Literature DB >> 32513788

Prenusinersen economic and health-related quality of life burden of spinal muscular atrophy.

Georgina M Chambers1, Stella Nalukwago Settumba2, Kate A Carey2, Anita Cairns2, Manoj P Menezes2, Monique Ryan2, Michelle A Farrar2.   

Abstract

OBJECTIVE: To quantify the economic and health-related quality of life (HRQoL) burden incurred by households with a child affected by spinal muscular atrophy (SMA).
METHODS: Hospital records, insurance claims, and detailed resource use questionnaires completed by caregivers were used to capture the direct and indirect costs to households of 40 children affected by SMA I, II, and III in Australia between 2016 and 2017. Prevalence costing methods were used and reported in 2017 US dollar (USD) purchasing power parity (PPP). The HRQoL for patients and primary caregivers was quantified with the youth version of the EQ-5D and CareQoL multiattribute utility instruments and Australian utility weights.
RESULTS: The average total annual cost of SMA per household was $143,705 USD PPP for all SMA types (SMA I $229,346, SMA II $150,909, SMA III $94,948). Direct costs accounted for 56% of total costs. The average total indirect health care costs for all SMA types were $63,145 per annum and were highest in families affected by SMA II. Loss of income and unpaid informal care made up 24.2% and 19.8% respectively, of annual SMA costs. Three of 4 (78%) caregivers stated that they experienced financial problems because of care tasks. The loss in HRQoL of children affected by SMA and caregivers was substantial, with average caregiver and patient scores of 0.708 and 0.115, respectively (reference range 0 = death and 1 = full health).
CONCLUSION: Our results demonstrate the substantial and far-ranging economic and quality of life burden on households and society of SMA and are essential to fully understanding the health benefits and cost-effectiveness associated with emerging disease-modifying therapies for SMA.
© 2020 American Academy of Neurology.

Entities:  

Year:  2020        PMID: 32513788     DOI: 10.1212/WNL.0000000000009715

Source DB:  PubMed          Journal:  Neurology        ISSN: 0028-3878            Impact factor:   9.910


  10 in total

1.  Cost-effectiveness analysis of gene-based therapies for patients with spinal muscular atrophy type I in Australia.

Authors:  Tianjiao Wang; Paul Scuffham; Joshua Byrnes; Martin Downes
Journal:  J Neurol       Date:  2022-08-18       Impact factor: 6.682

Review 2.  Parents as informal caregivers of children and adolescents with spinal muscular atrophy: a systematic review of quantitative and qualitative data on the psychosocial situation, caregiver burden, and family needs.

Authors:  Maja Brandt; Lene Johannsen; Laura Inhestern; Corinna Bergelt
Journal:  Orphanet J Rare Dis       Date:  2022-07-19       Impact factor: 4.303

3.  Costs of Illness of Spinal Muscular Atrophy: A Systematic Review.

Authors:  Erik Landfeldt; Astrid Pechmann; Hugh J McMillan; Hanns Lochmüller; Thomas Sejersen
Journal:  Appl Health Econ Health Policy       Date:  2021-02-12       Impact factor: 2.561

Review 4.  Systematic literature review of the economic burden of spinal muscular atrophy and economic evaluations of treatments.

Authors:  Tamara Dangouloff; Camille Botty; Charlotte Beaudart; Laurent Servais; Mickaël Hiligsmann
Journal:  Orphanet J Rare Dis       Date:  2021-01-23       Impact factor: 4.123

5.  Infantile spinal muscular atrophy - the potential for cure of a fatal disease.

Authors:  J J Volpe
Journal:  J Neonatal Perinatal Med       Date:  2021

6.  Systematic Literature Review to Identify Utility Values in Patients with Spinal Muscular Atrophy (SMA) and Their Caregivers.

Authors:  C Simone Sutherland; Pollyanna Hudson; Stephen Mitchell; Noman Paracha
Journal:  Pharmacoeconomics       Date:  2021-12-15       Impact factor: 4.981

7.  Eliciting health state utilities for Aromatic L-amino Acid Decarboxylase (AADC) deficiency: a UK vignette study.

Authors:  Adam B Smith; Andria Hanbury; Katharina Buesch
Journal:  J Patient Rep Outcomes       Date:  2021-12-11

8.  Systematic Literature Review to Assess the Cost and Resource Use Associated with Spinal Muscular Atrophy Management.

Authors:  Noman Paracha; Pollyanna Hudson; Stephen Mitchell; C Simone Sutherland
Journal:  Pharmacoeconomics       Date:  2021-11-11       Impact factor: 4.981

Review 9.  Systematic Literature Review of Clinical and Economic Evidence for Spinal Muscular Atrophy.

Authors:  Min Yang; Hiroyuki Awano; Satoru Tanaka; Walter Toro; Su Zhang; Omar Dabbous; Ataru Igarashi
Journal:  Adv Ther       Date:  2022-03-20       Impact factor: 4.070

10.  Modelling the Cost-Effectiveness and Budget Impact of a Newborn Screening Program for Spinal Muscular Atrophy and Severe Combined Immunodeficiency.

Authors:  Sophy T F Shih; Elena Keller; Veronica Wiley; Michelle A Farrar; Melanie Wong; Georgina M Chambers
Journal:  Int J Neonatal Screen       Date:  2022-07-20
  10 in total

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