Congenital Heart Disease: Madeline’s Story

Before Madeline joined our family, we were made aware of some very serious and complex medical conditions she would live with the rest of her life. We couldn’t discuss them openly until she was officially adopted, and so our posts about our month-long hospital stay and her surgery were vague at best. Now that she is legally ours, we hope to bring you up to speed. Get ready for a crash course in Cardiology.

Madeline was born with multiple and rare Congenital Heart Defects (CHD). Her most pressing are called Total Anomalous Pulmonary Venous Return (TAPVR), as well as the more well-known Hypoplastic Left Heart Syndrome (HLHS).

Maddie's heart (TAPVR and HLHS)

What does this mean? Most of the structures on the left side of her heart are small and underdeveloped; she basically has two out of the four “chambers” of her heart. More importantly, on their own, her pulmonary veins don’t return the oxygen rich blood that all bodies need to survive. I’ve discovered many thorough, medically detailed websites which describe her conditions well, but the gist of it is she is unable to circulate sufficient amounts of blood to maintain a normal level of oxygen saturation throughout her body without serious intervention.

Thankfully, modern medicine allows these sweet babies with CHD a chance to survive such conditions with an incredibly complex series of procedures to reroute the circulation process. It’s truly astounding what they can do. Her first open heart surgery took place the day she was born. She underwent the TAPVR repair in which they essentially joined the pulmonary veins into a common flow back behind the left atrium. She spent 1 month in the hospital recovering from this difficult procedure. And she was all alone while there. It still makes me cry when I think about that.

The second phase of recirculation is known as the Fontan operation, which is a two stage process.

The Fontan procedure

Just a few weeks after Maddie came into our lives, she underwent the 1st stage, the Bi-Directional Glen (or “the Glen,” for short) which decreases the volume load on her single ventricle.

In the CTICU after the Glen

We are anticipating that she’ll need the 2nd stage (the Fontan) in the near future, but it’s not for sure. It will all depend on how her body continues to handle this change. So far, she seems to be compensating well.

But that’s not all. If it weren’t enough for her to have a faulty heart, she also has other complications. She is asplenic, meaning that she lives without a spleen– a critical organ for fighting infection. And her organs are reversed– situs inversus, if you’re talking to the Radiologist and heterotaxy to the Cardiologist and surgeons. In conjunction, she has dextrocardia, which means that her little heart is on the right side of the chest instead of the left. To say that the surgeons have to be at the top of their game with her is an understatement.

Just to give you a sense of the severity of these defects, first know that an average, healthy body has an oxygen saturation level of 98-100%. When we rushed down to Children’s Hospital for her second surgery, Madeline was “satting” at 67%. Even now, as well as she’s doing, her “normal” range– a range that the cardiologist considers good— is 75 to 85%. I can’t even imagine what that feels like.

For the most part, we don’t allow ourselves to become overwhelmed. We take it as it comes and press forward. But it’s on our minds. We worry about things like her oxygen saturation levels and if she’ll become cyanotic again, whether she has a fever and needs to be rushed to the hospital, or if her pulmonary veins will ever grow. We see the cardiologist every 3 months, and sometimes every other, for an echocardiogram, hoping that her body continues to compensate for its limitations. Maddie takes amoxicillin daily to combat infection and aspirin to thin her blood and relieve the pressure on her poor little broken heart. But those are small interventions compared to what we could be facing. She eats, drinks, and breathes all on her own.

Her heart will never be normal, no matter how many surgeries she experiences. She’ll never have a spleen. We live each day hoping that the defects of her body will never become the defects of her life.

It all sounds so dire and I know that it can be and is for so many families. But to me, she’s just little Maddie. I look at her and see a beautiful baby girl. It’s hard for me to remember that she is sick when I hear her scream heartily for more food, or watch her crawl at top speed to get to a toy she loves, or see her giggle like mad when she watches her big brother act like a goof ball.

