When I called Sick Kids today to let them know Tyson was sick, the cardiologist on call said that if it was just a cough he could still come in on Monday and be assessed. (Sometimes a cough indicates heart failure and not a cold - in that case they would want to proceed with the cath!) But because it is associated with the sniffly nose and fever, they would have just sent us home if had we come in.
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 nine 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!
When I called Sick Kids today to let them know Tyson was sick, the cardiologist on call said that if it was just a cough he could still come in on Monday and be assessed. (Sometimes a cough indicates heart failure and not a cold - in that case they would want to proceed with the cath!) But because it is associated with the sniffly nose and fever, they would have just sent us home if had we come in.
Tyson is good to go for Monday's cath procedure, provided that he remains healthy. He has had a very slight sniffle but it's so hard to say if it's really a cold or not. We tend to over-analyze things when we get this close to the cath and every cough or sniffle causes our heart to jump. The problem is Tyson struggles with nose bleeds (because of the blood thinners) so he tends to freak out if he feels any moisture in his nose whatsoever. And because he's on the nebulizer mask every day, it can cause quite a bit of humidity in his nose and he does drip a little afterwards. Anyway, he looked very well at pre-op clinic today and he appears healthy enough to go ahead.
On the way to Sick Kids this morning, Tyson just about broke my heart with something he asked me. We've always wondered how much of his heart condition and his surgeries he truly understands. Today he asked me, "No open my heart, Mom??" I just about choked up , and quickly reassured him that there was no open heart surgery this time. But wow, what a question for a three year old to ask! When the Child Life Specialist explained the details to him today, he already knew exactly what was going to happen. Afterall, we did just review this with him in September when his last cath was cancelled, and he just had a cath in June too. He knows that he is going to wear a watermelon-scented mask which will make him fall asleep, and when he wakes up he will have an IV in his hand, a band-aid on his leg (they enter his heart through a vein or artery in his groin) and that he can lay down in bed and watch tv for a few hours with Mom and Dad until he feels better. He is excited to get to watch the movie Cars!
Tyson is first case on Monday morning. His procedure is scheduled for 8am, we have to be there for 7am. He will be having an MRI first (to assess the pulmonary vein situation) and then will go to the cath lab. The entire procedure will take about 2 1/2 to 3 hours. Then he will need at least 6 hour recovery time before we even think about heading home. And if they do close the fenestration it may be an overnight stay for observation purposes.
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.
Tyson went for his regular check-ups at Sick Kids on Wednesday, November 2 to see the cardiologist and respirologist.
Tyson didn't have an echo this time so there isn't a whole lot to report about his cardiac condition. (Other than he still has half a heart LOL.) He now weighs 31 pounds and his O2 sats in clinic were 87. We used to see numbers in the high 80s-low 90s but unfortunately that didn't last long and now at home we often see low - mid 80s again.
Dr McCrindle, cardiologist, made a special notation in his calendar to be available for consultation during Tyson's heart cath scheduled for Dec. 5. Last cath in June he was away on holidays. Again in September he was away, and the scheduled cath was postponed because the cardiology interventionist wasn't comfortable making decisions without Dr McCrindle around. Dr M said he is 100% certain they will be paging him from the cath lab to decide what to do about the fenestration closure. First they will perform a test occlusion (balloon it closed temporarily to watch how his body reacts,) then observe Tyson closely for about 10-20 minutes to see how the closure will affect his pressures. If his pressures remain stable, they will go ahead with placing the metal disc inside his heart to permanently close the hole. This will stop all mixing of red and blue blood (oxygenated and de-oxygenated blood) and help raise his O2 saturations, giving him a healthier winter, and hopefully buy him more time before another surgery. However, if the pressures elevate too much during the test occlusion, then fenestration closure is not possible for Tyson this time. Our hopes aren't set too high that the closure will be a success, because his venous pressures and pulmonary artery pressures are very high due to the stenosis in his left lung veins. So we won't be surprised if they cannot close the fenestration.
Dr M also reiterated that the results from this cath procedure will determine what is next for Tyson. He will be presented to the surgeons again for consideration. Consideration for either another open heart surgery on his pulmonary veins or for transplant surgery. Either way, his little chest will need to opened up once again :( I asked Dr M, "If we know that transplant is inevitable for Tyson's future, why don't we list him for a new heart/lung right now, even if that means he's on the bottom of the list. We don't want to watch Tyson get sicker and sicker and then have to go into such a huge surgery already weak." Dr M assured me that they will not wait for Tyson to be sick before they list him. This upcoming cath will be a deciding factor for what to do next.
