Respiratory Syncytial Virus in Infants and Children with Prader-Willi syndrome

ABSTRACT BACKGROUND Prader-Willi syndrome (PWS) is a complex disorder affecting approximately 1/15,000-1/30,000 people. Infants with PWS are at risk for serious complications with Respiratory Syncytial Virus (RSV) due to low muscle tone and a weakened pulmonary system. OBJECTIVES Understanding RSV incidence, hospitalization rates, lingering effects, and morbidity in children with PWS may help with planning health care, insurance and vaccine recommendations in children with PWS. METHODS Links to volunteer surveys were provided via direct email and social media to families throughout the United States with children having PWS. The contact distribution lists were provided by PWSA(USA) and the Foundation for Prader-Willi Research. RESULTS A total of 220 surveys were completed by the parents/caregivers of children with PWS. Of those respondents, 60 (27.27%) had contracted the RSV virus during early childhood. Of those with RSV, 44 children required hospitalization, with 16 reporting multiple hospitalizations, some for several weeks. Of those with the virus, 22 required PICU admission, 10 required intubation, 20 needed CPAP, and 46 children needed supplemental oxygen during the infection. Of those who had contracted RSV, 42% were over age 1 year at the time of infection, and 17 children developed chronic lung issues after the RSV infection. The case lethality was 1.37%. Only 38% had received the RSV Synagis shot, and 19% received more than one season of the vaccination. Prematurity prevalence was only 28%, but 99% reported that their child had significant hypotonia. Approximately 30% of parents sited lack of insurance authorization or failure of the physician to recommend the treatment. CONCLUSIONS The risk of contracting RSV for young children with PWS is high. The implications of contracting RSV include death or lung damage, along with high medical expenditures, which could be ameliorated with routine administration of the Synagis vaccine.