This is Ehlers-Danlos

#EDShsd
#NotJustBendy 
#IgnoranceIsNoExcuse
Complexly Simple
Unfortunately people with EDS/HSD are commonly judged and this is the main reason for such a delayed diagnosis. Because of the multi-systemic symptoms and the usual lack of medical knowledge, professionalsnot only misdiagnose but more damagingly, they often ignore or dismiss what the patient is saying, labelling them as a hypochondriac, drug- or attention-seeker, liar or as suffering from a purely psychological condition with no physical cause. This leads to a halt in medical care.

Even when professionals know a little about EDS/HSD, the attitudes of the medical profession towards EDS/HSD patients is abysmally disgusting and is one of the two driving forces of the #youseeus campaign. We will explore this subject in depth through our features in HEDS Up!.
The fact a plethora of scientific 
 EDS/HSD research exists 
reinforces that bad attitudes 
and behaviour goes beyond a lack of knowledge and into the realm of ignoranceFor this, there is  no excuse.
In an ideal world, doctors in the UK, for example would stick rigidly to the General Medical Council Guidelines and research the condition when EDS/HSD presents in their office, or at least refer them to a specialist. Too often, this is not the case, and EDS/HSD patients are discharged from NHS services when they should be receiving further care and management. 

It seems that rather than research or admit a lack in knowledge, some professionals prefer to exercise neglect and eject the patient from the service they are meant to be providing.

One of the most reported complaints of the EDS community is the way in which patients with confirmed diagnoses and years of EDS/HSD research are treated by medical professionals. In the UK, we have the 'Expert Patient Programme', a highly prized health initiative designed to and hailed as giving patients power over their care. What power do they have when too may doctors ignore credible knowledge?

The fact a plethora of scientific EDS/HSD research exists on the database that medical professionals all over the world use during their studies and career, serves to reinforce that this attitude and such behaviour goes beyond a lack of knowledge and into the realm of ignorance.  For this, there is no excuse.

Not Just Bendy
Life with EDS/HSD can be heartbreakingly hard. Pain often arrives around the onset of puberty, though it's been recorded that symptoms start at a mean average of 6.2 years-old. The social and psychological considerations that can accompany EDS/HSDaremany and complicated. While some Bendies can work full-time, go mountain biking, run marathons and live relatively normal lives, others are not so lucky. 

As the involvement of gastrointestinal issues (GI) in Ehlers-Danlos/HSD become increasingly recognised, documented and understood, and as the internet facilitates the exchange of information in the medical world and among online communities, we are seeing rising numbers of EDS/HSD patients needing surgical intervention in order to get fluids and nutrition into their bodies. This sometimes takes the forms of various tubes that go through the skin directly into the digestive system or, in even more extreme cases, it can mean a line into the heart and the patient being totally reliant on liquid administered that way to provide what they need to stay alive. 

The reason for this isn't simple and could be a number of things, or a combination of many. In EDSHSD, the oesophagus, stomach, intestines, bowel...From lips to lavatory...Could be structurally impaired - remember the brick analogy earlier? So the tissues making up the system stretch and sag when they shouldn't, perhaps, and are unable to move things along in the way they should. However, it could be something else...

Dysfunction of the Autonomic Nervous System (Dysautonomia) is massively associated with EDS'HSD and often comes along with it. The Autonomic Nervous System (ANS - another thing we'll cover in HEDS Up!) controls our GI systems. If it's not working as it should, then it can kill the appetite and kill your body’s ability to take in, retain and digest fluids and food. Obviously some individuals with GI issues will have both factors playing against them and causing problems. Just as GI issues are being seen as increasingly significant when it comes to EDS/HSD, as is Dysautonomia and the ANS. 
One of the hardest things about EDS is what goes on inside,while outside  individuals look totally healthy...
It's not unusual for those with EDS/HSD
 to be accused of exaggerating 
or lying about symptoms.
As you can see, on top of joints that dislocate or sublux (a full or partial dislocation without damage to the muscles, ligaments or tendons) in a crazily easy and frequent fashion, wounds that don't heal properly and reopen once stitches are removed, prolonged bleeding, pain all over the body, extreme exhaustion, fragile blood vessels, fragile internal organs...People with EDS/HSD have a lot to deal with, and it is not just about being bendy. 

