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!

Tyson is a Big Brother!




!~TYSO
N IS A BIG BROTHER~!




Addisyn Faith was born into the world on Wednesday, January 25, 2012 at 12:20 am, weighing 6 lbs 12 oz and 20.5 inches long.

This week was a life-changing week for the Kottelenberg family. On Monday, the surgical team at Sick Kids Hospital met to discuss Tyson's heart and what could be done to repair it. (The heart cath done on January 9th was their second attempt at closing his fenestration and because they were unsuccessful again, the surgical team met at conference to come up with a plan for Tyson.)
Due to the fact that I was bringing my kids to piano lessons at the time of Dr McCrindle's call, we had to wait until Tuesday morning at 7:30am for him to call me again so we could go over the details. Not only had I missed this very important phone call, but I also found out that afternoon that I was going to be induced the very next morning to deliver our 5th baby, because the ultrasound I had a week earlier revealed the suspicion that the baby had stopped growing properly. The midwives consulted an OB and they decided it was better for our baby to be outside the womb where she could grow and thrive rather than continue on with the risk of not growing properly in utero.

We were very nervous about an induction, but at the same time we didn't want to risk the health of our unborn child so we agreed to go to Alliston hospital for me to be induced, after discussing with Tyson's cardiologist the plan for his future.

The news we received about Tyson's future was good news (somewhat) and bad news. Bad news because the surgeons all definitively agreed that there is nothing more they can do to repair the pulmonary veins in Tyson's heart and the next stage for him is heart and/or lung transplant. This is heart-breaking to hear. "There is nothing more that can be done."

In other ways this is good news, because it means that, for the very first time in Tyson's life, there is no *immediate* open heart surgery looming in the near future! Dr McCrindle said that Tyson is not even close to being a candidate for transplant yet at this time, so they will not be placing him on the list immediately. He feels that given Tyson's healthy status right now, transplant could potentially be years down the road. This is so reassuring to hear! What a relief!

As long as Tyson does not require hospitalization for every little cold or flu, and we can keep him clear of pneumonia, he could potentially be surgery free for years. We are hopeful that as Tyson grows bigger, his airways will become less reactive whenever he catches a cold. However, he will continue to desaturate (lowered O2 sats) over the months and years. If he can live with his cyanosis and continue to stay healthy then we will just watch and wait. He will have to stay on coumadin his entire life but at least he's past the clumsy toddler years already (on the other hand, the dangerous, adventurous years are ahead of him yet!) While most kids are followed up once per year after the Fontan, Tyson will be followed up much closer than the average Fontan patient with appointments every 3 months instead.

For those of you who understand "heart talk," here is the scenario: During his cath, they found a significant amount of blood crossing through the fenestration. As a result, when they attempted the 2 test occlusions, his cardiac output declined, his heart and lung pressures rose, and his oxygen saturations through the Fontan decreased as well. Right now his pressures are between 12 and 14mm. They also found a small left pulmonary artery and some collaterals draining into his left pulmonary veins as well. There are no obstructions in the Fontan per se, but there is very high resistance going through the lungs. Because the resistance in the Fontan circulation is too high, Tyson is clearly not a candidate for fenestration closure. When the surgeons discussed the possibility of repeating another repair of his pulmonary veins in order to alleviate some of these pressures, they all agreed that it would be too difficult to repair. The left lower vein is being compressed by the aorta and other heart vessels and attempting a repair on that would not only be risky, but would also cause even more scar tissue than what is already there from his repair in December 2010. So it is unlikely to be able to repair the pulmonary veins enough to make fenestration closure successful.

After Brian and I had time to absorb what was discussed at the surgical conference, we rejoiced that Tyson won't need surgery in the near future, and put that part of our lives behind us for a time to focus on the birth of another child.

