We are still trying to get my daughter’s EM under control. It seems that she has developed a secondary “pain syndrome” along with the EM. Her pain is not limited to her burning hands, feet, scalp, ears, it takes off and goes systemic. It feels like every nerve fiber in every part of her “being” is ten feet tall! She equates it to someone having shingles in every part of their body, internally and externally. Her pain was so bad the other night that we had to go to the ER again. Third time in two weeks. ER doc essentially accused us of coming in and wanting meds. She called my daughter’s doctor at 2:30 in the morning! Talked to our doctor and she didn’t have too many good things to say about the ER doc. My daughter’s doctor is going to send us to a pain clinic to see if they can help the severe nerve pain while she tries to address the EM. I listened to a TED talk with an anesthesiologist who runs a pain center and he talked about a problem that happens with chronic pain where the pain no longer is a symptom of an illness, but becomes the illness itself. It was fascinating! Does anyone have pain to this degree? We are talking about a 20 out of 10. Does anything relieve it? Thank you for your help!!
Yes I can totally relate to what you’re talking about.
I do have severe small fiber neuropathy with my EM which now has also developed into autonomic dysfunction. I have tried all the meds and prednisone and opioids have help me. Because we think there is a large autoimmune component, I have tried IV IG. That works for me for a while it was like a total miracle. But then all of a sudden it stopped working. I am about to try plasmapheresis. I do have a gene mutation in the sodium channel that is not causal for EM but is commonly found in families and EM patients. We don’t know exactly what the gene mutation is doing but is probably related to hyperactive neurons and also gives me the diagnosis of a I Channel apathy
I have just started to take the drug Nucynta extended release and it seems to be helping .
We are so overwhelmed right now. Some days the pain is so bad that she blacks out (and she’s on pain meds). Other days the pain is a little less, but her limbs are all falling asleep and she looks as if she has no circulation. Extremely pale. She does have a positive ANA with a titer of 1:80, I know that’s low, but the first time she had it tested it was 1:40. So, it’s going up. She’s also lost about 1/3 of her hair. She’s not too happy about that. Her hematologist is considering IVIG if her blood work for GAD antibodies comes back elevated, but not for EM by itself. I do think she has an autoimmune component to hers, as well, and was hoping the doc would try the IVIG regardless. Evidently, it’s a problem that most insurance won’t cover it unless there’s irrefutable evidence that it’s absolutely needed. She’s looking for Stiff Person Syndrome, as my daughter has problems with her body “freezing” up in spasm when the pain is so bad. This all started with her body going into severe spasms (dystonia) even before the nerve pain hit. So, which came first, the chicken or the egg? Did I mention how overwhelmed we are?? Sorry to unload, but I’m at a loss as to what to do. Doctors don’t call back unless you harass them, and then you become “that” patient. To help get her through the weekend, we are trying higher doses of Benadryl. It helps, somewhat. Her primary neurologist had mentioned different channelopathies when we first started seeing him. May have to go back. We sort of stopped seeing him because we had exhausted his knowledge. Maybe this would be something he know? IDK… Thanks for your help!
Has she seen a rheumatologist? Her symptoms sound remarkably similar to mine. I hadn’t connected it with my problem but I have been losing my hair. When I comb it there is always quite a lot of hair on my comb. I have said this in other discussions that it took around 12 years to get a proper diagnosis. Mine is rheumatoid vasculitis. I have only started in August infusions of Rituximab which has only recently been used for any form of vasculitis originally it was a cancer drug. I just couldn’t believe how quickly and well it worked for me. I went from terrific pain 24/7 to hardly any pain. Of course it might not be but well worth seeing a rheumatologist and asking if it could be.
Thank you for your reply! We have seen three rheumatologists and none of them was worth anything! Two of them were at a major University in CA. I was furious when we left. They were absolutely sure that there was nothing autoimmune wrong with my daughter. They concluded this in a 1 hour visit! They couldn’t have possibly taken adequate time to come up with that conclusion, she’s too complex. The rheumatologist that my mom goes to is good, and I think she’s in the process of getting Rituxin infusions. Think that’s the same as Rituximab? The rheumatologist quit taking our insurance, though. Maybe if I mention the rheumatoid vasculitis to our hematologist, she might be able to help. Thank you so much for the suggestion, am going to read about it right now!
Something I am going to tell you will amaze you, or maybe not! It was over 12 months ago that I suggested to my rheumatologist that perhaps it could be vasculitis. At the time he said “Oh no certainly not”. He did at least apologise once he realised it was that. The only way I got there, which may not be possible as you don’t have the NHS as we do in the UK, was that there was a Multi Disciplinary meeting where a group of different types of specialists got together to discuss my case as non of them really knew what was going on. I was invited along. That’s when the idea of it being rheumatoid vasculitis came up. There was a haematologist, dermatologist, vascular surgeon and my rheumatologist present. The one thing I do have that you don’t mention your daughter has is ulcers on my feet and legs or should I say foot and leg as I had to have one amputated due to the ulcers turning to gangrene. They just appeared from nowhere and in the end they said it was due to the inflammation in my blood vessels.
