Monthly Archives: May 2020

We are chucking some meds and are down to only 7. Once stabilized, Zayne will only have 3 daily medications.

Thursday’s transplant clinic appointment shows no improvement since last week, but at least we didn’t see a decline. I was told that recovery could take a while. Also, blood work has stayed the same – once again, at least the bad numbers did not increase.

Zayne is already stressing over her heart catherization and next COVID test on June 8. This will be her 3rd COVID test (this test will be a 10 second swab far up her nose).
After the heart cath, she will have a 6 hr (maybe 4 hr?) Intravenous Immunoglobulin Therapy (IVIg) infusion to add antibodies to her system that were removed from other treatments. What is the source of the IVIg antibodies? The antibodies come from purified blood plasma donations (from people).

Today, we had an unexpected trip to the ER, but when is a trip to the emergency room expected? Zayne had more severe side effects from the vaccinations than is typical, but her high fever could be a result of an infection that has come about due to all of the treatments to suppress her immune system. Since the source of the fever isn’t know, we were asked to go to the ER ASAP.

Since hospital policy seems to change on a daily basis, Zayne was subjected another COVID test. On April 27, we were told that a COVID test was needed to be admitted to the hospital, but now a COVID test is needed just for showing up in the ER. This test was a little different – a squirt of saline and a tube stuck up her nose (as far as the swab method), but it was much quicker. My ear is still ringing from her screams. Unfortunately, Zayne will need the slower, 10 second swab on June 8 for her heart catherization.

For the good news of this post, Zayne’s PRA (panel-reactive antibody) for donor specific antibodies has gone down! We are stoked to hear that both PRA and BNP are going down.

This was a tough day for Zayne, but I just got a phone call that her BNP is down to 1,200 from Monday’s result of just over 2,000. This is great news (her heart is on the mend), and her donor specific anti-body results will be available on Friday. In other good news, her echo (heart ultrasound) looked better than Monday’s echo.

The morning started in lab for a blood draw. Three pokes later, we were able to leave. This time, I didn’t hesitate to tell the tech to “pull out” when she missed the vein (I’m learning), but she still managed to hurt Zayne. The next vein poke wasn’t very bloodfull, but filled the required tubes. A finger poke finished off the remaining vials.

Galaxy, MD came to help Zayne with a couple of vaccinations – Meningococcal B and ACWY vaccinations that she needs because of the eculizumab (complement inhibitor) treatment. Two nurses came in to give her the shots at the same time. She didn’t feel one of them, because the other hurt so much more.

Side effects from the vaccinations hit, and Zayne feels badly: fever, chills, tremors, aches, fatigue, etc.

I made Zayne cry when I told her she would need a booster next month for one vaccination, and the other vaccination would need a booster in 3 months.

The day before the latest hospital visit was more fun

Zayne’s big take away from her May 18, 2020 clinic appointment:
I can finally take a bath!

My Summary:

• ECG is improved
• Echo (ultrasound of heart) is unchanged
• Heart rate is still in the 130s
• Zayne lost 5 pounds in 4 days – I wish I could do that, but her weight is from excess fluids due to heart failure
• Lasix dose is getting cut by 50%
• Steroid dose will be cut in 1/2 by Wednesday
• Off of penicillin, then back on. Apparently, the pharmacist has some concerns about the amount of elapsed time since her eculizumab treatment. This one will be good to leave behind, because Zayne has a 3 hour No Food/Drink window. So, Zayne may get to stop taking penicillin, or may have to continue for a couple of more weeks.
• Thursday’s blood test for BNP (measures heart failure) and PRA (measures donor specific antibodies) will show if rejection is getting under control or not (we want lower numbers). PRA results will not be available until Friday.
• It is going to take Zayne a long time to recover – her heart is in bad shape, and she is still in severe heart failure.

Zayne is wearing her sun outfits to protect herself from the sun, while playing outside. She is trying to ride her new bike, but can’t do any hills right now. It is difficult to stay away from inclines, so I rode ahead, walked back down, and let her sit on the bike while I walked her bike up the incline.

Finally, I received a call today that had better news for Zayne. The CH50 and PRA results are so encouraging that Monday’s extra treatments are probably not needed. Zayne is extremely happy that Monday should only be a finger poke for a routine blood draw.

The CH50 blood test measures the activity of a group of proteins in the bloodstream. These proteins are part of the complement system, which is one part of the immune system. The complement system helps antibodies fight off infections and destroy substances (i.e., Zayne’s heart) that are foreign to the body. The eculizumab brought her level down to nearly 0, but now it is at 43 which is near normal. I couldn’t get a clear answer on what level she should be at, but this seems to be ok.

The great news is that the PRA has gone down by nearly 50%. The Panel Reactive Antibodies measures the amount of donor specific antibodies. We’ll measure these next Thursday, and I hope then continue to go down.

Zayne is still easily winded, and she doesn’t have any physical restrictions. Right now, she just needs to self regulate. I have to figure out how to get her moving in a fun way.

