Deep in the heart of Texas

Kaston & I will be heading down to Houston on Sunday for this next round of appointments and testing over the next 10-12 days at Texas Children’s Hospital.  Bob will be flying in Tuesday night for his heart cath Wed since they will be putting him under again, and of course heart caths are nothing to sneeze at.

Our tentative schedule is as follows:

Sun Feb 23 – travel

Mon Feb 24 – labs, EKG, Echo, exercise stress test, 24 hr Holter monitor

Tues Feb 25 – morning Ultrasound w/liver Elastography am & then afternoon MRI w/liver Elastography

Wed Feb 26 – cardiac catheterization

Thurs Feb 27 – Psychology consult

Fri Feb 28 – cardiology consult

Mon March 2 – 4 hour psych evaluation

Tues March 3 – travel home (hopefully)

(plus 3 more appointments that have not yet been confirmed)

 

It has been a long 2 weeks getting pre-authorizations, setting up a case worker with insurance to help manage everything, phone calls every day from different departments, arranging transportation, lodging, etc.  Lots and lots of logistics to take care of.  But thank goodness for his nurse coordinator Corey.  She’s a great advocate for Kaston and getting questions answered and taking care of all of the moving pieces in all the different departments.  She definitely has worked her tail off for our boy!

There has been some discussion of bringing 2 more doctors onto his team.  We are working to secure an appointment with one of cardiologists in the Adult Congenital Heart Program at TCH.  We also are trying to secure an appointment with the Medical Director of Liver Transplant at Baylor St. Luke’s Medical Center, Dr. Stribling.  St. Luke’s is next door to the TCH Women’s Pavilion and part of Baylor Medical (which also employees Kaston’s docs at TCH).  The TCH ACHD program works hand in hand with the Liver program at St. Luke’s.

So what does this mean?  Well, Kaston seems to have some crazy hemodynamics and side effects that they don’t typically see in patients this young.  We have known this for awhile.  They still can’t figure out why he is as far progressed in Fontan Association Liver Disease as he is or why he is having these sudden drops in blood pressure.  The Adult CHD doctors and the Liver Transplant Director are used to seeing this in much older Fontan patients, so they are thinking that we need to use their expertise in FALD since he is the size of an adult and almost legally an adult, and will eventually end up with them in the near future, although he is still considered pediatric (which of course makes insurance approvals a little more challenging).  That’s why they have not considered this an option yet.  But we are gonna try our best to convince insurance it’s necessary. So keep your fingers (and toes) crossed!

We will take all of the data we collect at the beginning of the week to these 2 new doctors, along with all of the data from the last 9 months to see if we can draw some better conclusions, see if the Sildenafil is working, see if there’s any improvements anywhere and see what our potential plan of care will be.

Does that mean transplant?  That’s the million dollar question.  Until we get all of this raw data put together we just won’t know.  Do I think he’s ready for transplant? My gut feeling is no.  I think we have a ways to go before he would even be considered to be listed and honestly would be shocked if they came back and said let’s list him.  Do I think they may want us to stay in Houston until they can pinpoint why he is having these crazy drops in blood pressure?  Possibly?  We are not currently planning on staying an extended time beyond the 10-12 days.  But if they find something conclusive that’s concerning and need us to stay, then we will just figure it out.

So, needless to say, we have a very long few days coming up.  Please keep the boy in your prayers.  It’s alot for anyone to take on, much less a 17 year old kid.  These appointments are so hard on him and he tends to be pretty emotional, and a little angry towards the end of it all.  We’ll be staying at the Ronald McDonald House again and I have some fun things planned to hopefully keep his mind off of everything.

Ob la di, ob-la-da, life goes on, bra

 

 

 

 

 

 

Upcoming Appointments update!

 

Kaston & his new pick-up!

As we enter into one of my favorite seasons of the year, I wanted to give an update on Kaston’s upcoming appointments.   Some of it is repeat info…

We leave next week for Houston and are driving the 600+ miles this time (anticipating an 11 hour trip with stops)…definitely not looking forward to this, but it’s necessary for this particular trip. We’ve been on the list to stay at the Ronald McDonald House since September, so they should have a room available for us.  But we won’t know until the night before we leave.

