An Aunt's Perspective on Congenital Heart Defects

When I couldn't find myself able to write for CHD Awareness Week, I went where I always go--to my sister. She has been a rock for me over the last couple of years. I know if I need her, despite living 6 hours away, she is here for me. Always. No questions asked.  And she was here for me today.  Please read the Editor's perspective.  CHD doesn't just stike the child born with a broken heart. It doesn't just hurt the parents and siblings. CHD affects the entire family.

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I don’t remember M.O.L. first telling me about being pregnant with DQ or when she and the Mad Scientist found out that DQ was a girl (I know she called and told me these things, but I don’t remember). What I do remember is standing in my kitchen talking on my phone and hearing my big sis break down in tears because her baby was going to be born severely sick. I didn’t understand a word of what she told me; all I knew was that there was something majorly wrong with the unborn girl that we now know as the Dancing Queen. Since I live 6 hours away from M.O.L., I felt helpless. My big sis who had always been my protector throughout my life had just been given the biggest blow from which I could not protect her—her unborn child was sick—and I couldn’t even give her a hug. I realize now that even if I was in the same room as M.O.L. when she delivered the news, I would still feel helpless. I had no idea what this illness was; I had no idea what the future would hold; I had no idea how to help my sister.

Even though DQ’s birth was a planned cesarean, I was not there; instead, I had to wait for the call that she was born and then hopped on the road the first chance I got. The first time I met DQ, she was a couple of days old and in the NICU; only two people were allowed to visit at a time, and at least one of those people had to be either M.O.L. or the Mad Scientist. I went through the long scrubbing routine required before entering the NICU and slowly walked to the room, feeling scared out of my mind because I still didn’t know what to do or say. I saw my sis and went and gave her a hug. I saw DQ in the little plastic hospital crib with all the wires and leads on her little body. To be honest, at that point, it didn’t hit me how sick she was. My own baby, The Chef, had been hit hard and hospitalized with pneumonia for an 8-day stint when he was 2 months old, so the wires and leads didn’t seem all that terrible. I knew intellectually that she was very sick, but I didn’t really feel it. The first time DQ’s illness hit me a little was when she had her first heart surgery at 7 days old. I don’t think I was there during the surgery; I think I was watching TRex. I do remember seeing her (now in the PICU, where there were more nurses per bed) after the surgery—she was so small and had even more wires and a breathing tube. I didn’t like seeing her like that, but I also didn’t want to leave her. I had to head back home to get back to work, so I visited DQ one last time in the PICU. I really didn’t want to leave. I hated that I lived 6 hours away. What if I never got to see this little girl again? But DQ is a fighter; we saw that early on. I don’t know all the stats, but I do know that we could have lost her at many points.

As time went on, my family and I got to visit some weekends, so we started to learn a little bit about the everyday routine to care for DQ. I saw the tons of syringes that were filled with various medicines; I saw the ng tube that went through her nose down to her tummy to nourish her. Most importantly, though, I saw the beautiful smile of my niece, DQ. She’s always had a way to suck me in with that smile.

When the time came for DQ’s “full-fix” surgery, I took a week off of work to be there. I again felt helpless but figured that maybe by being there instead of 6 hours away, I could be of some use. I mostly watched TRex while M.O.L. and the Mad Scientist were at the hospital. Little Car Guy, The Chef, and I tried to keep TRex occupied and having fun since he was too little to understand why his mommy and daddy weren’t with him—well, that was my thought process; my boys just wanted to have fun with their cousin.

I’ve tried to get out there as much as possible when DQ has needed a procedure of some sort, including another full week with DQ’s third open-heart surgery. There are times, though, that all I could do was pray from home and be available via phone. When I’m stuck at home, I feel helpless, but when I’m there in my home state, I still feel helpless. To this day, I don’t know what to do or say because there’s nothing I can do or say to make it all better. I can pray, I can be on the other end of the phone and listen, and I can cherish every minute I do get to spend with DQ and the rest of my family. None of it is ever enough, though. I feel like a constant failure to my family because I can’t do enough to make anything better. I long to be back in my home state, and a lot of that longing is because I’m not able to take the time out to visit on a random weekend—I don’t get to spend enough time with DQ. M.O.L. doesn’t know this yet, but Car Guy and I just made the decision this week that we’ll be moving back to my home state over the coming months. It still won’t be enough to live closer by, but maybe I can take more of the burden off of M.O.L. and the Mad Scientist. I’ll never be able to remove it completely, though.

