A rare life – day 4

If there were any questions about whether or not Miss Z had fully recovered from her 5-day week at school, they were answered this afternoon. We went to a birthday party and Miss Z completely failed to cope. On the positive side, it was the birthday party of a lovely girl with a similar condition to Miss Z’s, so everyone was kind and understanding and I didn’t feel that I had to try to hide my grumpy, coughing, crying child. She did rally briefly during pass the parcel, but it was all downhill after that. She fell asleep on the drive home and, as luck would have it, was much happier when she woke up.

This evening has been all about preparing for tomorrow. We are heading to the hospital very early tomorrow morning for the surgical re-insertion of Miss Z’s GJ tube (remember that thing she pulled out the other day?!) and if all goes well, she’ll then get her Pamidronate infusion (which strengthens her bones). Neither procedure is particularly high risk and she’s had general anaesthesia before, so I’m not too concerned. But it all amounts to a very, very long day at the hospital.

I hate packing for a trip to the hospital, even if it is only a day admission, like tomorrow. I find it as stressful as packing for a holiday – or even worse, a work trip. I am one of those people who worries about getting to their destination and realising they’ve forgotten some crucial item.

In my defense, Miss Z does not travel light. Nappies, wipes, sick bags, feed pump, formula, all the necessary tubing for her feeds, emergency seizure medication, and several changes of clothes are all must-haves. Then there are the things that life at the hospital more comfortable for Miss Z, like her bunny, extra socks, her iPad and her pink blanket.

So, our bag is packed and our wheelchair-accessible taxi has been booked (for a painfully early 5.30am!) and I’ve laid out clothes for both Miss Z and me. My lunch is made and waiting (hopefully not to be forgotten) in the fridge. I can hear QB getting Miss Z’s chair out of the back of the car as I write. So long as I remember to put a phone/iPad charger in the bag before we go to bed, the crisis of the forgotten crucial item should be averted…

I’ll let you know tomorrow how successful we were at remembering everything!

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Bunnies make hospital trips just a little bit better.

 

A rare life – day 3

Life with Miss Z can be quite messy. One of the less pleasant aspects of Miss Z’s life is her inability to manage her secretions. “Secretions” is one of those vague, polite words that actually mean the phlegm, snot and saliva that build up in the back of her throat. Most people swallow regularly throughout the day and so don’t even notice it, but Miss Z rarely swallows, and when she does, it often isn’t effective enough to get much down.

So, the secretions just sit there. You can hear them rattling in her upper airway. They make her cough. Eventually, she will cough, and then gag and then retch and vomit, getting rid of some of the goo. The rest ends up aspirated into her lungs or up into her nasal passages. The stuff in her nose gives her sneezing fits. The stuff in her lungs causes chronic respiratory infections.

Think of that horrible, congested feeling you get when you have a bad cold. Well, Miss Z has that feeling every day.

The tough part is that there isn’t anything that can be done to fix it. Her GJ tube will stop her refluxing and aspirating her food, but not her secretions. There are drugs that will dry up saliva which are used with kids who drool excessively. But Miss Z doesn’t have excessive saliva, she just can’t manage her ordinary amount. And the doctors are concerned that drying up what she has will only make the secretions thicker, which means she won’t be able to bring them up as easily. And if this happens, she will have the sensation of not being able to breathe in enough air, which to me sounds like the stuff of nightmares.

At the moment, we address the problem by using Ventolin (a drug many people with asthma use) and a special saline solution in a nubuliser. For those who aren’t familiar with them, nebulisers are (noisy) machines that administer medication by turning it into a mist to be inhaled through a face mask. The Ventolin is meant to open her airways, whilst the saline thins the secretions, making it easier for her to cough them out.

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Not fond of her nebs, our Miss Z

When using nebulisers and throughout the day as she coughs and vomits, we rely a lot on our suction machine. This is because her secretions can be quite thick and gooey, so she often needs help getting rid of them. We suction them from her mouth and her nose.

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Suction machine and a sick bag – they go hand in hand

I used to be quite squeamish about sticking a tube down Miss Z’s nose to suck out the snot. Used to be. Now I am the nasal tube master. I can do it on my own (even though Miss Z puts up a good fight). I have done it while she’s asleep – and not woken her until the suction machine roared to life. I’ve even done it in the middle of the night when I probably wasn’t fully awake.

