血友病,由凝血因子缺乏引起的遗传性出血性疾病,影响全世界数百万人。在低收入和中等收入国家(LMICs),受影响者的生活质量(QoL)仍然特别欠佳。本文深入探讨了中低收入国家血友病护理和管理中未满足的需求,突出各种挑战和潜在的改进策略。LMIC的主要挑战之一是获得全面护理的机会有限,其中包括涉及血液学家的多学科方法。物理治疗师,心理学家,和社会工作者。在许多LMICs中,医疗基础设施不足以提供这种综合服务,导致血友病患者的护理分散和健康状况较差。另一个重要问题是预防性治疗的挑战。预防,其中包括定期输注凝血因子浓缩物以防止出血发作,是高收入国家的护理标准。然而,在LMICs中,由于凝血因子浓缩物的高成本和有限的可获得性,预防性治疗通常是不可行的。这导致了对按需治疗的依赖,它仅解决出血事件的发生,并不能防止与频繁出血相关的长期并发症。疼痛管理是另一个存在显著差距的关键领域。由于反复的关节出血导致关节损伤,慢性疼痛是血友病患者的常见问题。在许多LMICs中,获得有效的疼痛管理策略,包括药物和非药物治疗,是有限的。心理健康支持也是血友病护理的一个关键但经常被忽视的方面。这种疾病的慢性性质,加上频繁的医院就诊和疾病造成的身体限制,会导致心理健康问题,如焦虑和抑郁。然而,精神卫生服务在低收入国家经常资源不足,并且对血友病患者的心理健康需求缺乏认识。照顾者的支持在管理血友病患者的日常需求方面发挥着至关重要的作用,是血友病护理的另一个重要组成部分,在LMIC中通常是不足的。许多低收入国家也缺乏对血友病的教育和认识。公众甚至医学界对这种情况的了解往往有限,导致误诊和延误治疗。就业和财政支持也是关键问题。与血友病相关的身体限制和频繁的医疗需求会使个人难以维持稳定的就业,导致财务紧张。在许多LMICs中,社会支持系统不足以应对这些挑战。最后,远程医疗和数字医疗技术的整合提出了一个有前途的战略,以克服这些挑战,提供远程访问专科护理,教育,和支持,这在医疗资源稀缺的地区尤其有价值。通过采取涉及政府之间合作的多方面方法,医疗保健系统,国际组织,和病人倡导团体,有可能应对这些挑战,并显著改善LMIC血友病患者的生活质量.
Hemophilia, a genetic bleeding disorder caused by a deficiency in clotting factors, impacts millions of people worldwide. The quality of life (QoL) for those affected remains particularly suboptimal in low- and middle-income countries (LMICs). This article delves into the unmet needs in hemophilia care and management in LMICs, spotlighting various challenges and potential strategies for improvement. One of the primary challenges in LMICs is the limited access to comprehensive care which includes a multidisciplinary approach involving hematologists, physiotherapists, psychologists, and social workers. In many LMICs, the healthcare infrastructure is insufficient to provide such integrated services, leading to fragmented care and poorer health outcomes for individuals with hemophilia. Another significant issue is the challenge of prophylactic treatment. Prophylaxis, which involves regular infusions of clotting factor concentrates to prevent bleeding episodes, is the standard of care in high-income countries. However, in LMICs, prophylactic treatment is often not feasible due to the high cost and limited availability of clotting factor concentrates. This results in a reliance on on-demand treatment, which only addresses bleeding episodes as they occur and does not prevent the long-term complications associated with frequent bleeds. Pain management is another critical area with significant gaps. Chronic pain is a common issue for individuals with hemophilia due to repeated joint bleeds leading to joint damage. In many LMICs, access to effective pain management strategies, including both pharmacological and non-pharmacological treatments, is limited. Mental health support is also a crucial yet often overlooked aspect of hemophilia care. The chronic nature of the condition, combined with frequent hospital visits and the physical limitations imposed by the disease, can lead to mental health issues such as anxiety and depression. However, mental health services are frequently under-resourced in LMICs, and there is a lack of awareness about the mental health needs of individuals with hemophilia. Caregiver support playing a crucial role in managing the day-to-day needs of individuals with hemophilia, is another vital component of hemophilia care that is often insufficient in LMICs. Education and awareness about hemophilia are also lacking in many LMICs. There is often a limited understanding of the condition among the general public and even within the medical community, leading to misdiagnoses and delayed treatment. Employment and financial support are critical issues as well. The physical limitations and frequent medical needs associated with hemophilia can make it difficult for individuals to maintain stable employment, leading to financial strain. In many LMICs, social support systems are inadequate to address these challenges. Lastly, the integration of telehealth and digital health technologies presents a promising strategy to overcome some of these challenges providing remote access to specialist care, education, and support, which is particularly valuable in regions where healthcare resources are scarce. By adopting a multifaceted approach that involves collaboration between governments, healthcare systems, international organizations, and patient advocacy groups, it is possible to address these challenges and significantly improve the QoL for individuals with hemophilia in LMICs.