It’s been a hard week…

Behind Ethan’s big smile there were so many tears, lots of frustration, tons of coaxing… and maybe a little (or a lot) of bribing. It has been a hard week. One of the hardest I remember in a long, long, LONG time.

My little seven-year-old went to bed last hating his life and hating hemophilia. And you know what? Right now I cannot blame him. I am utterly heartbroken and feel so helpless. What kid should have to deal with this? I know so many kids are not as fortunate as Ethan; so many kids are not as healthy. Ethan knows that too. But right now, he is focused on his life and cannot comprehend why so many things have changed in just a few short days. He is consumed by his hemophilia and the sudden changes it had brought his way.

You’re probably wondering what has changed so suddenly. I rarely take time to write about Ethan and his hemophilia anymore because he has been doing so well! Really, there has been “nothing” to write about – which has been good!

However, things have suddenly changed. And we knew this was coming sooner or later.

The last week or two Ethan has been having pain in his port when we give him his regular treatment. Not when we access it with a needle, though. It happens when we push the fluids through. He feels a lot of pain and pressure… to the point that he clinches his chest and cries. The needle goes in perfectly, we get good blood return, and the fluid goes in without having to force it… but it hurts. A LOT. We know this is not normal.

We contacted his hemophilia nurse Monday morning and that afternoon he was in for an emergency x-ray. While we haven’t received the results, they know something is not right. So, for now (and likely forever) we cannot use Ethan’s port. This happens. They usually give the port a lifetime of about 5-7 years. Ethan has had his for almost seven. He has done well. Really well. But, it is all we have known. It has been our life. We have ALL been so comfortable with it. It has been so easy! So routine. And while we knew this was coming and we chatted about it with Ethan regularly, we weren’t ready for it yet.

Wednesday, we were told Ethan would have to receive his factor by the means of peripheral IV. This means we would have to access his vein using a butterfly needle. This process is (normally) a lot simpler and quicker than accessing his port. It doesn’t require as many supplies or as much prep work. Wednesday went fairly well. He was upset about it, but he was brave. He was really nervous and shed a lot of tears, but he stuck it out and let me access his vein. The treatment went in nicely and he was done. Phewww.

Yesterday he bravely came up to get some Emla cream on the spot we would be injecting. This helps numb the area so it doesn’t hurt as much. However, once it was time to get his factor he totally shut down. He couldn’t bring himself to do it. We sat at the table for an hour trying to coax and bribe him to get his treatment with no luck. We tossed everything out and agreed to try later with fresh factor and supplies. So we did.

That evening we did the same thing. The vein he wanted to use wasn’t great, but I tried it anyway. I tried to let him have a little say in the process. I poked him, but had no blood return. I wiggled the needle around a little with no luck. Darn. He was getting more nervous and upset as time went on. We took a break… Many, actually. He cried. He hyperventilated. He had to get some fresh air, and even wanted lie down. He cried… a lot! He is a nervous kid anyway. He worries about everything (he inherited that from me, of course). After going back and forth to the kitchen table a number of times, we tried again. He let us use a different vein. I had a little blood return but lost it. As I was moving the needle he pulled away and the needle came out. Ughhh. He was frustrated and so were we. We hid it well, though. We tried to be as calm and comforting as possible. We tried to be empathetic. At this point nothing helped him.

An hour and a half in and he was exhausted. So were we. But I really wanted to get it done before he went to bed. I didn’t want him to have to face the same thing again in the morning. I really wanted to try the top of his hand. That’s where we accessed Peter’s vein and mine when we were practicing and it went so well! He even took a turn getting blood return from Peter and pushing some saline through. He smiled but the tears were still there.

He knows the vein in his hand is nice; it’s easily accessible However, for some strange reason he refused to let us access it. We literally bribed him to no end with no avail. So, after two hours this guy went to bed with no factor. And I went to bed and shed many, many tears. My heart was broke for him. It was so hard to have to see him go through this and see him feel this way. I wish I could take it all away, but we all know I can’t. He has been so good with hemophilia all along. I guess we’ve just hit a bump in the road for now. Hopefully one day – sooner than later – we can look back at it and say, “Remember that time when we first started giving you factor using your vein and you were so scared and upset… Well, look how far we have come!”

However, despite it all, I do have some positive news… maybe a little silver lining from having so much trouble with his port and having to go through all of this. We learned Wednesday that Ethan will be switching over to a new medication next week. He was supposed to switch over soon, but things got put on hold due to a shortage of the new drug. We were told recently (prior to his port issues) that it may not be until the spring or summer. And, at that time, we were totally ok with that since his treatments were going so well and Ethan was doing so well! Little did we know things would change so quickly. Because of these recent issues, he was bumped up the priority list and approved by Canadian Blood Services to transition over to Hemlibra.

Hemlibra is a fairly new drug that has recently become available in Canada. Most hemophilia clinics are just now rolling it out to patients with severe hemophilia. It is a huge breakthrough for hemophilia patients! It is given in the fat as a subcutaneous injection. Absolutely no need to find and access a vein. Yay! Annnnd, instead of having to receive treatment 3x a week like Ethan does now, he will only need to receive it once a week or once every two weeks (depending on the treatment regime we choose with his hemophilia team). While it will be hard to change the mindset of always guessing Ethan’s factor VIII levels (we have been doing this since he was born), we know Hemlibra works differently. When using this medication, it won’t increase his factor VIII levels. Rather, it will act like a bridge, bringing factor VII and factor IX closer together to allow the blood clot process to happen without needing to replace factor VIII.

It is crazy how it works, but we have heard wonderful things about it and are excited to see what this new medication will bring for Ethan’s quality of life.

Hopefully there will be brighter days ahead.

And hopefully he lets us access his vein today so we can give him treatment. Say a prayer for us. Please.