Medication
Cystic Fibrosis affects the lungs and the digestive tract - these areas are likely to require medication.
Lungs
Medication can be administered in various ways: inhaled into the lungs using nebulisers, taken orally, or taken intravenously.
These drugs treat the lungs in the following ways:
- Bronchodilator drugs open the airways by relaxing the surrounding muscle. They relieve tightness and shortness of breath.
- Antibiotics help to treat or control persistent infection.
- Steroids reduce inflammation in the airways.
Digestive System
Cystic Fibrosis affects the pancreas, so enzyme replacement capsules should be taken with meals and snacks to replace pancreatic enzymes and enable people with CF to gain more energy from the food they eat.
Pancreatic Enzyme Insufficiency
Pancreatic enzyme insufficiency is one of the common symptoms of CF. The CF gene defect results in a thickening of the pancreatic secretions. The accumulation of thick secretions causes obstruction and eventually leads to damage of the pancreas. Pancreatic enzyme insufficiency prevents digestion and absorption of nutrients. Large, bulky, smelly stools containing undigested nutrients are a direct result of a lack of pancreatic enzymes.
Pancreatic enzyme insufficiency is treated with pancreatic enzyme replacement capsules, which should be taken with every meal or snack. Dosing can be based on the fat content of the food items. Foods containing only carbohydrate, such as fruit, juices and boiled lollies will not require pancreatic enzyme replacement capsules.
Pancreatic Enzyme Replacement Therapy (PERT)
Specially coated enzyme beads (microspheres) have been a treatment breakthrough for people with CF. Effective and acceptable enzyme replacement therapy has been a significant factor in maintaining health for those with CF.
For infants and younger children, who cannot swallow capsules whole, the capsules can be opened and the enteric coated microspheres mixed with fruit gel. This is then fed to the infant before and during their feed. Older children can have the microspheres mixed with yoghurt or pureed fruit.
Dosage Of Enzymes
The physician and dietitian will monitor pancreatic enzyme therapy dosing. The dietitian will provide information on the correct dosage of enzymes for different foods.
Enzyme Storage
Australia has a unique climate which is not suited to preserving the potency of enteric coated microspheres. Parents and adults with Cystic Fibrosis need to be aware that this is costly medication and needs to be handled carefully. The Pancreatic Enzyme Replacement Capsules need to be stored below 250C, and kept in the dark and dry. They do not like to be left in the car. During summer, the preparations can go off quickly.
CREON® - Solvay Pharmaceuticals (available in several strengths)
PANZYTRAT - Technipro
COTAZYM® - Organon – Discontinued in Australia
PANCREASE - Janssen-Cilag - Discontinued in Australia December 2005
Your dietitian or doctor can advise you on the appropriate type/dosage of enzyme supplement
See also Nutritional Requirements for People with CF
Other Organs
Bones can be effected by a lack of minerals, which can cause osteoporosis (weak/brittle bones). Bisphosphonates, which are used to treat osteoporosis in post-menopausal women, have been shown to be beneficial for the treatment of osteoporosis in CF too. Research is being carried out to investigate the benefits of high doses of vitamin D and calcium.
If someone with CF is having liver problems, they will be treated in the same way as other people with this condition. Promising results have been reported following early treatment with ursodeoxycholic acid.
