Doctors

1 in 100

1 in 100…Times three. In my family, there are three of us born with CHDs, myself, my nephew, and my daughter, Amelia. Amelia is my cardiac kid born with complex congenital heart defects in May 2009.

My awesome nephew, Aidan, has a CHD that is electrical in nature, it’s called Wolff-Parkinson-White (WPW). In WPW, there is an extra electrical pathway between the atria and ventricles. A symptom of this syndrome is a rapid heartbeat. When he was around 3 years old, he started telling us that “his heart was beating” and our natural response was “that’s right, your heart is beating, good for you, what a smart kid”. Over a six month period or so though, he started mentioning his heart beating a couple of times like this. It wasn’t until he said it during no activity, my sister put her hand on his chest and could feel the rapid heart beat. She knew something wasn’t right and brought him to the emergency room at Sick Kids. In an incredibly fortunate coincidence, the ER doctor that examined Aidan that day had a 5 month old child that had recently been diagnosed with the same syndrome and spotted it right away. As with most people with WPW, he lived relatively symptom free with a few episodes of tachycardia yearly. At around 8 years old, he started taking medication but that seemed to make him more lethargic and out of breath. When he was around 17, his episodes started to increase in frequency and it was decided that he would go through with a heart catheter ablation that would destroy this extra pathway, with a success rate of about 95%. He was 3 days shy of his 18th birthday when he had the catheterization done at Sick Kids. Being able to have this procedure done at Sick Kids played a huge role in the decision to have it done at all. Any heart surgery or procedure is frightening so electing to have one is an extremely scary decision to make. What made his decision easier was his baby cousin, Amelia. Amelia was almost 2 at the time of his procedure and had already had more heart catheter procedures than I can remember, that’s not including surgeries. He gave himself the “suck it up” pep talk and was wholly inspired by our little trooper. It’s been 4 years since his heart catheter ablation and 4 years since he’s had an episode.

As I mentioned, Amelia was about 2 years old when Aidan went in for his procedure. That means I had also just spent about half of those two years in and out of Sick Kids, and a lot of time in that cath lab. So as an amusing side note, when I showed up to the recovery to see Aidan, the nurses instantly recognized me but scanned the beds looking for my little girl not expecting me to be there for the over six foot tall man, with his feet hanging off the bed. Also amusing is that his one complaint coming out of the catheter is that his ankles were hurting for that very reason.

Our sweet baby girl, Amelia was born with DORV (Double Outlet Right Ventricle) along with other heart defects that often accompany that diagnosis like AVSD, holes between the atria, holes between the ventricles. She also has mitral valve issues. Her condition is by far the most complex of our three diagnoses, requiring the most intervention, follow up, monitoring, lifelong care. In other words, completely terrifying, with an unknown outcome. What DORV means is that both her pulmonary artery and her aorta were attached to the same ventricle when each of them should have their own. One of the “fixes” for this defect is to do just that, attach one of the arteries to the other side but because of the other heart issues like the mitral valve problems, they could not do this type of surgery. They were always fairly certain that they were not going to be able to do this but they couldn’t know for sure that this wasn’t going to be an option until they actually went into her heart and took a good look. Again, what a terrifying thought. With the feeling that they were not going to be able to do the switch, we were told that Amelia’s best option would be to move towards a univentricular heart, ignoring the fact that she has that left ventricle at all. She has had three surgeries to “repair” her heart thus far, a PA (pulmonary artery) banding, the Glenn, and the Fontan. We don’t know, long term, what that will mean for that left ventricle as it shrinks inside of her not getting used.

