This is written with the hopes that one day, when we see light on the other side of this tunnel we’ve been placed in, we can show our sweet girl how far she has come and how proud we are of her fighting spirit. So, this site is dedicated to her fight and to all of the family and friends in her corner who loved her and prayed over her from the very beginning of it all.
Kendall’s Diagnosis
Kendall was born with a severe and complex congenital heart defect called Hypoplastic Left Heart Syndrome (HLHS). This is a condition that is usually diagnosed in utero but in Kendall’s case, it was not, so we went into this journey thinking we were going to be taking home a healthy baby girl and our biggest hurdle would be figuring out how to deal with sleep deprivation and handling diaper duty as first-time parents. Now we would give anything for these types of things to be our only challenges. After a 29-hour labor and a relatively smooth delivery, we met our sweet girl for the first time and everything first-time parents say is true - it's a love at first sight you can never explain. She received perfect scores on all her tests and Travis and I both got to have some skin-to-skin time. However, within the hour after she was born, she started to turn blue and our world flipped upside down. Long story short, she was transferred to a more advanced NICU at Cooks Children in Fort Worth and I'm forever grateful Travis was able to go with her even though I had to stay behind in the hospital and receive updates throughout the night from him and her many cardiologists and neonatologists. Eventually, we learned about the devastating HLHS diagnosis. With HLHS, the left side of the heart is not formed properly affecting oxygen-rich blood flow to the rest of the body. In Kendall’s case, she presents with the following issues: - The left ventricle is underdeveloped and too small. - The mitral valve is narrowed, blocking sufficient blood flow into the heart’s main pumping chamber, the left ventricle. - Aortic valve stenosis (narrowing) is present, blocking sufficient blood flow into the rest of the body. There is also coarctation present in the aorta, causing even less blood flow to the lower half of her body. - She has a ventricular septal defect (VSD), which is a hole between the left and right ventricles of her heart, and allows the mixing of oxygen-poor blood and oxygen-rich blood and forces her heart and lungs to work harder to remove waste from the blood.
Current Plan of Action (as of April 4th 2022)
With Kendall’s diagnosis, she will need to undergo a staged heart reconstruction, consisting of a series of three open-heart surgeries to reconfigure her heart to go down from two pumping chambers to one and re-route the circulation so her heart can properly do its job. Currently, Kendall is in the NICU and the predominant concern is monitoring her blood oxygen saturation levels to ensure proper oxygen-rich blood to the rest of her body. She is on medication to allow the right ventricle to provide this blood flow to the body. Because she is on a ventilator, has a PICC line, arterial line, and a million and one other things stressing her poor little body, she is pretty heavily sedated and of the many, many things that are hard about this, for me, I would just love to be able to see her little eyes open. I’ve only seen them open one time and that was directly after birth when they placed her on my chest for a few minutes.
3/1/23 Update: Kendall is currently not eligible for the Glenn or Fontan operations due to severe pulmonary hypertension and extremely small pulmonary arteries despite months of different treatments, an extra open-heart surgery, and continuous oxygen support. The working side of her heart will continue to get weaker and weaker and the shunt that was meant to hold her over for a few months will remain her lifeline. She is not eligible for a heart transplant and if she was, she would need a heart/lung transplant, which is extremely rare. We've been told it's important to preserve quality of life as 5-6 years is a good goal for life expectancy.
Stage 1: The Norwood Operation
Her first surgery, the Norwood, is scheduled for Monday, April 18th, when she is a little over a week old. During this surgery, the team will build her a new, larger aorta using the pulmonary valve. Because this pulmonary valve will go from the right ventricle to the body, a shunt will be needed to take blood to the lungs. Assuming all goes well, she will recover in the Cardiac ICU for 3-6 weeks before she’s allowed to come home with us until her next surgery. The doctors have been very forthcoming with us about the challenging nature of this surgery, so we are fervently praying for her to have a chance and hope others will join us in these prayers. While prayers are needed for Kendall’s health and safety and wisdom for the doctors first and foremost, we’d also appreciate prayers for our sanity during the waiting throughout the 10–12-hour long surgery, waiting in the critical 24–48-hour window where she comes off the heart/lung bypass machine and her heart adjusts to this change, and her recovery time. We are also terrified to bring our fragile baby girl home after being in the care of so many amazing doctors and nurses.
