TYSON MATTHEW KOTTELENBERG
"The Lord is faithful to all His promises, and loving toward all He has made." Psalm 145: 13b
This blog is about our eight year old son Tyson. Tyson was born with serious complex congenital heart defects, (Hypoplastic Right Ventricle, Tricuspid Atresia, Coarctation of Aorta, Transposed Great Arteries, with VSD and ASD.)
In short, the right side of his heart is completely under-developed (he has half a heart,) his main arteries are mixed up, and his aorta is narrow. He has undergone 3 open-heart surgeries and 5 heart cath procedures to try to 'repair' his heart. Sadly, Tyson has also been diagnosed with pulmonary vein stenosis, a fatal disease in the veins of his left lung. Typically, having the 3 palliative surgeries buys children with single ventricle hearts many years before eventually needing a transplant. But because of the narrow pulmonary veins, this increase in pressure is causing his blood to shunt the opposite way across his Fontan fenestration and surgeons are not able to close the fenestration. As a result, he remains on coumadin and is heading down the transplant road faster than we'd originally anticipated. He's still doing AMAZINGLY well all things considered. We entrust our dear son into the hands of God, knowing that in all things God works for the good of those who love Him!
Today Tyson had his first echocardiogram since discharge after his Fontan/Coles surgery. I was really hoping and praying for big, wide open pulmonary veins. As unrealistic as that is, I still prayed for a miracle for Tyson. But no, they are still very small. At this point we are more concerned with the size of the pulmonary veins than anything other part/defect in his heart. Pulmonary vein stenosis is a very scary disease and I really just wish I'd never ever heard those words in my life. But it's a reality, so we deal with it.
Lately every time Tyson goes for another test or procedure, it seems to reveal new problems that arise. There is concern about the size of the left pulmonary artery (which Dr McCrindle wasn't surprised about at all - and we knew it was a problem since almost the very beginning but apparently they don't always mention every single concern they have at every appointment unless we specifically ask.) The echo tech had to call in another Dr. to look at the echo with his own eyes because he himself was having a difficult time measuring/reporting to Dr McCrindle on the pulmonary artery.
On a good note, the echo revealed good left ventricle function (this is good - his left ventricle is the only working ventricle and it's currently doing the work of two! )
Next Tyson had an Ecg while he was still sleeping.
And of course, waking the bear is always fun. Tyson did not disappoint us in that department, it's always fun dealing with a grouchy, hungry, drunk toddler. I hate sedate echos. I cannot WAIT until we can do this without sedating him! Today it was a good thing he was sedated though (despite his coughing for the past few weeks, his lungs sounded clear upon examination) because the echo lasted an hour and a half! The "average" echo is 30-45 minutes long.
People in the 4A waiting room must have thought I was a horrible mother because Tyson said things and did things to me that I normally would never ever tolerate. But he honestly could not help his behaviour. The chloral hydrate he takes for sedation causes him to remain groggy/dizzy/nauseous for the entire day, and he can't get out of the stroller to walk around because his jelly legs would cause him to fall flat on his face (no need for brain bleeds...I know we'd be in the right place, but no thank-you.) Every time he closed his eyes his head would spin and he would whine and complain. We end up spending the entire day catering to him and just doing whatever we need to do to keep him somewhat tolerable. We pretty much had to do handstands today to keep him "happy." Thanks to my wonderful mother for her incredible patience. With the grouchy bear and his by-the-end-of-the-day-irritable-mother.
Tyson also needed bloodwork again, as part of his bi-weekly INR levels. His level has been slipping slowly so two weeks ago he was bumped up to 2 mg of warfarin every day. The level needs to be between 2.0 and 3.0. It was 1.9 today, so we will be waiting for a call from the thrombosis people to adjust his warfarin dosage again. We're trying to find the right dose for him but it's been taking this entire post-op time to find the right dose. We'll get there.
Next off to chest x-rays, which looked good despite his cough. Chest x-rays were to confirm that the chylothorax accumulation has stayed away. Which it has, thank the LORD! Eight weeks of strategic cooking and planning has paid off! I wish minimal fat diets on no one :)
Dr McCrindle, the cardiologist, couldn't give us really great answers regarding the blood flow into the pulmonary veins, which is what I was sort of hoping. An echo can't really get a good picture of the blood flow from the veins. (It shows more the heart function, how it's pumping, if the oxygenated and de-oxygenated blood are mixing, and can measure things like the pulmonary arteries, the size of the fenestration, among other things.)
Soooooo..........within the next 6 months Tyson will be going for another heart catheterization and MRI. The cath gives a good picture of the pulmonary arteries, while the MRI gives a good picture of the pulmonary veins. Both procedures will be done on the same day, under the same general anesthetic. During the cath, the cardiologists will make decisions about what interventions may need to be done, whether it's ballooning or dilating. At this time it is not smart to stent the pulmonary artery because Tyson is too small and the procedure would just need to be repeated in 3-6 months, as his heart grows. (The artery will grow as his heart grows, but still in proportion to how it is now...small.) And the veins are way too small to stent. The veins are probably small because the pulmonary artery isn't getting enough blood flowing through it, so in turn the veins are not growing to their full size. Dr M says it could also be vice versa (the veins are small because the artery is small, OR the artery is small because the veins are small. Tyson isn't old enough to really know what the trend is for his heart yet. But the concern lies within the entire left "pulmonary branch.")
