Caregiver Asking Questions

[Connie]

"Julie,

Thanks for all the great information. I will go to The Mastocytosis Society Website and check it out. Yes his cardiologist did mention a possible histamine problem but never addressed it or even recommended it be addressed. for time to time he had what we thought were allergic reactions to something - twice so bad we went to Dr and he had shots to alleviate his symptoms. One time we were out of town and it became so bad we had to call 911 and they meet us on the side of the road. His skin looked like he had been dipped in boiling oil, his lips were swollen and his ears were so swollen they were sticking way out. He had severe stomach cramps and nausea. They gave him two shots of epinephrine before we reached the hospital and the hospital kept him over night. I remember they kept asking me if we were sure he was having a allergic reaction to something and I kept telling I did not know for sure what the problem was. We could not figure it out. Since that time we have Epipins and he has not another reaction that bad since. If he feels like a reaction is starting he takes two benadryl tablets and that usually helps. He does take allergra/fexofenodine twice a day for allergies.

As for his stomach - I did forget he takes Prevacid and that helps him but sometimes he mentions excessive acid. There are days when he feels so bad I can not get him to eat very much. Either nothing sounds good or he is too tired. I will try to go out tomorrow and find some turmeric. That sounds very promising. Brain fog is a huge problem. We tried school work again today with science after 25 - 30 minutes he was shaking all over and getting very frustrated saying he had enough. That is very frustrating for both of us because he is very intelligent and I keep trying to help find a way he can learn without putting too much pressure on him. But hardly anyone understand why he can not go to school or at least do home school. But by the time we start he becomes so washed out. Hopefully the turmeric will help. He weighs 145 pounds - how much turmeric do you take?

I am going to talk to our primary care physician and see if we can schedule a sleep study. We found out about the bradycardia when we had the tilt table test done. His pulse dropped to 37 and then 40 -42. After having the halter test, the cardiologist said this was an additional problem and would account for him being so fatigued but could not recommend anything else except a stimulant for energy. He did mention a pace maker but did not think it would help.

Thanks for the suggestions. I am so glad I found the Forum. It is so great to be able to chat with people that have the same problems because so many people health care providers included do not understand.

Hope"

______________________________________________________________________________________________________________________________________

Hi Hope-

Wow, sounds like a lot of his symptoms may be mast cell related. I, too, have had episodes of anaphylaxis and I'm allergic to nothing. I carry an epi-pen and have had to use it. When my mast cells are acting up, my autonomic stuff is so much worse. There have been studies linking POTS and Mast Cell Activation Disorder/MCAD. I first learned about it on the "Causes" section of this DINET website.

The daily allegra (and benadryl as needed) are good; but may not be enough. In mast cell disorders, patients must take daily H-1's ( you have that covered with the Allegra) and H-2's (Zantac or ranitidine.) The combo is what calms the mast cells down. Since your son is having excessive stomach acid issues, adding a daily ranitidine would address both issues. My son takes a Nexium in the AM, (and because MCAD is suspected,) he takes a ranitidine in the afternoon- that's about the time he may have break through acid from the Nexium anyways. Many patients, myself included, also add an anti-leuketreine/Singulair. I have pain upon breathing in if I don't take it- despite the fact that I don't have asthma.

I would also recommend that your son keeps up the Allegra in the AM, but takes something stronger at night like Doxepin, Atarax, or Cyproheptadine. All are potent H-1's, will help with sleep, and appetite. Atarax in the PM was life-changing for me. As long as I tae my meds, I am almost symptom free.

Keep a tiny pediatric dose of Ery-Ped (liquid erythromycin) in mind as a GI prokinetic. My son takes a half teaspoon AM & PM and it irritates his stomach just enough to make him REALLY hungry. He can't eat w/o it. Some here use domperidone the same way.

Finding a doctor who understands the mast cell issues & how they relate to dyautonomia is difficult. Tell me what part of the country you live in & I may be able to recommend a physician. There is a new website called Mastopedia, that has a forum. Folks there could also help you find a doc.

I know how hard it is to see your son suffer. He's lucky to have a wonderful Mama fighting for him. I suspect he can get to a much higher level of functioning once he gets onto a more effective med regimen.

