Jacob

Jacob Christopher Wilkins 
5/27/2015 - 1/19/2021 

Hypoplastic Left Heart Syndrome was the diagnosis we were given on January 13, 2015 when we went for an anatomy scan to find out if our little peanut was going to be a boy or girl.   Big sister and big brother were with us, ages 7 and 5.  We all entered the CHD world together that day. And our lives were changed forever.  I was initially angry at God, confused and fearful. But we were determined to do everything possible to give Jacob the best outcome.  I saw a ‘Faith over Fear’ sign that night and it resonated with me.  I tried to adopt that mentality.  The worship song ‘Oceans’ became a battle cry.  Shortly before Jacob’s birth we moved from Colorado to Georgia where our family lives.  

Jacob was born on May 27, 2015 in Atlanta and moved to Children’s Hospital of Atlanta a few days later.  Oh, to not be able to hold our baby or have him in the same room was heartbreaking.  To see other babies and mamas on the unit made me tearful.  I kept asking God ‘WHY?’ Daddy split his time between the hospital I was at and CHOA until I was discharged. Our other children were with family members about two hours away.  

Jacob was fortunately stable enough to not have surgery immediately following birth.   His Norwood, the first of 3 palliative surgeries for HLHS,  was on June 3,2015 at one week old.  His heart was the size of a strawberry.  

That word palliative.  Pay attention to it.  It does not mean cure.  It means dealing with the symptoms and providing relief so as to improve and extend life for patients.   There is no cure for CHD, the most common birth defect that impacts more than 1 in 100 children every day. And their families. Every procedure is considered palliative and anyone with CHD requires life long care. 

We were discharged June 24 to go home.   We were seen by pediatricians, home health, cardiologists, and therapists multiple times a week prior to his next surgery at the end of September 2015. The time between Norwood and the next surgery, the Glenn, is highly critical. And many children end up hospitalized as things can deteriorate quickly.   We were blessed that we did not have this experience.  

We went in for a pre Glenn heart cath procedure at the end of September 2015.  Jacob did not tolerate the cath well and ended up being admitted emergently. We thought we would go home following the cath and return for the Glenn a month later.  But this is how quickly things can shift for someone with a CHD.  His Glenn procedure, the second palliative surgery,  followed a few days later.  The recovery was rocky.  It took a few days to extubate.  The CICU became like a war zone to me.  It was during this stay that the words from Psalms became real to…”Children are a gift from the Lord…”.  I recall sitting beside JACOB’s bed and physically opening my hands and telling God that I realized Jacob was His first. I wasn’t giving up.   I was trying to recognize His will over mine.  I realized I had no control over the outcomes.   None of us who enter this world of CHD do.   All we can do is show up, with grit and determination, ask for God’s grace and advocate for our children.   We went home on October 11.   At this point I felt like these stays were long.  Any stay with your child feels long.  Little did I know how short they really were.  

We worked through therapies the next few months, we re-established a routine and enjoyed the time until his Fontan at the age of 4. Jacob was a typical toddler with no outward signs of health issues. If you saw him, you would not have known he had half a heart. I saw the scars everyday. They were part of who he was. They were a sign of courage and hope.

I recall when he turned 1. It was a miracle to us based on all the stats we were given. And we felt like we could breathe a little easier. When Jacob turned 2, we returned to Colorado. The altitude didn’t impact Jacob, which was a concern because kiddos with HLHS have a lower oxygen saturation rate than everyone else. Many kids with varying CHDs do. We live and breathe by that number. Literally. It’s maddening.

The time for the third, and what we hoped, the final surgery arrived when Jacob was 4. The Fontan procedure. In July 2019, we drove from Colorado to Kentucky where his surgeon from Atlanta had taken a job. We had a pre Fontan cath that would determine if he was even a candidate for the next surgery. If children with HLHS do not have a large enough pulmonary artery he would not be a candidate for the surgery. This is just one of many roadblocks that can occur. Fortunately he was a candidate and we proceeded with the Fontan two days later. We were out of the hospital relatively quickly and returned home about three weeks later. And we thought we were in the clear. If only.

June 12, 2020 found us in Omaha for a heart cath procedure. In the middle of COVID. On that day we learned that Jacob was in acute heart failure and were immediately admitted on medication with discussions of heart transplant beginning. We went through the evaluation for transplant only to learn that Jacob had too many antibodies and would most likely have acute rejection. The antibodies were from blood products received during his previous surgeries. The very surgeries that were absolutely critical were now becoming a roadblock for transplant. We weren’t given many options and in the midst of this Jacob had a cardiac arrest and was placed on ECMO. We attempted to take him off a week later and ended up having to put him back on due to another cardiac event. We were in a very dark place without a lot of hope. We started reaching out to other institutions and were directed to Lurie in Chicago. Lurie accepted Jacob - on ECMO. Lurie is one of few hospitals that will transplant kids who have high antibodies. We just had to find a team to get him there. On July 18, a team from Texas flew Jacob from Omaha to Chicago -on ECMO - where our journey to transplant began.

