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!

Respirologist Appointment

We're feeling pretty optimistic that Tyson will have a much better cold & flu season this year than last. I know, I know, summer has only just begun and I'm talking in anticipation of this coming winter! But with Tyson's heart not working the way it should after the Fontan surgery, in addition to the fact that his left lung is in really rough shape, it's hard to not be anxious about what the upcoming cold & flu season will bring.

The respirologist at Sick Kids asked 101 questions about Tyson's past illnesses, when he started on puffers, how they have worked for him in the past, how many times he's had pneumonia, what triggers his wheezing, etc. From there he came to the conclusion that it does indeed sound like Tyson suffers from asthma. In light of what his cardiologist suggested about him needing aggressive lung therapy, the respirologist has prescribed him Singulair, a daily pill which will help relieve the inflammation in his bronchials (asthma preventative.) We are to begin this treatment as soon as the kids start school again and may come home with viruses harmful to Tyson. (Unless he gets sick before that time, we will begin it then.) He also prescribed some more liquid ventolin, to be administered through his nebulizer. One nebule of this ventolin is equivalent to 25 puffs from Tyson's ventolin inhaler. No wonder it worked wonders on Tyson in June when his family doctor prescribed it! The other medication is Pulmicort, a corticosteroid much like his Flovent puffer, used to control asthma symptoms and prevent asthma attacks; but again this is used with his nebulizer so it will be much more effective in controlling the symptoms. The respirologist also recommended that Tyson receive regular RSV shots again this winter, and of course the flu shot. Normally, RSV shots are only available for cardiac patients up to the age of two, but given Tyson's struggles with respiratory illnesses, they are going to apply for Tyson to have them covered again this year too. We'd rather do the RSV shots (which Tyson had for his first 2 winters and he tolerated them very well) than end up in Critical Care. We've seen what RSV can do to healthy children, I don't want to think about what it could do to Tyson!

We also spoke with Tyson's cardiologist, Dr. McCrindle again. He let us know that he put in the order for another heart catheterization to take place within the next 2-3 months. He also wanted to check Tyson's sats (they were 86! We haven't seen numbers that high in a long time!) I also managed to squeeze in a few questions, one of them being, "If, during his next cath the test occlusion goes well, and they are able to close his fenestration, does it still mean he'll need a transplant?" **hope in my voice** Dr. McCrindle hesitated, then wrinkled his nose, then shrugged his shoulders and said, "There are no guarantees. It's too soon to say." *sigh* Ok, so I tried :)

I left the hospital feeling optimistic and hopeful that the new medications will help manage his asthma and when cold & flu season hits us again, we'll be praying that Tyson can stay healthy and well. The longer he can stay healthy = the more stable his sats will be = the less his heart will have to work, and hopefully = the longer the "transplant road" will become.

Thanks for checking in on Tyson! Prayers are always appreciated! (Don't forget to ask for a miracle that the pulmonary vein problems will resolve, that's his biggest issue right now :)

Mel:)

Surgical Team Meeting

"For I know the plans I have for you,"
declares the Lord,
"plans to prosper you and not to harm you,
plans to give you hope and a future."
Jeremiah 29 :11



I don't really know where to start on this update. Everything is still very jumbled up in my mind. I finally received the call from Tyson's cardiologist, Dr. McCrindle late this afternoon (after sitting on pins and needles for at least a few hours...he finally called at 5:15 pm.)

The surgical team met today to discuss Tyson's case. This team is made up of a number of staff cardiologists, Dr. McCrindle himself, the cardiology interventionist who performed Tyson's recent cath Dr. Lee, and Tyson's cardiovascular surgeon Dr VanArsdell, among several other professionals.
After reviewing his latest echos, his cath report and his MRI findings, they've come to the conclusion that it is not only the left lung which is causing the high pressures but there is also something going on in the right lung as well. I don't think there is anything wrong with his right pulmonary veins, but Dr. M said that the entire Fontan circuit is not operating well. In order for you to understand exactly what I mean, let me explain an important detail to you. In your healthy heart, your right ventricle pumps de-oxygenated blood to your lungs, your lungs oxygenate that blood, and then your left ventricle pumps the oxygenated blood to the rest of your body. Your heart always get the exact correct amount of blood it needs in its lungs because your right ventricle is pumping exactly as it should. Because Tyson doesn't have a functioning right ventricle, he has nothing pumping the blood to his lungs. The only way blood can get to his lungs is through natural flow which the surgeons have created throughout the last 3 open-heart surgeries. The Fontan surgery completed the system, and Tyson's blood is now flowing (not pumping) to his lungs through a pathway which we call the "Fontan circuit."