Some people are unable to drink or eat, and then what goes in must come out, and that's a whole other world of unpleasantness...
When EDS/HSD is severe, it leaves its bodies unable to do the simplest things. It's well recognised in the EDS/HSD world that the pain it causes is relentless, severe, life-limiting and can be almost impossible to control. Often only combinations of the strongest opiate and narcotic medications offer any relief.  Unfortunately, this leads to many Bendies being accused of drug-seeking behaviour, and pain relief is refused or even totally withdrawn, leaving patients to an existence of uncontrolled, intractable agony. Why medication is less effective in some EDS/HSD bodies is still not understood [but could it be related to certain enzyme levels in Bendy bodies?)

It's also reported that nervous systems in EDS/HSD can become hypersensitive to externally inflicted pain - a Bendy may be functioning with a level of pain most would be hospitalised at, but if they stub their toe, their pain will feel amplified. Other sensitisation shows up in other ways too. 

Experts understand that some with EDS/HSD experience a hypersensitivity to sound, light and physical sensation. What may be a comfortable volume or ambient noise one minute could be intensely uncomfortable the next. Those socks that were so cosy last week are back in the draw because every knitted stitch is rasping on the soles of the feet today. While the physical sensitisation may be down to neurological damage or alteration in some cases, in general, it's not known what causes them. The perception of internal sensation can also be different in an EDS/HSD body, and some people will feel certain bodily functions that we don't normally feel. This is similar to some symptoms associated with Autism and interestingly, specialists say that the number of individuals with EDS/HSD and Autism is so statistically relevant that a link exists between the two. What this is and what it means for the future is still a mystery.  

Arguably one of the hardest things about EDS/HSD is that all this can go on inside, while on the outside, individuals look totally healthy and well. Aids like crutches, wheelchairs and splints all add a visibility, but it's not unusual for those with EDS/HSD to be accused of exaggerating or lying about symptoms by strangers in the street, loved-ones and medical professionals.
Challenge And Change
Ehlers-Danlos/HSD may have no cure, though that’s not to say there’s no hope. Research is ongoing, even if bigger budgets would see bigger benefits for patients. The most significant and helpful thing we can now do for people with EDS/HSD is to challenge current medical attitudes and create awareness of the condition. Doing these two things will bring about the change sufferers so desperately need. 

Bendies and their loved-ones can empower themselves by reading about the illness. In doing so, friends and family are more equipped to empathise and appreciate that stripey struggles are very real, down to the most basic physical actions. It enables better, more effective practical and emotional support to be given.  

Even just knowing the basics and communicating them to medical teams can alter experiences of official care and treatment massively. If patients appear without any EDS/HSD knowledge, generally the professionals are not going to fill in the gaps and research it. When a patient presents with knowledge of their condition, they have a chance of spotting when mistakes are made or important factors are ignored - after all, if you don’t know what’s right, how can you know what’s wrong? This also imbues the situation with a level of expectation that the professionals will then research it too.

By showing the medical field that patients understand Ehlers-Danlos/HSD, we’re telling them they should understand it too. And we won’t settle for anything less. 

The wonderful thing about raising awareness and really helping the EDS/HSD community is that you don’t have to have the syndrome to do it. Simply sharing features like this online and using our hashtags brings EDS/HSD into the public psyche, and while there is a long way to go in EDS/HSD medicine, these small things are the important first steps.

words : #ysuEditor

  Rundown
say :
'err-lers dan-loss'
cause :
Production of faulty collagen and/or Tenascin X.
Inherited - so you're born with it, or you're not (congenital).
Can happen when a gene mutates - so no need for a family history of it. If a mutation causes it, future generation can then inherit it.  
Some generations may show no/few symptoms.