We phoned Alliston hospital and they told us to come in at 1:00pm to begin induction. Since I had been having early labour signs for a few days already, and since this is my fifth pregnancy, we all assumed that labour would begin on its own with the breaking of my waters. They broke my waters at 2:00pm and we walked around waiting for labour to begin. But after several hours of labour starting and stopping, things did not progress at all. We discussed with the midwives our options to get labour going. At about 9:00pm we asked for me to be induced with the drip. (I never thought I'd ever ask to be induced with the oxytocin drip but I was just eager to get the show on the road and have our baby! I knew I was not going to sleep that night until our baby was in my arms.) So I was finally induced at 10:00pm and Addisyn made her appearance at 12:20am. There were a few complications throughout the process, but God indeed provided for us once again.

The immense relief and peace that I now feel is overwhelming. All of the stressful events of this pregnancy and labour - the antibody issue, the extra tests, fetal echo, extra ultrasounds, measuring considerably small towards the end, the realization that our baby was no longer growing adequately, the frustrating induction - then her heart rate kept dropping during the last number of contractions, the cord was wrapped around her neck *twice*, and I hemorrhaged pretty badly afterwards (which resulted in some panicked nurses re-inserting an IV, a few bags of pitocyn to stop the bleeding, and some other not-so-gentle interventions to stop the bleeding) - all of these events combined made for a lot of anxiety and worry throughout the pregnancy and labour.

As soon as she was born, however, a huge load was lifted! In the same day we experienced two life-changing events. Not only did we learn that we don't have another open heart surgery for Tyson looming over our heads, but we were also blessed with a beautiful, healthy baby girl! From the moment Addisyn was in my arms, the burdens were lifted and I felt FREE! We are so ready to move on to the next phase of our lives, not having to look ahead to another impending surgery, just enjoying our new little bundle and adjusting to being a family of five wonderful blessings. I am so unbelievably happy, I simply can't put it into words. :D :D :D :D :D

THANK-YOU FATHER!


Our Brave Warrior

"No king is saved by the size of his army;
no warrior escapes by his great strength."
Psalm 33:16


Recovering on 4D, eating
freezies and watching tv


Every time I hear the word "warrior" I think of Tyson. He sure is one brave boy! Yesterday again he showed us how strong of a fighter he truly is. When we reflect on the psalm above, we see that victory does not belong to us, but to the LORD. In Tyson's life too, we acknowledge God's mighty hand in him, leading him, guiding him, carrying him through the difficult times, giving him the will to keep on fighting. We know that Tyson is not a warrior in and of himself, but God keeps him strong. God deserves all the glory and praise! It is through God's grace that I can call Tyson my little heart warrior :)

Monday was a very LONG and difficult day for us all. We left the house at 5:30am and didn't get home again until 12:15am Tuesday morning. Tyson was admitted to 4D in the evening to continue under observation, but by 10:30 pm the cardiologist fellow on call gave us the go-ahead to go home if we wanted to. Since nobody really sleeps well in the hospital, we chose to make the trek home and curl up in our own beds. Tyson fell asleep on the way home, woke up at 6:45am and has been back in bed napping since 9:15am. He is zonked and understandably so!

It was a LONG morning, with Tyson not able to eat since 6pm on Sunday evening, and nothing to drink since 6am Monday morning. He wasn't allowed even a small sip to wet his lips. Overall he managed well because we were able to distract him quite well, until about 10:30am. We took along his hockey gloves and stick so he passed a lot of time playing a fair bit of hockey in the hallways, charming the desk clerk and all the other parents in the cath lab waiting for their children, and watching tv.

Once he finally got called in for his MRI at 11:15, he was past the point of hungry, thirsty and grumpy and it all went downhill from there. If you know Tyson, you know what I mean! Brian was amazing with him, and I am so glad he was there. His lap is much bigger than mine these days, and his back much stronger! I'm usually the one who goes with Tyson into the anesthesia room but since I'm pregnant, I was not allowed this time. Brian said Tyson fought the mask for quite some time, kicking and screaming that he wanted to go home, but once he got a few deep breaths of the gas, he was limp and asleep. From there, Brian had to leave his son in the hands of the doctors and nurses and the long wait began.

