PDF(Pubmed)
Abstract：
The heritable condition epidermolysis bullosa (EB) is a rare but potentially devastating and life-threatening condition that is characterized primarily by cutaneous fragility, manifested when the dermis and epidermis fail to adhere properly. EB has no cure, and because of its rarity, few healthcare professionals have experience in treating it. Most families with an EB child are forced to rely on family caregiving which can be disruptive to family routines but, more importantly, place enormous time and emotional and financial burdens on the family. EB can be extremely painful, and families are often caught in the bind of trying to manage overwhelming financial burdens in an effort to help their children cope with excruciating pain. For many years, the nonprofit organization NoBabyBlisters.org has worked on five continents with families caring for EB children. Many of these families reside in under-developed nations with hot climates and limited healthcare resources. Over time, the healthcare professionals with NoBabyBlisters.org have worked with EB families both internationally and in the United States to develop a series of simple tips or \"hacks\" that may provide relief or great benefit to these children. The objective of this article is to share these field-tested tips with a wider audience. This is not a scientific study or a systematic review and is offered as a companion article to an earlier article on the same subject.
摘要：
遗传性疾病大疱性表皮松解症（EB）是一种罕见但潜在的破坏性和危及生命的疾病，其特征主要是皮肤脆性，当真皮和表皮不能正确粘附时表现出来。EB没有治愈方法，因为它的稀有性,很少有医疗保健专业人员有治疗经验。大多数有EB儿童的家庭被迫依靠家庭护理，这可能会破坏家庭日常生活，但是，更重要的是,给家庭带来巨大的时间、情感和经济负担。EB可能会非常痛苦，家庭经常陷入试图管理压倒性的经济负担的困境，以帮助他们的孩子应对痛苦。多年来,非营利组织NoBabyBlisters.org在五大洲与照顾EB儿童的家庭合作。这些家庭中的许多人居住在欠发达国家，气候炎热，医疗资源有限。随着时间的推移,NoBabyBlisters.org的医疗保健专业人员与国际和美国的EB家庭合作，开发了一系列简单的技巧或“黑客”，可以为这些孩子提供救济或巨大的利益。本文的目的是与更广泛的受众分享这些经过现场测试的技巧。这不是科学研究或系统评价，而是作为同一主题的早期文章的配套文章提供。