Hugs-

Julie

Julie,

Thanks again for the information. I am almost in tears to find there are other things to check to help him. I kept reading everything I could find but not finding answers until I found this site. I had the feeling there were other issues other than POTS. Since we have started back trying to do school assignments he has passed out twice in two weeks. He had not passed out since end of grade test last May. School does him in, even in small doses. I feel like PotsMom, my son even made the same comments to me "School is not worth it. You do not know how I feel." My son loved school and was in the top of his classes. Another reason why the school here cannot understand his medical problems. Last year he passed three end of grade test without any instruction or even opening class books the last 5 months of school. But just like PotsMom even trying to complete work in small doses does not work. He loved school and misses it. Also like PotsMom, he watches educational program and seems to learn from those but has huge problems with recall and memory.

We live in NC. His cardiologist is at Duke but the last 3-4 appointments we had to cancel because he was too ill to go. Also I feel like he "too busy" with heart patient and patient that are critically ill to have time for us. I have waited 3 - 6 weeks for return phone calls and never received answers at times. I would like to find someone who we could work with who understands and has time to work through all of his problems. I am thankful the cardiologist was able to give us the original diagnoses and then later did the tilt table test for us. At the time we saw him, in fact the same day - we saw our regular Pediatric GI Specialist, Pediatric Neurologist, and Infectious Disease Physician - all of whom told me to "make him go to school" and we needed to see a psychologist to help us with "our problems." In fact a primary physician we were seeing was looking at me for Munchausen Disease. I am a single mom with a full time job and I would "want" to create all this problems - get real. I am very thankful for the cardiologist diagnosis but I would greatly appreciate any names of doctors you could provide for me to find more answers. Our primary doctor would be more than willing to have test ran, work with another doctor anything to help us manage my sons condition. Our doctor has been wonderful trying different medication, etc.

I am calling this afternoon to see if I can find turmeric. How much do we start taking?

Thanks again Julie for all your help and support. I can't imagine how hard it is for you to deal with your health issues and your son's health issues and have time to try to help all of us. Thank you so much. I would greatly appreciate any suggestions.

Hope

[kjd111]

Julie,

thank you so much for the reply. My daughter actually wasn't on any meds at the time for those anaphylactic attacks. She had one last August, one in January and one in February. We started with our current endo in March and to be honest, she hasn't had a lot of difficulty since. She had whopping spring allergies (for the 4th year), and we have been oh so careful to keep her away from the things we know did it last time - grapes, apples and dairy. So far, so good on that at least. Her IgA was normal, as was her IgG series. The IgM was very low (in the 20's) and her IgE slightly elevated (by like 2 points - a 159 when the top of the range was 157 or something). The pneumoncoccal titre test was the one that really opened the allergists eyes a bit. If i remember correctly, there are 10 or so parts to it and she was basically a zero response on 6 of them. The other weird part was that she started having small reactions to things like apples that she had been able to eat forever, long before they showed up on the skin tests. We took her in at 13 to a very well known immunologist where we are (he's not got the best patient manners so we didn't choose to go back when things got bad last year) and despite literally not being able to be around the smell of apples without her throat closing up, the skin test was negative. Last year it was hugely positive, but the grapes weren't and yet it was grapes that sent her to the ER - twice! The other oddity, was that all last year, the only major things that the other specialists could find wrong with her before her diagnosis, were low red blood cell counts, somewhat low hematocrit (35) and protein in the urine. We did the rounds of nephrologists, who couldn't find anything wrong with her kidneys (scans, test, you name it) but she consistently showed up with 2+ for protein in the urine - normal albumin in the blood though. After she started on the meds in March, the summer urine test showed negative protein and the one in August only a trace. It is the endo's theory that her kidneys have been "denervated" by the dysautonomia and the meds have helped - but the kidney issues were apparently never the cause of the water retention/angioedema. Its this latter part that we are becoming somewhat frantic about trying to solve. If you can help steer us to someone who could look at her for the mast cell issues, that would be great. We are in Dallas Tx.

