Effects of a personalized home-based training program among patients suffering from Marfan syndrome: a pilot randomized and controlled study

Abstract

Marfan, telerehabilitation, personalized trainingMarfan syndrome (MFS) is an autosomal hereditary pathology affecting 1:5000 peoples. Alteration of the fibrillin 1 gene (FBN1) results in haplo-insufficiency of the FBN1 protein mainly altering the vascular system. International recommendations have gradually allowed MFS patients to perform training programs because of its potential benefits. However, to date, there are no data on the effect of a long training period in these patients. The aim of the present study is to investigate the effect of a 3-month personalized home-based training on quality of life (QoL) of patients suffering from MFS. At least 50 MFS patients were included in the study. They were randomly placed into 4 groups: control group; endurance; resistance and endurance + resistance training groups. The training program lasted 3 months and is performed at patients' home. There were 2 training sessions per week telemonitored by a specialist of physical activity and cardiology. Pre and post-training evaluations were performed at the Bichat-Paris Hospital, France. They consisted of assessing psychometrics based on self-administered questionnaires (FiRST, GPAQ, ISP-25, MOS SF-36) and physiological parameters such as the peak oxygen consumption, aorta diameter, cardiac ventricle function and skeletal muscle power at rest and during exercise. Our preliminary results showed an improvement of 50% in QoL, cardiorespiratory fitness and skeletal muscle power in a patient who completed the combined training program. This experimental approach might be a new alternative way for MFS patients' care that may improve their QoL, cardiorespiratory fitness and skeletal muscle power.

Health Status and Individual Care Needs of Disabled Elderly at Home in Different Types of Care

“…At the community level, more elaborate and personalized care programs should be developed for the disabled elderly [ 47 , 48 , 49 , 50 ]. From the three levels of individual, family, and community, the disabled elderly should be guaranteed continuous access to appropriate care [ 51 , 52 ].…”

Online Personal Training in Patients with Marfan Syndrome: A Randomized Controlled Study of its Impact on Quality of Life and Physical Capacity

“…In addition, recent animal studies suggest that regular endurance exercise may be beneficial 11,12 . Therefore, a personalised online training program in combination with standard care may be beneficial in patients with MFS 13 .…”