Department of Pediatrics, PGIMSR and ESIC Model Hospital, Basaidarapur, New Delhi, India.
Department of Pediatrics, Maulana Azad Medical College and associated Lok Nayak Hospital, New Delhi, India.
Indian J Pediatr. 2023 Dec;90(12):1227-1231. doi: 10.1007/s12098-023-04595-9. Epub 2023 May 3.
Thalassemia is one of the most common hemoglobinopathies affecting a large number of people in India and other countries of South-East Asia. For patients with most severe form of the disease- Transfusion Dependent Thalassemia (TDT), stem cell transplantation or gene therapy are only curative treatment which are not available to most of the patients because of lack of experts, financial constraints and lack of suitable donors. In such situations, most cases are managed with regular blood transfusion and iron chelation therapy. With this treatment, over the years, survival of the patients has improved and 20-40% cases are entering into adulthood. In the absence of structured transition of care programs, currently most adult TDT patients are being managed by pediatricians. This article highlights the need for transition of care for TDT patients, barriers to transition and how to overcome the barriers and process of transition of care to adult care team. The importance of empowering the patients in self-management of the disease and educating the adult care team to achieve the desired outcome of transition program is highlighted.
地中海贫血是影响印度和东南亚其他国家许多人的最常见血红蛋白病之一。对于大多数严重形式的疾病——输血依赖性地中海贫血(TDT)患者,干细胞移植或基因治疗是唯一的治愈方法,但由于缺乏专家、经济限制和缺乏合适的供体,大多数患者无法获得这些治疗方法。在这种情况下,大多数病例都通过定期输血和铁螯合疗法来治疗。随着这种治疗方法的应用,多年来,患者的生存率得到了提高,有 20-40%的病例进入了成年期。由于缺乏有组织的过渡护理计划,目前大多数成年 TDT 患者由儿科医生管理。本文强调了 TDT 患者过渡护理的必要性、过渡的障碍以及如何克服障碍和过渡到成人护理团队的过程。强调了赋予患者自我管理疾病的能力和教育成人护理团队以实现过渡计划预期结果的重要性。