Stage 2: The Glenn Operation
While we haven’t even tried to comprehend much beyond her first surgery, the second surgery, the Glenn, will take place when Kendall is 4-6 months old. The Glenn procedure makes blood from the upper half of the body go directly to the lungs, so blood can pick up oxygen without passing through the heart. This takes away some the extra work from the right ventricle as up until this procedure, it was pumping blood to both the lungs and the body.
Stage 3: The Fontan Operation
The third surgery, the Fontan operation, will take place when Kendall is 2-3 years old. It will make blood from the lower half of the body go directly to the lungs, and at this point, the right ventricle has become the main pump and can send blood out to the body so that there is no more mixing of oxygen-rich blood and oxygen-poor blood.
Our Prayers
With these three surgeries, if 100% successful, it is likely Kendall will survive to adulthood and live a full and somewhat normal life. There are concerns about the lifelong struggles HLHS patients face as a result of having a heart with only one pumping chamber. This could include lung, liver, gastrointestinal diseases or problems with neurodevelopment and while we are devastated to learn our baby girl will most likely not have the quality of life we dreamed about for her, we are focusing on one day at a time and one milestone at a time. However, as we pray for Kendall’s survival, please join us in praying for no long-term complications that might impact her quality of life.
We are crushed, we are in a phase where this is feeling all very unfair as she is a beautiful, innocent baby who has only known pain and suffering. Our souls are heavy and we are tired, both physically and emotionally, but we will fight to the ends of this earth for Kendall and do whatever it takes to make sure she knows joy, love, excitement, and to eventually see her open those beautiful eyes and smile. We won’t pretend to understand why this is happening to her, but faith that God walks with us is the only thing we’ve got right now. Please join us in a fight for Kendall’s life and pray with everything you have.
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Nothing is ever super straightforward in the heart world and we saw that again today with Kendall’s heart cath results. But first and foremost, Kendall’s doing well. She tolerated the procedure well even though she had some ballooning done in both central branch pulmonary arteries (PAs) and in her shunt, which had shrunk from 5mm to 4mm. Luckily, they were able to open the shunt back up to 5mm without any issues. However, her central branch pulmonary pressures were slightly worse than we saw in her last cath back in June despite being on the continuous Remodulin infusion for the last 5 months. Not at all what we wanted to hear as this doesn’t give us any surgical options to help Kendall and further solidified the team’s 4–5-year outlook as a “best case” scenario. That conversation will never get any easier no matter how many times we have it.
After we got to join Kendall in her room in cardiac stepdown (yay for not being admitted to the CICU! - though after seeing Kendall’s name on the procedure list, the CICU was all prepped and ready for her as they know she likes to keep everyone on their toes…), I got a call from Kendall’s pulmonary hypertension specialist at Children’s Dallas. She was pleased with Kendall’s distal pulmonary pressures out into her lungs, which is where Remodulin is most effective. In June, those pressures were 19-23 and they are now 10-12 on both sides. The talk about the distal pressures was relatively new to us as we have always been focused on the central branch pressures.
Unfortunately, the improvement in the distal arteries still doesn’t really change the game plan since the central branch pressures are still way too high. The central branch PAs are just too stenosed from an anatomical perspective for the pressures to decrease. The only thing that could help is another open-heart surgery to try to augment the central branch PAs, but we’ve already been down that road and it didn’t help.
Next Steps: Kendall’s cardiologist will discuss her case with the larger cardiology group at Cook Children’s to get a group consensus on Wednesday, but I think we’re looking at just continuing to do a cath every 9 months to optimize things as best they can and try to get the most longevity out of her shunt as possible. We’ll hopefully have a better update (or at least a more clear update) after the conference on Wednesday.
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Monday, March 25th - Before heart cath #6
Monday, March 25th - Resting after heart cath #6
