Patient-Reported Outcomes and Medical Provider Satisfaction Among Adult and Pediatric Ataxia-Telangiectasia Patients.

Ataxia-telangiectasia Inborn errors of immunity Medical provider satisfaction Patient-reported outcomes Primary immunodeficiency Quality of life

Journal

Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137

Informations de publication

Date de publication:
17 Jan 2024
Historique:
received: 06 08 2023
accepted: 06 01 2024
medline: 17 1 2024
pubmed: 17 1 2024
entrez: 17 1 2024
Statut: epublish

Résumé

Ataxia-telangiectasia (A-T) is a rare genetic condition with malfunctioning DNA repair processes resulting in significant clinical findings, including progressive neurologic decline, elevated malignancy risk, immunodeficiency, oculocutaneous telangiectasias, and severe pulmonary disease. Research has been limited into the quality of life of such patients and yet to be completed are studies quantitatively analyzing psychosocial, physical, and cognitive patient-reported outcomes (PROs) within the A-T population. PRO evaluations of 90 international adult and pediatric A-T patients and their caregivers were completed via secure online administration of Patient-Reported Outcomes Measurement Information System (PROMIS) short forms evaluating anger, cognition, mood, social health, fatigue, pain, anxiety, and upper extremity function. The impact of age, gender, race/ethnicity, prior malignancy diagnosis, and current supportive treatment interventions on such PROs was additionally assessed. Finally, given the importance of medical providers in the care of A-T patients and the impact of patient satisfaction on healthcare outcomes, we further analyzed, via a novel survey, how patients and caregivers perceived their primary A-T healthcare provider's A-T expertise, trustworthiness, accessibility, and level of compassion. It was found that a diagnosis of A-T complexly impacts patient PROs, but such data offers the potential for preventative and therapeutic interventions to improve the care of such patients. While most A-T patients and their caregivers feel their primary A-T medical provider has expertise and compassion in addition to being accessible and trustworthy, a significant percentage of study subjects did not agree that their provider was an expert in A-T or overall trustworthy.

Identifiants

DOI: 10.1007/s10875-024-01652-6 PMID: 38231422
pubmed: 38231422
doi: 10.1007/s10875-024-01652-6
pii: 10.1007/s10875-024-01652-6
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

