IceLizard

You're welcome . To get the most out of your appointment I would send your most relevant test results and medical records regarding POTS, EDS, and MCAS to Dr. Grubb so that he can know where to begin with you. You may also want to include a letter saying what medications or other things you have tried and that you want to make progress but your current doctors are not sure what to do next. At the time that I had met him I was newly diagnosed and did not have the years of trying things out that you have.

I do know that he will to the best of his ability try to find a way to help you improve. He cares very much about his patients and their well-being.

Hi SpinnyC,

I asked where you are because I have seen both Dr. Grubb who is about 1000 miles away from me and Dr. Suleman who is only 284 miles away from me.

I saw Dr. Grubb nearly 10 years ago. He is a leading researcher in the area of dysautonomia and is very familiar with the possible causes including EDS. He was the doctor who first diagnosed me with JHS/EDS after I had already been diagnosed with POTS. I had already had tilt table and catecholamine testing and he did not order any more tests. He spent a lot of time with me explaining the connection between EDS and dysautonomia. He is very compassionate, will work with the local doctor you have, and his nurse will answer any questions you have via email. He told me to do 20 minutes of aerobic exercise 3X a week, try Cymbalta, and see a counselor (since being disabled by POTS at 19 is distressing). Unfortunately I was in an unstable position at the time and did not have a good primary care doctor to work with. Because of this I was unable to have him continue my care through a local doctor.

I saw Dr. Suleman for the first time just last week. He did a complete workup on me including full autonomic testing (17 tests over 4 days in all). He told me that my previous tilt table tests were incomplete. He is very, very precise and thorough. Through his extensive testing we have a much better picture of what is happening with me. He also wants to look for a cause of my POTS to be able to address it directly. However, he does not really want to work through other doctors with continuing treatment. If I am to try a new medication he wants me back in Dallas. He sees more patients with EDS and POTS than anyone else in the south and is very aware of the connection between EDS, POTS, and MCAS. He also did an exercise stress test on me and I scored very poorly. Based on my results his exercise physiologist wrote a very specific exercise regimen for me outlining what I am to do each day. He also wants me to see a nutritionist, be tested for gastroparesis, and work on voluntarily lowering my heart rate and breathing rate by biofeedback training. He says we can also try to add medications later if I need to.

A lot has changed in the the realm of EDS, POTS, and MCAS in 10 years, so I can't say what Dr. Grubb is doing now compared to back then. If I had continued to work with him, he may have ordered more testing to get a better picture of what was going on. Both doctors are very knowledgeable about the connections between EDS, POTS, and MCAS and will compassionately listen to your concerns. But, I felt I got more specific information about what was going on with me and what to do about it from my visit with Dr. Suleman.

However, given that you are also far away from Dr. Suleman, I would see Dr. Grubb because he will work with your local doctors to continue your care. (At least he did this 10 years ago, you may want to call to make sure.)

Where are you located?

My cardiologist had me do a stress test on a stationary bike, but I also had the option of the treadmill. My O2 levels and heart rate were monitored the whole time. They pushed me to my limits, until I got weak and nearly passed out. They wanted to see where my limits were. It turns out that my limits are much lower than a normal person my age, only about 47% of normal. I do know that cardiologists routinely perform these tests on certain patients, but I am not sure about your particular one. It would be a good thing to bring up since your episodes occur when you exert yourself .

I think you should ask for a stress test with pulse-oximetry so that doctors can see your problems develop while exercising.

If you have puffy, purple legs that means the blood is pooling in your legs. As a result your body produces adrenaline to combat the lower blood volume in your head, causing high blood pressure and a high heart rate in the second instance. In the first instance it seems you body adapts some to being upright and the heart rate lowers. Narrowing pulse pressure is common in POTS.

I have a similar reaction to your 2nd instance.

My doctor suggested Clonidine for the adrenaline surges and high bp, but that does not address the cause of the adrenaline surge which is the blood pooling when you stand. So, he suggested I pair Midodrine with it to constrict my blood vessels and help prevent the surges from being triggered in the first place.

He also gave me instructions for exercises to tone the muscles that help prevent blood from pooling. You can also try compression hose, but that would be uncomfortable in the summer.

Best wishes!

Hi Jadecat,

Has your son tried fludrocortisone (Florinef)? It acts like aldosterone in the body and causes the body to retain more salt and therefore increase blood volume and blood pressure. I am on this to keep my blood volume up.

