Just a "zebra" trying my best to live life to the fullest with EDS and POTS...and loving the ride.

Ehlers-Danlos in Cincinnati

Written by Katie. Posted in Ehlers-Danlos Appointments

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Well, I have been putting this post off because I knew it was going to be a long one.  One with a lot of information; some of which I honestly did not want to hear.  Since my appointment last Thursday, I have been mentally processing a lot of the information I learned at my appointment with Dr. Tinkle. This “processing” has led to a few mental breakdowns emotional moments, and I felt it was best to blog about it when I was feeling much more positive and ready to break it all down in writing. 
So this is the morning.   

I started out the day at the physical therapist’s office.  Dr. Tinkle likes to send his Ehlers-Danlos patients to a physical therapist first to learn how to best tone muscles and protect the joints to achieve joint stabilization without stressing or damaging the joints more.  In EDS, the joint ligaments are too lax, which creates instability in the joints, which leads to pain.   I spent two hours with a therapist, who was kind enough to allow Brad to video tape me doing each exercise.  A lot of the exercises she said I needed to focus on were for my back and neck instability.  Paired with knowing I also had POTS, we did a mixture of exercises that required standing and sitting.  A lot of the exercises involved a medicine ball which I will be investing in for home use.  Besides learning certain exercises that will hopefully help me with joint stabilization and protection, the key to doing physical therapy, or any activity for that matter with Ehlers Danlos was definitely ingrained into my head: EVERY EXERCISE OR ACTIVITY IS TO BE DONE MID-RANGE.  Exercising to full flexion and extension is a no-no.  Carrying grocery bags with my arms hyper-extended is a no-no.  Standing with my knees locked is a no-no.  This is something I will have to train myself to do.  As a gymnast for 14 years, everything was always carried out to full extension, so this will be something I will need to constantly pay attention to until my body is retrained.  I left the physical therapy session with many exercises that I will bring back to my physical therapist at MSU to tackle. 

A few hours later it was time to see Dr. Tinkle.  So before I go into all the nitty gritty details, I do not think I ever explained what Ehlers Danlos is. 

Ehlers-Danlos is a genetic condition, so I have had it since birth.  I either inherited it from my Mom or my Dad, which we are not sure who, because whomever has it has been asymptomatic.  I am willing to bet my Dad has it though because ever since I can remember my Dad has been able to put his legs behind his head.  I will probably never know which side it came from, and really, at this point, does it even matter? 

Hopefully I will explain this accurately, but please keep in mind I am not a doctor 🙂 Ehlers Danlos is a connective tissue condition that causes a defect in the synthesis of collagen.  Our whole body is made up of connective tissue; it is like the glue that holds our bodies together.  Therefore, muscles, ligaments, blood vessels, the skin, the stomach, the visceral organs, and pretty much any body system can be effected.  Basically since there is a defect in collagen, my EDS body is too stretchy, which is why I have had major issues in the last year, feeling like my body has been coming “unglued” or like what used to feel tight and stable, now feels extremely unstable.  For me, my back, GI tract, hips, and neck have been effected the most, but each EDS person is different as to how they are effected.  Also, my autonomic nervous system has been effected, causing the POTS (Postural Orthostatic Tachycardia Syndrome).

There are six different types, but I really only know about the first 4 types.  Type 1 includes extreme skin involvement (slow wound healing, easy bruising, abnormal scar formation, stretchy and smooth skin) and joint hypermobility.  Type 2 includes mild skin involvement and joint hypermobility.  Type 3 is extreme joint hypermobility.  Type 4 is the vascular type which is the most serious type.  Many people do not live past 50 with type 4.  I have been diagnosed as either a type 2 or type 3.  I definitely have the extreme joint hypermobility but both geneticists I have seen have both agreed that I have some skin involvement (I have a couple scars that have opened up a little, I have smooth and mild stretchy skin, and I bruise easily). 

So how was my Ehlers Danlos diagnosed?  For me, and again, I can only speak about my experience, I was asked to pull skin away from my neck and elbow.  My scars were examined.  And I was given the Beighton Scale which tests hypermobility.  There are 9 points on the Beighton Scale and I scored 9/9.  Overachiever, huh?  One thing I could not do was touch my tongue to my nose so of course I am working on that 🙂

Below you will find the Beighton scale.  So paired with the scale, my skin, and a few other markers such as my palate, my symptoms, my POTS which is found VERY commonly in EDS people, my flat feet, and certain bumps that I have on my feet that are markers of EDS, a diagnosis of type 2/3 EDS was confirmed by the Fort Wayne geneticist and by Dr. Tinkle, the geneticist in Cincinnati. 

