Has anyone tried immunoglobulin therapy for dysautonomias?
Interesting question! Immunology has me on longterm 3-weekly 4 hour IgG replacement therapy infusions (IVIG) for my Primary Immunodeficiency (pan hypogammaglobulinaemia) which is simultaneous with my other 4 very early onset primary illnesses (DES Syndrome, hEDS, Systemic Lupus Erythematosus)
My dysautonomia had already been responding positively so quite damped down thanks to my daily lupus meds (hydroxy + prednisolone + mycophenolate) and when i began IVIG i felt as if my dysautonomia became even more well-medicated
Are you in the UK? Is Immunoglobulin G Replacement Therapy being proposed by your medics? If yes, for which condition - because doses vary from mine (for PIDs: 25mg gamunex) to much higher for neurological illnesses
PS do you know this official article:
We are from New Zealand. An Australian integrative medical doctor has recommended the immunoglobulin. The dysautonomias are very severe- she has major abdominal pain and can’t seem to digest or absorb food and has lost a massive amount of weight. She also has Behçet’s disease.
I see, thanks. My feeling is that if you are NHS patients over here, your daughter may already be in the care of hEDS-expert rheumatologists & gastroenterologists. Is a MCAD (Mast Cell Activation Disorder) being considered?
Where are you in the UK? because hEDS gastro etc experts are mainly in the london area, but there are 1 or 2 up north.
In case it’s useful:
At my NHS univ hosp, my lead consultants are in rheumatology (a lupus expert) + immunology (a PID expert) + gastroenterology (an IBD expert). I’m on long term Elemental Enteral Nutrition (EEN) due to a lifetime of mouth to exit slow transit dysmotility + inflammatory crohns-like CTD+AID+PID Intestinal Insufficiency manifestations segueing 6ish years ago into Intestinal Failure (EEN is prescrip predigested amino acids taken by mouth because i can no longer digest food and am hyper-sensitive+reactive to food residue of all kinds). Our goal is to keep me able to be on EEN, avoiding segueing onto tube feeding aka PEN (Parenteral Nutrition) etc, but, lucky for me, my univ hosp has a world-leading Gastro Feeding Issues Multidiscipline Team (FIMDT) so will get extra tertiary care from that should i need PEN
I can suggest various support groups & sources of info..but maybe you’re well orientated already
Hope something in there can be useful. 🍀❤️🍀❤️
PS hope i’ve understood:
you’re from NZ, but based in the UK now?
Thankyou Barnclown. You seem to provide so much helpful information to many. 😊
Thank you...i do my best...this life is that bit easier to manage when we support one another 🤝❤️🍀
We are still living in NZ. I am desperately trying to find the right doctor/clinic/treatment. Her main issue is dysautonomias, with gastrointestinal dysfunction being life threatening. I have joined many online groups for Ehlers-Danlos syndrome, GI dysmotility, dysautonomias. I am trying to figure out if IVIG, stem cell therapy have been successful for patients with patients with EDS and autoimmune diseases (she has Behçet’s disease as well). It has taken me 5 years to get these diagnoses. I have been fighting hard to get doctors to see past the anorexia nervosa label. There have been no longterm treatment options offered to her. The EDS specialist suggested a PEG J tube for life. We are not going to settle for this. She deserves so much better. I joined this group because I saw how insightful and knowledgeable you are actually! You have been and are going through such a lot- an understatement for sure. Our daughter has been robbed of her teenage years- from age 13 years she’s been very unwell. New Zealand is a terrible place to be if you have rare diseases. Do you know much about stem cell therapy? Thanks for all your help and support. Janine
Oh janine: am greatly feeling for you all
Will priv message you here now XOXO
I have not heard of this yet.
Useful to know.
I had seen an article on the "first patient" who had EDS receiving IVIG in Japan. The article was so brief and it did not say what IVIG was prescribed for. EDS-related disorder, I did gather.
IVIG seems to be commonly prescribed in the USA/or Australia where people pay a hefty sum of money for their healthcare cover.
Yes the USA is very ahead with using IVIG for many off- label conditions. Thanks
Yes, it seems to be.
