Reassuring News!

I got a very reassuring phone call today from Lauren, Ty's cardiac nurse. (side note: she used to work on 4D when he was a baby. She was awesome back then and she's even more awesome now as his cardiac clinic Nurse Practitioner.) Lauren reviewed all the details of his heart and liver MRI and said that his heart function is similar to his last MRI in 2021, with no major changes or new concerns. His liver did show some increase in stiffness, but she assured me that it's still an ok range for a Fontan. This is the news we wanted to hear because given the way things were worded on his MRI report, it looked like he was heading into Fontan failure or liver failure. 

MRI report said that Tyson's liver stiffness was in the upper limits of normal at 6.2 kPA, with most adult Fontans ranging from 4.3 - 5.5 kPA, with liver stiffness >4.5 kPA in one study correlating to Fontan failure. So you can see how we would look at his number of 6.2 kPA and start to worry a little! We got the MRI results in his MyChart medical app and although the numbers were immediately concerning, we decided we'd just wait for a call from the cardiologist before we started to worry. BUT then he got sick and then he presented with (still unexplained) blood in his vomit. So I was a bit of a mess for the past few days since his incident, worried he was in Fontan failure or liver failure. We know that many Fontan livers can eventually fail because The Fontan circulation creates a lot of congestion in the liver.  When that happens, they talk about heart transplant because if they give a new liver, congestion will just build up again eventually due to the Fontan blood flow.

Lauren said that the majority of the Fontans she sees have numbers in the 6's but that can also change from year to year depending on what's going on in their life at that time. However, out of an abundance of caution, she wants a liver ultrasound done. Because his spleen was also enlarged and his platelets are low (they have actually been low for several years) but it could have also been early signs of infection. So just to be sure she wants him to have a liver ultrasound when we go for clinic in October to see if they need to refer to hematology (liver) clinic for advice.

Tyson will also be going for a cardiac catheterization (angiogram/heart cath) before leaving SickKids as part of their new proactive care plan for all Fontans graduating SickKids. They just decided on this new plan within the last 6 weeks and Tyson will be one of the first patients to have a cath as part of this new plan. The reason they're now implementing this is because they're finding that 50% of patients need a cath shortly after being transferred to Toronto General anyway. And as adults, it is done with no general anesthetic (they're completely awake.) So they want to help guide SickKids patients through their very first awake cath with teaching and explanation from a child life specialist so that they're not nervous and understand what's happening for down the road when they need a cath at an adult hospital.  They'll still use numbing creams or calming meds if needed but he will be awake for the whole procedure. They can also dilate vessels or identify any problems and fix them prior to transitioning to adult care. 

So all in all, Tyson is still within the normal limits they'd expect to see in a Fontan and there's no need to worry that what happened this past weekend is a result of a worsening heart or liver condition. Lauren thinks it's more related to whatever is happening in his lungs and that maybe he burst a vessel while he was puking. He'll be going for a chest CT and pulmonary function test at SickKids so the respirology team will try get to the bottom of it. 

I noticed in MyChart that he's already booked for his chest CT scan and his liver ultrasound in October when we go for his other cardiac tests. Sometimes we complain about our healthcare system...but we can also attest to the fact that things can also happen quite quickly when they need to. 

Brian and I are both very reassured with this news and are feeling a lot less nervous about Tyson having a reoccurrence of this past weekend. It feels like I can breathe again, I think I was holding my breath the whole time waited for that phone call! 

One other quick thing to note is that Tyson is also being referred to the orthopedics department because it's recently been discovered that he has pretty significant scoliosis, a curvature in his spine. His curve is angles 24 degrees to the right and then 22 degrees back to the left. He's been seeing a chiropractor for about a year, as soon as we noticed him walking crooked, and he is pretty sure it's a result of having his sternum dissected three times and fused back together with sternal wires. It likely wasn't noticeable until he started growing. We saw our family doctor today for follow-up appointment and he put in the referral for Tyson to see the orthopedics team at SickKids. Even though Ty is technically graduating from SickKids in just over a year, his family doc feels more comfortable having him go see the pediatric experts first, before sending him to an adult orthopedic team. It just means more appointments in his final year at SickKids, but that's ok.

One final note, if you want to receive notifications when this blog is updated, you can click the 'follow' button. I try to keep everyone up to date as much as possible and this blog really helps with that! Thanks again for following along on Ty's journey. 

Home Again

Praise God, Tyson was able to come home this afternoon. There are still some unanswered questions but we are trusting God's timing with those answers. All of the various teams at SickKids were confident that he's doing well and can go home. They've also done their due diligence in trying to get to the bottom of what brought on the hematemesis in the first place. When blood appears like that, it can be a sign of all kinds of other serious issues like internal bleeding, stomach ulcers, liver disease and the list goes on.  Tyson hasn't had any pain in the stomach or other organs, the vomiting stopped soon after a dose of IV gravol, and his O2 saturations improved quickly with oxygen therapy. They also ran a ton of labs to look for signs of other underlying concerns and came up with nothing. 

