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!

Another Happy Fontan-iversary!



Last week Tyson had his annual ECHO and cardiologist follow-up appointments as well as a bone density scan and visit with the thrombosis team too. 

CARDIOLOGY:  Tyson's ECHO looks the same as last year!!! Which means the mitral valve and pulmonary veins are behaving themselves. The mitral valve leakage is still mild to moderate, but no worse than last year.  The pulmonary veins are not shrinking anymore, and they seem to be growing at the same rate as the rest of his body.  They will still always be much narrower than they should be, but right now they are holding their own.  Dr McCrindle is still scratching his head about how Tyson's doing so well for a post-Fontan kid, but we'll take it!! We don't have to see cardiologist again for another year! Next year will be fun - Dr McCrindle wants an exercise test, blood work, holter and MRI in addition to all else. Tyson hasn't had a cardiac MRI since he was in Kindergarten, so this is just a routine 'every few years kind of test.'  The exercise test should be interesting!  I'm looking forward to seeing if Tyson really does understand his body's limits. 


 
The best news of the day (for Tyson, not me ) is that Dr. McCrindle told him that he's allowed to go on larger roller-coasters!


After 9 years of running this blog I STILL haven't figured out why some pics go sideways!
THROMBOSIS:  Tyson had another bone density scan and his density has improved over the last two years since he's started supplementing with calcium and vitamin D.  The reason for these regular bone scans is because one of the long-term side effects of coumadin is low bone mass.  Tyson was already showing low bone density at the age of 5,  just three years after starting the coumadin.  His density seems to be better than what it was two years ago, so that is good news. We will see thrombosis again in one year. 

APIXIBAN STUDY:  We are excited to be participating in a study for a new anticoagulant drug called Apixaban.  Dr McCrindle has been talking to us about this study for a few years already so we knew it was coming.  This is a new anticoagulant that isn't affected by diet and drugs in the same way as coumadin is and it doesn't require any INR level checks! The biggest downfall of coumadin is how the INR levels have such high and low peaks.  We have finally found some stability with Tyson's levels but anything can cause it to go crazy out of whack - food intake, vitamin K rich foods, cranberries, grapefruit, Tyson's overall health, and the use of other medications (like antibiotics.) Apixaban isn't affected by diet and drugs like coumadin.  It has been approved for use in adults but has not yet been approved for children.  This testing is phase 3 of the study, with phase 1 being 24 hours and phase 2 being one week.  Phase 3 of the study lasts for 12 months. We'll go to sick kids for screening and blood work on day 1, day 7 and day 14. Then month 3, 6 and 12. We are hoping to co-ordinate his Day 1, 7, and 14 with the Christmas holidays so that Tyson won't miss so much school. 


There are 3 different randomized groups in the study - 2 groups on apixaban and 1 group stays on coumadin. A bone density scan is done before the study begins so that they can also see how apixaban compares to coumadin.  There's a 1 in 3 chance Tyson will be chosen to stay on coumadin for the study, but since all drugs and supplies are covered in the study,  at least they'll pay for the coumadin and the test strips for the next year (those strips are NOT cheap!)

The hospital also compensates for gas, food and parking and Ty will receive a monetary gift for each study visit too.   So it'll be a lot of travel but so worth it if this drug gets approved! 
Tyson was a little apprehensive about getting poked so much (he's not used to that anymore) but when we explained to him that other kids participated in studies so that he could be alive today, he was very mature about it and said he really wanted to help. I'm sure the monetary gift helped coax him too :)

Tomorrow we celebrate Tyson's 7 year Fontan-iversary!!  It's been seven years since his chest was last opened and we pray for at least seven more!  A sea of memories come flooding at me at this time of year as we remember everything that happened surrounding his first and third surgeries (both in December.)  For his Fontan he was barely two years old and didn't understand what was happening - why did the doctor "open my heart?"  All he wanted was his Mommy and some apple juice.  Though Tyson will never remember,  we will never forget.
Happy Fontan-iversary buddy!