Far more enlightened,
and it shows their commitment to
get patients better sooner than later!
Here is a link to the latest official UK Immunoglobulin therapy guidelines:
Thanks Barnclown. We have a compassionate supply of immunoglobulin therapy from a US biosupply company. We have not managed to get any doctor in NZ yet to consider it for EDS/autoimmune Behçet’s disease. We are trying to get it registered in Australia as there are 2-3 doctors who may be willing and able to help us get it to her. The more I read online about patient experiences with it though the more I am not sure about it. Very mixed responses and side effects. Don’t know if I want to put her through all that and have to re locate overseas etc. she’s very low body weight right now and been so traumatised by many doctors.
My feeling is that you are thinking this through with great common sense + acute intelligence + compassion. All the research i’ve managed has me thinking this way too. My impression is that bad reactions tend to happen most with the higher doses of IgG given to neuro patients who have history of hypersensitivity (eg i personally know one such patient). saying that, i hyperreacted to IVIG when infusion clinic tried giving it to me without premeds (100mg hydrocortisone + 20mg piriton + 1000mg paracetamol), so, after that reaction, immunology decided i am given premeds before each 3-weekly 25mg infusion for my PID.
What makes it more difficult is that every doctor has a different opinion. In the end I have to respect our daughter’s the most. She’s a bright girl, having been the one to put Behçet’s and EDS to the doctors. It’s her body/mind.
Am constantly thankful for internet resources + in awe of younger patients navigating their way through the diagnostic & treatment process!
YAAAAY: I’ve had medics tell me younger patients don’t put up with being messed around (ie the gaslighting, stonewalling & occam’s razoring mine endured)...i say: Well done!
I cannot imagine what you have had to put up with over the past years, decades..... You have certainly gathered a wealth of knowledge, experience and compassion. 🤗
💐 thanks: i just try my best to follow the wonderful example set by mothers (sisters, friends, colleagues, medics, support group staff etc) who show they really care & want to acknowledge + support those of us beset by the spectrum of challenges the overlapping nature of rare diseases present to most of us. Eg my poor dear mother’s despairing choices directly resulted in one of my rare conditions (DES Syndrome) which rendered me infertile and probably triggered my simultaneous very early onset AID/PID...her reaction was: shame + denial + withdrawal. On the other hand, there are mothers like you who sensitively acknowledge + encourage + engage in positive direct action: you give me hope. I think you’re wonderful
I’ve made some pretty controversial choices- with daughter’s consent. Namely going to an integrative medical doctor who ordered expensive tests in the US and could not interpret them. And failed to refer when she was out of her league. I also got fooled into going to an eating disorders/rehabilitation centre in Australia that was extremely expensive and not helpful. So I guess you could say I’ve learned a few lessons. I only made those choices because mainstream medicine was failing us. And it still is. Our daughter is dying. She’s 33kg BMI 11, in chronic pain and all that is being done is more tests and the mention of a feeding tube for life. And yet we hang on to hope. Thanks for being there xx
This is really hard - tragic. I wish with all my heart science could do better to help us. But we’re right at the cutting edge
I’ve been following mothers & daughters in very similar situations to yours on my PID & MCAD forums, eg those who have forced there way through the health system to the best advanced care at tertiary gastro centres with feeding issues multidiscipline teams. am humbled by everything involved
You’re all too aware that patients like us are right at the cutting edge of science. mistakes are made by all involved: medics, patients...this is how understanding advances...eg all the secondary & tertiary gastro investigations i’ve endured over the past 6 years have got me teetering on the edge of tube feeding & ileostomy neither of which make me safe because my primary immunodeficiency disease means these are both ultra high risk. it is what it is
Am wishing you all best wishes ...and thanking you for offering me up so much understanding & acknowledgement & inspiration 🍀❤️🍀❤️
I will never give up hope and positive thinking 😊Thanks you are such a great support x
Am RIGHT by your side 👭‼️💞
Your positivity is incredible.
where do you get this "power" or drive?
Did IVIG help for EDS symptoms if it's okay to ask?
