Good afternoon,

I'm here to bring you guys a pretty positive update today, with only minor setbacks.

Last update our biggest struggle was Annabelle's HD line for her dialysis. Well, the line continued to give us trouble through the weekend, and it was decided on Saturday that the team would like to replace the line. At first, I was completely against it seeing as switching legs didn't seem like a good option to me. But after talking with the IR team, they discussed moving the line from her leg to her chest, this line would run parallel to her central line and would be much less problematic. Again, I never want more holes than there needs to be, but this seemed like a pretty good option since her dialysis machine was spending more time off than on.

Annabelle was taken down to get the line replaced around 11:30 and was back in her room settled on CRRT by 2. She did great and the line placement went as expected. Since the placement of the line the CRRT machine has been running great with no alarms or down time. Now that the line is out of her leg Annabelle has been much more comfy and able to relax a bit more since she is able to move both her legs freely. The team did check her leg for any clots caused by the HD line, and she does have 2, one that is inclusive and one that is non-inclusive. The plan is to put her back on blood thinners and hopefully that will help break down the clots in the leg.

Since her new line was placed and her circuit has been more stable, I was able to hold her at the bedside this week. It's not for very long and I'm not sure she even likes it, but it does some good for this momma's heart. Being able to be close to her again after such a long time watching her lie in a bed paralyzed was amazing. She’s coming off a lot of sedation and with every day I can see more and more of her personality coming back. We were able to play with PT and OT this week and working on moving our arms and legs again. Sometimes it feels like we are back at step one, but they remind me daily that she is so much stronger than when we first got here and it's only going to get better.

Our new goal this week has been to start trialing Annabelle at a lower rate on her ventilator to prepare her for extubation. She has been doing CPAP trials for the last 2 days and doing really well all things considered. They were able to wean her rate down and her pressure support, which allows her to take more of her own breaths and work out her lungs. Due to being intubated for a month now she has lost a lot of muscle tone, along with having her chest open for 3 of those 4 weeks. It is going to take her a little longer to get back to her baseline which we already expected. We are proud of how much work she is doing but understand it can be a lot all at once so we will continue to take things at her pace and hopefully, we can get this tube out soon.

Her PD line is still healing, and we do have to wait for the wound vac to come off her chest before they are comfortable trialing PD again. The surgical team should be up to replace it tomorrow and take a look at the skin and staples. If everything is looking good, we could have it off in as soon as a week. Once her chest is healed enough, we can start thinking of taking her off CRRT, getting PD going and getting her listed for kidney transplant. We were informed that the team expects her to wait about 1 to 2 months for a kidney. A kidney transplant is also a much simpler procedure then a heart transplant. The hope is that she will be transplanted, peeing, and extubated within 5 days of surgery. It feels like such a long ways away but I know once everything starts falling into place it's going to go so fast.

Since Annabelle has gotten her new heart going home has been very heavy on my heart. I know we are still probably 9 months out at the least from being able to go home but we are closer than we have ever been. With the plan to bring her home as soon as possible we have been working on getting our home ready. Due to water damage this spring we had to pull up the flooring in our basement which led us to finding a cracked foundation and now we have a wet basement anytime it rains. We have had 2 different companies come give us quotes for the repair which now also included some concern about the structural integrity to our garage. With only Luke working for the time being our funds for a project like this are extremely low, but we know this needs to be fixed before we can bring Annabelle home.

I bring this up because I know there are programs, foundations, and assistance available for these types of things, but I have no idea where to start. If anyone has a lead on where I could look, or even a family member or friend who could give us a quote to make sure we are on the right track with the amounts we are being given I would be so grateful. We have contacted two companies and gotten 4 different quotes for things they could do. I have also tried reaching out to Anoka County and am waiting to hear back. I know we have an amazing community behind us that has got to know of some way we can get help. We want to make sure everything is safe and sound when we bring this baby girl home.

If you know anyone, please feel free to let me know so I can reach out or they can reach out to me. Our house is in Anoka County, Ramsey MN.

For now, we keep taking it day by day, thank you for all the love and support and help. We appreciate all of you and pray that Annabelle can be at home with us soon.

Love,
Sidney & Luke

Good afternoon, everyone.

