Hypermobility Ehlers-Danlos Syndrome

Tips for surgery (aimed at surgeons and
medical people):

IVs may not flow normally with EDS patients. Have an IV pump handy in
case you need it. Gravity fed IVs don’t work for me.

Check with the person and see if they have problems with medical
adhesives. We don’t enjoy our skin coming off with the medical tape.

Taking blood and siting IVs can be problematic. Remember arms are not
the only option. There are other limbs. Is a central line an appropriate
route for this patient? It an arm has undergone a great deal of surgery,
the other arm is likely to be more difficult to get a good stick. My
left arm is almost impossible to use for IVs and blood tests, but we
hope it will keep going until after my next surgery on my right arm. If
not, we will have to get creative. There is always another option.

Try to keep any surgery as blood free as possible. People with EDS often
have bleeding problems. Schedule some extra time for the surgery so the
surgery is not rushed. That way, adequate time can be taken to go around
or to take an alternative route and time is available if any
complications arise. The laxity of the tissues make complications more
likely than for the ‘normal’ patient. As long as you plan ahead and know
what you need to do in all circumstances, this will not be a problem.
And you knowing these things will give us the confidence to trust you
and your judgment. Don’t do something stupid and prove yourself to be
unworthy of our trust.

A person having EDS is not a good excuse to not operate. If the surgery
is needed, the EDS should not be a bar to appropriate treatment. If you
find yourself thinking “if this was a normal patient, I would
operate” ask yourself why the EDS makes a difference. If the risks
are well understood by both parties and the possible benefits are worth
the risk, then think again. And there is no such thing as being too
young for a surgery, either.

Stitches too easily rip our skin. If Steri-strips can be used, use those
instead and use loads more than you would with a ‘normal’ person. There
is a world wide shortage after my surgeries. While this will not
eliminate the cigarette paper scaring and other EDS oddities, it will
help to minimize the problem. Leaving them on longer than normal can
also help.

If bone is being moved or fixed do NOT try and get away with just a few
wires. Use plenty of metalwork to be sure that something that should no
longer move will not, even if it is “impossible”. A piece of
bone with six K wires shouldn’t be able to move, but it did. Permanent
metal work is the better option, even if you will need to remove it for
further
surgeries. Don’t remove internal metal work unless you absolutely have
to. If it is not causing problems, leave it alone. Removal to allow the
next surgery proceed is a different matter. It is best to do this at the
same time as the next surgery if at all possible.

If external fixation is required, make sure all the pins and wires are
accessible and can be thoroughly cleaned/sterilised multiple times a
day. It is worth the hassle if the alternative is a bone infection. I
have been there and done that and it is not fun. External hardware with
a non-removable dressing is not worth the risk. Accessible external
hardware or all hardware internal.

Don’t try to turn the EDS patient for the perfect x-ray. Be willing to
move the machine to fit what the EDSer can do. These machines move for a
reason. Once damage has set in, our range of motion lessens, even though
it may never approach normal range of motion, unless fusions are present
when different rules apply. Also dislocations and subluxations are
easily provoked and the EDSer will not be amused if you refuse to
believe it until _after_ you have injured them. People that do this are
Class 1 idiots and will be treated accordingly.

If in doubt, don’t. Unless you are sure you know all the possible
consequences good or bad for any treatment. Don’t forget all the rare
problems. Those EDS bodies are tricky. Time preparing and reading up is
never wasted. Talk to the person with EDS. They may have suggestions
that just might work, even if they are a bit off the wall. Lateral
thinking is useful if you have EDS patients. We do it all the time in
normal life. If you can’t see a solution, ask the EDSer. While our ideas
can be wild and wacky, they often point to a usable solution, even if it
is somewhat unorthodox.

Don’t assume you know more about EDS than the patient. This patient was
BORN with this condition and is much more aware of their body than you
will ever be. Listen to this person. Discuss everything thoroughly. This
is not a patient to dictate to unless you fancy being injured in the
near future. This person will know more about EDS that you ever will, so
listen and communicate. Make sure you are BOTH happy with any treatments
and what have you. And just because a thing is “not possible”,
that doesn’t mean it is not possible with an EDSer. Our bodies can do
all sorts of things. If a patient says that they are resetting multiple
dislocations a day, then they are. This is normal for many of us. I
don’t even bother counting them anymore.

