Patients with the more severe forms of epidermolysis bullosa (EB) are at risk of developing osteopenia, osteoporosis and fractures. The cause is likely to be multifactorial and includes reduced mobility, a generally proinflammatory state, poor nutrition and hormonal factors. Monitoring this group with dual energy X-ray absorptiometry (DEXA) scans and plain radiographs is necessary to detect these changes. Data are lacking about the optimal approach to managing poor bone health in EB, although it seems that encouraging mobility, supplementation of calcium and vitamin D where necessary, with the addition of a bisphosphonate when there is evidence of fractures, may be helpful.
The problem of poor bone health in EB
Osteopenia, osteoporosis and fractures may occur in patients with epidermolysis bullosa (EB), particularly in the more severe forms of disease. The incidence of vertebral fractures in these patients is unknown but was observed in 13 of 42 children with severe generalized recessive dystrophic EB (RDEB) at Great Ormond Street Hospital (AE Martinez and JE Mellerio, personal communication, 2009) ( Fig. 1 ). Abnormal bone health in patients with EB may not be surprising as it is well documented in association with other chronic inflammatory diseases of childhood such as juvenile idiopathic arthritis (JIA) and inflammatory bowel disease (IBD). The combination of inactivity with reduced muscle mass, nutritional compromise with altered nutritional use, chronic inflammation, abnormal growth, and pubertal delay is known to alter bone metabolism. These factors hinder the acquisition of peak bone mass during childhood and adolescence, which is an most important determinant of long-term skeletal health.
The effect of reduced mobility on bone health
Physical activity is critical for skeletal development: mechanical loading promotes increases in lean tissue mass, which is directly related to total bone mineral content. Exercise and mobility have been shown to increase bone mass in healthy children. Pain, chronic anemia and contractures all contribute to an often restricted and limited mobility in patients with severe types of EB. In a study of 39 children with different forms of EB (RDEB, junctional EB (JEB), EB simplex Dowling-Meara (EBS-DM)), Fewtrell and colleagues found lower bone mineral density (BMD) in patients with RDEB and JEB compared with patients with EBS-DM and controls, even after correcting for size. The strongest predictor of bone mass was mobility level. Two studies have investigated the effect of vibration therapy in children. Ward and colleagues performed a trial on 20 disabled ambulant children who were randomized to standing on an active or a placebo vibrating device for 10 minutes per day, 5 days a week, for 6 months. Those on treatment had increased tibia and spine trabecular BMD, whereas those on placebo had reduced BMD (measured using a three-dimensional quantitative computed tomography scanner). A second study investigated the effect of 6 months whole-body vibration therapy in a total of 6 immobilized children and reported similar results; vibration therapy improved children’s mobility and muscle power, and increased whole-body BMD. Even though there are limited data on the effectiveness of vibration therapy in children, platforms are well tolerated in children and seem to have a positive effect on bone status, muscle strength, and mobility. In EB, where skin involvement and contractures often limit or reduce mobility and physical activity, vibrating platforms may be a useful therapeutic approach to consider using in future clinical trials.
The effect of reduced mobility on bone health
Physical activity is critical for skeletal development: mechanical loading promotes increases in lean tissue mass, which is directly related to total bone mineral content. Exercise and mobility have been shown to increase bone mass in healthy children. Pain, chronic anemia and contractures all contribute to an often restricted and limited mobility in patients with severe types of EB. In a study of 39 children with different forms of EB (RDEB, junctional EB (JEB), EB simplex Dowling-Meara (EBS-DM)), Fewtrell and colleagues found lower bone mineral density (BMD) in patients with RDEB and JEB compared with patients with EBS-DM and controls, even after correcting for size. The strongest predictor of bone mass was mobility level. Two studies have investigated the effect of vibration therapy in children. Ward and colleagues performed a trial on 20 disabled ambulant children who were randomized to standing on an active or a placebo vibrating device for 10 minutes per day, 5 days a week, for 6 months. Those on treatment had increased tibia and spine trabecular BMD, whereas those on placebo had reduced BMD (measured using a three-dimensional quantitative computed tomography scanner). A second study investigated the effect of 6 months whole-body vibration therapy in a total of 6 immobilized children and reported similar results; vibration therapy improved children’s mobility and muscle power, and increased whole-body BMD. Even though there are limited data on the effectiveness of vibration therapy in children, platforms are well tolerated in children and seem to have a positive effect on bone status, muscle strength, and mobility. In EB, where skin involvement and contractures often limit or reduce mobility and physical activity, vibrating platforms may be a useful therapeutic approach to consider using in future clinical trials.
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