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 nine 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!
The roller coaster has been huge. Ups and downs. High mountain peaks and low, deep valleys. First, preparing for a surgery 6 weeks in advance is hard to do. It's almost easier to have short notice, you have little time to fret and worry. Then, we received the call that they had an opening for October 5th, which we couldn't accept because of a family important wedding. Then this past Tuesday (two days ago) the surgical coordinator called to say that Dr V had a very long, difficult surgery planned for Thursday so there might not be time for Tyson on Thursday. Would Tyson be ready tomorrow morning? (Wednesday) That was a very anxious day too, knowing we could have to give up our "Family Day" we had planned with the kids for Wednesday, and having very little time to finish all the last-minute details. By lunch time that same day, we received word that Dr V felt he could handle both the long surgery and Tyson's surgery on the same day after all, meaning Tyson would still be in on Thursday but he would be second-case. Although he wouldn't be in till later in the afternoon, we could handle that. It would mean Tyson would have to fast longer and he could get grumpy but oh well, at least it was still happening on Thursday and we could enjoy our family day on Wednesday!
Until next time,
Brian and Mel:)
Tyson's pre-op appointment went very well today. He had blood work done, chest x-rays, an ECG and we had meetings with the various health care professionals such as cardiac nurse, anaesthetist, cvs fellow (cardiovascular surgeon fellow) and of course, Tyson's surgeon Dr. VanArsdell.
Tyson was in a very good mood and did a good job charming the nurses as always, and even met some fellow heart patients who will have their surgeries on Monday. We also had the opportunity to meet Parker, a heart buddy from New Brunswick who is a few months older than Tyson. Parker had his Fontan in September but suffered from some serious chylothorax leaking which prolonged his stay at Sick Kids. I've been in contact with his Mom over facebook and we've been able to share our stories and be a support to one another. Parker was officially discharged today and is flying home to NB on Saturday. It was refreshing to meet another heart mom whose son has a similar diagnosis to Tyson's (Parker has Hypoplastic Left, Ty has Hypoplastic Right, among other things :)
We found out today that Tyson will most likely be on the blood thinner Coumadin for a few months after surgery, until his heart gets used to the new blood flow. The Fontan procedure will completely separate the red blood from the blue blood (this is how a normal heart works, but in Tyson's heart right now the red blood and blue blood are mixing.) We will also need to get a weekly INR level checked, done by a visit to a local lab for bloodwork, who then fax the results into Sick Kids, who will then tweak the dosage of Coumadin if necessary.
We also found out today that the anaesthesia process will be easier on us than we thought. I expressed to the anaesthetist that there was no way I was letting them peel a screaming boy from my arms, only to have them fight with the sleeping mask over his face..and then next thing he knows, he wakes up in immense pain. I just thought that this would be way too traumatic for Tyson, and for us as well. I am happy to have learned that they will first give Tyson a sedative that will make him fall asleep (in my arms) within about 10 minutes. Ah, I love the description she gave us: She said he would feel sort of like he was really drunk and doesn't remember a thing by morning. You know, the kind of night where you drink about 24 beers within 10 minutes and in the morning, you don't remember that you were dancing on the tables all night :)
We had the blessed opportunity to talk to Dr. VanArsdell, who was able to answer most of our questions, but of course some questions are still left unanswered. We'd love for him to be able to tell us that the procedures are 100% effective, that Tyson will be miraculously healed, that he will make a speedy recovery, and that he will live a very long, healthy life! But we entrust all those details into God's Almighty hand and cast all our anxieties on Him!
I will do this in question and answer format again as that seems to be the most comprehensive way.
Dr. VanArsdell, you've mentioned that Tyson has small pulmonary veins (pv's) and will need a Coles procedure to try to fix them. We have been researching and have found that this is called pulmonary vein stenosis and it is a rare condition that has a very guarded prognosis. Does Tyson have pulmonary vein stenosis (pvs)? Where is the pvs and how bad is Tyson's case?
Yes, Tyson has significant pulmonary vein stenosis in both his left pulmonary veins (pv's). The stenosis is at the opening of where the veins meet the heart. In many cases of single ventricle patients, since the one ventricle is small, the functioning ventricle will be extra large. Because Tyson's heart is large on the left side, it is compressing the pv's and restricting the blood flow from the left lung to the heart. This is very concerning and needs to be repaired. We need to remove the pvs from the equation in order to have a successful Fontan operation.
We've read that those with pvs involving all 4 veins have a very high mortality rate. Is this true?
Yes. Unfortunately that is true. But Tyson's pvs involves the 2 left pv's. So far his right lung is not affected.
The heart cath done in August revealed that there has been no growth in the pv's since his Glen surgery in May 2009. That's a year and a half with no growth. Is it possible that the Coles procedure will stimulate the veins to begin growing on their own?
No, it is not likely that the veins will begin growing now after all this time. That is why the Coles procedure is being done.
Is it still the plan to perform both the Coles procedure to fix pvs and then proceed to the Fontan? How do you know it's safe to perform both procedures?
