ASD

We Didn’t Know

Alexander Day 1

The more heart families we get to know, the more I think about a simple division that exists for parents in our situation; those who knew about their child’s condition before they were born, and those, like us, who did not. Alexander was born in May of 2009, and as time passes and I see new families step into the shoes we wore six years ago, I find myself giving a lot of consideration to those early days after Alex was born. I remember it being very difficult at times to sort out my feelings. The analogy of a balloon comes to mind. All the joy, excitement, and pride of becoming a father again lifting the balloon off the ground, but this time fighting against a giant weight called CHD that houses feelings of confusion, fear, and despair, and prevents the balloon from lifting as fast as it should. The passage of time also allows for some internalizing and self discovery. I don’t think I realized it at the time, but it turns out I was angry. I think my family and friends would tell you I’m a pretty calm guy. I actually don’t remember the last time I got really mad. I don’t like conflict, and typically try to solve problems through compromise and consensus building instead of arguing a point. I don’t like feeling angry. But it turns out, I was, and here’s the strange thing… I wasn’t angry at the world for putting us in this situation, I wasn’t even angry at God for implementing this plan without running it past me first. I was angry because most of the other parents we spoke with knew this was coming, and we didn’t.

For a long time, we just accepted that Alexander’s CHD was simply missed during the routine prenatal Ultrasound. After all, the heart of a baby is a tiny little thing, and surely the differences between a healthy heart and a CHD heart are small and subtle when seen through sound. After a while though, things began to suggest that Alex’s condition was more conspicuous than that. Right Atrial Isomerism is very complex from a physiological standpoint. It’s one of those CHD’s that bring with it multiple complications, and lots of fun acronyms (ASD, VSD, DORV, TAPVR, etc.). There are also several anomalies present not related to his heart. His liver and stomach are reversed, and he’s got no spleen, just to name a couple. Surely some sort of flag should have gone up in the Ultrasound right? Looking back, things started to hit home when Alexander was moved to the NICU at our local hospital for evaluation a few hours before being transferred to SickKids for the first time. The sonographer performing the echocardiogram was very honest. “I’m not qualified to offer any feedback. I don’t understand what I’m seeing”. If it was so obvious just after Alex was born that something was really wrong, why didn’t they see it before?

Well, it turns out some signs were present. During our first prenatal ultrasound the results stated that the pictures of the heart were “unclear”. They needed to schedule a follow up after he developed a bit more to get a better look. I’ve often wondered if the initial pictures were in fact, unclear, or if the radiologist simply assumed they couldn’t be right. We’ll never know. It was during the second ultrasound that it appears the mistake was made. “We got a clear picture, and everything looks fine…”

It wasn’t until after Alexander’s Glenn/Coles procedure that we eventually decided to ask the blunt question. After one of Alex’s cardiology clinic appointments, we simply asked his cardiologist. “Should someone have seen this coming? Should we have known about Alexander’s condition before he was born?” Our doctor paused, I think considering his response. He then deferred to the cardiology fellow shadowing him… “What do you think?” he asked the other doctor.

“In my opinion, the physiology of the top part of the heart could have been missed in a routine prenatal ultrasound. However, under no circumstances should the single ventricle physiology, or the configuration of the pulmonary veins have been missed on a follow up ultrasound focused specifically on the heart,” he said.

Our cardiologist agreed. He then asked us a question I’d never really considered.

“Now that you know, you need to ask yourself… what would have changed?” he asked.

It was such a simple question, and I should have had an answer. I know that Alex would have been born at Mount Sinai and immediately moved to SickKids, but in the end everything worked out okay with him being born in Newmarket too. So what would have changed? Why was I angry about something I really hadn’t properly considered?

“We would have been prepared,” was all I could come up with. Our doctor smiled, likely at the absurdity of my response. We wouldn’t have been prepared, nobody can be. Sure, we might have been all set up logistically. Grandparents would have been on call, time would have been booked off from work, maybe we would have had a pediatrician all set up… but prepared? I doubt it.

And what about all the countervailing “what ifs?” What if we’d been assigned a different cardiologist? What if we never met Alex’s amazing pediatrician, who we actually pretended to know just to get an initial appointment? What if the added stress to the end of Michelle’s pregnancy had resulted in something catastrophic? What if we’d been told Alexander was unlikely to survive?