To encourage Zayne and to help motivate her through difficult hospital procedures, I promised to buy her a new mountain bike. I told her that I would find the color that she wanted, but had no idea that there is a bicycle shortage. I could not find the gloss black bike with blue trim anywhere in the Denver area, so I ended up driving to Cheyenne, Wyoming to pick up her new bike (delivered to my car). I thought we could all go, but her team does not want her so far away, so Aunt Vanessa watched her while I made the 4 hour (round trip) journey.

I told Zayne that we would leave today’s transplant clinic appointment with another medication, and I was correct. I noticed that Zayne seemed to be getting more tired and not eating as much. Her stomach also seemed to be sticking out, and Zayne was proud that she was at 61 pounds. I didn’t really think that she gained a healthy 4 pounds in one week. We left the appointment with a new medication to take at home – Lasix. I’m happy to report that Zayne seemed to perk up and have an increased appetite increased after taking the diuretic. Unfortunately, she couldn’t visit Aunt Stacy (a non-contact visit), because she needed to get that extra fluid out of her body.

Zayne is swallowing pills like a champ. She is using applesauce, and will work on water when she is down to taking fewer pills. I’m very impressed with her progression to pills.

Today’s blood draw was another vein poke taken from the top of her wrist. Unfortunately, Zayne was dehydrated (all the water was in the wrong place), and filling 5 tubes was a very slow, painful ordeal. Mondays are supposed to be a finger poke day, and that will happen at the lab. However (there is always a “however”), it looks like Zayne will need special treatment on Monday that will require an IV and an injection. Her strong, little body has powerful donor specific antibodies that are still a big problem.

Depending on the results of her CH50, she may need another Eculizumab infusion on Monday The panel-reactive antibody (PRA) will determine if she will need a new treatment – a shot of Bortezomib.

PRA = High = Bad
PRA = Low = Good

CH50 = High/Low = I don’t know [I couldn’t get a specific action number from the doctor]

From what I’m reading, bortezomib therapy can help to treat severe antibody-mediated rejection. I knew that Zayne’s rejection is bad, but seeing the words “severe antibody-mediated rejection” just drives the point home. I fully expect that Zayne will spend all Monday in a hospital bed to have her body infused with eculizumab and injected with bortezomib.

I asked if Zayne’s donor specific antibodies could be targeted, but that technology doesn’t exist. All of her antibodies need to be targeted to get at the bad ones. It seems so archaic.

After one full day at home, we were back at the hospital bright and early for a blood draw and transplant clinic. Unfortunately, the blood draw was not a finger stick that Zayne is used to getting, but a venous blood draw. Zayne doesn’t remember giving venous blood in the lab, so she was nervous and scared.

Heart transplant clinic involves the usual height, weight, questions, ECG, Echocardiogram, and exam. On Sunday, and at the hospital, Zayne just doesn’t seem as peppy, and doesn’t want to go for a walk or spend much time outside. We tried to walk up the stairs, and took lots of breaks, but had to get out at the next floor to take the elevator. Basically, her heart numbers are not improving (they were a little worse since Friday), but the doctor couldn’t visually see a decline in the echo – it looked the same to her. So, maybe that is OK news?

Right lung is back to having fluid, but no where near what it was at the beginning of her hospitalization. On Thursday, there will be some additional blood tests to check on her heart function and donor specific antibodies. If the fluid is still there, she will probably be put back on Lasix (a diuretic) to help remove the fluid.

Listening to the doctor, and learning that she will need another vein poke on Thursday (and then once a week for the next 6 weeks), and another Covid test at her heart catherization (no more than 4 weeks from now), Zayne kind of shut down. She is dreading going to the hospital for procedures that are painful, and this is causing her stress and anxiety.

This morning, I woke up to find hooked up to another magnesium IV. Not only was her magnesium levels a little low, but she had arrhythmia last night. The arrhythmia could have been caused by low magnesium. This is the first arrhythmia that Zayne has had. Not the best news to hear upon waking up.

Unlike the ICU, there is sleep is better in the CCU. I used medical tape to close the gap in the blinds in the nurse fishbowl area – the room is much darker. Zayne and I are happy to have a garbage can in the bathroom (and thrilled to actually have a bathroom with a door) – little things that make life easier. I also love using the toilet in the room, but I’m not supposed to use it. I live in fear of getting caught.

Zayne is extremely fearful of having her IV and PICC lines removed. She is wary of anyone telling her “it won’t hurt”, because that is usually not the case. Today, she asked me why she has so much pain in the hospital. She was very down after her transplant cardiologist talked with us, and spent a good part of the day moping around, and not wanting to explore or go outside. She knows that I worry if she doesn’t want to do things, so she told me that she is OK, but just doesn’t feel like doing anything.

Plan to get rejection under control

• Monthly IVIG infusion for the next 1/2 year. This means a monthly IV, which means pain.
• For the next 6 weeks, we will be coming to Children’s twice each week for early morning blood tests and a transplant clinic visit (ECG, Echo, exam).
• Another heart catherization in June to check heart function and collect biopsy samples

Plan for Friday, May 8, 2020

• The final plasma exchange
• IVIG infusion – this treatment puts antibodies into her system in the hopes that her body stops producing donor specific antibodies
• If there aren’t any problems, go home? If not going home on Friday, then go home on Saturday.

Zayne is much better than when she came into the hospital, but still has significant issues. It could take weeks, months, or over a year to recover. Will she ever fully recover?