Table 1. Selected articles.
| Author (Year) | N | Main findings |
|---|---|---|
| Handisides et al 18 (2019) | 321 | Children (5–18 y) with MFS scored lower than healthy population norms for physical and psychosocial domains of quality of life. |
| Stanišić et al 27 (2018) | 16 | MFS patients had higher than average satisfaction with life. |
| Treasure et al 28 (2018) | 119 MFS patients, 25 physicians | An active lifestyle was reported to be more important in male MFS patients, compared with female MFS patients. Patients placed more importance than doctors on not deferring surgery and on avoidance of anticoagulation in the interests of childbearing. |
| Ratiu et al 20 (2018) | 318 | Executive function difficulties and metal fatigue are associated with MFS symptoms and affect quality of life. |
| Speed et al 19 (2016) | 245 | 89% of respondents with MFS reported having pain while 28% of individuals reported pain as their presenting symptom of MFS. |
| Goldfinger et al 22 (2017) | 389 | College education, marital status, higher household income, private health insurance, full-time employment, moderate alcohol use, fewer prior surgeries, fewer comorbid conditions, absence of depression, and less severe MFS manifestations were all reported as positive predictors of quality of life in MFS patients. |
| Benninghoven et al 25 (2017) | 18 | MFS patients were highly satisfied with the inpatient rehabilitation program and resulted in significant positive changes for mental health, fatigue, nociception and vitality domains for quality of life. |
| Benke et al 11 (2017) | 45 | MFS patients experience increased trait anxiety compared with healthy individuals after acute life-saving surgery. |
| Velvin et al 49 (2016) | 73 | Adults with MFS experienced decreased satisfaction with life compared with the general Norwegian population. Only fatigue, aortic dissection, and having regular contact with psychologist showed significant effect on satisfaction with life. |
| Rao et al 21 (2016) | 230 | Across all quality of life domains, MFS patients scored significantly lower than United States population norms. |
| Mueller et al 17 (2016) | 42 | No significant overall reduction of quality of life was found in MFS patients from (4–18 y) of age. |
| Moon et al 23 (2016) | 218 | In patients with MFS, the quality of life was affected significantly by social support, disease-related factors, and biobehavioral factors, such as anxiety and depression. |
| Ghanta et al 24 (2016) | 49 | After life-saving aortic surgery, MFS patients reported slightly worse physical scores with slightly better mental scores than the general population. |
| Nelson et al 48 (2015) | 993 | 67% of MFS participants reported pain in the preceding 7 days. 56% of participants noted analgesic use to control pain related to MFS with 55% reporting <50% pain relief. |
| Velvin et al 57 (2015) | 70 | 59% of participants were employed or students, significantly lower work participation than the general Norwegian population. Age, lower educational level, and severe fatigue were significantly associated with low work participation; not MFS related health problems or chronic pain. |
| Kelleher et al 29 (2015) | 147 posts | Social media content related to MFS can identify various common emotions, such as anxiety and depression |
| Bathen et al 10 (2014) | 72 | Participants reported significantly higher prevalence of severe fatigue compared with the general Norwegian population and patients with rheumatoid arthritis, but lower than in other chronic conditions. |
| Johansen et al 26 (2013) | 11 children, 10 Caregivers | MFS was found to have a negative impact on the quality of life in these children, however, there was no impact identified in their caregiver's satisfaction with life. |
| Song et al 30 (2013) | 194 | MFS patients who had undergone emergent aortic surgery had a significantly lower quality of life. |
| Schoormans et al 31 (2012) | 121 | MFS patients' quality of life score was significantly lower in the physical domain which was weakly related to age and presence of scoliosis. |
| Rand-Hendriksen et al 55 (2010) | 84 | MFS patients, compared with the general population, demonstrated lower quality of life domains for social function, vitality, general health, bodily pain, and role physical. |
| Giarelli et al 80 (2010) | 37 | The frequency and range of self-monitoring increased with the age of the child with MFS. |
