Monday, January 31, 2011

Best Bi-directional Glenn and Fontan descriptions I've found...

Without grabbing a napkin and pen, I still find I have a hard time concisely explaining in words the surgeries Luke has had and still needs to people who are curious. It's such a simple question: "So, how do they fix that??" But the answer is confusing, even after four years of reading and researching! I thought I would pass on this explanation of the staged Fontan palliation for other heart families to use, or for those reading this blog who understandably don't understand the single ventricle heart!

"Dr. Francois Fontan first performed the Fontan procedure in 1971 on children with tricuspid atresia, or lack of a tricuspid valve. Fontan redirects venous blood from the right atrium to the pulmonary arteries, without pumping it from the lungs to the heart.

The Fontan Procedure is a palliative surgery; in other words, it doesn’t focus on curing the disease, rather, it reduces the symptoms and severity of it, resulting in an improved quality of life for the patient. It’s generally performed on children with complex congenital heart defects. Specifically, it’s used when a child only has one effective ventricle.

The Fontan is typically done as a two-stage repair. The first stage is referred to as a Bidirectional Glen procedure, or Hemi-Fontan. In this stage, oxygen-poor blood is redirected from the upper part of the body to the lungs. The pulmonary arteries are disconnected from their blood supply and the superior vena cava is removed from the heart and directed into the pulmonary arteries. The inferior vena cava transports blood from the lower body and remains connected to the heart. This redirection allows the single ventricle of the heart to do much less work. The second stage is known as Fontan completion, and it also redirects blood from the inferior vena cava to the lungs.

Although there are many different types of Fontan operations, they all serve a common purpose: to cause one effective ventricle to pump oxygen-rich blood to the aorta and into the body.

Patients who undergo Fontan surgery require life-long management to address any problems that may occur, such as heart rhythm issues, a weakened ventricle, or blockages and/or narrowing in the Fontan circulation. In many cases, children who undergo these procedures will require some form of Fontan revision surgery later in life.

It’s difficult to predict exact outcomes for Fontan children—in some cases patients will be able to participate in sports and vigorous activity; however, others may be severely limited in terms of exercise. Because this procedure is still relatively new, there are still many questions as to how well single ventricle heart/Fontan patients will fare as they enter their 30s and 40s, which is why ongoing monitoring is so crucial."

Tuesday, January 25, 2011

If the Lord allows

If the Lord allows, it could it be that three ultrasounds is all this baby girl will experience. Her big brother beats her by six! Not that we are in any way encouraging sibling rivalry =)

We had a follow-up ultrasound this past Monday at Maternal-Fetal Medicine. My OB sends all her patients there (high-risk or not) for their 18-20 week ultrasounds. We were thankful to be getting good, level II pictures of little girl. As emotionally difficult as it was to know about Luke's heart in utero, we wouldn't have wanted it any other way. For us, the benefits of having a team put together, being at the best hospital for Luke's arrival, having family prepare with us, all outweighed the anxieties, doctors appointments and unknowns. This time around, we knew we would want to know with this little one if anything was wrong.

At our first ultrasound with the Maternal-Fetal specialists, I was 19 1/2 weeks pregnant. They confirmed what our fetal echo tech thought, that this peanut is truly a girl and then went ahead with all the measurements. Once you've had a child where every ultrasound is a breath-holding, hand-gripping experience, Roger and I found we couldn't quite relax on this one, either. Even though we already knew her heart was a-okay, it was still a tense experience. After 45 minutes or so, and several position changes, the tech let us know she couldn't get a good shot of baby girl's spine because she was all curled up "toes to nose". She assured us this wasn't concerning, that babies love this position in the first 20 weeks, but we would need to come back in two weeks for another ultrasound. We took reassurance in the fact that the doctor never came in to see us or talk with us, but still we couldn't shake our feeling of unease. Both my OB and my sister's boyfriend's dad (who is a perinatologist in Virginia) confirmed that having to go back for further measurements is very common, but honestly, we did not want to go back.