When I asked Dr M what kind of procedures he anticipated for Tyson in the immediate future (the next 2 years) he couldn't give a prediction of what is to happen, since he'd have to wait to see what the cath reveals. After the cath and MRI, Tyson's case would be presented to the surgeons again if any other interventions were needed. It could be that they try to do the Coles procedure again. I hope not, that means another open-heart.
When I asked what the small arteries/veins means for Tyson's long-term survival, Dr M couldn't give a prediction for that but admitted that he really wished the pulmonary vein stenosis was not in the picture. I asked him if the stenosis comes back, would Tyson be on the list for a transplant? Dr M said that he is not thinking at all about transplant in the near future. He said if that were the case, we'd be talking years down the road. But hopefully never :) He seems to think that one-lung Fontans are ok. Not the best situation, but ok. It's just a really good thing that Tyson's right lung is perfect so far!
All in all, we didn't really receive much more information than we already know. But I feel more peace in knowing that the pulmonary vein stenosis isn't going to cause Tyson to go into heart failure as quickly as we had originally thought. If that were the case, we wouldn't be waiting 3-6 months for the cath procedure.
People ask me all the time, "So his heart is fixed now?"
No, his heart is not fixed. He will always have a half of a heart (unless transplanted - even then it means life-long echos and anti-rejection medication.) Congenital Heart Defects have no cure. The series of 3 surgeries aim to repair the anomalies temporarily, until transplant is needed. Sometimes that is within 2 years, sometimes 5 years, sometimes maybe 20 years. Sometimes maybe even never? We are praying that by the time Tyson's half-heart starts to fail there will be new technology out there which will prolong transplant even longer.
But no, his heart is not fixed. Not even close. He may look great on the outside, but inside his heart is fighting a huge battle. The pulmonary vein stenosis also complicates things. It means Tyson needs closer follow-up than the usual post-Fontan patient. So we're looking at cardiologist appointments every 3 months at the maximum, regular echos and ECGs, and quite possibly multiple procedures to keep the arteries/veins stable.
Tyson's next cardiologist appointment is in early May. No sedate echo this time, thankfully. But from there a date will be set for his cath and MRI.
That is all I had to say today. I honestly went into this blog thinking it would be just a quick one. I forgot who I am for a minute :)
Till next time,
Tyson hugging his new "Heart Buddy" whom
he calls "Buddy."
Buddy accompanied us to Sick Kids today :)
"My flesh and my heart may fail,
but God is the strength of my heart
and my portion forever."
Psalm 73: 26
February is Heart month, and February 7-14 is Congenital Heart Defect (CHD) Awareness week. I simply have to do a blog post for CHD awareness.
Right now I am feeling pretty passionate about CHD awareness. I just can't believe that even though CHDs are the #1 birth defect, there is still no mandatory testing at birth.
You know the PKU heel prick that they do to your newborn shortly after birth? The one that makes your baby scream because it hurts when they try to draw blood from his foot? That is to test for a rare disease called Phenylketonuria, which affects about 1 in every 10,000 to 15, 000 babies worldwide.
CHDs AFFECT 1 TO 3 IN EVERY 100 BABIES IN CANADA ALONE, yet no mandatory testing is done for this. There is a very simple way to test for CHDs - a pulse oximetre reading given shortly after birth. It involves wrapping a band-aid type probe around the newborn's finger or toe and it reads the oxygen saturations in the blood. Simple. Painless. Non-invasive. No screaming involved. This non-tormenting device is sitting inside hospital cupboards.
PKU is 1 in 15,000. CHD is 1 in 100. Hmmm....
Ok, now that that is off my chest, I will continue :)
CHDs affect 1 in 100 births and kill more children each year than ALL childhood cancers COMBINED. This year alone, 4,000 babies will not live to see their first birthday because of complex CHDs.
There are 35 known types of CHDs, ranging from small holes in the heart that may never require surgery to complex CHDs which require surgery within days after birth. Tyson has 4 of these complex CHDs, as well as two holes in his heart.
This week was an important week for Tyson and our family, and for all families affected by CHDs. With the help of another heart Mom Shannon Heighington from Orangeville, both mayors of Orangeville and Shelburne have officially declared February 14, 2011 as Congenital Heart Defect Awareness Day! This morning Tyson had a "pow-wow" with Shelburne's mayor Ed Crewson so that we could be present for the signing of the declaration. A reporter from the Orangeville Citizen was also there, so look for a picture of Tyson high-fiving the mayor in the next edition of the Citizen :)
I am so excited that these two small communities have acknowledged the importance of CHD awareness. Next year it's our goal to raise awareness at a provincial level, and perhaps even make February 14 CHD Awareness Day nation-wide. I'll be working alongside another woman named Laura who is also very passionate about raising CHD awareness. In fact, she has been imperative in starting a foundation called "Wyatt's Warriors Congenital Heart Defect Foundation of Canada." For more info on this foundation or how you can donate, go to http://www.wyattswarriors.ca/.
The reason I am so fired up about awareness is because I believe that awareness leads to support, education and research. Research leads to funding. And funding leads to HOPE.
Please join me in raising CHD awareness. On Monday, February 14th, please wear the colour RED. It's easy to do because it also happens to be Valentine's Day. And if someone comments on your bold red clothing, please explain to them what you have learned about Tyson and CHD.
The following are links to some local newspaper articles that have been printed recently in view of CHD Awareness Day (two of them were written by yours truly :)