Over the course of the 11 days we’ll be gone, Kaston will be seeing Dr. Dreyer in Cardiology, Dr. Hertel in Hepatology, Dr. Cutitta in Psychology and Dr. Kim in Electrophysiology. It’s definitely been challenging keeping everything straight now that he has 4 – well actually 5 doctors.  He only sees his heart cath cardiologist when he has a cath.  Thank goodness for Dr. Dreyer’s Nurse Coordinator, Corey!  He’ll be having the normal echo and EKG and lab work and I don’t anticipate him having any further testing beyond that. Which is a nice break for him! We spread everything out this time instead of stacking everything in because we have found those long days of back to back appointments are daunting and exhausting for him.

These appointments are basically them just checking his liver status and if they are seeing any improvement from him being on Sildenafil and making sure cardiac and liver status is still stable. They want him seeing his Psychologist each visit as well, just to keep him in check. It’s definitely alot for a 16 year old kid to manage and we are always open for getting him any additional help that might help him manage his sometimes difficult life. The MAIN reason for these appointments is to see if he is a candidate for a pacemaker.

After his last 24 hr Holter monitor, it indicated that his minimum BPM was 36 and his highest was 105, with an average of 53. This is dangerously low for someone his age. The good thing is that he’s in normal rhythm. However, his rates just keep getting lower and lower. So it was something that Dr. Dreyer wanted to explore a little further, so that’s why he referred us to an Electrophysiologist. They basically are cardiologists that deal with the electrical side of things in the heart.

We want his heart to be at an optimal level, and having a Congenital Heart Defect means that we will do what we need to do until what we did stops working. The surgeries and procedures he’s had up to this point are only Palliative, meaning a temporary fix to a permanent problem. I am pretty anxious about that particular appointment, because a part of me wants him to have a pacemaker with the hope that it will make him feel better and improve his heart function, and possibly his liver function. But of course, I definitely don’t want him to have to have another major surgery. People like him with his cardiac anatomy getting a pacemaker is a major undertaking. So we are just in the hurry up and wait mode in life.

My gut feeling… I do not believe that they will decide to do the pacemaker.

We are already working on his January appointments. Talked to the cath lab today. For now, January looks like he will be having a cath, another liver biopsy, an MRI w/Elastography, a sonogram w/Elastography, cardio, liver & psych visits. And then if they determine he needs a PM, then there will be additional testing on top of that. We will be in Houston for an undetermined amount of time, but we are thinking around a month or so. Just because we will be so far away from home. We figure a week for testing & pre-op, a week for cath, biopsy and PM, and then 2 weeks of follow-up appointments. If a PM is not warranted, probably another week to 10 day stay in Houston and are planning on flying.  Depending on how long we are down there will depend on when Bob is with us.  There’s no way he can stay the entire time, but will definitely be there for any surgeries or major procedures.

We also have been intentional on scheduling these appointments around some other activities that we wanted him to be involved in! Hunt with Heart will be having their annual Christmas party for the families and Sporting Clays shoot while we are there. And he should be getting his Corsican Ram mount presented to him at the Christmas party. He actually was supposed to go on another hunt last week-end with them, but since Bob’s accident has left him somewhat immobile, they had to cancel it. But hopefully he will have another opportunity to go on another hunt at some point. On Saturday they have their big charity fundraising event and Kaston will be shooting with some of the sponsor’s teams. We were able to attend both events last year and had just the greatest time and are so appreciative of all they do for our kiddos.

Also while I am there, I am hoping to do some Mended Little Hearts work with our Houston Chapter. With my new role being on the Mended Hearts Board of Directors, it has definitely come with lots of conference calls, emails and webinars, which I have enjoyed tremendously and learned tons. But it definitely gives me a sense of responsibility to help any groups in whatever area I may be in. So I am looking forward to meeting the Houston leaders and staff that work with MLH in the hospital and see where I can be of assistance in my position.

We will also be there on Veteran’s Day, and you all know how big of a Patriot Kaston is! There is a Veteran’s Day benefit concert that we are planning on taking him to with Joe Walsh, ZZ Top, Sheryl Crow, the Doobie Brothers, and Brad Paisley. The proceeds benefit several military charities and should be a really great show. The tickets are only $25 so it will be a great economical evening out! And then there is a Veteran’s Day ceremony and parade in downtown Houston that we are planning on going to. We have a couple of other things planned…we still have to do school while we’re gone! So we’ll take in a couple of museums and do some educational exploring.

