A Whole New World: the World of Neurology

It has been quite an eventful summer to say the least. So much has happened in just a few months but I will try to keep this update as short as I possibly can. (You know how hard that is for me, right?) 

In the spring Tyson started experiencing absence seizures. These aren't the convulsing type of seizures but they're similar to focal awareness seizures, where he stops what he's saying and doing, has a blank stare, and is unresponsive to sound or touch. It looks like he's completely ignoring us. When it's over, sometimes he remembers what's happened or what we've said to him, sometimes he doesn't. 

His family doctor suspended his driver's license, ordered a brain MRI, and referred him to a neurologist. We got an appointment for a brain MRI at Orangeville Headwaters for June 29 but couldn't get in to see a neurologist until August 17 at Brampton Civic. 

The first week of July,  Tyson had a cardiac catheterization procedure at SickKids for some 'routine stents.' Unfortunately we learned that it was too risky to proceed with the stents for various reasons, so he's looking at open heart surgery sometime in the near future.  I already blogged about all of those details here. 

The same week that we learned he would need open heart surgery, we got a phone call from his family doctor about the brain MRI results. The MRI revealed a very large cyst in the left frontal lobe of Tyson's brain. The cyst is 8 cm large (8cm X 6cm X 6cm.) so his referral to a neurologist would be advanced straight to a neurosurgeon. 

This was a lot for us all to process...not understanding much about brain cysts at all...could it be cancerous...can they remove or drain the cyst?...will Tyson need to undergo brain surgery...all the while we're still processing that he's going to need open heart surgery... and Tyson is not yet back to work because he's still recovering from his heart procedure...so he's still having to take it easy...

Lots of stress and uncertainty. 

Last week Tyson saw a neurosurgeon at SickKids. We learned that the cyst is not cancerous, and is an arachnoid cyst that was most likely caused by an ischemic event during one of his heart surgeries as a baby. Meaning: he likely had a "silent" mini stroke during one of his surgeries, some of the brain tissue died, and in its place a cyst formed. Because the cyst has been there so long, Tyson's brain has remodeled around it and the surgeon said that surgery is not the recommended treatment plan for a cyst of this size because it would create more problems than it would solve. There are too many risks associated with removing or draining it. So the current treatment plan is medication with the goal of seizure management. Rather than experimenting with different meds (which have side effects,) Tyson will first need to see a neurologist who will order an overnight EEG to record his brain activity while he sleeps. This will help them figure out exactly where the mis-firing in the brain is happening and prescribe the right med for him.

Of course we are all relieved that Tyson won't need to undergo brain surgery at this time, but the thought of the cyst just hanging out in his brain is kinda eery and still leaves us with a level of uncertainty and unanswered questions. These will have to wait until we can talk to the neurologist assigned to him.

In the last five days, we have taken Tyson to the ER twice for different kinds of, potentially progressing, seizures.  Last week in the middle of the night he was having very strange symptoms, so we drove him straight to SickKids quickly.  After testing and making sure he was stable (and losing a whole night of sleep) they allowed him to go home. Then yesterday morning when he got up for work, he told us he was pretty sure he had a convulsing seizure, so we brought him to Headwaters. Based on the work-up and tests that were done, it indeed looks like Tyson has experienced a tonic clonic seizure (the convulsing kind.) So right now his seizures are getting more frequent and more serious. It's imperative that he has an EEG as soon as possible. 

Unfortunately SickKids neurology is extremely backed up and they won't be able to prioritize Tyson for an EEG because he's almost 18 and is soon phasing out of SickKids. But thankfully we kept that appointment with an adult neurologist for August 17 at Brampton Civic hospital that was booked way back in May. They will be able to order him an EEG a lot sooner than SickKids can. 

So we hope to learn a bit more on Monday about what the plan is to manage these seizures. Until then, we keep taking it one day at a time. And some days it's one moment at a time. We keep putting one foot in front of the other. And somewhere between one step and the next, God keeps meeting us along the way. 

Please pray that He continues to give our family strength for today and hope for whatever tomorrow holds.

An Unexpected Turn

This week has been a wild rollercoaster ride. We went from expecting a diagnostic cardiac catheterization... to being told Tyson would need one, then possibly two, then three stents... only to learn after the procedure that stenting isn't the safest option after all.  Now we're shifting gears and awaiting discussions with the surgical team, as open-heart surgery may be the next step for him.

That was the short version. If you like more details of how the week went down, read below.

Tyson had SickKids appointments on Thursday and Friday. Thursday was a pre-cath appointment with an MRI, chest x-rays, bloodwork, etc and Friday was the scheduled cardiac catheterization (moving forward I will call this a 'cath.')  You might remember me talking about this planned cath and it's purpose on a past blog post here.  These were scheduled a few months back because his care will be transferred to Toronto General in the fall, so it's all part of his "exit plan." 