Before my daughter was born, I hadn’t given much thought to the fact that I had heart surgery as a baby. I didn’t know the statistics of 1 in 100. I didn’t know because my case was a relatively common and easy “fix”. I had surgery in 1980, at the ripe old age of 2, and have had no follow up since. We didn’t ever talk about it growing up beyond anecdotal stories and there was no internet or Dr. Google back then for me to do extensive research of the topic. I had a PDA (Patent Ductus Arteriosus) closure. If you happen to google PDA, it’s third on the list behind Personal Digital Assistant and Public Display of Affection, can’t be that serious of a defect, right? The DA is something that usually closes within the first few weeks of life, it’s one of those things we need in utero but as soon as we start breathing air, it’s no longer required. Normal blood flow is affected if it does not close and my family doctor heard that through a murmur. The same family doctor that heard Amelia’s thundering murmur as well. As I saw when strolling the halls of the cath lab, they do the PDA closures there for the most part. I couldn’t believe that the surgery is done through cath when I have a scar that traces my entire left shoulder blade – check it out. It’s obviously much more faded now, 35 years later but still very visible. Now the procedure leaves no scar at all, unbelievable. Oh, and my surgery was done at, you guessed it, Sick Kids!

3 - #rockyourscar 2 - tarahomefromsurgery

I plan to eventually write about Amelia’s surgeries here as well. I have many notes from those days but in all honesty, I fear for the safety of my laptop when I start reading them over again, it is not waterproof afterall. The Glenn was the worst of the three surgeries for her which leaves me with some of the most traumatic memories from our many stays at Sick Kids. One of the lines I repeat over and over again about the first 3 ½ years of Amelia’s life is that her time in hospital recovering from surgeries is something she’ll probably never remember and something I’ll never forget. At least she won’t remember the same things I do about her stay. Since I have a basis for comparison, I was 2 when I had my surgery, there are 3 things I remember from my stay:

1. waiting in line to have blood drawn and dreading it

2. a nurse scolding me for taking another child’s scissors in the playroom (or possibly for just      having scissors, I was 2 after all)

3. my daddy brought me a pint of strawberries (this one seems sweet but it was traumatic because he told me he had gone strawberry picking for them which meant to me that he had gone strawberry picking, without me, while i was stuck in the hospital. Having fun without me. It wasn’t until many, many years later that I realized he was just kidding about having gone, ha)

That’s us, three CHDers, one family. I mentioned to a CCU nurse one time that I had had heart surgery as a child and she asked me if it made me feel better about Amelia and her condition, knowing that I came through it okay. I remember replying that no, it did not, it made me feel guilty and responsible for causing this to happen to her somehow. I know logically that is not true and I’ve been told time and time again by doctors and nurses that is not true but that’s how I felt at the time. Now that we’re almost 6 years into this journey with Amelia though, I can say that having been born with CHD as well does make me feel better. Amelia and I can share a sense of camaraderie through our battle scars or as she said to me the other day, “me, you, and Aidan are the lucky ones in the family because of our special hearts”.

1 - 1in100

Tara

Tara is a regular blogger for Cardiac Kids. Click here to read her bio.

Stay tuned later this week for a post from Heather 

Compassionate Care

Today I want to tackle a subject that has haunted me for many years.  When my daughter was born with HLHS, we were given three options, a heart transplant, three open heart surgeries or to do nothing and let her die, also called compassionate care.  We chose the surgical repair and never did I ever think of the last option until years later when I was once again haunted by these words.  Our journey continued with HLHS and many years after her successful repair I took her to an Oakville hospital to meet her new cousin who was just born.  Once in the hospital one of the nurses came up and knew about my daughter had a heart defect and asked which one.  I told her she had Hypoplastic Left Heart Syndrome and her eyes looked very perplexed.  She then said to me that a baby was born here also who had the same condition.  I asked her why the baby was born here and she pointed to one incubator on the other side of the room and stated that the family did not want to pursue any medical interventions for their child.  At that moment I sat silently and stared at that incubator in the back of the room.  I couldn’t understand why no one was holding that baby and then understood why that baby did not cry.   This beautiful gift would never see the sunshine, would never play on a slide or ever have the chance to survive.  I guess I never thought about this option but if chosen I now know that families can choose to leave their baby at the hospital for their last moments.  I remember leaving and getting lost on the way home.  My mind never stopped wondering who that child was of could have been.  I do not want to open a discussion or argument on this subject but these are my true feelings and it still haunts me as to why.