It's about time for another update. There has been so much happening around here and honestly, I just haven't had the energy or desire to update as frequently as I normally would. In my last update I left off with a long list of things to do and unfortunately, we really haven't made many moved towards a lot of them.

Starting off with our one positive, on Monday the surgical team was able to close Annabelle's sternum completely and place a wound vac over the open skin. She has been closed for 4 days now and had her first wound vac change this morning. The skin is healing great, but we do not know if the surgeon will be able to completely close the skin yet or not. They will continue to do bi-weekly dressing changes to see how the skin looks and if enough new skin grows and the surgical team feels confident, they will look into closing the skin late next week. If not, they will let the skin come together and heal on its own.

Along with the sternum being closed they were able to remove all three of Annabelle's chest tubes. The berlin canula sites have also been closed and are healing amazing. Her skin has been doing so much better since the transplant and the team is optimistic that her scars will heal very nicely.

Now let’s get into everything else that's been going on.

Since the transplant Annabelle has had issues with her blood pressure. Her MAP (mean arterial pressure) should be between 55 and 70 but at times she had been as low as 35 or as high as 120. These swings in her blood pressure happen when she is being assessed but also were happening randomly with no external stimulation. The team was struggling to keep her within her goal MAP and were constantly going up and down on her EPI and NOREPI. It was discussed many times at rounds what could be causing such drastic swings but there was no clear answer.

Next Annabelle started experiencing huge temperature swings, being as low as 34 to as high as 38 in a matter of a day. The team worried that she might have some kind of infection and started treating her with antibiotics and steroids again. While none of the cultures have come back positive, the clinical appearance of Annabelle has improved so I feel like I can safely say something was bothering her. She also have large parts of the day where she was thrashing, delirious, and upset. These episodes meant more sedation and more drugs to try and capture her to keep her safe.

Yesterday afternoon after an extremely tough day managing her blood pressure MAP and temperature the team decided to put on EEG again to monitor Annabelle's brain during these episodes to make sure there was no seizure activities. The EEGs were on for about 8 hours and the Nuro team confirmed today that they saw no seizure activities during the episodes that they captured. They did see some delay in her brains response but that is to be expected with all the drugs she is on and how sick she has been her whole life. They did mention that once she is in a stable spot before we go home, they will want to do an MRI to check that they haven't missed any brain injuries.

Our biggest issue this week has been the HD line that they are using to do her dialysis. Due to her thrashing and being upset quite often this week the line has been having problems clotting off. The issue with this is there is no other way to run dialysis without this line. The team did try to use her PD, but it is believed that the suction from the wound vac on her sternum caused the peritoneum to pop and leak PD fluid into her chest cavity. It is recommended that the peritoneum is given at least 2 weeks to heal before trying to be used again. We did turn down the suction on the wound vac in hopes it wouldn't cause any additional holes in the time being.

In the last 4 days her dialysis machine has gone down 5 times. Each time she is off dialysis for around 2 to 3 hours. This causes problems with her becoming too fluid positive since she doesn't pee. When she is fluid positive her blood pressures are harder to maintain and there is more pressure on her heart which is still having some difficulties on the right side. All these things alone are manageable but when everything is happening at once it becomes a daunting task. The team is doing their best to keep the line healthy and running but each time they have to restart the machine there is a risk of losing the line access completely. I have talked with the team and made my wishes clear that I do not want another HD line placed. Annabelle has very little access left and her only place still open is her right leg. The issue with her right leg is due to the pulmonary embolism that happened last year her tissue and blood flow to that leg have been severely affected and the likelihood that the HD line would last is low. I don't see any good coming out of trying to place a line in that leg when we know it is already compromised.

So, for now we will continue to TPA the line and try to keep it healthy as long as we can while we wait for the PD line to heal and for Annabelle's chest to close. We ask for prayers that Annabelle can remain stable to continue to heal from her transplant and get her blood pressure and sedation to a good spot. We pray that she remains safe and that we have no more risks of brain injury.

Love,
Sidney & Luke

Good evening everyone,

I'm here to share another update, please bear with me while I try to organize all the things that have happened this weekend.

