Poor Immune System & Pots?

[anna]

Hello all,

I posted this on another thread, but I thought I would like to start a thread to see if any of you have a low immune system or are diagnosed with a Primary immune disorder.

MY other post:-

Ok for a while now I have been looking at PRIMARY IMMUNODEFICIENCIES as a possible answer to my families POTS. We have a diagnosis of Classical EDS and I am sure this goes back a long way in my family, but I do not feel CEDS really explains the Pots symptoms, I am sure something else is linked here. Any way does anyone have any info linking POTS wit PID or CVID.

I know this might be a really stupid theory, but I think some forms of EDS have a fault on the same coding sequence as some forms of PID, this might explain why many HEDS/JHS patients have POTS and MCAD type conditions as well.

It seems people with PID or CVID have a 20% chance of having autoimmune disorders! Does anyone see a pattern forming.

Can any one out there that has a good understanding of genetics see any possibility that my mad idea might have some scientific credence!!!!

Anyone have any ideas about this?!!

Anna

[s-pot]

Hi Anna,

Id be interested in finding out if you get any links on this! I havent got any info to give but my POTS was diagnosed in March, however in my journey to get diagnosed I visited an Immunologist and had a full immunology screening (still awaiting some results) I have a dysfuctioning immune system and always have, prior to any POTS symptoms. Tests showed low T-helper and C4 cells however i continue my journey to see is some Immunodeficieny/Autoimmune problem my core illness setting off POTS>

I have a massive family history of Autoimmune problems...sister 21 diagnosed Multiple Sclerosis (progressive) last year, an aunt wheelchair bound with MS, grandmother died with rapid deterioration of Motor Neuron disease (6months after diagnosis), pernicious anaemia among other diseases.

I am still early on in trying to find out exactly what is going on in my body! However I do suspect my family genetic line involves some defected gene causing autoimmune problems and suspect this is the core of my problem!

Just to find out now exactly what that is is the toughie!

ill be keeping an eye on this link to see if anyone comes up with anything...sorry cant be of more help!

s-pot

[juliegee]

I have an impaired immune system; hypogammaglobulinemia, low igG (around 475.) I think normal is 750-1500. My igA is also intermittently low. I think you're on to something.

[anna]

I see a pattern in my family of so many odd symptoms that must be interlinked!! but I am not able to take this idea beyond just an idea point as my knowledge and understanding of genetics and well every thing is limited!! It would be really good if anyone with an understanding of genetics could bounce my idea about friends etc. to see if any one is willing to look into it more.

S-pot,

My family have lots of Autoimmune problems, we also have odd rare cancers, which is a big concern!! I will keep looking for leads on this one.

Mack's mum,

My GP clinic is not very receptive of my ideas they think I am a mad attention seeking mother! ha ha oh well their loss, so we have not had a chance to see anyone regarding our immune system, the only thing that I have noticed is that me and my kids are consistently on very low end of normal total white blood count, Plasma viscosity and Erythrocyte sedimentation rate is also on the very low end of range. my daughter also has a very low IgE but shows hugh symptoms of allergies.

Anna

[juliegee]

Anna-

Big me too. My white count has gone down to 2.5, very low sed rate, and plasma viscosity, and IgE- with severe allergy symptoms. I'm actually DXed with MCAD. You need to look into that. There is a strong correlation between POTS/NMH and chronic Fatigue Syndrome (CFS)/Chronic Fatigue Immune Deficiency Syndrome (CFIDS). CFS/CFIDS is strongly linked to a problem with the immune system. Maybe rather than trying to link POTS and an immune deficiency, look into that research- there's oodles out there.... Maybe if you bring some printed medical research into your next appointment and you will look slightly more sane I feel your frustration.

Re. the connection to EDS or any connective tissue disorder- YES- done. http://dynakids.org/Documents/pots_article2.pdf Many researchers are already linking autonomic dysfunction to connective tissue disorders, especially EDS. That's something a good POTS doc will rule in or out on a first visit.

Docs have established that autoimmune disorders can cause autonomic dysfunction. I don't think that the relationship or causation is well understood at this point. You have good instincts and much of what you've discovered on your own, with your own observations has already been established and is currently being researched. Make sure your doubting docs know that! Sounds like YOU may be able to teach them a thing or two. Just saying...

Julie

[HopeSprings]

I was thinking about this this morning. (well sorta the same topic, not really) It's bothering me how many children are getting POTS - I just can't believe it. And what do children get alll the time - viruses! It may be overly simplistic, but I wonder if there's some nasty virus that targets the ANS.

[Sallysblooms]

I have CFS, 19 years now.

[anna]

[sounds like YOU may be able to teach them a thing or two. Just saying...

Julie

Ha ha Julie I may well be able to!!

I think I will be busy with this idea for a while!! I would like to look into MCAD but this is not a condition the UK Dr.'s seem to be that aware of, am that sounds all too familiar!

