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Low bone mineral density

The problem

Children and teenagers with CF have increased risk of low bone mineral density, which can lead to early osteoporosis in about 25% of young adults with CF with an increased risk of fractures, especially vertebral crush fractures. Risk factors are:

• Poor nutritional state
• Frequent or persistent chest exacerbations
• Frequent or long term systemic steroid use
• Low levels of vitamin D and calcium
• Low levels of weight-bearing exercise
• Delayed puberty
• Possibly low vitamin K levels (not proven)

Diagnosis

There are rarely any symptoms. Fractures that occur with less than the expected degree of force should raise concerns. Diagnosis is otherwise based on DEXA scans. The CF Trust recommend that these are done from 10 years of age, and the Brompton do them from 8 years of age. We are currently doing them from 14 years of age, unless there is a history of long term steroid use, or easy fractures.

Management

The reason we do not screen at an earlier age is that intervention is currently limited to doing things we would try and achieve in all chidren, which is to:

• optimise nutritional support
• minimise systemic steroid use
• optimise vitamin D and calcium status
• encourage weight-bearing exercise

One specific intervention that would do recommend is to:

• start vitamin K as menadiol phosphate 10mg/day

Bisphosphonate use is currently reserved for children with a low bone density (-2SD or worse) who have not responded to the measures described above and who have a history of easy fractures or for children who are waiting for lung transplant.