Another Mama With A Potsy Kid. Please Help Me Understand.

[gnomegarden]

Hello. I have been lurking for a bit, and want to say hello. I have a 12.5 year old daughter who was diagnosed with POTS at Hopkins just before the Holidays. She saw dr Abdallah in Reston last Friday and he dx'd severe hyperadrenergic pots and recommended seeing a genetics dr for looking into Ehlers-danlos. I think what I mostly am looking for is help with understanding how to best proceed in all of this. From reading here, I understand that there is usually something else that causes the pots to occur? How does one go about trying to figure that out?? I just want to help my sweet daughter-- it's as if she has been taken away by this. She is mostly bedridden. Losing weight. Her chief comPlaints are feeling super dizzy all the time with hot flashes and feeling disassociated. She can't even go to the restroom across the hall without hanging on to things. She has started midodrine and toprol. She says she thinks it has helped some, but she is also recovering froma horrid stomach flu that she came down with fri night. Her ttt was very hard to get through. I remember only that her hr jumped to 174 on tilt, which was at least 50 Pts. I am not sure what else to add-- just that I am so sad and feel so ineffective in helping her at this point. Any moms that might have advice, I'd love some!. Please excuse my typing--banana fingers on an iPhone! Dawn

[juliegee]

Welcome, Dawn-

Sorry for all you are going through. My son was also DXed at Hopkins at the same age. So hard to see our kids suffering like that You are right. There is almost always something underlying the POTS DX. If her doc suspects EDS- he's probably onto something. Has he recommended a geneticist to firm up that DX? My guess is that would be your next step.

Your daughter's level of functioning seems very low- so was my son's at that age- something about the onset of puberty and growth spurts. It's like the body doesn't have enough blood to meet all of those new demands. I suspect that she will get much better once you figure out a good med regimen. Is she drinking plenty of fluid and taking extra salt? My son is still managed by a wonderful ped at Hopkins- Dr. Peter Rowe, who does a wonderful job of helping kids with dysautonomia. Have you heard of him? Any chance your daughter could get in with him?

Better days are ahead. Stay strong. Hugs to you & your daughter-

Julie

[Sarah4444]

Hi Dawn-

So sorry that your daughter and family are going through this. I just wanted to let you know that I was in the same condition as your daughter last summer and I am gradually doing better thanks to info I gathered from people here. There seems to be a growing number of people who have lots of trouble with POTS, a hereditary disorder of connective tissue like Ehlers-Danlos, and a mast cell disorder. If she is one of us, apparently we have to be careful about taking beta blockers.

I found that the increased fluids and salt, and midodrine made it possible for me to do very light water exercise (but I still would have been too unwell to travel to a pool). Then the mast cell meds allowed me to start getting back some orthostatic tolerance. In terms of what causes POTS, there is a good list on the dinet site. I found it most helpful to try to find people here whose symptoms and signatures were most similar to what I was feeling. I am nowhere near functioning at a normal level yet, but feel at least like I am improving.

My favorite POTS article is the 2006 one by Dr. Raj (Dr. Grubb's are also good), and you should probably take a look at the 2005 one by Shibao et al called "Hyperadrenergic POTS in MCAD". Let us know how we can help.

Sarah

[gnomegarden]

Thanks Julie and Sarah. This seems such a super nice community! I really appreciate your welcome and thoughts.

Julie-- are you local to Hopkins then?? We are. In fact employed there. Much to our sadness, dr Rowe would not see our daughter, even after pleadings and campaigning from colleagues Just too busy. It is very frustrating If you are local I'd love to maybe talk to you about other local resources. Yes, we are seeing a geneticist as soon as I can get the paperwork done-- I thought we'd try dr francomano at Gbmc as I have heard good things of her. I am open to ideas though!! My daughter is so low functioning right now. It is hard and confusing as a parent to not really know when to " push them" an when to not. She has begun home schooling for now and manages to keep entertained pretty well lying in bed. I just want her to get some of her life back. I know she feels awful. It just seems like so much inactivity cannot be ok. She is drinking a lot of fluids and higher salt in diet. She does not tolerate the salt pills at all. As i mentioned the meds are not obviously helping at this point. She is having nightmares and is exhausted. I am wondering if this is the toprol's influence. . I don't mean to blah on and on. If you have any other thoughts on how to make things easier for them in the acute swings, I'd love to hear. Thanks again.

[gnomegarden]

Oh sorry-- sarah, I wanted to respond and thank you as well! Can you tell me how you coped with being so affected?? I hope that we can look forward to her getting better as well! I will check out those papers, thanks. What are the problems withhyper adrenergic pots and beta blockers?? I am still trying to figure out how to understand this very undefined illness... I will have to read about mcad, as I have no idea what it is. The first dr who dx'd her with POTS was so reassuring. "she just needs more salt and fluids, and she'll be fine. She will outgrow it very quickly" is the way I remember it . I had of course never heard of it, and sure had NO idea that those first few dizzy spells would so quickly turn into such a life altering problem. For the whole family! I try not to get too discouraged, but it is hard when it's all been so bad thus far! Thanks again.