CONTRARY TO POPULAR MEDICAL BELIEF, A PATIENT DOES NOT HAVE TO HAVE STRETCHY SKIN TO HAVE EHLERS-DANLOS.

CURRENT KNOWLEDGE STATES TENASCIN X INVOLVEMENT MAY LEAD TO AN INCREASED RISK OF INTERNAL ORGAN/VESSEL RUPTURE (EVEN IN HYPERMOBILE TYPES).
description : 
A common degenerative connective tissue disorder, which effects every system of the body, and thus requires a multi-disciplinary medical approach to management. 
symptoms :
Note: This list does not include all symptoms.
- Widespread, intractable musculoskeletal and neurological pain.
- Hypermobilty. Means abnormally bendy joints (people say 'double-jointed')
- Bruises easily. Wounds heal poorly. May reopen after stitches removed
Local anesthetics less effective or don't work at all 
- Abnormally stretchy skin. 'Velvety' soft. Scars easily
- Overcrowding of teeth and high arched palate (roof of mouth)
- Frequent, easy dislocations and subluxations
- Hypovolaemia (low blood volume)
- Prolonged bleeding
- Dysfunction of the Autonomic Nervous System
- Gastrointestinal Issues (nausea, vomiting, constipation, pain, bloating, no appetite, inability to eat or drink)
- Sleep Disturbances
- Tachycardia and Arrhythmias
- Dizziness
- Seizures [not Epileptic, possibly cardiogenic/neurological?]
- Fainting
- Fidgeting (yes, fidgeting)
- Vitamin D Deficiency
- 'Allergies' - actually Mast Cell Activation Disorder/Syndrome

Not all people will have all the symptoms. 
Some may have EDS and have no/few problems. 

Can cause death through internal organ rupture, aortic dissection or rupture and through complications such as infection, poor healing, malnutrition, dehydration, seizures, etc. 
diagnosis :
Clinical examination using the Beighton and Brighton Scales. Genetic testing for types other than the Hypermobile Type (HT-EDS). Including urine test for the very rarely recorded Kyphoscoliotic type.
treatment :
THERE IS NO CURE.
Ongoing detailed cardiac scanning (aortic risks).
DEXA bone scans.
Ongoing blood vitamin/mineral/full counts.
Ongoing specialist physio/hydrotherapy, orthotics.
Pain Management input, medications.
Specialist liquid diets (delivered via various tubes in to the body), increased water and sodium intake (under medical guidance).
other :
In 1998 the Villefranche Nosology identified ten types of EDS. In 2016, a New York Symposium reclassified them, and now seven subtypes exist. 
Vascular (VT-EDS)                      
Classical (CT-EDS)
Hypermobile (HT-EDS)             
Athrochalasia (AT-EDS)
Kyphoscoliotic (KT-EDS)            
Tenascin X Deficient (TNXB EDS)
Dermatosparaxis (DT-EDS)

Some types are rarer than others. Each have specific traits but all carry common characteristics too. The gene for the Hypermobile Type has not been found.
Patients sometimes show symptoms associated with another subtype, though the official line currently states an individual can only have one type of EDS. Genetic theory shows mutation of more than one 'collagen gene' is possible, and a UK family with 
HT-EDS were identified as carrying the COL3A1 gene which causes VT-EDS. 

NEW CONTROVERSIAL DIAGNOSTIC CRITERIA ANNOUNCED 15TH MARCH 2017, IN WHICH NUMEROUS NEW HYPERMOBILITY SPECTRUM DISORDERS ARE INTRODUCED AND OTHER CHANGES MADE.
Find support and further information on causes and symptoms at:
The Ehlers-Danlos Society (USA, formerly the Ehlers-Danlos National Foundation)

PubMed (US National Institute Of Health) is the database medical professionals use for research 
on EDS/HSD. Visit it to do the same. We've linked you to an EDS search.
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