I admit, I assess these from the position of a parent whose child has done extremely well given the complexity of his disease. It’s easy to look back without regret given that Alexander has, so far, exceeded even the most optimistic predictions. I also realize now that not knowing was likely the better situation for me personally. Months of additional opportunity to consult Dr. Google about Alex’s diagnosis would not have been a beneficial experience.

It’s okay that we didn’t know. I might feel differently if we had, after all I doubt anyone has ever said “I wish we’d been surprised,” but I’ve accepted the way things worked out; in large part due to the conversation we had with our cardiologist that one day in clinic. What would have changed? Probably nothing. What I have trouble accepting is that for a long time I defaulted to anger, and even jealousy that other parents knew about their child’s CHD ahead of time, and we didn’t. It sounds ridiculous, I know.  I’ve given a lot of thought to this over the years, trying to understand the reason why. All I can come up with is that it’s for the same reason parents snap at nurses sometimes, or fly into a rage when a custodian rearranges the furniture in the CCU waiting room, disrupting the temporary home base they’ve established for themselves. It’s a simple reality that we’ve all faced at one time or another.

It’s easier to be angry than scared.

There’s one more reason I’m okay with how things worked out for us, and it’s not something I’ve shared before. When people ask how we cope with Alexander’s CHD, I’ve often said that Alexander’s condition has just always been there, that he’s always been our heart baby. That’s not entirely true. I hold dear a photograph that immortalizes a memory of my young family sitting in our hospital room in the hours after Alexander was born. It was only for one day of naive bliss, but for that one day Alexander was simply our beautiful new baby boy. His older sister was not the sibling of a heart baby, we were not heart parents. We had no idea what was to come in the next 24 hours. I don’t regret what became of that family; in fact I treasure our experience since more than anything. But I also treasure the memory of that day, and the perfection of that single moment in time, a moment made possible because we didn’t know.

Matthew
Matthew is a regular blogger for Cardiac Kids click here to read his bio

Check back next week for a post from Tara 

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Tyson’s Story

Congenital Heart Defects (CHDs) are the world’s leading birth defect; more common than spina bifida or Downs Syndrome, and are the leading cause of infant deaths in Canada. CHDs kill twice as many children each year as all childhood cancers combined. The term “congenital” means that the defect is present at birth.   One in 100 to 3 in 100 Canadian children are born with one or more of the 35 known types of CHDs, representing 1-3% of births. These heart defects vary in severity, ranging from a tiny hole in the heart that may never require surgery, to life-threatening defects which require open-heart surgery within hours or days of birth. Sadly, more than 4,000 babies born this year in Canada will never see their first birthday because of complex congenital heart defects, and every year hundreds of CHD children die before ever reaching adulthood.

Our family’s CHD warrior Tyson is one of the fortunate ones, who has been blessed with five birthdays so far. Tyson was born in November 2008 with complex CHDs. Even after two prenatal ultrasounds, he remained undiagnosed until he was 10 days old. He was born in the comfort of our own home, with my husband and our two midwives in attendance; all of us unaware of how sick our son’s heart really was. We had no idea that our world was about to be turned upside down.

Immediately after he was born, Tyson began having problems. He had a hard time keeping his body temperature stable, he never woke up to eat, and he slept 7-8 hours through the night. The red flags were there, and blood tests were run to check on other things, but nobody thought of checking his heart. He was even seen by the hospital pediatrician at one day old, but the symptoms at that time didn’t directly point to his heart, so he wasn’t concerned. A few days later Tyson developed bad jaundice and was increasingly lethargic. Bilirubin levels tested over the next few days all came back in the normal range so nobody really knew what was wrong.   At 6 days old, we noticed he was breathing really fast as he drifted off to sleep, but we wrote it off as something we thought most babies did from time to time. At eight days old, we were tired of worrying, and he was increasingly sleepy and lethargic, never opening his eyes or spending any time awake, and not latching well to nurse, so our midwife suggested a trip to the local hospital for some phototherapy to treat his jaundice. Blood tests were run once more and his bilirubin levels again came back normal – a little on the high side but not overly concerning – so the ER Dr was prepared to send us home. We were certain that something was wrong with our baby and insisted that we see the pediatrician. The pediatrician came in, and within less than five minutes of examining Tyson, he whisked our baby away into the trauma room, put him on oxygen, and told us “You’re right, your baby is not going home tonight,” and told us he needed to undergo some tests. The next time we saw our newborn son, his head was shaved at the sides and we saw two IV lines running from blood vessels in his head, because his arms were too tiny to find a vein. A spinal tap was done, and various tests were run. The conclusion was that our eight day old baby was in heart failure and his other organs were beginning to shut down. A preliminary echocardiogram at our local hospital revealed that he had “a rather large hole in his heart.”

