure and insufficient intake of vitamin D-containing foods and/or supplements.

The CF Foundation established recommendations for vitamin D supplementation

in 2002 and again in 2005 but it quickly became apparent that the amount of vitamin D recommended for treatment and prevention of vitamin D deficiency was insufficient.

According to Tangpricha, associate professor of medicine (endocrinology), Emory University School of Medicine, there are a number of major differences between the old and the updated guidelines.

“This is a step-wise approach in the dosing of vitamin D. The previous guidelines only had one dose of vitamin D that was continuously repeated if levels were low,” says Tangpricha.

Tangpricha says the most interesting change is the emphasis on the prevention of vitamin D deficiency versus the treatment of vitamin D deficiency.

“The key is that we want to make sure that all patients with cystic fibrosis get enough vitamin D so they are not deficient rather than waiting for them to become deficient and then treating them,” he explains.

He also says the new guidelines recommend vitamin D3 as opposed to vitamin D2. It also offers clear recommendations on when vitamin D status should be determined and when follow-up levels should be performed.

Vitamin D deficiency in CF has been associated with decreased bone mass in children, failure to achieve expected peak bone mass in young adults, and osteoporosis in mature adults, and it may impact other serious illnesses common in people with CF.

According to the CF Foundation, cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States. A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. It also obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF.