Just when we thought we were done with hospital visits for a while… BAM, Ethan’s port is the size of a golf ball!
We infused Ethan on Friday as scheduled, and everything went absolutely perfect. I hit the port in the first try and immediately got blood return (which is exactly what is supposed to happen).
After that, things were great and nothing happened out of the ordinary over the weekend – at least not that we are aware of.
Ethan and I were getting ready to attend his little friend’s first birthday party on Sunday afternoon. As I started to change his clothes, I noticed his port was swollen. Everything was fine up until Saturday night, and I knew this was not normal. Peter immediately grabbed the phone and called the Janeway to speak to the hematologist on call. She assured us things were okay and that we could go about our normal plans for the day. She just wanted us to touch base with her later that evening if things got any worst; otherwise she would talk to us the next morning.
So, off we went. Ethan had a blast at the birthday party and everything went great. He had so much fun he slept for almost two hours when we got home!
That evening as I was getting Ethan ready to have his bath, I noticed the area around his port had swollen even more! This time it was more than double the size… Maybe even triple. It was easily the size of a golf ball. The skin was so tight that the scar from his surgery was starting to stretch. I panicked at the sight and immediately called the Janeway again!
I have to say, I have been really laid back and calm about everything we have been through so far, but this time I was legitimately worried. I felt sick to my stomach just looking at it. Oh dear! I was ready to scoop Ethan out of the tub then and there and take him directly to the hospital.
Thankfully, the hematologist assured us that things were fine and that there was no need for us to rush in with him. Aside from the noticeable swelling, there were no other signs of a bleed. He was still his normal, happy self. She advised us to keep an eye on it and to make a visit to Medical Day Care in the morning whenever we were up and straightened away. She wanted to see Ethan not so much because she was concerned with the swelling, but because she knew we wouldn’t be comfortable accessing his port to infuse him.
This morning when we woke, although Ethan’s port was still very swollen it had decreased significantly – which was a really good sign!
We made the hour trek over the highway to visit the doctors and nurses at the Medical Day Care unit once again. He really does love those nurses, ha ha.
After they checked Ethan out and assessed him, it was determined that he had a bleed around his port… For no known reason, other than the fact he is a little boy with hemophilia that has a port. He may have bumped it, we may have hit a small blood vessel while accessing his port (where his body isn’t used to it), and it may have just happened on its own.
Thankfully it was nothing too serious.
They decided to give Ethan his Advate while we were there, and rather than giving him the usual dose they doubled it to be safe. Since the swelling was significant, they left the needle in place so they wouldn’t have to re-access the port tomorrow. They just covered it with gauze and Tegaderm to secure the area.
For now Ethan has to go back again tomorrow for another dose of Advate, and again on Thursday for a checkup. Hopefully the swelling will be gone down by Wednesday when we are scheduled to do his next at-home infusion.
The area has, however, started to bruise. The hematologist warned us that it will likely bruise very badly before it fully heals.
Aside from the bleed in his port, the hematologist also wanted to do some bloodwork to make sure Ethan hasn’t developed an inhibitor. While this test was done just before he had his port surgery and came back negative, they want to be certain things haven’t changed since he has been on prophylaxis (and has been receiving factor treatment much more frequently than what his body is used to).
Hopefully this is not the case.
If it is, we will cross that bridge when we get to it. And, if by some chance Ethan has developed an inhibitor (which essentially means his body blocks the Advate from working), there are treatment options available. Ultimately the goal would be to get his body, and immune system, used to the infusion of Advate so that it no longer rejects it. This would be done by infusing Ethan with high doses of factor for long periods.
I will be sure to let you know how tomorrow goes. Fingers crossed for no inhibitor and that the swelling has gone down.