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Journal of Inherited Metabolic Disease

, Volume 24, Issue 7, pp 733–747 | Cite as

Self-rated psychosocial consequences and quality of life in the acute porphyrias

  • L. M. Millward
  • P. Kelly
  • A. Deacon
  • V. Senior
  • T. J. Peters
Article

Abstract

A battery of self-report psychosocial measures was mailed to 116 patients who had been referred for clinical management (clinic attenders) or laboratory diagnosis (non-clinic attenders) to the London Supraregional Assay Service Centre for Porphyria over the past decade and who tested positive for porphyria. Usable replies were received from 81 (70%) patients. Our interest focused on the prevalence of psychosocial symptoms in acute porphyrias and the perceived effects of porphyria on quality of life and patient experience. Research questions examined included (i), lifestyle factors; (ii) life events; (iii) mental health; (iv) general health; and (v) perceptions of illness of patients receiving specialist clinical management compared to respondents referred for diagnostic investigations, between patients with latent or manifest symptomology and between patients with different types of porphyria.

Patients with porphyria have an impaired quality of life, particularly manifest cases, compared to controls and to diabetic patients. Depression, and particularly anxiety, is more common than in the general population or general medical outpatient attenders. Quality of life is lower in acute intermittent porphyria (AIP) than in other forms of porphyria and a significant number of patients had major life event consequences, e.g. failure to secure, or loss of, employment, limitation of family size. Patients attending a clinic providing specialist porphyria advice, management and counselling received some perceived lifestyle benefits.

Keywords

Porphyria Laboratory Diagnosis Major Life Diagnostic Investigation Event Consequence 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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REFERENCES

  1. Allen JP, Litten RZ, Fertig JB, Babor T (1997) A review of research on the Alcohol Use Disorders Identification Test (AUDIT). Alcohol: Clin Exp Res 21: 613-619.Google Scholar
  2. Anderson JSC, Sullivan F, Usherwood TP (1990) The Medical Outcomes Study Instrument (MOSI)—use of a new health status measure in Britain. Fam Pract 7: 205-218.Google Scholar
  3. Andersson E (1993) The hospital anxiety and depression scale. Homogeneity of the subscales. Soc Behav Personal 21: 197-204.Google Scholar
  4. Babor T, de la Fuente JR, Saunders J, Grant M (1989) The Alcohol Use Disorders Identification Test: Guidelines for Use in Primary Health Care. Geneva: World Health Organisation, publication no. PSA/92.4.Google Scholar
  5. Burgoyne K, Swartz R, Ananth J (1995) Porphyria: reexamination of psychiatric implications. Psychother Psychosom 64: 121-130.Google Scholar
  6. Crimlisk HL (1997) The little imitator—porphyria: a neuropsychiatric disorder. J Neurol Neurosurg Psychiatry 62: 319-328.Google Scholar
  7. Department of Health (1998) Health Survey for England 1996. HMSO: London.Google Scholar
  8. Doss MO, Kuhnel A, Gross U (2000) Alcohol and porphyrin metabolism. Alcohol Alcohol 35: 109-125.Google Scholar
  9. Elder GH, Hift RJ, Meissner PN (1997) The acute porphyrias. Lancet 349: 1613-1617.Google Scholar
  10. Estrov Y, Scaglia F, Bodamer OAF (2000) Psychiatric symptoms of inherited metabolic disease. J Inherit Metab Dis 23: 2-6.Google Scholar
  11. EuroQol Group (1998) EuroQol ED-5D User Guide. University of York, UK: Centre for Health Economics.Google Scholar
  12. Kind P, Dolan P, Gudex C, and Williams A (1998) Variations in population health status: results from a United Kingdom national questionnaire survey. Br Med J 316: 736-741.Google Scholar
  13. MacKenzie DM, Langa A and Brown TM (1996) Identifying hazardous or harmful alcohol use in medical admissions: a comparison of AUDIT, CAGE and brief MAST. Alcohol Alcohol 31: 591-599.Google Scholar
  14. McEwin R, Lawn J, Jonas CT (1972) A survey of porphyria among psychiatric patients. Med J Austr 2: 303-306.Google Scholar
  15. Patience DA, Blackwood DHR, McColl KEL, Moore MR (1994) Acute intermittent porphyria and mental illness—a family study. Acta Psychiatr Scand 89: 262-267.Google Scholar
  16. Piccinelli M, Tessari E, Bortolomasi M, et al (1997) Efficacy of the alcohol use disorders identification test as a screening tool for hazardous alcohol intake and related disorders in primary care: a validity study. Br Med J 314: 420-424.Google Scholar
  17. Regan L, Gonsalves L, Tesar G (1999) Acute intermittent porphyria. Psychosomatics 40: 521-523.Google Scholar
  18. Rufener EA (1987) Erythropoietic protoporphyria: a study of its psychosocial aspects. Br J Dermatol 116: 703-708.Google Scholar
  19. Sack GH (1990) Acute intermittent porphyria. J Am Med Assoc 264: 1290-1293.Google Scholar
  20. Santosh PJ, Malhotra S (1994) Varied psychiatric manifestations of acute intermittent porphyria. Biol Psychiatry 36: 744-747.Google Scholar
  21. Saunders JB, Aasland OG, Babor TF, de la Fuente JR, Grant M (1993) Development of the Alcohol Use Disorders Identification Test (AUDIT). Addiction 88: 791-804.Google Scholar
  22. Stewart AL, Hays RD, Ware JE (1988) The MOS short-form general health survey. Med Care 26: 724-735.Google Scholar
  23. Stewart AL, Greenfield S, Hays RD, et al (1989) Functional status and well-being of patients with chronic conditions. J Am Med Assoc 262: 907-913.Google Scholar
  24. Thadani H, Deacon A, Peters T (2000) Diagnosis and management of porphyria. Br Med J 320: 1647-1651.Google Scholar
  25. Thunell S, Floderus Y, Henrichson A, Moore MR, Meissner P, Sinclair J (1992) Alcoholic beverages in acute porphyria. J Stud Alcohol 53: 272-276.Google Scholar
  26. Tishler PV, Woodward B, O'Connor J, et al (1985) High prevalence of intermittent acute porphyria in a psychiatric patient population. Am J Psychiatry 142: 1430-1436.Google Scholar
  27. Weinman J, Petrie KJ, Moss-Morris R, Horne R (1996) The Illness Perception Questionnaire: a new method for assessing the cognitive representation of illness. Psychol Health 11: 431-445.Google Scholar
  28. Wilkin D, Hallam L, Doggett MA (1992) Measures of Need and Outcome for Primary Health Care. Oxford: Oxford University Press.Google Scholar
  29. Zigmond AS and Snaith RP (1983) The hospital anxiety and depression scale. Acta Psychiatry Scand 67: 361-370.Google Scholar

Copyright information

© Kluwer Academic Publishers 2001

Authors and Affiliations

  • L. M. Millward
    • 1
  • P. Kelly
    • 1
  • A. Deacon
    • 1
  • V. Senior
    • 1
  • T. J. Peters
    • 1
  1. 1.Departments of Clinical Biochemistry and Psychological Medicine and Psychology Research Group, GKT School of MedicineKing's CollegeLondonUK

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