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Journal of Clinical Immunology

, Volume 39, Issue 8, pp 805–813 | Cite as

Provider Perceptions of Quality of Life, Neurocognition, Physical Well-being, and Psychosocial Health in Patients with Primary Immunodeficiency/Immune Dysregulation Conditions

  • Thomas F. MichniackiEmail author
  • Kelly J. Walkovich
  • Lauren E. Merz
  • Julie Sturza
  • Roshini S. Abraham
Original Article
  • 52 Downloads

Abstract

Purpose

Both pediatric and adult patients with a primary immunodeficiency/immune dysregulation (PID/PIDR) diagnosis report inferior quality of life (QOL) and patient-reported outcomes (PROs) as compared with their healthy peers. Recognition of the negative impact on QOL and PROs provides an opportunity for clinicians to intervene with supportive measures. However, provider perceptions of PID/PIDR patients’ quality of life, physical well-being, psychosocial health and neurocognition, and access to supportive resources have yet to be systematically evaluated.

Methods

We report specialty providers’ perception of the QOL and psychosocial and physical well-being of their pediatric and adult patients with PID/PIDR through the utilization of an online survey assessing QOL and the impact of disease or its associated treatment on their physical well-being, mental health, social relationships, neurocognition, and work/school performance.

Results

Clinicians trended towards believing adult PID/PIDR patients had worse overall QOL than children with PID/PIDR. Providers additionally identified their adult patients’ QOL to be more deleteriously affected by co-morbidities than their pediatric patients. Clinicians distinguished anxiety and social relationships as the psychosocial aspects most often affected by a complex immunological diagnosis in all patients. Of physical health considerations, energy, rather than mobility or pain, was perceived to be more negatively influenced by PID/PIDR in both adult and pediatric patients.

Conclusions

Knowledge of these clinician perceptions can affect communication of findings with patients, as well as ongoing management, and thus, it is important to understand these fully to improve healthcare delivery to, and clinical management of, these patients.

Keywords

Primary immunodeficiency/immune dysregulation quality of life patient-reported outcomes provider perceptions 

Abbreviations

CIS

Clinical Immunology Society

HRQoL

Health-related quality of life

IAPIDS

International Alliance of Primary Immunodeficiency Societies

PID/PIDR

Primary immunodeficiency/immune dysregulation

PROs

Patient-reported outcomes

QOL

Quality of life

Notes

Acknowledgments

We thank the survey respondents and the Clinical Immunology Society and International Alliance of Primary Immunodeficiency Societies for facilitating this study.

Author Contributions

Drs. Michniacki and Walkovich conceptualized and designed the study. Dr. Michniacki drafted the manuscript as well as analyzed/interpreted the study data. Dr. Abraham, Dr. Walkovich, Dr. Merz and Ms. Sturza assisted in editing the manuscript. Dr. Merz also assisted in constructing the online surveys. Ms. Sturza additionally provided statistical support.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflicts of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee (University of Michigan; HUM00148246) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

