Tabor Aaron, LeQuang Jo Ann K, Pergolizzi Joseph
Research, No Baby Blisters, Colorado Springs, USA.
Scientific Communications, NEMA Research, Inc., Naples, USA.
Cureus. 2024 Feb 7;16(2):e53774. doi: 10.7759/cureus.53774. eCollection 2024 Feb.
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.
大疱性表皮松解症(EB)是一种罕见的遗传性疾病,其特征是由于真皮和表皮之间的粘附受损而导致皮肤脆弱。EB在出生时或出生后不久就会出现。目前尚无治愈方法,治疗主要是支持性的。EB患儿患鳞状细胞癌的风险较高。在理想情况下,EB患者受益于能够提供最先进治疗的跨学科护理团队。但在现实中,尤其是在欠发达国家,护理可能会受到限制。在所有情况下,照顾EB患者的家庭在护理方面面临巨大挑战,其中大部分护理工作在家中进行,并且在敷料、设备、交通和自付费用方面承担巨大的经济开支。虽然研究团队正在努力寻找治愈EB的方法,但世界各地治疗EB患者的临床医生已经找到了一些实用且相对便宜的小贴士,以使EB患者的生活更轻松。NoBabyBlisters.org是一个非营利组织,积极为五大洲的EB儿童每月提供医疗用品,并致力于EB研究。该组织创新、开发、收集并在此提供了七项这样实用且可行的建议,这些建议是从其在现实世界中帮助欠发达国家(通常气候炎热)儿童的经验中学到的。这些建议基于在具有挑战性的情况下应对复杂疾病的实际临床经验。这篇短文的目的是为EB患者的护理人员及其亲人提供建议,这些建议可能会使事情变得更轻松并提高生活质量,包括减少水疱和疼痛。