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!

Cold & Flu Season Has Begun....

It's here, folks. The time of year I dread the most. Cold and flu season. As much as we try to stay cooped up in the house to avoid all the bugs flying around, it's very difficult to avoid everything completely because Tyson and I often have to be out for appointments, and our other kids go to school and bring home all sorts of nasty things. It's inevitable. Tyson will get sick. The question is: can he fight it this year? Will he spend much of the upcoming winter in the hospital, or on antibiotics, or can his immune system handle the slightest cold this year?

Tyson came down with his first cold a week ago. He was the first in the family to catch it so we're not sure where it came from! We let his symptoms go for a few days because his 02 sats were still low 80s (Sick Kids doesn't want them lower than 80 or Tyson will have to be on oxygen.) We immediately began the ventolin mask every 4 hours as soon as the cold symptoms began, but by Thursday he was coughing pretty badly and didn't sleep well the night before so I had to take him in to see his Dr. Dr said he had a slight pneumonia in the upper lobe of his right lung, so prescribed him an antibiotic to help him heal, despite Tyson's energetic and bouncy behaviour at the Dr's office. He sure didn't look sick when we brought him in, but we know how quickly that can change with him. Anyway, we debated on whether or not we should actually fill out the prescription because he seemed to be on the mend. And I hate the idea of having Ty on drugs all winter long. But then Braden came home from school with a sore throat and barky cough, so we continued the antibiotics for Tyson to be proactive and hopefully keep Braden's cold away from Tyson. So far all of us in the family have had the sore throat cold that Braden started with last week, so we are glad that Tyson can be protected from it. However, he is still coughing and I'm not convinced that the antibiotics are actually treating his pneumonia. It is hard to tell if the pneumonia is viral or bacterial unless they swab him. If it's viral (which I think it is since he's been on antibiotics for 5 days now with no huge improvement) then the antibiotics really do nothing. Except keep this other cold away from him. We will continue to keep an eye on him and if the coughing doesn't stop by the time he's through with the 10 days of antibiotics, we'll have him seen again. His sats are still hovering in the low 80s, occasionally dipping to 78-79 at night when he's sleeping. Tyson is pretty good at letting us know when he's not doing well, so we are constantly assessing his behaviour for signs of distress.

We decided this past March when Tyson was hospitalized into Cardiac Critical Care Unit at Sick Kids with Influenza A and pneumonia, that he would absolutely be getting the flu shot this year. Anyone who knows us, knows we have never been big on the flu shot. But I would rather have Tyson covered for this season's most common colds and flus than spend another 2 weeks at Sick Kids because we didn't take the necessary precautions. Tyson will also be getting the RSV prophylaxis shots once every month this season too. RSV is a serious respiratory tract infection that affects the lungs and can be fatal in children with severe cardiac and pulmonary issues like Tyson. He received these shots during his first two winters, from November to April. Ontario Disability Support Program (ODSP) covers the RSV shots for anyone with chronic lung or heart disease, premature babies, and infants with auto-immune deficiencies. Because Tyson turned 2 last November, I assumed he was no longer covered so we did not ask about the shots and prayed he didn't catch RSV. However, both his respirologist and cardiologist (as well as family doctor and pediatrician of course) feel it's imperative that Tyson receive these shots again because of his pulmonary vein stenosis. His cardiologist is writing a special request to ODSP to make sure the shots are covered (they are $900 for each monthly shot!) We are thankful for that. Please continue to pray with us that Tyson has a healthy winter and that he can be kept away from any serious respiratory illnesses. We love Sick Kids but really don't want to spend the winter there!

