Social Indicators Research

, Volume 120, Issue 3, pp 871–882 | Cite as

Relationships Between Disease Severity, Social Support and Health-Related Quality of Life in Patients with Amyotrophic Lateral Sclerosis

  • Benjamin Ilse
  • Tino PrellEmail author
  • Mario Walther
  • Viktor Hartung
  • Susanne Penzlin
  • Florian Tietz
  • Otto-Wilhelm Witte
  • Bernhard Strauss
  • Julian Grosskreutz


Quality of life (QOL) is an important issue in patients with amyotrophic lateral sclerosis (ALS). QOL measurements can help the support care team understand how to maintain or improve QOL in ALS patients. The purpose of this study was to describe the relationship between health-related QOL, disease severity and social support in ALS patients. In the current study, 49 German ALS patients were evaluated using the European quality of life score (EQ-5D), ALS Functional Rating Scale in its revised form (ALSFRS-R), Social Support Questionnaire (F-SozU K-14) and the Beck depression inventory. Data concerning patient history and socioeconomic background were collected using a semi-structured interview. Age, gender, number of children, habitation, socioeconomic status and disease duration were not significantly related to health-related QOL (EQ-5D). Positive correlations were found between the ALSFRS-R, social support and health-related QOL, whereas depression was negatively correlated with the ALSFRS-R score. A multiple linear regression model indicated a significant influence of the ALSFRS-R score on health-related QOL in ALS patients, with an additional trend for social support as a predictor. These results suggest that because of the logarithmic association between measures, compensatory therapy for loss of health-related QOL should be optimised during the course of the disease in ALS patients.


Quality of life Health-related quality of life Social support ALSFRS-R Motor neuron disease 