Tyson had heart and liver MRIs at SickKids last week, and there is some worsening in liver stiffness, which is how they measure the degree of his Fontan Associated Liver Disease. A lot of you have been asking how his liver fits into the picture. When the surgeons replumbed his heart during his last open heart surgery in 2010, his new Fontan circulation (though life-saving!) often creates a challenge for the liver. The blood flow basically bypasses the right ventricle which causes increased pressure in the central veins, especially those draining into the liver.  Chronic venous congestion, reduced blood flow, and the body's response to the new Fontan circulation can lead to a range of liver problems, from mild fibrosis to potentially serious complications like portal hypertension and liver cancer. This is why it was so important that someone review the report from his liver MRI before sending Tyson home. We wanted to make sure that the hematemesis wasn't a symptom of a declining liver. His cardiologist was not available yesterday to meet with us in person, but she was consulted and she was happy with the course of action taken by the other doctors.  I have asked for her to call me next week sometime so we can go over whether or not the MRI report is something to sound alarm bells over or if we're still playing the "watch and wait" game. 

Tyson is doing really well and was very ready to go home. We are going to be pretty protective of him over the next few weeks while he makes a full recovery. He's on antibiotics in case this was a result of a bacteria, as his bloodwork showed an increase in neutrophils which means bacterial infection somewhere in his body. And we are going to really focus on nutrition, hydration, and rebuilding the gut microbiome over the next few months. We really don't want a replay of this to happen again, and especially not the week before his brother's wedding.

That's the update for today. Please continue to pray that Tyson can make a full recovery and that his heart and liver can continue to function well.  

Admitted to SickKids

Ty was admitted to SickKids yesterday. It’s very hard to make a long story short without including all the details because he has a lot going on right now, but basically after a period of hematemesis and extremely low oxygen levels at home yesterday morning, I had to call 911. The ambulance brought him to Headwaters where they did bloodwork, a chest xray, put him on IV fluids and antibiotics, and then transferred him to SickKids. Once at SickKids, he had to go through emerg to decide which department they were admitting him to. He ended up on 7D, the general pediatrics ward (not the cardiac ward as much as I tried to advocate for.) We didn’t get to 7D until 10:00pm so it was an extremely long day. By evening he was already looking much better so it was clear that the IV fluids and antibiotics were helping him. He didn’t have an amazing night’s sleep but the first night is always rough.

This morning when the pediatric team did their rounds, I questioned their diagnosis of pneumonia because Tyson hasn’t been coughing any worse or more than what he has been for the past several months. And the vomiting wasn’t brought on by a cough at all, he literally woke up feeling nauseous and that’s how it started. The staff paeds doctor said that the xray showed an 'aspiration pneumonia,' which is different from a viral/bacterial pneumonia, so it wouldn’t necessarily produce a cough the same way as his usual pneumonia.They think that when he was puking yesterday, he likely aspirated into his lungs and that's what caused his O2 sats to tank so badly. Bloodwork also found increased white blood cells, particularly neutrophils, which indicates some sort of bacterial infection. So given his respiratory history, they’re treating him with IV antibiotics and oxygen. 

Today he’s looking very well! He’s in good spirits and wants to go home. He honestly looks like he could just put his shoes on and go home right now. But they ran a bunch more tests today and asked about a thousand questions about what happened yesterday, and have decided to keep him for another night for observation. Given the extensive MRI findings with increased liver congestion, and the unexplained hematemesis, they really want to make sure they’re not missing anything. (I’ll save the liver MRI findings for another time.) Right now nobody can explain the hematemesis so they want to get to the bottom of it before sending him home. And we want this too, because the last thing we want is to go home prematurely only to have to come back next week with the same thing. We have a wedding to celebrate next week so we want to be sure he’s 100% ready to go home and can stay home!

We’ve seen about 20 fellows and resident doctors today from multiple teams. The paeds team, infectious disease team, cardiology team and respirology team all came by a few times and are digging into what could have been the cause of the hematemesis.  

With respect to the chronic cough he’s had for the past several months, the respirology team wants to wiggle their way back into his life. They were a big part of his care from 2011-2016 when he had asthma and recurring pneumonia. They ordered a sputum culture so they can evaluate if there’s a viral, bacterial, or fungal aspect to this cough. (Plastic bronchitis, a side effect of the Fontan, is always a concern in the back of my mind, so hopefully it’s not that!) And later as outpatient, he'll be going for a chest CT scan and a pulmonary function test. The respirologist says those can wait till after the wedding 🙃

So we are here one more night for sure, with the plan to go home tomorrow as long as everything goes well. There are still some unanswered questions, but our cardiologist is going to call next week to explain the findings from his liver MRI and heart MRI that Ty had done last week. And we will follow-up with the respirology team to see if we can do something about this chronic cough. 

Thank you for all your prayers, they are greatly appreciated. We will update you about the MRI findings another time once our cardiologist has a chance to explain everything to us.