Many years later one of the teachers at our local school told me someone she knew was expecting a child like Colena.  She asked if I would mind speaking with them and I eagerly said yes.  I never heard anything for a couple of days until she told me that they decided to terminate the pregnancy since they did not feel that they could financially handle it.  Walking home I once again wondered why two children who I never knew came into my world with this situation and why the choices were made.  Current statistic say that HLHS occurs in 3% of life births and I met two families, what are the chances of this. I once again want all readers to know that I am not here to judge these parents and I am not trying to open a discussion on choices, I only want to express how I felt knowing these private matters. I will openly admit that I do not feel that doctors should even offer compassionate care as an option. I have always felt very strongly about this.  In the past the odds of HLHS children surviving with either option were very poor, but that is not the case now.  They do however go through extreme pain and a cautious future but everyday the medical world is finding new answers so they must be given the chance.

One thing I certainly know is that my life has been enriched by not only my daughter but by the doctors, nurses and all the staff that worked so hard to keep my angel with me.  These special children are gifts for all people who surround them.  Rage on Heart Warrior!

Jean is a regular blogger with Cardiac Kids read her bio and Colena’s story click here 

Check back next week for a very special guest post from Kathryn about being a “CHD Sibling”

You’re Doing Great

Lily

There was a steep learning curve when we first became Lily’s mom’s. While we had known from the beginning that Lily had “heart issues,” we didn’t truly begin to understand what that meant until our first appointment at Sick Kids. We met Lily’s foster mom in the waiting room of the ECHO lab and were perfectly happy to let her take the lead, comfortable playing the role of the casual observers. To our shock though she was just as happy to hand the reigns over to us and forced us to jump head-first into our new role as mothers. In the hour that followed, I learned on the spot how to try and comfort Lily during a needle poke, how she preferred to be swayed instead of rocked as she drifted off to sleep and how quickly she could squirm out of the most tightly swaddled blanket. I felt totally and completely out of my element and it wasn’t long before my self-confidence, which had been high after years of hearing how great I was with kids and what a great mother I would be, started to plummet. In the moment where I truly became a mom, I had never felt more like a fraud.

And this is the truth: the learning curve is steep for all CHD parents. Even when you have the diagnosis early: you’ve prepped yourself with all of the reading and research and you’ve reached out to other parents who have travelled this road before us, there is nothing we can do that can prepare us for these first moments. Alongside the “normal” firsts: counting fingers and toes, trying to distinguish between cries, changing diapers while trying not to turn on every single light, you are now thrown head-first into this unexpected world. While I was shocked at how quickly this world became our new normal, standing beside Lily during that first ECHO, I felt as though I had just jumped off the edge of a cliff and didn’t have a clue what was waiting for me at the bottom. Then, out of sound of the wind rushing through my ears, came a firm but gentle hand on my arm and the most incredible words I had ever heard: “You’re doing great Mom.” It was exactly what I needed in that moment, a small bit of encouragement to help me see that I wasn’t a fraud, that Lily and I just had to figure this road out together. We learn fast that we can’t always protect them the way we want to, that we can’t keep bad things from happening. But we also learn, just as quickly, that we can hold them closer, comfort them, find ways to help them work through their own fears and know, always know, that they will never face any of this alone. We don’t always know the right steps to take, the right path to follow and we often find ourselves relying on people to help guide our decisions in the hope that it will help our children survive, but we do know how to bring real life into this medical world; we know how to help them not just survive, but live.

When I think about Lily, at 5 days old, going through her first heart surgery alone, it breaks my heart. While I know that there has always been a “Team Lily”, thanks to the many nurses who stopped in her room on 4D to tell us about how they were there to watch her — spending time snuggling her up while they we were working, on their lunch breaks or even once their shifts were done, there is nothing I wouldn’t give to be able to go back in time and be with her, to experience those first moments right alongside of her. I want to be the voice gently whispering in her ear, “You’re doing great baby” and bring her the same comfort that a nurse named Jane once brought to me.

Crystal

Crystal is a regular blogger with Cardiac Kids read her bio and Lily’s story click here 

Check back next week for a very special guest post from Kathryn about being a “CHD Sibling”