From there, Tyson was transported via ambulance to Toronto’s Hospital for Sick Children, where it was discovered that his condition was much more serious than a large hole in his heart. He was born with four severe CHDs: Hypoplastic Right Ventricle, Tricuspid Atresia, Transposed Great Arteries, Coarctation of Aorta, as well as an ASD and VSD. In short, he’s missing the tricuspid valve which caused a complete under-development of his right ventricle (basically, he has half of a heart.) In addition, his main arteries are mixed up, his aorta is narrow, and he has two holes as well.

We were told that Tyson would need to undergo a series of at least three open-heart surgeries in the next two to three years of his life, the first one being scheduled in a matter of days, once the surgeons could organize his schedule. The goal of the surgeries was to re-plumb his heart, so that the under-developed right side of his heart would be essentially by-passed, leaving the left side to do all of the work.

Tyson underwent his first open heart surgery at 13 days old, spent three weeks recovering at Sick Kids, and made it home at just over one month old; just in time to spend Christmas with his mom and dad, two older brothers, and one older sister.

 

4 week pic
Four weeks old

The winter months at home were extremely busy; Ng tube feeds, low-fat portagen formula every 2 1/2 hours round the clock, pumping breast milk, cleaning up the aftermath of reflux problems, and administering the seven different medications that Tyson had to take.  The medications were to strengthen his heart, prevent fluid retention, help with reflux, and prevent blood clots since he now had a Gortex shunt placed in his heart. The blood thinner used to prevent the clotting was an Enoxaparin needle which we had to inject into his legs twice a day, morning and night.

If that wasn’t enough to keep us hopping, Tyson was re-admitted three days later for an infection in his sternal incision. Once home again, we had to go for weekly appointments at Sick Kids, then we graduated to bi-weekly, and then monthly check-ups. This was on top of his regular appointments to the pediatrician, regular appointments to his family doctor for his immunizations, and monthly doctor’s appointments for the Synagis injections to prevent RSV (a virus that causes very serious respiratory tract infections.)

Despite our attempts to keep him in a bubble, Tyson struggled with his health through much of his first winter because of his compromised immune system. At four months old, his life was spared again when he was hospitalized because of a serious pneumonia infection, causing him to be intubated and in Intensive Care at Sick Kids for almost two weeks.

In May 2009 when he was five months old, Tyson had his second open-heart surgery, after which he developed another pneumonia infection. After his second surgery, his health was much better; he still seemed to catch quite a few colds but thankfully, he avoided any hospital stays for about ten months.

His third open-heart surgery was in December 2010. During this surgery, the surgeons at Sick Kids attempted to re-plumb his heart so that the left side would do all the work since his right side was effectively useless. They also attempted a repair on his left pulmonary veins, the veins responsible for carrying oxygen-rich blood from his lungs back to his heart. These veins are dangerously small and are only carrying about 18% blood flow to his heart. Unfortunately, this surgery didn’t go quite as they had planned and they were unable to repair these veins as well as they’d hoped. After several cardiac catheterization procedures to measure his pressures again and attempt a fenestration closure, it was decided that there are no more surgical options left for Tyson besides a heart & lung transplant.   So for now we are on a ‘watch and wait’ plan of action. Right now his heart function is borderline, but he continues to grow and thrive despite his many obstacles.

