Quality of Life Research

, Volume 12, Issue 5, pp 565–574 | Cite as

Patients' health-related quality-of-life and health state values for motor neurone disease/amyotrophic lateral sclerosis

  • C. Green
  • G. Kiebert
  • C. Murphy
  • J.D. Mitchell
  • M. O'Brien
  • A. Burrell
  • P.N. Leigh


Motor neurone disease (MND) has a severe impact on patient quality of life, especially in later stages of the disease. This study assesses the health-related quality of life (HRQL) of MND patients, and for the first time elicits health state values from patients for their present health state. A structured interview was conducted with 77 patients. Patients completed a disease specific health status measure (ALSAQ-40), a generic health status measure (EuroQol EQ-5D), a visual analogue scale (VAS) and a standard gamble (SG) exercise. The ALSAQ-40 was sensitive to disease severity. Patients' mean VAS rating of their own health ranged from 0.74 for stage 1 (early) disease severity (n = 15), to 0.37 for stage 4 (late stage) disease severity (n = 19). Utilities elicited via SG varied from a mean of 0.79 for stage 1 disease severity to a mean of 0.45 for stage 4 disease severity. The EQ-5D derived single index ranged from a mean of 0.63 for stage 1 disease severity to a mean of −0.01 for stage 4 disease severity. This study demonstrates that it is feasible and practical to obtain health state values from MND patients and it provides evidence that patients place a high value on their HRQL, even in cases where health status is very poor.

