Monday 20 May 2019

Living with HSD // EDS/HSD Awareness Month 2019

May is EDS/HSD Awareness Month.

I wanted to do a post on HSD as there's so much out there about EDS (Ehlers-Danlos Syndrome) but little about HSD.

The zebra is the symbol for people with HSD and EDS as we are 'medical zebras' because doctors don't automatically look for conditions like EDS or HSD.

I was originally diagnosed with Joint Hypermobility Syndrome aka EDS Hypermobility Type by my neurologist who referred me to an hypermobility specialist rheumatologist.  As well as the hypermobility type of EDS my rheumatologist suspected I may have vascular EDS so as per the guidelines I was sent of for genetic testing to rule out other types of EDS before giving a diagnosis of hypermobile EDS; thankfully that come back clear. When I went back to my rheumatologist the new 2017 classification had just come in. I met many of the criteria for hEDS but I missed out on two points so I was given the diagnosis of HSD.

In the new 2017 classification they made the criteria for hEDS much stricter than before in an attempt to find the genetic marker for hEDS as all of the other 12 subtypes of EDS already have one (hence why I was sent for genetic testing for vEDS).

Hypermobility Spectrum Disorder is largely an invisible illness. You might see me in my wheelchair, walking with crutches or the braces and splints that I wear but you don't see all the other symptoms and complications that I'm having to deal with.

HSD is caused by faulty collagen. Collagen is the glue of the body and because mine is faulty is causes my joints to be lax and as a result the sublux or dislocation and my organ are too stretchy so I have problems with different organs such as my digestive system, heart and bladder.

It's an incredibly painful condition and because my joints hurt all the time and every movements exacerbates the pain because my joints are so lax. Even putting on a t-shirt or reaching for a plate can cause a a joint to sublux or dislocate! It's also tiring because my body is constantly trying to repair itself.

I sublux/dislocate joints multiple times a week, sometimes in a day and it something that I find never gets any easier to deal with.

On top of that are the complications that I have secondary to my HSD.

I have gastro issues so I have 'delayed gastric emptying' which means that my stomach empties much slower causing me to feel constantly full like I've eaten a 5 course meal even though I only eat small portions; it also causes me to feel nauseous all the time, sometimes leading to episodes of vomiting. Them I have intestinal dysmotility so like with my stomach my digestive system is sluggish and doesn't work as well as it should causing discomfort, cramping and bloating (which doesn't help when you already have IBS).

I also have baller issues which I find quite difficult and it's not helped by my neuro disorder. Because of my faulty collagen my organs are stretcher than normal so my bladder is stretchy which causes it to leak so I have incontinence. This is quite emotionally difficult to deal with and one I hope my specialist can find an answer to.

As well as that I have dysautonomia which is also referred to as autonomic dysfunction so the body's automatic functions like temperature control, blood pressure, heart rate ect doesn't work as well leading to me developing orthostatic intolerance and POTS (Postural Orthostatic Tachycardia Syndrome).

I also have TMJD with is a problem with my jaw joint, again because it's so lax just eating or taking can cause it to slip out of place. Even when I'm just sat watching TV my jaw hurt.

I want to raise more awareness of HSD as there's lots about hEDS and EDS but little about HSD and often HSD isn't taken as seriously or is overlooked compared to hEDS. 
I wrote a post about this which you can find here.

HSD and my other illness all interlink so sometimes it can be hard to work out what's causing what symptom or my illnesses will aqeccct each other for example I'll have a seizure (unrelated to my HSD) but it will cause my joints to sublux or dislocate exacerbating my pain levels.

I live in constant pain; some days I can cope with it and the times the pain will be so immense I just want to curl up and cry.

Other symptoms of HSD is that I injure more easily so I'm forever covered in mysterious bruises. I also have poor vein access - I have on 'good' vein and if that's gone then it's become a challenge; I've had annuals inserted into all sorts of odd areas and often after on blood draw or medication amiss the vein will blow and then it's a hunt for another IV access point.

Another really random symptom of HSD is that local anaesthetic has no effect on me which completes things like when I go to the dentist.

Medication helps me and keeps me functioning and I've found physiotherapy helpful. I'm waiting to start a specialist pain management programme and once my pain is better managed I can go on a hypermobilty exercise programme at UCLH and I'm looking forward to giving hydrotherapy a go. I also find very gentle yoga help me stretch but I have to be super careful not to over do it and cause my joints to sublux/dislocate. As well as medication and physio and yoga I find heat, deep breathing and my TENS machine helps. Occupational therapy has also helped me too.

Once my seizures and other illnesses are better controlled I hope to do an inpatient rehab program for patients with chronic pain and hypermobilty disorders, but I try not to look too far ahead. 

I try to just just take each day as it comes and deal with the challenges that present that day.

I feel very lucky to be under the expert care of specialists at UCLH who within their own field have an interest in EDS/HSD. The travelling is a pain but it worth it.

A few years back I saw my local rheumatologist who told me that EDS is a condition you grow out of! I firmly believe that in cases of suspected EDS/HSDpatients should be referred to EDS/HSD specialist rheumatologist to ensure patients are given the right diagnosis. It's great that there's now more awareness of EDS, especially hEDS but I wonder if in some cases people are seeing professionals like their GP or local rheumatologist who lack to expert knowledge of EDS and HSD and are misdiagnosing EDS when people may have HSD instead or may just simply be hypermobile. (You can be hypermobile and not have a hypermobilty disorder - just look at dancer, gymnasts and athletes.) Especially with the new 2017 EDS classification some GP and rheumatologists may not be aware of this and I know in some cases people are still now being diagnosed with the old classification of EDS Type 3.

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