Although all this information is not at all new to us, it is still difficult to hear and accept. It's so hard to believe that our little Tyson, who is growing and developing so well right now, has such a sick little heart. He runs around without a care in the world like any other healthy boy, doing all the things other boys do at this age. He's talking a mile a minute, he's outgrown his afternoon nap, and he's completely toilet trained. (Yes, the boy who adamantly declared that "underwear is DUMB!" was toilet-trained within two days!) How can this boy, who looks so normal on the outside, who can for the most part keep up with other children his age, be so sick on the inside? I don't get it either. It's hard to accept. But, as my insightful husband reassures me, "We don't have to accept anything yet. We accept that we don't know." Ok, true.
The appointment with the respirologist also went off without anything new to report. Tyson's asthma is very well under control since he's been on the nebulizer twice a day as preventative therapy. He has caught a few small colds this fall and although he does get wheezy from it, the ventolin and pulmicort are doing their jobs and he hasn't had to be hospitalized from anything so far.
Dr M did stress keeping Tyson away from sick people, especially with his upcoming cath. They won't put him under general anesthesia or intubate him if he catches the slightest thing. So it's officially "lock-down" time for Tyson. He'll only leave the house when necessary.
That's the latest news on Tyson. We'll report more after his pre-cath appointment on November 30. Keep him in your prayers please!
(Sorry, for some reason the link won't work...)
It basically says that parents of children with severe congenital heart defects experience more stress, as well as more sadness and anger than parents of healthy children. Though I didn't read the article in its entirety, and though I do agree with this statement, I have to hope they didn't spend too much money researching this "phenomena" because I could have made this same conclusion for them for *FREE*! Of course parents of children with severe heart problems experience more stress! I shake my head that they actually took the time to research this query!
One thing that the article did fail to point out is the immense JOY that parents of heart babies can feel over the simplest things in life. Like today, for example, Tyson walked with me all the way to Henry and Marg's driveway and back again without getting tired. Like, he walked it himself, no stroller involved. He actually wanted to walk farther and longer but the clouds were threatening rain so I coaxed him back inside again. This is huge for Tyson, who normally can't walk the length of our driveway, or the church parking lot without turning funny colours. The things most parents take for granted are huge milestones for us heart parents. This gives me so much joy. When Tyson nursed for the first time again after his first open-heart surgery, the first time he waved bye-bye, sat independently, pulled to a stand, took his first steps and spoke his first words, the tears just come from out of nowhere. No warning. No "look out, I'm coming!" They're just *there.* Perhaps because ever since I've become a heart Mom I've always been on the verge of tears. Not always happy tears, mind you. Sometimes the tears flow while I'm in the middle of a tight hug with Tyson, but they aren't only tears of joy. Tears of fear more like it. Tears that make me wonder, "How long will I get to hold onto my little miracle?" LORD, help me to hold on to these memories, to appreciate every single moment of his life, and never forget what a blessing Tyson is.
I am guessing that the reason for my emotional ride today is because Tyson's third birthday is quickly approaching. THREE! My miracle baby is going to be THREE!! I am so thankful that God has blessed us with three years with this wonderful kid. And he really is a wonderful kid. For those of you who know Tyson, you know how much of a character he is. He is just great! He keeps us laughing all the time. He has this thing where he gives people the thumbs-up at the funniest times. Most of the time he will give me the sign on his way up the stairs, with his hockey stick and ball in the other hand...knowing he's not allowed to come upstairs with them. Or he'll try to butter me up first with an "I love YOU, Mom!" and a sparkle in his eye. I just look him straight in the eyes and stare. And then he slowly withers his way back downstairs with said hockey stick and ball and a hurt look on his face. But the thumbs-up sign is the cutest. He will see someone he knows sitting on the other side of the church and give a big cheeky grin accompanied by the thumbs-up sign. It sure generates a lot of smiles from others too. He even gave one minister the thumbs-up sign when he looked down at him from the pulpit.
The other day, I was thinking it's about time I try to toilet train this kid; he's almost three and it can be done. (Besides, I'd love to enjoy at least a few diaper-free months!) So I've been talking with him about the toilet, wearing underwear, and I said that if he learns to do all his jobs on the toilet, he gets to wear his "Cars" underwear. To which he responds, "No! Underwear is DUMB!" Gotta love that kid! Toilet training him could prove to be a very interesting experience...
