Cystic Fibrosis or CF is an inherited disease that affects the normal movement of salt (sodium chloride) into and out of certain cells, including those that line the lungs and pancreas. This results in thick, sticky mucus and other secretions. The mucus clogs the lungs, causing breathing problems. It also provides a breeding ground for bacteria to grow. This leads to frequent lung infections, which eventually damage the lungs and contribute to early death.
Thick digestive fluids also may clog ducts leading from the pancreas to the small intestine. This prevents the fluids from reaching the small intestine, where they are needed to digest food. This can cause digestive problems and slow growth. Some CF patients also suffer from poor liver function which may eventually become chronic liver disease.
Malabsorption of fat soluble vitamins is likely in most patients with cystic fibrosis, especially for those who are pancreatic insufficient. Vitamins A, D and E have been found to be deficient in early diagnosed infants as well as older patients. Different levels of these vitamins are prescribed depending on the age of the patient, whether the patient is pancreatic insufficient or sufficient and other health factors that can be affected by cystic fibrosis. Vitamin K may also be prescribed under certain conditions.
The recommended daily supplements which usually achieve normal plasma levels in infancy are vitamin A 4000 iu (120 mcg), vitamin D 400 iu (10 mcg) and vitamin E 37 – 75 iu (25 – 50 mg). The recommended doses for children over 1 year of age are vitamin A 8000 iu, vitamin D 800 iu and vitamin E 100 – 200 mg.
These doses are considerably higher than the usual dietary intake and generally are adjusted to meet the needs of the patient as they get older. Vitamin K is generally prescribed for those cystic fibrosis patients who suffer from liver disease or who are going to undergo a surgical procedure, or whose blood tests do not me certain standards.
* Vitamin A: Vitamin A deficiency may cause night blindness in older patients and can progress to severe xerophthalmia if not checked.
* Vitamin D: Vitamin D deficiency may cause rickets which is very rare and osteomalacia. Although in the past there is little clinical evidence of vitamin D deficiency is rare in cystic fibrosis new research and studies osteoporosis, osteopenia and low levels of vitamin D metabolites are being increasingly recognized in children and adults with cystic fibrosis.
* Vitamin E: Vitamin E deficiency may cause neurological problems in older CF individuals. Correction of vitamin E deficiency improves hemoglobin levels. Vitamin E is an antioxidant and protects cell membranes from oxidative damage. Because of this role vitamin E may be important in controlling the progression of lung disease in cystic fibrosis.
Recent studies have suggested that cystic fibrosis patients have inadequate antioxidants defenses to cope with certain stresses on their system. Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress and higher levels of supplementation may be required.
* Vitamin K: Regular vitamin K supplements are not given unless there is chronic liver disease, a prolonged prothrombin time or an upcoming surgical procedure. Given any of these conditions an oral daily supplement of 5-10mg is given for a week prior to the procedure. Vitamin K is required for the formation of osteocalcin which is involved in bone metabolism.
As you can see vitamin supplementation is essential for Cystic Fibrosis patients. It is crucial for you to discuss any changes to your current vitamin regimen with your physician prior to adding, removing or changing the doses of any vitamins. The balance of vitamins in your system can be very delicate and should be closely monitored by your physician.
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