PDF(Pubmed)
Abstract：
Epidermolysis bullosa (EB) is a rare genetic condition characterized by fragile skin caused by impaired adhesion between the dermis and epidermis. EB is present at or near birth. There is no cure and treatments are supportive. Children with EB are at elevated risk of squamous cell cancer. Under ideal circumstances, EB patients benefit from interdisciplinary care teams who can offer state-of-the-art treatments. In reality and particularly in less-developed nations, care can be limited. In all cases, families dealing with a member with EB face great challenges in caregiving, much of which is managed at home, and incur great financial expenses for dressings, equipment, transportation, and out-of-pocket expenses. While research groups are working to find a cure for EB, clinicians working with EB patients around the world have found practical and relatively inexpensive tips to make life easier for people with EB. NoBabyBlisters.org, a nonprofit organization actively supplying monthly medical supplies for EB children on five continents and working on EB research, has innovated, developed, collected, and now offers here seven such practical and actionable items learned from its experience in the real world assisting children in less-developed nations, typically with hot climates. These are based on real-world clinical experience dealing with a complex disorder under challenging circumstances. The goal of this short paper is to provide advice to EB caregivers and their loved ones that may make things easier and enhance quality of life, including blister and pain reduction.
摘要：
大疱性表皮松解症（EB）是一种罕见的遗传病，其特征是真皮和表皮之间的粘附受损导致皮肤脆弱。EB在出生时或接近出生时存在。没有治愈方法，治疗是支持性的。EB患儿患鳞状细胞癌的风险较高。在理想情况下,EB患者受益于跨学科护理团队，他们可以提供最先进的治疗方法。在现实中，特别是在欠发达国家，护理可以是有限的。在所有情况下,与EB成员打交道的家庭在护理方面面临巨大挑战，其中大部分是在家里管理的，并为敷料产生巨大的财务费用，设备,交通运输,和自付费用。虽然研究小组正在努力寻找治疗EB的方法，世界各地治疗EB患者的临床医生发现了实用且相对便宜的技巧，可以使EB患者的生活更轻松。NoBabyBlisters.org,一个非营利组织，积极为五大洲的EB儿童提供每月医疗用品，并致力于EB研究，创新，开发,收集,现在在这里提供了七个从现实世界中帮助欠发达国家儿童的经验中学到的实际和可操作的项目，通常气候炎热。这些都是基于现实世界的临床经验，在具有挑战性的情况下处理复杂的疾病。这篇短篇论文的目的是为EB照顾者及其亲人提供建议，使事情变得更容易，并提高生活质量。包括水泡和减轻疼痛。
