Kelebek Hastası Çocuklarda Günlük Yaşam Sorunları ve Çözüm Önerileri: Mühendislik Bakış Açısı

Abstract

Amaç: Nadir hastalıklar, toplumda çok az kişide görülen hastalıklardır. Nadir hastalıklardan biri olan epidermolizis bülloza, yaygın adıyla kelebek hastalığı veya lupus, genetik bir deri bozukluğudur. Hastalıkta, yapısal proteinleri kodlayan genlerdeki mutasyonlar nedeniyle cildin dayanıklılığını sağlayan proteinler doğru şekilde üretilemez, bu da cildi son derece kırılgan hale getirir. Hasta çocukların bakım ihtiyaçları, yalnızca tıbbi tedaviyi değil, aynı zamanda kapsamlı psikososyal, gelişimsel ve etik yönetim gerektiren çok boyutlu bir yapıdır. Bu ihtiyaçlar, çocuğun kronik hastalığının tanısından başlayarak yaşam sonu sürecine kadar bir süreklilik içinde ele alınmalıdır. Bu derleme, kelebek hastası çocuk hastaların kapsamlı bakım gereksinimleri ve günlük yaşam sorunlarını, mühendislik bakış açısıyla ele alınmayı ve konuyla ilgili farkındalık yaratmayı amaçlamaktadır. Yöntem: Çalışma, literatür taraması ile hazırlanmış bir derlemedir. Ulusal ve uluslararası makalelerde konu ile ilgili makaleler incelenerek, kelebek hastası çocuklarda yaşam kalitesindeki değişiklikler ve mühendislik bakış açısı ile ele alınmıştır. Bulgular: Kelebek hastalığı olan çocuklarda günlük yaşamda en sık karşılaşılan sorunlar cilt ve yara bakımı, el deformiteleri, uyku ve basınç bölgelerinin korunması, güneşe karşı aşırı duyarlılık, ağız içi lezyonları içermektedir. Sonuç: Kelebek hastası çocuklarda günlük yaşam sorunlarının giderilmesinde ve yaşam kalitesinin arttırılmasında mühendislik açısından geliştirilebilecek ürünler yararlı olacaktır.

Keywords

• Kelebek hastalığı
• nadir hastalıklar
• çocuk yaşam kalitesi

Supporting Institution

Destekleyen kurum bulunmamaktadır.

Daily Living Problems and Solutions for Children with Epidermolysis Bullosa: An Engineering Perspective

Abstract

Objective: Rare diseases are illnesses that affect very few people in the population. Epidermolysis bullosa, commonly known as lupus or butterfly disease, is a genetic skin disorder. In this disease, due to mutations in the genes encoding structural proteins, the proteins that provide skin resilience cannot be produced correctly, making the skin extremely fragile. The care needs of affected children are a multifaceted structure requiring not only medical treatment but also comprehensive psychosocial, developmental, and ethical management. These needs should be addressed in a continuum from the diagnosis of the child's chronic illness to the end-of-life process. This review aims to address the comprehensive care needs and daily living problems of children with lupus from an engineering perspective and to raise awareness on the subject. Method: This study is a review prepared through a literature review. Articles on the subject in national and international publications were examined, and changes in the quality of life of children with lupus were addressed from an engineering perspective. Findings: The most common daily problems encountered by children with epidermolysis bullosa (butterfly disease) include skin and wound care, hand deformities, protection of sleep and pressure points, hypersensitivity to sunlight, and oral lesions. Conclusion: Engineering-developed products that can address daily living problems and improve the quality of life for children with epidermolysis bullosa would be beneficial.

Keywords

• Epidermolysis bullosa
• rare diseases
• children quality of life

Ethical Statement

There is no supporting organization.

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