"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!

Annual Cardiologist Appointment

Brian, Tyson and I headed out at 6:00 this morning for a long day of tests and discussions at Sick Kids.  The day started with the thrombosis team to review Tyson's anti-coagulation and any concerning symptoms or side effects.  We're so thankful that the study drug is having many positive effects for Tyson.  He's growing and gaining weight,  bruising less, having fewer nosebleeds, and we don't have to test his levels as often.  The apixaban also isn't affected by other factors such as illness, diet, plants, or essential oils, while coumadin had limited our oil choices especially when Tyson got sick, because some oils have blood-thinning properties.  Apixaban allows us to use whatever kind of essential oil we want :)
 Next we went to cardiology where Tyson had an echocardiogram, ECG, pulmonary function test, stress test and holter monitor. Tyson's echo looks good and there are no changes from last year.  The mitral valve is still leaky and could cause troubles down the road but so far so good!

We had some time at lunch hour to play in our favorite place in the hospital - Marnie's Lounge!

Good ol' air hockey
Never too cool for a picture with Mickey Mouse

Tyson was such a trooper during his stress test and completed his bike run, biking uphill for 11 minutes in a row without stopping.  He did this while attached to ecg leads and a tube in his mouth, both to monitor his heart rate and function. He was quite exhausted when he was done and the cardiac nurses assisting were VERY impressed that he was able to complete the whole test!  We're happy with the results and so is Tyson!

Dr McCrindle said Tyson did better than the average Fontan kid!! You can't get much better of an answer than that!

I have to share with you a couple of things we're really excited about!

Firstly, it's possible that once the apixaban study is over, the sponsor company might be able to supply Tyson with apixaban for the duration of time while we wait for the FDA and Health Canada to approve apixaban in children.  This is exciting because with all of the positive results we've seen so far, we do NOT want to have to go back on coumadin again.  UGH I can't EVEN!!!

Second, Brian and I are going to be participating in a study at Sick Kids in January. It is a "whole genome sequencing study" to see if there is a relationship between congenital heart defects and parents' genetic makeup. Brian, Tyson and I will all be having an echocardiogram and bloodwork done so they pick apart our genomes looking for any genetic predisposition to any particular heart defects.  The flip side of this study is that it could potentially detect other illnesses we may possibly be predisposed to (breast cancer, etc.) and we're going to have to decide if this is something we desire to have them disclose to us.  Regardless of what we decide about the disclosure, we're always honoured to help with research in any way we can to help our cardiac communities at large.

Tyson signing the consent forms for the genome study

And lastly, I'm REALLY excited about something else I'll be busy with over the next while. Dr McCrindle and some other practitioners at SickKids are spearheading a new Fontan Registry Database to bring together heart centers and pull together Fontan research across the world.  They currently have no Fontan database and this makes it very difficult if they ever want to do any research on post-Fontan challenges, medications, symptoms, or even ways they can improve on their practices, etc. For example, right now Sick Kids is one of the few hospitals that does fenestrated Fontans where other hospitals perform non-fenestrated.  There is currently no concrete research proving one way or the other which method is superior.  This database of information would help cardiologists across the globe make educated decisions based on past experiences and research.

This is SO exciting, but do you know what is even MORE exciting? Dr McCrindle asked me to sit on the Parent Advisory Committee for this research database!! My role is not 100% clear as of yet as the registry is still in the works, but the committee would serve as an advocate for other heart families who want to express their concerns or pose questions about post-Fontan research.  What an honour to be able to serve the cardiac community in this way!  And you bet, I said Yes!

It was a long day today, I think the longest one yet!  Leaving just before 6am and because traffic was a gong show and it took us AN HOUR to get to the Lakeshore from Sick Kids, we didn't get home until 8:15pm :(  Sooooo tiring!

So we'll be back at Sick Kids in January for the genome sequencing study and finalizing details about the apixaban study.  Then again in May for a kidney ultrasound and appointment with the nephrologist.  We'll keep you posted as time moves on.

Thanks for checking in!
Melissa :)