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!

Cardiac Cath and MRI - June 23

Tyson finally went in to the cath lab at 1:45pm. They were running behind schedule. Thankfully he did fall asleep in Brian's arms at about 12:30pm so he wasn't TOO cranky. For most of the morning all he repeated over and over was, "Juice. Juice." Of course, he's been fasting since last night and his last drink was at 8am in order to prepare for the 11am procedure. It's always tough being the second case of the day, we all much prefer first case but unfortunately we don't have a choice. Brian and I also fast along with him (other than our quick bowl of cereal at 5:30 am before we woke Tyson:) so by the time he went in, we were all very hungry, thirsty, and tired.
So Tyson has his MRI once he's under general anesthetic and intubated, and then they take him straight from MRI to the cath lab to perform the fenestration closure. We were told it would be about a 4 hour wait. We'll update again once we hear something. With the surgery running behind schedule, plus factoring in the minimum 4-6 hour recovery time (depending on if they went in through an artery or a vein,) we won't be going home till evening at the very earliest. We will update again once we hear something.

8:15 pm

First the good news, then the bad news. The good news is Tyson made it into recovery room at just after 5pm, has to stay for observation for 4 hours, and then we will get to go home later this evening.

The bad news is very devastating to us and we are still trying to process it all. There is a huge problem with his left pulmonary veins; they are not getting better and it appears the Coles procedure he had done in December didn't really help at all. The left pulmonary artery seems to be damaged by the descending aorta, so it's small from other heart anatomy pressing against it. However, it appears that the pulmonary vein stenosis is indeed caused by the unwanted growth of cells inside these veins. So the problem will not get any better, but we need to find a way to prevent it from getting worse. They did try to send the catheter into his veins to see if they could dilate them or balloon them in any way, but they were unsuccessful at this.

As a result of the very small pulmonary veins, most of Tyson's blood is going through the right side of his heart and lungs, causing very high pressures in his right lung. It was not safe to proceed with the fenestration closure today because it would have caused dangerously high pressures in his right lung. So he will remain with low O2 saturations until there is a plan in place for what to do next.

The report from today's procedures have been emailed to Tyson's staff cardiologist Dr McCrindle, who is away on holidays till next week. He will need to meet with his team of experts to present Tyson's case to the surgeons at their next meeting and come up with a plan. No one has outright said this to us, but we are most likely looking at another open-heart surgery in Tyson's future; but we don't know at this point how urgent this is. The cardiologist who performed that heart cath was very clear and direct with us that his left lung is not functioning well at all and they cannot leave things this way. Having said that, when I mentioned the word "transplant" to her, she did say that she believes this is still a few years down the road, and that there are probably other surgeries they can try instead of opting for transplant quite yet. As much as it may seem that having a heart/lung transplant may "solve" the problems, there are extreme risks that go along with it too, especially on a young boy like Tyson. Plus the risk of rejection, and a lifetime of anti-rejection medications. We're not there yet in our minds anyway; we're hoping and praying there is another way around this.

Tyson is recovering well. He's been drinking well this evening and starting to eat a few things. He will need to resume his warfarin (blood-thinners) tonight and continue with them until the fenestration is closed, whenever that will be, if that will even take place. It's not a pressing matter at this time to close it, but it is imperative that we keep Tyson very healthy and continue with aggressive lung therapy treatments. Even though the ventolin mask (nebulizer) can cause elevated heart rates, the cardiologist feels that a rapid heart rate is better for Tyson than reoccurring pneumonia. Tyson will see the respirologist at Sick Kids on July 6, so we can make a plan as to what to do to keep his airways open. In the meantime, we will be giving him a ventolin mask a minimum of twice daily to keep those airways open and prevent pneumonia from settling in.

That's the update for today. Not the best news we've heard. We're trying to let it soak in and choosing to trust God with this as well.

Prayers are always appreciated!

Mel:)

Pre-Cath Day, June 21

Tyson's pre-op tests went extremely well today. As sad as this sounds, it really is a blessing that Tyson is so familiar with his "home away from home." The day included a multitude of tests and appointments, including ECG, chest x-rays, bloodwork, meeting with the cardiologist interventionalist fellow, the cardiac nurse, a Child Life Specialist, and his first "official non-sedate echo." He was a trooper! For his chest x-ray, he just wrapped his arms around the chest plate without even being given any instructions, and he was so quiet and still during his echo. Blood work is always a different story but, the LORD willing, that will be the last time he has blood work in a long time. (He won't need the blood-thinners, or the bi-weekly bloodwork that goes along with it, after his fenestration closure!) During his non-sedate echo, he laid quietly watching tv for over 40 minutes, then another 25 minutes while the sonographer wrote her report and discussed the echo with the staff cardiologist. Sick Kids policy is that all children under age 3 be sedated for their echos. (At least for kids with as complex of defects as Tyson's, since the echos are usually quiet lengthy.) We popped in a Franklin DVD and he laid on the bed with his soother and blankie, rolled over to his side when asked to, and lifted his head so they could get at his neck to look at the pulmonary arteries. What a good kid! It has been a blessing in some ways that he gets these echos so frequently because he was so comfortable having her work with him. No more groggy, angry drunk! I think we can kiss the chloral hydrate good-bye! The best part of the non-sedate echo is that he remained in a relatively good mood ALL day because he wasn't fighting the spinning head he would have had if he'd been given the chloral hydrate.


