Abstract
Rombaut L, Malfait F, De Wandele I, Cools A, Thijs Y, De Paepe A, Calders P. Medication,
surgery, and physiotherapy among patients with the hypermobility type of Ehlers-Danlos
syndrome.
Objectives
To describe medication use, surgery, and physiotherapy, and to examine the effect
of these treatment modalities on functional impairment and amount of complaints among
patients with the hypermobility type of Ehlers-Danlos syndrome (EDS-HT).
Design
Cross-sectional study.
Setting
Physical and rehabilitation medicine department and center for medical genetics.
Participants
Patients with EDS-HT (N=79; 8 men, 71 women) were recruited for this study.
Interventions
Not applicable.
Main Outcome Measures
Patients filled out questionnaires regarding type of complaints, medication use, surgery,
physiotherapy, and outcome of treatment. Functional impairment in daily life was measured
by the Sickness Impact Profile. Pain severity was assessed with visual analog scales.
Results
Patients reported a large number of complaints, a considerable presence of severe
pain, and a clinically significant impact of disease on daily functioning. Most patients
(92.4%) used medications, among which analgesics were the most prevalent. Fifty-six
patients (70.9%) underwent surgery, including mainly interventions of the extremities
and abdomen. Forty-one patients (51.9%) are currently enrolled in a physical therapy
program, mainly comprising neuromuscular exercises, massage, and electrotherapy. Patients
with a high consumption of analgesics, who visited the physiotherapist, or who underwent
surgery had a higher dysfunction in daily life. Only 33.9% of the patients who underwent
surgery and 63.4% of patients in physical therapy reported a positive outcome.
Conclusions
Patients with EDS-HT have numerous complaints and an impaired functional status that
strongly determine their high rate of treatment consumption. The outcome of surgical
and physiotherapy treatment is disappointing in a large percentage, which illustrates
a strong need for evidence-based therapy.
Key Words
List of Abbreviations:
EDS (Ehlers-Danlos syndrome), EDS-HT (hypermobility type of EDS), NSAIDs (nonsteroidal anti-inflammatory drugs), SIP (Sickness Impact Profile), TENS (transcutaneous electrical nerve stimulation), VAS (visual analog scale), WHO (World Health Organization)To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Archives of Physical Medicine and RehabilitationAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Molecular nosology of heritable disorders of connective tissue.Am J Med Genet. 1992; 42: 431-448
- The Ehlers-Danlos syndrome.in: Royce P. Steinmann B. Connective tissue and its heritable disorders: molecular, genetic and medical aspects. Wiley-Liss, New York1993: 351-407
- Heritable disorders of connective tissue.Best Pract Res Clin Rheumatol. 2000; 14: 345-361
- Ehlers-Danlos syndrome.Ann Rheum Dis. 1970; 29: 332-333
- International nosology of heritable disorders of connective tissue, Berlin, 1986.Am J Med Genet. 1988; 29: 581-594
- Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997.Am J Med Genet. 1998; 77: 31-37
- Evaluation for sleep apnea in patients with Ehlers-Danlos syndrome and Marfan: a questionnaire study.Clin Genet. 2001; 60: 360-365
- Neuromuscular involvement in various types of Ehlers-Danlos syndrome.Ann Neurol. 2009; 65: 687-697
- Fatigue is a frequent and clinically relevant problem in Ehlers-Danlos syndrome.Semin Arthritis Rheum. 2010; 40: 267-274
- Musculoskeletal complaints, physical activity and health-related quality of life among patients with the Ehlers-Danlos syndrome hypermobility type.Disabil Rehabil. 2010; 32: 1339-1345
- Both pain and fatigue are important possible determinants of disability in patients with the Ehlers-Danlos syndrome hypermobility type.Disabil Rehabil. 2010 Nov 15; ([PubMed Epub ahead of print])
- Subjective health complaints in individuals with Ehlers-Danlos syndrome: a questionnaire study.Int J Nurs Stud. 2010; Nov 20; ([PubMed Epub ahead of print])
