Systematic review of the psychosocial aspects of living with Marfan syndrome

Velvin, Gry; Bathen, Trine; Rand‐Hendriksen, Svend; Geirdal, Amy Østertun

doi:10.1111/cge.12422

Cited by 57 publications

(78 citation statements)

References 42 publications

(192 reference statements)

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“…So far, rehabilitation programs specific for MFS appear to exist only in Norway and scientifically evaluated programs do not exist. However sparse studies and case reports emphasize the need for psychosocial support of affected persons [3–5]. All authors agree on the crucial value of a multidisciplinary approach to rehabilitation for MFS [2, 6].…”

Section: Introductionmentioning

confidence: 99%

“…These are predominantly characterized by uncertainties regarding physical fitness, body image, sexuality, and occupational abilities. Also, questions of how to deal with the inheritance of MFS and of how to inform others of their condition are central to MFS patients [3]. The primary aim of this study was to confirm that our rehabilitation program was feasible and medically save.…”

Section: Introductionmentioning

confidence: 99%

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Inpatient rehabilitation for adult patients with Marfan syndrome: an observational pilot study

Benninghoven

Hamann

Kodolitsch

et al. 2017

Orphanet J Rare Dis

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BackgroundAdvances in medical, interventional and surgical treatment have increased average life expectancy of patients with congenital heart defects. As a result a new group of adult patients with congenital cardiac defects requires medical rehabilitation. Patients with Marfan syndrome (MFS) are a relevant group among these patients. So far, no reports on the effectiveness of specialized rehabilitation programmes for MFS patients exist. We implemented an inpatient 3-week rehabilitation program for MFS patients at the Muehlenberg-Clinic for rehabilitation and assessed the medical safety as well as the impact of the program on physical fitness and psychological wellbeing of participants by means of an observational pilot study. The comprehensive multidisciplinary program included medical, physiotherapeutic, psychological and social issues. Two groups including 8 and 10 individuals with verified MFS attended the programme. Medically adverse events that occurred during the rehabilitation were registered. Adverse events were defined as: any new cardiac arrhythmias such as atrial fibrillation, ventricular tachycardia, cardiac syncope or any complications located at the aorta. Psychological assessment was performed using Short Form-36 (SF-36), hospital anxiety and depression scale and other psychometric questionnaires. Medical examinations included assessment of maximum power in bicycle ergometry. All assessments were performed at the beginning and at the end of the rehabilitation. Psychometric assessments were repeated 1 year after the end of the programme for both groups, respectively.ResultsPatients were highly satisfied with the programme and improved in almost all psychological and physical fitness assessments. The pre-post-comparison resulted in significant positive changes for mental health (p < .001 for SF-36 Mental Health), fatigue (p < .05 for Fatigue Severity Scale), nociception (p < .05 for SF-36 Pain) and vitality (p < .05 for SF-36 Vitality). Physical fitness improved from admission to discharge (p < .001 for maximum power in bicycle ergometry, p < .05 for maximum nordic walking distance). Considerable improvements persisted through 1 year follow-up. Medical assessments excluded medical problems or adverse events caused by participation in the programme.ConclusionsIn our study, inpatient rehabilitation was both safe and helpful for MFS patients. They benefited in terms of physical fitness, health related quality of life and in terms of psychological wellbeing. An evaluation of the efficacy of the programme in a controlled design as well as further conceptual improvements of our current program is desirable.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Inpatient rehabilitation for adult patients with Marfan syndrome: an observational pilot study

Benninghoven

Hamann

Kodolitsch

et al. 2017

Orphanet J Rare Dis

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“…There is some evidence that patients with MFS show a lower quality of life and a higher level of psychopathology, mainly anxiety and depression, than comparable healthy subjects. In addition, some limited evidence exists showing that patients with MFS may also have neuropsychological deficits 3,5,7,104,105. But these findings need further confirmation through studies with larger sample sizes.…”

Section: Team Membersmentioning

confidence: 96%

“…The main goals here are to overcome patients’ uncertainty regarding their physical abilities and, thereby, to improve patients’ body image. Twice a week, patients take part in a psychological group therapy focusing on coping with MFS 105. Additionally, a psychologist trains patients in a relaxation technique.…”