I have to keep searching and hoping for something better for our daughter. All that is promised here in New Zealand is a feeding tube for life. We have a compassionate supply of immunoglobulin therapy and are going to get it subcutaneously either in Australia or USA. am seriously looking into stem cell therapy as well.
💁🏼♀️ I feel same way about ALL of YOU 🤩👏👏👏 it’s you folk here & on my other forums who inspire me & keep me coping 💐💐💐💐💐
Also, my hero husband has v early onset severe crohns (terrible stuff involved: peritonitis + several more big bowel resection ops etc etc) and he copes with this in a very inspiring way...he is also v understanding + supportive about my health stuff
Yes, IVIG is helping to some extent with what i consider my hEDS-related multisystem pain. This benefit is extra important cause i aversely react to all prescrip analgesics (eg am now recovering from hEDS scoliosis instrumented lumbar spine fusion totally without analgesics...and my shingles flared with the neuropathy pain persisting...am sure IVIG is helping with this pain too...but, as i say in my reply below, the IV premeds are also helping!
Boy you and your husband are going through a lot - but together I am sure you are stronger. Great to hear that you have such a support. xx
He is my ROCK...he can’t even begin to understand the science re my overlapping CTD/AID/PID comorbidities inc why their v early onset is debilitating all my body systems so dreadfully, but his decades of severe crohns & crohns treatment side effects mean he comes closest to understanding my intestinal failure and he has been very good about discussing strategy & tactics re navigating nhs gastro investigations etc. But he really is allergic to discussing stuff re all my other clinics..which is why my online support forums are such lifesavers!
He really great thing is that after 4 decades of severe disease, approx 15 years ago his crohns seemed to have possibly finally gone into remission: his gastro team encouraged hum to try coming off daily steroids, which he slowly & painfully managed to do...since then he only needs the crohns meds that keep his lower GI functional despite having had so many crohns-ridden lengths removed surgically over the decades. as a result he has been fit enough to enjoy a retirement full of physical activity (he is a long distance walker, and has planted a native wood around us, which he tends all year. His experiences of immune dysfunction illness have taught me there is always hope XO
I agree with you.
Some people got worse.
This says a lot. . .
I also heard that they also had IV steroid infusion first,
followed by IVIG.
The next would be plasma.
Personally, I prefer the idea of Plasma.
You are not adding anything, potentially
I can’t access plasmapharesis
They say that it only lasts about 2 months.
And, it is invasive.
It is interesting to read EDS patients accessing the stem cell therapy. I get to read that some clinics offering stem cell therapies. These "clinics" are not even medically certified clinics.
I wondered if there's anyone who had it in the UK.
If your daughter has Behcet's, has she been given any biologic?
No she’s not been offered a biologic yet. The prescribing protocol is such that many medications including steroids immunosuppressants 5-ASAs etc all have to be trialled first. The rheumatologist we have seen is also very accurate conservative prescriber. We would also not likely get funding for biologics- approx $2000 a month. That’s the harsh reality of living in such a small country like NZ.
I always have IV steroid (hydrocortisone) + IV antihistamine (piriton) premeds before each of my 3-weekly IVIG session. Most IVIG patients din’t need premeds ongoing, but i am so hyperreactive to meds generally and have proven to need these premeds in order to get max benefits from IVIG. So far there is no evidence of any negative effects from any of these IV treatments: i feel very lucky to meet NHS criteria for IVIG long term: it is helping me enormously
IgG is refined plasma serum:
Here is an excerpt:
Immune globulin products from human plasma were first used in 1952 to treat primary immune deficiency. Intravenous immunoglobulin (IVIG) contains the pooled immunoglobulin G (IgG) immunoglobulins from the plasma of approximately a thousand or more blood donors.
IVIGs are sterile, purified IgG products manufactured from pooled human plasma
What pain medication (s) are you managed on? I am still hoping to get the neurologist to test for small fibre neuropathy with a skin biopsy. Currently all she is on tramadol which is taking the edge off her pain and not causing any constipation or nausea. I would really appreciate any input from you.