He's also been extremely healthy the past few weeks. With 02 sats back up in the high 80s-low 90s, we are ecstatic about the work God is doing in his heart and lungs. Brian and I can't help thinking that either his fenestration is closing on its own, which it can sometimes do, or that God is working a miracle in his left lung and the disease is not progressing. His next cath is scheduled for Dec 5 in which they plan to attempt a fenestration closure again and assess the severity of the stenosis (narrowing) in his left pulmonary veins. The fenestration closure, if successful, will bring his 02 sats even higher and give him more breathing room for this winter when he inevitably will get sick. I am feeling anxious about this procedure already, and we're still well over a month away. I hate that this amazing little boy has to endure such invasive procedures so often. And I also hate admitting to the possibility that he may face another open-heart surgery in the future. The cath in December will give the cardiologists a good indication of what exactly has been going on in his hearth and lungs the past six months. Is his heart finally adjusting to the Fontan circuit? Can they indeed close the fenestration now? Has the stenosis in his left lung progressed? Sadness and Joy. Two emotions we feel at the exact same time each and every day. We confess and acknowledge that God is so good, yet each and every day I am still so scared for his future.
Ok, now on to more joy. I like that topic better :) Each and every day Tyson's appetite seems to increase. This is a huge blessing because a LOT of heart babies (and I mean A LOT...probably the majority of heart babies) cannot eat normally but are fed by either Ng tube or a permanent G-tube surgically placed into the stomache. Or they suffer from severe reflux issues. Tyson is also beginning to become more independent when he eats, doing most of the work himself. Now at almost three years old, most kids have been eating on their own for about a year or more. But not Tyson. He has been finicky and picky and very difficult at times. He continues to gain weight despite his heart burning off more calories than the average child. When Brian and I watch him eat at dinner time these days, we can't help but be thankful and reflect on how far he's come. It's amazing that a kid who has gone through three open-heart surgeries in the first two years of his life can still be so "normal."
Tyson's speech is continuing to improve every day too. He seems to be talking a-mile-a-minute lately, especially on days when our three older kids are in school and it's just Tyson and me together all day. During our short walk today, Tyson did not close his mouth from talking for even a second. We are still waiting for a call from Headwaters to get him in weekly speech therapy sessions, but I am optimistic that they will quickly discharge him once they see how much progress has been made over the summer.
Anyway, I promise to post some more pictures soon. That is, if our family pics turn out ok. We had our cousin Brandy over two weeks ago and I'm not sure we could get a very good shot of Tyson; he was too busy being a big goof. When she wanted to take some pics of him by himself, he absolutely refused to take off his hockey gloves. Thankfully he did listen better to her than to me; she was able to bribe him that if she let him wear the gloves for a few pics, he would take them off to get some pics without the gloves. Later when trying to get a nice family shot, when the photographer said, "Everybody ready? Say cheese!" Tyson would either pull a funny face or burp loudly. Yes, regrettably he has somehow mastered the skill of burping on demand. Of course his brothers and sister also thought it was quite hilarious, so we'll either have a family pic with everyone laughing hysterically, or with Tyson making a funny face. I just love this kid!
Won't you please pray that we get to keep our joyful little guy for a really long time? It would break our hearts if the joy he exudes to us was someday gone. Also in the way of prayer, we'd also appreciate prayers that his heart adjusts to the Fontan circuit so that he can avoid future surgeries, and that the pulmonary vein stenosis problem will miraculously disappear.
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.
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 :)
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
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 :)
We'll keep you posted again once Tyson's goes for his heart cath on September 12 (pre-op on September 7.)
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!
The respirologist at Sick Kids asked 101 questions about Tyson's past illnesses, when he started on puffers, how they have worked for him in the past, how many times he's had pneumonia, what triggers his wheezing, etc. From there he came to the conclusion that it does indeed sound like Tyson suffers from asthma. In light of what his cardiologist suggested about him needing aggressive lung therapy, the respirologist has prescribed him Singulair, a daily pill which will help relieve the inflammation in his bronchials (asthma preventative.) We are to begin this treatment as soon as the kids start school again and may come home with viruses harmful to Tyson. (Unless he gets sick before that time, we will begin it then.) He also prescribed some more liquid ventolin, to be administered through his nebulizer. One nebule of this ventolin is equivalent to 25 puffs from Tyson's ventolin inhaler. No wonder it worked wonders on Tyson in June when his family doctor prescribed it! The other medication is Pulmicort, a corticosteroid much like his Flovent puffer, used to control asthma symptoms and prevent asthma attacks; but again this is used with his nebulizer so it will be much more effective in controlling the symptoms. The respirologist also recommended that Tyson receive regular RSV shots again this winter, and of course the flu shot. Normally, RSV shots are only available for cardiac patients up to the age of two, but given Tyson's struggles with respiratory illnesses, they are going to apply for Tyson to have them covered again this year too. We'd rather do the RSV shots (which Tyson had for his first 2 winters and he tolerated them very well) than end up in Critical Care. We've seen what RSV can do to healthy children, I don't want to think about what it could do to Tyson!
