TYSON MATTHEW KOTTELENBERG

This blog is about our 14 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 catheterization procedures to try to 'repair' his heart. Tyson also has severe narrowing in his pulmonary veins which are causing higher venous pressures. 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!

GUESS WHAT CLINIC WE SAID GOOD-BYE TO TODAY?!?!


Did you guess correctly? That's right, the haematology/thrombosis clinic!! We're ecstatic to report that after 11 long years, Tyson is officially OFF of coumadin!!!!!

His cardiologist Dr. Emilie says he can start taking a low dose aspirin instead. This news both excites us immensely and scares the crap out of us at the same time. How can we be sure that he's fine being off the anti-coagulant that he's been on for almost his whole life?  

Well, remember that study Tyson participated in a few years ago? There were 3 drugs being studied at that time - rivaroxaban, apixaban, and aspirin - all comparing their risks and benefits vs. coumadin.  Because of the findings of that research study, SickKids has decided that aspirin is as good as coumadin to prevent stroke in Fontan patients. Health Canada has finally approved the use of rivaroxaban (aka Xarelto) and apixaban (Eliquis) in children, so from now on SickKids will be prescribing one of those to Fontan kids INSTEAD of coumadin! They'll take that for approx a year while the risk of clotting is high, and then transition to low dose aspirin after a time (except in rare cases of history or risk of blood clots/stroke.) Tyson doesn't have those risk factors so Dr. Emilie is confident that he will do great on aspirin. She thinks that soon prescribing coumadin for anyone will be a thing of the past!! This is such good news for Fontan kids!! 

What does this mean for Tyson and for us as a family? It means:  

  • No more INR level checks (finger poke and/or blood work)
  • No stressing about out-of-wack levels when he’s sick or doesn't eat a consistent diet
  • It's not as serious if he misses a dose
  • No more spending hundreds of dollars a year on test strips
  • No more nosebleeds 🤞
  • Better appetite 
  • Less risk of heavy bruising
  • Less risk of brain hemorrhage (still slight risk but it’s greatly reduced)
  • No limit to what plant medicines he can use
  • Don’t have to accompany him to every school field trip
  • No more bone density scans to monitor for low bone mass (brittle bones)
  • No more thrombosis clinic appointments at SickKids!
If you've been following Tyson's story for a while, you'll know how much I hate coumadin. I don't use the word 'hate' very often, if ever. But when it comes to coumadin I truly do hate it. It's the reason for so many decisions we've made over the years; why we had go to every sports game or field trip with him, why we never went skiing until last year, why he wears a helmet and shin pads when skating, etc. 

All of this comes just in time because our CoaguChek machine was on the fritz and was no longer reliable. We crossed our fingers and said a little prayer every time we used it for the past few months because it would randomly turn off in the middle of the test. I was going to ask for a prescription for a new one today but we won't be needing one afterall!

Just a baby aspirin

Overall his cardiac tests today looked good. His heart function is unchanged from previous ECHOs and he's gained 5.5 kilos since this time last year - that's 12 pounds! I can only imagine how much my grocery bill will go up now that he's no longer on coumadin. (To that I say, BRING IT!)

Dr. Emilie Jean-St-Michel

Today we said good-bye to Jennifer, our thrombosis clinic nurse of many, many years. She's the one we report every INR level and bounce ideas around about how to get him back on track if we miss a dose. She's more than just a nurse to us, she's like family. 

Good-bye Jennifer, we love you!

Tyson also had a long MRI today (it turned out to be 3 hours long...) He fasted from 8:30am, had tests and clinic appointments all morning, and then the MRI started at 12:30.  He wasn't too thrilled that he had to have an IV for the contrast dye, but was brave nonetheless.  



What does a 13 year old boy do in an MRI machine for three hours? 
Watch Harry Potter, of course!

Going in!

Finally eating at 4:00 pm

Tyson came out of the MRI machine quite stiff and sore, with pressure marks on his behind from laying so still in one spot for so long. He literally wasn't allowed to move an inch, not even to adjust his legs. I'm thankful they have movie goggles to keep him distracted. 

Ok, one final detail and then I'm signing off. We don't have the results of the MRI yet, but as long as things look good, Dr. Emilie is going to present Tyson's results to the cardiac team to see about the possibility of closing his open fenestration. Maybe.... Depending on results... 🤞🙏... We don't want to get our hopes up too high, but this would mean there'd be no need for aspirin because he'd no longer be at higher risk of stroke. AND it would mean his oxygen levels would get closer to 100%. 

All in all, a very long, emotional day.  We left at 5:45 and weren't back till 6:30pm. Tyson and I are both exhausted (my eyes are starting to close...) but all of us are extremely excited for NO MORE COUMADIN!  

Chow for now,

Melissa

PS If you're on Instagram, check the Reel I made with highlights from our day.

https://www.instagram.com/reel/CW9p2coA0Cu/?utm_medium=copy_link


Annual Health Update

It's time for my annual update about Tyson's health.  So at the end of November we went to SickKids for a few appointments. November 23 we went for genetic follow up and Tyson's respiratory stress test. December 1 we went for all his cardiac appointments, bone density follow-up, abdominal ultrasound (liver, kidneys) and thrombosis clinic. 

