Hypermobile Ehlers–Danlos syndrome (hEDS) includes physical symptoms of chronic pain, fatigue, gastrointestinal dysfunction, and joint subluxations/dislocations. This study aims to fill a research gap regarding the psychosocial well-being in pediatric hEDS by assessing relationships between functional disability, social support, and mental health. Increased functional disability is hypothesized to be associated with increased mental health challenges, specifically anxiety and depression, and general social support is hypothesized to moderate this relationship, such that higher perceived social support will mitigate the negative psychological impacts of functional disability. Gender’s influence on mental health in pediatric hEDS is also explored. Thirty-four youth with pediatric hEDS recruited from a United States Midwest multidisciplinary genetics clinic completed self-report questionnaires. Results demonstrate associations between functional disability and mental health, and social support and mental health independently; however, moderation was not found. Functional disability and social support each have a unique influence on the mental health of children with pediatric hEDS and should each receive clinical attention. Exploratory analyses into the influence of gender provide a groundwork for future studies.
AndersonLKLaneKR (2022) The diagnostic journey in adults with hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders. Journal of the American Association of Nurse Practitioners34: 639–648. DOI: 10.1097/JXX.0000000000000672
2.
Baeza-VelascoCBourdonCMontalescotL, et al. (2018) Low-and high-anxious hypermobile Ehlers–Danlos syndrome patients: comparison of psychosocial and health variables. Rheumatology International38: 871–878.
3.
BeightonPPaepeADSteinmannB, et al. (1998) Ehlers‐Danlos syndromes: revised nosology, Villefranche, 1997. American Journal of Medical Genetics77: 31–37.
4.
BieniakKHKovenMBedreeH, et al. (2022) Experiences with pediatric hEDS: parent-child concordance across challenges, readiness to change, coping, and implications for functioning. Clinical Practice in Pediatric Psychology10: 139–149.
5.
BulbenaABaeza‐VelascoCBulbena‐CabréA, et al. (2017) Psychiatric and psychological aspects in the Ehlers–Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics175: 237–245.
6.
CastarlenasEJensenMPvon BaeyerCL, et al. (2017) Psychometric properties of the numerical rating scale to assess self-reported pain intensity in children and adolescents. The Clinical Journal of Pain33: 376–383.
7.
CastoriMCamerotaFCellettiC, et al. (2010) Ehlers-Danlos syndrome hypermobility type and the excess of affected females: possible mechanisms and perspectives. American Journal of Medical Genetics. Part A152A: 2406–2408.
8.
CederlöfMLarssonHLichtensteinP, et al. (2016) Nationwide population-based cohort study of psychiatric disorders in individuals with Ehlers–Danlos syndrome or hypermobility syndrome and their siblings. BMC Psychiatry16: 207.
9.
ChuPSSaucierDAHafnerE (2010) Meta-analysis of the relationships between social support and well-being in children and adolescents. Journal of Submicroscopic Cytology and Pathology29: 624–645.
10.
CohenSWillsTA (1985) Stress, social support, and the buffering hypothesis. Psychological Bulletin98: 310–357.
11.
FalesJLEssnerBSHarrisMA, et al. (2014) When helping hurts: miscarried helping in families of youth with chronic pain. Journal of Pediatric Psychology39: 427–437.
12.
FishbainDACutlerRRosomoffHL, et al. (1997) Chronic pain-associated depression: antecedent or consequence of chronic pain?The Clinical Journal of Pain13: 116–137.
13.
FriedrichsdorfSJGiordanoJDesai DakojiK, et al. (2016) Chronic pain in children and adolescents: diagnosis and treatment of primary pain disorders in head, abdomen, muscles and joints. Children (Basel, Switzerland)3: 42.
14.
HarrisMAAntalHOelbaumR, et al. (2008) Good intentions gone awry: assessing parental “miscarried helping” in diabetes. Families, Systems & Health: the Journal of Collaborative Family Healthcare26: 393–403.
15.
HarterS (2012) Social Support Scale for Children: Manual and Questionnaires. Denver, CO: University of Denver.
16.
HershenfeldSAWSMcNivenVParikhM, et al. (2016) Psychiatric disorders in Ehlers‐Danlos syndrome are frequent, diverse and strongly associated with pain. Rheumatology International36: 341–348.
17.
IrwinDEStuckyBLangerMM, et al. (2010) An item response analysis of the pediatric PROMIS anxiety and depressive symptoms scales. Quality of Life Research: An International Journal of Quality of Life Aspects of Treatment, Care and Rehabilitation19: 595–607.
18.
Kashikar-ZuckSFlowersSRClaarRL, et al. (2011) Clinical utility and validity of the functional disability inventory (FDI) among a multicenter sample of youth with chronic pain. Pain152: 160–1607.
19.
Kashikar‐ZuckSLynchAMSlaterS, et al. (2008) Family factors, emotional functioning, and functional impairment in juvenile fibromyalgia syndrome. Arthritis Care & Research: Official Journal of the American College of Rheumatology59: 1392–1398.
20.
KeoghEEcclestonC (2006) Sex differences in adolescent chronic pain and pain-related coping. Pain123: 275–284.
21.
