Factors affecting quality of life in children and adolescents with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders,2019,Mu et al

[Andy]

Abstract
Hypermobile Ehlers‐Danlos syndrome (hEDS) is a hereditary disorder of connective tissue, often presenting with complex symptoms can include chronic pain, fatigue, and dysautonomia. Factors influencing functional disability in the pediatric hEDS population are incompletely studied. This study's aims were to assess factors that affect quality of life in children and adolescents with hEDS.

Individuals with hEDS between the ages 12–20 years and matched parents were recruited through retrospective chart review at two genetics clinics. Participants completed a questionnaire that included the Pediatric Quality of Life Inventory (PedsQL™), PedsQL Multidimentional Fatigue Scale, Functional Disability Inventory, Pain‐Frequency‐Severity‐Duration Scale, the Brief Illness Perception Questionnaire, measures of anxiety and depression, and helpful interventions. Survey responses were completed for 47 children and adolescents with hEDS/hypermobility spectrum disorder (81% female, mean age 16 years), some by the affected individual, some by their parent, and some by both. Clinical data derived from chart review were compared statistically to survey responses.

All outcomes correlated moderately to strongly with each other. Using multiple regression, general fatigue and pain scores were the best predictors of the PedsQL total score. Additionally, presence of any psychiatric diagnosis was correlated with a lower PedsQL score. Current management guidelines recommend early intervention to prevent disability from deconditioning; these results may help identify target interventions in this vulnerable population.

Paywalled at https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.61055

 

[Jonathan Edwards]

I strongly suspect that the patients studied are in no way representative of hypermobility in children (forget the EDS). They will be children with fatigue and pain problems who have been referred to a local physio who has it in their head that pain and fatigue go with 'EDS' and so get referred on to a genetics clinic. Children with hypermobility and no pain or fatigue do not go to such clinics.

So this is a complete waste of time. Moreover:

Current management guidelines recommend early intervention to prevent disability from deconditioning; these results may help identify target interventions in this vulnerable population.

Is all too familiar.

 

[rvallee]

Current management guidelines recommend early intervention to prevent disability from deconditioning; these results may help identify target interventions in this vulnerable population

Where are the studies showing normal deconditioning from a sedentary lifestyle leads to this much measurable disability? To properly test this level of deconditioning participants in studies are made to remain bedbound under constant surveillance for months at a time (lots of it comes from NASA because it compares well with what weightlessness does to the human body). Less than a quarter of us are bedbound so this whole obsession is just bizarre.

None of the psychosocial studies ever include any deconditioned controls for comparison. Likely because it's not actually possible to find any population that would actually fit those requirements and even a modest exercise training program would have any such controls soar immediately past any chronically ill population. That exercise doesn't help most is precisely the biggest tell of all that there is something else going on, it's completely abnormal, any healthy person should and does benefit from exercise.

Why does this deconditioning assumption have such a long shelf life when its impact is so grossly exaggerated? In the case of ME it literally requires people to become deconditioned to the point of being bedbound within a matter of days. That's not how any of this works. I hate suspension of disbelief.

 

[Cinders66]

Even the

Where are the studies showing normal deconditioning from a sedentary lifestyle leads to this much measurable disability? To properly test this level of deconditioning participants in studies are made to remain bedbound under constant surveillance for months at a time (lots of it comes from NASA because it compares well with what weightlessness does to the human body). Less than a quarter of us are bedbound so this whole obsession is just bizarre.

None of the psychosocial studies ever include any deconditioned controls for comparison. Likely because it's not actually possible to find any population that would actually fit those requirements and even a modest exercise training program would have any such controls soar immediately past any chronically ill population. That exercise doesn't help most is precisely the biggest tell of all that there is something else going on, it's completely abnormal, any healthy person should and does benefit from exercise.

Why does this deconditioning assumption have such a long shelf life when its impact is so grossly exaggerated? In the case of ME it literally requires people to become deconditioned to the point of being bedbound within a matter of days. That's not how any of this works. I hate suspension of disbelief.

even the astronaut study by nasa, 3 months in bed did not cause ME symptoms or any major symptoms. This directly contradicts Information such as that in the GP document trying to suggest that muscle pain is caused in healthy people if they rest a lot and drawing equivalents to ME pain. Firstly, as you say the symptoms usually preceed the deconditiong, secondLy many with ME are not deconditioned and thirdly as I said even significant stays in bed do not cause the distressing symptoms of severe MEor lasting disability, causing mainly just autonomic issues with being upright. I don’t understand how For years we have been side lined by a bullshit model and seemingly applying to others too

 

[Dr Carrot]

This guy reported high heart rate on standing, weakness, coordination issues: https://www.vice.com/en_uk/article/jma83d/nasa-patient-8179-200

Crucially, it took him 3 days to feel relatively normal, and only two weeks to be back to how he felt before he spend 70 days in bed for the test.

 

[rvallee]

This guy reported high heart rate on standing, weakness, coordination issues: https://www.vice.com/en_uk/article/jma83d/nasa-patient-8179-200

Crucially, it took him 3 days to feel relatively normal, and only two weeks to be back to how he felt before he spend 70 days in bed for the test.

Within a few days of casual strolling and formal reconditioning exercise, my balance returned and my endurance began to recover. By the end of the two-week post–bed rest period, I felt 95 percent physically normal. I was ready to go.

So how does it make sense to pursue this idiotic model where bedbound patients are asked to gradually work their way up from brushing their teeth over several months when the normal expected course of an otherwise healthy deconditioned person is "95% back to normal after a few days". There's something deeply broken here.

Why is this complete nonsense going unchallenged for decades? Are we truly deserving of so much contempt that people need to twist reality just to stick it to us? It's basically a giant exercise in mixing cause and effect with absolute confidence and the superficial appearance of science. "You're bedbound because you're deconditioned and you're also clearly deconditioned because you're bedbound" isn't any more credible just because someone really wants it to be true and has built their career on it.

 

[Dr Carrot]

So how does it make sense to pursue this idiotic model where bedbound patients are asked to gradually work their way up from brushing their teeth over several months when the normal expected course of an otherwise healthy deconditioned person is "95% back to normal after a few days". There's something deeply broken here.

Because it was cheap and no one in medicine bothered to challenge it.