Hello!
My name is Sidney, my husband Luke and I are parents to a very special heart warrior named Annabelle. For heart month we want to share her story and help bring awareness to CHD. Our story is filled with the highest highs and the lowest lows. We currently live on the CVCC floor of Childrens Hospital while we wait for a heart transplant for Annabelle. Here is her story so far.
A little background on Miss Annabelle and where we are in her journey. Annabelle was born 6/29 and came home on 7/1 a seemingly healthy happy newborn. Within the first 2 weeks we would struggle with feeding, have multiple doctor visits including a lactation specialist. We tried multiple different routes to get her eating, but she continued to have slow weight gain and struggled to meet her feeding goals.
On August 15th her doctor recommended we go to Children's Hospital for observation to help them better understand her feeding struggle and how we could help her gain weight. Upon admission Annabelle was observed taking her regular bottles of 1oz to 1.5oz without much fuss. We saw speech therapists, lactation specialists, and a cardiologist, all who were determined to help us get home and back to normal life. An ultrasound was done of her chest to try and locate a possible reason for her small feeds/fussiness. That ultrasound revealed that her heart was enlarged and struggling to pump. We were then informed by a cardiologist that she would be moving up to the cardiovascular floor for more intense treatment to try and help her heart as she was seriously struggling.
We were in shock. How could our seemingly normal baby have a problem with her heart? We asked so many questions, "How was this missed?" "Why didn't we know sooner" "Could we have prevented this?" All these were met with unknowns or simply more questions.
Around 5pm on August 17th we were moved to our new room and doctors got to work placing ivs and a central line to get the medication needed to help our little girls heart. It was the longest day of our lives. Due to being dehydrated and uncomfortable it was very hard for the team to place the IVs and Annabelle was so upset. Her heartrate was dangerously high and her O2 stats dropped many times. She was placed on oxygen to help supplement her and once her lines were placed, she was immediately given pain medication and sedation to help calm her.
We were informed she has dilated cardiomyopathy. Her heart was enlarged, and the ventricles were weak and struggling to pump blood out of the heart and lungs. Leaving her with fluid in her lungs, causing her little body to be working overtime. This is something she was likely born with that would no correct itself and would eventually require a heart transplant to save her life.
We met with the transplant team during our first week in the hospital and were quickly started on the transplant evaluation so we could make sure Annabelle was eligible for a transplant and be listed as soon as possible. Through this evaluation we discovered that Luke has a genetic mutation that was given to Annabelle, and they are 2 out of 10 people known to have this mutation. Annabelle is the only person currently genetically tested with this gene mutation with Cardiomyopathy. There is still so much unknown when it comes to genetics so we can't say for sure this is where her cardiomyopathy came from but for now, she is quite literally one of a kind.
Annabelle was approved and listed for transplant on September 8th 2023. It was such an emotional day; we finally were able to take a deep breath as we settled into what could be a very long wait. We were told it could take between 6 to 9 months to get the call, even more if there were complications. We were informed that there was a possibility that she would need a surgery before the transplant to place a VAD (ventricle assist device) called a Berlin Heart. This device would help support the left side of her heart while we wait since it was already so much weaker than the right side of her heart.
After 3 weeks of little improvement with meds and Annabelle still struggling to get to full feeds, despite a feeding tube being placed, we started discussing her VAD surgery. It was decided that her heart needed the help and on September 26th 2023 she went for her first open heart surgery. The surgery took just over 10 hours to complete and we were a ball of nerves the entire time. We were updated every hour or so that she was handling everything great and the surgery was going exactly to plan. We took our first deep breath that night when we saw her again, she was so small and hooked up to so many devices but we were reassured that she was doing great. The doctors were watching her closely and she was showing them she was a fighter; even thought she was still struggling. Her urine output became almost zero and her berlin was struggling to fill and empty. Her doctors made the decision to take her back again not even 12 hours after her first surgery.
The following days would include 3 more open heart surgeries, another VAD being placed on her right side and so much waiting. After what felt like a lifetime Annabelle was settled into her room, a RVAD and LVAD supporting her heart, her chest still open due to the swelling, and a breathing tube. It was so hard watching her lay perfectly still for days on end due to sedation. We held our breath while we waiting for her to start waking up. After another week we were able to start see her open her eyes, her chest was closed and it felt like we were finally on our way to recover and waiting for a new heart.