Let me just say that my appointment with Dr. Tinkle did not disappoint.  He is obviously very passionate and knowledgeable about what he does, and he is really committed to helping his patients.  My appointment was at 3:30 and Brad and I left the office at 6:30.  I learned so much and it was such a breath of fresh air to have a doctor really, really GET what I was talking about.  When I left, he told me he wants to know how I am doing and to send him email updates about what is happening and any questions I have.  He said patients usually come back yearly but he can help from afar with issues that arise.  I would tell anyone who has EDS to make the trip to see Dr. Tinkle because he obviously fully understands the complexity of the disorder (including the POTS relationship with EDS).
Here are most of the issues we talked about. It was much easier for me to spilt the info into categories.

 Why do I have so many GI issues?

The POTS (dysautonomia) and the EDS both effect the GI tract.  The POTS causes an abnormal amount of blood flow to my stomach for digestion, while the EDS also causes my stomach to be too stretchy as well.  Dr. Tinkle said a lot of my symptoms present as a hiatal hernia, which is common in EDS patients.  In a hiatal hernia, the connective tissue around the diaphragm becomes too stretchy and therefore the diaphragm becomes weakened.  So then the stomach is able to pass upwards into the chest cavity which creates all sorts of GI issues.  So even my stomach is hypermobile.  We discussed that taking something for acid should help some of my symptoms as well as eating smaller meals more frequently should help.  The smaller meals will help my stomach empty faster and the stomach will not need as much blood flow to digest the food if not a lot of food is in the stomach to begin with. 

What should I do about all of the instability in my joints?

Physical therapy is key to toning many of the muscles, so that I can return to being active.  EDS people must find ways to be active without hyper extending or damaging joints further to keep muscle tone.  In EDS there is nothing wrong with the muscles themselves so it is extra important to have the muscles as toned as possible since they have to work extra hard to hold the body together. Suggestions include water activities, walking, Pilate’s, and cycling. No more running 🙁

Because the muscles work overtime, they are often spasming and feel hard as rocks (which is a major problem I have).  Massage therapy was ok’d as well as chiropractic care for any area of my body besides my neck. 

So that brings me to my neck…

Why the cervical collar and what about the neck instability?

The fact that the cervical collar reduces my symptoms, helps keep my head up, and reduces my headaches, along with the fact that I have a resolution of symptoms with neck traction seems to show that I am having cranial settling and cranio-cervical instability because of the weakened connective tissue (I think).  The top two vertebrae (C1 and C2) are moving too much and the ligaments are too lax.  Dr. Tinkle explained that I can try all the conservative treatments: pain management doctors with injections, physical therapy exercises, and continued use of the collar, but it is his experience that all of his patients with the same issue have been able to resolve the instability and symptoms with a C1/C2 fusion surgery only.  He wants to refer me to a neurosurgeon in Cincinnati who also understands EDS for a consultation to see what he thinks about a C1/C2 fusion.  So I pretty much got the idea that to ditch the collar for good, surgery is in my future.

This was item #1 I was not so thrilled about.  I told Dr. Tinkle this and that it scared me to death and he definitely understood.  But I came for information from the expert, and information is what I got.

What did he think about prolotherapy?

First of all prolotherapy “uses a dextrose (sugar water) solution, which is injected into the ligament or tendon where it attaches to the bone. This causes a localized inflammation in these weak areas which then increases the blood supply and flow of nutrients and stimulates the tissue to repair itself.” The hope is that it tightens the weakened areas up, providing more stability (Thank you Prolotherapy.com).

Dr. Tinkle pretty much said that it is still unknown if prolotherapy will help Ehlers-Danlos patients.  He said it seems to him that it would be more beneficial for the joints that are not meant to move as much like the ribs or the tailbone.  He explained that it is expensive, since insurance does not usually cover it but if I wanted to try it, to go for it…EXCEPT in my neck.  It was clear that he did not think it was a good idea to stick the needles in my neck. 
(Something else I was bummed to hear because I was hoping that he would have said that prolotherapy would be something less invasive than surgery, that could help). 

Why POTS and EDS?

Basically, it is my understanding that the abnormal connective tissue causes the blood vessels and veins to become stretchier so it becomes harder for the vessels to maintain the right amount of pressure to constrict in order to push the blood back up to the heart and brain normally when standing.  Dr. Tinkle reiterated that it is important to get into Dr. Grubb in Toledo since he not only is an expert in POTS but how POTS and EDS play into each other.  So I will continue to make it my mission to get into Dr. Grubb and/or his nurse practitioner Beverly. 

