Psychosocial adaptation in adolescents and young adults with Marfan syndrome: an exploratory study.

Tongerloo, A. Van; Paepe, Anne De

doi:10.1136/jmg.35.5.405

Cited by 49 publications

(80 citation statements)

References 13 publications

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“…The study was designed to evaluate the psychosocial impact of MFS in different European populations. Earlier studies indicated that the majority of Marfan patients cope well with their condition, although they perceive the syndrome as having adverse consequences on their lives [2,3,4,5]. …”

Section: Discussionmentioning

confidence: 99%

“…The many other physical problems that go along with MFS are not life threatening, but can cause severe distress and, thus, affect the quality of life and form a potential burden to the psychosocial well-being of the patients. There are many published reports on clinical manifestations, molecular background, and medical management of MFS, but surprisingly limited studies regarding the psychosocial burden experienced by MFS persons [2,3,4,5]. …”

Section: Introductionmentioning

confidence: 99%

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Marfan Syndrome in Europe

Bie

Paepe

Delvaux

et al. 2004

Public Health Genomics

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Objectives: Marfan syndrome (MFS) is a relatively frequent systemic connective tissue disorder with an important physical morbidity and mortality. The influences of MFS on physical problems, perception of severity, and impact on the quality of life and psychosocial well-being have been studied only limitedly. The aim of this study was to assess the association between the severity derived from the reported symptoms and subjectively experienced severity of MFS (expressed as a global judgment), with special emphasis regarding impact on relationships and pregnancies, psychosocial adjustment, and differences between the seven European countries. Methods: A questionnaire designed specifically for this study and translated in each of the native languages was sent to 2,080 members of one of the patient support groups in Belgium, Denmark, France, Germany, The Netherlands, Switzerland, and the United Kingdom. 857 MFS patients of 13 years and older completed the questionnaire and were included in the data analysis. Results: Physical impairments were scored by perception of severity of physical symptoms by the patients (physical severity perception score) and by their perception of the influence of MFS on their life (subjective severity score). Main discrepancy between physical severity perception and subjective severity score was the higher percentage of patients scoring in physical severity perception as severe (53.5%) compared to subjectively severe (26.5%). 61% of those who scored on the physical severity score as severely affected were designated as being mildly-moderately affected on subjective scoring. Both severity scores increased significantly with age. Two hundred-twenty women have carried 430 pregnancies (1.95 pregnancies/woman), with cardiovascular complications in 1.6%. Prenatal studies for MFS were rejected by 7.6% of MFS patients of 25 years and older, 33.6% were undecided, and 48.5% favored prenatal diagnosis for MFS if available. A positive general self-image was reported by 91.5% of patients. However, more than 90% stated that MFS had a negative influence on their sexual relationships, which they ascribed to negative perception of their body image. Conclusions: MFS has significant impact on daily life activities, but the majority of patients come to terms with their condition. Acceptance is mainly determined by subjective severity, and less by physical symptoms as reported by the patients themselves. It is important to stimulate a positive attitude towards MFS.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Marfan Syndrome in Europe

Bie

Paepe

Delvaux

et al. 2004

Public Health Genomics

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“…National Marfan Foundations can provide information and support. Adequate information about the disease and good medical and social support can help children and adults to cope with MFS [119].…”

Section: Special Concerns Psychosocial Alterationsmentioning

confidence: 99%

Marfan syndrome: what internists and pediatric or adult cardiologists need to know

Kaemmerer

Oechslin²,

Seidel

et al. 2005

Expert Review of Cardiovascular Therapy

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Marfan syndrome (MFS) is one of the most frequent connective tissue disorders, showing striking pleiotropism and clinical variability. There is autosomal dominant inheritance with complete penetrance but variable expression. Approximately 25% of MFS patients have no family history of the syndrome and represent sporadic cases due to new mutations. This hazardous condition is often associated with premature cardiovascular death unless surveillance and management are optimized. The fibrillin gene (FBN1) encodes the structure of the connective tissue protein fibrillin. MFS is caused by mutations in the fibrillin gene, located on chromosome 15 at locus 15q21. Fibrillin abnormalities reduce the structural integrity of different body systems, primarily involving the heart valves, blood vessels, lungs, bones, tendons, ligaments, cartilages, eyes, skin, spinal dura and the CNS. Patients with MFS are likely to have too little fibrillin within these structures, resulting in clinically relevant problems. For example, in the aortic wall, deficient fibrillin may trigger progressive aortic ectasia and may result in aortic dissection.

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“…We selected the BDI because it contains few somatic items, an important consideration when assessing psychological symptoms in chronically ill patients. The BDI has been used with adolescents with medical illnesses (25)(26)(27)(28). The instrument has been administered extensively and shown to have good reliability coefficients ranging from 0.73 to 0.92 (29).…”

Section: Measures Of Psychological Distressmentioning

confidence: 99%

Psychological Distress and Adherence to the Medical Regimen Among Adolescent Renal Transplant Recipients

Penkower¹,

Dew²,

Ellis³

et al. 2003

American Journal of Transplantation

105

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This longitudinal pilot study of adolescent renal transplant recipients (a) describes the prevalence of psychological distress, (b) describes the prevalence of nonadherence, and (c) explores the association between the recipient's psychological distress and his/her subsequent medical adherence. Twenty-two adolescents, aged 13-18 years, completed two interviews that were separated by approximately 12 months. Psychological distress was assessed in three domains: symptoms of depression, anxiety, and anger. Adherence was assessed in three domains: medication taking, blood work, and clinic attendance. At the initial interview, 36.4% had symptoms of depression, 36.4% endorsed anxiety, and 18.2% endorsed excessive state anger. Non-adherence rates were 13.6% for medication, 22.7% for blood work, and 50% for missed clinic. At the second interview, nonadherence with medication remained the same and the other domains decreased. Our small pilot sample, however, limited our ability to detect statistically significant changes over time. Predictive analyses demonstrated that adolescents with excessive anger were at greater risk for subsequently missing medications than adolescents without excessive anger. These findings suggest that while symptoms of depression and anxiety are observed among some adolescents with renal transplants, only anger is associated with elevated risk for nonadherence with medication.

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Psychosocial adaptation in adolescents and young adults with Marfan syndrome: an exploratory study.

Abstract: We conducted a pilot study to evaluate the psychological effects and consequences of

Cited by 49 publications

References 13 publications

Marfan Syndrome in Europe

Marfan Syndrome in Europe

Marfan syndrome: what internists and pediatric or adult cardiologists need to know

Psychological Distress and Adherence to the Medical Regimen Among Adolescent Renal Transplant Recipients

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