Follow-Up and Community Care of the Child With a Chronic Disease

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DOI :10.26650/B/CH32.2024.010.025   IUP :10.26650/B/CH32.2024.010.025    Full Text (PDF)

Follow-up and Community Care of the Child with Hemophilia

Ayşegül Ünüvar, Serap Karaman

Hemophilia is a chronic disease that is caused by a hereditary deficiency or the absence of coagulation factors VIII and IX that are crucial in the blood coagulation system. It is X-linked recessive, that is, females are usually carriers, and males manifest the symptoms. However, some children do not have a family history, and it is caused by de novo mutation. Hemophilia can be severe, moderate, and mild. Trauma-related or unrelated bleeding events of different severity levels may occur in different organs. Intra-articular hemorrhage is one of the most crucial problems; if untreated, disability is inevitable. Treatment is prophylactic and/or on-demand, and the primary aim is to prevent bleeding. Regular factor therapy as prevention is essential for patients with severe bleeding phenotype to lead a healthy life. Given the life-long persistence of the disease, patients should adhere to treatment throughout life. Accordingly, a multidisciplinary approach is very important. Compliance with the treatment initially starts in the family, and as the child grows, it should continue at school age, adolescence, and adulthood. Parents, children, and teachers should know the treatment approach in emergencies. Teachers should be properly informed about the disease and should explain the disease to other students correctly. It may be necessary to seek help from the healthcare team. An overprotective attitude can lead to a lack of self-confidence in the child, and the child may feel lonely. Physician-family relationship is very important for children with hemophilia to have normal mental and physical health and be beneficial to society. Therefore, they should be supported psychologically and socially at all ages, as in other chronic diseases.


Keywords: Chronic disease, hemophilia, bleeding, factor, follow-up
DOI :10.26650/B/CH32.2024.010.025   IUP :10.26650/B/CH32.2024.010.025    Full Text (PDF)

Hemofi̇li̇si̇ Olan Çocuğun İzlemi̇ ve Toplumda Bakımı

Ayşegül Ünüvar, Serap Karaman

Hemofili, kanın pıhtılaşmasında görev alan pıhtılaşma faktörü VIII ve faktör IX’un kalıtsal eksikliği ya da yokluğu sonucunda meydana gelen kronik bir hastalık olup X’e bağlı resesif geçişlidir. Bu nedenle genellikle kızlar taşıyıcı, erkekler hastadır. Ancak bazı hastalarda aile öyküsü olmayıp, de novo mutasyon sonucu hastalık ortaya çıkar. Hastalığın, ağır, orta ve hafif tipleri mevcuttur. Farklı şiddette ve farklı organlarda, travma ilişkili ya da ilişkisiz kanamalar görülebilir. En önemli sorunlardan biri eklem içi kanamalar olup tedavi edilmediği takdirde sakatlık kaçınılmazdır. Tedavi, kanamayı önleyici ve/veya kanamayı tedavi edici şeklindedir. Öncelikli amaç kanamaları önlemektir. Ağır kanama fenotipi olanlara koruyucu olarak düzenli faktör tedavisi verilmesi, hastaların sağlıklı bir yaşam sürmeleri için şarttır. Hastalığın ömür boyu sürmesi nedeniyle, tedaviye uyum da ömür boyu devam etmelidir. Bunun için multidisipliner yaklaşım çok önemlidir. Tedaviye uyum öncelikle ailede başlamalı, çocuk büyüdükçe oyun çağı, okul çağı, ergenlik ve erişkin dönemde de devam etmelidir. Acil durumlarda tedavi yaklaşımı, anne, baba, çocuk ve okul yönetimi tarafından bilinmelidir. Öğretmen hastalık hakkında doğru bilgilendirilmeli ve diğer öğrencilere hastalığı doğru anlatmalıdır. Bu konuda sağlık ekibinden yardım alınması gerekebilir. Aşırı koruyucu tutumlar, çocukta özgüven eksikliğine yol açabilir ve çocuk giderek yalnız kalabilir. Hemofilisi olan bireylerin bedenen ve ruhen sağlıklı olması ve topluma yararlı birey olarak kazandırılması için hekim-hasta-aile ilişkisi çok önemlidir. Bu nedenle hemofilisi olan bireyler, diğer kronik hastalıklarda olduğu gibi her yaşta psikolojik ve sosyal yönden desteklenmelidirler.


Keywords: Kronik hastalık, hemofili, kanama, faktör, izlem

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