Other than the cold this week, Tyson is doing very well. He is talking more and more each day, especially on the days that he is home alone with me and all his siblings are in school. He will be starting weekly speech therapy sessions this fall, we're just waiting for the call from Headwaters to find out when he starts. I don't think he'll be in therapy long since he's not doing that badly. He sure has come a long way! Tyson continues to make us laugh and cry. When he's happy, the world sure knows about it; and the same goes for when he's upset. His personality reminds us a lot of Merrick when he was this age, just goofy and silly and active, and really into sports. He also enjoys reading books. Lots of them. I get so tired of reading the same books all the time! Last winter I took him to Story Time at the library so I could take out some different books, but I am so paranoid about taking him anywhere for fear of what he could pick up while we're out. The smallest little sniffle or cough to a healthy child could mean terrible things for Tyson. We're out often enough these days keeping up with all his appointments and he's at a babysitter often enough while I have my appointments for this baby.

Before his cold, Tyson's 02 sats were definitely improving from this past spring and summer. In June when he had his heart cath, they were low 80s at best. That is why they had originally scheduled another cath in September, to try close the fenestration so his sats could hopefully increase. However, since about August or so and he's been on the nebulizer mask 2X per day, his 02 sats have improved to the high 80s, low 90s. We've seen a 92 even! During his pre-cath appointment his sats were 90, which is probably the main reason they postponed his cath. Why fix what isn't broke?

Tyson's new heart cath date is December 5th. Hopefully that gives enough time for some of the pressures in his heart and lungs to decrease so they can go ahead with fenestration closure. If not, I'm sure the surgical team will come up with a wonderful plan for Tyson. It may involve transplant. Brian and I have been discussing the idea of asking that they place Tyson's name on the transplant list now while he is still clinically well; even if his name goes on the very bottom of the list. Why wait till he is unwell and his heart or lung is failing before deciding he needs to be on the list? I know things go by priorities, but we'd rather not watch our child get very sick and wait months in the hospital for a transplant. Many infants/children die while waiting for their gift of a new heart. So after his cath, if things get to that point, we will have a long talk with Tyson's cardiologist.

Please continue to keep Tyson in your prayers. Pray that he may be spared from any serious illnesses this winter, that his heart and lungs will continue to function well and that the path down the transplant road can be as long as possible. Also while you're praying for him, put in a word or two for our family too. That we can all remain healthy too, that our unborn baby can continue to grow healthy and strong, that God will prepare us for whatever He has in store for us all this winter, that we can experience God's peace knowing that all things come not by chance but by His Fatherly hand.
Thanks,
Mel:)





Tyson's Heart Cath Cancelled

I got a phone call this afternoon from Tyson's cardiologist Dr McCrindle about Tyson's heart catheterization set for Monday. The cardiology interventionist performing the cath, Dr Lee (who also did Ty's cath in June) spoke with Dr McCrindle today and she is uncomfortable doing Tyson's cath this week because Dr McCrindle will be away on holidays during the procedure. They both feel that attempting a closure at this time would be borderline, because the pressures have likely not changed much since his last cath in June. Dr. Lee would need to make a decision right there in the cath lab, while Tyson is intubated and under general anaesthetic, about whether or not to go ahead with the closure. It would all depend on how his heart and lungs react to the temporary test occlusion they do during the first 20 minutes of the cath procedure. So it would be a case of "should we or shouldn't we?" Dr Lee wants to wait till Dr McCrindle is available for consultation about whether or not to go ahead with the closure, and he won't be back till the end of September. They also feel that there is time to wait because right now Tyson is healthy and doing so well, and his 02 sats are generally sitting in the mid-to-high 80s. (Whereas in June when they planned this cath, his sats were just at 80-81. Since he's been on his nebulizer mask he has been so healthy and not wheezing or struggling at all.)
Brian and I are feeling so relieved about this cancellation. We both wondered whether or not the timing was right for Tyson, since he seems to be doing so well lately and his 02 sats are generally sitting in the mid-to-high 80s. At Sick Kids yesterday he was 90. So why fix what isn't broken, right? If you read my last blog post that I wrote just last night, I expressed a number of fears about this procedure, mainly due to the risks the cath poses, and God has indeed calmed those fears for us today. We've been feeling pretty nervous about having the fenestration closed, because if done prematurely it can lead to serious complications afterwards, but at the same time we prayed "Your will be done, O LORD." God has again shown that His will is the way. The time is not right for Tyson yet.
Dr McCrindle will see Tyson again at his next cardiologist appointment in November and then will most likely make plans closer to December to attempt a closure. In the meantime, once he gets back from holidays at the end of September, he will consult an oncologist at Sick Kids to discuss the possibilities of prescribing Tyson a chemotherapy drug called Gleevac to potentially prevent his pulmonary veins from narrowing even further. There are laws and regulations that must be followed because a cardiologist can't just prescribe a chemo drug to a cardiac patient without reason. Since pulmonary vein stenosis mimics cancer in the way that unwanted cells grow inside the vein tissue, there is a theory out there that suggests that chemotherapy can prevent the cells from multiplying further. It doesn't un-do what is already damaged, but it could stop the veins from narrowing further. Gleevac would also come with risks and side effects (it's a chemo drug afterall) and Tyson would need to be followed very closely with an oncologist to make sure his white blood cell counts stay where they should be and to make sure the risks aren't greater than its benefits. I will update on that once we hear more about if Gleevac is a possibility for Tyson. (I do know that the drug costs $200 per day...so we're hoping Tyson's ODSP benefits will cover it!)
In all this, we still don't save ourselves a trip to Toronto on Monday, because I still have an appointment at Mt Sinai in the afternoon to follow-up on my ultrasound and more bloodwork. But at least it saves our family from being separated again for a night or two, and we can let go of the stress of an upcoming heart cath procedure.
Have a great weekend, ours now looks a whole lot brighter :)

