Exercise as it relates to Disease/Implications of physical activity for women with Ehlers Danlos syndrome hypermobility type

This is a critique of the research article 'Muscle Mass, Muscle Strength, Functional Performance, and Physical Impairment in Women With the Hypermobility Type of Ehlers-Danlos Syndrome' by.

This critique was written as an assignment in Health, Disease and Exercise at the University of Canberra - September 2019.

Background to the Research

The article defined a distinct gap of knowledge between the understandings of Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT) and the implications of health and physical activity. As the condition is becoming easier to diagnose, greater understanding of the associated symptoms need to be addressed. This article aims to provide that, though suggests further research may be required.

Ehlers-Danlos Syndrome may vary in many ways, as each type of EDS is not yet fully understood. Treatment and lifestyle are very difficult for those with EDS, which is defined by;

• Joint hypermobility
• Skin hyperextensibility
• Tissue fragility

Diagnosis of EDS-HT is very complicated, it is known it is an inherited autosomal dominant pattern and will consist of one, two or all of the features;

• Systemic manifestations of a more generalised connective tissue disorder
• Positive family history, with one or more first degree relatives independatly meeting the current diagnostic criteria for EDS-HT
• Musculoskeletal complications

Research

Research was conducted by Rombaut et al, she is a leading physiotherapist in Belgium that has conducted multiple research programs to develop the understanding of Ehlers Danlos Syndrome. Her postdoctoral work has been done at the Department of Rehabilitation Sciences and Physiotherapy at Ghent University and Centre for Medical Genetics of the Ghent University Hospital.

The article was published by the American College of Rheumatology in 2012, under Care & Research. The focus of this article was muscle strength and pain implicating physical activity levels for women with Ehlers-Danlos Syndrome, sponsorship from the American College of Rheumatology may have influenced bias and therefor findings.

This case-control study compared 43 women with to 43 women without EDS-HT with significant differential findings. The research strongly recommended more research needed to be conducted for treatments. This belief was also supported by other studies.

Methods of Study

Participants of the study were measured by their characteristics such as height, body mass, age, body mass index, Beighton score and physical activity levels using the Baecke questionnaire. Followed by these general assessments were several physical tests to assess the following;

• Muscle Strength. Was assessed by a Biodex Medical System used to calibrate force. Participants were asked to sit upright in a chair with the lever arm of a Biodex Medical System attached at the ankle. Followed by repeated maximal voluntary concentric knee flexion and extension. Repeated 5 times at 60 degrees per second and 5 times at 180 degrees per second.
• Muscle Strength Endurance. Used the same set up to evaluate isokinetic muscle strength endurance performed at 240 degrees per second over 30 second period of maximal concentric contractions at the knee.
• Pain and Fatigue Associated with Muscle Strength and Endurance Tests. Assessed associated pain with the above tests. This was measured using a visual analog scale (VAS) immediately before and after the test occurred. Simultaneously using the Borg Scale fatigue was measured.
• Muscle Mass. Was measured using a Dual X-Ray Absorptiometry (DXA).
• Functional Performance. Was assessed by participants performing 5 chair rises as fast as possible whilst being timed. Women who exceeded 2.5 seconds during the test were categorized as having a higher falls risks.
• Physical Impairment. Using the Arthritis Impact Measurement Scale (AIMS) questionnaire, functional impairment was evaluated. This questionnaire has been adapted and validated and is now used widely amongst the field of rheumatology.

Aspects and reliability of methodology were strong and concise. Given there were so many tests performed in this article, clear explanation was beneficial to understand what was asked of the patients. Accountability of mistakes and mishaps were noted during the method and discussion.

Results

Different statistical analyses were used to analyse the various tests. Pain and muscle fatigue scores were calculated using PASW statistics shown in clustered box plots. Physical characteristics used independent t-tests which provided evidence of considerable variation of physical activity levels and muscle function (P<0.001). Univariate analysis of covariance was used to determine differences between groups for muscle strength, muscle strength endurance, and functional performance.

For Subjects characteristics, findings proved that the subject group had higher Beighton scores (1.5-9.7 <0.001) and considerably lower physical activity levels (6.1-11.5 <0.001). As expected women with EDS-HT had muscle strength weakness, -33.3% to -42.9% lower than the control group. Though it is noted that the ratio between quadriceps and hamstring strength was not significantly different between the two groups. The Muscle strength endurance test proved women with EDS-HT have considerably weaker isokinetic and static endurance and therefor expressed a lower total work. For Pain and Fatigue assessments women with EDS-HT said they felt mild lower extremity pain at rest before the test, after the test their pain increased to moderate (between 40-60 of 100 - using VAS). Some of the subject patients had fatigue even before the test was performed, the subject group were completely exhausted after the endurance test and remained exhausted one-minute post. Many of the subject group were over the 2.5 second marker time for chair raises, increasing the risk of falls for this population.

Conclusion

Conclusively participants of the study with EDS-HT are significantly limited by pain, fatigue and muscular weakness. Greatly impacting their in-ability to participate in physical activity and a functional life. As this is the first extensive study in lower extremities of EDS-HT there was limited comparison of correlated research. Given the 13 different types of Ehlers Danlos Syndrome, relevancy of results in various articles is divided by focuses, which is a limitation.

Various hypothesis questions the reason for muscle weakness in EDS, whether it is due to muscle weakness as a lack of exercise, collagen abnormalities or mitochondrial dysfunction. It is yet to be determined, and the limited knowledge implicates treatment management and lifestyle.

Practical Advice

The article provides strong evidence that women with EDS-HT have chronic moderate-severe pain worsened by physical activity. Understanding of pain is limited, with various suggestions listed above. Several studies on patients with rheumatoid arthritis and fibromyalgia determined light aerobic and resistance training both on land and aquatically are beneficial for improving muscular strength and endurance.

Suggested treatment includes pain prescription medication and sometimes blood pressure medication. Physical therapy is also recommended to strength joint stability and potentially braces, frames or crutches. This can be prescribed by Physiotherapists and/or Exercise Physiologists. In severe cases, surgery can be considered to repair damage. Though due to collagen dysfunction this is not strongly recommended because of the associated healing issues.

With this evidence, recommendation of a repetitious light intensity exercise program prescribed by a Physiotherapist or Exercise Physiologist will be beneficial.