The MRI and cath both took exceptionally long. During the MRI, Tyson participated in a study to see if doctors can in the future predict which Fontan patients will experience protein losing enteropathy (PLE - a common side effect of the Fontan surgery, involving bowel and kidney issues/ complications.)
The cath also took extremely long because they not only went into the vein in his groin, but also the artery. I didn't ask why, but we believe this was to obtain different measurements and pressures by accessing different areas of the heart. They also performed the fenestration closure test occlusion not just once, but twice, to make sure they were 100% sure how Tyson's heart and lungs would react. The test occlusion caused his pressures to elevate and there was also a decline in his cardiac output, so they decided not to permanently close the fenestration. Because Tyson suffers from pulmonary vein stenosis, a lot of the blood that should be going through his pulmonary veins is instead escaping through his fenestration hole (which is useful in relieving some pressure) and once the hole is blocked it causes even more pressure through his veins - picture the extra pressure in a garden hose once you kink the house for a long time. This means Tyson will remain on his coumadin for much longer term than anticipated. Most kids can come off the blood thinner within 6 - 12 months of the Fontan surgery upon successful fenestration closure, but Ty will have to stay on them for much longer. I truly dislike having my toddler on blood thinners; it adds so much more stress to our lives whenever he falls...but we choose to accept this as God's will too.

At about 4:00pm we talked to Tyson's staff cardiologist Dr McCrindle who had heard nothing yet about how things were going. He went in to talk to the cardiologist in the cath lab, and then came out to report to us that they were just beginning the test occlusion. They tested it for a good long time because there were still no answers yet by 5:00pm. It was a VERY long wait! Usually cath procedures aren't that long...but this time the wait was pretty excruciating. Not knowing exactly what kind of results we should pray for, we kept praying that God would give the cardiologists clear answers as to what was best for Tyson. As much as we wanted Tyson to have the closure so that he can be off the blood thinners and we can put the "Fontan chapter" of his life behind us and begin moving forward, we were also scared that his heart and lungs would not be able to handle it and it would cause problems for him down the road. We know that God's will was done.

So now we wait for the surgical team meeting for them to discuss what is next. They meet every Monday at 3pm. If there is room to discuss Tyson at next Monday's meeting, they will. If not, it might wait till the following Monday. Either way, we are confident that the cath and MRI both have provided adequate information for the team to decide exactly what to do. Considering how long both of these procedures took, we know they have all the information they need to move forward with a plan for Tyson. We believe they will need to give Tyson a large time slot during their team meeting, since they have had extensive conversations about Tyson's condition in the past and this will be a big, life-altering decision to make.

Overall, Tyson did extremely well under the circumstances. His strength continues to amaze us! When we walked into recovery to see him at about 6:00pm, the first thing he said was "Owie. My tummy hurts." Then he cried for his Mommy. I walked over to his bedside and leaned over to kiss him and told him I am so proud of him and he is such a brave, brave boy. He looked up at me, then his eyes began to close again and he said to me softly, "Mommy. Please hold my hand." Sweet, sweet, sweetness. Oh, I just love that kid! Even under those circumstances, he remained tender and lovable. He did a fair amount of vomiting at first in reaction to the anesthetic, and he did spike a fever (which is normal) so the nurse gave him Gravol intravenously and some Tylenol for pain and he did well after that. He laid quietly for a few hours watching Franklin and the hockey game, dozing off here and there. By the time we got onto 4D at about 9:00pm his goofy personality had come back, he told us some knock-knock jokes, and he continuously gave us the "thumbs-up" and winked whenever we looked at him. He mowed down a snack bucket of Cheerios, had 4 freezies and some crackers. Once his stomach had settled he must have realized how hungry he was!


All back to"normal" and ready
to go home! The spider man hat
is his gift from us for being so brave.


This morning he was up by 6:45 burning up with a fever. We gave him some Tylenol and his nebulizer mask (lots of coughing after being intubated and laying flat on his back for so long), let him watch tv while we tried to catch a few more zzz's, and he feel asleep again by 9amish.
He has to take it pretty easy today and tomorrow but in the next few days he will be back to his normal active self.

We are so relieved to have this cath behind us now, that Tyson pulled through again with flying colours, and that we can now move on to the next stage of our lives: a new baby. The last few days I've been paranoid that I'd begin labour before Tyson's cath but thankfully baby is not ready yet and has stayed put. (Ever thought you'd hear a pregnant woman say that?!?) In a few days though, when Tyson is completely recovered, my tune will change and I will be twiddling my thumbs waiting for baby!