Another project we will be doing while we are there is that we will be serving breakfast each morning at the Ronald McDonald House, on behalf of The Children’s Heart Lane! Dinners are always covered by other volunteer organizations, but there is never breakfast available to the families. They do provide eggs each morning.  So we are going to make up some breakfast burritos & sandwiches that parents can grab n go on their way out, along with some fruit and pastry trays.  We are always looking at ways to give back and this fits inside the mission of what we do at The Children’s Heart Lane.

We will finish up our Houston “tour” by leaving on Friday the 15th and meeting our dearest friends in College Station, where they both graduated from Texas A&M. They are taking us to our very 1st A&M game ever for Kaston’s 17th birthday. Needless to say we are over the moon excited and extremely grateful to our sweet friends for this opportunity. Gig ’em!  And…did I mention Kaston is turning 17!! WHAT IN THE WORLD…HOW DID THAT HAPPEN??

We also wanted to say thank-you so so much to those of you who have made deposits into Kaston’s benefit account. We appreciate your generosity more than you know.  It definitely helps ease one more burden on our plate, especially in this season of our lives we are going through. We have about $500 in there and that will help us so much on this trip! We do have to pay to stay at the RMDH, and then of course we’ll have gas and food expenses to cover, so that will help ease the burden for sure. Kaston’s insurance does not provide any travel allotment at all, so all of it comes out of our pocket.  And whatever is left over will carry over into his January visit.
His benefit account is at Amarillo National Bank and we set up a Paypal account that is attached to that bank info. The email to use is kastonlane@gmail.com.

Thank-you to everyone that asks about him and prays for him regularly, especially the sweet Pastor at our community church that the kids go to youth group at and his congregation.  They pray for him every week in church and in their Bible study classes.  We absolutely love our community and are blessed to live where we do.

Will of course provide updates as we continue this journey over the coming weeks.

the Snyders

The anatomy of a Teen-Aged Fontan

So we FINALLY heard back from the hepatologist from TCH in Houston, Dr. Hertel.  We wanted to update everyone about what this means and what Kaston’s future plan of care is.

Her main goal was to get an overall picture of the liver status.  There is not just 1 test that can show us this, and that’s why he had so many different tests done in May.  So the first puzzle piece is that his liver biopsy showed mild, stage 1 fibrosis.  But as she indicated, this is really not an accurate picture because they take only a portion of the liver to sample.  There can be many different variances throughout the entire liver. The second piece is that the MRE showed that he has an enlarged diseased liver and it’s appearance was “bumpy and abnormal appearing”, which is a common characteristic of a Fontan liver.  His spleen is also enlarged.  The next piece of the puzzle is his blood work.  It’s not ideal, and she wants to keep an eye on his levels periodically, particularly his INR, or blood clotting, level.  The final piece was the ultrasound with Elastography, which measures stiffness.  He currently is showing severe stiffness, much more than what he should be at at this point.  This we knew from his very first Elastography from Dallas.

All of this has given us a good baseline to work from.  She did say that his liver is at a good place – not great, but not terrible – even despite the severe stiffness, and that these results are not indicative that he would need a transplant at this point due to liver status.  So that’s really really great news!!!

So what is her plan of care for him?  Let’s tie in cardio for a minute.

Kaston’s Fontan is failing somewhat.  Meaning his heart is just not functioning the way we really need it to.  His pressures are high due to the 2 areas that have narrowed in his pulmonary artery and his ejection fraction is really low, which is likely the culprit of the advanced stiffness in the liver.  They are putting ALOT of faith in this new medication, Sildenafil (aka Viagra), to help relieve the stress on his heart.  Right now we are just going through the motions and taking baby steps, but at least we know that we have options.

Both the liver doc & cardio are keeping their fingers crossed that the Sildenafil is opening up those arteries.  So far he seems to be responding well to the medication with no obvious or troublesome “side effects”.  The 1st couple of weeks while his body was getting adjusted he had a few headaches, likely as a result in blood pressure changes, a couple of bloody noses and some nausea.  But he seems to be tolerating it well now at the full dose.

Another thought is that we can always go back in and stent open the narrowed artery to help alleviate the higher pressures and hopefully improve the pumping of that single left ventricle.  But they want to give the Sildenafil a chance first.