Throughout this journey, I’ve learned more about CHDs (nowhere near the amount of knowledge that M.O.L. has accumulated, but at least a little). The stat that still shocks me the most is that nearly twice as many children die each from CHDs as from ALL forms of childhood cancers combined; yet funding for pediatric cancer research is five times higher than funding for Congenital Heart Defects. Why is it that it took my niece being born with a severe CHD before I started to hear anything about CHDs? Why don’t other people know more? I see commercials and have coworkers constantly participating in walks and other fundraisers for one type of childhood cancer or another. I don’t want the work with pediatric cancer diminished, but I want more awareness of CHDs and more research money for CHDs. It may be selfish, but I want DQ to have every chance she can get because I’m not ready for her to be gone. I won’t be ready for that until I’m looking down from heaven and Miss DQ is the ripe old age of 80 or so. I also want her to be able to play with my kids and have fun like a normal kid without swelling and coughing because her heart can’t keep up. I want some new answers from researchers. I want to stop feeling helpless.

Why I Can't Do What I Want for CHD Awareness

I had every intention of starting heart week off by giving my tips for advocating for your child--strategies I've used, methods of persuasion where you don't look like a raving lunatic, but an engaged, well-informed member of the team. Yah, that was my plan.

I can't do it. This heart week has been hard for me.  I can't put my finger on it, but it has been much, much harder.

Apparently, my emotional baggage is greater this year. Each story I read makes my heart hurt a little more. Each time I post something on FB, a friend comments and I realize just how many people I know who have lost their child to CHD.  I counted--about one out of every ten of my heart friends has lost a child to the demon that is CHD. While I fight most days for CHD awareness so that someday it won't be one in ten, right now, it is scary and sad and emotionally overwhelming.***

Children whose stories I have followed for years are very, very sick. My heart breaks for their families. I want to do something or say something, but there is nothing. I've been on this road long enough. I've seen miracles happen and kids get better against the odds, but I also know, many times, there is nothing.

All of these awareness stories are touching my heart so much this year. Perhaps it's because  CHD is all encompassing in my life still.  CHD won't leave us alone.

I've read so many stories of kids having their "fix" and going one to two years between cardiac appointments and then finding all is good at the check up.  I want that! There are many kids who don't take any medicines after their fix. I want that! There are kids who aren't reminded daily that they were born with a broken heart. I want that!

Here we are, four years in, "fixed" and "revised", and still presented with constant reminders that CHD controls our lives. Each morning, for the last couple of weeks, I wake a sleeping DQ, too tired to get up (despite 11.5+ hours of sleep) and face swollen. She has not been my happy girl. And later, each morning, her fingers tips and lips turn dark blue, almost black. I know, she is getting worse, but I can't articulate it. It petrifies me.

I have longed for the day when DQ can just be a regular girl. I mean, I treat her the same as I do TRex. I send her to school. I punish her and let her scream and cry at me (in her room) for 45 minutes if she doesn't behave. I let her play until she tires, but when it comes down to it, DQ tires out after a couple of minutes of running. And no matter how "normal" we make taking 10 medicines a day, DQ still takes 10 medicines a day. We can't escape it.

And while I don't want my daughter to be defined by her CHDs, it is hard to escape. I can't pretend nothing happened. Frankly, DQ isn't fixed. She won't be fixed and she will always have limitations.  Since she tends to ignore her limitations to her detriment, we have to remind her.  She will dance, run, and play until she is coughing. We've been told not to let her do that. How can you tell an almost 4 year old "you have to stop playing now" without an explanation? Not possible! And I'm not going to lie to DQ. So when she asks "why?", I have to tell her "your heart and lungs can't handle it, we don't want you to start coughing."