I've posted a few pictures of our kids from the Labour Day weekend. We went to the air show in Toronto and then we enjoyed some rides at Ontario Place. Tyson was in such a good mood that day and loved the water park. It was a really hot day and was wonderful to enjoy some family time before the cold and flu season hits us and we are forced to stay cooped up more.


Kenya (5) and Tyson


Braden (10) and Ty

Merrick (8) and Ty


Cooling off on the last hot day
of summer :)




Pre-Cath Appointment, Sept 7

Tyson had his pre-cath appointment at Sick Kids today. Since he was just there for a cardiologist visit in early August, he didn't need to repeat his echo, ECG or chest x-rays today so all he needed was some bloodwork to make sure his counts were good to go for his upcoming cath on Monday.

Tyson had a pretty good day, spending most of his time in the CDIU (cath lab) waiting room watching tv and playing with toys. We didn't have a lot of tests for him to repeat but we did have to wait for the cardiologist who was held up in the cath lab for a few hours.

We did finally get to speak with a cardiologist fellow who will be aiding in the cath. She went over the procedure again (briefly - since we've done this 4 times already and we're old hat at it!) and all the risks involved. So we signed consent and Tyson is good to go for Sept. 12.

I also asked that a Child Life Specialist visit Tyson today and explain to him what was going to happen to him, so that he is prepared for Monday. It turns out we probably didn't need to go over it again since he just had a cath in June and he remembered everything that she told him from the last time. He knew exactly where to put the gas mask on the "heart buddy's" face and he knew that the IV would be in his hand when he woke up. When we told him he would go for a little sleep and when he woke up he could lay in bed and watch tv, he started crying a very sad cry and pointed to his chest and pleaded, "No owie heart!" Then I knew for sure he understood exactly what was going on and he was probably remembering his last open-heart surgery in December. We had to reassure him that this time they wouldn't be hurting his chest but he would just go for a long sleep and have some band-aids on him and an IV in his hand when he woke up. He did calm down after that but he is still awfully unsure about whether he wants this procedure or not. It's amazing what little ones can comprehend!

Brian and I can't decide whether we pray for them to close the fenestration this time, since that can lead to very serious complications if Tyson's heart and lungs are not ready to handle it yet, (and the only way to retrieve it is through an open-heart surgery;) or if we pray that they NOT close it, since that won't keep his O2 sats very stable over the winter months when he tends to catch every virus that flies around. Either way, we do trust the cardiologists involved in the decision-making process and even more, we trust that God will lead and direct them to the right decision.