So what would the criteria be for a heart transplant?  I have no idea.  That is something we will be discussing with Dr. Dreyer.  But do we think that is the path he is on?  Absolutely not.  We at least have options to help improve function, especially now knowing the status of his liver.  So to say we have had a huge burden on our shoulders lifted is an understatement!

What happens next?  We still will be going to Houston every couple of months for the next unknown time frame.  We basically will schedule from appointment to appointment.  We have usually had the “see ya in 6 mos.” for years on end.  Right now they have us scheduled for the first week of September, where he will have another full cardiac work-up and another ultrasound with Elastography and full blood panel to check his liver status.  I don’t think they will do a cath at that point, so it might just be me & Kaston going down.

And then we will be down for a full week in November where I believe he will be having another heart cath procedure.  And more liver testing.  He will have been on the Sildenafil for about 5 months at that point. So they will want to check and see if they see any improvement on reducing pressures.  After the November appointment we will reevaluate again.

Alot of people have asked if there is anything that he do nutritionally or supplementally to help the liver function (like limit red meat or salt).  There is absolutely nothing.  This liver disease is not due to diet or external causes, like Hepatitis, or alcohol or a lifelong diet of crappy food.  It is 100% due to the higher than normal pressures in his heart.  And until we can reduce those pressures, it’s going to continue to damage the liver.  Actually some homeopathic treatments can cause more harm to than good to livers like his, because some of them can alter blood flow and counteract the vasodialation that the Sildenafil is creating.  He can take Tylenol, but not in large amounts and no alcohol.

So if you’re still with me here, thanks so much for following along!  Thank-you to everyone that has been praying for our boy and been so generous to us.  It means the world.  We still have a long journey ahead of us.  And I can’t imagine us having to go to Houston every couple of months, but we will make it happen.  CHD is not a sprint, it’s a lifelong marathon with large mountains.  And we just entered a really long stretch of a gradual uphill incline.

Jamie

 

Alot of people the last couple of months have asked if we have a GoFundMe set up or how they can donate to help.  We decided to open up a benefit fund at Amarillo National Bank to help offset some of our travel expenses.  You can send donations to:

Benefit Fund for Kaston L Snyder

Amarillo National Bank

PO Box 1

Amarillo, TX. 79105

The funds will be strictly be used for airline tickets for Kaston & I to fly to Houston in September, fuel for when we drive down in November, Ronald McDonald House/lodging fees, and meals.

 

 

Day 2 & 3

Well, we have had an interesting couple of days.  So much to share.  It’s hard to know where to even start.

Yesterday started off great.  Did the abdominal ultrasound.  He had been complaining that he was cold all morning.  But it’s a hospital and they’re typically always cold, even in his hoodie.  Met with cardio/transplant nurse Corey, who is btw Amazing.  We have spent HOURS with her these past 2 days and I don’t know how she got all of this done, but wowza…she is a miracle worker!

Did an echo and then decided to go grab a late lunch before we did labs and stress test.  He drank a little gatoraide after he was done with the ultrasound, as he had been NPO since 7:00 am.

At lunch he started feeling really bad and nauseous.  So he really didn’t eat anything.  So we went back and he did the stress test on the treadmill and it was brutal.  He ended up puking, his temp dropped to 95 degrees and his blood pressure tanked.

He turned blue-grey and was shaking uncontrollably.  We got some fluids down him and piled warm blankets on him.  Finally got his BP stabilzed and his temp up after about an hour.  So we chilled in the echo holding area for a couple of hours.  The MRI and labs didn’t happen. There was talk of admitting him, but it is such a delicate balance rehydrating a Fontan person and they can get overloaded super easily.  He rested well at the House and we were in bed early.

We headed back this morning, had labs drawn…I never have seen that many vials come out of his arm ever.  Dr. Dreyer, his new cardiologist visited with us at length.  He has reviewed all of his previous records, and everything they have done since we’ve been here.  He still has more labs to go over when they get in.

His concern is that his left ventricle, his only functioning ventricle, is not pumping efficiently.  It squeezes fine but it’s not releasing properly. Which then is potentially backing up the liver, causing the scarring. Or at least that’s how I understand it.

So there is a definite concern with his cardiac function and the cath will give us a better idea of what’s exactly going on.