Three weeks after her RVAD was placed her team decided to take her for a surgery that would help widen her pulmonary artery. They had been seeing lower flows and filling in the RVAD and assumed that was because of the size of the cannulas and it pushing up against the wall of the artery. Unfortunately, this surgery would leave her with pulmonary edema and on ECMO do to the damage done to her lungs by being over flooded with blood from being on bypass. She spent 24 days on ECMO before they were able to take her off for a trial that lasted 5 days. She was emergently put back on ECMO due to O2 stats in the 30s and work on breathing. She remained on ECMO for another 37 days before her body was stable enough to take over the work on her lungs again. During her ECMO stint she had 4 bronchoscopy procedures to help clean out clots from her lungs that was impeding her ability to breathe. Due to the clot burden from the pulmonary edema and her enlarged left ventricle her lungs were only at about 50% over the course of 8 weeks. Thankfully her last bronchoscopy removed a huge clot that was blocking the lower 25% of her right lung and since then she has had much better O2 stats. Her team is optimistic that with the continued work on strengthening her lungs we can see her extubated before transplant.
In November, we received more bad news. Annabelle’s kidneys were failing and without immediate intervention she would likely die from the flood overload and waste her body was not able to expel. We prayed that her kidneys would start to recover with the medication and treatments we were trying but say little improvement. After four days it was decided that she needs to be started on dialysis right away to help her body remove the fluid and waste. We were informed while on dialysis Annabelle would not be eligible for transplant as the anti-rejection meds that they are put on after surgery are extremely hard on kidneys. There for going into a transplant with already damaged/failing kidneys was not an option. We cried and prayed for some sort of light. We couldn’t simple give up and say no to dialysis even though we knew it meant she wouldn’t be able to accept a heart.
We accepted that dialysis was the only way to help her body even if it meant temporarily removing her from the transplant list. Within a few days we were able to see improvement in her labs. She started to be less puffy and able to open her eyes again without being in pain. Her skin started to be less shiny and stretched. The dialysis was working! We were happy and sad all at the same time. Happy something was working, sad because it confirmed her kidneys were damaged and we would need to figure out how to heal them before a transplant. If they weren’t able to heal, we talked with our doctors about a heart/kidney transplant. Unfortunately, they do not do heart/kidney at Children’s so the topic of being transported to another hospital has been talked about. Our hopes are to see improved kidney functions in the coming months that will give her team an idea if she will be able to accept a heart.
While we waited for her kidneys to heal the team continued to work on her lungs and getting her stronger and bigger incase she needed to be transported. She was doing PT and OT almost every day. We were finally able to hold her again after what felt like a lifetime. She was started on feeds again to try and help her grow and keep her stomach active. Her team was happy with how stable she was after what had seemed like a very up and down journey. Everything was going well until December 22.
On December 22 Annabelle experienced a pulmonary embolism after a clot on one of her VAD cannulas traveled into her heart and blocked 95% of her pulmonary artery. She was taken to an emergency Heart Cath Lab where they tried to stabilize her and remove the clot burden from her heart. She was gone for 6 hours and when her surgeon came to talk to us, he was very serious about the complications that could arise from this event. We were informed that she was without good cardiac output for over 35 minutes. There was no way to tell yet what the impact would have on her body. She showered clots into her legs, which were likely cause the tissue in her legs to die and break down. There was also a high likelihood that clots had traveled to her brain, and she would have brain bleeding. We were gutted. In a matter of hours our hopes and dreams of taking our daughter home one day were ripped from us. Our doctors sat us down and told us the likelihood of her being okay after this was slim. They prepared us to be ready to say goodbye and that while they would do everything they could, there might be too much damage. She was placed on EEG to monitor her brain activity for seizures and anything else abnormal. We spent the night holding her hand and telling her how much we loved her. There was very little we could do but wait and see what happened next.
The next morning, we were informed that no seizures had been captured on the EEG and that her head ultrasound came back normal. The relief and disbelief was felt by everyone! Her doctors were shocked to see her awake and moving around as if nothing had happened. Her tests and labs came back relatively normal, and she seemed in good spirits. It was unexplainable but we didn’t care. She was alive and fighting to show us that she was still here. The EGGs were removed, and her team continued to monitor her but over all she was doing very well. Her leg would need some intense wound care, but it was believed that she would regain normal function.
This little girl has been through setback after setback since the beginning of her heart failure journey, but she has also shown us time and time again that she is a fighter. Her wait for a new heart continues to be met with challenges and victories every day. On her bad days we hold her close and support her in every way we know how. On her good days we celebrate in every way possible. We celebrate monthly birthdays with treats and decorations. Her room is decorated with her bows from home, blankets for every occasion, clothes and toys and so much more. We have been in patient for 179 days with little to no end in sight so anything we can do to make this room feel like home we do.
I hope by sharing Annabelle’s story for heart month we can bring awareness to the amazing things these babies do every day. We constantly say how strong Annabelle is and that she is our little superhero. We hope one day soon to be able to share her complete transplant story with the world, until then we continue to pray and support this little heart warrior through every challenge she faces.
#AnnabelleStrong