We have also learned to always have a sick bag on hand, as Miss Z is fond of the stealth vomit, where she sounds fine one minute and is projectile vomiting down the front of your shirt the next. So, if you visit our house, you’ll notice strategically placed sick bags in every room as well as the car. We have learned from the Boy Scouts – its always best to be prepared.

Her secretion management problems (aka coughing and vomiting) also makes it tricky to take her out and about. I’ve learned to more or less ignore the surprised looks when Miss Z starts retching and gagging in the middle of a shop. However, Vegemite still gets very embarrassed when her sister starts throwing up in public.

Rare may be beautiful, but no one said it wouldn’t be messy, too.

 

 

A rare life – day 2

I love someone rareWelcome to day 2 of my 25 day challenge to give you a glipse into life with a child with a rare (in this case undiagnosed) disease. For those of you who weren’t paying attention yesterday, I’m going to try to write every day up to 29 February, which is World Rare Disease Day.

I had planned on writing about our Friday routine – which always makes me smile. However, events again unexpectedly presented themselves and were just too good to resist writing about.

Today was the fifth consecutive day of Miss Z attending school. She began Prep (aka Kindergarten or Reception in other parts of the world) last week, which was only a three-day week, so this week was her first full week of school. Even though she became increasingly tired as the week wore on, she did really well.

So well, in fact, that at the school assembly today she was named Student of the Week!

Of course, being Miss Z, she had to do things her way. She was awarded Student of the Week for adapting to the transition of attending full-time school so well. And she slept through assembly and the awarding of her certificate. Her teacher said that at one point she cracked one eye open, decided she didn’t want to get involved, and closed her eyes again! At any rate, the photos of my sleeping daughter receiving a reward are hilarious and QB and I are hugely proud of her.

Everyone in our house has been worn out by the first full week of school. Even I’m struggling to keep my eyes open as I write and it isn’t even 10pm on a Friday night here! I knew the girls would be tired after school, so we watched movies all afternoon. They were both happy enough, but Vegemite later cracked completely when trying to do her piano practice. Irritation at not being able to play a piece turned into frustration, anger and then there were tears… which eventually turned into wails of anguish. All over piano practice.

I eventually cut my losses and stuck Vegemite in the shower and then sent her straight to bed. She cried a bit because I was paying more attention to Miss Z (who I’d just taken out of the bath) and took “too long” to put all of Z’s bath things away before going to have a cuddle with her.

Which made me thing: everyone always assumes it is Miss Z who is the difficult child, but that is far from the case. In fact, Vegemite is quite often the one who is drives me around the bend. Miss Z is actually quite happy and easy – so long as you follow her rules.

 

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A rare life

I love someone rareThe 29th of February is World Rare Disease Day. The day is intended to raise awareness for rare diseases and to improve access to treatments and medical representation for individuals with rare diseases and their families.

Miss Z is rare. So rare, in fact, that the doctors aren’t sure what she has – they’re just confident that it isn’t any of the usual suspects. Usually, being rare isn’t a problem – her doctors and therapists and teachers treat her as Miss Z, not a diagnosis. However, being rare leaves Miss Z without a prognosis, without any idea of what might happen in the future. It also means that the doctors and therapists and teachers who haven’t met Miss Z before always tend to be a bit befuddled and struggle to find a box in which to put her.

It also means that we, as her family, live a less than typical life. Life is never dull, or easy, or straightforward with Miss Z around. But it can be quite beautiful.

To give you a better understanding of what being rare means to us, I have decided to give you a glipse of our daily lives during the 25 days leading up to World Rare Disease Day. We have a very busy February ahead of us, so there should never be a dull moment!

I was going to kick off my 25 days of living rare by telling you about my big accomplishment for the day – getting a date for Miss Z’s surgery to have her GJ tube re-inserted (and yes, I do mean re-inserted – she got it last Monday and managed to accidentally pull it out on Saturday). However, this evening Miss Z and I had a mishap, and I know that mishaps are always more interesting than achievements (especially when the achievement involves hospital bureaucracy), so….

Miss Z and I were both tired at bedtime tonight, but everything went smoothly. I even remembered to take Miss Z’s feeding connector tube downstairs with me when I put her to bed. I like to have her all connected up, so that I can give her meds and her feed later in the evening without waking her up. Usually, I forget the tube upstairs, which means I have to run back upstairs for it – not a big deal, but one of those little, annoying things in life.

