Zayne's Heart

Just as we were getting IVIG down pat, we (I should say, Zayne) is done. I was told this is the last one, but I have learned that things can always change.

We got to try virtual reality (VR) for the first time. I tried for 3 minutes with Zayne screaming at me to give it back – I thought I was fine, but after I went back to my chair, I realized that I was motion sick (lasted for a couple of hours). Zayne loved it and was instantly addicted.

The new med, Entresto, is being slowly increased in hopes that we can make some progress. “A tad better” is the first positive news from her echo since we left the hosptial. Maybe Entresto (consists of two different medications) is working? We can no longer use BNP to measure heart failure, because Entresto increases BNP. The new measurement NT-proBNP is going down – that is good. I’m not explaining, because I would have to look up the technical reasons.

Still have to go to transplant clinic this week, since Entresto has been increased. Probably will have another clinic before the Nov 2 heart cath and Covid test #4. Children’s Hospital is not using the spit test ?

We had a little scare last week when riding bikes from parking lot to the pump track. There was a small incline and Zayne couldn’t catch her breath. I could hear her wheeze when she tried to breath. It was very scary for Zayne. It reminded me of the first symptom that I noticed the week before we were in the hospital for rejection – the same thing happened while biking up a steeper hill. She didn’t feel well for 4 days (even had to sleep with 2 pillows to breath better at night).

She still had fun at the track, but only 1 loop and rest at a time. Very different from the last time we visited when she rode her bike a couple of miles and then rode non-stop for an hour before riding back to the car.

Yesterday, IVIG #5 was completed. Once again, the IV went in nicely since Zayne worked to hydrate herself. Tylenol yesterday and today have helped with headaches. This time, the headaches weren’t as bad (the brand was changed since side effects were bad last time).

Because of last week’s biking incident, Zayne was given IV Lasix and has had her meds changed up. As usual, heart diagnostics aren’t showing any improvement (or un-improvement). Med changes include a Lasix increase and replacing Enalapril with Entresto. Entrosto was approved for pediatric use last October, and it has been shown to more effective in treating heart failure. She starts the new med on Sunday morning. Hopefully, Entrosto can help with heart failure and we can get her BNP to go down.

Another heart cath is scheduled in November instead of next June. Docs want to check out her heart to see if there is a reason for the lack of improvement.

The first couple of years we walked for the first day of school. The last couple of years, we rode bikes. This year, she sat in a chair, and I taped her schedule to the wall.

Exciting at first, but soon realized all day of school without other people can get boring. Especially for ADHD kids.

Last week’s clinc visit brought tears because Zayne learned she will be getting another COVID test in November. Since Zayne isn’t getting better, she is going to get another heart catherization (7 month earlier than anticipated). Maybe there will be another test by November that doesn’t require a cotton swab to the brain.

Since rejection med is a little low in her blood work, we have to head out to Children’s Hospital for blood work this week (only a finger poke so no big deal). Last week, we arrived for blood work at 6:45 am when the lab opens at 7 am. Blood draw did not happen until 7:45 – that is a whole lot of uncomfortable time in a waiting room with other people. Next week is IVIG #5

The day was spent watching TV and snacking. Water was guzzled the night before and in the morning – no tears this time around – IV went in smoothly on the first try. It was a good day (good for a 6-hr infusion).

I read some of an archaeology magazine to Zayne (we have a subscription), but after an hour it was time for TV and pizza for breakfast.

All the numbers were good (not better, but not worse). No donor antibodies. Heart failure indicator (BNP) still holding in the 800s. Heart rate still in the 130s. No change in echo or EKG.

No stomach issues this time around.

Last week there was some unexplained bad joint pain. Some extra tests showed nothing. Fingers crossed that joint pain doesn’t return.

The 3rd IVIG is done, and there are 3 more to go. I think Zayne learned the importance of drinking a lot of water prior to an IV poke. She refused to guzzle water the night before and the morning of the procedure; the IV insertion did not go well. On the positive front, there wasn’t a COVID test. Even though we were told that there wouldn’t be a COVID test, Zayne was stressing about it.

IVIG – what is it?
Intravenous immune globulin is a product made up of antibodies that can be given through a vein. Antibodies are proteins that your body makes to help fight infections. Each antibody made by your body is slightly different, because it fits like a lock and key to every foreign substance (such as a virus or a donor heart) that gets into your body. Since Zayne created antibodies to destroy her donor heart, all of her antibodies had to be wiped out (unfortunately, there isn’t a way to only target the wayward antibody). It is bad timing to have antibodies wiped out when there is a pandemic.

IVIG is prepared from the blood donated by thousands of people, to make a super-concentrated and very diverse collection of antibodies.

Only three more 6-hr monthly sessions needed. After IVIG is completed, then back to getting vaccinations (the first round landed Zayne in the ER and the dreaded COVID test). Zayne was only the 2nd kid at Children’s Hospital Colorado to undergo Eculizumab treatment for heart rejection. This is a chemotherapy drug that stops antibody production (need to stop all antibody production since the donor specific antibody cannot be targeted), and has a side effect of allowing meningococcal infections. After Zayne, there have been other kids to have this treatment, but Zayne was the only one that had a severe reaction to the first set of meningococcal vaccinations. She is not looking forward to the next set of vaccinations.

How is she doing?
Numbers are holding steady and not getting worse. Heart Echo and EKG show no change. Donor specific antibodies are dormant, resting heart rate is still in the 130s (occasionally high 120s), and BNP is below 1,000 ng/l for the first time since rejection. BNP is a protein that measures heart failure. Before rejection, her level was below 100. Zayne still gets out of breath, and has to ask me to slow down.

Other exciting news is getting a new, smaller pill box with removable sections. Zayne is doing great swallowing all pills with water. She is down to regular rejection meds, except for one. When she gets out of heart failure, then she can stop taking that extra medication.

June 29, 2020 marks the 5th anniversary of Zayne’s heart transplant surgery. This year, the celebration seems dampened by the rejection episode. For the first time, I didn’t bake a cake because she told me that she didn’t want one (we just had a “pill” celebration cake, and I would end up eating most of it). She wanted to celebrate by going back to the Wild Animal Sanctuary after today’s transplant clinic appointment.

Results from today’s heart transplant clinic appointment: no change, everything is the same. However, another medication bites the dust – Lasix was stopped on Friday, and isn’t needed anymore since Zayne is the same as last week. Down to 4 meds.

Another week of the same or slightly better results. We’ll take it. Her echo show very slight improvement, BNP managed to go down from the unexpected high a couple of weeks ago (still a little higher than the lowest number after rejection). No nasty donor specific antibodies – we want to keep those at zero.

Last week, we went to visit the Wild Animal Sanctuary It was our first public outing that didn’t involve the hospital (besides walks and bike rides). We went on a cold, rainy week day – perfect for us! It was empty, and the animals were active. Zayne loved it.

Recovery is going slowly, but it is going. We’re hoping that last week’s high BNP (measures heart failure) is an anomaly, because all other indicators are the same or improving. We’ll get PRA (antibodies) and BNP next Monday (oh no, that means a vein poke).

Zayne attempted to eat the sidewalk (bike fall), and it looks like she only has some sore teeth. I told her “next time, turn your head”.

Medical News

• Aspirin has been stopped
• Magnesium is stopped for now – the team is researching different types of magnesium with less side effects.
• Switched from Tacro liquid to capsule (levels have been stable, so the change was allowed)
• Dropping down to 1 hospital appointment every week
• Every other week vein poke
• Next cath in 1 year