End of the Year

The end of the school year is sneaking up on me. I was forewarned, when Mercy entered Kindergarten, that the end of the year tends to be a very busy time. The term "busy" is an understatement!  There have been assemblies to attend, parties, field trips, sports day and countless little girls and boys birthday parties to attend.

Don't get me wrong, I am not complaining. Far from it! I enjoy these little activities that remind me that life goes on. Sometimes it feels as if life drums along to a different beat for my family. Depending on whether or not Brian is having a "sick week". The possibility of a blitz week always looms over my head like a dark cloud. So when we have a week filled with normal activities, I celebrate!

*yawn*

I yawn because I'm tired. I'm tired because this learning curve is getting the best of me. Learning curve? Yes. I'm learning all kinds of things right now. What exactly Brian's disease means, and what forms of treatment are there in other parts of the world, what kinds of natural remedies treat symptoms he has and what are the best DIRECT forms of getting the nutrition he needs, how to cook these "different" foods (and how to afford them on said limited budget!), how to be the "man of the house" when Brian is sick (for example, I tried to learn how to run our rickety old lawn mower last week. Tried and failed, I might add). It's frustrating and overwhelming some days. Days like today I'm just completely tired out.

So the blog has kinda been one of the balls that has been dropped, obviously. A LOT has been dropped. I'm no longer a good sister, a good friend, or a good daughter. Not that I ever claimed to be perfect in the first place. I've forgotten birthdays. I even (almost) forgot Koby's Well, I was a day late. ON his birthday it took a school friend to remind me that it wasn't the NEXT day, it was TODAY! Well, I threw something together last minute and I don't think he noticed. Poor little neglected man...

Well. There you have it. A post!

Whoa

Okay. It's been awhile. Has it really been almost a month since I posted? Honestly, I haven't run out of things to say or share. I have just been busy doing LIFE lately. The new-new-NEW normal. It's an adjustment.

Brian ended up struggling again with his platelet count. Now it's stable. Not a HIGH stable, but it's stable. And that's good enough for us, I suppose.

On Monday we got the news that the genetic testing came back. Nothing prepared us for the news that it IS genetic and my kids have a 50% chance of having the same genetic mutation. What that means exactly? We don't quite know. But 50% just sounds frightening. I've had questions swimming around my head since the news, and according to Brian we'll be having a conference call with some specialists who can answer all our questions. It would've been nice to have questions answered the very next day, but I am getting used to playing the medical waiting game. Everything takes time.

For those of you that may like medical mumbo jumbo, and are research buffs and/or curious... The gene affected by the abnormality is in the C3 gene sequencing: the Exon 14 gene. Most sufferers of HUS/TTP have issues with their Adams 13 gene, but Brians case is (of course) different. Of the 6 categories under Atypical HUS, Brian's genetic mutation doesn't fit in with any of them. So the very rare disease he has is even more rare.

If that went over your head, I don't blame you!

bad days

It's been hard, watching the progression of Brian's disease. Not that things are changing rapidly, but the last few weeks we've noticed a trend. What used to be his bad days are now his good days. Does that make sense? On a bad day, he'd be tired and winded, feel nauseated and look pale. But recently that's been the norm. On a bad day he's been feeling worse. Sometimes MUCH worse.

Just over two weeks ago his numbers started a decline. I thought nothing of it this time. Every other time I'd panicked, thinking he'd have to have a "blitz" of treatments (three treatments in one week, usually). But every time the numbers came up again on their own. I guess you can say I got overconfident that it would work itself out. Surprise, surprise - his platelet count dropped down to 78. And I didn't even notice. I've been less and less diligent with checking his lab numbers, so it took us by surprise. To remind you, his platelet count was in the 60's when he was rushed to VGH the first time. So it was pretty close for a second time. 6 treatments later (make that 7, counting today's regular treatment) and the numbers still aren't where they should be.

I'm frustrated. I feel so helpless, so STUCK. I want to get off this roller coaster, or I want to be useful somehow, and help Brian get better. But there's nothing I can do, except try to support him. It's not enough.

very bad news indeed

I just got a call from my favorite Mother in law (even if I had more than one, I just KNOW she'd be my favorite). Brian was at the hospital having his weekly PLEX treatment and the kids were with Gramma. I've been hit by a nasty flu bug that's pretty much landed me flat on my back! I can't remember the last time I felt so miserable. Cold, then hot, sore throat, nauseated, headaches, body aches, fevers... But I digress...

Brian just picked the kids up from Gramma's house and they're on their way home. She phoned me because she wasn't sure Brian would tell me himself. He got bad news today (we've dealt with it before, but I'm nervous anyway) His platelet counts are 80 today. 80! To put it in perspective, again, platelets are supposed to be anywhere from 150 - 400. At about 60 you start to bleed out. Scary. So he's going to be going back to hospital Wednesday and Friday, she tells me. We're having a "blitz week" of PLEX treatments. I hate those kinds of weeks... The fact that he can't work 3 out of 5 days adds an extra stress. Money stress.

claw

What's new on our front? Well, good and bad (as usual!). Brian and I were just at the hospital today. We got a call last night that he was booked for a test - nice of them to give us notice! A dye had to be inserted into the veins in his arm to see if he can have surgery to create a fistula in the near future. We haven't spoken to the specialist but we already know the bad news - his veins are just too small! They may still be able to do a fistula in his upper arm, but it requires much more work and much more time off to heal! So I'm praying something miraculous happens in the meantime.

We have also been waiting to hear about the genetic testing. It was done months ago. There is a very good chance his type of Atypical HUS is genetic, and could be passed down to my kids. First they'd test his siblings, if he tests positive for the genetic code, to see if any of them carry the same trait. THEN my kids would get tested. I have been waiting on pins and needles to hear those results. They were supposed to come in last Friday, but we've heard nothing.

I've made this post long enough after weeks (months?) of silence. Thanks for being "out there".

On

- On the Brian front: Amazingly enough, he is doing really well! With a few treatments postponed due to holidays and one appointment accidentally missed, his numbers are holding steady. We're very happy about that, as you can imagine.

- In the next few weeks Brian will be seeing a surgeon, who will set up an appointment for the fistula to be created in his arm. This means he'll be able to have the tubes taken out of his chest, which decreases the possibility of infection (a huge concern, especially since they go directly to his heart) This ALSO means easier access when blood needs to be taken, or when he needs a future PLEX treatment. but it takes months for it to be ready after surgery, so it will be ready for use in a few months.


- My time at the keyboard is coming to and end, I have two kids crawling on me. Thus the reason my blog has been abandoned, for the most part! Maybe I'll add that to my list of things to do while the kids are in school!!