For Hope, your story with docs makes me deeply angry. While i have yet to hear that to my face (munchausen), we have had our share of docs blow us off and one particularly nasty neurologist last December at one of the MAJOR children's hospitals in our area accuse my daughter of "needing a shrink". He told her that to her face. Screamed it actually. She is in school; in fact that is what set him off - it was 2 days before finals when we went to see him in the middle of the day and my daughter was a 9th grader in pre-AP chemistry. She brought her chemistry and he told her that between that and being a competitive skater (or trying to still be one - rather unsuccessfully due to the health) that she had enough stress for a "holocaust survivor" and "needed help" and was a spoiled (name of town) kid who should get to a "shrink". Then he said that he didn't doubt she had some sort of angioedema but what we were describing was "impossible" (yeah - made it up just to hack you off buddy) and he wanted a drug screen on her. When i insisted she wasn't taking drugs - he said "performance enhancing" drugs. When i then said "i am at the rink in the viewing area every day", he said "her coach is probably slipping it to her". I was like "where, in her water for goodness sake"? This guy had clearly read too many dime store novels. And besides, if he knew anything about skating, they don't want to be bulky and strong - just the opposite - lithe and lean. We get eating disorder issues in this sport, not steroids! Our endo and the people who sent us to this guy were livid, and deeply embarrassed and apologetic, but as you can see, i'm a little sensitive about it still and that was 9 months ago.

Whew. Deep breath. Off soap box now.

[juliegee]

Hi Hope-

I so get you with the Munchausen's thing. In my past life, I was a therapist & realized I appeared to meet the criteria- esp. as Mack often "seemed" OK at doctor's appt's. Little did that doc realize how much it took for him to even be there. Grrrrrr. i was never accused, but fully expected to be. I have met other Mom's who've encountered the same thing. You are so right. Who'd ever make this stuff up????

I understand about the school stuff. Mack only missed a year, but trying to teach him was torture. He did best lying down with his feet elevated. I am not kidding, there were times when I'd have him hang his head upside down over the sofa- legs in the air to get blood to his brain. It is so sad what these kids go through and no one seems to understand or empathize. If you haven't done it yet, I'd probably line up to do a psycho-educational assessment so that his academic deficients can be quantified. If you are in the US, this should be free through your school system. Your child will need accomodations when he gets back to school both for academic and medical reasons. The two are inextricably linked. Tell me if you want links to studies, etc. Mack, too, has awful memory issues and very slowed speed processing. He is entitled to extra time on quizzes/tests, even standardized ones like PSAT, SAT, and the ACT. He can also take frequent breaks and even got to take his ACT on multiple days. You need to do the testing to line this all up. I took the turmeric from GNC & followed the dosing instructions on the bottle.

You are really lucky to be near Duke. I have heard of an allergist there who deals with mast cell issues named Patricia Lugar. Here is her contact info: http://www.dukehealth.org/physicians/patricia_l_lugar

Sounds like you could use an ANS specialist to put it all together for your son. We never found cardiologists or neurologists to be of much use either- aside from DXing. They seem to have little understanding of how this condition affects our children overall. We are blessed to have a brilliant ped at Johns Hopkins, who totally "gets" it and is a huge advocate for our kids. Unfortunately, he is not accepting new patients...but I'd be happy to ask him for a recommendation for someone in NC. There is a doc in upstate New York, Lyndonville I think, who specializes in these kids. His name is Julian Stewart. Google him to learn more. Maybe Dr. Lugar will know of someone at Duke who can help put it all together for your child

Hang in there, Hope. You are doing a great job finding help for your son.

Julie

[juliegee]

Julie,

thank you so much for the reply. My daughter actually wasn't on any meds at the time for those anaphylactic attacks. She had one last August, one in January and one in February. We started with our current endo in March and to be honest, she hasn't had a lot of difficulty since. She had whopping spring allergies (for the 4th year), and we have been oh so careful to keep her away from the things we know did it last time - grapes, apples and dairy. So far, so good on that at least. Her IgA was normal, as was her IgG series. The IgM was very low (in the 20's) and her IgE slightly elevated (by like 2 points - a 159 when the top of the range was 157 or something). The pneumoncoccal titre test was the one that really opened the allergists eyes a bit. If i remember correctly, there are 10 or so parts to it and she was basically a zero response on 6 of them. The other weird part was that she started having small reactions to things like apples that she had been able to eat forever, long before they showed up on the skin tests. We took her in at 13 to a very well known immunologist where we are (he's not got the best patient manners so we didn't choose to go back when things got bad last year) and despite literally not being able to be around the smell of apples without her throat closing up, the skin test was negative. Last year it was hugely positive, but the grapes weren't and yet it was grapes that sent her to the ER - twice! The other oddity, was that all last year, the only major things that the other specialists could find wrong with her before her diagnosis, were low red blood cell counts, somewhat low hematocrit (35) and protein in the urine. We did the rounds of nephrologists, who couldn't find anything wrong with her kidneys (scans, test, you name it) but she consistently showed up with 2+ for protein in the urine - normal albumin in the blood though. After she started on the meds in March, the summer urine test showed negative protein and the one in August only a trace. It is the endo's theory that her kidneys have been "denervated" by the dysautonomia and the meds have helped - but the kidney issues were apparently never the cause of the water retention/angioedema. Its this latter part that we are becoming somewhat frantic about trying to solve. If you can help steer us to someone who could look at her for the mast cell issues, that would be great. We are in Dallas Tx.