Had lunch and actually ran into a heart friend of mine I have “known” for years but never met in person!  She is 31 yrs old with the same condition Kaston has. I have learned a tremendous amount from her about all of this liver business and am so appreciative of her advocacy for adults with CHD as well as these kiddos!

After lunch we met with Dr. Hertel, the Hepatologist – or liver Doc.  We really like her, she is extremely thorough and friendly and outgoing.  She is still waiting on quite a few labs to come in, but she is pretty concerned about a few that are already in.  His INR (blood clotting) is elevated – and it shouldn’t be, even being on the low dose aspirin he’s on.  His platelet count is low, and his spleen is enlarged.  Having an enlarged spleen can cause lack of appetite. Which is of great concern right now too, because he’s lost about 8 lbs in a month.  And his sickness at the beginning of the month might have not truly been a virus, it might have been something to do with this. So his labs are still showing he’s dehydrated, even though he’s drinking and his urine output is good and clear.  So we think it’s starting to compound. He doesn’t want to drink anything because he is so feeling full because of the spleen, which then keeps him in a chronic dehydrated state and is causing him not to want to eat.  Which all could be why his blood pressure is all over the place.  While we were with the liver doc, his BP was 87/58…dangerously low.   But then we checked it an hour later and it was normal.

So IDK.  There’s still so much to find out.  Everyone involved in his care is concerned and today is the first time the word transplant has ever been used.  Of course, nothing will be conclusive until we can get inside of this kids organs and take a look around.  She also is going to check for esophageal varices (not even sure what that is), but basically he will swallow a capsule with a camera inside and then 💩 it out.  😳

And with all of this we have had insurance issues.  But we got all of that straightened out after both docs called the insurance company.  🙄

So the plan is Tuesday, first case cath & liver biopsy and NPO again (nothing by mouth for 8 hours, Wed will be MRI and esophageal varices test. We hope to still be home by Thursday.

Been a long day, and we’re at the airport to pick up Korah for the week-end.  Tomorrow we will go to Beaver Creek Ranch for the annual family Hunt with Heart picnic.  But they will be watching him closely while we are there.

Then depending on how Kaston feels, we may go to Galveston on Sunday to chill.

If you’re still reading allll of this…thanks for hanging in there!  I know it’s alot of info, but it’s easy for me to communicate to everyone that is wanting to know the nitty gritty details.  And thanks to all who have reached out and are praying for him/us.

Day 1

4:00 came early today that resulted in puffy, burning eyes after only 3 hours of sleep, but we were in Houston by 10:00 this morning.  Was able to get a rental car and get checked in to the House just in time for lunch.  And then took a short nap. 

I have to say, being on the other side of the serving line for the first time is quite humbling.  Today we were one of “those” families. 

The facilities are super nice, the kitchen is equipped with 12 cooking stations, we have our own assigned fridge and cabinet totes where we can store our own food.  Of course there’s plenty of shared public space like TV rooms, game rooms, play rooms, outdoor grilling area, playground & patio area.  Our room has 2 queen beds, private bath, drink fridge, recliner, dresser, end table & decent sized open closet, but man do we have the best view!!! One whole wall is nothing but floor to ceiling windows that faces downtown Houston and medical center! We literally lay in bed with an entire panorama of downtown Houston! 

And this evening we had a fantastic surprise! One of our best friends just happen to be in Houston for work today so she took us to a wonderful dinner before she headed back to CO!  Was such a treat to get to visit with her, even if only for a couple of hours. 

Thursday’s agenda:

NPO (nothing by mouth) at 6:30 so Bob’s gonna get up at 6:00 and make breakfast. 

11:15 sonogram Elastography in Radiology

12:00 check in to cardiology

1:00 labs

1:40 EKG

2:00 echocardiogram 

3:00 exercise stress test

5:30 MRE (MRI of the liver) – Kaston’s a little apprehensive about having to lay perfectly still in a tube for an hour.  We may ask to give him something to help him relax and not be so fidgety.  Will be a really full and busy day! Hope to be back to the House by about 8:00.

Friday we will go over all of the testing results with the transplant doctor and the liver doctor.