That's all for now. I will update again once we hear something from Dr McCrindle about the plain for Tyson.
Thanks so much to everyone for your prayers, love and support. They mean so much to us!
Mel:)



January Heart Cath

1:45 pm
Tyson finally made it into the cath lab at 12:15pm. Poor guy was very hungry, thirsty and past the point of grouchy, (nothing to eat since dinner last night, and some apple juice at 6am) but thankfully Brian is a wonderful Dad and was great with him :) We won't hear anything for a few hours yet. The cardiologist doing the cath forewarned us that he didn't think a fenestration closure would be possible given his pulmonary vein stenosis and high venous pressures. We completely expected this, but it's good for them to have a look around and re-assess his pressures and measurements anyway so they can discuss where to go from here. Keep praying :)

5:45 pm
Tyson is still in the cath lab; he should be in recovery very soon. His Fontan pressures are slightly lower now than they were in June. But they are still too high for fenestration closure. Most of this is caused by the stenosis (narrowing) in the left pulmonary veins. They did a test occlusion and watched his pressures for about an hour, and his pressures did increase too high. So if they permanently closed the hole it would affect his cardiac output. They also concluded that his cardiac output is slightly decreased since his last cath. I can't explain what they mean by that right now....no time. Given how well Tyson is doing at the moment, everyone agreed not to close the fenestration and put him at risk for even lower cardiac output. He is healthy now, let's not fix what isn't broken.

Dr VanArsdell (surgeon) happened to be walking by just as Dr McCrindle was giving us the news, and so they both agreed that Tyson will be discussed at Monday's surgical team meeting. Maybe there is something they can still do for the pulmonary veins, another open heart surgery possibly. They will see what the entire team thinks. If they don't fit him in the schedule for discussion for this coming Monday, it will be the following Monday. We may have to wait a few weeks to know for sure. But we are ok with that because, even though this isn't the best news, it was not at all unexpected. All day we prayed that God would give the cardiologists wisdom to know what is best for Tyson. We believe and trust that this is the best decision for Tyson for now. Even though it means another open heart.
We should be able to see him again soon. I miss him :( It's been an incredibly LONG day. Thanks for your prayers!

For those of you watching our children, please hug them for me....

Pre-Cath Appointment, January 6, 2012

Tyson had his pre-cath appointment today at Sick Kids and all went well. He is scheduled for second case on Monday morning, at 10am. He will have an MRI first to assess the status of the pulmonary veins in his left lung, and then while he is under the same anaesthesia and intubation, he will proceed to the cath lab for his heart catheterization. Again, the cath is to attempt a fenestration closure, to examine how his Fontan circulation is working, and to measure his heart and lung pressures. From this, we hope to learn what the next step will be for Tyson surgically. The entire process will take 2.5 - 3 hours roughly, and then he will have a minimum of 4-6 hours recovery, with the possibility of an overnight stay if fenestration closure is successful.
Please continue to keep Tyson and our family in your prayers. Please pray that Tyson will remain healthy and ready for this procedure, that his heart & lung pressures are low enough for him to have the fenestration closure. Please also pray that the pulmonary vein stenosis has not progressed but that the veins remain stable, so we can hold off on any talk of heart & lung transplant in the near future.
(When praying for our family, please keep our other children in mind as well. They deal with more anxieties than most kids their ages, and our oldest especially understands more and worries more than he should have to at his age.) Our comfort is knowing that God is in control of every situation and we pray for His peace to guard our hearts and minds!
Tyson is coping with things very well. The Child Life Specialists at Sick Kids are wonderful at explaining things to him at an age-appropriate level and he seems very comfortable with what is going to happen. He even said on the way home today that he likes Sick Kids because it's fun. We feel so blessed to have an amazing Children's Hospital that even a three year old boy is not scared to go there!
Thank-you also to those who are looking after our other three blessed cherubs while we travel back and forth to Sick Kids for the pre-cath appointment and for the MRI & cath procedures! It's great to know our kids are happy and in good hands!
Brian and Mel