Primary immunodeficiency disorders

Hi, maybe these will be of help here and here. Excuse the sheer garishness of the last website, maybe you'll find something useful on it though! You may have seen those already in which case I apologise - what level of information is it you're looking for. The former website seems to have a pretty thorough resource section at "learn about primary immunodeficiencies". If this isn't the kind of thing you're looking for please let us know, I'm not sure what level of information you're shooting for.

As for Ireland-specific information, I'm afraid I don't have much.

Usually these things are diagnosed by a canny practitioner suspecting someone who's too sick for too long with various infections and the patient is sent to an immunologist. I'd imagine you'd be heading to a bigger city hospital for that and probably also to receive the treatment (which might be immunoglobulin transfusion). If the patient's old enough it is possible (at least in the UK) to teach them to administer the immunoglobulin themselves subcutaneously (much like diabetics must do with insulin) which is often great because they can give themselves smaller doses more frequently, which avoids the peaks and troughs in getting large doses in the hospital once every few weeks. It also might make life handier not having to travel to a hospital I'd imagine! This is if immunoglobulin is needed in the first place.

cyning wrote: »

Thank you... That really helps. I guess what I'm looking for is anything: I have an almost 15 month old who has needed 7 hospital admissions for infections, constant viral infections, constant bacterial infections: even when on a prophylactic antibiotic. She has also ended up quite sick after every vaccination requiring hospitalisation after two of them (pneumonia after bcg, severely neutropenic after 4 months).

We have been referred to a paedeatric immunologist but even privately there is up to a nine month waiting list. Hopefully won't be that long, but I need to have an understanding of it so I know what questions to ask.

Wow, that's a frightening waiting list, I can't imagine how frustrating that is. Hopefully once you do get seen things will move quickly!

It's often difficult to tell between sicker-than-normal children (very common) and primary immunodeficiency (quite rare) so primary immunodeficiency is very often missed and can go on until it's eventually recognised in adulthood after years of misery - so it's great if it can be picked up and treated early in life.

An immunologist can do further assessment of the problem by looking at the various components of the immune system in the blood (complement proteins and immunoglobulins), as well as looking at things like family history. GPs should be careful interpreting immunoglobulin results to rule out primary immunodeficiency based on immunoglobulins as an acute illness can push the immunoglobulin levels into the "normal" range. Things like this is why referral is best if immunodeficiency is suspected.

Usually you've to be careful with live attenuated vaccines (MMR, BCG and varicella, etc), because while most people's immune systems could deal very easily with the weaker virus, people with primary immunodeficiency may find it a struggle. This is also the rationale as to why vaccinating as many children in a community as you can is so important as herd immunity can then protect the very few children unable to tolerate the vaccine, and why anti-vaccine groups annoy doctors so much...

Blogs can also be a good source of information and what life with a condition is like (the one I linked was just found with a quick google search - it could well be rubbish). But beware the information on them isn't always right and sometimes the truth is blurred by personal bias! (Also worth noting - people in blogs might not have the same condition as your child - primary immunodeficiency is an umbrella term for a number of conditions!)