A questionnaire survey in 367 patients with hereditary epidermolysis bullosa / 中华皮肤科杂志

ABSTRACT

Objective To investigate the nursing care burden,medical assurance and comorbidities of patients with hereditary epidermolysis bullosa (EB) in China.Methods From September to December in 2018,a cross-sectional telephone/internet-based questionnaire survey was conducted in EB patients registered at DebRA China between January 2013 and September 2018.Results Totally,377 participants (EB patients or their parents) were enrolled into this survey,and a total of 367 valid questionnaires were collected.Of the 367 registered patients with EB,275 (74.9％) were minors,and 319 (92％) reported wound care costs per month less than 3 000 RMB Yuan,and 211 (57.5％) reported that medical expenses were mainly paid by themselves.These patients were distributed in 30 provinces,autonomous regions or municipalities directly under the central government of China,and no patient was from Tibet.The largest number of registered patients was from Jiangsu province (49/367),followed by Henan (35/367),Guangdong (27/367),Hebei (23/367) and Shandong (20/367) provinces.Of the 367 patients,168 received genetic testing.The most common comorbidities of EB patients were nail peeling and itching,with the prevalence rate being 66.2％ and 55.3％ respectively.Disabilities occurred in 122 (33.2％) patients,102 (27.8％) of which had physical disabilities.Conclusion Most of EB patients cannot obtain appropriate diagnosis,nursing care and medical assurance,comorbidities and disabilities are common,and their quality of life is very low.