Testing for c1 inhibitors ? angio odema

[AuntieMonica]

DD (3.11yrs) has had 2x anaphylactic episodes in the past 4mths, has very dry skin, occasional eczema, gets extreme prickly heat (10 mins exposure to sun on her neck made her scratch to bleeding) amongst other minor (ish) ailments.

We attended our 1st Allergy clinic appt yesterday, where she had the prick test for grass, cows milk and wheat allergy, and these were clear.

He did that test where he ran his nail up her skin, and it came up red and lumpy, instead of just a white mark like it should do....(i'm sure there's a medical name for this...)

They've said it's important we keep the epipens close at hand, that it obvious she is 'allergic' but not sure why/what to.

I asked him what the consequence of this test would be, and he was very vague......said there may be a treatment that would help her. I've googled and trying to unread what's out there IYGWIM.

The consultant now wants her to be tested for C1 inhibitors - C50 incl and wondered if any other parents were about who've been through this too?

Was the consultant just being really thorough in trying to find an allergen or am i now a google-victim?

Hope someone can help me here

bump

another try.....

no expert, but is angio-edema where there is like internal tissue swelling? Have you considered wheat intolerance (not allergy)?
I knew someone who went gluten-free and the swelling gone away. I am sure I also read about it on gluten free forum a while back.

The bugger with allergic and anaphylactic reactions is that they are not always caused by the same things and don't always manifest in the same way. It's incredibly complicated.

I reckon if your consultants thinking out of the box and not just chucking epi-pens at you and saying come back in a few years then you've got yourself a good one. I agree I think angioedema is tissue swelling, and it sounds like your consultant is being sensible and thinking through all the possibilities.
My DS is a tricky one too - he's now deemed to have food alelrgies, pollen allergies and to be idiopathically anaphylactic as there are situations where we just cant work out what happened to him. When my DS' consultant's not had time to answer my questions fully I've gone to my GP and talked things over, it's really helped me and kept my GP on-board and in the loop.
If it's any consolation, your daughter is safe as long as you have the epi-pens. And until they work out what is wrong you can keep her safe. I'm sorry it's not easy. Good luck and tell us how you get on

thanks for these replies, have to go collect her from nursery now but will be back later

we have found that in hereditary angio-edema (our case) that DHEA was out of whack -- far too low. Taking DHEA as a supplement monitored by regular blood tests has greatly reduced symptom occurrences and has stopped the near death swelling.

Few doctors understand HAE, C1 inhibitor testing and treatment is another approved treatment

Hi Auntiemonica,

My DS1 also has chronic ideopathic angio-edema, we have been tested for C1 esterase deficiency and C50 amongst many many other blood tests - he does not have hereditary angio-edema, but we still have no explanation.

It sounds like you have a consultant who knows what they are talking about and hopefully you will get some answers soon. The test you are talking about is dermographism and good old wikipedia explains it nicely.

Hi my DS has the same allergic symptoms, including the welts on skin being scratched. 2 seconds it took when the GP tested Monday! He also tested negative (RAST) to tomato, grass and peanuts.

He was tested for angioedema (C1 test) and it was negative.

My DS also is very similar to texas DS. Goes to show that the same symptoms can have different causes.

Best of luck in your journey for some answers.

the docs have just ruled out HAE with our son and have come up with some other explanations. its been a long and difficult year so far!

Sorry for hijack - what other explanations eragon ? Might help us on our quet for an answer - thanks in advance

well , apparently there are many different types of anti histamine and his may be having some war, so is on diff combination of anti histamines are being taken at the moment.

also back to the drawing board and having food challenges of mystery poss causes of epi level reactions.

who knows what they really sort out and pin point to the problem!

make that 'different types of histamine ' not anti, am tired its been a looong day!

ah thats interesting eregon I'm watching with interest too as we are no further with DS allergens/ allergies. He seems generally better now than previous years but still has sudden reactions.

Hi Eragon

Just wondering about this as my mum used to get idiopathic urticaria with tongue swelling and breathing difficulties but fortunately, once she recognised the start of a reaction she would dose up on piriton which would stop it developing into an anaphylactic type reaction. I saw her have a couple of ana ones and I can still remember how awful they were. It explains why I still(after 4 years) dont deal with DD's allergies so well in terms of my own anxiety :(

My mum was never prescribed epipens but was told to take 1 piriton everyday for a while. She was on this for about 3 years and touch wood has been attack free since. She does now have Oral Allergy Syndrome and asthma so always has her meds with her.

Can a person have both allergy induced angioedema and HAE or is it one or the other?

With HAE is it a case that the body produces too much histamine ? or is it still external factors that cause histamine to be released?

I remember reading in a paper on the AllergyUK forum written by a guy who suffered with too much histamine in his body, who suggested that Vit C supplements can help 'mop up' excess histamine but not sure if that would help in cases of HAE?

