Characterization of pain, disability, and psychological burden in Marfan syndrome

Speed, Traci J.; Mathur, Vani A.; Hand, Matthew; Christensen, Bryt A.; Sponseller, Paul D.; Williams, Kayode; Campbell, Claudia M.

doi:10.1002/ajmg.a.38051

Cited by 42 publications

(86 citation statements)

References 55 publications

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“…A subgroup analysis of opioid users compared those currently using one opioid with those currently using two or more opioids. Similar to previous work [8], psychological variables are reported as median with Interquartile Range (IQR), and compared between groups using the Mann-Whitney U test. Spearman bivariate correlations were conducted to examine the association between pain treatments and pain characteristics.…”

Section: Discussionmentioning

confidence: 99%

“…As previously reported [8], 441 respondents completed an online survey over a 15-month period. Since the survey was anonymous, all data was self-report, and not verified by medical record.…”

Section: Participantsmentioning

confidence: 93%

“…Self-reported clinical pain severity was assessed as the average of patients' ratings of "current pain", "pain at its worst", least pain", and "general pain" using a 5-point scale from [1] mild to [5] excruciating [8].…”

Section: Pain Severity (Bpi)mentioning

confidence: 99%

“…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%

“…Pain symptoms frequently occur at the onset of MFS, and individuals with pain at symptom onset tend to have greater pain severity, pain-related disability, and worse physical and mental health [8]. Given the high incidence of pain in MFS and the medical comorbidities and life-threatening complications associated with the syndrome, it is essential to better understand how pain is managed in this patient population.…”

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

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Participantsmentioning

confidence: 93%

Section: Pain Severity (Bpi)mentioning

confidence: 99%

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

confidence: 99%

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

confidence: 99%

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Survey of Pain Therapies in Marfan Syndrome

Speed¹

2017

J Musculoskelet Disord Treat

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Parenting a child with Marfan syndrome: Distress and everyday problems

Warnink‐Kavelaars

Oers

Haverman

et al. 2020

American J of Med Genetics Pt A

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Marfan syndrome (MFS) is a multisystemic, autosomal dominant connective tissue disorder that occurs de novo in 25%. In many families, parent and child(ren) are affected, which may increase distress in parents. To assess distress, 42 mothers (29% MFS) and 25 fathers (60% MFS) of 43 affected children, completed the validated screening‐questionnaire Distress thermometer for parents of a chronically ill child, including questions on overall distress (score 0–10; ≥4 denoting “clinical distress”) and everyday problems (score 0–36). Data were compared to 1,134 control‐group‐parents of healthy children. Mothers reported significantly less overall distress (2, 1–4 vs. 3, 1–6; p = .049; r = −.07) and total everyday problems (3, 0–6 vs. 4, 1–8; p = .03; r = −.08) compared to control‐group‐mothers. Mothers without MFS reported significantly less overall distress compared to mothers with MFS, both of a child with MFS (1, 0–4 vs. 3.5, 2–5; p = .039; r = −.17). No significant differences were found between the father‐groups, nor between the group of healthy parents of an affected child living together with an affected partner compared to control‐group‐parents. No differences in percentages of clinical distress were reported between mothers and control‐group‐mothers (33 vs. 42%); fathers and control‐group‐fathers (28 vs. 32%); nor between the other groups. Distress was not associated with the children's MFS characteristics. Concluding, parents of a child with MFS did not show more clinical distress compared to parents of healthy children. However, clinical distress was reported in approximately one‐third and may increase in case of acute medical complications. We advise monitoring distress in parents of a child with MFS to provide targeted support.

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Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective tissue disorders

Koning

Warnink‐Kavelaars

Rossum

et al. 2023

American J of Med Genetics Pt A

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The aim of the present study was to investigate the nature and prevalence of nonspecific somatic symptoms, pain and catastrophizing in children with Heritable Connective Tissue Disorders (HCTD), and to determine their association with disability. This observational, multicenter study included 127 children, aged 4-18 years, with Marfan syndrome (MFS) (59%), Loeys-Dietz syndrome (LDS) (8%), Ehlers-Danlos syndromes (EDS) (12%) and hypermobile Ehlers-Danlos syndrome (hEDS) (23%). The assessments included the Children's Somatization Inventory or parent proxy (CSI, PCSI), pain visual-analogue scale (VAS), SUPERKIDZ body diagram, Pain Catastrophizing Scale Child or parent proxy (PCS-C, PCS-P) and Childhood Health Assessment Questionnaire (CHAQ-30). Data from children aged ≥8 years were compared to normative data. In children ≥ 8 years (n = 90), pain was present in 59%, with a median of 4 (IQR = 3-9) pain areas. Compared to normative data, the HCTD group reported significantly higher on the CSI (p ≤ 0.001, d = 0.85), VAS pain intensity (p ≤ 0.001, d = 1.22) and CHAQ-30 (p ≤ 0.001, d = 1.16) and lower on the PCS-C (p = 0.017, d = À0.82) and PCS-P (p ≤ 0.001, d = À0.49). The intensity of nonspecific somatic symptoms and pain explained 45% of the variance in disability (r 2 = 0.45 F(2,48) = 19.70, p ≤ 0.001).In children ≤ 7 years (n = 37), pain was present in 35% with a median of 5(IQR = 1-13) pain areas. The mean(SD) VAS scores for pain intensity was 1.5(2.9). Functional disability was moderately correlated to the number of pain areas (r = 0.56, p ≤ 0.001), intensity of nonspecific somatic symptoms (r = 0.63, p ≤ 0.001) and pain (r = 0.83, p ≤ 0.001). In conclusion, this study A complete list of the Pediatric Heritable Connective Tissue Study Group members appears in the Acknowledgements.Raoul H.H. Engelbert and Lies Rombaut contributed equally.

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Characterization of pain, disability, and psychological burden in Marfan syndrome

Cited by 42 publications

References 55 publications

Survey of Pain Therapies in Marfan Syndrome

Survey of Pain Therapies in Marfan Syndrome

Parenting a child with Marfan syndrome: Distress and everyday problems

Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective tissue disorders

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