I just wanted to share too that this week has been a huge week of blessings to our family.  Just getting to stay at the RMDH will save us well over $1000.  We didn’t find out until late yesterday that we would get to stay here.  We also had a precious friend from high school send us a couple of gift cards and a sweet note.  And, our friends rallied everyone in our community together and gave us a basket full of goodies and cards filled with cash & gift cards.  Some people we don’t even know, but they know Kaston and have been praying for him!   Such sweet sweet notes of encouragement and prayer.  

And one of the best surprises was that my dad decided to give his very nice “Zero Turn” mower to Kaston.  Who would have thought a lawnmower would make a 16 yr old boy so happy!  You woulda thought he gave him a dirt bike the way he carried on! Hopefully he can use the mower to provide a little summer income for himself. 

It’s definitely been a week full of gratitude to have such supportive friends, family & neighbors. We have been on the giving end for so many years, it’s a little difficult to be on the receiving end again. But it fills my heart with joy to know that so many care for our boy. 

Kaston is by far the oldest by quite a few years I have seen here at the House.  Lots of littles walking the halls with their bald little heads, some in wheelchairs, some just walking around with no outwardly visible battle, like Kaston.  Lots of stressed out, tired parents cooking dinner, or maybe gathered around a table visiting with one another or spouses having a late night meal on the patio.  

One thing I love about RMDH is that anyone can stay here.  It doesn’t matter how much or how little money you have.  It just matters if you need some sort of resemblance of a home environment while families have to travel far distances away from home to get the best medical care for their kids.  And this is our very first experience on this side of the House.  We never were able to stay when Kaston was little because we could never get on the list.

So many unknown stories of sick kids.  I look at these tired parents and wonder where their child is and what illness brings them here? And how long have they been here?  Sometimes I wonder how did WE ever fit in to this scenario all of these years later? Just seems a little too surreal for me.  

 

TCH is a go!

SO much of this feels like I am in a time warp from eons ago!  Time for another update, as I find myself repeating this information over and over.  So it’s just better to get it out there for everyone, and then feel free to ask questions.

I have been consulting with the wonderfully amazing people at Texas Children’s Hospital in Houston.  Man…the attention we are getting is remarkable.  Either Kaston is something special, or they are just that good!  I’ve been working with 5 different departments to make all of this happen and it has been a full-time job the last couple of weeks!  They are pretty much contacting every day either by phone or email.  Dr. Dreyer’s nurse Corey is uh-mazing to help get all of this coordinated and accomplished!  So, for info purposes and for those that have an interest in who we are seeing and why, here is Kaston’s medical team:

Dr. Dreyer – Medical Director, Heart Failure, Cardiomyopathy, Cardiac Transplantation, and his nurse Corey

Dr. Paula Hertel – Pediatric Hepatologist, and her Liver Center Referral Specialist Denise

Dr. Manish Bansal – Intervention Cardiologist and the Cardiac Cath Lab nurse coordinator – Ericah

David – research study coordinator for the FUEL/FALD study

Elena – social work

WHEW!!!!

So here’s the run down of what’s about to happen:

Wed May 22 we are hoping to fly to Houston through an Angel Flight of some sort or fly commercially if possible.  (Looking for airline miles through different organizations or if anyone has any they want to donate) Just really not wanting to make the 10 hour drive if any way possible. And booking commercially this close to the time is way expensive.  Plus we’re not sure when we will be coming home so it’s hard to book a commercial flight.  I submitted our info to a couple of different organizations today.  Fingers crossed we can find something private.

Kaston is going to be entering into a clinical trial, which will hopefully also help pay for some expenses, including a hotel for a couple of nights.  Other than that, we are working to see if there’s a spot for us at the Ronald McDonald House since we will be there for awhile.

Thursday May 23 he will be meeting with the cardio team for testing…lab work, EKG, Echo, exercise stress test.  This is all primarily to check cardiac function prior to the cath/biopsy.  More about that in a minute.

Friday May 24 will be a super full day.  He will have an ultrasound Elastography.  He had this done in Dallas in March.  They want to do a MRE (Magnetic resonance elastography – *a non-invasive medical imaging technique that measures the mechanical properties (stiffness) of soft tissues by introducing shear waves and imaging their propagation using MRI.  Diseased tissues are often stiffer than the surrounding normal tissue.*  The MRE may or may not happen due to the mesh Amplatzer closure device that is in his right atrium.  They are still consulting with Dallas to determine if that is feasible and if it will be compatible.  You know, because metal in his heart and a super strong magnet 🧲 probably isn’t safe.  But he can go to 3.0T, whatever that means.