Yes Rutuxan as it is called here with an a not an i is the same as Rituximab. So, it might be worth a mention to your haematologist.
Actually, I am not surprised! We have seen some of the finest doctors the US has to offer recently, and, for lack of a better way of putting it, they are all idiots! I should say that I don’t think they are stupid, they are highly unobservant and have their noses stuck in whatever project they are currently involved in or are still stuck in the “textbook” mode of diagnostics, instead of practicing the “art” of medicine. My daughter is anything but textbook and she needs someone or a group of people to think way outside of “the box”. I would love for her primary care doctor (whose known her since she was 4), her hematologist, dermatologist, a really good rheumatologist to get together with a white board and a few pads of sticky notes and start slapping up all of her symptoms on the board. Maybe if they saw them all laid out, something would click. One thing I’ve been so frustrated (could you tell I was frustrated :)) with lately is that they “part” you out. You’ve got this doctor looking at this aspect, another at a different one, and nobody communicates. I would LOVE to be in a meeting like you had and be able to make suggestions, correct misconceptions, and just lay the whole thing out to them all at once. One thing they are missing is that this is systemic - all caused by more than likely one condition.
My daughter doesn’t have ulcers on her feet/legs just yet, anyway. I’m so sorry that you had to lose your leg. That must have been incredibly difficult! May I ask, did you ever have high ESR readings on labs? My daughter has not. But, she had a situation a couple of months ago with inflammed optic nerves in both eyes and blurred optic discs in both eyes. At our follow-up appointment about a month later, the neuro-opthalmologist checked her eyes and said there was no inflammation now and only slight blurring of one of her discs. She had been on a very low dose of Prednisone for about a month, and I think that if she hadn’t been on that, her eyes would now be a far different story.
Last night, her left ear was really red and hot. This morning when she woke up, the left side of her face was red, hot, her left eye blood shot and everything was burning. Her left arm and hand are still burning. Any EM doesn’t usually show up with any severity until night, but today seems to be a turning point. Also, when her pain is bad, any nicks from shaving, hair follicles on her legs and even around all her fingernail beds are super red and inflammed. Any thoughts on any of these symptoms? I’m sorry this is so long, but I can see that you have been dealing with this a long time and are a wealth of information! Thank you for your help!!
I will reply tomorrow it’s nearly 01.00 here so I am attempting to sleep now.
Sorry I couldn’t have answered last night, but it would have taken me too long at that time. That’s the problem with time differences!
You are quite right about lack of communication between different people. Something that has been talked a lot about here. I was lucky I got there in the end. However, the same happens with care at home. Our local council just don’t communicate with doctors. I have failed to find a care company to take me on and I should be a priority, but the social workers probably don’t know that I have had an amputation etc.
It was hard to lose my leg, but the good side is I don’t have pain now on my right side! Or have to deal with the ulcers. I didn’t even get the phantom pain that most people get. I did have high ESR readings, but I believe that people don’t always have high readings even if they have the illness. Your daughters eyes sound very much as though she might have inflammation problems and as you say the Prednisilone would have saved them. Plus the fact that any nicks end up inflamed that shouldn’t happen.
I really do hope you get there soon.
I am sorry to butt in on this conversation, but in some occasions there are multi discipline groups available at different hospitals. My daughter has a vascular malformation on her shoulder, since birth. We had been told a strawberry patch, port wine, and many other diagnoses. She had multiple laser surgeries, which accomplished nothing. Fast forward. About 6 years ago she calls me ( in Pittsburgh) telling me her shoulder hurt and was swollen. I started investigating online. After research, I felt it was this vascular malformation, but with lymphatic involvement. And that one of the children’s hospitals in Ohio had one of these multi discipline teams. So, I call children’s in Pgh. They also have one, that team finds one in San Francisco, which is where she is. And boom, there we are. Diagnosis and treatment. Which 6 years later is on going. Being as she was born with it, she can be treated at children’s forever. Now though, she seems to have inherited my em. Whomever she has consulted with is telling her to cut nerves. Grrrr. Moral is… we must be super proactive. I have mixed connective tissue disease. Raynauds and em in same places. I still have not found any answers, but keep trying. Are you near a children’s hospital?
Please don’t apologise for butting in the conversation. That’s what it’s all about a discussion for anybody to join in. If people want to keep it between themselves they should be using private messaging.
Hi Marasmom!
