Bone Abstracts

Fibrodysplasia ossificans progressiva (FOP; OMIM #135100) begins in childhood and leads to irreversible restriction of movement, functional impairment, and shortened life-span. Individuals with FOP develop progressive limitations in chest expansion, resulting in restrictive lung disease. Current management guidelines published in 2011 (1) highlight that Influenza may be a causative factor for FOP flare-ups, and can also cause potentially deadly cardiopulmonary complications, especially in individuals with severe restrictive chest wall disease. It is therefore recommended that patients with FOP should consider receiving influenza immunisations annually, and that unaffected household members of patients with FOP should also consider annual immunisations to decrease the risk of spreading the influenza to highly susceptible FOP patients. The aim of this of this study is to investigate the vaccination rates of UK patients with FOP and their household contacts. Telephone interviews were conducted with patients with FOP or their parents/guardians. All patients were resident in the UK and attended a single specialist centre. We interviewed 17 of 20 patients (85%). Of these 10 were children (age < 18 years) and 7 adults (age ≥ 18 years). Seasonal influenza vaccination rates were 23.5% (n=4) in children and 11.7% (n=2) in adults. The main reasons for not having the vaccine were: anxiety about the injection causing a flare of the disease (n=9, 52.9%), recurrent flares of the disease (n=3, 17.6%), and previous administration of influenza vaccine via the intramuscular route rather than subcutaneous, resulting in an acute flare (n=2, 11.7%). Influenza vaccination rates among household contacts (n=39), including parents, partners, children or siblings were 71.4% (n=5) in children and 31.2% (n=10) in adults. This study highlights the need to provide education to individuals with FOP and their close contacts regarding the importance of influenza vaccination. There may also be an added role for education of the primary care/family physician involved in their care. In FOP individuals who are not vaccinated, consideration should be made to have access to antiviral medication if they develop influenza.

Disclosure: The authors declared no competing interests.

Reference
1. The Medical Management of FOP: Current Treatment Considerations. Clin Proc Intl Clin Consort FOP 2011 4 1–100.