Surgery is not the only option. What about splinting? Will that be a
better answer than the scalpel? Sometimes it will. But if the patient
cannot stand the pain and disability any more, do the surgery as long as
both of you are aware of the risks and possible outcomes.

The laxness most EDSers have means that a partial or mini arthrodesis is
more likely to have a bad result than a full arthrodesis. Fusion will
stabilise the joint much more effectively and avoids further unnecessary
surgery and the risks that this entails. Loss of function vs. a stable
joint is something we are able to judge for ourselves. Just give us the
chance and we can make the right decision FOR US. That may not be as the
same thing as the right decision FOR YOU. We have the right to choose.
Please give us the information we need, untainted by your opinions, so
that we can do so.

Expecting anyone with chronic pain to show overt pain symptoms is
ridiculous. We evolve ways to mask how we feel. People don’t want
friends who moan about being in pain all the time. We learn to hide
these things so we are accepted by ‘normal’ people. So, if an EDSer says
they are in pain, then they are. We are not drug seeking idiots, so
don’t treat us as if we are. People who know us well can see through the
mask to the pain. My Mum can tell by my voice alone how I am feeling.
You may not have enough contact with us to learn to read those very
subtle signs. So please listen.

The standard approaches to problems may well not be appropriate for
EDSers. Learn to think “outside the box”. Look at the problem
in a
different way. Unconventional approaches may well be the answer.

Every patient deserves good pain control, whether they have EDS or not.
If possible, allow the EDS patient to self medicate, either with their
own medication or with patient controlled medication. EDSers know what
they need to take and when. And if an EDSer is in pain, don’t tell them
to live with it. We live with it every day of our lives. We do not
deserve to be in pain and uncontrolled pain needs to be addressed. So
address it. And uncontrolled pain may well impair the healing process.
Most EDSers do not like to stay in hospitals and want to be home as soon
as possible. Adequate pain control will facilitate this.

All superb specialists (surgical or no) need an EDS patient. A patient
where normal treatments will have abnormal outcomes and where straight
forward things don’t quite work as planned. It helps to keep your feet
on the ground. No matter how good you are, things will go awry with this
patient and you will need to courage to admit when you are wrong and
will have to try and put things right. And see every encounter with this
patient as a learning opportunity. And it will be. Not only will you
learn that you are not omnipotent and will learn to be humble (in a good
way), you will learn how much strength of faith and character it takes
to live with a body like this. And you will learn how lucky you are that
you do not need to learn this first hand.

Just because our bodies do not work properly, this does not mean that
the brain is affected. We are just normal people with an abnormal
condition. While this makes what we find humorous a bit unusual, we do
have brains and they do work properly. We just see life a bit
differently than most other people do. Treat us with the respect we
deserve.

Local anaesthetics do not work for all EDSers. Talk to the person and
find out if this person is one of those for whom this does not work. And
some of us sometimes will and sometimes will not freeze on application
of a local. So if I say the local isn’t working for me, please pay
attention. It is not pleasant to feel every cut of the scalpel.

When intubating an EDS patient, please be very careful that you do not
bruise or tear the wind pipe. Care, attention and patience will mean
that you will not further damage the patient. While we may not say so,
we will be grateful. And if you do not take care, we may never trust you
ever again.

While most people can (and often are) be handled like slabs of meat when
moving them when they are unconscious. Doing this to an EDS patient will
injure them and may cause permanent damage. While someone with EDS
subconsciously holds the body together, a general anaesthetic removes
that control. You must move us carefully, positioning us such that we
are supported properly. If the body naturally curves one way or the
other, don’t try to realign the patient into a ‘normal’ position. My
spine curves to my right. Trying to straighten it is very painful and
will not be appreciated.

If a limb has to be elevated, please make sure that the next joint
(shoulder or hip) is supported properly. Pillows and other padding can
make the difference between a joint dislocating or not. Being in
hospital is bad enough. Accumulating further injuries while we are there
will not be appreciated.

If a joint dislocates or subluxates, we may wish to self reduce. We know
what does and does not work for us and we also know what methods to use
to not make matters worse. The brute force method often causes further
damage. If we want help, we will ask for it. Please make sure you
understand what we are asking for and do it for us. We may just need you
to hold something in the right position to make resetting the joint
easier. We have much more experience doing this than most doctors get in
a life time, so please listen to us.

One last thing: The only stupid question is the one you do not ask.