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!

Cardio Appt - August 10

Tyson had another cardiologist appointment this week, as well as an ECG, chest x-ray and blood work.

There are really no changes in his condition, except that his O2 saturations have been sitting a little higher these days. We praise God for that and are thankful that most of the time we see numbers in the mid-to-high 80s! At his cardio appt his O2 sats were 88 :) The nebulizer mask for his asthma is doing a good job!

Tyson didn't need an echo today because he just had one in May and he is having another heart cath procedure on Sept. 12.

Tsyon still goes for blood work bi-weekly at the lab in Shelburne. His INR level has been therapeutic (between 2.0-3.0) for the past number of weeks on 3 mg of coumadin per day. I like the 3mg/day dose much better than the 2 mg-2 mg-3 mg rotation we've been doing all summer. It's so hard to remember what he had the night before and we would always have to write it down, such a pain. Now it's a consistent 3 mg every day.

I talked to Dr McCrindle again about the Gleevac study being done at Boston Childrens' Hospital. (The chemotherapy drug being used to treat pulmonary vein stenosis.) He hadn't heard back yet from the cardiologist in charge. So I emailed the nurse in Boston again and also forwarded her email address on to Dr McCrindle. If we are going to participate in this study, we need to get acting on it quickly. PVS is a notoriously progressive disease and we are wasting precious time. Before we know it, fall/winter will be here and with that comes all kinds of colds and flus. I sent Dr McCrindle an email suggesting that if Tyson does qualify for the Gleevac study, would they consider ballooning or dilating his pulmonary veins during his cath procedure on Sept. 12 so that they are as open as possible before starting the chemo drug. He said Dr Benson (the cardiologist performing the cath) will assess the veins and may consider dilation or a stent implantation during his cath, but this may disqualify him for the Gleevac study. Having said that, if the stent is successful he may not need the Gleevac to keep his veins open.

We're not exactly sure how we'd pay for all the tests that will need to be done in order to prepare for the Gleevac study. First we'd have to send off CD copies of all his recent echos/MRIs/heart cath/chest x-rays, etc. to Boston for their information. Once they have all his info, Tyson would need an echo/lung scan/bloodwork/CT scan at Boston Childrens Hospital. (I'm not sure we want to know how much all that costs! So thankful for OHIP here!) After that initial testing there, Sick Kids can do all the follow-up testing and send their results to Boston. Brian doesn't have group health benefits so we're not exactly sure how much all this would cost but we know that the drug itself is paid for by the drug company. Good thing too! It's over $200 per day and the study requires being on the drug for 48 weeks...close to one year! If the drug wasn't being covered, we'd never consider being part of this study because that's way more than we can even earn in a year!

For more information explaining the Gleevac study, visit this website from Boston Childrens Hospital. Don't worry, it's very easy-to-read :)

http://www.childrenshospital.org/clinicalservices/Site683/mainpageS683P5.html

We'll keep you posted again once Tyson's goes for his heart cath on September 12 (pre-op on September 7.)

Mel:)




Tyson's Camping Experience & Other News

We got home from a 2 week holiday a little while ago and I've got to tell ya, we sure could use another holiday! Tyson was out of sorts the entire week at Earl Rowe and kept asking to go back home. He is such a home body and doesn't enjoy big crowds so we weren't too surprised that he didn't enjoy camping all that much. He was just not himself at all. He took two nasty falls to the back of his head onto a concrete pool floor, resulting in a trip to Alliston hospital. Thankfully there was no evidence of head injury or brain bleed (something we have to be very careful with since he's on blood thinners.) Oh, the joys of a 2 1/2 year old on blood thinners!