So, if the fenestration closure is successful, because Tyson is sort of "borderline," we would most likely need to stay the night at Sick Kids for more observation. If they can't close it, we will go home within 4-6 hours after he enters the recovery room, and the surgical team will have yet another meeting to decide what the next step is for Tyson.

I myself really struggle with the "what-ifs." What if they close the fenestration and Tyson's heart and lungs can't handle it and he develops PLE? (protein losing enteropothy - a common side effect from fenestration closures that affects the way their bodies digest proteins; it requires life-long management.)
What if he goes into cardiac arrest during the procedure and he ends up with severe damage to his heart, and we have to stay in the hospital all winter long waiting for another heart?
What if he can't handle the fenestration closure and his heart and lungs start to fail over the next few months but we don't recognize the signs and symptoms?
What if he has a stroke while in the cath lab and we lose the Tyson we know and love? Or he becomes a totally different boy to us than the one we knew before? What if he loses his ability to walk or talk or be the fun-loving kid he is to us?
What if while they are trying to close the fenestration, the disc slips off the catheter and it flies off into another portion of his heart, causing either stroke or aother open-heart surgery to retrieve it?
What if complications arise and he starts severely bleeding and he ends up staying at Sick Kids for a lot longer than what we're prepared our other children for?

These questions are never far from my mind, and while they don't last long they are still there. At the same time, just as I am breathing the worries and doubts to my Father, I feel His presence and know He is a loving and powerful God who reassures us time and time again:
"Be strong and courageous. Do not be afraid; do not be discouraged, for the LORD your God will be with you wherever you go.” Joshua 1:9
"In this world you will have trouble. But take heart! I have overcome the world.” John 16:33

Our baby has a healthy heart!

Today Brian and I went to Toronto for a few tests and appointments to check on the health of our new baby. We had a fetal echocardiogram of our baby in the morning at Sick Kids. We're happy to report that our baby has a healthy four-chamber heart! It sure is amazing how they can get such a good picture of a fetal heart that is only about 2 cm in diameter, all from within a womb that is only 19 weeks pregnant!
Next we had an anatomy ultrasound done at Mt. Sinai to make sure that all the baby's other parts are developing normally. Here again, we are rejoicing in the news that everything appears to be healthy and well! The baby was very stubborn and would not move out of his/her fetal position, was curled up in a ball laying spine-down, so the sonographer didn't get a very good look at his spine. But everything else looks wonderful so far.
As far as the "anti-c antibody" levels in my blood, the antibody has seemed to have disappeared for now. For how long, we don't know. The OB at Mt Sinai would like me to re-do the blood work again in two weeks to double check that it wasn't a mistake, and continue to monitor the levels throughout the pregnancy to make sure the baby is not harmed. My first two blood work levels indicated very low anti-c levels, ( 1:1 - almost the lowest titre level you can have) low enough that we can continue with midwifery care. If the levels get higher than 1:16, it will mean a Mt. Sinai delivery, with a blood transfusion to baby if needed. If the antibody stays gone, or the levels remain very low, we can still have midwives deliver the baby but he would need to be seen by a paediatrician shortly after birth, have bloodwork taken to check on his red blood cell levels, and then if jaundice begins to develop phototherapy would take place without any hesitation.
So, since I have to have the blood work re-done in two weeks, and since the ultrasound didn't give a good picture of the spine, we have another Mt Sinai appointment booked on Sept 12, the same day as Tyson's heart cath at Sick Kids. This will save us a trip to Toronto (we're already making 3 trips in 3 weeks this month!) and Brian can stay with Tyson in recovery while I get my ultrasound and another anti-c level done. Brian had more bloodwork done today, since his first draw only reported his blood type and not if he had the anti-c in his blood, so they would like to have on file where the little c antibody came from.
All in all, it was a very long but productive day. We're thrilled that our baby has a healthy heart and appears to be growing and developing well so far. God has answered our prayers in a huge way today. We're also praying that He will answer our prayers about Tyson's heart and lungs, and that his heart cath procedure on Sept 12 will go well.
That's the update for today! YAHOO! Praise the Lord!
Brian and Mel:)