Our heart baby

In reality, the current ease of her condition is simply a miracle. There is no other explanation. Most babies living with a CHD as severe as hers are not as fortunate. We are blessed beyond measure that she is functioning so well. It is a constant reminder that she is truly in the hands of our Father in Heaven.

I can’t tell you how wonderful it feels to be able to write openly about Maddie. Though we still plan to leave our last name and other identifying information off our blog, I feel strongly about posting and discussing issues that affect our family so greatly.

And please ask questions. I never tire of informing people and am always grateful to share the message that life goes on despite such trials.

14 thoughts on “Congenital Heart Disease: Madeline’s Story”

  1. She has a lot more going on than I realized. What a strong girl! (And family!)

    And, that’s a great picture. She sure is a looker.

  2. What a good post. I still don’t have the cardio knowledge to understand most of it, but the difference between the two pictures of Maddie is a great representation of what you are saying – that it could be so bad, but somehow she seems just like a regular little kid. We love Maddie and are so grateful she is doing well.

  3. Wow, that photo of her first surgery is heart wrenching. I’m going to blame it partially on the hormones I can’t seem to control, but I started crying. I’m so happy she’s so healthy despite being dealt a tough hand, and even happier she’s a part of your family.

  4. You said it best – It is so astounding what modern medicine can do (By the way, do you feel like you’ve been through a year of medical school?), and it’s astounding what faith can do. And prayers. She is one extraordinary little girl.

  5. wow, I’m so glad you guys are able to pull through this. I had a nephew die of congenital heart disease, so I am familiar with the terrible process, experience, and seeing such a tiny baby full of tubes and needles. Good thing she has you guys.

  6. She is very lucky to have parents who love her and have invested so much in making sure that her spiritual and emotional health are cared for so she can focus her energy on keeping that little body working.

    I loved this post, thank you for sharing.

  7. oh Jeannie, I read the header and didn’t want to continue because I knew I would need some tissue….I finally came back to it and I don’t think any doctor or nurse could’ve written this process so condensed and concise. Maddie is so lucky to have you and Colin as parents. Maddie truly a miracle and much more.

  8. I love that last picture of her! She truly is such a strong one! I’m so glad you guys are in such a good place right now and she’s so happy and I pray it lasts forever!

  9. Oh Jeannnie….How lucky little Maddie is to have a great family. To think that all of that is going on inside of that tiny little body is overwhelming. That picture of her smiling says it all…..I’m cute, I’m strong, and I love life! I’m so thankful she has such a strong family that can teach her the “bigger picture” when life gets tough. What a blessing the gospel of Jesus Christ is in our lives always, but more so when in life we face trials! Love you Jeannie…thanks for posting!!

  10. What a beautifully written story about your sweet baby girl. I came across your page in my research for TAPVR for a class I am taking. Maddie is simply a miracle and as much as she blessed your lives by becoming your adopted daughter, you blessed hers by being such wonderfully loving and compassionate parents.

    I would love to know how Maddie is doing today.

    1. @Melissa, thank you for your sweet comment. Maddie is a little miracle and just continues to amaze us by how well she copes with her conditions. She is actually going in on Wednesday for a heart cath to determine if she can undergo the Fontan. We’re anxious to know. Hopefully, I can update the blog soon with the latest information.

  11. Thank you gor sharing Madfies story! Shes the forst baby that I have found with the EXACT diagnosis she has single ventricle, No septum. Small Aurota and Hetrotaxy disease. Top small for the Glenn so she’ll receive it around 4-6 mths. The the fontan. Your story gives me hope!

  12. Good Afternoon, Maddie story is very inspirational. I noticed that Maddie was going in to see if she needed the heart cath. How is she doing?

  13. Thank you for sharing this story. We found out yesterday that our unborn little girl, already diagnosed with HLHS, may also have TAPVR. We weren’t given too much hope even though the doctors insist they will do everything they can to help her live. Seeing Maddie gives me hope that we will get to bring our little girl home. Thanks for sharing!

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