“So, I’ll see you at four”.

He seemed unimpressed when all of the lackey doctors fell about laughing and I knew I would pay for it the next day.

We didn’t get discharge until 2.

The ENT doctor came in earlier and advised that she stay away from  all social activities for five days.

When I told him she had none, that if we went to the supermarket, she came home with pneumonia, his response was;

“Well, that won’t happen anymore, I’ve cured her”.

Ahhh, yes.

He has a very healthy ego.

I’m sure he said the exact same thing the last time he operated on her.

but

in a bold move of unnerving positivity, I put both Noah and Ivy’s name down for preschool next year.

Next week Ivy goes in for her first infusion of IVIG

and so my plan for normality begins.

It’s tomorrow!

Wish them luck. They are two of 700 applicants for dance alone!

Have fun Immy and Maddy!

Today we went to the paediatrician.

Ivy has lost just over a kilo.

Her cortisol levels are out of wack.

Her iron stores are low.

Her chest x - ray looks better, the pneumonia is clearing.

Her kidney functions are still okay.

Her  left ear is discharging, her other is infected.

Her white cell count still high.

She is still sick.

But

it’s time to move forward.

We are going back to the immunologist on the 22nd of July.

After that we are hopefully going to start IVIG or Intragam as it is commonly known.

Before she has her operation on the 7th of August.

What is IVIG?

It is a blood product, a plasma that is rich with antibodies. Ivy will have this infused into her body every four weeks. It will help to boost her own immune system and with any luck she will be able to have something of a normal life.

Sure, there are risks.

There are with any blood product

but

She might be able to go to pre school next year, without fear of catching every nasty bug under the sun.

She might be able to go to play group, go to a play centre, a shopping centre, a doctor’s office, visit friends and not walk out with some disease that might not have made the carrier sick but most certainly would Ivy.

It might mean less of the hospital and more of the sunshine.

I’ve never wanted anything other than to see her healthy.

I’ve never wanted anything more than this. 

This is our chance, I think.

This is her chance.

Ivy was well (touch wood touch wood touch wood).

Her chest was clear.

Her stomach not too tender.

Her ear looked much better. Still not fabulous but he said he could see a clear spot.

Her bottom was clear of any blisters, clear of any trace of the pemphigus, just a small amount of scarring.

She didn’t have to have bloods this week.

Her blood pressure was ok.

Her heart rate was ok, no sign of a murmur. Still a little high.

She actually smiled at him and gave him a hug when it was time to go.

She wasn’t scared for the first time in weeks.

So now what?

Now it’s time to start reducing the Prednisone again, now it’s time to  push the Micophenolate up.

Micophenolate is the immune suppressant that she trialled in hospital. It has lots of neat side effects too.

Now it’s time to reduce the antibiotics.

Now it’s time to upset the apple cart, take chances, take risks, find a balance, play with fire.

And we have a plan (of course we do).

For when she gets sick

and for a new doctor, an endocrinologist

and for another visit to the immunologist.

Am I nervous?

You bet I am, after the Dapsone, I am down right scared.

For now, though, I am going to bask in her wellness and try not to think about the  what if’s or the next time.

Today, I am just going to enjoy her.

We met with the guru of all things blistered. She asked us some questions. She took lots of photos. She made some suggestions.

Our heads did spin with possibilities.

What it came down to, what is our (limited) understanding and what has been confirmed this evening is this;

If an immunoflourescence (direct) comes back positive for pemphigus then it is pemphigus. Just because it presents differently from the common form does not mean it can’t be pemphigus. Nor does it have to be something else just because the Ig’s are different from typical presentations. She didn’t come straight out and say the other derm was an idiot but she did, if you know what I mean.

David, Imogen and  now, Ivy, all have confirmed ectodermal dysplasia. Not just any ED but a type that also causes,

among other things, pemphigus. Considered genetic and not auto immune.

Among other things heart abnormalities.

Among other things gut problems.

Among other things immune deficiency.

We were going through the history. Ivy history and family history and we were not getting far. Then the good doctor asked about William and when she discovered that he died from an aortic stenosis, everything fell into place. Like the final piece of the jigsaw puzzle. My William - the missing link.  Literally and physically.

I have said to the doctor’s all along that she presented to me like Immy had all those years ago. Today I cried tears of relief, of validation, of hurt for my baby girl, past and present. I cried for her future.

I cried because when the doctor’s told us that everything had come back ‘normal’ they didn’t actually mean that. Her IgG was low, she had IgE’s all over the place and her IgA was not normal either.

For Ivy, the queen of all things genetically bizarre, it means a few things…

It  means she will possibly have issues with skin, teeth, hair, nails and temperature regulation as part of the ED

It means that we can treat the pemphigus with a mix of prednisone and dapsone. We just have to iron out a few creases;

* that she does not have a missing enzyme, which would cause the dapsone to make her very unwell

* she is anaemic already and the medication can make it alot worse. This will need to be treated.