Another option for raising blood pressure is midodrine, which causes the blood vessels to constrict.

My doctor also told me that I need to include exercise in my recovery plan. In part to tone the muscles around the blood vessels to keep blood from pooling and my blood pressure from dropping. I had an exercise stress test done that showed I am operating at only 47% of my functional capacity for my age (28). My doctor wrote me a exercise regimen which included what exercises to do when (both strength and aerobic).

The regimen is tailored to the fact that I have POTS and EDS, so I must use low impact exercises and small weights (2 lbs to start). I am supposed to be very careful to not overdo the exercise because I will crash. The goal is to exercise gently and slowly build up myself by adding more reps or more time when I am ready. I do exercises that help prevent blood pooling in the legs such as heel raises and ball squats (squats done up against a wall using an exercise ball to keep knees from bending forward, which can hurt the joint). I also do ab crunches, back extensions and various upper body moves with the small weights.

Since I can't run and the weather is too hot for me to use my recumbent tricycle right now, I have ordered a DeskCycle. It will allow me to do aerobic exercise inside sitting in a chair. I have to keep my heart rate in range of 140-160. Going above that risks triggering symptoms and a crash later.

Our hope is that over time (months really) I will improve my functional capacity to the point where I can get out and do things without paying for it as much afterward.

I hope your son can find a way to feel better!

Glad you made it through! I hope you recover easily and find that the surgery has improved your symptoms!

Hi MakeMeErised,

It does sound like you could use oxygen based on what I read here: http://www.webmd.com/lung/copd/oxygen-treatment-for-chronic-obstructive-pulmonary-disease-copd

It is about COPD, but has guidelines for how low the O2 sats need to be before oxygen is considered.

If your oxygen level stays too low for too long it can become dangerous, like you read. Do you know how long your spells last? Can you instruct the people around you to call for help or drive you to the ER if you do not come around within a minute or so? It is better to be seen by EMTs or doctors when in doubt than to risk damage.

If you do call an ambulance but recover somewhat by the time they get there you can choose whether or not to be transported to the ER.

Also, it should make your doctors take you more seriously if you are treated by EMTs or the ER for low oxygen.

Hi everyone. This syndrome is described and listed here as a cause of dysautonomia:

http://www.dysautonomiainternational.org/page.php?ID=150

Thank you everyone. I was so nervous before the last appointment. I knew that my blood pressure did not drop like in previous tilt table tests, but I still either passed out or got really close to it. I was afraid he would say it was all in my head or that he didn't know the reason. It turned out there was a very good reason for the way I was feeling.

Also we found that breathing into a paper bag helped bring my CO2 levels closer to normal and keep me from passing out. This is what is done when people hyperventilate during panic attacks.

I can see why some people mistake POTS for panic, the adrenaline surges create a lot of the same symptoms.

My sister recently developed POTS as well. In our case it is EDS. Since EDS is inherited and 60% of people with EDS develop POTS, it unfortunately makes sense.

Hello everyone,

This past week I have had about 16 different medical tests done at Dr. Suleman's clinic to help determine how my autonomic nervous system is functioning and rule out other problems. My doctor was the most thorough I have ever had.

What he found was this: I do have POTS, and most likely EDS (as my previous doctors said) but he wants it confirmed by a geneticist.

This is what he has shown:

When I am upright blood drains from my head and pools in my legs. This prompts an adrenaline surge which causes my heart rate and breathing rate to increase dramatically (hr to 180 bpm). My blood pressure also increases; he said that my body was over-compensating. My increased breathing rate leads to low blood CO2 levels, dropping so low as to cause vasoconstriction in my brain, reducing its blood flow. This reduced blood flow causes my fainting and convulsions even when my blood pressure does not read low.

He told me that about 60% of people with EDS also develop POTS, but that sometimes there is an underlying cause that can be found separate from EDS. He wants to find that cause if possible so that it can be treated directly.

He wants me to tackle this problem from several directions: exercise, diet, biofeedback and lastly medications.

My exercise stress test showed that I am operating at only 47% of my functional capacity for my age. So, his exercise physiologist wrote me a very specific prescription for an exercise regime for me, taking into account my POTS and unstable joints. Several exercises were modified to produce less stress on my joints while building the muscles around them. I am to keep my heart rate inside a target zone of 140 - 160 bpm at all times to avoid triggering symptoms.

