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the effects of CF on bone density.
Normal: Normal bone is composed of a meshwork of collagen and calcium.
With CF: The bones of patients with CF are less dense leading to osteoporosis. |
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Muscle
Muscle weakness, particularly in the arms, legs and diaphragm, is common in CF patients. Malnutrition contributes greatly to muscle dysfunction. Chronic illness leads to reduction in muscle mass and strength, and impaired regulation of electrolytes, the hallmark of CF, also contributes to muscle weakness. Medications used to treat CF complications, like steroids, may worsen underlying muscle weakness. Proper nutrition and regular exercise help maintain conditioning and retain muscle mass. Bone
Adult CF patients are at a high risk for reduced density of their bones, known as osteoporosis. Up to 77 percent of adult patients will develop the condition. Thinning of the bones makes them more prone to fractures that may occur even without trauma. Low levels of vitamin D—due to malabsorption—impair bone formation. CF patients are further prone to low vitamin D levels because they may not receive adequate sunlight exposure required for converting vitamin D into its active form.
This accelerated bone breakdown increases with inactivity, chronic lung disease, and steroid use. Also, inflammation from chronic lung disease may increase bone breakdown. Lung transplant recipients using immunosuppressive drugs like corticosteroids also have an accelerated rate of bone loss. Low levels of sex hormones important to bone development and delayed onset of puberty limit the time for adult bone formation, more often in men. Cystic Fibrosis Related Diabetes, alcohol use and cigarette smoking may also hasten the onset of osteoporosis. CF patients should be screened every two years with a DEXA (Dual-energy radiograph absorptiometry) scan. Using two separate X-rays, it measures bone density at the spine, femur, and other sites. Patients should also have serum vitamin D levels checked yearly along with diabetes screening. Prevention of osteoporosis involves implementation of a health plan that emphasizes diet and weight-bearing exercise. 
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