Patel Archana B, Pathan Habib G
Indira Gandhi Medical College, Nagpur, India.
Indian J Pediatr. 2005 Jul;72(7):567-71. doi: 10.1007/BF02724180.
To identify specific domains and traits that are most affected in patients with sickle cell anemia and traits with respect to normal children.
Children attending the regional hemoglobinopathy center at IGMC, Nagpur in age group of 8-14 years were assessed. Of 52 children studied, 25 had sickle cell anemia (SCA), 12 had sickle cell trait (SCT) and 15 wre normal control. The (quality of life (QOL) was assessed using multidimensional interview based questionnaire.
All domains, physical, psychosocial, cognitive and morbidity were affected. In SCA playing and mobility were most affected. There was feeling of sadness or disinterest and lack of support from teachers. The school attendance, vocational achievement perception, entertainment and participation in cultural activities were also affected. The intensity of weakness and pain was greater in SCA children who left that they were affected by a major illness. The unusual finding was that the SCT children also showed affection of all domains as compared to normal children, which was perhaps due to the stigma of the disease.
QOL is affected in children with sickle cell disease (SCD) and to a lesser extent in SCT. Interventions to improve QOL should target the affected items. Improving awareness of the disease and its manifestation will help to alleviate the psychosocial affliction of children with SCT.
确定镰状细胞贫血患者及镰状细胞性状患者相对于正常儿童受影响最严重的特定领域和特征。
对在那格浦尔IGMC地区血红蛋白病中心就诊的8至14岁儿童进行评估。在研究的52名儿童中，25名患有镰状细胞贫血（SCA），12名患有镰状细胞性状（SCT），15名是正常对照。使用基于多维访谈的问卷评估生活质量（QOL）。
所有领域，包括身体、心理社会、认知和发病率均受到影响。在SCA中，玩耍和活动能力受影响最大。存在悲伤或无兴趣的感觉，且缺乏教师的支持。学校出勤率、职业成就认知、娱乐和参与文化活动也受到影响。与认为自己受到重大疾病影响的SCA儿童相比，虚弱和疼痛的程度更大。不同寻常的发现是，与正常儿童相比，SCT儿童的所有领域也受到影响，这可能是由于疾病的污名化。
镰状细胞病（SCD）患儿的生活质量受到影响，SCT患儿受到的影响较小。改善生活质量的干预措施应针对受影响的项目。提高对该疾病及其表现的认识将有助于减轻SCT患儿的心理社会痛苦。
