You would think I would learn a lesson…

When you tell the universe you can’t take much more of the coughing and snot, the tears and grumpiness of one child, you expect a little reprieve.

Well, I do, anyway.

Instead the universe smirks at you in that ‘you’ll be sorry you said that’ kind of way and throws not one but three more sicky kids your way.

One with an ear ache,

and two with wind and weather induced asthma

and another who is threatening to come down with something.

All I can say is thank goodness the boys come from healthier stock.

The ear ache is easy, panadol, warm, comforting things, with the possibility of a visit to a doctor and antibiotics in the next 24 hours. In the mean time, TLC, in metered doses.

Can I just say, right here and now, that I hate asthma?

It starts off innocently enough - in Noah it usually starts with a tight cough which then becomes a wheeze and a cough. The ventolin puffer comes out but it quickly moves to the nebuliser and then the inevitable Prednisone.

*sigh*

One child on pred is not nice but it is doable.

When Noah is on prednisone, he is, how can I put it delicately?

A monster!

He is aggressive and loud and cranky and loud and moves a mile a minute.

Did I mention loud?

My sweet little guy turns into… Damien (you know, from Omen?) I have to stop myself from searching for the triple six in his hairline.

Ivy is the brunt of most of his aggression but we all cop it. Except for Dave, he can do no wrong. Noah hero worships his Dad, and rightly so, he has done most of the nebbing over the weekend.

I had to start Maddy on the ’sone today. It’s been a while since she’s needed a course and to tell you the truth, I’ve forgotten how she reacts to the steroid. Guess I’ll find out.

So, Ivy and Noah are a bad combination on Pred. I’ve known that for all of their Winters that have gone before this but this time, it seems especially…violent. no matter what I do to keep them apart, well, the image of two billy goats butting horns springs to mind when trying to describe them. Add to it insomnia and you can imagine, can’t you?

All things said, Noah has recovered quite well. Saturday was a bit touch and go, we had to push his ventolin right up to 2nd hourly and there were a couple of one after the other nebs. Today he looks like he has gone a round or two with a world champion boxer, the circles under his eyes are so dark but he is in good spirits and his wheezing is settling.

It can only get better, can’t it?

Please, placate the crazy mother and tell me it will.

Also, you have to remind me not to keep daring the universe. She has a mean streak.

She must be on Prednisone.

Here is a picture of Ivy when she started the prednisone

.

This was about a month in, January, weighing just under 12 kgs

This is Ivy at the beginning of May.

Weighing it at 15kgs.

See her face, the roundness, the loss of her neck? It’s known as ‘moonface’ and is a typical sign of Cushing’s.

I know, what she really needs is to get off the Pred but that is not an option, right now.

She also has;

rapid weight gain (obviously) but only on her trunk and in her face, it’s not all fat, it’s mostly fluid,

increased hair growth on her body,

joint pain,

and fatigue

and it’s getting worse.

With each day that she stays on the high levels of prednisone, the disease progresses.

It puts her at all kinds of risk for other things too, like stroke, heart attack, diabetes and brittle bones.

She is displaying symptoms of diabetes, with increased thirst and increased urine output.

That is why we need an endocrinologist.

Now.

Not in five months.

We got our letter today, giving us a date for our first appointment for the 22nd of September.

Am I the only one who thinks this is not ok?

What is your best bet, for a waiting time, my friends?

Six weeks?

No.

Try six months!

That’s right, six months, that will see us just about up to Ivy and Noah’s 3rd birthday

in November.

The paed’s idea that we see this new doctor before we see him next, at the end of May, is one of pure fantasy. In fact, I think I heard the endo’s receptionist fall to the floor in a fit of hysterical giggles, when it was mentioned.

David was told, in no uncertain terms, that we could not actually make an appointment, that Ivy’s referral would be sent to a booking commitee (yes, you read it right) and we would then be allocated a time and date to which we would attend.

Bwa hahahahahahahahahahahahahaha!!!!!!!!!!!!!!

OMG, where are we living, people?

This insane life that we lead just got a little bit crazier.

Of course, Ivy is not a high priority.

I mean, what could possibly go wrong, with a two year old, on large doses of prednisone, in the next six months?!?!?!?!

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.

It was a plan, some pathology results and a script.

That’s right, my friends, you can doctor a patient via fax now.