EQ-5D Health state valuation Motor neurone disease Quality of life Standard gamble 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Riviere M, Meininger P, Zeisser P, et al. An analysis of extended survival in patients with amyotrophic lateral sclerosis treated with riluzole. Arch Neurol 1998; 55: 526–528.Google Scholar
  2. 2.
    Donaghy M. The motor neurone diseases. In: Wetherall DJ, Warrell DA (eds), Oxford Textbook of Medicine, 3rd, edn. Vol. 3. Oxford: Oxford University Press, 4087–4090.Google Scholar
  3. 3.
    Bernat JL, Vincent FM. Neurology. Oradell, New Jersey: Medical Economics Books, 1987.Google Scholar
  4. 4.
    Jablecki CK, Berry C, Leach J. Survival prediction in amyotrophic lateral sclerosis. Muscle Nerve 1989; 12: 833–841.Google Scholar
  5. 5.
    Norris F, Shepherd R, Denys E, et al. Onset, natural history and outcome in idiopathic adult motor neuron disease. J Neurl Sci 1993; 118: 48–55.Google Scholar
  6. 6.
    Benzimon G, Lacomblez L, Meininger V and the ALS/ Riluzole Study Group. A controlled trial of riluzole in amyotrophic lateral sclerosis. New Eng J Med 1994; 330: 585–591.Google Scholar
  7. 7.
    Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V for the Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Dose-ranging of riluzole in amyotrophic lateral sclerosis. Lancet 1996; 347: 1425–1431.Google Scholar
  8. 8.
    Shields RK, Ruhland JL, Ross MA, et al. Analysis of health-related quality of life and muscle impairment in individuals with amyotrophic lateral sclerosis using the medical outcome survey and the Tufts Quantitative Neuromuscular Exam. Arch Phys Med Rehabilit 1998; 79: 855–862.Google Scholar
  9. 9.
    Damiano AM, Patrick DL, Guzman GI, et al. Measurement of health-related quality of life in patients with amyotrophic lateral sclerosis in clinical trials of new therapies. Med Care 1999; 37: 15–26.Google Scholar
  10. 10.
    Green C, Brazier J, Deverill M. Valuing health-related quality of life: A review of health state valuation techniques. PharmacoEconomics 2000; 17: 151–165.Google Scholar
  11. 11.
    Jenkinson C, Fitzpatrick R, Brennan C, Swash M. Evidence for the validity and reliability of the ALS assessment questionnaire: The ALSAQ-40. ALS Motor Neuron Disorders 1999; 1: 33–40.Google Scholar
  12. 12.
    The EuroQol Group. EuroQol-a new facility for the measurement of health related quality of life. Health Policy 1990; 16: 199–208.Google Scholar
  13. 13.
    Kind P. The EuroQol instrument: An index of health-related quality of life. In: Spilker B, (ed), Quality of Life and PharmacoEconomics in Clinical Trials. Philadelphia: Lippincott Raven, 1996.Google Scholar
  14. 14.
    Williams A. A social tariff for EuroQol: Results from a UK general population survey. York Centre for Health Economics Discussion Paper No. 138. University of York, 1996.Google Scholar
  15. 15.
    Dolan P. Modeling valuations for EuroQol health states. Med Care 1997; 35(11): 1095–1108.Google Scholar
  16. 16.
    Von Neumann J, Morgenstern O. Theories of Games and Economic Behavior. New York: Wiley, 1953.Google Scholar
  17. 17.
    Torrance, G.W. Measurement of health state utilities for economic appraisal: A review. J Health Econ 1986; 5: 1–30.Google Scholar
  18. 18.
    Llewellyn-Thomas HA, Thiel ED, McGreal MJ. Cancer patients' evaluations of their current health states: The in-fluence of expectations, comparisons, actual health status and mood. Med Dec Making 1992; 12: 115–122.Google Scholar
  19. 19.
    Ramsey SD, Patrick DL, Lewis S, et al. Improvement in quality of life after lung transplantation: A preliminary study. J Heart Lung Transplant 1995; 14: 870–877.Google Scholar
  20. 20.
    Furlong W, Feeny D, Torrance GW, Barr R, Horsman J. Guide to design and development of health-state utility instrumentation, CHEPA Working Paper, McMaster University, Hamilton, Ontario, Canada, 1989.Google Scholar
  21. 21.
    Jenkinson, C, Fitzpatrick R, Brennan C, Bromberg M, Swash M. Development and validation of a short measure of health status for individuals with amyotrophic lateral sclerosis/motor neuron disease: The ALSAQ-40. J Neurol 1999; 246: III/16–III21.Google Scholar
  22. 22.
    Dolan P, Gudex C, Kind P, et al. Valuing health states: A comparison of methods. J Health Econ 1996; 15: 209–231.Google Scholar
  23. 23.
    Richardson J. Cost utility analysis: What should be measured? Soc Sci Med 1994; 39: 7–21.Google Scholar
  24. 24.
    Brazier JE, Deverill M, Green C, et al. A review of the use of health status measures in economic evaluation. NHS Health Technol Assess 1999; 3 (9).Google Scholar
  25. 25.
    Nord E. Methods for quality adjustment of life years. Soc Sci Med 1992; 34: 559–569.Google Scholar
  26. 26.
    Loomes G, McKenzie L. The use of QALYs in health care decision making. Soc Sci Med 1989; 28: 299–308.Google Scholar
  27. 27.
    Sackett DL, Torrance GW. The utility of different health states as perceived by the general public. J Chron Dis 1978; 31: 697–704.Google Scholar
  28. 28.
    Boyd NF, Sutherland HJ, Heasman KZ, Tritchler DL, Cummings BJ. Whose utilities for decision analysis? Med Decis Making 1990; 10: 58–67.Google Scholar
  29. 29.
    Hurst NP, Jobanputra P, Hunter M, Lambert M, Lockhead A, Brown H. Validity of EuroQol-ageneric health status instrument-in patients with rheumatoid arthritis. B. J Rheum 1994; 24: 973–980.Google Scholar
  30. 30.
    Jenkinson C, Gray A, Doll H, Lawrence K, Keoghane S, Layte R. Evaluation of index and profile measures of health status in a randomized controlled trial: Comparison of the Medical Outcomes Study 36-item short form health survey, EuroQol and disease specific measures. Med Care 1997; 35: 1109–1118.Google Scholar
  31. 31.
    Gold MR, Siegel JE, Russell LB, Weinstein MC (eds), Cost Effectiveness in Health and Medicine. New York: Oxford University Press, 1996.Google Scholar
  32. 32.
    Nord E, Pinto JL, Richardson G, Menzel P, Ubel P. Incorporating societal concerns for fairness in numerical valuations of health programmes. Health Econ 1999; 8: 25–39.Google Scholar
  33. 33.
    Dolan P. Whose preferences count? Med Dec Making 1999; 482–486.Google Scholar
  34. 34.
    Froberg DG, Kane RL. Methodology for measuring health-state preferences-II: Scaling methods. J Clin Epidemiol 1989; 42: 459–471.Google Scholar

Copyright information

© Kluwer Academic Publishers 2003

Authors and Affiliations

  • C. Green
    • 1
  • G. Kiebert
    • 2
  • C. Murphy
    • 3
  • J.D. Mitchell
    • 4
  • M. O'Brien
    • 4
  • A. Burrell
    • 5
  • P.N. Leigh
    • 3
  1. 1.Wessex Institute for Health Research and Development, BoldrewoodUniversity of Southampton, SouthamptonLondonUK
  2. 2.MEDTAP InternationalLondonUK
  3. 3.Department of Neurology, Guy's King's and St Thomas' School of Medicine and the Institute of PsychiatryKing's CollegeLondonUK
  4. 4.Department of NeurologyRoyal Preston HospitalPrestonUK
  5. 5.Aventis Pharma LtdKentUK

Personalised recommendations