  1. Averill, A. J., Kasarskis, E. J., & Segerstrom, S. C. (2007). Psychological health in patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 8(4), 243–254.CrossRefGoogle Scholar
  2. Cedarbaum, J. M., Stambler, N., Malta, E., Fuller, C., Hilt, D., Thurmond, B., et al. (1999). The ALSFRS-R: A revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS study group (phase III). Journal of the Neurological Sciences, 169(1–2), 13–21.CrossRefGoogle Scholar
  3. Chio, A., Gauthier, A., Montuschi, A., Calvo, A., Di Vito, N., Ghiglione, P., et al. (2004). A cross sectional study on determinants of quality of life in ALS. Journal of Neurology, Neurosurgery and Psychiatry, 75(11), 1597–1601.CrossRefGoogle Scholar
  4. Clarke, S., Hickey, A., O’Boyle, C., & Hardiman, O. (2001). Assessing individual quality of life in amyotrophic lateral sclerosis. Quality of Life Research, 10(2), 149–158.CrossRefGoogle Scholar
  5. Cupp, J., Simmons, Z., Berg, A., Felgoise, S. H., Walsh, S. M., & Stephens, H. E. (2011). Psychological health in patients with ALS is maintained as physical function declines. Amyotrophic Lateral Sclerosis, 12(4), 290–296.CrossRefGoogle Scholar
  6. De Groot, I. J., Post, M. W., van Heuveln, T., van den Berg, L. H., & Lindeman, E. (2007). Cross-sectional and longitudinal correlations between disease progression and different health-related quality of life domains in persons with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 8(6), 356–361.CrossRefGoogle Scholar
  7. Donyavi, T., Naieni, K. H., Nedjat, S., Vahdaninia, M., Najafi, M., & Montazeri, A. (2011). Socioeconomic status and mortality after acute myocardial infarction: A study from Iran. International Journal for Equity in Health, 10(1), 9.CrossRefGoogle Scholar
  8. Dupuis, L., Dengler, R., Heneka, M. T., Meyer, T., Zierz, S., Kassubek, J., et al. (2012). A randomized, double blind, placebo-controlled trial of pioglitazone in combination with riluzole in amyotrophic lateral sclerosis. PLoS One, 7(6), e37885.CrossRefGoogle Scholar
  9. Epton, J., Harris, R., & Jenkinson, C. (2009). Quality of life in amyotrophic lateral sclerosis/motor neuron disease: A structured review. Amyotrophic Lateral Sclerosis, 10(1), 15–26.CrossRefGoogle Scholar
  10. Fegg, M. J., Kogler, M., Brandstatter, M., Jox, R., Anneser, J., Haarmann-Doetkotte, S., et al. (2010). Meaning in life in patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 11(5), 469–474.CrossRefGoogle Scholar
  11. Fydrich, T., Sommer, G., Tydecks, S., & Brähler, E. (2009). Fragebogen zur sozialen Unterstützung (F-SozU): Normierung der Kurzform (K-14) Social Support Questionnaire (F-SozU): Standardization of short form (K-14). Zeitschrift für Medizinische Psychologie, 18(1), 43–48.Google Scholar
  12. Ganzini, L., Johnston, W. S., & Hoffman, W. F. (1999). Correlates of suffering in amyotrophic lateral sclerosis. Neurology, 52(7), 1434–1440.CrossRefGoogle Scholar
  13. Gignac, M. A., Cao, X., Mcalpine, J., & Badley, E. M. (2011). Measures of disability: Arthritis Impact Measurement Scales 2 (AIMS2), Arthritis Impact Measurement Scales 2-Short Form (AIMS2-SF), The Organization for Economic Cooperation and Development (OECD) Long-Term Disability (LTD) Questionnaire, EQ-5D, World Health Organization Disability Assessment Schedule II (WHODASII), Late-Life Function and Disability Instrument (LLFDI), and Late-Life Function and Disability Instrument-Abbreviated Version (LLFDI-Abbreviated). American College of Rheumatology, 63(Suppl 11), S308–S324.Google Scholar
  14. Goldstein, L. H., Atkins, L., & Leigh, P. N. (2002). Correlates of quality of life in people with motor neuron disease (MND). Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders, 3(3), 123–129.CrossRefGoogle Scholar
  15. Green, C., Kiebert, G., Murphy, C., Mitchell, J. D., O’Brien, M., Burrell, A., et al. (2003). Patients’ health-related quality-of-life and health state values for motor neurone disease/amyotrophic lateral sclerosis. Quality of Life Research, 12(5), 565–574.CrossRefGoogle Scholar