20102011 Winter 206
Tyson, 2 years old. Three days after his third open heart surgery.

Today, Tyson is an energetic five year old who is enjoying Grade One. He is developing almost normally and enjoying all the things that typical five year old-boys love: soccer, books, Superheroes, and Ninja Turtles. He tires more easily than healthy children, and his medication prevents him from doing some activities, but overall he is living life as normally as can be expected. Unfortunately, because of the severity of his heart and lung conditions, Tyson’s biggest struggle is recurring pneumonia, and he has had more hospital stays due to this than most kids with his condition. We make use of all kinds of alternate remedies to boost his immune system and keep him healthy, and of course we have a strict hand-washing policy in place in our home, but there is only so much that we can do to keep him healthy. It seems that whenever he does catch a cold, the virus attacks the weakest part of his body, his lungs.

Having a child with severe heart defects has taught us not to take one single day for granted, to enjoy every possible minute with Tyson that we can, and to appreciate every day as a blessing from God. Every morning we wake up and are thankful that Tyson can enjoy another day with us; another opportunity for us to love him and care for him, and to see the laughter and joy that he brings to our lives.

We are often reminded that if Tyson was born in 1978 instead of 2008, there would have been nothing that they could have done for him as an infant and we would have had to take him home under compassionate care. But over the last twenty or thirty years, there have been so many advancements in medicine and technology that doctors have been able to come up with ways to prolong the lives of children with severe congenital heart defects.

Even in the early 1980’s when these types of ‘palliative’ heart surgeries were first being performed, survival rates were very low and many babies did not make it. Now however, more and more babies are surviving the first and most risky surgery called the ‘Norwood’, usually performed within days of birth. Then they are able to move on to the ‘Glen,’ typically performed between 4-6 months of age, and the ‘Fontan’ performed between 18 months – 3 years of age. Even still, Hypoplastic Right or Left Heart Syndrome is responsible for 25% of all cardiac deaths under one year of age. With those kinds of odds, we spent hours in prayer, begging God that Tyson could beat those odds, and asking Him to spare his life.

Over time cardiologists and surgeons are perfecting the techniques used to perform these risky surgeries. More and more children are making it into adulthood, resulting in a growing population of young adults who require life-long cardiac care. According to the Canadian Congenital Heart Alliance for Life, there are an estimated 100,000 adults with CHD across Canada (outnumbering CHD children.) At least half face the prospect of complications, multiple surgeries, and premature or sudden death.

In Ontario, there are roughly 40,000 adult CHDers, as well as 40,000 CHD children. However, there are only seven cardiologists who are formally trained in the care of adult CHD patients, and only four surgeons with adult CHD expertise. There are far fewer resources allocated for the care of adult CHDers than other cardiac patients, resulting in long wait times for clinic appointments and surgeries. This puts these adults CHDers at greater risk of death.

There is still so far that we have to go to raise awareness of congenital heart defects. Many people aren’t aware of the dangers of CHDs, and their cause is still unknown. Some are known to be associated with genetic disorders such as Down syndrome, but the cause of most CHDs is unknown. Often prenatal ultrasounds are not sufficient in diagnosing the problem. If a woman is at higher risk for carrying a baby with CHD (for example, if she already has a child with CHD,) she should have a fetal echocardiogram done to determine whether or not there are cardiac concerns. As was the case with our son, I had a routine prenatal ultrasound at 20 weeks pregnant, but was sent for an additional ultrasound since the first one didn’t give an adequate view of my baby’s heart. Ironically, this repeat ultrasound reported that our baby had a healthy, four chamber heart.

Since CHDs often go undiagnosed prenatally, I believe that funding should be made available for all babies to have a pulse oximeter reading shortly after birth. This would allow the doctor or midwife to monitor the baby’s oxygen saturations in the blood, and therefore aid in early detection of many congenital heart defects. In this way, hundreds of babies’ lives can be saved. I cannot explain how or why Tyson was able to live undiagnosed for 10 days, since his CHDs are so severe that he could have died within hours of birth. But I do believe with all of my heart that it was only by the grace of God that he is here today, and I now that He has big plans for our little heart warrior.

I am 1 in 100

 

Melissa

Melissa is a regular blogger for Cardiac Kids. Click here to learn more about her and her family.

Check back in 2 weeks for a post from Caitlin.

Sources:

http://www.cchaforlife.org                                                www.heartandstroke.com

www.congenitalheartdefects.com