References

  1. 1.
    Similuk MN, Wang A, Lenardo MJ, Erby LH. Life with a primary immune deficiency: a systematic synthesis of the literature and proposed research agenda. J Clin Immunol. 2016;36:123–33.CrossRefGoogle Scholar
  2. 2.
    Peshko D, Kulbachinskaya E, Korsunskiy I, et al. Health-related quality of life in children and adults with primary immunodeficiencies: a systematic review and meta-analysis. J Allergy Clin Immunol Pract. 2019;7:1929–1957.e5.CrossRefGoogle Scholar
  3. 3.
    Kuburovic NB, Pasic S, Susic G, Stevanovic D, Kuburovic V, Zdravkovic S, et al. Health-related quality of life, anxiety, and depressive symptoms in children with primary immunodeficiencies. Patient Prefer Adherence. 2014;8:323–30.CrossRefGoogle Scholar
  4. 4.
    Barlogis V, Mahlaoui N, Auquier P, et al. Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study. J Allergy Clin Immunol. 2017;139:1275–1281.e7.CrossRefGoogle Scholar
  5. 5.
    Quinti I, Pulvirenti F. Health-Related Quality of Life and Patients’ Empowerment in the Health Care of Primary Immune Deficiencies. J Clin Immunol. 2017;37:615–6.CrossRefGoogle Scholar
  6. 6.
    Molzahn AE, Northcott HC, Dossetor JB. Quality of life of individuals with end stage renal disease: perceptions of patients, nurses, and physicians. ANNA J. 1997;24:325–33 discussion 334-335.PubMedGoogle Scholar
  7. 7.
    Lucchesi F, Figueiredo MS, Mastandrea EB, et al. Physicians’ Perception of Sickle-cell Disease Pain. J Natl Med Assoc. 2016;108:113–8.CrossRefGoogle Scholar
  8. 8.
    Jones JM, McPherson CJ, Zimmermann C, Rodin G, le LW, Cohen SR. Assessing agreement between terminally ill cancer patients’ reports of their quality of life and family caregiver and palliative care physician proxy ratings. J Pain Symptom Manag. 2011;42:354–65.CrossRefGoogle Scholar
  9. 9.
    Ewing G, Rogers M, Barclay S, McCabe J, Martin A, Campbell M, et al. Palliative care in primary care: a study to determine whether patients and professionals agree on symptoms. Br J Gen Pract. 2006;56:27–34.PubMedPubMedCentralGoogle Scholar
  10. 10.
    Horton R. Differences in assessment of symptoms and quality of life between patients with advanced cancer and their specialist palliative care nurses in a home care setting. Palliat Med. 2002;16:488–94.CrossRefGoogle Scholar
  11. 11.
    James BC, Aschebrook-Kilfoy B, White MG, Applewhite MK, Kaplan SP, Angelos P, et al. Quality of life in thyroid cancer-assessment of physician perceptions. J Surg Res. 2018;226:94–9.CrossRefGoogle Scholar
  12. 12.
    Oliva EN, Finelli C, Santini V, Poloni A, Liso V, Cilloni D, et al. Quality of life and physicians’ perception in myelodysplastic syndromes. Am J Blood Res. 2012;2:136–47.PubMedPubMedCentralGoogle Scholar
  13. 13.
    Sneeuw KC, Aaronson NK, Sprangers MA, Detmar SB, Wever LD, Schornagel JH. Evaluating the quality of life of cancer patients: assessments by patients, significant others, physicians and nurses. Br J Cancer. 1999;81:87–94.CrossRefGoogle Scholar
  14. 14.
    von Eisenhart RA, Bielitzer M, Meinertz T, et al. Predictors of discordance between physicians’ and patients’ appraisals of health-related quality of life in atrial fibrillation patients: findings from the Angiotensin II Antagonist in Paroxysmal Atrial Fibrillation Trial. Am Heart J. 2013;166:589–96.CrossRefGoogle Scholar
  15. 15.
    Wilson KA, Dowling AJ, Abdolell M, Tannock IF. Perception of quality of life by patients, partners and treating physicians. Qual Life Res. 2000;9:1041–52.CrossRefGoogle Scholar
  16. 16.
    Srikrishna S, Robinson D, Cardozo L, et al. Is there a discrepancy between patient and physician quality of life assessment? Neurourol Urodyn. 2009;28:179–82.CrossRefGoogle Scholar
  17. 17.
    Berry LL, Deming KA, Danaher TS. Improving nonclinical and clinical-support services: lessons from oncology. Mayo Clin Procs: Innov, Qual Outcomes. 2018;2:207–17.Google Scholar
  18. 18.
    Schor EL, Lerner DJ, Malspeis S. Physicians’ assessment of functional health status and well-being. The patient’s perspective. Arch Intern Med. 1995;155:309–14.CrossRefGoogle Scholar
  19. 19.
    Detmar SB, Muller MJ, Schornagel JH, Wever LD, Aaronson NK. Health-related quality-of-life assessments and patient-physician communication: a randomized controlled trial. JAMA. 2002;288:3027–34.CrossRefGoogle Scholar
  20. 20.
    PedsQL TM Pediatric Quality of Life Inventory TM, https://www.pedsql.org/index.html (accessed 13 July 2019).
  21. 21.
  22. 22.
    Nicolay U, Haag S, Eichmann F, et al. Measuring treatment satisfaction in patients with primary immunodeficiency diseases receiving lifelong immunoglobulin replacement therapy. Qual Life Res. 2005;14:1683–91.CrossRefGoogle Scholar
  23. 23.
    Quinti I, Pulvirenti F, Giannantoni P, et al. Development and initial validation of a questionnaire to measure health-related quality of life of adults with common variable immune deficiency: the CVID_QoL questionnaire. J Allergy Clin Immunol Pract. 2016;4:1169–1179.e4.CrossRefGoogle Scholar
  24. 24.
    Ballow M, Conaway MR, Sriaroon P, et al. Construction and validation of a novel disease-specific quality-of-life instrument for patients with primary antibody deficiency disease (PADQOL-16). J Allergy Clin Immunol. 2017;139:2007–2010.e8.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Pediatrics, Pediatric Hematology/OncologyUniversity of MichiganAnn ArborUSA
  2. 2.Department of Internal MedicineBrigham & Women’s HospitalBostonUSA
  3. 3.Department of StatisticsUniversity of MichiganAnn ArborUSA
  4. 4.Department of Pathology and Laboratory MedicineNationwide Children’s HospitalColumbusUSA

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