Some New Concerns

Hi all, Tyson had his annual cardiologist appointment at the end of November and there are some new concerns.  It’s taken us a bit to write the update because we had to process and understand the information before we could accurately pass it along to others. 

Tyson’s being monitored for 2 complications that can happen after the Fontan surgery: PLE and FALD. The cardiologist is initially more concerned about PLE, but the two issues are related to one another so it could actually be both. 

FALD (Fontan Associated Liver Disease) happens to many (most) Fontan patients due to hepatic congestion (increased blood flow to the liver.) Because the Fontan circulation changes the direction of blood flow of the heart and to other organs, the new blood flow increases hepatic congestion and it can cause the liver to stiffen, not function as well, and eventually leads to liver cirrhosis. Because FALD happens in almost all Fontan patients at some point in their life, usually as adults, SickKids monitors the liver enzymes regularly. Liver cirrhosis isn’t something that occurs overnight, and is generally a process that can take many years.  The only cure for FALD is a heart transplant.  If they transplanted the liver, the problems would just happen again, because it is directly related to his Fontan circulation. 

PLE (Protein Losing Enteropathy) is when the body loses proteins into the GI tract.  PLE is a common post-Fontan complication but it's more common in kids who have higher venous pressures (which Tyson has because of pulmonary vein stenosis - narrowing of his pulmonary veins.) This is of more immediate concern to us right now because it can happen quickly and causes extreme abdominal swelling and pain as well as malnutrition.  Again, because it's directly related to his Fontan circulation, the only cure is a heart transplant. 

When diagnosing FALD, they look at 3 different liver enzymes and one of Tyson’s levels (GGT) is outrageously high but the other 2 are still within normal range. His GGT has been extremely high for the past 2 or 3 years but now that he also has decreased proteins, he will need an MRI of the liver within the next year to see how it’s functioning.  If the MRI finds decreased liver function, the cardiac team will refer Tyson to a liver specialist for closer monitoring. He will also need stool testing to see how much protein is being lost.

Since both PLE and FALD are due to the current anatomy and circulation of his heart, there is no cure for either of these issues besides eventual heart transplant. 

Right now he doesn’t have any alarming symptoms of liver failure (fluid retention, discoloration, abdominal pain and swelling,) but he DOES experience fatigue, nausea, vomiting (which I assumed was heart related because it usually happens after he has over-exerted himself.)  We talked to his cardiologist and asked him if it's ok that Tyson often feels nauseous after physical exertion (which is a relatively new symptom) and his cardiologist stated that this just might be part of his new normal and something he will have to get used to. 

So the poor guy's got quite a lot going on with his little body: half a heart, weak lungs, stress on the liver, stomach issues, and a cyst on his kidney that he's still being monitored for yearly. 

When Tyson goes for the MRI on the liver, they will also MRI the heart at the same time because during his last ECHO and ECG he had sinus bradycardia, which is a low heart rate.  They also like to keep an eye on his narrow pulmonary veins and the mitral valve regurg (leaky valve) at the same time.

I can't believe how tall he looks in this picture!  Every year he fills the bed a little more.

Signing the wall of courage after bloodwork

We’ve also been informed that Tyson will be getting a new cardiologist as of January 2020.  His new cardiologist will be Dr. Emily Jean St-Michele who specializes in post-Fontan heart failure and transplants.  Dr McCrindle is scaling back his work load and because Tyson will eventually need to see another cardiologist later on due to his post-Fontan complications, Dr McCrindle felt this is an inevitable switch.  This is so hard for us because we have come to know, love, and trust Dr. McCrindle.  It is also hard for us because…well… she specializes in heart failure and transplants and we weren’t really ready to have to think about this quite yet.  We’ve been in the honeymoon phase for a while now with Tyson’s health and energy being good and not being hospitalized in almost five years. (Praise God for that!) 

If you have questions for us about this, we don’t mind discussing but we would appreciate if it doesn’t get brought up in front of Tyson.  We haven't told him everything we've written here because he wouldn't understand it all and he already struggles with anxiety.  He is aware that there are concerns with his liver that are being watched and that is all we've told him.  We don't want to give him too much information because it's likely to cause even more anxiety and magnify the stomach pain. (We haven't even told him that he's being watched for PLE and that it causes stomach pain.  He's experiencing it already, so no need to give him reason for even more stomach pain!)  

On a more positive note, Tyson got to present the SickKids Foundation with a cheque in the amount of $3553.00 for our Get Fit 4 SickKids fundraiser earlier in November!  The money is earmarked to Cardiac Kids, a charity under the SickKids foundation.  It really is a special feeling to be able to give back to an organization that has done so much for us and it definitely won't be our last fundraiser! 

We will keep you posted about MRI dates or anything else they may have planned for him in the near future. Prayers are always appreciated ❤