Last updated I had mentioned that her chest was scheduled to be closed. Well big shocker that didn't happen. The team was not comfortable attempting to close her chest with her numbers being high. They also did an echo that showed decreased function of the right ventricle. While an echo on an open chest in difficult in of itself, the decreased function had the team extremely worried. We were told they were worried about rejection in the heart and wanted to get her scheduled for Cath lab and biopsy asap.

Normally post-transplant kids will get a Cath lab with biopsy to check for rejections at 2 weeks. Due to everything weird going on with Annabelle the team decided to move the Cath lab up to Friday. We were told the results likely wouldn't be back till Monday but that they would start her on a high dose steroid and raise her immunosuppressants to help fight any rejection that could be happening.

The Cath lab went well, Annabelle remained stable and comfortable the whole time. Her pressures in the heart were more stable than the team expected and gave them the impression that we might not be dealing with sever rejection. There was more concern that the right ventricle was being compressed due to fluid/the chest wall along with some possible rejection. The Cath lab also showed that the left ventricle, left atrium, and right atrium all had great function. The team continued to treat as if there was rejection just to cover all bases and keep Annabelle safe, but we were relieved to know there could be another answer for why the right ventricle was struggling.

Side note, because I know the average person is not super well versed in all things pediatric transplant. Rejection can be treated. It can get worse, and it can be better. At any time in a transplant recipient’s life they can deal with rejection. Rejection is measured in stages, 0, 1R, 2R, and 3R. Obviously we would love to never deal with rejection but that likely isn't going to happen. Annabelle also is a bit of a complicated case due to her kidney failure. With a normal heart transplant patient, the team would use three immunosuppressants, Thymoglobulin, Tacrolimus, Cellcept. Due to her needing a kidney the team held the Thymoglobulin because that will be used with the kidney transplant. If they were to use Thymoglobulin, we would have to wait for up to six months to get a kidney transplant. They teams hope is that we can manage Annabelle's antibodies with only Tacrolimus and Cellcept until she receives her kidney.

Due to them holding the Thymo everyone has been extremely on top of all her levels, but I also believe that this is why everyone is so jumpy about the decreased function in the right ventricle. While we are so grateful to have everyone watching her closely it has caused increased stress for us. We knew that this transplant was only the beginning of a very different journey, but I don't believe we truly understood how traumatizing the first 6 months of Annabelle's life were to us. Seeing her paralyzed and intubated with alarms going off by the minute and at least 10 people in and out of our room is a lot. Simply put its a lot of everything all at once.

Over the weekend while we waited for the results to come back, we got word that the preliminary results of the biopsy that was done showed 1R rejection. We were told this wasn't the final results but either way they would keep her on steroids an extra 3 days and keep working on the immunosuppressants. This wasn't the news we wanted to hear especially since we know her crossmatch was negative, and she clinically has improved since transplant. I took some time to talk with her transplant doctor and he explained that this was very treatable, and he would tell me if we needed to worry. He was very open and positive that this level of rejection is manageable and could very likely be due to the fact she didn't receive Thymo after transplant and that she is on CRRT for dialysis.

While the news of the possible rejection hit hard, I was reminded that clinically Annabelle looked great and was improving daily in her levels, vent support, and fluid balance. I took all these as small wins and tried to let go of that fact that we still had a very long way to go before we would see her awake again.

This morning at rounds there was talk of trying to close her chest as long as her pressures allowed, and she tolerated it. Around 11am they were able to close part of her chest. She experienced a couple hours of lower blood pressures but with fluid and a little EPI she was able to level out and has been doing great. They also removed her paralytic, and she has been morning around a bit more. Still very sedated but it's been nice to see her twitch a hand or foot every once in a while.

 The final results from the biopsy also came back confirming the 1R rejection. Hearing that news this afternoon did sting a little but after the talk with her doctor on Sunday I feel like we did everything possible to treat it. I also see that she is continuing to improve clinically so worrying about it helps nothing.

Our next big steps are to get her chest closed completely, get back to using our PD drain for dialysis, remove her breathing tube, and turn off all sedation. That is a LOT of big steps, and we will likely be working on them for a month or so but for now we are happy to take the small steps forward.

Please continue to pray for Annabelle and that she can keep getting stronger and healthier. We will update when more things happen.

Love,
Sidney & Luke