[Sarah4444]

I'm happy to help, the thought of your poor daughter feeling so bad is so upsetting. I wanted to respond to a couple of things in both of your replies. Dr. Francomano is the doctor who diagnosed my hereditary connective tissue disorder and I would strongly recommend her. Not only is she very nice, but because she has a couple of patients with HDCT/POTS/MCAD, she is becoming interested in what is going on with us.

With regard to pushing your daughter academically, I wanted to mention that with severe POTS/MCAD I had significant cognitive problems (particularly with concentration and memory) on top of all the physical misery and exhaustion. I am the kind of person who always pushes myself, but with this illness, the harder I pushed, the sicker I got. I know this is so hard to balance with her academic responsibilities.

I also had insomnia and terrible trouble with falling blood pressure and feeling terrible at night. Taking 20 mg of doxepin at night really helped with that, but I don't know if they would want a child to take that. However, sleep is also an important part of recovery from this.

The problem with beta blockers is that they can make the allergic-type responses in MCAD worse apparently. MCAD is very confusing. I learned what I could about it but am still frustrated by how little I know. However, treating it has really helped.

I gradually got sick over a very long time, getting worse with traumas and illnesses. By mid 2010 I was totally bedridden and with two young kids, it was very hard to cope. My husband, mother and aunt had to look after me and my children. I am not back to normal, but am doing much better and at least feel like I am no longer going downhill.

It's hard, but for this you likely won't be able to find one doctor who will have all the answers. You have to find a committed doctor who is willing to read articles you bring in. I am still having trouble with that. In the meantime, if you feel she might have MCAD I would look into Gastrocrom for her. Apparently it's an older med with a good safety record and not too many side effects.

Please just let me know if I can help, and as frustrating as this is, know that you're on the right track to helping her recover.

[dakota]

Hello Dawn,

Let me first say that I am sorry for everything that you and your daughter are going through. You certainly are on the right track, though. My 16 year old daughter also sees Dr. Abdallah and he is the best that we have found. We were not able to get in to see Dr. Rowe either. Like Sarah, I would also recommend Dr. Francomano -- she is very good. And I also can't say enough about the people on these boards -- they are helpful, knowledgeable and compassionate.

I know how hard it is to watch your child suffer through this. We are working with Dr. Abdallah to try to find a combination of meds that will make my daughter feel better. A lot of it is trial and error. What works for one person does not work for another. It sounds like you are already doing all that you can to get help for your daughter. I hope that you will be able to find things that help.

I think we must be in relatively the same area. If you'd ever like to talk, just let me know.

Lynn

[juliegee]

Hey Dawn-

No, I live in GA. We had to travel to get help for Mack. His initial symptoms were an inability to eat w/o nausea and vomiting. He lost tons of weight and got dangerously thin. We saw Dr. Anil Darbari (with Kennedy Kreiger) at Johns Hopkins, where he held monthly clinics. He's a pediatric motility specialist. He was able to DX the nerve damage to Mack's small bowel AND connect it to the dysautonomia. THAT'S the only way we got in with Dr. Rowe. It was an "in-house" referral for a desperate case. I know your daughter's case is equally desperate & I wish there was good help easily available for all of our kids When you told me that she was very thin, I wondered about a slowed GI motility tract. What are her GI symptoms?

I also understand about the confusion. We regularly struggle with extracting exactly WHAT is his illness and WHAT is teenage laziness It sounds like your daughter is at a place where she needs all of the help she can get. Have you tried florinef yet? That one really turned things around for Mack. Maybe consider it. My son worked his way up to a pretty strong dose initially. It allowed him to function and begin attending school again.

I've also heard great things about Dr. Francomano. She'd be a great geneticist to see. Sarah might be onto something.....with your daughter's hot flashes. Maybe MCAD is playing a part in her illness. Have you learned much about that yet? Maybe a med like doxepin, atarax, or ciproheptadine taken at night would help control that, aid in sleep, and increase her appetite during the day.

BTW, we love Baltimore. It is beautiful, especially by the harbor. It is a beloved place to us- as that is where we finally found help.

Hugs to you both-

Julie

[gnomegarden]

Thanks Lyn and Julie. I really appreciate the kindness.

I was reading a bit today and stumbled upon a post here on "motivating a 13 year old" I think what I was trying to communicate in my post above was very much in line with the sort of things I was reading there versus trying to push my daughter academically. It is a much lower bar I am afraid. I completely acknowledge that she does not feel good, but she is so content to just lay around in bed for days upon days. I worry about the fact that she sees no sunlight, or people that don't venture up to her bed; I know that the loss of muscle tone and such only adds to the problems also.... She also just doesn't naturally possess a "motivated" sort of temperament. She will just lie there and read or ipod or craft forever... I worry that she is becoming a bed-bound invalid! She has already had to stop going to school and has been home schooling for the past month.Her initial docs were very concerned about her not getting up and about. I am also not sure if she is depressed-- I know that she feels physically uncomfortable at the least, but when we do manage to get her downstairs with the rest of the family, she does seem more alert/active and engaged in "life", even if its just from the sofa instead of bed.

Any thoughts or advice is very much appreciated, I am just so worried about her.

I am going to post a separate thread on figuring out how to coordinate her care. Please see and respond if you have any ideas. Thanks so much!!!!

Dawn