So, this heart week has been hard. I read so many wonderful stories of odds being overcome and it makes me so proud of these mighty warriors. But it also makes me sad and a little jealous because of where we still sit. Then I read the heart wrenching stories of loss and I fear the worst. But I'm so grateful for these strong women sharing their warrior-angels to help the greater good. And I feel guilty and sad and happy and grateful that my child survived and she is living life to the fullest.

At the core of it all though, the CHD Awareness stories leave me sad. Every story this year is making me sad. So, please forgive me for not doing as much for CHD Awareness as I would like. And please see Stefenie's blog, where she is strong enough to tell the heart stories, to spread awareness, and link to others who are stronger than me.



*** I know several years ago, the odds were one of ten CHD babies would not make it to their first birthday. I've heard that statistic has changed for the better through research. I don't know the new statistic. I just know my personal experience. Please understand that the odds are not 1 out of 10 babies die from all CHDs, but my heart friends tend to have children with complex CHD or complications from their CHD, which is not as easy to survive.

A Glimpse

Below are some to the questions and things I hear in a typical week from the Dancing Queen. A sorta glimpse into what it means to have an almost 4 year old with complex heart and lung disease and 22q11.2 deletion syndrome.

Mommy, we can't walk to the candy store. The cold air makes me cough and my chest hurts.

Translation: Between the high pulmonary pressures and asthma, when the Dancing Queen is out in the cold for more than a couple of minutes, her lungs can't handle it.

Mommy, can I see videos of myself as a baby in the hospital?

DQ always wants to look at videos and pictures of herself as a baby, in the hospital.  She always points out when she sees baby DQ with a pacifier. This is one of the hardest things she asks the Mad Scientist and I to do. The pictures are painful reminders.

Mommy, I get my army, it's just one little poke. It doesn't hurt a lot, only a little bit.

 Translation: Her weekly Hizentra infusions that are done via subQ button (small needle that only goes into the skin). DQ gets donated human immunoglobulin once a week in her belly or leg. The needle stays in for 2 to 2.5 hours, but it only takes one poke.While DQ says it doesn't hurt a lot, when it comes time to give the "one little poke" she fights us.

Mommy, Daddy always says "a double shot of enalapril."

Mommy, I need my lasix to help me pee. I need my aspirin to help my heart.

Translation: DQ takes about 10 medications a day. At breakfast every morning as I give DQ her morning meds, she tells me these things.  My husband always uses two 1mL syringes to give enalapril and I use one 6 mL syringe; hence double shot of enalapril.

Mommy, I need to eat, sleep, play, and take my medicines so I can grow big, big, big.

 For some reason, this is DQ's normal topic of conversation on the way to dropping TRex off at latchkey in the morning.

Mommy, I'm little because I don't eat. If I eat, I'll be big like TRex.

Translation: DQ rarely eats as much as she should. It is a constant struggle and quite the opposite of her brother.

Mommy, Mickey is sick. I'm taking him to the hospital. His sats are 35. He is almost dead. He'll be better in a week. 

 Translation:  DQ and TRex playing with their animals. Mickey Mouse was sick. It doesn't matter the character, but someone is always sick, they always go to the hospital, they always get shots or surgery.

Mommy, my army kills the bugs, but I still have to take yucky medicine. That's not like my yummy medicines. 

Translation: Yucky medicine=antibiotics; yummy medicines=enalapril, lasix, aspirin, calcium carbonate, calcitriol, and prevacid.

Mommy, why do I have heart disease?

 When we wore red for heart disease, DQ wanted to know why she had heart disease. I had to explain that she is missing a piece of chromosome, so her body didn't know how to build her heart properly. That wasn't a satisfactory answer, so she asked me over and over and over again. Finally, I told her I don't know. She wasn't happy with that, but accepted it.

Heart Month

February is Heart Month. We try to raise awareness of Congenital Heart Defects (CHDs) this month. Since today is "National Wear Red for Heart Disease Day", our family is doing our part.  Both kids plan to tell people they are wearing red for heart disease and our favorite heart warrior, the Dancing Queen.

Today, I'm also featured in Heart Mom interviews for CHD Awareness Month on When Life Hands You a Broken Heart.  Please follow me there.