We then moved from Earl Rowe to Horseshoe Lake Camping and Cottages, where Tyson was not much happier. The first day we were there he was complaining of a headache and owie tummy (which also could mean he has a tight chest.) In the back of our minds we were still thinking about his head injuries, since symptoms can sometimes take 24 hours to show up. And he wouldn't settle down to sleep at night even after we gave him Advil, and he then spiked a fever. He had been coughing for a few days prior and the nebulizer mask we were giving him managed to keep his O2 sats stable, but his heart rate that evening was out of control. So we took him in to Parry Sound hospital where we told him our suspicions of either a head injury or pneumonia. The doctor immediately assessed him for a head injury right there in triage. Tyson was given an OK in the head injury department, but guess what the chest x-rays found?....Take a guess now....pneumonia! Antibiotics and regular ventolin masks through the nebulizer had him feeling better and enjoying his holiday by about Thursday afternoon.....and we left to go back home on Saturday :)

I have to admit that I'm a little worried about this upcoming cold & flu season. If Tyson can catch pneumonia in July, what is October or November going to bring?

We got a call from Sick Kids cath lab regarding Tyson's next heart catheterization. He will be going in for his 5th heart cath on Monday, September 12. During this procedure the cardiology interventionist will attempt, once again, to close his fenestration (the surgically-made "hole" used to relieve pressures in his lungs until his body gets used to his new Fontan circulation.) If they can indeed close the fenestration, it will make his O2 sats increase by about 5 - 10 % and will give him some more "breathing room" in the event that he does catch a respiratory illness this fall/winter. We are trying not to be pessimistic about it, but we're not setting our hopes up too high regarding this fenestration closure. If the pressures in his lungs were too high in June because of the pulmonary veins stenosis, it's not too realistic that they will suddenly be ok to proceed with the closure. This is an attempt to buy Tyson more time before he will eventually need to be listed for heart and/or lung transplant.

Tyson is now back to his normal self again. He is currently being toilet-trained and things are going relatively well. He is enjoying having his older brothers home for the summer and loves swimming in our backyard pool. His energy levels have been good again, and since he's been on the nebulizer mask, his O2 sats have been sitting in the mid-to-high 80s! So we're keeping him on the mask twice daily to keep his airways open and clear.

In other news, in case you haven't heard yet, Brian and I are expecting another baby in January 2012! We are trusting God's timing in this too, and have several appointments set up to ensure that this baby is healthy before proceeding with plans to have midwives deliver him/her.

First of all, my prenatal bloodwork revealed that my body is producing an antibody called "anti-c" which could potentially cross the baby's placenta and cause harm to our baby. Right now the level of anti-c is still quite low but often increases as we go farther along in pregnancy. In order to manage this minor road block, I have been going for monthly bloodwork to keep a close eye on the anti-c levels, and may need regular ultrasounds to make sure that the anti-c is not attacking our baby's red blood cells. In the event that the anti-c levels continue to rise and could cause harm to the baby, I would need to be induced at Mt Sinai in Toronto and our baby would need a blood transfusion immediately following birth. In extreme cases where the anti-c levels get dangerously high but it is too early to induce, baby can have in utero blood transfusions.

Our midwives in Alliston consulted their team of OBs, who have recommended that I consult with an OB at Mt Sinai in Toronto and also have a level 2 anatomy ultrasound there at the same time. This way, if the anti-c becomes a problem and I do have to be induced there, they will already know me and our baby. In addition, Brian and I have requested to have a fetal echo of our baby so that if we do have another baby with a congenital heart defect, we are well-prepared.

Fortunately, we have all these appointments scheduled for the same day in Toronto, Thursday September 1, 2011! Our baby's fetal echo (ultrasound of his/her heart) is scheduled for 8am, a procedure which can take a couple of hours because we already have a child with complex CHDs. They will want to be extra cautious and look for absolutely everything.
In the afternoon I will have a level 2 anatomy ultrasound (a specialized ultrasound which looks at placenta, umbilical cord, all the organs, etc with higher-tech equipment.) Thankfully Kenya was born into this world healthy and well, as an unplanned emergency home-birth; but after she was born the midwives found that she had only a 2-vessel umbilical cord! This is another reason we'd prefer a level 2 ultrasound as opposed to what we'd have in Orangeville. After the ultrasound, we will meet with an OB at Mt Sinai, Dr. Windrim, who will discuss the findings of the ultrasound with us, as well as go over more about this anti-c antibody and how to manage it.

Thanks for checking in again! Prayers for a successful heart cath for Tyson, a healthy summer, and a heart-healthy little baby would be much appreciated!

Mel:)