Bone disease and cystic fibrosis
As we get older our bones become thinner and weaker, and may be more likely to fracture. In people with cystic fibrosis (CF) this can happen at an earlier age.
Osteopenia and osteoporosis in cystic fibrosis
Osteopenia and osteoporosis are terms that mean bones are ‘thinner’ than they should be. A more scientific description is that the skeleton has a low bone mass, which means that the bones are more fragile and more likely to fracture.
What is bone mineral density?
Bone mineral density (BMD) is a measure of bone mineralisation. Bone mineralisation is the level of minerals contained in bone. These levels are an indicator of the bone mass and therefore how strong the bones are.
How are bones affected by cystic fibrosis?
People with CF might experience early thinning of the bones. BMD is usually normal in children with CF with a healthy BMI and lung function. Even so, many people with CF fail to gain bone normally or experience premature bone loss when they are teenagers.
About one-third of adults with CF have low BMD, which may mean they are more likely to get bone fractures.
What causes low bone mineral density in cystic fibrosis?
There are many reasons that people with CF might have low BMD, including:
- Poor overall health
- Poor nutrition
- Low physical activity
- Delayed puberty
- CF-related diabetes
- The CF gene
What is a BMD measurement?
BMD measurements compare your bone density with the bone density expected for a young, healthy adult or a healthy adult of your own age, gender and ethnicity. BMD results can be reported as ‘Z-scores’ or ‘T-scores,’ but Z-scores are usually the most appropriate method in people with cystic fibrosis.
How is bone mineral density measured?
BMD is usually measured by ‘dual energy X-ray absorptiometry’ (DEXA) scans. DEXA scans are used to measure BMD in the lumbar spine (below the chest and above the pelvis), the top of the leg (hip), the wrist, and the whole body.
How can low BMD by prevented and treated?
Screening for low BMD at regular DEXA scans and chest X-rays.
- Monitoring and treating low vitamin D and calcium levels.
- Improving nutrition.
- Ensuring people with CF have access to a specialist dietitian.
- Preventing and treating lung infections.
- Encouraging weight-bearing physical activity.
- People with CF being provided with appropriate physical activities by their CF physiotherapist.
- Keeping Glucocorticoid treatment (prednisolone and inhaled steroids) to a minimum.
- Looking for and treating delayed puberty and low blood levels of testosterone and oestrogen.
- Potentially avoiding contraceptive injections and progesterone-only contraception
- Avoiding smoking and alcohol.
How do bisphosphonates help low bone mineral density?
Bisphosphonates are drugs that reduce bone breakdown. Studies in the general population have shown these drugs to help with the treatment of postmenopausal osteoporosis, osteoporosis in men and glucocorticoid-induced osteoporosis. There is also increasing evidence that they may be effective in adults with cystic fibrosis.
Download our bone health factsheet for detailed information about bone health in cystic fibrosis
Joint pain and arthritis in cystic fibrosis
Five-to-ten percent of people with cystic fibrosis will develop joint pain or joint disease (arthropathy) such as arthritis, with the risk increasing with age. The most common form of joint pain in CF is an arthritis that affects large joints, such as the knees, ankles, wrists, elbows and shoulders.
Arthritis in CF tends to come and go, with no symptoms between attacks. Joint pain can flare up during infections and periods of ill health.
Find out more about our joint pain Strategic Research Centre.
Our bone health factsheet
This factsheet describes how CF can affect bone health, what this means for people with CF, and ways to prevent and treat thinning bones.
Research we fund
We fund research to tackle some of the most pressing issues in CF today. Find out how your donations are making a difference.
What is CF?
Cystic fibrosis, or CF, affects the lungs, digestive system and other organs, and there are over 10,600 people living with it in the UK.
Get in touch with us to speak to someone on our Helpline, find out about an event or speak to our Press Team.