Thank you so much for sharing your story, and, as Sheltielife said, you are never butting in! I love forums for the fact that it is one, huge, international brainstorming session! When I need to learn what people are dealing with day to day, I consult the forums. If I need technical info, I start reading medical journal articles. Yes, we are in an area with a Children’s Hospital. We’ve had good and bad experiences with them, as my youngest was hospitalized/treated there a couple of times. I so agree with you in that we all must be proactive. A doctor told me once that I needed to worry more about being a mom and let the doctors do the doctoring. I have taken that advice as fuel for the fire. I can be a mom AND the doctor, as I’m sure most of us have had to be for quite some time.
In hindsight, we believe that my daughter has had EM for several years, as she has always had hot feet after showers, never has liked to wear slippers because they make her feet hot. When going to bed, always started out with her feet outside the covers. It wasn’t until about July of this year that she just “burst into flames” as I say. But, here’s the thing, she has multiple things going on (Ehler’s Danlos Syndrome, Dysautonomia, POTS) and I’m wondering if it is “just” her Dysautonomia flaring up in a wild way (although she had a positive ANA and low Complement 4 levels). Around June of this year, she developed tremors in her hands. It then escalated to full on dystonic contracturing. This started with muscles, then progressed to the tendons doing the same thing, then the blood vessels started spasming shut (she looked very pale as if no blood was circulating) and limbs were falling asleep, that switched and started up the EM. So, I do think that hers is secondary EM, most likely having to do with everything else that’s going on. It’s a neurovascular-musculoskeletal-with a side of tenosynovitis disorder. Her nerve pain makes her feel like she’s got shingles on the outside of her skin and internally, the bone pain is excruciating, the muscle spasms have slowed somewhat, the tenosynovitis comes and goes. The nerve pain makes her black out when it’s too bad. We are in the process of getting an appointment with a Pain Clinic, and also a referral to a GI doc. The spasms are mostly internal at the moment and are making her stomach very distended and painful. My mom has connective tissue disease also, with an overlap of Lupus. We’ve tried several university clinics (we are also in the SF area) to no avail. They just can’t process this many symptoms at once. Their answer is to take her off all her meds and see what happens (not quite that extreme, but almost). I’ll keep Children’s in mind, though. They might accept her even though she’s 20. Any thoughts or suggestions are always welcome!!!
I cannot imagine what she is going through. How overwhelming. What about Mayo Clinic? I wouldn’t even know where to start. Maybe contacting different doctors via email first, seeing if one is more knowledgeable or interested in her case. What is the Dr that everyone tries to see at Mayo? Dr ? Davis ? I am totally blank. You would probably almost need to move to the area. I wish I lived there, you could stay with me! Maybe there is housing. Employment or school is probably not possible for your daughter? Mine and my daughters issues are petty compared to what your family deals with.
Joanne
Thank you for all of your kind thoughts! People on forums are like family because we are the only ones that truly understand what we are experiencing. I’ll have to check out Dr. Davis at Mayo. There is another doctor that started his own private medical research/clinic company in Silicon Valley. Both of my girls are involved in a CDC study that he is conducting on chronic illness and chronic fatigue syndrome. Might be interested in her case, never know. My daughter actually attempted to take one college course at our local community college. After the second day, she was in the ER. That was sort of the beginning of her POTS. In the two years since, though, she has managed to complete her first novel. Who says you need college to write. She lives to write. Just need to edit it and get it off to the publishers. Employment, we hope, will come in the form of her writing. It would have to be freelance, due to her health, but hoping that might be a possibility some day.
Happy Thanksgiving to you and your family!
I am so pleased that people are finding help here. You put it well FireGirl that we are like a family and feel that we can pour our thoughts out to each other. Of course that’s as important as finding medical things out as quite often you can’t talk to people who are close in the outside world and certainly if other people haven’t experienced EM they really don’t know how bad it is. I don’t know about anybody else but I get fed up of people asking what sort of pain it is, as I find it very hard to describe I have never had anything else like it in my life.
That would be great FireGirl if your daughter managed to get her novel published. It’s not easy as publishers get so many things sent to them. A friend of mine started self publishing and did really well here in the UK anyway I don’t know what it’s like in other countries. There is also Blurb if you have ever heard of it. If not look it up on the internet. That’s a way of self publishing that doesn’t work out expensive.
I can second Mayo - one of my friends lives in MN and her daughter (who is 18 now) had some major issues with POTS. If you want, I can ask who the Dr is that she saw.
please be sure to rule out lyme disease and coinfections, I have EM,peripheral neuropathy, raynauds, autonomic dysautomia,interstitial cystitis hashimotos thyroid, lumber back damage, severe allergies , asthma, cardio issues and more disabling conditions severe pain everywhere- all a result of undiagnosed long standing bacterial and parasitic infections and lyme- lyme is known as the great imitator- be sure to get testing done at a good lab like Igenex- go to a lyme literate Dr not an infectious disaease dr