The original plan was for Tyson to have this cath awake so that he would get used to having them in the adult world where they don't use general anesthetic. However, we learned at his pre-cath appt on Thursday that he would be put to sleep with general anesthetic after all, because the plan was to place a stent, potentially even two. One in the Glen/Fontan pathway to widen it (because he's outgrown it since they did these surgeries as a baby) and another stent in the left pulmonary artery (LPA) which has been narrow for years. We were told it's not a matter of "if" he needs a stent, but "that" he will need it. We were caught a little off-guard about that, but we were all comforted knowing that his heart would get a good tune-up before going to an adult hospital when he turns 18. 

Right before the cath we met with the cardiac interventionist doing the procedure, Dr Benson who has done at least two of Ty's caths as a baby.)  He told us that they would also be stenting the left pulmonary veins which are narrow as well. He says there's no sense stenting the LPA which carries blood into his left lung, without also stenting the pulmonary veins to bring the blood out of the lungs and back into the heart. So he said we're looking at three stents for sure. 

After the procedure we learned that the repairs they wanted to make to the pulmonary artery are too risky to do in the cath lab, which means he will need a more invasive surgery down the road. They need to discuss with the cardiac surgical team, but unfortunately it's likely he will need this repaired by open heart surgery.

The GOOD news is that it doesn't appear to be an IMMEDIATE concern and surgery could be a few years away yet. So it wouldn't be done at SickKids, it would be done at Toronto General. If they don't repair it at some point, his heart will fail in 10-15 years. So they'll keep monitoring it and decide when the timing is right. Dr Benson also does caths at Toronto General so that's good news too.

We were really hoping Tyson could get this little 'tune-up' in the cath lab yesterday and enjoy many years as an adult without needing to talk about surgery, but we also know Ty has defied the odds before so who knows how long it'll be before this becomes a concern. We've kinda always been on a "watch and wait" plan, so we will continue to trust God's timing. And as always, we entrust Tyson into God's hands.

We arrived home at around 7:30 last night. Overnight he slept in his lazy boy chair so we could be close by because the catheterization site was bleeding for a bit after we got home, just from the activity of walking from the car to the living room. It takes him a bit to clot on a normal day, but yesterday his body was pumped full of heparin which is a very strong blood thinner. It's going to take a day or so for his body to flush everything all out. 

He has to rest this coming week so he won't be able to go to work, and he can't have a bath or go swimming for 5 days until the catheterization site is healed.  We leave for camping at the end of the week, so by then he'll be able to resume activity and go swimming again. 

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For those of you who understand the heart anatomy and would like more detail about why the procedure didn't go as planned, Dr Benson gave these reasons for why they did not place any stents: 

They could not stent the Fontan conduit without covering the fenestration (the hole, or man-made pop-off valve, that was created during his Fontan surgery in 2010.) The fenestration is still needed to help relieve pressures in his heart, so it can't be covered. There's some calcification in the Fontan area, which is normal after so many years, but his Fontan circulation still looks OK and he's not concerned about it being an immediate concern. He said it actually looks better in the cath lab than it did on the MRI images. A lot of Fontan conduits at his age are between 12-18 mm so they like to widen it as they reach adulthood. Tyson's conduit is measuring 20mm and they were hoping to widen it to 22mm to give him the best possible outcomes, but 20mm is still better than most Fontans. Dr Benson is very happy with the images they got today, and we are so thankful they were able to get a full diagnostics and lots of pictures so that his new cardiac team at Toronto General will know exactly what they're getting. 

Now let's talk about the left pulmonary artery (LPA) which has been narrow for years and years (like, since he was two years old.) Meaning the blood flow to his left lung is substantially less than the right lung. It was 82% (right) and 18% (left) for quite a long time (several years.) Then last year's MRI showed it was 88% to 12%, and his MRI from Thursday shows it's at 91% to 9%. So it's getting worse, which is why they wanted to stent it to widen it and improve blood flow. 

However, Tyson has unique anatomy and his descending aorta, his LPA, and his left bronchus are all quite close together. Because the LPA is being pushed down by the aorta above, they cannot stent the LPA without the stent getting too close to the bronchus below (the bronchus is the airways into the left lung.) Because the aorta, LPA, and bronchus are all really close together, if they stent the LPA, it won't leave enough room for the bronchus. They cannot have a metal stent touching the bronchus because it will lead to infection, clots, etc. So it was too risky to try to repair this in the cath lab and it's likely he will need this repaired by open heart surgery down the road.  

Dr Benson says if they don't repair it at some point in the future, in 10-15 years he's going to go from a "good Fontan" to a "failing Fontan." He doesn't think it needs to be repaired immediately but the key is not to leave it too long. Dr Benson is also a cath doctor at Toronto General, so that's very reassuring.