OP, sorry to butt in on this thread. I hope you're DD is okay and that you are too. Sounds like a worrying time for you, but it does sound like your consultant is in the know about all this so fingers crossed they can help you manage it.

no HAE is not related to to the allergy side of things at all. it a genetic disease that passes in family, most commonly people find out they are carriers after someone dies from it in the family. Has different emergency meds than adrenaline injectors.

sounds like your mother is lucky to still be here, why hasnt she got epi pens now?

living on piriton antihistamine every day is old fashioned advice these days, should be on a non sedative one, as it will have less side effects, not just the sleepy feeling.

on the vitamin front, vit D is very low in allergic people, so my son is on vitamin D after a blood test . Have read up on vit D and have asked everyone in family to take it!

I have used the epi 3x on son, and am so glad we have them!

Hi Eragon,

I give my DD and DS vitamin D most days. I am also very low in it and like you read up on it and am completely convinced that we should all take it.

I read somewhere that people who burn easily probably have inadequate levels of Vitamin D. I am indian and in the last few years I used to burn easily, even with my colouring and was conviced that the sun was getting stronger.

I had my vitamin D measured last year as part of my hypo thyroid problems and my GP said my results were the lowest he'd seen. I had a Vit D booster injection and was on prescription vit D for 6 months. The consultant at the hospital told me to take it for the rest of my life which I will do. The GP stopped prescribing it as soon as I reached the right levels and seemed surprised that the consultant had told me this. He was also confused as to why the consultant had given me the blood test in the first place so I suspect GP's don't really understand the benefits of this.

I asked at DDs allergy appt if they would test her vit D and she came back 'slightly low' so they said there was no need to do anything. [sceptical] I started giving her some drops anyway as we'd had no summer, were going into the latter part of the year so figured there wouldn't be many opportunities for her to get adequate sun exposure.

Can I ask, how much do you give your DC and how much do you take? Also, have you seen the spray versions? Apparently they are more effective as they don't get affected by stomach acid but I've not tried them yet.

Thanks for your reply by the way and hope you and your family are having a lovely long weekend. :)

Take care

We have just brought a supplement from the supermarket, one with states 200% RDA for son,(takes one a day)

my daughter who cant have milk is on a calcuium and vit D which provides 100% RDA (takes 2 a day)
she also has a small glass of calcuim enriched orange juice just to make sure the calcuim is ok.

As a middle aged woman i take a cocktail of supplements! I take evening primrose for my painful near menopause boobs... and Glucosamine sulphate for my crunchy , painful knees. the vit Dplus calcium and multi vit and iron.
i am on a ibs exclusion diet so am not eating a wide ranging diet, low in wheat and milk, and only eating 3 types of veg and 3 types of fruit. its all quite grim really.

Gosh Eragon, that sounds tough :(

I hadn't heard of calcium enriched orange juice. That sounds like a good thing to have.

I have crunchy painful knees too and used to get alot of joint pain but found that getting my Vit D levels up was a really big helped with joint pain. My knees are better but still a bit dodgy.

It sounds like a tough time for you at the moment esp if you're also on an exclusion diet. I hope you're ok though. Any more plans to do any more radio works again?

Take care and hope you've had a good weekend. Big Hugs to you all. xxx

you know, i forgot to mention the probiotics i take, they really seem to help with the nasty ibs.

am hoping to do some more radio stuff in the future but have no set dates yet. am also hoping to help more with the alex curtis trust which is really moving on a pace these days. very exciting stuff going on at the moment!!

That's great Eragon. It's good to hear the Alex Curts Trust is doing well and hope you enjoy the radio stuff. :)

I have just discovered this thread, so will bump. Wondered if the OP was any further forward?

I have severe HAE (Hereditary Angioedema), as do 4 of my dcs (varying severity). As said, it is unrelated to allergies or any type of anaphylaxis, being caused by a deficient or non functioning protein and not helped by meds to treat those conditions. Due to its rarity, it often goes undiagnosed or is confused with other conditions, if your area/hospital doesn't have an immunologist/specialist in HAE you can really struggle.

I am under a fab consultant, one of the world's leading specialists of this rare condition, she is very progressive and is great for the dcs. I have been managing our treatment under 'best practice' for many years and have considerable knowledge of HAE (type I in particular) self treatment (home therapy of C1 INH), children's treatment etc so if anyone has any questions/worries please feel free to message me.

See also haeuk and we have a very friendly, helpful and supportive HAE facebook site you can be added to.

footphobic

thanks for that post and link. we are still visting the royal hos in london as still waiting for test results to come back, just to finally confirm that he doenst have this condition.
can you re-post every so often on mumsnet, esp in childrens health and special needs?
am sure more parents will need support with this.

thanks again.

Yes I will eragon. I hope you get some answers for your DS. I see the immunology team at the RL (though we live Norf/Suff border) and the same team plus a paed cons with the dcs and they are world leading in HAE knowledge and care. I have no experience of other depts there but I hope they can help you.