Naomi Yes it does seem odd that we are seeing more and more children with POTS and alike, it is so sad.

Sallysbooms,

I am coming across more and more people with a diagnosis of CFS or ME.

[arizona girl]

Hi anna, from my own personal medical history I know there is a link. It has been proven by my doctors by skin biopsy and by blood work. I had positive ANA for years treated me as lupus patient didn't get better, rheumy's were never able to define the underlying autoimmunity. Years later the pots symptoms became so apparent that TTT was done proving the autonomic dysfunction.

That led to a neurologist who tests for autoimmune neuropathies, beyond large fiber nerves. Skin biopsy proved small fiber autoimmune autonomic neuropathy. While treating for that the immune deficiency was unmasked. Total Quantitative Immunoglobulins were tested and I had no IGM and very low IGG. Diagnosis of hypogammaglobulinemia or CVID a PID was made. So there you have it an autoimmune illness with low immunity. As research suggest they tend to go together. I was lucky thanks to dinet, and my current neuro who knows how to diagnosis small fiber neuropathy. I had no idea I should see a neuro, no one ever suggested it, I didn't even know there were neuropathic autoimmunities.

I'm about 9 months into treatment with IVIG. We've also been treating with antibiotics, as I have had undiagnosed infections all along as well. You don't get much of a fever, if one at all when you get an infection and you have low immunity. Which means you have to get pretty sick before someone recognizes you have an infection brewing. I've posted about this a bit, if you click on my name, you can see the posts I've made in that last year.

BTW if your looking for a doctor to diagnosis, look for a Neuro who specializes in neuromuscular autoimmune neuropathies, it is a subspecialty and there are not a lot of them and/or an immunologist who treats low immunity with IVIG.

[Sallysblooms]

Yes, on my CFS forum, there are a lot of us with POTS.

[Annaliese]

I think you are correct. Ive been researching this myself recently and im 100% convinced.

[icesktr189]

I was diagnosed with CFS but it wasnt until after my pregnancy. Before it, with my POTS, I never got sick. I think its def. autoimmune because usually it gets better during pregnancy and worse after, which mine did. It wasnt just POTS symptoms worsening. I have multiple EBV positives with mono, severe allergies out of nowhere, and fevers on and off. My immune system went nuts and hasnt been the same since...

[rubytuesday]

I have neg. ANA and multiple allergies/sensitivities but IgEs are ok. WBCs are ok. I do have CFS, fibro, EDS, and have Delta Granule Storage Pool Deficiency (platelets) and low T cells, T helper cells, IgG1, IgG2 and other IgG subclasses and low gamma globulins. I have to see hemeoncologist day before Thanksgiving.

[juliegee]

Hey Ruby-

With normal IgE and multiple allergies/sensitivites....you could have a mast cell disorder. Be sure your hemo checks your serum tryptase to rule out mastocytosis. If that's normal, you could still have MCAD.

I've got lots of the same stuff you do- with the exception of the bleeding disorder. Let us know how you fare & what you learn from the hemo.

All the best-

Julie

[rubytuesday]

Hey Ruby-

With normal IgE and multiple allergies/sensitivites....you could have a mast cell disorder. Be sure your hemo checks your serum tryptase to rule out mastocytosis. If that's normal, you could still have MCAD.

I've got lots of the same stuff you do- with the exception of the bleeding disorder. Let us know how you fare & what you learn from the hemo.

All the best-

Julie

Julie,

I also have colon polyps, GERD, osteoarthritis (early onset), osteopenia (early onset), chronic sinusitis,intermittent bronchitis, chomophobic microadenoma, IBS-D, chostochondritis, tendonitis (DeQuer Veins), tenosynovitis, pleuritis bursitis, gastritis, pericapsulitis, osteomylelitis, seborrhea (that waxes and wanes), eczema (that waxes and wanes), dry eyes, endometritis, endometriosis, pericardial effusion, cataracts (early onset), extremely myopic and cysts on ovaries, and on heart, lung, kidney that my PCP (IM) tells me are nothing to worry about and appear, self-absorb in everyone over time. I'd also had 2 or 3 Baeker's (sp?) cysts. I have fibromyalgia and chronic myofascial pain with EDS and the platelet

disorder I'd mentioned above.

My insurance company does not cover antihistamines and I've tried all the OTC stuff just for seasonal nasal allergies or hives/rashes but the only thing that seems to help is 50mg Benadryl around the clock (and that makes me very drowsy). I was reading about this MCAD and see that antihistamine of some sort being part of treatment. And I have no clue (other than the drugs and certain animal/environmental things) that could be triggering GI symptoms. I had pill cam that GI doc initially read as Celiac sprue but the cam was defective and repeated but was normal. My Endocrinologic testing (including thyroid and for pheochrom) was negative. I've lost so much weight (unintentional--and eat a lot when I can, small feedings when I can't but it's a challenge when your daily meds include meds at times of day when stomach needs to be empty and then the absorption time needed for that med when having delayed gastric emptying).