But we did, armoring ourselves with prayer, and God gave us an amazing appointment. Our tech this time was exactly who we needed. She was chatty, talking to us and the baby throughout the ultrasound, and believe it or not, has had three open heart surgeries herself! She was born with transposition of the great arteries and has also had two aortic valve replacements. Right away, we could tell baby was in a better position, and she was able to get the spine pictures out of the way first. She continued taking a few more measurements, and even turned on her 3D camera so we could watch sweet girl rubbing her eyes and waving her fingers. After printing out a few more pictures, she went to talk with the doctor, and within a minute or two, came back in and said, "You guys are outta here!" Sweeter words we have never heard. We rejoiced together as we walked out to the car gave thanks and praise to our God.

If the Lord allows, this baby girl will be healthy. That is our daily, sometimes hourly, prayer.

But what of the families that were also at the Maternal-Fetal Medicine office, who hadn't just received the good news they so desperately wanted? What if our baby's spine had not been okay? What about my dear friends, the Lester's, who lost their sweet Maddie to a CHD exactly three years ago?

I love this paragraph by Max Lucado, answering the question, "Do you trust Him?":

"Scripture, from Old Testament to New, from prophets to poets to preachers, renders one unanimous chorus: God directs the affairs of humanity. No leaf falls without God’s knowledge. No dolphin gives birth without his permission. No wave crashes on the shore apart from his calculation. God has never been surprised. Not once."

I honestly can't say I understand it. But I believe, with every fiber of my being, that God was not surprised when the doctors saw a missing ventricle. And I believe just as strongly that God was not surprised that this second baby has four healthy chambers.

My job, then, is not to dwell in the "whys" and "why nots". God wants me to dwell on this, instead:

"I am the one who creates the light and makes the darkness. I am the one who sends good times and bad times. I, the Lord, am the one who does these things." —Isaiah 45:7

My job, then, is to ask Him to comfort me in the bad times, so that I can be a comfort to others when they are suffering.

My job is trust Him. Our family is on-our-knees grateful for this baby girl's healthy heart, but that doesn't give me license to think we had anything to do with that outcome. It doesn't give me license to forget that every life is in His hands and it certainly doesn't give me license to forget that He wants glory for this girl's life as well as Luke's.

Sunday, January 9, 2011

Roooaaaarrr! You are FOUR!


How is it possible that in four short years you have woven yourself into our hearts so intricately and changed our hearts so immensely? Your fight through adversity has taught us about the Lord's strength within us. Your enthusiasm for life has caused us to laugh more and see the joy in the small and simple. Walking with you through the hard stuff — three surgeries, two heart catheterizations, 70 nights in the hospital, NG tubes, countless appointments — has been a privilege. Walking with you through the good stuff — first steps, first words, discharge days, family prayers, wrestling matches, morning snuggles — we count as miraculous.

Your fourth year was a big one. You gave up your mimi's (pacifiers) in July. You started preschool in September. You had an angioplasty at Mary Bridge Children's Hospital in June. Your sense of humor started to really blossom. You had your first babysitter that wasn't related to you. You jumped to size 8 shoes, size 3T pants and size 4T shirts. You were potty-trained and you moved to a big boy bed. Your opinion became even stronger and no amount of persuasion can convince you otherwise when you've made up your mind. You discovered peppermint hot cocoa from Starbucks and played even more often with your best buddy, Marcus. You put hours, and I mean hours, on your Fisher Price jeep. For the most part, you are completely nap-free and have learned to play independently for extended periods of time. You passed the 30 pound mark and are currently creeping up on 33 pounds.

Four years ago today, you were in the Tacoma General NICU, waiting for your PDA to close so we would have a better idea when your first open heart surgery would be. Today, you are blowing out candles on your dinosaur cake, playing with your new train set sporting your new Lightning McQueen light-up shoes. You have no planned surgeries in the next year or two and you have a baby sister on the way. One who you've most recently dubbed Slappy Hammer.

You amaze us. We love you more than you could ever know, only out-loved by God Himself.

Happy Birthday, sweet Luke.