For Hope, your story with docs makes me deeply angry. While i have yet to hear that to my face (munchausen), we have had our share of docs blow us off and one particularly nasty neurologist last December at one of the MAJOR children's hospitals in our area accuse my daughter of "needing a shrink". He told her that to her face. Screamed it actually. She is in school; in fact that is what set him off - it was 2 days before finals when we went to see him in the middle of the day and my daughter was a 9th grader in pre-AP chemistry. She brought her chemistry and he told her that between that and being a competitive skater (or trying to still be one - rather unsuccessfully due to the health) that she had enough stress for a "holocaust survivor" and "needed help" and was a spoiled (name of town) kid who should get to a "shrink". Then he said that he didn't doubt she had some sort of angioedema but what we were describing was "impossible" (yeah - made it up just to hack you off buddy) and he wanted a drug screen on her. When i insisted she wasn't taking drugs - he said "performance enhancing" drugs. When i then said "i am at the rink in the viewing area every day", he said "her coach is probably slipping it to her". I was like "where, in her water for goodness sake"? This guy had clearly read too many dime store novels. And besides, if he knew anything about skating, they don't want to be bulky and strong - just the opposite - lithe and lean. We get eating disorder issues in this sport, not steroids! Our endo and the people who sent us to this guy were livid, and deeply embarrassed and apologetic, but as you can see, i'm a little sensitive about it still and that was 9 months ago.

Whew. Deep breath. Off soap box now.

Hi Kate-

You definitely need a mast cell doctor to sort things out. Unfortunately, I don't know one in TX, but I will refer you to a few websites where you can ask others to get that info. Here is a link to one: http://mastcelldisorders.lefora.com/forum/category/mast-cell-disorder-doctors/ The other is new, but the forum is not, it is called "Mastopedia." Just google that for a link. The folks at The Mastocytosis Society are also very helpful. Valerie Slee, the board chair in particular, will help to find you a doc. Their site is: www.tmsforacure.org

In my last post, I meant to mention that a normal serum tryptase test does rule out mastocytosis (except in rare cases), but it does NOT rule out mast cell activation disorder/MCAD which is linked to autonomic dysfunctions. The fact that your daughter's IgE was close to normal does point to a mast cell issue. Additionally, she clearly has immune issues. She may even meet the criteria for Common Variable Immune Deficiency or CVIDS. I also have impaired immunity, but not CVIDS (go figure ) My immunoglobulins are quite low, but I do make antibodies to pneumonia. Some here have that DX and are treating with IVIG therapy, which has greatly improved autonomic dysfunction symptoms in some patients. This is an avenue that you should consider. All mast cell docs are also immunologists so the same one should help you untangle things.

I hear you about the Munchausen's. Grrrrrrrrr. My son was also told that he was emotionally ill (by a pediatric GI that we travelled to see)- THAT was why he couldn't stand upright, eat food, or attend school. I was furious. It ultimately led us to Hopkins and the best teams of doctors in the world. LONG road getting there. The doc that made such crazy accusations towards your daughter should be prosecuted. What ever happened to the Hippocratic Oath- "First, do No harm." We turn to these docs in desperation....

My son was a big time skateboarder and straight A student when he was struck ill. It was heartbreaking to see him have to drop everything that he loved. i know your daughter is dealing with all of that as well. Keep fighting for her.

