The British Society for Pediatric and Adolescent Rheumatology (BSPAR) issued Guidelines for Management of Joint Hypermobility Syndrome in Children and Young People which caused quite a ruckus, panic and protest among my people. For good reason.

This paper made some highly incorrect statements about Ehlers-Danlos Syndrome.

The last thing we need is wrong information, when we already spend our patient careers trying to educate doctors who know nothing. Misinformation makes our lives worse.

I pasted the full document from the BSPAR at the bottom of this post.

The Ehlers-Danlos Society is preparing a statement.

Here is mine.

Let’s Break It Down

Okay, good start, BSPAR.

You touched on an important issue: some people are hypermobile and fine. Others are hypermobile and very, very sick.

Already going off track, BSPAR.

Suffering with hypermobility likely depends on what gene is making that stretchy collagen in you, not because hypermobility is benign.

The Ehlers-Danlos Society is trying to solve this mystery with the HEDGE Study, to discover what versions of genes are disease causing. Please enroll and donate your DNA sample if you have Hypermobility EDS.

Here I am getting ready for my blood draw at the 2019 Ehlers-Danlos Learning Conference in Nashville.

You’re getting it backwards, BSPAR.

Many doctors don’t even know there are deadly forms of EDS, so patients aren’t given the care they need in an emergency to treat life-threatening complications from Vascular EDS. New diagnostic codes were created to help save lives.

Diagnosing HEDS

Diagnosis of EDS is tricky because few doctors are trained on how to. Until we know more about HEDS, it probably shouldn’t be diagnosed until adulthood.

I know many an HEDS parent who does not want to burden their child with an EDS label so long as the kid is getting along well enough. Isn’t adolescence enough to go through without really bad medical news?

I was not diagnosed until I was 33.

By then, my brain was fully developed (happens around 25). I was married to the kind and generous Mr. Pennington and my life was stable, even though it totally sucked because I had already been completely disabled for years with the misdiagnosis of “chronic fatigue/fibromyalgia.”

When I got the bad news, that this was a genetic disorder, not an illness that was going to go away, I had the maturity to put off my plans of suicide and focus on trying to get better. Why thoughts of suicide? Because I had zero quality of life.

Back then it was really hard to get information about EDS, and it didn’t seem like I would ever get any quality life.

But I was wise enough to realize that since I had lived with EDS torture for this long, I could certainly go on for a while longer just to see what would happen. No point in getting dramatic and carried away.

As a teenager, I was about as sick as they get with EDS.

I was too sick to even notice how different I was from the other kids at school. My parents were troubled people. Neglectful and self-absorbed is as kindly as I can put it. Definitely not up to the task of caring for children. I had to beg and beg to be taken to the doctor. They didn’t involve themselves in my medical care. All decisions were left to me. They didn’t tell doctors how I spent all my time out side of school resting, although they did ridicule me about it at home. They didn’t talk to my physical therapists and try to make sense of why I never got any better. Nobody showed me any compassion or kindness. I carried on, not knowing anything else.

Was this damaging?

The PTSD about that time in my life continues to haunt me. It was seriously traumatizing that no one acknowledged my suffering.

Minimizing EDS does not make it go away.

Collagen-typcials have a much different experience with pain, dysautonmia, fatigue, etc. than we floppy people, because they do not have our genetic disorder.

Personally, I think we need some new words to describe the agony and suffering of HEDS because the way we feel is bad unlike anything.

What if parents get the idea from your paper that their super sick kids are just malingering, refusing to benefit or have psychological problems, not physical ones? That may lead to child abuse. Disabled children are already more likely to suffer maltreatment.

Suicide and EDS.

People with Ehlers-Danlos don’t commit suicide for any other reason than the pain and suffering is too much.

BSPAR, here’s a suggestion on how to address this:

Suicide is a big problem with Ehlers-Danlos, so best to keep it light with the kiddos and don’t upset them too much. They must have hope. Let’s get them some gentle relief with these guidelines and keep it positive, give them encouragement. They are going to have to come to terms with their diagnosis and struggles, but no rush. This will take time. They need to grow up.

BSPAR, you don’t seem to know.

That many kids born with Ehlers-Danlos Syndrome may get further along in life than I did before falling apart. But they do fall apart.

Many of us have adult relatives who are undiagnosed yet suffering tremendously and trying to cope with what they don’t know is EDS. And they don’t want to hear that they have it.

Over-medicalization of HEDS kids.

It seems the BSPAR is trying to prevent kids from getting excessive medical treatment that will make them worse physically and psychologically.

This is a whole other conversation that we are not currently having in our community, so I’m glad you brought it up, BSPAR.

Many medical interventions for Ehlers-Danlos symptoms make us worse.

You are better off without a port for IV fluids, without TPN, without a chiari decompression, passing on a pain pump, passing on a benadryl pump, going without joint surgeries, refusing opiates, skipping the spinal fusion and staying out of a wheelchair. But what is a doctor to do when faced with a patient who is suffering so, except to reach in his tool bag and do his best?

Our condition is so difficult to treat, and we are so peculiarly weak and sensitive, that efforts to give us relief do not return us to health, but create a bigger physical burden.

I sure AF wish I had never been on long-term opiates, but when you are in so much pain you cannot tolerate being alive, what choice do you have. Yes, they made my health worse. But don’t worry about me. I now am off and functioning better than ever. Do take a moment to explore my blog.

Wait, what?

