Handling It Like A Champ - Tyler Cohen Wallace

~by Jennifer Wallace

When I found out I was pregnant, it was one of the happiest days of my life. My husband and I did everything new parents do when they first learn they are having a child. It was a miracle, until our 20-week ultrasound that changed everything.

We were told our baby had clubfeet, his left hand wasn’t opening and they suspected he had some urological issue. What could it be? What was wrong? The Doctors told us these could all be unrelated and could be fixed after birth. Distraught and yet hopeful, we tuned out the doctors, gave up on further testing and hoped for the best.

Tyler’s delivery was a long 48 hours of labor, and as soon as he was born, everyone in the delivery room could see something was wrong. He was immediately whisked away from us for a full work up. My placenta did not deliver and I began to hemorrhage losing almost two liters of blood. Hours later, exhausted, I finally got to meet our son for the first time in the PICU. I couldn't hold him, but I could see how adorable he was, and how peacefully he was sleeping.

In the middle of the night, the doctor woke me with the news that Tyler had to be rushed to Sick Kids for cardiac surgery. I tried to get out of bed, but was too weak and in too much pain. I never got to hold him or even kiss him goodbye. I called Eric and told him he needed to get to Sick Kids to meet the doctors immediately. Neither of us knew what to expect.

When I finally got to hold Tyler it was a magical moment, but short lived. His Clubfeet were severe, his hand was closed and his chest was concave. Swaddled he looked so peaceful, but naked he was so frail. It was heartbreaking.

Before we could bring Tyler home, he would have to undergo an inguinal hernia repair and what should have been routine, only led to more speculation. The Surgeon told us his tissue was very difficult to stitch – he likened it to a wet tissue. What was going on with our son? We were frightened of what the future held for our son.

The Cardiologist was quite confident Tyler had a rare connective tissue disorder called Loeys-Dietz Syndrome and Tyler was one of the most severely affected cases he’d seen with a very high risk of fatality. It was if a bomb went off in my head. We took our baby home and tried to settle into life with a newborn, while desperately searching for answers.

Tyler's first MRI imaged his heart and part of his spine. Cardiology had been tracking a PDA aneurysm and it was reaching a dangerous size, which meant that surgery was imminent. Both the MRI and CT imaging of his cervical spine deemed it was too high a risk, so at six weeks old, Tyler went in for a routine PDA ligation surgery. An hour into the procedure we were told that the aneurysm had already started to rupture, and Tyler would have to be put on a heart lung bypass machine so they could remove the aneurysm. After many grueling hours, the surgeon reported that Tyler was okay. Finally, something positive!

At four months old, we received his confirmed diagnosis of LDS with a TGFBR2 mutation. For the next year, every three months Tyler underwent an echocardiogram and ECG, and every six months an MRI. In the meantime, doctors started him on Losartan in an attempt to slow down the rapid growth of his aorta, but it was still enlarging.

In June of 2012 we attended the Loeys-Dietz Conference in Baltimore and met other children and adults with LDS. It was nice to talk to people who understood what we were going through, and witness first-hand the quality of life they were enjoying. We knew it wasn't going to be an easy road to travel, but now we had found companions to share our experiences with.

I’ll never forget the first time we met Dr. Loeys and Dr. Dietz, whose research discovered LDS. We had two pages of questions prepared but didn’t get to ask any of them. Dr Dietz walked into the room somberly and told us he was very concerned about Tyler’s heart, "It is not a matter of if he dissects, it’s a matter of when he dissects." he said, and advised us to arrange surgery upon our return home.

Surgery was booked for the end of August. It was a very scary two months of waiting, knowing that he could dissect. At 15 months of age he was being prepped for open-heart surgery. They replaced his entire ascending aorta with a Dacron graft. The anesthesiologist told us the aneurysm was as large as his fist and if it had ruptured, it would have killed him instantly. To this day I still feel the hairs on my arms and neck stand up when I think about that.

We got a surgical reprieve for about a year but Tyler's condition was replete with complications and over the next few years he would endure multiple surgeries. At the age of two, a sleep study revealed severe obstructive sleep apnea requiring an adenoidectomy as well as urological and dental surgeries. At the age of three, Tyler underwent one of his most difficult surgeries to correct a problem with his kneecaps and achilles tendons. Although his clubfeet had been corrected when he was younger, he had difficulty walking and required full leg braces.  

As difficult as these surgeries were, they changed his life. Today he is able to run independently; jump and play just like all his friends; sleeps much better and is always smiling and happy. We still have our struggles but are amazed every day at how resilient he is and how well he copes with his condition.

Tyler is now five and requires a g-tube to assist with the treatment for Eosinophilic Esophagitis – a condition that makes it difficult for him to eat. That makes 12 surgeries in his short five-year life but he is still full of life. We still have another surgery planned for the New Year and are hoping he can have a long reprieve after that. Through it all, Tyler has handled it like a champ.

When we write it all down, we realize how much he has gone through and how far he has come. Almost every system in his entire body is affected by LDS. He presented with low tone; extreme hypermobility of all joints; aortic enlargement; bilateral club feet; floating knee caps; pectus excavatum; webbed fingers and a bifid uvula, just to name a few.

LDS is a multidisciplinary syndrome where each patient can have a varying degree of symptoms. Tyler has almost every manifestation ever written about and some have been added based on his presentations. He is followed by 16 Specialists between Sick Kids and Holland Bloorview in the areas of Complex Spine, Plastics, Cardiology, Respirology, Urology, Endocrinology, Orthopedics, Nutrition Clinic, Feeding Clinic, Eye Clinic, Dermatology, GI, Dental, Genetics and Complex Care as well as his Pediatrician which makes Tyler a very busy guy.

In spite of everything we are humbled and comforted by how brave and resilient Tyler is. As difficult as the surgeries were, they changed his life. He goes to school, has friends, plays, runs, jumps, and enjoys life like any other child his age. He is the sweetest and happiest little guy, always smiling. Many people have told us there is something special about him and we couldn’t agree more. We are truly blessed to be his parents and couldn't be prouder of all the odds he has beaten. 

We are grateful for all his therapists, doctors, nurses, surgeons, and hospitals that have saved his life on countless occasions and are thankful for foundations such as GADA Canada, the Loeys Dietz Syndrome Foundation and The Loeys Dietz Syndrome Foundation of Canada who have helped educate and support us on our journey. As Tyler’s parents, we have learned so much medical information. Knowledge is power. 

Organizations such as GADA Canada play a huge role for patients and families. They are a trusted source of information. It’s essential to donate to these organizations in order to support research, find better treatments, more effective medications and improve quality of life, as well as extend the lifespan of patients.