Then we will visit with the clinical research team to start the process of the FUEL/FALD clinical trial.  After alllllll of that, we will then be meeting with Dr. Dreyer and Dr. Hertel to come up with a game plan and go over any results that may have come in by that point and to determine the need for the cath & biopsy.

Sat, Sun, Mon we’ll hang in Houston, go to the Hunt with Heart picnic at Beaver Creek Ranch and enjoy some down time.  Korah will be joining us for the week-end as well.  So it will be some much needed family time!

Tuesday the tentative plan is to be in the cath lab with Dr. Bansal by 7:30 to do the cardiac cath as well as the liver biopsy.  It is a transjugular biopsy, meaning they will access the liver through his jugular vein in his neck.  We are preparing for an overnight stay in the hospital for monitoring, but hopefully the procedure goes well and his recovery goes smoothly and we can consider heading home Wednesday or Thursday.

This past week Kaston has been to the doc twice.  He’s been running a fever, has a sore throat, has zero appetite and everything makes him nauseated. 🤢 And he has a nasty cough.  We went in a couple of days ago and they started him on antibiotics but the cath lab doesn’t want him on antibiotics prior to the cath!  So, we are trying to #1 figure out EXACTLY what’s going on and #2 what’s the best and most aggressive way to treat this so that they don’t have to reschedule alllll of this.  Which has been crazy insane to even just make happen for us.  He seems to be doing better today, but has literally been in bed for 5 days now.  It has totally kicked his butt.  Talked to the cath lab today and she said she will consult the interventionalist that’s doing his cath but she felt he should be good by the end of the month but to monitor him closely and report any declining changes. His chest xray was clear and his blood counts seem to be within normal range, of course except his liver stuff being out of whack.

SO, why are they wanting to do all of this testing you may be wondering? Based on the preliminary work-up in Dallas, for some odd reason he is more advanced in Fontan Associated Liver Disease than he should be. Twice as advanced.  We have really struggled with putting him through so much testing, and risky testing at that.   But we also feel that they would not be doing this extensive of testing if they weren’t seeing something that didn’t warrant it.

TCH is the best in the country, if not the world, for pediatric cardiology and extremely knowledgeable in FALD and are the leaders in research and treatment.  So we feel will be at the best possible place and in the best possible hands.

The cardiac cath will help determine what his pressures are and give them a better idea of what’s going on inside.  And there’s always the potential they can tweak a few things while they’re in there if they are able to.

The liver biopsy will go through his jugular down inside of his liver where they will take multiple samples of different areas.  Going inside the liver is a less risky procedure, as the bleeding will be contained inside of the liver (or at least it’s how I understand it).  Whereas if they took a biopsy from outside of the liver, the risk of bleeding out into the abdomen would be a significant problem. Especially him being on aspirin.

This will give us more accurate information on the status of the liver and if it is cirrhotic and just an overall better picture.  It’s not 100% accurate but it’s the closest they can get.  All of this testing will come together to give us a better picture.  None of the tests are conclusive by themselves.

There is definite risks involved in these procedures, especially since he will be put under anesthesia again.  Which is always risk for heart patients. And then there are risks involved anytime you are going inside of organs with foreign objects. So I’d be lying to say we aren’t a little anxious

I anticipate the plan of care following this testing will be to put him on some medication to help with the elevated pressures caused by the Fontan to help slow down the aggressive progression. And they can’t put him on these meds until they do this testing.

As far as possibly being listed for a heart transplant…I don’t think that is going to be on the radar for awhile.   Although I suppose there is always that very slight possibility, that I try really hard not to think about what that would look like for our family.  But I just honestly don’t think he’s that bad off and would be shocked for them to come back and say they want to list him.

Some repeat info…they won’t do just a liver transplant because the funky heart would just damage the new liver.  As far as double organ…it’s extremely rare and only a few have been done.  So the tricky part of it all is not letting the liver get too far gone before the heart needs transplanted.  But to qualify for a heart transplant, your heart has to be really really sick.  Usually by that time, there’s no way for the liver to handle the trauma and stress of a heart transplant.  So it’s finding the delicate balancing act of not letting the liver get too far gone before they can be listed. This is all so new that there really isn’t any guidelines set in place by UNOS to address this problem, but I’ve heard there are a few doctors that are working on getting some of this changed and they can potentially qualify them before the cardiac and liver function deteriorates too much.