| Giarelli et al 38 (2008) A | 39 parents 37 adolescents (14–21y), 16 young adults (22–34y), health care professionals 15 |
Obstacles to transition self-management for MFS patients can believe related to three categories; person with MFS, parents, and healthcare professionals. |
| Giarelli et al 13 (2008) B | 39 parents 37 adolescents (14–21y), 16 young adults (22–34y), health care professionals 15 |
Caregivers of individuals with MFS reported that the financial concerns with one of their largest stressors. MFS adolescents were all involved in daily self-monitoring. The parent's fears and need to stay involved in the child's health care slowed the child's independent work on self-management responsibilities. |
| Van Dijk et al 52 (2008) | 59 | MFS patients reported fatigue which was significantly correlated with low orthostatic tolerance. There was no relationship between β-blocker use and fatigue. |
| Fusar-Poli et al 9 (2008) | 36 | MFS patients reported an impaired health-related quality of life in the psychological domain but not in the physical domain. Being male and older was significantly associated with a poorer perceived mental quality of life. |
| Percheron et al 50 (2007) | 21 | MFS patients had significantly higher fatigue than the controls. |
| Rand-Hendriksen et al 51 (2007) | 16 | Self-reported fatigue was comparable with fatigue reported in other severe chronic diseases and disabilities and was primarily in the mental/psychological domain. Psychological distress was higher than expected compared with the population at large. |
| Peters et al 40 (2005) | 174 | Fifty-six respondents (32%) reported feeling discriminated against or socially devalued because of having MFS. Endorsement of discrimination was significantly correlated with having depressive symptoms, low self-esteem, the view MFS has had significant negative consequences on one's life, striae, and perceptions of workplace discrimination. Instances of workplace discrimination were perceived by 20% of respondents, and 23% reported that they remained in a dissatisfying job due to having MFS. |
| Foran et al 53 (2005) | 22 | Symptoms associated with dural ectasia had a marked impact on the overall health of patients with MFS. |
| De Bie et al 57 (2004) | 857 | 61% of those who scored on the physical severity score as severely affected were designated as being mildly-moderately affected on subjective scoring. Two hundred-twenty women have carried 430 pregnancies (1.95 pregnancies/woman), with cardiovascular complications in 1.6%. 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. |
| Peters et al 12 (2002) | 174 | Approximately 62% of the patients agreed that having MFS significantly affected their reproductive decision-making. 69% of MFS patients in the study reported personal interest in prenatal testing for MFS. Age, striae, back pain, and low quality of life were each independently correlated with lack of sex drive. |
| Verbraecken et al 56 (2001) | 15 | Health related quality of life scores were significantly lower in MFS patients compared with healthy controls, except for emotional problems. Sleep apnea was significantly increased in MFS patients compared with controls. |
| Peters et al 6 (2001) | 174 | The presence of cardiovascular symptoms and fatigue were positively correlated with medication use (β-blocker and calcium channel blocker) in MFS patients. |
| Peters et al 6 (2001) | 174 | 83% of the respondents perceived MFS as having had significant adverse consequences on their lives. Having striae, pain (sore joints), and depression were each independently correlated with this view. |
| Van Tongerloo et al 8 (1998) | 17 | During childhood, most MFS patients were insensitively teased by peers because of their typical phenotypic features. In female MFS patients, the risk associated with child bearing represented a major concern. |
| Schneider et al 7 (1990) | 22 | MFS patients perceived that their lives would be significantly better without MFS, especially in the areas of physical activities and self-image. Their medication compliance was suboptimal, similar to prior publications on description of compliance among teenagers with other chronic illnesses. |
| Reviews | ||
| Velvin et al 32 (2019) | N/A | |
| Velvin et al 49 (2016) | N/A | |
| Velvin et al 57 (2015) | N/A | |
| Gritti et al 16 (2015) | N/A |
Abbreviation: MFS, Marfan syndrome.