* her zinc levels are questionable also, so these need to be addressed.

It means that she will need further investigation, with the possibility of having IVIG  or the need for IV antibiotics when she becomes unwell, as she has a questionable level of IgG deficiency that some would treat and other’s would play the watching game with.

It means a label, a box, a place to belong. If that seems weird then let me explain; I can tell doctor’s she has this…thing…and they will listen. They will treat her accordingly, instead of putting  her in the too hard basket and patting me on the shoulder like a neurotic mother and asking me if she is my first. A diagnosis is everything when you need help.

We are going to see the paediatrician on Monday, so hopefully I will have a better understanding then but for all of our lovely people out there. Our friends who have seen us up to this point, I wanted to tell you what we know so far. I wanted to include you in this because without you all it might not have ended so well. Some of you have been with us in body, some in mind and all of you in soul, to which, I am truly grateful.

I’m going to Sydney to take Ivy to an Immunologist.

It seems that when you stand up for yourself it earns you the right to be fast tracked.

I’m not quite sure how it happened. My head is still spinning.

David is skeptical. He thinks the paed has an agenda. Whatever. So do I;

To get my baby well before Winter.

My hectic social life (can anyone hear that sarcasm echoing in the background?) will have to take a back seat this week.

Today we will meet an immunologist for the first time in our journey to Ivy’s health.

We have to go to Westmead Children’s Hospital; the hospital that William died in. The hospital that induces a panic attack everytime I think about walking in there. The hospital that whispers of hope and yet…and yet snatches it away all too quickly.

If this is some kind of weird cosmic emotional test, please let it play out well.

Damn, where are those crazy pills, now?

The paed gave us directions to the clinic. When I say that, I mean it went something like this;

Paed: (please read with almost no breaths in between words and very fast) “What I remember of Westmead is that you walk through the doors and it’s big. Very big. You turn right and walk up the corridor, past a coffee place… I think it’s a Starbucks. I can’t remember the exact directions but I can remember the type of coffee they sell, how wrong is that? Says alot about my life. Anyway, you turn right after the Starbucks. I think. If that’s not right, there is an information desk in there somewhere”.

Me: (please read in a very slow, country bumpkin way) ”Yeah, r i g h t… thanks for that”.

Note to self: Do not ever, ever ask a doctor for directions.

On Wednesday or Thursday we will go to see the paed. I need to see him. I want to address trust issues, make sure that everything is… not too weird.

Dave and I have had huge discussions on the paediatrician’s personality type (a hobby of Dave’s) and how this would be best addressed. I just know that I need to work out if I can trust him anymore because when it comes down to it, if there is no trust, there is nothing.

The following Friday - not this one, it’s Mum’s 60th 15th birthday. Think it through, my friends. Have you worked it out yet? Anyway, that’s another post. The following Friday we are off to meet the leading expert in Australia on pemphigus. What’s more, I think she is even more excited to meet us than we are to meet her!

Can I just pause here to say I really appreciate the paediatrician when he is organised.

Someone commented that perhaps we could send some ‘balls’ to the paed… I think Australia Post is on it!

My mum knits yellow booties.

She started knitting them after William died. She doesn’t do it for money, she does it because she loves me and I asked her to.

When I was trying to conceive I read about an old wive’s tale. It went something along the lines of;  a pair of yellow booties knitted and given to a couple trying to conceive, would bring them good luck in fertility and a pregnancy would occur soon after the gifting.

Pffft, you might be saying, that’s stupid, just a myth… but is it?

The first pair of booties she knitted was for a friend, who was struggling to conceive.

She fell pregnant the month after they were given to her. When she and her partner started to try for number two, the booties went straight into their pillowcases and now another baby is on the way.

She has knitted about a dozen pairs now, all of them resulting in beautiful babies. The second last pair she knitted was for a friend who had already done three cycles of IVF and nothing had worked. I knew she was very fragile, not knowing whether to try again and so it was with trepidation that I gave her the booties with the story attached.

I didn’t see her again until late last year, with her daughter.

She came up and gave me the biggest hug and said that they had taken the booties home and slept with them leading up to the forth and final cycle. When she went for the transfer of the embryo, they were in her handbag and in there still when she got a positive blood test. She said they brought her hope.

Maybe that’s what it is. Maybe it is the symbol of hope, these tiny little yellow booties that brings about a mindset, a little bit of magic.

You want more proof?

Still not a believer?

The last set of yellow booties she made were given to me on Mother’s day, 2005. Not one pair but two, presented in a little box.

On June 1st, I discovered I was pregnant again and about two weeks after that, I found that we were expecting twins.

Got gooseys?

I always get them when I think of all the babies that have worn mum’s yellow booties home from the hospital.

Now she’s knitting them again because there are new friends who need to hope, just a little.

I think the Prednisone is working! I’ve updated the photos if you want to look.