KingSChambersCTHuguetA, et al. (2011) The epidemiology of chronic pain in children and adolescents revisited: a systematic review. Pain152: 2729–2738.
López-MartínezAEEsteve-ZarazagaRRamírez-MaestreC (2008) Perceived social support and coping responses are independent variables explaining pain adjustment among chronic pain patients. The Journal of Pain9: 373–379.
MalfaitFFrancomanoCByersP, et al. (2017) The 2017 international classification of the Ehlers–Danlos syndromes. American Journal of Medical Genetics Part C: Seminars in Medical Genetics175: 8–26.
26.
MuWMurielloMClemensJL, et al. (2019) Factors affecting quality of life in children and adolescents with hypermobile Ehlers‐Danlos syndrome/hypermobility spectrum disorders. American Journal of Medical Genetics Part A179: 561–569.
27.
MurrayBYasharBMUhlmannWR, et al. (2013) Ehlers–Danlos syndrome, hypermobility type: a characterization of the patients’ lived experience. American Journal of Medical Genetics Part A161: 2981–2988.
28.
PavlovaMFerenceJHancockM, et al. (2017) Disentangling the Sleep-Pain Relationship in Pediatric Chronic Pain: The Mediating Role of Internalizing Mental Health Symptoms. Pain Research & Management.
29.
PollackWS (2006) The “war” for boys: hearing “real boys” voices, healing their pain. Professional Psychology: Research and Practice37: 190–195.
30.
RidenourTAGreenbergMTCook, et al. (2006) Structure and validity of people in my life: a self-report measure of attachment in late childhood. Journal of Youth and Adolescence35: 1037–1053.
31.
RubinKHBurgessKB (2001) Social withdrawal and anxiety. In: MWVDMR (ed), The developmental psychopathology of anxiety. New York, NY: Oxford University Press.
32.
SimmondsJVKeerRJ (2007) Hypermobility and the hypermobility syndrome. Manual Therapy12(4): 298–309. DOI: 10.1016/j.math.2007.05.001
33.
SimonsLESiebergCBClaarRL (2012) Anxiety and functional disability in a large sample of children and adolescents with chronic pain. Journal of pain research17: 93–97.
34.
StanfordEAChambersCTBiesanzJC, et al. (2008) The frequency, trajectories and predictors of adolescent recurrent pain: a population-based approach. Pain138: 11–21.
35.
SyxDDe WandeleIRombautL, et al. (2017) Hypermobility, the Ehlers-Danlos syndromes and chronic pain. Clinical and Experimental Rheumatology35: S166–S172.
36.
TegethoffMBelardiAStalujanisE, et al. (2015) Comorbidity of mental disorders and chronic pain: chronology of onset in adolescents of a national representative cohort. The Journal of Pain16: 1054–1064.
37.
TinkleBCastoriMBerglundB, et al. (2017) Hypermobile Ehlers–Danlos syndrome (aka Ehlers–Danlos syndrome type III and Ehlers–Danlos syndrome hypermobility type): clinical description and natural history. American Journal of Medical Genetics Part C: Seminars in Medical Genetics.
38.
TranSTJagpalAKovenML, et al. (2020) Symptom complaints and impact on functioning in youth with hypermobile Ehlers–Danlos syndrome. Journal of Child Health Care24: 444–457.
39.
U.S. National Library of Medicine (2020) Ehlers-Danlos syndrome. U.S. National Library of Medicine.
40.
VarniJWMagnusBStuckyBD, et al. (2014) Psychometric properties of the PROMIS® pediatric scales: precision, stability, and comparison of different scoring and administration options. Quality of Life Research: an International Journal of Quality of Life Aspects of Treatment, Care and Rehabilitation23: 1233–1243.
41.
VinallJPavlovaMAsmundsonG, et al. (2016) Mental health comorbidities in pediatric chronic pain: a narrative review of epidemiology, models, neurobiological mechanisms and treatment. Children3: 40.
42.
VoermansNCKnoopHBleijenbergG, et al. (2010) Pain in Ehlers-Danlos syndrome is common, severe, and associated with functional impairment. Journal of Pain and Symptom Management40: 370–378.
43.
Von KorffMSimonG (1996) The relationship between pain and depression. The British Journal of Psychiatry168: 101–108.
44.
WalkerLSGreeneJW (1991) The functional disability inventory: measuring a neglected dimension of child health status. Journal of Pediatric Psychology16: 39–58.
45.
WallanderJLVarniJW (1992) Adjustment in children with chronic physical disorders: programmatic research on a disability-stress-coping model. In: La GrecaAMWallanderJLWalkerCE (eds), Advances in pediatric psychology. Stress and coping in child health. The Guilford Press, pp. 279–298.
46.
WallanderJLVarniJWBabniLV, et al. (1989) Family resources as resistance factors for psychological maladjustment in chronically ill and handicapped children. Journal of Pediatrics Psychology14: 157–173.
47.
ZhangBYanXZhaoF, et al. (2015) The relationship between perceived stress and adolescent depression: the roles of social support and gender. Social Indicators Research123: 501–518.
Supplementary Material
Please find the following supplemental material available below.
For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.
For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.