I have not yet met with the nutritionist, but so far he wants me to increase my salt and protein intake (he said that people with EDS tend to not absorb protein which can cause muscles to not improve with exercise).

He has also loaned me a biofeedback program to help me train myself to lower my heart rate and breathing rate during surges of adrenaline.

Lastly he says we can try some new medications, but only under his supervision. He also wants me tested for gastroparesis and MCAD.

It has been a very difficult week, but I happy to have endured it because I now have some concrete answers and a plan for improvement.

Thanks everyone! I will let you all know how it goes.

@Ctat333, thank you for the tips! I will be sure to follow them.

Hello everyone,

I have been struggling more lately with dysautonomia, neck pain, and migraines, but I have managed to keep up with my students that I have been tutoring. I still have 2, and today I am adding one more. I have been having to lie down and rest quite a bit during the day and I try to pace myself so that I feel well enough to tutor. Unfortunately I have also been passing out more and I have had to cancel 2 sessions, but so far I have managed to work around that.

Everyday is a struggle, though, and I can only be upright and active for short periods of time. Getting everything ready (including my toddler) in the morning is hardest and I feel like I am being pushed to the edge of falling over. When I tutor my students propping my legs in a nearby chair seems to help.

I want to improve my functioning overall so I can participate more in life (family, work, social, ect.), so that is why I have scheduled to see Dr. Amer Suleman in Dallas. He specializes in treating disorders of the autonomic nervous system and has quite a bit of diagnostic equipment, including equipment to measure blood flow in the brain. I have been told to set aside three to four days for testing. I have never had this extensive of a workup so I am eager to see what it will show. I have already seen many doctors for my condition, including some considered to be the best in their field, and I have tried most of the conventional medications for POTS. So, at this point I am stuck. I am hoping this appointment will help me become "unstuck" and allow me to make progress once again. My appointment is June 2nd and I will be posting about it and the tests he orders so that other people can learn about what Dr. Suleman does.

Hi Northerndarlene,

It almost sounds like you had a mast cell reaction to the food. See symptoms here: http://www.mastocytosis.ca/signs.htm. The same website also has information on the low-histamine diet. Since I am not a doctor, I would ask yours about whether it really is a mast cell reaction.

Hope you feel better.

Hi Lyla,

I don't consider myself to be that flexible, and I do not dislocate, but I was flexible enough (5/9 Beighton) and had other signs such as scoliosis, hernias, and skin differences to be diagnosed with JHS. My sister is extremely hypermobile (9/9 score) and has more problems with unstable joints than I do. My doctor said that sometimes JHS/EDS manifests itself differently between relatives. That's why doctors will also want to look at your family history.

Best wishes!

An increase in the Florinef caused headaches in me as well. It's a common side effect. I can only tolerate .1 mg once a day. Maybe the headache will improve if you continue to keep the dose lower... Beta blockers are also sometimes used to prevent migraines. Not sure whether you tolerate those.

Yes. Sometimes I get tremors, or convulsions and my diastolic bp can be high. I take Librium for these attacks, which enhances the effect of GABA.

Sorry to hear you are dealing with this again. Have the doctors ruled out other causes of headache and nausea besides migraine? I hope you get to feeling better soon.

You made it! Congratulations. Just want to let you know that it is normal to have exhaustion and brain fog post partum. I am less sure about the headache and shakiness, maybe ask your doctor about those. It took me several months to fully recover from delivery. Your body has to adjust to different hormone levels and your blood volume drops. Enjoy your baby boy!

Yes, sometimes I feel really hot just before I pass out. I also get very thirsty and drink frequently. Sorry I don't know specifically why this happens.

Yes, if you are getting O2 readings that low, its best to talk with your doctor. Please take care!

Hi Tuesday,

I know you are not sure whether you have EDS or not, but hiatal hernia is common in the EDS population. So, if you'd like to talk to more people about their experiences with the surgery, you can head over to these forums: http://www.inspire.com/groups/ehlers-danlos-national-foundation/

Best wishes!

Hi Katybug,

For that test do you have to be on a treadmill? I last all of 2 minutes when I try to run on one (I don't try to run anymore, just walk). I hope the test does not make you feel more ill, and I hope your surgery in June helps relieve some of your symptoms!

Best wishes!