It is still the plan to do both at the same time, but I won't know for sure if that's possible until I see it with my own eyes. If it takes me two and a half hours to do pvs repair, then we will absolutely not proceed to the Fontan. But if I can do the pvs repair in half an hour, we have lots of time and will continue on with the Fontan. Because pvs affects the lung function, we want two healthy lungs before proceeding to Fontan. Because having two healthy lungs is paramount to a successful Fontan.
What exactly is done in the pvs repair (Coles' procedure) ?
The Coles procedure was developed by Dr. Coles here at Sick Kids and is a relatively new procedure. The heart is surrounded by a membrane called pericardium membrane. We take this membrane around the pv's and stretch it over to the pv's opening and reconstruct the openings, making them larger. Because Tyson's large heart is compressing these openings and putting pressure on them, we have to shift the opening to a slightly different area in the heart, hopefully relieving that pressure. The heart may heal well enough that it is happy with the new position of pv's openings. Or it may not, and begin to compress the new openings as well. We won't know how Tyson's heart will heal and how it will react to this procedure. Because it's a relatively new procedure, we can't give statistics on how successful it is in the long-term. We do know it is a better option than and the results last longer than a stent. Since his heart is still growing, we'd have to re-stent too frequently.
If you can't proceed with the Fontan, how long after this open-heart surgery (OHS) would you wait before completing the Fontan?
6 months to a year. Depending on his recovery. We'll need the scar tissue to heal properly first. Buuuut, if he recovered quickly and he was strong enough, we could do it as soon as 10 days later.
(Ok so that answer REALLY confused us....but we trust he knows what he's talking about. He judges everything case by case. It would all depend on Tyson's recovery and strength. We'll cross that bridge if we get there.)
How long would it take before we know if the Coles has been successful? Does it CURE the pvs?
3-6 months. Maybe 12 months. If re-stenosis will occur, it's usually within 3-6 months. Hopefully this doesn't happen, but it is fairly common. How Tyson's veins heal will determine his long-term survival rate. A successful Coles will mean a successful Fontan.
If his pv's re-stenose, could you do another Cole's procedure at that time?
Mmmm....we could consider it. We will have to re-evaluate at that time and make the best decision at each stage. If pvs was a recurring problem with Tyson and his pv's became blocked (which they often do with recurring pvs) we would at that time opt for a heart transplant.
Wouldn't we have to consider a heart transplant PLUS a lung transplant, since the pvs affects the lungs more than the heart itself?
No. Many people live with only one good lung and will never need a transplant. A healthy heart plus one strong lung have a good long-term survival rate, provided that the right lung stays healthy. But a single-ventricle heart with only one good lung is not a very good prognosis.
When we first learned of the pvs, and that there was a possibility of another surgery before his Fontan, I cried and thought that was the worst thing in the world. But at this point, I don't care how many procedures it takes...I want my son to live as long as he can! (*tears*)
Of course! And I want that too. How Tyson's heart heals is what determines the next step. I can only do the best I can. The rest is not dependent on me. I really wish that I could heal it all for good. (In light of this statement, I found out today that Dr. VanArsdell is such an amazing man that he is traveling to China in February and performing heart surgeries on little babies there!!! Wow, how God is using him!!!)
What are some common complications with the Fontan (besides the obvious risks involved) ?
The 2 most common complications are chylothorax leaking and a condition called Protein Losing Entropothy (PLE). Chylothorax leaking is the most common, occuring in 10-15% of cases. If the leak occurs, it is managed by a low calorie diet (we've been there, done that with the Norwood!) and perhaps another surgery involving a side chest incision. The PLE is managed by medication, a low calorie diet, and if it gets serious enough, and the body cannot handle the protein loss, a transplant is sometimes done. We will cross those bridges if we get there.
So...that was our day. Information overload! We are thankful that so far Tyson has been blessed with one healthy lung. If the pvs repair is successful, he will have two healthy lungs again and that will mean a typical single ventricle life for him! It can be a long life, but it could also include various complications or future surgeries. All that is up to God. He leads and we follow :)
If all this overwhelms you, as it does us, we were reassured about Dr. V's abilities when he stated, shaking his head matter-of-factly, "Tyson doesn't scare me. His condition is well within the range of what I've seen before." :) :) :) :) :) :) !!
We are confident in his abilities and honestly, we really, really, really have one of the world's BEST surgeons working on Tyson's little heart. Please pray that God will use Dr. V to work miracles on our little boy :)
As the big day approaches, please pray for
1) steady hands for the surgeons (especially Dr. VanArsdell of course)
2) that the Coles procedure will be successful and it fixes the pvs problem
3) that the Fontan can also be completed, eliminating the need for another OHS
3) and a speedy recovery
4) strength for Brian and I as we wait by his bedside for him to recover
5) strength for our children, who love their brother so much and would love to enjoy many more years with this blessed, joyful little boy :)
Brian and Mel:)