Tyson's heart is not handling the Fontan circuit well. This is why he has been sicker more often and had more health struggles after his Fontan than before. Most kids do very well after their Fontan and are in the hospital much less often. Most kids graduate to annual appointments after the Fontan. But not Tyson. Tyson is one of those kids who is just not handling the Fontan circuit well. His lungs are not getting the right amount of blood, causing resistance in his lungs, which is why it was not safe to close the fenestration. Most of the concern is with his left lung because of the pulmonary vein stenosis, but it looks as though the right lung is also not doing what it's supposed to do.

Dr. VanArsdell (surgeon) does not feel that another attempt at repairing the pulmonary veins (Coles procedure) will be successful. He would like to see Tyson in the cath lab again in the next 2-3 months to try to do a test occlusion before the fenestration closure. Again, let me explain. Before doing a fenestration closure, which is usually permanent, the cardiologist will perform a test occlusion (occlusion means closure.) Through the cath lab (heart catheterization) they will temporarily balloon the fenestration closed while the patient is under the general anesthetic, and keep it closed long enough to assess the heart and lung pressures, see how the pressures change, see how the patient's body handles it. Then, after they've decided that the test occlusion was successful, they will go ahead with placing the metal discs in the fenestration to close it permanently. During Tyson's cath 2 weeks ago, they didn't even try a test occlusion because they thought the pressures were just too high, there was no point in even trying. However, Dr Van Arsdell feels that they should at least attempt a test occlusion to see if they can go ahead with a fenestration closure. This would make his sats come up, and hopefully keep him healthy for a little while. With Tyson's O2 saturations hovering in the low 80s, there is not a lot room for him to get sick and he will most likely need hospitalization if his sats dip much lower.

Even so, this second attempt at a fenestration closure would still be a short-term solution. The fenestration clsure (IF successful) will not repair the pulmonary vein problem. The problem with Tyson's pulmonary veins is serious and will affect Tyson's long-term life.

The surgical team then discussed putting Tyson on the list to be transplanted and would consider two options when doing so.

Option 1: Transplant just his heart, and only connect it to his right lung, leaving the left lung completely out of the picture so the pulmonary vein stenosis problem becomes obsolete.

Option 2: Transplant both a heart and a left lung.

For now, clinically speaking Tyson is doing well. He is not sick enough to transplant yet. If during his cath in the next 2-3 months, the veins look worse, we would need to look at transplant as an option sooner rather than later. No one knows how long this long-term solution will take to become a reality. But it looks like he's definitely heading down that road.

Brian and I very much get the impression that the team looks at transplant as a last resort only. Obviously, a transplant is a very serious operation with numerous risks, way more risks than any of the surgeries Tyson has ever had. But, before opting for transplant, Dr. V wants to at least rule out the fenestration closure. If indeed they are able to close it, it still only buys Tyson time before he inevitably will need a transplant.

For a long time, Brian and I were of the understanding that a 3 year old could take an adult heart. But Dr M didn't seem to agree with that. I'm sure I've heard that from a cardiac critical care nurse way back when Tyson was born...but either way, Tyson's heart would need to come from another child his age. It's scary to think that some day I might be tempted to pray for another child to lose his life so that my child can have another chance at life. *tears* Ok, I'm getting ahead of myself. He's not there YET.

In the meantime, Tyson sees the respirologist at Sick Kids this coming Wednesday. They will assess his lung function and come up with a plan to protect him from his reactive airway syndrome, or asthma, or whatever it is he has going on with his lungs. We are to mention to them what the cardiologist said about "aggressive lung therapy" for Tyson. Hopefully they will come up with a plan to keep Tyson's airways nice and open so he can have a healthier winter/spring this coming year. We want Tyson to stay healthy and strong for as long as possible. The sicker he gets, the quicker he'll travel down the transplant road. Dr. M also wants to take another quick peek at Tyson at that time too, since respirology and cardiology are right beside each other on 4A. I said, "Yeah, I was hoping it would be a short day. We'll come by if we have time." To which Dr. M said, "No, I want to see Tyson on Wednesday. I'll be here all day. Come and find me."

That's the update for today. I'm not really sure what prayers to ask for. Today I was praying for wisdom for the surgical team and patience and acceptance for us. I believe the team was given the wisdom to find the best solution, and we have been given the patience and acceptance...or at least for today...but we're still praying for a miracle. Is that wrong? To pray for wide open pulmonary veins even when the doctors have given us little hope? To pray that Tyson lives a long healthy life just the way he is, when the doctors think he will require transplant? "What is impossible with man is possible with God." We have to be realistic of course, but we cannot deny the power of prayer.

Mel:)