Section: Team Membersmentioning

confidence: 99%

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

Kodolitsch¹,

Rybczynski²,

Vogler³

et al. 2016

JMDH

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Marfan syndrome (MFS) is a rare, severe, chronic, life-threatening disease with multiorgan involvement that requires optimal multidisciplinary care to normalize both prognosis and quality of life. In this article, each key team member of all the medical disciplines of a multidisciplinary health care team at the Hamburg Marfan center gives a personal account of his or her contribution in the management of patients with MFS. The authors show how, with the support of health care managers, key team members organize themselves in an organizational structure to create a common meaning, to maximize therapeutic success for patients with MFS. First, we show how the initiative and collaboration of patient representatives, scientists, and physicians resulted in the foundation of Marfan centers, initially in the US and later in Germany, and how and why such centers evolved over time. Then, we elucidate the three main structural elements; a team of coordinators, core disciplines, and auxiliary disciplines of health care. Moreover, we explain how a multidisciplinary health care team integrates into many other health care structures of a university medical center, including external quality assurance; quality management system; clinical risk management; center for rare diseases; aorta center; health care teams for pregnancy, for neonates, and for rehabilitation; and in structures for patient centeredness. We provide accounts of medical goals and standards for each core discipline, including pediatricians, pediatric cardiologists, cardiologists, human geneticists, heart surgeons, vascular surgeons, vascular interventionists, orthopedic surgeons, ophthalmologists, and nurses; and of auxiliary disciplines including forensic pathologists, radiologists, rhythmologists, pulmonologists, sleep specialists, orthodontists, dentists, neurologists, obstetric surgeons, psychiatrist/psychologist, and rehabilitation specialists. We conclude that a multidisciplinary health care team is a means to maximize therapeutic success.

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“…The scale tributes to significant disability, psychological burden [8][9][10], and reduced quality of life [8,11]. To our knowledge, only one study has investigated the treatment of pain symptoms in MFS [6] and no studies exist on the use of opioid therapy in this patient population.…”

Section: Short Form-12 (Sf-12)mentioning

confidence: 99%

Survey of Pain Therapies in Marfan Syndrome

Speed¹

2017

J Musculoskelet Disord Treat

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Objective: Pain in Marfan syndrome is common, although frequently under diagnosed and undertreated. Few studies have investigated the treatment of pain symptoms in Marfan syndrome and no study has reported on the use of opioid therapy in this patient population. This study aims to characterize the use of pain treatment interventions, including opioid use in individuals with Marfan syndrome. We hypothesize that opioid use would be associated with worse pain and greater psychological burden and that pain-related disability would moderate opioid use.Methods: Individuals with Marfan syndrome completed an online questionnaire assessing pain severity, psychological burden, and pain management therapies. The sample reporting pain (N = 218) were 74.5% female, average age ranging from 35 to 44 years, predominantly white (91%), and majority are employed (53%).Results: Participants frequently use a combination of pharmacological and non-pharmacological therapies, and the most commonly used treatments are over the counter medications and opioid medications. Use of opioids, and non-opioid medications, but not non-pharmacological treatments, increases with pain severity. Individuals who use opioids (34%) report more severe and frequent pain compared to non-opioid users, perceive greater pain-related disability and psychological burden, and more frequently utilize the emergency department. Conclusion:Pharmacological and non-pharmacological treatment for pain in Marfan syndrome is common, although psychotherapeutic interventions are significantly underutilized. Further studies are needed to address pain management in Marfan syndrome. Pain therapies should be multimodal and include adjunctive interventions that reduce pain and suffering, improve function, and enhance quality of life in this patient population.

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Systematic review of the psychosocial aspects of living with Marfan syndrome

Cited by 57 publications

References 42 publications

Inpatient rehabilitation for adult patients with Marfan syndrome: an observational pilot study

Inpatient rehabilitation for adult patients with Marfan syndrome: an observational pilot study

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

Survey of Pain Therapies in Marfan Syndrome

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