Nijmegen Centre for Evidence Based Practice, Department of Rehabilitation/Occupational Therapy 898, Radboud University Nijmegen Medical Centre, P,O, Box 9101, 6500 HB, Nijmegen, The Netherlands.
BMC Neurol. 2011 Jul 13;11:86. doi: 10.1186/1471-2377-11-86.
Myotonic dystrophy type 1 (MD1) is one of the most prevalent neuromuscular diseases, yet very little is known about how MD1 affects the lives of couples and how they themselves manage individually and together. To better match health care to their problems, concerns and needs, it is important to understand their perspective of living with this hereditary, systemic disease.
A qualitative study was carried out with a purposive sample of five middle-aged couples, including three men and two women with MD1 and their partners. Fifteen in-depth interviews with persons with MD1, with their partners and with both of them as a couple took place in the homes of the couples in two cities and three villages in the Netherlands in 2009.
People with MD1 associate this progressive, neuromuscular condition with decreasing abilities, describing physical, cognitive and psychosocial barriers to everyday activities and social participation. Partners highlighted the increasing care giving burden, giving directions and using reminders to compensate for the lack of initiative and avoidant behaviour due to MD1. Couples portrayed the dilemmas and frustrations of renegotiating roles and responsibilities; stressing the importance of achieving a balance between individual and shared activities. All participants experienced a lack of understanding from relatives, friends, and society, including health care, leading to withdrawal and isolation. Health care was perceived as fragmentary, with specialists focusing on specific aspects of the disease rather than seeking to understand the implications of the systemic disorder on daily life.
Learning from these couples has resulted in recommendations that challenge the tendency to treat MD1 as a condition with primarily physical impairments. It is vital to listen to couples, to elicit the impact of MD1, as a multisystem disorder that influences every aspect of their life together. Couple management, supporting the self-management skills of both partners is proposed as a way of reducing the mismatch between health services and health needs.
1 型肌强直性营养不良（MD1）是最常见的神经肌肉疾病之一，但人们对 MD1 如何影响夫妻生活以及他们如何单独和共同管理自己的生活知之甚少。为了更好地根据他们的问题、关注点和需求提供医疗保健，了解他们对这种遗传性、系统性疾病的看法非常重要。
本研究采用目的抽样方法选取了五对中年夫妇，包括三名男性和两名女性 MD1 患者及其伴侣，对他们进行了定性研究。2009 年，在荷兰两个城市和三个村庄的夫妇家中，对 15 名 MD1 患者、他们的伴侣以及他们作为夫妇的双方进行了 15 次深入访谈。
MD1 患者将这种进行性神经肌肉疾病与能力下降联系起来，描述了日常活动和社会参与的身体、认知和心理社会障碍。伴侣们强调了照顾负担的增加，为了弥补 MD1 导致的缺乏主动性和回避行为，他们会给出指示并使用提醒。夫妇们描绘了重新协商角色和责任的困境和挫折感；强调了在个人和共同活动之间取得平衡的重要性。所有参与者都经历了来自亲戚、朋友和社会（包括医疗保健）的不理解，导致他们退缩和孤立。医疗保健被认为是零碎的，专家们专注于疾病的特定方面，而不是试图了解系统性疾病对日常生活的影响。
从这些夫妇身上吸取的经验教训提出了一些建议，这些建议挑战了将 MD1 视为主要有身体损伤的疾病的倾向。倾听夫妇的意见，了解 MD1 作为一种多系统疾病对他们共同生活的各个方面的影响是至关重要的。提出夫妇管理，支持双方的自我管理技能，作为减少医疗服务与健康需求不匹配的一种方式。
