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 12 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 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!