We also spoke with Tyson's cardiologist, Dr. McCrindle again. He let us know that he put in the order for another heart catheterization to take place within the next 2-3 months. He also wanted to check Tyson's sats (they were 86! We haven't seen numbers that high in a long time!) I also managed to squeeze in a few questions, one of them being, "If, during his next cath the test occlusion goes well, and they are able to close his fenestration, does it still mean he'll need a transplant?" **hope in my voice** Dr. McCrindle hesitated, then wrinkled his nose, then shrugged his shoulders and said, "There are no guarantees. It's too soon to say." *sigh* Ok, so I tried :)
I left the hospital feeling optimistic and hopeful that the new medications will help manage his asthma and when cold & flu season hits us again, we'll be praying that Tyson can stay healthy and well. The longer he can stay healthy = the more stable his sats will be = the less his heart will have to work, and hopefully = the longer the "transplant road" will become.
Thanks for checking in on Tyson! Prayers are always appreciated! (Don't forget to ask for a miracle that the pulmonary vein problems will resolve, that's his biggest issue right now :)
declares the Lord,
"plans to prosper you and not to harm you,
plans to give you hope and a future."
Jeremiah 29 :11
The surgical team met today to discuss Tyson's case. This team is made up of a number of staff cardiologists, Dr. McCrindle himself, the cardiology interventionist who performed Tyson's recent cath Dr. Lee, and Tyson's cardiovascular surgeon Dr VanArsdell, among several other professionals. After reviewing his latest echos, his cath report and his MRI findings, they've come to the conclusion that it is not only the left lung which is causing the high pressures but there is also something going on in the right lung as well. I don't think there is anything wrong with his right pulmonary veins, but Dr. M said that the entire Fontan circuit is not operating well. In order for you to understand exactly what I mean, let me explain an important detail to you. In your healthy heart, your right ventricle pumps de-oxygenated blood to your lungs, your lungs oxygenate that blood, and then your left ventricle pumps the oxygenated blood to the rest of your body. Your heart always get the exact correct amount of blood it needs in its lungs because your right ventricle is pumping exactly as it should. Because Tyson doesn't have a functioning right ventricle, he has nothing pumping the blood to his lungs. The only way blood can get to his lungs is through natural flow which the surgeons have created throughout the last 3 open-heart surgeries. The Fontan surgery completed the system, and Tyson's blood is now flowing (not pumping) to his lungs through a pathway which we call the "Fontan circuit."
Tyson's heart is not handling the Fontan circuit well. This is why he has been sicker more often and had more health struggles after his Fontan than before. Most kids do very well after their Fontan and are in the hospital much less often. Most kids graduate to annual appointments after the Fontan. But not Tyson. Tyson is one of those kids who is just not handling the Fontan circuit well. His lungs are not getting the right amount of blood, causing resistance in his lungs, which is why it was not safe to close the fenestration. Most of the concern is with his left lung because of the pulmonary vein stenosis, but it looks as though the right lung is also not doing what it's supposed to do.
Dr. VanArsdell (surgeon) does not feel that another attempt at repairing the pulmonary veins (Coles procedure) will be successful. He would like to see Tyson in the cath lab again in the next 2-3 months to try to do a test occlusion before the fenestration closure. Again, let me explain. Before doing a fenestration closure, which is usually permanent, the cardiologist will perform a test occlusion (occlusion means closure.) Through the cath lab (heart catheterization) they will temporarily balloon the fenestration closed while the patient is under the general anesthetic, and keep it closed long enough to assess the heart and lung pressures, see how the pressures change, see how the patient's body handles it. Then, after they've decided that the test occlusion was successful, they will go ahead with placing the metal discs in the fenestration to close it permanently. During Tyson's cath 2 weeks ago, they didn't even try a test occlusion because they thought the pressures were just too high, there was no point in even trying. However, Dr Van Arsdell feels that they should at least attempt a test occlusion to see if they can go ahead with a fenestration closure. This would make his sats come up, and hopefully keep him healthy for a little while. With Tyson's O2 saturations hovering in the low 80s, there is not a lot room for him to get sick and he will most likely need hospitalization if his sats dip much lower.
Even so, this second attempt at a fenestration closure would still be a short-term solution. The fenestration clsure (IF successful) will not repair the pulmonary vein problem. The problem with Tyson's pulmonary veins is serious and will affect Tyson's long-term life.
The surgical team then discussed putting Tyson on the list to be transplanted and would consider two options when doing so.