51

Informations de copyright

© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

Références

Petley E, Yule A, Alexander S, Ojha S, Whitehouse WP. The natural history of ataxia-telangiectasia (A-T): a systematic review. Plos ONE. 2022;17(3):e0264177. https://doi.org/10.1371/journal.pone.0264177 .
doi: 10.1371/journal.pone.0264177 pubmed: 35290391 pmcid: 9049793
van Os NJH, et al. Classic ataxia-telangiectasia: the phenotype of long-term survivors. J Neurol. 2020;267(3):830–7. https://doi.org/10.1007/s00415-019-09641-1 .
doi: 10.1007/s00415-019-09641-1 pubmed: 31776720
McGlashan HL, et al. Quality of life and neurological disability in children and young people with ataxia telangiectasia. Eur J Paediatr Neurol. 2022;40:34–9. https://doi.org/10.1016/j.ejpn.2022.07.004 .
doi: 10.1016/j.ejpn.2022.07.004 pubmed: 35932633
Félix E, Gimenes AC, Costa-Carvalho BT. Effects of inspiratory muscle training on lung volumes, respiratory muscle strength, and quality of life in patients with ataxia telangiectasia. Pediatr Pulmonol. 2014;49(3):238–44. https://doi.org/10.1002/ppul.22828 .
doi: 10.1002/ppul.22828 pubmed: 23956159
Robinson CA. Trust, health care relationships, and chronic illness. Glob Qual Nurs Res. 2016;3:2333393616664823. https://doi.org/10.1177/2333393616664823 .
doi: 10.1177/2333393616664823 pubmed: 28508016 pmcid: 5415286
Doyle C, Lennox L, Bell D. A systematic review of evidence on the links between patient experience and clinical safety and effectiveness. BMJ Open. 2013;3(1):e001570. https://doi.org/10.1136/bmjopen-2012-001570 .
doi: 10.1136/bmjopen-2012-001570 pubmed: 23293244 pmcid: 3549241
Banka G, et al. Improving patient satisfaction through physician education, feedback, and incentives. J Hosp Med. 2015;10(8):497–502. https://doi.org/10.1002/jhm.2373 .
doi: 10.1002/jhm.2373 pubmed: 26014339
“Our Mission,” A-T Children’s Project. Available:  https://www.atcp.org/aboutus/our-mission/ . Accessed 19 Jul 2023.
“Global A-T Family Data Platform," A-T Children’s Project Webpage. Available:  https://www.atcp.org/about-ataxia-telangiectasia/get-connected/global-a-t-family-data-platform/ . Accessed 28 Oct 2023.
“Why Use PROMIS,” PROMIS Health Organization Webpage. Last updated on 3/27/2023. Available:  https://www.healthmeasures.net/explore-measurement-systems/promis . Accessed 13 Jul 2023.
Rothrock NE, Amtmann D, Cook KF. Development and validation of an interpretive guide for PROMIS scores. J Patient Rep Outcomes. 2020;4:16. https://doi.org/10.1186/s41687-020-0181-7 .
doi: 10.1186/s41687-020-0181-7 pubmed: 32112189 pmcid: 7048882
“What is PROMIS?,” PROMIS Health Organization. Available:  https://www.promishealth.org/57461-2/ . Accessed 28 Oct 2023.
“Scoring Instructions.” PROMIS Health Organization Webpage. Last updated on 10/20/2023. Available: https://www.healthmeasures.net/score-and-interpret/calculate-scores/scoring-instructions . Accessed 27 Dec 2023.
Manusama OR, van Beveren NJM, van Hagen PM, Drexhage HA, Dalm VASH. Psychological symptoms in primary immunodeficiencies: a common comorbidity? J Clin Immunol. 2022;42(3):695–8. https://doi.org/10.1007/s10875-022-01207-7 .
doi: 10.1007/s10875-022-01207-7 pubmed: 35043302 pmcid: 9016014
Michniacki TF, Merz LE, McCaffery H, Connelly JA, Walkovich K. Quality of life and patient-reported outcomes in chronic severe neutropenia conditions. Int J Hematol. 2021;113(5):735–43. https://doi.org/10.1007/s12185-021-03089-8 .
doi: 10.1007/s12185-021-03089-8 pubmed: 33587282
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(2):123–33. https://doi.org/10.1007/s10875-016-0241-1 .
doi: 10.1007/s10875-016-0241-1 pubmed: 26873708
Martinez W, Carter JS, Legato LJ. Social competence in children with chronic illness: a meta-analytic review. J Pediatr Psychol. 2011;36(8):878–90. https://doi.org/10.1093/jpepsy/jsr035 .
doi: 10.1093/jpepsy/jsr035 pubmed: 21745809
Salk RH, Hyde JS, Abramson LY. Gender differences in depression in representative national samples: meta-analyses of diagnoses and symptoms. Psychol Bull. 2017;143(8):783–822. https://doi.org/10.1037/bul0000102 .
doi: 10.1037/bul0000102 pubmed: 28447828 pmcid: 5532074
Bensing JM, Hulsman RL, Schreurs KM. Gender differences in fatigue: biopsychosocial factors relating to fatigue in men and women. Med Care. 1999;37(10):1078–83. https://doi.org/10.1097/00005650-199910000-00011 .
doi: 10.1097/00005650-199910000-00011 pubmed: 10524374
Faro M, Sàez-Francás N, Castro-Marrero J, Aliste L, Fernández de Sevilla T, Alegre J. Gender differences in chronic fatigue syndrome. Reumatol Clin. 2016;12(2):72–7. https://doi.org/10.1016/j.reuma.2015.05.007 .
doi: 10.1016/j.reuma.2015.05.007 pubmed: 26190206
Rafla-Yuan E, et al. Striving for equity in community mental health: opportunities and challenges for integrating care for BIPOC youth. Child Adolesc Psychiatr Clin N Am. 2022;31(2):295–312. https://doi.org/10.1016/j.chc.2021.11.007 .
doi: 10.1016/j.chc.2021.11.007 pubmed: 35361366
Maura J, Weisman de Mamani A. Mental health disparities, treatment engagement, and attrition among racial/ethnic minorities with severe mental illness: a review. J Clin Psychol Med Settings. 2017;24(3–4):187–210. https://doi.org/10.1007/s10880-017-9510-2 .
doi: 10.1007/s10880-017-9510-2 pubmed: 28900779
Gliwska E, Guzek D, Przekop Z, Sobocki J, Głąbska D. Quality of life of cancer patients receiving enteral nutrition: a systematic review of randomized controlled trials. Nutrients. 2021;13(12):4551. https://doi.org/10.3390/nu13124551 .
doi: 10.3390/nu13124551 pubmed: 34960103 pmcid: 8705712
Ojo O, Keaveney E, Wang X-H, Feng P. The effect of enteral tube feeding on patients’ health-related quality of life: a systematic review. Nutrients. 2019;11(5):1046. https://doi.org/10.3390/nu11051046 .
doi: 10.3390/nu11051046 pubmed: 31083338 pmcid: 6566785

Auteurs

Nadine Kridli (N)

University of Massachusetts Chan Medical School, Worcester, MA, USA.

Julie Sturza (J)

Department of Statistics, University of Michigan, Ann Arbor, MI, USA.

Thomas F Michniacki (TF)

Department of Pediatrics, Pediatric Hematology/Oncology, University of Michigan, D4202 Medical Professional Building, 1500 E. Medical Center Drive, Ann Arbor, MI, 48109-5718, USA. tmich@med.umich.edu.
ORCID: 0000-0001-8919-5243

Classifications MeSH