GENETIC FOLLOW-UP:  You might remember that back in January of 2019 Brian and I participated in a whole genome sequencing study to help with cardiac research. They're trying to see if there are any genetic links to certain heart defects. We didn't participate because we're personally needing to know how or why Tyson's defects happened, but more so to help out with SickKids' research. The results showed nothing to conclude a genetic link to his heart condition, but they did find a genetic link to his chronic respiratory issues.  

PRIMARY CILIARY DYSKINESIA: They found that he has 3 damaged genes that indicate he likely has/had something called PCD (Primary Ciliary Dyskinesia.) PCD is basically when the cilia in the sinuses and lungs don't clear mucous very well and this results in a chronic wet cough, nasal congestion, asthma,  and frequent infections like sinus infections, ear infections, and pneumonia.  We found this data VERY intriguing!  Remember how long Tyson struggled with his respiratory health and they just kept prescribing asthma meds, and recommended chest physio and breathing exercises, none of which really seemed to help? If they discovered this PCD earlier in his life they may have prescribed heavier medication, although from my research about it, there really is no 'cure' for PCD. 

The genetic team suggested that we follow up with the respirology department for further follow-up to run some further tests.  However, we've found that the essential oils we use daily are very effective at keeping his airways open and preventing pneumonia so we decided not to pursue anything with respirology.  In fact, he was discharged from that department in 2016 because he's been doing so well! We doubt that anything would change in his treatment plan even if they did confirm that he has PCD so we don't feel it's worth visiting another department at SickKids. If hospitalizations become a problem again down the road, we may pursue it then. But it sure does explain a lot about his respiratory struggles in the past! 

PULMONARY STRESS TEST: Showed good physical working capacity and aerobic capacity for a Fontan patient. 


Pulmonary function test


Some traditions never die!
Some traditions never die ;)

CARDIAC:  We're still seeing many of the same things as always, but some newer things too. His Fontan pathway is unobstructed, which is good, but his blood is shunting through the fenestration right-to-left instead of left-to-right. This is probably due to the high venous pressures in his left pulmonary artery and stenosis in the left pulmonary veins.  This has been going on since a few months after his Fontan surgery in 2010 and we're not sure how it's working for him, but it is. The mitral valve is still showing mild-moderate regurgitation which may need some attention down the road. 

Some newer concerns are that they've been watching are that Tyson has mild bradycardia. Bradycardia  means his heart pumps fewer than 60 times per minute (sometimes in the 30s or 40s when he's sleeping.) He also has biventricular hypertrophy, meaning the walls of his heart are thickening and that makes it hard to pump effectively. He still has good ventricular systolic function but the hypertrophy shows there is wear and tear on his heart from years of having one side of his heart doing all the work. 

KIDNEY CYST: Tyson has had a cyst on his right kidney for years and it is being monitored yearly.  So far doesn’t seem to be giving him troubles, but it’s 3.5 cm in size on his 9cm kidney, so it’s taking up 1/3 of his kidney.  As Tyson’s body grows, his kidneys grow, and unfortunately so does the cyst.

FONTAN ASSOCIATED LIVER DISEASE: Because of the way his SVC is connected to the lower organs, the hepatic pressure is high which causes weakening of the organs like liver, kidneys, spleen, etc.  His recent abdominal ultrasound showed that he has some 'lesions' on his liver and it is starting to stiffen. The report said the stiffening of his liver is ‘severely abnormal.’ I scheduled a phone call with his cardiologist to get an explanation as to why it was written that way and if his liver is in danger of failure soon. She said that unfortunately, liver failure is par for the course with single ventricle patients who’ve had the Fontan surgery. The longer time passes after the Fontan, the greater the likelihood of liver problems. It's best to be proactive and run some more tests, so Tyson is scheduled for an MRI in November to get a clearer understanding. 

Waiting for the cardiologist to see him


CARDIAC AND LIVER MRI:
The MRI will be of his heart and his liver. He hasn’t had an MRI of his heart since 2015 so it’s high time he has one again to check out what’s happening with those pesky pulmonary veins that gave him troubles when he was younger. The pulmonary veins are the veins that carry oxygenated blood from the lungs to the left side of the heart, and his left sided veins are extremely small, causing a lot of pressure within the heart. His last MRI showed borderline ejection fracture, meaning borderline heart function. I’d like to think he’s doing a lot better now than in 2015 but that’s the trouble with heart conditions…sometimes you can’t tell what’s going on in the inside just by looking at the outside 😉 I’m sure during the MRI they’ll also look at the cyst on his right kidney. 

PSYCHOEDUCATIONAL ASSESSMENT: Tyson will also be scheduled for another psycho-educational assessment with a SickKids psychologist. He had this done once already when he was starting Grade 3 and things looked good all things considered, beyond a bit of anxiety which we’ve been working on, but they also like to do another one as kids prepare for high school. We don’t have a scheduled appointment for that yet but if I don’t hear back soon I’ll call to see if we can have that scheduled in during one of the times we’re there this year.

That's the clinical update for this time.  We're thankful to have such a world-renown pediatric hospital right in our own backyard. Ok, not quite 'in our backyard,' but pretty close! Considering some people have to travel 5 hours to get to SickKids, we're thankful we can usually do the trip in two. 

Thanks for reading!