Hugs-

Julie

[kjd111]

Julie - I will definitely see if i can contact someone to see about the MCAD. I have actually lurked here for a few months now, and have been trying to get a handle on what the folks with MCAD are taking/doing that has helped. Based on that, we added Zantac and Singulair to the mix. Zantac was given in the ER, and was available over the counter and you know, i think it has helped some. In fact we ended up trading it out for the Zyrtec - which she was taking at night because we ran out of zyrtec one night. She was actually a little less swollen the next am and while i've no idea if the zantac did some of that, we went with it. The singulair she had been given by an endo last year who didn't know what was wrong with her but figured that the allergy thing was serious and wanted her to take it. At the time, the problems felt less immune related and more endocrine or something else, so (bad me) we kind of didn't take it. I read up on the MCAD thing and singular seemed to target something "leukin" or some such and i read that thing could cause swelling, so we rolled with that one and i think there's been a little progress there too. She still swells. It really bugs her because she swells from her mid thighs through her abdomen and into her chest. It changes her whole body shape and you know.........teenage girl..........body shape.........the tears are beyond when it happens. But, i've noticed that the more activity she pushes herself to do, the swelling swings seem a little smaller (4-5 pounds of fluid vs. 10). The endo is baffled because her blood pressure holds pretty well now when upright, but we know her capillaries still aren't behaving. Sometimes when she does spins on the ice, she'll get little broken blood vessels all around her eyes. She's skated since the age of 6 and never had that happen. Also, her exercise tolerance is still not great. But, given what i've heard on these boards, i think perhaps we should be thankful we have gotten as far as we have. And, despite the holes in her progress, i am so, so, so thankful for finding our endocrinologist - who not only didn't want to send her to a shrink, but knew in about 5 minutes based on his first pass at heart rate and her pallor. He is a little older, but has the patience of a saint and while we will keep looking until we can plug those holes, i feel blessed. He is worth his weight in gold. I have debated for some time how to handle the neurologist. I wanted to wait until i was 100% sure of our diagnosis and progress and then thought i would write to the medical center where he is on staff, show them her diagnosis, and describe what he said to her.

[juliegee]

Julie - I will definitely see if i can contact someone to see about the MCAD. I have actually lurked here for a few months now, and have been trying to get a handle on what the folks with MCAD are taking/doing that has helped. Based on that, we added Zantac and Singulair to the mix. Zantac was given in the ER, and was available over the counter and you know, i think it has helped some. In fact we ended up trading it out for the Zyrtec - which she was taking at night because we ran out of zyrtec one night. She was actually a little less swollen the next am and while i've no idea if the zantac did some of that, we went with it. The singulair she had been given by an endo last year who didn't know what was wrong with her but figured that the allergy thing was serious and wanted her to take it. At the time, the problems felt less immune related and more endocrine or something else, so (bad me) we kind of didn't take it. I read up on the MCAD thing and singular seemed to target something "leukin" or some such and i read that thing could cause swelling, so we rolled with that one and i think there's been a little progress there too. She still swells. It really bugs her because she swells from her mid thighs through her abdomen and into her chest. It changes her whole body shape and you know.........teenage girl..........body shape.........the tears are beyond when it happens. But, i've noticed that the more activity she pushes herself to do, the swelling swings seem a little smaller (4-5 pounds of fluid vs. 10). The endo is baffled because her blood pressure holds pretty well now when upright, but we know her capillaries still aren't behaving. Sometimes when she does spins on the ice, she'll get little broken blood vessels all around her eyes. She's skated since the age of 6 and never had that happen. Also, her exercise tolerance is still not great. But, given what i've heard on these boards, i think perhaps we should be thankful we have gotten as far as we have. And, despite the holes in her progress, i am so, so, so thankful for finding our endocrinologist - who not only didn't want to send her to a shrink, but knew in about 5 minutes based on his first pass at heart rate and her pallor. He is a little older, but has the patience of a saint and while we will keep looking until we can plug those holes, i feel blessed. He is worth his weight in gold. I have debated for some time how to handle the neurologist. I wanted to wait until i was 100% sure of our diagnosis and progress and then thought i would write to the medical center where he is on staff, show them her diagnosis, and describe what he said to her.

Hi Kate-

I highly suspect that she will improve on the zyrtec (H-1), zantac (H-2) and singulair (anti-leuketreine.) All of those should be taken in the AM. Consider adding a stronger H-1 at night. i used a benadryl for years. These are all preventative and need to be taken daily. Weird you mention the broken blood vessels around her eyes because I've been dealing with that too. I'm well enough now (on my med regimen) that I exercise really hard- run, bike, weights, etc. If I push it, I get those dots around my eyes. Luckily, they don't last long- must be a part of this thing.

She's so lucky to have a Mama on the ball whose figuring it out w/o the docs. It was other parents who initially DXed my son and then steered me to docs who got it...Sad, but it worked.

Hang in there & post her progress to us all.

Hugs-

Julie