Don’t get me started.

Yes, I am disgusted.

Anyhoo, the plan; was to wean Ivy off Prednisone because she is at risk of diabetes and brittle bones and a plethora of other nasties the longer she stays on the pred. Our alternative is a drug called Dapsone.

Never heard of it?

That’s because it is not widely used these days. It is mostly prescribed for Leprosy (We don’t call it that anymore, we call it Hansen’s disease because that just sounds so much better).

Dapsone can also be used for other skin diseases, one of which is pemphigus.

The paed has sent a general information sheet about Dapsone, which tells me very little about what my baby girl is about to ingest. It doesn’t matter though because I have researched it and have all that I need to know that this is one scary drug.

Lets throw around a few terms I have learnt about today, shall we?

Yes, let’s.

Agranulocytosis. This means a deacrease in granulocytes or leucocytes or in layman’s terms, it reduces the number of cells that can fight infection. Great for someone who is immune deficient, wouldn’t you say?

How about, aplastic anaemia, which is the decrease of red and white blood cells in the bone marrow or dyscrasias -  the abnormal mix of blood lymph and bile in the body?

They sound like crackin’ side effects,  don’t you think?

Then there is hemolysis which is the disruption of the red cell membrane, causing release of haemoglobin. This means that the red blood cell essentially explodes and cannot carry iron anymore and it has a great sidekick: methemoglobin, which means the haemoglobin can no longer carry oxygen.

It sounds good.

A real easy sell.

Add to that the risk of liver disorders, nausea, vomiting, abdominal pains, pancreatitis, vertigo, blurred vision, tinnitus, insomnia, fever, headache, psychosis (now that’s an extra special one)  and a whole lot of other exciting things, including carcinoma and I am really starting to see that this is a wonderful medication for Ivy.

Not.

So, what the paed is saying, is that this medication is the better choice over Prednisone and all I am wondering is how he can say that?

Just one last thing, if you are a ‘learning doctor’ out there, or even if you are an ‘already doctor’; If you say you are going to be in contact on a certain day, be in contact.

Don’t send through a fax and think that will be adequate. Because it won’t be. Especially if the patient involved is little and is about to take Dapsone and has any parent worth their salt. It will just make them angry.

Very angry.

Very rare they say, very complex.

Can anyone say understatement of the year?

They talk of DNA testing and OPG to confirm and publication and recognition of their ‘discovery’.

But what of the little girl in all of this?

Now she is sick, does her diagnosis change anything?

 No.

More antibiotics. More pain relief and *sigh* more blisters. Plus an over the top, over active prednisone induced zombie of a child.  I know that doesn’t make alot of sense, stay with me, visual coming up.

Cranky, confused, sleep deprived baby.

Not fair.

One minute she crawls up onto my lap in misery, searching for sympathy in cuddles and kisses and the next she is scampering away to spin and dance to the beat of the prednisone god’s ever compelling drum.

I am tired just watching her.

I guess it’s good she is upright but is it because of the pred that she cannot let her body rest?

She did not sleep last night and made very sure that her parents didn’t either. 

Moaning and groaning and rolling about our, now seemingly too small, queen size bed. (Too small because the Queen likes to sleep in a starfish shape).

*Bless her cotton socks*.

Her breakfast consisted of (over the course of two hours - I didn’t hit her with it all at once); liquid iron (ironically based in  sorbitol - a laxative), liquid zinc, vitamin C, Bactrim, EES, Prednisone, Singulair and two bites of a piece of pineapple.

A cocktail.

Pina wedon’tknowwhatelsetotrysowewilljusthityouwitheverythingandthensome.

The paed offered me up a hospital bed with all the dressings, on a silver platter but I turned him down. Now I am thinking maybe we should have gone.

Maybe .

I also know that when you take Pred induced children into a hospital setting you get mean, sideways glances from parents of limp, sickly, bed ridden children and throw away comments of your child not looking sick. Oh, if only they knew!

 And, providing she can hold her own, I have been given release from the crazyhouse home tonight to take Mum to see Billy Elliot.

Is that wrong to want her to hang in there just one more day, so I can paint the town red?

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.

These are strong words, my friends and if they are too painful or heavy then, please don’t read. I need to write my thoughts down and push them out into the night.