- Combating the burden of musculoskeletal conditions.Ann Rheum Dis. 2004; 63: 329
- The bone and joint decade.J Rheumatol. 2003; 30: 6-9
- Natural history and manifestations of the hypermobility type Ehlers-Danlos syndrome: a pilot study on 21 patients.Am J Med Genet A. 2010; 152A: 556-564
- Living a restricted life with Ehlers-Danlos syndrome (EDS).Int J Nurs Stud. 2000; 37: 111-118
- Dignity not fully upheld when seeking health care: experiences expressed by individuals suffering from Ehlers-Danlos Syndrome.Disabil Rehabil. 2010; 32: 1-7
- Ehlers-Danlos syndrome, hypermobility type.(2004 Oct 22)in: Pagon R.A. Bird T.C. Dolan C.R. Stephens K. GeneReviews. University of Washington, Seattle, Seattle1993 (Accessed April 27, 2010)
- WHO's pain ladder.(Accessed April 28, 2011)
- Management of musculoskeletal pain.Best Pract Res Clin Rheumatol. 2007; 21: 153-166
- The Sickness Impact Profile: development and final revision of a health status measure.Med Care. 1981; 19: 787-805
- The Sickness Impact Profile; results of an evaluation study of the Dutch version.Ned Tijdschr Geneeskd. 1990; 134 ([Dutch]): 1950-1954
- A French version of the Sickness Impact Profile (SIP): stages in the cross cultural validation of a generic quality of life scale.Fundam Clin Pharmacol. 1992; 6: 319-326
- Symptoms and functional health status of individuals with Ehlers-Danlos syndrome (EDS).J Clin Rheumatol. 2001; 7: 308-314
- Chronic pain is a manifestation of the Ehlers-Danlos syndrome.J Pain Symptom Manage. 1997; 14: 88-93
- Pain in Ehlers-Danlos syndrome is common, severe, and associated with functional impairment.J Pain Symptom Manage. 2010; 40: 370-378
- Opioids in rheumatic diseases.Ann N Y Acad Sci. 2010; 1193: 111-116
- Psychosocial functioning in the Ehlers-Danlos syndrome.Am J Med Genet. 1994; 53: 149-152
- Antidepressants for neuropathic pain: a Cochrane review.J Neurol Neurosurg Psychiatry. 2010; 81: 1372-1373
- Antidepressants and antiepileptic drugs for chronic non-cancer pain.Am Fam Physician. 2005; 71: 483-490
- Orthopaedic manifestations of Ehlers-Danlos syndrome.Clin Orthop Relat Res. 2000; 376: 213-221
- Total knee arthroplasty in Ehlers-Danlos syndrome.J Arthroplasty. 2004; 19: 190-196
- Joint hypermobility and rectal evacuatory dysfunction: an etiological link in abnormal connective tissue?.Neurogastroenterol Motil. 2010; 22 (1085-e283)
- Unexplained gastrointestinal symptoms and joint hypermobility: is connective tissue the missing link?.Neurogastroenterol Motil. 2010; 22 (252-e78)
- Overall management of the joint hypermobility syndrome.in: Keer R. Grahame R. Hypermobility syndrome Recognition and management for physiotherapists. Butterworth-Heinemann, Philadelphia2003: 27-33
- The sensorimotor system, part I: the physiologic basis of functional joint stability.J Athl Train. 2002; 37: 71-79
- Joint position sense and vibratory perception sense in patients with the Ehlers-Danlos syndrome type III (hypermobility type).Clin Rheumatol. 2010; 29: 289-295
- Reduced quantitative muscle function in tenascin-X deficient Ehlers-Danlos patients.Neuromuscul Disord. 2007; 17: 597-602
- Duffield's exercise in water.3rd ed. Baillière Tindall, London1993
- Effectiveness of manual therapies: the UK evidence report.Chiropr Osteopat. 2010; 18: 3
- Transcutaneous electrical nerve stimulation for knee osteoarthritis.Cochrane Database Syst Rev. 2009; (CD002823)
- Physical therapy in the treatment of fibromyalgia.Scand J Rheumatol. 2000; 113: 78-85
- Chronic musculoskeletal pain in chronic fatigue syndrome: recent developments and therapeutic implications.Man Ther. 2006; 11: 187-191
- Time to take hypermobility seriously.Rheumatology. 2001; 40: 485-491
Article info
Publication history
Published online: June 03, 2011
Footnotes
No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit on the authors or on any organization with which the authors are associated.
Published on-line June 2, 2011 at www.archives-pmr.org.
Identification
Copyright
© 2011 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.