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!

The Pre-Plan Plan

I got a call from Tyson's cardiologist on Saturday to go over Tyson's most recent MRI results and discuss a plan for Tyson's future.  It turns out they're not quite in the planning stage yet, but more like the pre-plan stage.

I blogged about the MRI results already, so if you need to review what the MRI found, click here.
I'd rather not talk about it again :P

So, our biggest concern and question for Dr McCrindle is how do we prevent losing function of Tyson's left lung altogether?  The pvs is worse, there's more narrowing in the pulmonary artery...so how do we know he's not going to lose his lung function completely?  Dr McCrindle said that it is very unlikely in this scenario for Tyson to completely lose function of his left lung and that it's unlikely that the lung develops no perfusion.  He also said that the MRI is not exactly the most precise test.  It can give measurements and pressures, yes, but for a full diagnosis of how the disease is progressing you have to compare multiple tests done over a span of time.

When looking at the last 3 or 4 MRI reports, we can see that there is less and less blood flow going in and coming from the left lung.  This obviously concerns us and we don't want things to get worse, so I asked about the possibility of stenting or ballooning the pulmonary veins to widen them and perhaps improve blood flow.  Dr McCrindle feels this is not a viable solution because 9 times out of 10, it needs to be re-stented every 4-6 weeks and this would cause way too much scar tissue. 


He did mention (again) that the current narrowing in the veins is irreversible, they will not just suddenly start to grow,  and then he reminded me (again) that pulmonary vein stenosis can be very tricky.  We are in uncharted territory. There is not a lot known about how to treat pvs. Sick Kids sees about 1 or 2 kids per year with pvs.

Dr McCrindle assured me that the decision-making is always on the table and every time there is a test or procedure which offers differing results, there is a discussion about what to do next.  He said that he plans to pull all Tyson's files and reports together and then he will present Tyson's case to the cardiology interventionists at Sick Kids (the cath lab team) to see if there would be benefit in perhaps stenting his left pulmonary artery, since it also appears to be getting smaller.   There is some evidence that if you stent the LPA, it could prevent further blood flow problems in the small pulmonary veins.

We then talked discussed the Gleevac trial being done at Boston Children's Hospital in Boston, Massachussets and whether or not Tyson can access this chemotherapy drug here in Canada to prevent the progression of pvs.  Dr McCrindle asked me to get a hold of the contact person for the Gleevac study and forward the information on to him, which I have done this morning.  He will contact them to see if there are any reports about results/benefits for children using the drug.  I've been talking to Dr M about Gleevac off and on for over two years, continuing to ask him to keep this therapy in mind if the pvs gets worse, and each time he says Tyson is not at that point yet.  So now that he's asked me for the contact info, I feel like we may be taking things to the next level, if Tyson qualifies and can access the drugs here in Canada.  The ability to afford it may be a whole other story.
At the time that I first discussed the Gleevac, Dr M felt it was not the same type of pulmonary vein stenosis as most of the children in the Gleevac study have.  He felt Tyson's narrowing was more due to other heart anatomy pressing onto his veins, squishing them, and causing them to have lack of flow. But then, during a cath and cardiac MRI in June 2011, it was revealed that unwanted cells are indeed growing abnormally inside his veins, thus causing more narrowing than before.  Tyson only has 18% blood flow from his left lung (even less now!)  At that time the cardiologist recommended intense lung therapy treatments, especially during cold and flu season.


With regards to the decrease in heart function, Dr McCrindle said that although he moved from the 'normal' category into the 'borderline' category, he is still by no means in the 'reduced function' or 'ventricular dysfunction' category.  That would mean congestive heart failure.  Thankfully, Tyson is not there yet.

Dr McCrindle said that his heart function is actually still pretty good considering, and that Tyson's primary problems with his heart are a result of his pvs.  His pvs is causing high venous pressures, poor blood flow in the left lung, and it is also causing his blood to shunt the opposite way across his fenestration (which is also why his sats don't respond well enough when you crank up the oxygen in the hospital.)I asked if pvs is also the cause of his recurring pneumonia, but he didn't think there is a link between the two.  He said from his heart condition in general he has a weaker immune system and that is why he is catching colds and spending time in the hospital.  I do know that once you catch pneumonia, it seems to be easier and easier to develop.  There is no real plan for how to keep Tyson out of the hospital.  Dr McCrindle can't think of anything that would prevent him from getting sick, other than the obvious things.  We do have him on immune system builders, probiotics, and vitamins to build a strong immune system and hopefully keep him from catching every little thing that goes around.

Given all of this information, I asked Dr M if he could foresee there being a need for another open heart surgery in Tyson's very near future, but I could not get a straight answer.  There was hesitation, and then he finally said, "Immediate open heart surgery is not currently on the table, but I have to first talk to the cardiology interventionist to be sure." 

So that is our news.  Nothing alarming.  But no definite plans as of yet.  I will keep you posted when I hear anything back from him regarding the Gleevac or perhaps a trip to the cath lab :(

As always, we covet your prayers for a healthy season for our whole family.





 

MRI results


Tyson’s MRI results are in and the news isn’t as good as we had hoped.  We’re still trying to process what it all means exactly, and the news comes as a bit of a shock to us considering Tyson has been doing so well.

There are two significant changes from his last MRI to this one.  

First, there is more narrowing in his left pulmonary veins and his left pulmonary artery.  As a result, he has even less blood flow going into and coming out of his left lung than before.  His last MRI indicated a blood flow percentage of 18% from his left lung.  (Most healthy lungs have 45-55% blood flow.)  Tyson’s lung now has even less flow than that and with minimal drainage. When comparing the speed of blood flow between his right veins to his left veins, the blood flowing from his right pulmonary veins (his healthy lung) flow 8-10 times faster than the blood from his left lung. 

Secondly, the function of his heart has deteriorated some.  The doctors diagnose this by measuring the ventricle’s “ejection fraction.”  Ejection Fraction determines how well your heart pumps blood with each beat.  Most healthy hearts will have a left ventricular ejection fraction of 55-75% (because the left ventricle does most of the heart’s pumping) and you would be considered in heart failure if it got down to 40% or under.  With Tyson’s heart, because of the Tricuspid Atresia and Hypoplastic Right Ventricle, the left ventricle is the only ventricle that pumps so it would have an ejection fraction of about 60% at best.  Tyson’s heart is 47%, down from 57% on his last MRI.  This is a significant drop, not enough to consider him in heart failure, but enough that his function changed from ‘normal’ to ‘borderline.’   

So what’s next?  Tyson has another ECHO and appointment with his cardiologist again in November, at which time Dr McCrindle will have had ample time to discuss the findings with his team and decide what to do next.  When I asked him about how close we are to transplant, he said “we’re still a ways away.”  We are very thankful to hear that!  But the possibility of another surgery is not entirely out of the question.   He will have another MRI in 9-12 months, as long as he is relatively asymptomatic.

We are trying not to let this news get us down, knowing that sometimes we have to look past the test results and just enjoy the life that we see right in front of our eyes. He is super healthy right now, is LOVING Kindergarten, his antics make me laugh every single day (and sometimes his antics make me holler too!)  and we haven’t seen a decrease in his energy levels compared to before.   So we will keep our chins up and keep praising God for his goodness in Tyson’s life!

As always, we appreciate your prayers. 
Brian and Melissa and family