  16. Grehl, T., Rupp, M., Budde, P., Tegenthoff, M., & Fangerau, H. (2011). Depression and QOL in patients with ALS: How do self-ratings and ratings by relatives differ? Quality of Life Research, 20(4), 569–574.CrossRefGoogle Scholar
  17. Hecht, M., Hillemacher, T., Grasel, E., Tigges, S., Winterholler, M., Heuss, D., et al. (2002). Subjective experience and coping in ALS. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders, 3(4), 225–231.Google Scholar
  18. Hjermstad, M. J., Fayers, P. M., Bjordal, K., & Kaasa, S. (1998). Using reference data on quality of life: The importance of adjusting for age and gender, exemplified by the EORTC QLQ-C30 (+3). European Journal of Cancer, 34(9), 1381–1389.CrossRefGoogle Scholar
  19. Hogg, K. E., Goldstein, L. H., & Leigh, P. N. (1994). The psychological impact of motor neurone disease. Psychological Medicine, 24(3), 625–632.CrossRefGoogle Scholar
  20. Hunter, M. D., Robinson, I. C., & Neilson, S. (1993). The functional and psychological status of patients with amyotrophic lateral sclerosis: Some implications for rehabilitation. Disability and Rehabilitation, 15(3), 119–126.CrossRefGoogle Scholar
  21. Jelsone-Swain, L., Persad, C., Votruba, K. L., Weisenbach, S. L., Johnson, T., Gruis, K. L., et al. (2012). The relationship between depressive symptoms, disease state, and cognition in amyotrophic lateral sclerosis. Frontiers in Psychology, 3, 542.CrossRefGoogle Scholar
  22. Jenkinson, C., Fitzpatrick, R., Swash, M., & Peto, V. (2000). The ALS health profile study: Quality of life of amyotrophic lateral sclerosis patients and carers in Europe. Journal of Neurology, 247(11), 835–840.CrossRefGoogle Scholar
  23. Kaub-Wittemer, D., Steinbuchel, N., Wasner, M., Laier-Groeneveld, G., & Borasio, G. D. (2003). Quality of life and psychosocial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. Journal of Pain and Symptom Management, 26(4), 890–896.CrossRefGoogle Scholar
  24. Kiebert, G. M., Green, C., Murphy, C., Mitchell, J. D., O’Brien, M., Burrell, A., et al. (2001). Patients’ health-related quality of life and utilities associated with different stages of amyotrophic lateral sclerosis. Journal of the Neurological Sciences, 191(1–2), 87–93.CrossRefGoogle Scholar
  25. Konig, H. H., Bernert, S., & Angermeyer, M. C. (2005). Health Status of the German population: results of a representative survey using the EuroQol questionnaire. Gesundheitswesen, 67(3), 173–182.CrossRefGoogle Scholar
  26. Logroscino, G., Traynor, B. J., Hardiman, O., Chio’, A., Couratier, P., Mitchell, J. D., et al. (2008). Descriptive epidemiology of amyotrophic lateral sclerosis: New evidence and unsolved issues. Journal of Neurology, Neurosurgery and Psychiatry, 79(1), 6–11.CrossRefGoogle Scholar
  27. Lule, D., Pauli, S., Altintas, E., Singer, U., Merk, T., Uttner, I., et al. (2012). Emotional adjustment in amyotrophic lateral sclerosis (ALS). Journal of Neurology, 259(2), 334–341.CrossRefGoogle Scholar
  28. Mahoney, F. I., & Barthel, D. W. (1965). Functional evaluation: The barthel index. Maryland State Medical Journal, 14, 61–65.Google Scholar
  29. Matuz, T., Birbaumer, N., Hautzinger, M., & Kubler, A. (2010). Coping with amyotrophic lateral sclerosis: An integrative view. Journal of Neurology, Neurosurgery and Psychiatry, 81(8), 893–898.CrossRefGoogle Scholar
  30. McDonald, E. R., Hillel, A., & Wiedenfeld, S. A. (1996). Evaluation of the psychological status of ventilatory-supported patients with ALS/MND. Palliative Medicine, 10(1), 35–41.CrossRefGoogle Scholar
  31. McLeod, J. E., & Clarke, D. M. (2007). A review of psychosocial aspects of motor neurone disease. Journal of the Neurological Sciences, 258(1–2), 4–10.CrossRefGoogle Scholar
  32. Neudert, C., Wasner, M., & Borasio, G. D. (2004). Individual quality of life is not correlated with health-related quality of life or physical function in patients with amyotrophic lateral sclerosis. Journal of Palliative Medicine, 7(4), 551–557.CrossRefGoogle Scholar