Jacob received a Berlin heart a few days after arriving at Lurie. This is a ventricular assistive device connected to a machine that takes over heart function. It is a bridge to transplant. Look it up. It allowed Jacob to come off ECMO only to learn that he had suffered a stroke somewhere along the way. This was hard to see because he had been on heavy sedatives and paralytics. He began therapies for feeding and motor activities. The stroke had only impaired his motor ability. He made amazing strides, was running around the unit, racing with a doctor while she walked on her hands, and being a normal 5 year old (all the while connected to a machine that only allowed us to be out of the room 20 minutes twice a day). We received the gift of a new heart on October 21, 2020. We were absolutely ecstatic. Heartache and hope intersected in a way that we never experienced before. To know that one family was saying good-bye and giving life to our baby boy was the most surreal experience.

It was a 15 hour procedure. Transplant is not a cure. Due to Jacob’s antibodies, he had to go through a desensitization regimen using chemotherapy drugs for about two months. He was placed on ECMO immediately following transplant. It was scary. He came off ECMO about a week later. He had monthly cath procedures to measure rejection. His kidneys were impacted from the length of time on bypass during transplant and ECMO. He was placed on dialysis. We weren’t able to extubate. But overall we were happy because his heart function was good. No signs of rejection. We were thankful. The rest we could deal with. But the devil that is CHD has a way of creeping in. He was tired. His other organs were compromised due to multiple bypass surgeries, single ventricle circulation, and ECMO. He was at a high risk of clots due to the multiple times his veins had been accessed for ECMO. All of this was being managed via medications. We were 7 months into our hospital stay. His chest and tummy were covered in scars. I started to question what scar his belly button was. Only to realize it was his belly button. The very place he had been connected to me. The place I would have let him stay forever because it meant he was safe.

We decided to have a trach placed and started discussing options for something other than continual dialysis that would allow us to go home and begin intensive outpatient rehab. And on January 13,2021 everything shifted again. Literally 6 years to the day we had received his HLHS diagnosis. He had a pulmonary embolism that led to a cardiac arrest. He was placed on ECMO for the fourth time. Four code calls, 4 ECMO runs. His brain suffered terribly. And we knew. We knew he was tired. We knew God would take him home. And heal his heart for eternity. Jacob was our glimpse of heaven here on earth. He taught us so much. He taught us to be thankful everyday. To not take for granted the small details. That boring is good. We said good-bye to our beautiful boy on January 19. I took the heart stickers off his chest and told him to go be free and whole and kissed him once more. Daddy kissed him once more. His older brother and sister made imprints of his hand and a memory box. And their friends helped them. Yes, other kids my children's age joined them in their grief that very day at the hospital. They are amazing kids. Not many adults can join someone in grief. And they did. A lesson to be learned. Sit with people in their grief. It was an absolute blessing. Jacob fought with a determination and resilience that was and is inspiring. His big sister always said he just rolled with things. And he did.

I share our story to raise awareness. The most common birth defect that impacts babies is the most underfunded. It has the least amount of awareness and a very low detection rate compared to how common it is. These babies and families deserve more. They deserve the best that the medical community has to offer. They deserve advocates and voices. Palliative care is what we have now and it has come far. But we need a cure. CHD is a thief and creeps in quietly, stealth like. Parents who are fortunate to receive the diagnosis at the 20 week anatomy scan need hope. Not termination as the first option. ‘Would you prefer comfort care?’ don’t need to be first words a surgeon says to a family because the surgeries are risky and complex. The Norwood is the most complex surgery performed on an infant. Necessarysurgeries shouldn’t become a possible obstacle for a transplant. Every heart warrior’s story does not end up the way we beg God for. I begged God to heal Jacob on this side of heaven. We also prayed for a whole heart. Which he now has for eternity. But that does not mean the story is not good. I know that Jacob presented many ‘firsts’ for the team at Lurie. If those firsts lead to hope for another child then that is good. That is grace. That is a step forward. It is my family's mission to advocate and raise awareness. As we come out of our grief, I am not sure what that looks like yet but we will find it. And we will hope and we will be a voice. We will carry Jacob with us forever. Our courageous lion. He had courage. He was loved deeply. And will be forever. Heartache and hope intersect again, but in a different way. Heartache in the saying good-bye to our beautiful boy and hope in his healing for eternity. Heartache and hope. It is the constant theme in the world of CHD. And we will continue to advocate for more hope.