Tonight, however, I was on the ball and remembered to grab the tube before going downstairs. Miss Z and I read a bedtime story (we’re very much into Lynley Dodd at the moment) and then I put her to bed, connected up the feeding tube, and kissed her goodnight.

An hour later, she was still awake and rocking around in her bed, so I went downstairs to check on her. She seemed a bit uncomfortable, so I rolled her onto her other side – which often helps her go back to sleep. Instead, I discovered a puddle. I had failed to close the clamp on the connector when I’d connected it to Miss Z’s tummy and the med and food ports were open too. In short, I’d managed to drain the contents of Miss Z’s stomach out onto the bed. Yes reader, I un-fed (de-fed?) my child.

Miss Z wasn’t terribly concerned about the puddle, but she did get very cross when I heaved her out of bed in order to change the sheet and her pajama bottoms. She is back in bed now, warm and dry, fast asleep and keeping the contents of her stomach to herself.

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Leakage – though not from un-feeding. And a view of the connector tube.

Leaving Z

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Nooooo, Mummy! Please don’t go!

Tomorrow, Vegemite and I will board a plane and fly to Michigan to visit my family for nearly 3 weeks, leaving QB and Miss Z in Brisbane.

I really wish it didn’t have to be this way. I wish we were all going as a family. But it quite simply isn’t possible. Miss Z isn’t healthy enough for long-haul international travel, even if we could somehow manage to get her and all her equipment and supplies on an airplane and find travel health insurance to cover her.

Vegemite has a very simple, yet quite genius, solution to this problem. We need a private jet. Then Miss Z wouldn’t be exposed to all those germs from other passengers, she wouldn’t gross anyone out when she vomited mid-flight, we could take as much luggage as we wanted, and if she got sick during our trip, we’d just put her back on the plane and fly home.

Unfortunately, QB informs me that it may be some time before we are able to purchase a private jet. So, it looks like Vegemite and I are taking the trip by ourselves.

But in all seriousness, this breaks my heart. I’m happy that Vegemite gets to see her extended family, spend time with her grandparents, and enjoy time in the state where I grew up.

But I wish Miss Z could do all that, too. She has only ever been to Michigan once – when she was 3 months old – so her extended family in the US has never really met her.

Even more difficult for me, however, is being separated from Miss Z for nearly three whole weeks.

Recently, I’ve started to use the phrase “if you can pry my hands off her” a lot. Yes, Miss Z is going to start school full time next year – if you can pry my hands off her. And yes, she will be taking the bus to school – at least, she will if you can pry my hands off her…

Leaving her to go to the airport is going to feel a lot like that.

I am Miss Z’s primary carer. We spend a lot of time together. And since she is reliant on others for all her needs, I am often the one who is making sure that she is washed, dressed, wearing a dry nappy, fed, watered, and comfortable. That she gets the right medications at the right time in the right amount and that the nebuliser and suction are used as necessary. That her stoma dressings are changed daily and the water levels in the balloon in her mickey button is checked regularly. I also make sure that she spends her day in a variety of different positions (even when she would prefer to lay on her mat on the floor all day) and doing a variety of activities.

That isn’t to say that there aren’t other people caring for Miss Z – there are. QB does his part, of course, and we have two fantastic support workers who help with Z. But Miss Z needs help with everything – from getting fed to changing position in bed at night – and it is a huge responsibility that looms large in my life, every single day.

And it also means that I am very intimately involved in Miss Z’s life. I know her better than anyone else.

When babies are first born, they don’t see themselves and their mother as individuals, but rather as one unit. It is only later in their development that they understand that they are separate people. However, for Z and me, it feels like we are still that one unit. Going away for three weeks makes me feel like an individual again, but that can be bittersweet. Because I don’t mind being part of the Z unit, if I’m honest. And since we don’t know for how long we have Miss Z, I inately resist losing even the tiniest bit of our closeness.

None of this will stop me from going. I want to see my family, to have respite from being Z’s primary carer, and to spend some one-on-one time with Vegemite. I know QB will look after her well – and that in fact, it will strengthen their bond, which is equally important.

But it still may come down to if you can pry my hands off her.