Dear Daniela,

I am so glad to hear that a higher and morefrequent dose is helping your son to
have less suffering on a daily basis. It sounds like he is still getting NOMID
flares with the rash off and on, which is unfortunate.

Do you have a NOMID diary to keep track of your son's symptoms from Dr
Goldbach-Mansky? SHe has all of us that go to the NIH asking our child's
temperature, and rating various symptoms on a 0-4 scale, plus an area to record
other unusual symptoms.

I was wondering if you kept track of his symptoms, and would also suggest noting
more details whenever you see him with more rash, fever, and pains if he was out
in the cold air the night or day prior, or even a few hours earlier, or was
getting over an illness, teething (getting his teeth) or anything like that to
see if there are trends.

I know this sounds odd, but it can help to see if there is a connection. My
son, Seth is currently on the Novartis canakinumab Interleukin-1 monoclonal
antibody drug trial, but used to be on the anakinra.

He rarely ever got the rash once he went onto anakinra, or even with this new
medication, but did occasionally have break-through flare symptoms on the
anakinra, and sometimes on this new drug too.

Before he was on any medications, we had no idea what was setting him off, as he
was constantly rashy and having fevers and other symptoms almost al the time.
But we did notice an increase in symptoms after he was recovering from a bad
cold, teething, or stressed and overheated in the summer.

We did not notice cold setting him off prior to him starting the anakinra or
this new drug. However, once he became very stable on the anakinra, ( on the
Canakinumab now), he has had a few times with NOMID flares after being outside
in very cold wind for prolonged periods of time.

Seth just recently had a flare with a morning headache, nausea and red/pink
cheeks on his face, but no other rash the morning after he was out in the
evening in some very cold winds for an hour. He also got some joint pain later.
I kept him home from school for it, as he was miserable for awhile, but got
better later after hydrating well and resting.

This happened a few weeks ago, with the same scenario when he was out after
school in the cold wind, with no jacket on (boys and jackets-when will they
learn to put them on?) and he had the same reaction by the evening that day.

This is more in line with what people with Familial Cold Urticaria Syndrome
experience, and we had not noted this until Seth was very stable on his
medications. He did not get the normal rash, and only had a slight elevation in
his temperature, and it did not get to the fever point, but the other symptoms
were definately like he was having a mild flare.

As this has happened before with him on the anakinra too, and now on the
Canakinumab, I am not sure if we will ever have him totally free of this
happening.

This occurred last year on the anakinra when he was out in the ocean swimming
(cold here) and again after being out in the garden in the cold air for a long
time. I don't keep him from things, but realize it could be much worse. His
flares are not too bad as compared to the past, and he is realizing that if he
dresses warmer he can prevent this from happening (except the swimming, unless I
get a wet suit for him.)

His labs are excellent right now, and his MRI and spinal tap too, and are as
good as they were on the anakinra. I need to talk about this with his doctor to
see if they want to do any adjustments, but it will be hard to convince them
unless I can bring him in right after this happens! I need to keep tracking
things for a bit to prove it is cold triggered, as it has only happened a few
times this year, and also last year on the anakinra.

When Seth first went on the anakinra at 4 1/2 yrs of age, we had to re-think
things and the diaries helped to better understand when he was having flares on
the anakinra, since he did not get the rash with flares anymore. The rash used
to be a big indicatior of his flares increasing, so we had to look closer at
other things.

To me, it sounds like your son is still having some breakthrough flare symptoms.

I hope that this helps you, and that he can feel better all the time soon! I am
so pleased to hear he is sleeping better, and enjoying himself more. Please
keep us updated on his progress. I hope that you are doing better now too, as
it is so hard to have your baby suffer.

Karen Durrant


--- In nomidsyndrome@yahoogroups.com, "daniela.spalt" <daniela.spalt@...>
wrote:
>
> Hello to everybody!
>
> I want to give you an update on Jan and ask for your help again:
>
> Jan is on 6 mg Anakinra at the moment. We need to inject him twice. Since then
his rash has vanished and he has improved a lot. He started to discover his body
and can sleep when he is well.
>
> Unfortunately he still has bad hives. They seem to develop every 10 to 14 days
- which is still not satisfying as you can imagine. He is very unwell for one
day then (fever, pain, eats and drinks little ...), paracetamol helps him for
2-3 hours.
>
> How often do NOMID - hives occur in your children and how long do they take?
Every 10 - 14 days is too frequent, isn`t it?
> Could it be that his hives get less when he gets old better?
> Do you have an idea about how we could help him better?
>
> I`m grateful for any advice, perhaps you have made the same experience???
>
> All the best to everyone - esp. to the kids ...
>
> Daniela
>