I am not writing for sympathy. I don’t need help. I need to work through all of this.

I am writing so that I can process my feelings…

When William died, I wanted to die.

I wanted it to be me.

I literally could not see past myself.

I didn’t want to see the other beautiful children who were waiting for me to pull myself together. Of course, kids have a way of pulling you back to the present. Their life force buoying me up when I felt like sinking.

For almost four years I have been trying to get a grasp on the grief I feel for a baby who is not here, who I never really had the time to get to know. I failed dismally, of course and so I went to the medical profession for help. My GP put me on antidepressants after I lost the plot last October and I started to see a psychiatrist.

The GP moved and the psychiatrist sucked.

She wasn’t interested in teasing out the knots of grief, that had gone unattented for so long. Her answer to my weeping was to push the medication up further. All my own attempts at dealing with having William’s blood on my hands had not worked as they relied on my obstetrician to help me.

So.

Now I am off the medication. For whatever reason, it doesn’t really matter.

Have been for over a week and I am not feeling very good. Somedays, yes, I will admit it, I have been feeling… ’suicidal ideation’ creeping back into my being. I don’t like that feeling either.

I have cried buckets. I’m not ashamed to say that.

I’ve cried because;

I cannot trust any of the doctors and

I do not trust my own self.

This is so complex for me. It has everything to do with William and the choices I made for him. In his birth and in his death.

When you have to turn off the ventilator that is sustaining your son’s life, it stuffs up your own for what seems like forever and it becomes part of who you are. Seeps into your everyday.

 Now I am second guessing myself with Ivy.

I hate it.

I want to be confident in her care. I want that confidence who whispers in my ear that my gut feelings are right, that I am doing everything I can for my girl and what I am doing, is great.

I want to jump up and down and tell these doctors to do a better job.

I am her mother, for goodness sake.

But I can’t.

The doctor’s don’t help, with their mistrust but when it comes down to it, it’s me who doesn’t believe me.

That little voice, ever steadying, says, wait, maybe you are wrong. Maybe what you want for her is the wrong thing. Maybe your gut feelings are wrong.

Yesterday was a low point.

Lower than last week. Whether it was because the last of the crazy pills had left my system or because I felt as though we were not getting anywhere fast and I finally lost the plot or both. I was a howling mess of self pity.

Today is a new day though and the sun is out.

So what happened yesterday?

I got in the bus with the toddlers.

On the way to the appointment, Noah puked up his breakfast in a fetta - type pile of cark sicky goodness. Word of warning, my dear friends, do not feed your child seed bread toast after a nilla shakey and then pile him in the car for an hour long drive. This is the third time in as many months of early morning appointments that Noah has protested. No more sitting up the back of the bus for that little guy.

I kept on driving though until we found a parking spot.

I changed the boy and stripped the carseat… or was that stripped the boy and changed the carseat???

We got there and waited.

About an hour later the paed turned up and we were invited into the hornets nest of dermatologists.

Ivy had a small erruption of blisters on her bottom, which I exposed to… ooh, lets say, half a dozen derm residents and registrars, two medical students, the paed and the head honcho, King DermGuy.

They bantered around what is was, what it could be, what they would do.

Hello?!?!?!?!

Parent here.

Talk here!

But No.

After a lengthy discussion, that didn’t involve me at all and only briefly involved Ivy’s bum, it was decided that they would do nothing.

Yep, you heard right.

Nu - uh - uthin’.

Let’s pause here briefly to go over the short history leading up to this appointment…

1. The paed would not alter Ivy’s meds without us seeing this guy.

2. We were told that once we had seen this doctor, a decision would be made and Ivy would be treated accordingly.

3. Seeing this guy is easier said than done.

It was decided that we would keep reducing the prednisone and induce a blister attack! (Because the blisters that were already there were not acute enough). When she was all blistered up and in pain they would do another biopsy.

Huh?

I guess it’s a decision.

David and I have been scratching our heads and wondering why.

The last biopsy she had on her bottom was on Christmas Eve. They said it was perfect and that we could not ask for better.

The last biopsy she had has left a giant keloid scar.

The last biopsy gave us a diagnosis of pemphigus.

Why is all of that not good enough now?????

So here we are, waiting for our baby to blister and it feels wrong, so very wrong.

So there it is. The ugly just got uglier.