I did not know if this hemeoncologist (James Cancer Center who has clinical interest in bleeding/platelet disorders), with this info about my low immune function (that I'm taking that was done by my ID) would consider bone marrow/needle bx as beneficial or not (I think I read that mast cell activation might not show since it too waxes and wanes and is affected by PPIs). I'm not going to take tonight or tomorrow night's Zantac and I won't take tomorrow's or Wednesday's Prilosec OTC (til after hemeoncologist draws any more blood if he thinks he needs to).

Thanks for mentioning the tryptase. I wrote it down and will take it with me to ask when I meet this hemeoncologist. I've never met him before and am going to a clinic not on Columbus campus, so I don't know if their lab will be able to do the test or if he'll have to get me over to the Columbus campus or what. Am scrambling to try to get anything in before new insurance/higher deductibles and co-pays start in Jan. and with holidays, while I'm open (save Dec. 8th when I see genetecist), I don't have anything that cannot be changed to get the pricier things done. (But with ID and genetic tests and hemeoncologist and ENT endoscope that's due--it's all pricey). I think that one Delta Granule Storage Pool Def. test was over $1300. I don't remember how much those T cell, T helper cell and IGg subclass and gamma globulin tests were but I do remember they were not cheap.

I will try to start a post after I've seen hemeoncologist and what, if anything was done (and same with genetecist at Children's Hospital in Cincy).

On a side note, you must be my guardian angel as DH's mother is passed and DHs name is 'Mac' and my DSs name is Julie. She was serving our country in Iraq--so concerned about the POTs my cardiologist/IM med had told me I had this summer all the while having more faints, more debilitation over the summer that she (a veterinarian), found a link to EDS forum discussion on EDS/POTs. I had never told her I had been diagnosed with EDS in 2005 because my doctors here in the boonies (PCP and ortho who'd referred me to the rheume who specialized in fibro who added the EDS III, really didn't know what it was as they'd never had any patients with it that they were aware of). So between that forum (who put me onto this forum), I have been soaking up info like a sponge. And pieces of this puzzle are coming together. I have cognition issues (when once sharp as a nail medical professional) but now this thing with mast cells (when I am only beginning to get my DH to see orthostatic hypotension/dysautonomia stuff when he finally was with me one time when I fainted). The son mirrors me and was born (just found in his 30s having slow healing rib/chest injury) to have congenital dilated aorta that he disregards as EDS potential--saying he's nothing like me healthwise. He even took me to see Dr G (cardiac specialist at UTMC) who talked to him about it. He is starting to get some of my dysautonomia--the fact that I'll have fewer warnings. He used to fuss at me for exercising or going out on my bike when he'd walk in and I'd be on the floor--conscious. But he's never seen me pass out (although his grandson has).

We are here in boonies and neuro dismissed me as nothing neurologically wrong as did endocrinologist the cardiologist sent me to to R/O other things than 'POTS'. So if the hemeoncologist or genetecist doesn't pick up on this mast cell stuff, my family will think I'm neurotic.

Thank you again.

Judy

[juliegee]

Wow, Judy-

We do have lots on common- many of the same weird DXes.

Don't stop taking any of your meds prior to testing. I doubt your hemo checks your prostaglandin D-2. If s/he did, aspirin or other pain meds could artificially lower that. Nothing else should affect mastocytosis/MCAD testing. Keep yourself safe. THAT is most important.

You might want to check out a site/group for those with mast cell disorders, autonomic dysfunction, and connective tissue disease: http://uk.groups.yahoo.com/group/theelephantproject/ Lots of great info & support.

Sorry you are dealing with so much-

Julie

[kmichaelson]

I almost started a topic similar to this, but I'm glad I didn't since yours is so much more articulate and knowledgable than anything I could have posted! I confess, I don't know most of the acronyms or medical terminology I've seen in this thread (I just recently found out about my POTS & am still learning), but I've had a pretty messed up immune system as well. I have recurrent EBV and CMV (both mono-causing viruses), and I think I've had a sinus infection my whole life pretty much.

Other things that I think are linked are that I get canker sores/ulcers in my mouth every few weeks (which I've read can be related to immune problems), lots of joint pain, and I get a sore throat and achy/feverish feeling most days when I'm tired (like CFS symptoms). Also, I have a lot of digestive problems, like IBS... dunno if that's related and endometriosis. Basically I feel as if my immune system is attacking my own body and causing a lot of this, but I haven't actually seen an immunologist to be able to back this up. When I was tested for mono, my antibody levels were actually extremely high, so that makes me think it's almost an overraction of my immune system. Anyway, I certainly don't know as much about it as others in the thread, but I wanted to chime in!

[Annaliese]

Dizzyblonde, re mouth ulcers, i used to get loads until i swapped to herbal toothpaste without sodium lauryl or (laureth) sulphate.