BSPAR, you do not understand HEDS.

When I was the unfortunate teenager very sick with EDS, my problems did not come from becoming weak and deconditioned. EDS took me down as I transitioned into puberty, for reasons not understood, but this phenomenon is well-observed.

It was becoming very, very sick which led me to becoming weak and de-conditioned, which then made my quality of life worse and my ability to function even less.

But thanks for the victim blaming, BSPAR.

Yes, I am a victim of EDS.

I am from a different generation, before cute language was thought to solve real problems.

I totally have EDS. Saying “it doesn’t have me,” or calling it “ an advantage,” I mean, who has the time for such nonsense?

Treatment by redefinition is not treatment. It is dismissive and patronizing. Maybe it works on people who have an attitude problem. It does not work on people who are actually physically sick.

I am a victim of Ehlers-Danlos. It did ruin my life. Best to acknowledge it and move on, like an adult, which I am. Is anyone at the BSPAR?

In my adulthood, I tried and tried to get stronger but it didn’t work. You have no idea how hard I tried. I am naturally such a weak person who doesn’t build muscle mass on my own. I am not unusual for HEDS.

After I began injecting Vitamin C everyday, I was able to get stronger and then benefit from the increased strength and circulation. But this is only because the intramuscular ascorbic acid seems to be compensating for whatever is causing HEDS in me, which was an accidental discovery.

Now that I am a very high functioning EDSer and no longer disabled, I still would not describe myself as having a connective tissue advantage except if we are referring to my beautiful, smooth, very envied, ageless skin. And my narrow rib cage. And my long elegant fingers. And giraffe neck. As if that eases any of my suffering! But the collagen-typicals who are as uninformed as the BSPAR tell me I am lucky. 🙄

Yes, I am the most graceful person in the gym swinging around kettle bells with my fluid, smooth, full range of motion.

But I seem to ache more than a normal person, have more muscle soreness, experience occasional burning neuropathy, react too strongly to all stimuli and break out in rashes if I get overwhelmed. All of that is an uncomfortable burden.

I also have an unstable nervous system which gives me something resembling a panic disorder, entirely generated by my wacky body. I do my best to smile over it and manage it quietly. I see this is many other EDSers. Our nervous system involvement is highly under-appreciated and possibly a big factor in our suffering, but that’s just my thought on the matter.

What BSPAR got right.

The guidelines in this paper would have helped me as I was growing up. There’s actually a lot of good advice here.

I’ll touch on the highlights:

• Splinting made me weaker and worse, plus it was humiliating.

• Using a wheelchair made me weaker and worse, plus it was humiliating.

• The exercise recommendations in here would have helped me. I was on the swim team growing up. In my teenage years, my knees and feet hurt so much I could hardly walk. Swimming was a bad choice: too repetitive, too big a range of motion and not weight bearing. Flip turns in the pool and dolphin kicking were terrible for my flexible spine. Ouch the low back pain!

• Doctors told me to quit swimming, so I did. I got worse from getting weaker. I got more fatigued. And felt more alone.

• OMG I wish I had help with posture, balance and strength training.

• Proprioception work is one of the greatest things I have done, as it has improved the function of my nervous system. Wish I had received that when I was growing.

• OMG I wish I had been salt-loading as a kid. Now I dump salt on everything I eat. It helps my hydration and blood pressure so much. Plus it tastes good.

• I had terrible abdominal pain in adolescence which later required two emergency surgeries: I had an ovarian cyst that was scarring up my abdomen and a hernia which later became incarcerated. The pain I was suffering wasn’t from constipation, although I had that too.

• We have fear of moving, fear of injury, fear of pain. Kinesiophobia does cause us to become weaker and then experience a lower quality of life.

We suffer a much bigger medical complication from chronic pain now described as Central Sensitization. One cannot just power through that.

I wish BSPAR had added a position on joint surgery: avoid it. Adolescents get talked into those all the time.

Joint surgeries may be of no value for an EDSer in any event, but for God’s sake, let the kids stop growing first.

When I was 14, orthopedic surgeons at UCLA suggested I get lateral release on my kneecaps, but luckily I was too afraid to do it. The last thing I need is more kneecap instability. Then I outgrew much of that severe knee pain. Growing is a terrible burden for someone with HEDS.

Childhood is your one shot at building strong bones. Best to take advantage of that. You didn’t mention that, BSPAR.

BSPAR, I agree.

Keep the kiddos active, feeling normal and participating as much as possible.

Loading up a kid on meds and keeping him at home won’t help anyway and can be very psychologically harmful. What does it do for those of us that are adults? I am still haunted by my years of isolation due to disability. No need to get the kids started on that early.

Fellow EDSers

You have to dig deep if you have HEDS. Which you already know.

Maybe we should just let the kids in on that. Tell them that life is going to be physically painful, but you can get better at dealing with that. Growth spurts are particular agony for an EDS body. Hang in there.

Maybe we should tell the kids the truth that many babies are born with rare genetic disorders and it is unfair and awful, but what choice do we have except to deal with it.

Many kids are born into circumstances unfair and disadvantaged in other ways. Such is life.

But medical research is advancing so quickly.

For sure the kids will have a better quality of life than we did.

Maybe we should teach the kids to be kind to their delicate bodies, work within their limitations, to dream big but make realistic plans and be smart about life choices.

Not pretend EDS isn’t torture and make them feel like they are lairs if they admit they are suffering.