  33. Nygren, I., & Askmark, H. (2006). Self-reported quality of life in amyotrophic lateral sclerosis. Journal of Palliative Medicine, 9(2), 304–308.CrossRefGoogle Scholar
  34. Olsson Ozanne AG, Strang, S., & Persson, L. I. (2011). Quality of life, anxiety and depression in ALS patients and their next of kin. Journal of Clinical Nursing, 20(1–2), 283–291.Google Scholar
  35. Peric’, S., Rakocevic-Stojanovic, V., Stevic, Z., Basta, I., Pavlovic, S., Vujanac, V., et al. (2010). Health-related quality of life in patients with myotonic dystrophy type 1 and amyotrophic lateral sclerosis. Acta Neurologica Belgica, 110(1), 71–77.Google Scholar
  36. Rabkin, J. G., Wagner, G. J., & Del Bene, M. (2000). Resilience and distress among amyotrophic lateral sclerosis patients and caregivers. Psychosomatic Medicine, 62(2), 271–279.CrossRefGoogle Scholar
  37. Robbins, R. A., Simmons, Z., Bremer, B. A., Walsh, S. M., & Fischer, S. (2001). Quality of life in ALS is maintained as physical function declines. Neurology, 56(4), 442–444.CrossRefGoogle Scholar
  38. Rothwell, P. M., McDowell, Z., Wong, C. K., & Dorman, P. J. (1997). Doctors and patients don’t agree: Cross sectional study of patients’ and doctors’ perceptions and assessments of disability in multiple sclerosis. BMJ, 314(7094), 1580–1583.CrossRefGoogle Scholar
  39. Sangha, H., Lipson, D., Foley, N., Salter, K., Bhogal, S., Pohani, G., et al. (2005). A comparison of the barthel index and the functional independence measure as outcome measures in stroke rehabilitation: Patterns of disability scale usage in clinical trials. International Journal of Rehabilitation Research, 28(2), 135–139.CrossRefGoogle Scholar
  40. Simmons, Z., Bremer, B. A., Robbins, R. A., Walsh, S. M., & Fischer, S. (2000). Quality of life in ALS depends on factors other than strength and physical function. Neurology, 55(3), 388–392.CrossRefGoogle Scholar
  41. Taylor, L., Wicks, P., Leigh, P. N., & Goldstein, L. H. (2010). Prevalence of depression in amyotrophic lateral sclerosis and other motor disorders. European Journal of Neurology, 17(8), 1047–1053.CrossRefGoogle Scholar
  42. Tedman, B. M., Young, C. A., & Williams, I. R. (1997). Assessment of depression in patients with motor neuron disease and other neurologically disabling illness. Journal of the Neurological Sciences, 152(Suppl 1), S75–S79.CrossRefGoogle Scholar
  43. Tramonti, F., Bongioanni, P., Fanciullacci, C., & Rossi, B. (2012). Balancing between autonomy and support: Coping strategies by patients with amyotrophic lateral sclerosis. Journal of the Neurological Sciences, 320(1–2), 106–109.CrossRefGoogle Scholar
  44. Van den Berg, J. P., Kalmijn, S., Lindeman, E., Veldink, J. H., de Visser, M., van der Graaff, M. M., et al. (2005). Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology, 65(8), 1264–1267.CrossRefGoogle Scholar
  45. Wilson, I. B. (1999). Clinical understanding and clinical implications of response shift. Social Science and Medicine, 48(11), 1577–1588.CrossRefGoogle Scholar
  46. Winter, Y., Schepelmann, K., Spottke, A. E., Claus, D., Grothe, C., Schroder, R., et al. (2010). Health-related quality of life in ALS, myasthenia gravis and facioscapulohumeral muscular dystrophy. Journal of Neurology, 257(9), 1473–1481.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2014

Authors and Affiliations

  • Benjamin Ilse
    • 5
  • Tino Prell
    • 1
    Email author
  • Mario Walther
    • 2
  • Viktor Hartung
    • 1
  • Susanne Penzlin
    • 1
  • Florian Tietz
    • 1
  • Otto-Wilhelm Witte
    • 1
    • 4
  • Bernhard Strauss
    • 3
  • Julian Grosskreutz
    • 1
  1. 1.Hans-Berger Department of NeurologyJena University HospitalJenaGermany
  2. 2.Institute of Medical Statistics, Computer Sciences and DocumentationJena University HospitalJenaGermany
  3. 3.Institute for Psychosocial Medicine and PsychotherapyJena University HospitalJenaGermany
  4. 4.Center for Sepsis Control and Care (CSCC)Jena University HospitalJenaGermany
  5. 5.Department of Palliative MedicineUniversity Medical CentreGöttingenGermany

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