What about diet and supplements?

The only thing Dr. Tinkle stressed was to take a multi mineral. 

What about hormones and EDS?

Dr. Tinkle said there is a definite relationship between hormones and EDS, and it makes sense that I hurt more and sublex more when I have my period.  He explained that both estrogen and progesterone have an effect on collagen, and he suggested I go on a certain type of birth control that helps regulate my hormones, which would hopefully make me less “stretchier” at my time of the month.  It was interesting that he explained that men with EDS hardly ever have symptoms, and they know it has to do with hormones.  Testosterone seems to help men with stability (which if my Dad has it, may be one of the reasons why he has not had problems).

What about Pregnancy and EDS?

Dr. Tinkle started out by saying that there are a lot of complications with pregnancy.  Since I have POTS, with pregnancy the POTS gets worse, which can not only be dangerous to me but also to the baby.  Then with EDS, because you get stretchier anyways with pregnancy, this is not good if you already have a problem with being too stretchy.  He said people with EDS do choose to get pregnant and have babies, but there are some things I must really understand first. 

If that is what Brad and I were to choose, he would harness the heck out of me; hips, pelvis, belly, etc.  The extra weight would most likely cause more damage to the joints.  I would have to count on being on bed rest for AT LEAST the last trimester.  So because of the bed rest, all of the muscle tone I would have worked my butt off to regain through therapy and being active would disappear, resulting in my body being a mess again after the baby.  He then explained that after the pregnancy, not only would my body be a mess but I now would have an infant to care for.  He also explained that because I have EDS, our child would have a 50% chance of inheriting it. 

Sigh.  That’s a lot to consider and I’m just gonna leave it at that for now….and say again, Santa, I would like a surrogate for Christmas.

I asked about work, and if he has teachers who work with POTS and EDS?

Dr. Tinkle said he has one teacher; a music teacher, that chooses to work.  But that is all she does.  All her nights and weekends are spent recovering.  He stressed that part time jobs usually work best if I can swing it, and working usually requires some doctor ordered job restrictions. Double sigh. 

But I have to keep in mind that he is talking about one person, and everyone is different so that is not to say I won’t get to the point of being able to work again, right? He did say I have a LOT of work to do, and management is not a quick fix.  But I definitely have hope.

I asked what else helps…

Other ways to manage EDS and POTS Dr. Tinkle explained include antidepressants because of the effect they can have on the Autonomic Nervous System and pain (He likes Cymbalta).  Other things he mentioned include, a good pain management doctor,  acupuncture, and to have my other doctors communicate with him.  I love that Dr. Tinkle said he would talk to any doctor, therapist, etc about any questions they have, and that he would rather talk to them directly than for me to try and explain something that may be confusing.

I asked why at 28, did everything fall apart if I have had EDS all my life.

Basically Dr. Tinkle explained that the fitness I enjoyed all my life through gymnastics and running kept my muscles in good shape but damaged the joints so once I stopped running, the muscles lost their strength and tone and everything fell apart.
Other interesting points… 
I find it funny that many of the doctors I have seen in the past year have commented on my Type A’ness.  Maybe it is the tabbed binder I bring to each appointment, or the audio recorder, or my long list of questions that clue them in on this personality trait of mine… Well, Dr. Tinkle also brought my type A personality up, and said that Type A people often struggle the most with POTS and EDS, because they push and push their bodies to keep going despite pain and sickness, and then pay for it more later.  He wanted me to be aware of this, and that it is so important to listen to my body and find ways to slow down.
Something else I found interesting…I was asked the country of origin of my Mother (Ireland) and the country of origin of my father (Italy).  Then I was asked, “So it is safe to say that they are not related?”  Hmmm, Brad and I looked at each other on that one.
Dr. Tinkle said it does make sense that my POTS is worse when my neck instability is worse but at this point they are not sure the relationship between the neck and the autonomic nervous system.
Dr. Tinkle made the comment that I will hopefully grow out of the POTS by my forties.
So I think that pretty much covers it.  I am so glad I went and I came away with so much information.  But like I said, there was quite a bit of information I did not want to hear, which was really overwhelming as I left the office.  In the elevator, on the way out, I couldn’t help the tears as I felt so overwhelmed about the many lifestyle changes and realities that are ahead of me. 
And then we reached the ground floor of Cincinnati Children’s Hospital. As we left, I saw children in wheelchairs, with breathing devices, bald from chemo…and I saw their exhausted parents. 

And I gained the perspective I needed for that moment. 

It IS overwhelming and it DOES suck at times but it really is not THAT bad, and is not something I can’t handle. 

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