Quality of Life Research

, Volume 26, Issue 5, pp 1337–1348 | Cite as

Factors associated with health-related quality of life (HRQOL) in adults with short stature skeletal dysplasias

  • Nitasha Dhiman
  • Alia Albaghdadi
  • Cheryl K. Zogg
  • Meesha Sharma
  • Julie E. Hoover-Fong
  • Michael C. Ain
  • Adil H. Haider
Article
First Online:
Accepted:

Abstract

Introduction

Numerous factors associate with health disparities. The extent to which such factors influence health-related quality of life (HRQOL) among adults with short stature skeletal dysplasias (SD) is unknown. In an effort to update and clarify knowledge about the HRQOL of adults with SD, this study aimed to quantify HRQOL scores relative to the American average and assess whether specific indicators are associated with lower scores.

Methods

Members (>18 years) of Little People of America were invited to complete an online survey assessing HRQOL using the SF-12 supplemented with indicator-specific questions. SF-12 components (Physical Component Summary, PCS; Mental Component Summary, MCS) were compared to the standardized national American mean. Scores were divided at the median to identify factors associated with lower scores using multivariable logistic regression, adjusting for age, gender, race, education, and employment.

Results

A total of 189 surveys were completed. Mean and median PCS and MCS were below the national mean of 50 (p < 0.001). Advancing decade of age corresponded to a significant decline in PCS (p < 0.001) but not MCS (p = 0.366). Pain prevalence was high (79.4%); however, only 5.9% visited a pain specialist. Significant factors for lower PCS included age >40 years (p = 0.020), having spondyloepiphyseal dysplasia congenita (SED) or diastrophic dysplasia relative to achondroplasia (p = 0.023), pain (p < 0.001), and “partial” versus “full” health insurance coverage (p = 0.034). For MCS, significant factors included a lack of social support (p = 0.002) and being treated differently/feeling stigmatized by health care providers (p = 0.022).

Conclusions

Individuals with SD face documented disparities and report lower HRQOL. Further research and interventions are needed to modify nuanced factors influencing these results and address the high prevalence of pain.

Keywords

Health-related quality of life Skeletal dysplasia Dwarfism Disparities 
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Notes

Funding

Cheryl K. Zogg is supported by NIH Medical Scientist Training Program Training Grant T32GM007205. Adil H. Haider is the PI of a contract (AD-1306-03980) with PCORI entitled “Patient-Centered Approaches to Collect Sexual Orientation/Gender Identity in the ED,” a Harvard Surgery Affinity Research Collaborative (ARC) Program Grant entitled “Mitigating Disparities Through Enhancing Surgeons’ Ability To Provide Culturally Relevant Care,” and a collaborative research grant from the Henry M. Jackson Foundation for the Advancement of Military Medicine in conjunction with the Uniformed Services University of the Health Sciences. Adil H. Haider is also the co-founder and an equity holder in Patient Doctor Technologies Inc., which owns and operates the Web site www.doctella.com.

Author contributions

ND, JEH, MCA, and AHH made substantial contributions to the conception or design of the work. ND and AHH participated in the acquisition of the data. ND, AA, CKZ, and MS were responsible for data analysis. ND, AA, and CKZ drafted the manuscript, and MS, JEH, MCA, and AHH critically revised the manuscript for intellectual content. All authors provided final approval of the version to be published and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Compliance with ethical standards

Conflict of interest

The authors declare that we have no conflicts of interest relevant to the analysis to report.

Ethical approval

All procedures involving study participants were performed in accordance with the ethical standards of the LPA Board of Directors, Johns Hopkins University School of Medicine Institutional Review Board, and 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study. Completion of the survey required provision of written informed consent.

Supplementary material

11136_2016_1455_MOESM1_ESM.pdf (108 kb)
Supplementary material 1 (PDF 108 kb)

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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Nitasha Dhiman
    • 1
  • Alia Albaghdadi
    • 2
  • Cheryl K. Zogg
    • 3
    • 4
  • Meesha Sharma
    • 4
  • Julie E. Hoover-Fong
    • 5
  • Michael C. Ain
    • 6
  • Adil H. Haider
    • 4
    • 7
  1. 1.Virginia Tech Carilion School of MedicineRoanokeUSA
  2. 2.Department of SurgeryJohns Hopkins University School of MedicineBaltimoreUSA
  3. 3.Yale School of MedicineNew HavenUSA
  4. 4.Department of Surgery, Center for Surgery and Public Health, Brigham & Women’s Hospital, Harvard Medical SchoolHarvard T.H. Chan School of Public HealthBostonUSA
  5. 5.Greenberg Center for Skeletal Dysplasias, McKusick-Nathans Institute for Genetic MedicineThe Johns Hopkins Children’s CenterBaltimoreUSA
  6. 6.Department of Orthopaedic SurgeryThe Johns Hopkins School of MedicineBaltimoreUSA
  7. 7.Department of Surgery, Brigham & Women’s HospitalKessler Director Center for Surgery and Public HealthBostonUSA

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