Option 1: Transplant just his heart, and only connect it to his right lung, leaving the left lung completely out of the picture so the pulmonary vein stenosis problem becomes obsolete.
Option 2: Transplant both a heart and a left lung.
For now, clinically speaking Tyson is doing well. He is not sick enough to transplant yet. If during his cath in the next 2-3 months, the veins look worse, we would need to look at transplant as an option sooner rather than later. No one knows how long this long-term solution will take to become a reality. But it looks like he's definitely heading down that road.
Brian and I very much get the impression that the team looks at transplant as a last resort only. Obviously, a transplant is a very serious operation with numerous risks, way more risks than any of the surgeries Tyson has ever had. But, before opting for transplant, Dr. V wants to at least rule out the fenestration closure. If indeed they are able to close it, it still only buys Tyson time before he inevitably will need a transplant.
For a long time, Brian and I were of the understanding that a 3 year old could take an adult heart. But Dr M didn't seem to agree with that. I'm sure I've heard that from a cardiac critical care nurse way back when Tyson was born...but either way, Tyson's heart would need to come from another child his age. It's scary to think that some day I might be tempted to pray for another child to lose his life so that my child can have another chance at life. *tears* Ok, I'm getting ahead of myself. He's not there YET.
In the meantime, Tyson sees the respirologist at Sick Kids this coming Wednesday. They will assess his lung function and come up with a plan to protect him from his reactive airway syndrome, or asthma, or whatever it is he has going on with his lungs. We are to mention to them what the cardiologist said about "aggressive lung therapy" for Tyson. Hopefully they will come up with a plan to keep Tyson's airways nice and open so he can have a healthier winter/spring this coming year. We want Tyson to stay healthy and strong for as long as possible. The sicker he gets, the quicker he'll travel down the transplant road. Dr. M also wants to take another quick peek at Tyson at that time too, since respirology and cardiology are right beside each other on 4A. I said, "Yeah, I was hoping it would be a short day. We'll come by if we have time." To which Dr. M said, "No, I want to see Tyson on Wednesday. I'll be here all day. Come and find me."
That's the update for today. I'm not really sure what prayers to ask for. Today I was praying for wisdom for the surgical team and patience and acceptance for us. I believe the team was given the wisdom to find the best solution, and we have been given the patience and acceptance...or at least for today...but we're still praying for a miracle. Is that wrong? To pray for wide open pulmonary veins even when the doctors have given us little hope? To pray that Tyson lives a long healthy life just the way he is, when the doctors think he will require transplant? "What is impossible with man is possible with God." We have to be realistic of course, but we cannot deny the power of prayer.
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.
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.
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.
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.
Prayers are always appreciated!
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.
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.
The MRI will give a good picture of his pulmonary veins and will be able to tell us how well the sutureless repair worked and how it's holding up, or if the stenosis has reoccurred.
The answer as to whether or not this applies to Tyson is very complex. This study assumes that pulmonary vein stenosis is caused by a build-up of cells lining the veins. In some patients, that is true. However, for patients with complicated heart disease, such as Tyson’s, there are other mechanisms which are more likely.
1. The lung arteries are small and underdeveloped, and hence the reduced flow into the lung and out of the lung means that the pulmonary veins are also small and underdeveloped (not likely to be helped by these meds).
2. The pulmonary veins are stretched by other structures or scarred from surgery (again, not likely to be helped by these meds).
I have had patients with the pulmonary vein “disease” which they are interested in, but I think it unlikely that Tyson has this kind of problem and would be unlikely to benefit from these meds (but would have all of the risks). I think we should wait and see what we find on the cath and MRI first, and then decide. I do know these people from Boston.
Dr. Brian McCrindle.
Anyway, Brian and I are both happy to hear that the cause of Tyson's pvs isn't 100% necessarily from an unwanted build-up of cells. We went down to the records department on Wednesday and received copies of ALL Tyson's reports from echos, ecgs, heart catheterizations, MRIs, chest x-rays, etc. I just really want to understand what exactly we're dealing with here. We learned that both his left pulmonary artery and his left pulmonary veins are about 1/3 of the size of his right artery and veins, and he has only 18% blood flow from this left lung. (This was as of his pre-Fontan cath report.) This is substantial. I know that the cardiologist is concerned about this, as he writes in his clinic reports to our family doctor. We also have copies of all those letters too, so we know exactly what we're having to deal with. Now we just have to wait and see what is going to be done about it.
Prayer Request: Please pray that Tyson's pulmonary veins are ok, that the surgery done to repair them was sufficient for him, that the function of his left lung will improve, and that there is no further re-stenosis in the veins. Please also pray for patience for Brian and me as we await his upcoming cath date. Here we go again :(