We got to see the "fenestration occluder" today. This is the mesh-like discs that Tyson will have placed in his heart to block his fenestration hole. It is about the size of a small button, made out of nickel and titanium. It can stretch into a skinny cylinder shape to fit inside the catheter and from there the catheter is threaded through his artery into his heart. Once the catheter secures the occluder in place, (I don't even want to think about how they do that!) the round discs dilate, (pop out) blocking the hole, and will remain in his heart forever. His heart tissue then grows around it, but the good news is he will not need to be on bloodthinners because of it. Although he may need to take one aspirin per day for the next six months, the danger of him banging his head and causing bleeding in the brain will no longer be a concern. Surprisingly enough, this small piece of metal costs around $5,000. Not to mention how much the procedure itself will cost. By the time Ty is all good-to-go he will be probably be worth a million dollars! Good thing for OHIP!

It's very likely that we will get to go home the same day as his cath, unless they do more intervention than they anticipate. For example, if they decide to stent his PA or PV, (unlikely) then we may have to stay overnight under observation.

Tyson's surgery is 11:00 am on Thursday, with his cardiac MRI immediately following while he is still intubated and under general anesthetic. I'm hoping we can be discharged and make it home in time to pick our kids up before they have to go to bed, but we'll see what the day brings.

Please pray that Tyson will tolerate the fenestration closure well, that there is no danger of stroke, that the closure will improve his O2 saturations, and that during his MRI they find wide open pulmonary veins so that he won't require further open-heart surgeries.

Mel:)

Summer is on Its Way...

With spring weather officially here, we're hoping that summer is right on its heels so that Tyson can get into his "healthy season." It's too bad that summer is so short, it's really the only time of year that Tyson is sniffle-free, cough-free, and he doesn't rely on his inhalers as much.


Back when Tyson had his hospital stay in March, his nurse practitioner referred him to a respirologist at Sick Kids, to figure out why he always has a cough all winter long. His appointment with the respirologist is Wednesday, July 6. They are going to do PFT (pulmonary function test) called a spirometry test. This test measures how well the lungs take in and release air and how well they move oxygen from the environment into his body's circulation. Hopefully that will give us a few answers and help us better prepare for next winter.


Tyson has also been seen regularly by a speech pathologist to assess his speech and language development as time goes on. Up until this point, he was being seen every 3-4 months just to keep an eye on him. However, at his last appointment a few weeks ago, the speech & language pathologist recommended him for regular, weekly speech therapy sessions. She wanted to start him already this summer, but I showed some hesitation having to truck three kids off to a babysitter once a week so that I could take Tyson to speech. So we've decided to wait until fall when all three of our older children will be in school and I can just take Tyson out by himself. The pathologist says that Tyson's cognitive understanding and vocabulary development are right on par for his age; it's the articulation of his words that needs work. She suspected it could be from some hearing loss, so she referred Tyson to an audiologist.


Tyson had his appointment with the audiologist last week and his right ear did not pass the test. So they are re-testing him again in case it was caused by left-over congestion from his pneumonia 2 weeks prior to that appointment. So we will go back again at the end of June to get his hearing checked again. If he indeed has hearing loss, that will definitely contribute to speech delay!

These days it seems we're off to at least one appointment every week for Tyson. If it's not the speech therapist, it's an audiologist appointment, or the pediatrician, or the family doctor, the cardiologist, the infant-child-development worker...and now we're adding a respirologist to the list too!
Tyson's oxygen saturations have been less than glorious these past few weeks. At his May 11 cardiologist appointment, his saturations were low 80s and Dr McCrindle wasn't entirely happy with those numbers, even though I tried to pass it off as being from Tyson's cold. Either way, he wanted Tyson into the cath lab to undergo a procedure to assess whether or not they can close the fenestration at this time, and also an MRI of his heart and lungs to assess the status of the pulmonary veins which are severely stenosed.


We bought a pediatric finger pulse oximeter probe that we clip onto Tyson's finger to monitor his oxegyn saturations at home. When we first got it, we were checking all the time of course...and checking my sats, and Brian's and the kids'... Now we only pull it out when we are concerned about his colour. Which is quite frequently lately. Today his sats were 76 for some reason, with no symptoms of a cold. His baseline with this pulse ox seems to be 80 or 81. But we often see numbers in the high 70s too. I've been in regular contact with Stephanie at Sick Kids, cardiac nurse, because I don't like those numbers at all. Those are pre-Fontan numbers, not post-Fontan. I've also been hounding them a little bit because they said Tyson should be in the cath lab for his heart catheterization procedure within the next 6 weeks (they told us this 3 weeks ago.)


I have a feeling Stephanie called the cath co-ordinator and asked her to book Tyson in quickly, because we got a call yesterday saying his heart cath procedure will be on Thursday, June 23. That's 3 weeks away. Pre-op for the heart procedure is Tuesday, June 21.


We'd appreciate prayers for Tyson to stay healthy in preparation for this heart cath procedure; they won't follow through if he gets sick. Please also pray that the doctors will find good heart & lung pressures, that they can close the fenestration so his sats will get back into the 90s where they should be, and that when they look at his pulmonary veins through MRI, that there is no further re-stenosis and the surgery they did in December is sufficient for him for now. We're praying he will not need further open-heart surgeries on these veins.

We'd also appreciate your help with a contest being run by the Heart & Stroke Foundation. As you know, Brian and I led a team called "Heart Warriors" who rode the big bike this week, raising $9,345.00 for Heart & Stroke. It was a lot of fun! Altogether, Orangeville 10 teams raised over $33,000! In this contest, Heart & Stroke is giving out an AED to the winning team in each province, to be placed anywhere in their community. Right now we are in SECOND place in Ontario, SO close to winning! If you could go to this website (you sign in with an email address and password) and vote for ALL 23 of Heart Warriors' stories. You can vote for ALL 23 stories EVERY 24 hours. Click on "♥ this story" to vote. Please help us win the AED for our community!
http://www.bigbikecontest.ca/teams/4170883-Heart-Warriors


Mel:)