At 66 pain meds are now a BIG NO NO for me...they’ve always been a big prob, but as i’ve aged, my reaction to all analgesics except paracetamol (IV & oral) has made medicating any type of pain extremely dangerous. Eg just 24 hours of IV fentanyl last june during&post my hEDS scoliosis spine op on gave me a full on bowel obstruction....and late last year high dose Celecoxib caused terrible side effects, although it helped me survive the entrapped lumbar nerve root that qualified me for that spine surgery in june
i have a lifelong history of being hypersensitive to all prescrip & PTC meds and always hyperreact to them. Which makes all treatments very risky. This is due to my immune dysfunction & connective tissue disorder illnesses + cause i was exposed daily for 5 months in utero to the notorious artificial osetrogen DES (diethylstilbestrol) which my medics think tipped my genetic predispositions over into the v early onset SLE & PID etc etc:
LUCKY for me, some of my daily SLE meds (the prednisolone, mycophenolate & low dose amitriptyline) + my 3-weekly PID IVIG (inc IV hydrocortisone & piriton premeds) ALL do help somewhat to damp all my multisystem pain down. Eg this summer i have had to recover from major spine surgery + terrible shingles without any pain meds except oral paracetamol + my SLE meds + IVIG meds. NB my medics are very keen on paracetamol for me generally eg in june preop i responded v positively to IV paracetamol
But my whole life has been 66 years of studying pain science enough to learn the diff between pain that can & can’t kill me ...AND how to lifestyle/self help manage the multisystem pain i get but can’t medicated due to my hyperreactivity...AND how to find which surgical procedures are worth risking (eg over the decades i’ve had success with pain consultant bilateral cervical & lumbar facet joint OR denervations...& this instrumented lumbar fusion surgery seems to have helped a lot)...so decades of all this help me to tend to cope fairly calmly & strategically with pain of most types....
Saying that, because i am getting older so am prone to even more health ’surprises’, it’s harder to rely on me now to collaborate with medics as effectively re my pain issues...so my immunology chief is arranging for me to see clinical pharmacology re setting up personalised documented pain meds strategies. Fortunately my nhs univ hosp is among the first hospitals to launch on online Patient Portal where all my details should now be more accessible to all medics coping with me
I’ll just add that there are 2 versions of NSAID i rely on long term:
- Mefenamic acid for childhood onset migraine, but only VVVVV short term
- 10% NSAID gel for soft tissue inflammation pain...i use this twice daily pretty much all the time
Generally, i have to shun all NSAIDs, all opiooids, all antiseizure meds, all tranquilisers for neuropathic pain, all forms of CBD - especially now my slow transit dysmotility is SO vulnerable to intestinal obstruction due to analgesics slowing my body systems down even more in the course of trying to give me pain relief
Apologies for such a long detailed reply, but maybe something in there can be useful to you or others here ...i am greatly feeling for you, your daughter & everyone living with our sort of probs long term...this really is hard going XOXO
Good to hear that your symptoms are a lot better.
I hope your medical problems would soon disappear one by one.
💐 THANKS...& Me too: hoping this for us all...wish i could magic 🌈💫 it away for 🦓s EVERYWHERE ❤️🍀❤️🍀
Thanks so much for taking the time to explain the complexities of managing your conditions. It is unbelievable what life is throwing at you. xx
🤷🏼♀️ Am feeling the same about you & your daughter...🤝✊✌️🤞we are stronger together ❤️🍀❤️🍀❤️
If BC publishes a book via SLE hub, I will definitely get one.
One I saw a few months back, published by Oxford ** on EDS. Whilst I did not read it fully (only browse through), there's nothing "new" about the contents. Not surprised, however.
I am in an older bracket, coming to an age group where honesty is necessary as we face more life's challenge. I am grateful that there's a Charity that exists, but it would be useful if they do more in terms of scientific/medical discussions, providing far more treatment options for the EDS sufferers.
To do this, they have to abandon medics, who advocate psych/functional labels on EDS. It's just so UNSCIENTIFIC. Quite frankly, quite childish, even. BC is a model, who defied all this BS "stereotype".