On Wednesday he phoned David and among other things, told him that Ivy’s colonoscopy showed a thing called melanosis coli.

Now, before you go and google that, you should know, that everything that comes up talks about laxative abuse.

Yep. Laxative abuse.

As you can imagine, for the last couple of days, all hell has broken out here.

I have cried a million tears. I have been angry and hurt because I thought that the paed knew me better than that and I thought my  husband most certainly did.

It has been like a big slap in the face.

No one directly accused me but it was there.

Hanging in the air.

I guess though, when you can’t make a diagnosis, it’s easier to blame the mother.

I still feel sick just thinking about it.

Anyhow, I’ve just spoken to the paed, challenged him and told him the distress he has caused and he has discounted laxative abuse, right off the bat.

Thank God for small mercies.

Why he couldn’t tell David that is beyond me.

He said that it can be caused by a whole lot of  other things and she would need to be tested for something in particular, that I can’t remember the name of right now because I was so cranky. Something granulosis.

We have started her on a gluten, wheat and lactose free diet. On Monday we are going to do a ‘big shop’ of all things free of wheat, gluten and lactose.  At the moment it’s just Ivy but come Monday we will be eating…ummm, not sure what.

I need help for good recipes, so if anyone out there is eating gluten free, throw me your tips and ideas, okay?

We have had a really quiet day today because the erythromycin has been causing her to vomit and I am now worn out from being upset with the paed.

To leave you on a happy note here is a photo of my beautiful fairy princess.

Please excuse me for a while while I regain faith in myself, the trust of my family and tend to my little girl.

The paed phoned today. That was good.

He asked how Ivy was and how she had faired over the weekend. Also good.

He said he had spoken to the dermatologist about Ivy’s pemphigus and he still wasn’t convinced that is what we are dealing with! What is with that?

The dermatologist, who wiped his hands clean of us, several weeks ago, now wants to see with his own eyes, how the pred has worked for Ivy.

The paed asked if we had heard from the dermatologist. No, no, a thousand times NO.

We have been on our own coping with prednisone highs and devastating lows. A discharging ear and an obvious infection. Diarrhoea worse than it has ever been, and the poor little girl who is getting lost in the middle of all this since the world’s most expensive test to find nothing!

ARRRRRRGGGGGHHHH!

So, if the derm doesn’t call by Wednesday, I have to call the paed and he will phone the derm and ask what the heavens is going on.

R i g h t, like the paed is going to speak with me on Wednesday, his busiest day. I know what that means. It means we will have to play message tag with the receptionist.

Nice.

So what does it all mean for Ivy?

Staying on the prednisone until Friday, full dose.

This might not seem so bad to whoever is still reading this rant but for Ivy it means roid rage, joint pain, her face has ballooned out with all the fluid retention and because of this she has to be seen by a renal specialist because her kidneys aren’t functioning like they should.

Yep another doctor! (Put your hands in the air like you just don’t care! Everybody say Yeah! (yeah)).

Because her infection hasn’t cleared, QID (four times a day) of the erythromycin for another few days.

EES is terrible for the gut and causes her already gross poo to become worse. Stomach cramps etc.

Back to a doctor that I don’t trust and who obviously does not like small children.

(When I told the paed I didn’t want to see the derm, he laughed but I was deadly serious. What can he do that our paed can’t? He just wants to ogle Ivy’s backside and put us off for another six weeks or so.  He is not a very friendly person, yes, he is a good dermatologist, who knows his stuff but I want more from someone who is dealing with my baby).

 I don’t think so. I’m sick of seeing my baby in pain. I’m sick of going to this doctor and that doctor and never getting anywhere.

I want our paed to grow a pair and make a decision and treat my baby.

I don’t like this ride anymore and clearly, Ivy does not either. Doctor after doctor after doctor and we still come up with nothing much of anything.

‘Round and ’round and ’round.

No diagnosis to work with, no defining blood tests. Nothing on the colonoscopy and endoscopy. Nothing.

And yet, she remains sick.

We can’t go anywhere or do anything. She’ll get sick, she screams because she is in pain, whatever the reason we are virtually house bound.

Unless we risk it and then we get in trouble for risking it.

I could just cry.

Join Ivy in her prednisone fuelled rages.

Seriously, I am beginning to lose hope.