The clinical trial, if he meets the criteria, will allow him to possibly be placed on trial medications.  I really have no idea what any of that means or what exactly it entails.

He seems to be handling everything ok, although he is getting frustrated with his body not cooperating with what he wants it to do.  His appetite is pretty much non-existant, which upsets him and he is tired beyond belief.  But he sure puts up a good front and pushes himself to the max, so it appears to most people that he seems fine.  Bob and I definitely see the struggle and the decline.  He’s good at masking things for sure.  He seems to be emotionally prepared for everything he is about to endure and we have been keeping an open line of communication with him, hopefully.

It is alot, I won’t pretend to say that it’s not. We feel like we’re brand new heart parents all over again.  There’s so much to learn, so much to keep track of.  So much information being thrown at us.  But, we are seasoned veterans and just take it day by day.  Our main thing is that we just hate seeing him struggle.  And if there’s anything we can do to help him feel more like himself, then as his parents we owe that to him.  Whatever that may look like, the good, the bad, and the ugly.

 

 

Where do we go from here?

Lots of folks have asked about Kaston over the past couple of weeks, so I wanted to give everyone an overview of where we’re at and what we’ve been up to on the liver front.

We definitely have mixed emotions, because we see this vibrant, hard working 16 year old that has far surpassed what we were told he would be able to do. But we know his body is failing him, which is starting to show outwardly.  But he still is above what the standard Fontan patient looks/acts like.  I think it’s a delicate balance that maybe is difficult for doctors to understand. Our opinion is, let’s compare him to HIM, not compare him to other kids that are similar to him…he is definitely unique in his activity level and cardiac output. But we are seeing changes in him that are unusual and concerning.   Which then comes to the question of are we missing anything with these subtle changes we are noticing?

So that brings us back to Children’s Dallas. They don’t really have a Fontan program in place yet and they don’t have a protocol set up for monitoring the liver in these kids. After messaging with his cardio in Dallas, he advised that we really don’t need to be seen by GI for 6 more months.  And to reschedule his GI appointment that was set up for us April 9 in Dallas to coincide with his cardio appointment in September.  We really didn’t like that answer, because we feel that there might be something we can start doing now instead of playing the waiting game based on lots of conversations I’ve had with other doctors and others adults and parents of kids that are going through the same thing we are. 

So we decided to reach out to 3 other top rated centers that deal with FALD and have certain protocols set up for monitoring the liver. I sent messages to Boston Children’s Hospital (BCH), Children’s Hospital of Philadelphia (CHOP) and Texas Children’s Hospital of Houston (TCH). And surprisingly, I heard back from all of them the very next day. Either they are THAT good or they are seeing something that needs to be addressed sooner rather than later.

2 doctors from Boston replied to my email almost immediately and one actually called me and we had about a 40 minute phone conversation. He is head of their single ventricle program and running a clinical trial for patients like Kaston. He is super interested in taking Kaston on as a patient and gave me some much needed information and advice.  Of course all 3 centers need Kaston’s full medical records from Dallas and that takes time. But Dr. Rathod in Boston absolutely said he would take Kaston on as his patient. But…it’s in Boston, so there’s that.  We could probably swing going there once, but to have his care moved there and then following up every 6 months could be challenging financially.  Plus there’s insurance.  Just a lot of logistics that would be involved. But he did say that he would most definitely collaborate with the Houston docs if we decided to transfer his care there.

He said they are finding that sometimes they can go in via heart catheterization and tweak some stuff so that they can reduce the heart pressures.  He also was interested in putting Kaston in a FALD clinical trial, which Houston is also a part of. (Dallas is not). So again, maybe collaborating with Houston would be our best option.

He also works close with CHOP and refers some of his own patients with FALD there, because they seem to have the most aggressive protocol for their Fontan patients and are kinda the experts. They start liver screening all of their Fontan patients at 10/12 years old.  So we are 4-6 years behind the curve already.  They even do liver biopsies on all of their Fontan patients across the board so that they can use that information for research and have some sort of idea of what they’re up against.

All 3 centers do extensive blood work, sonogram Elastographies, MRIs, biopsies, and transplants for FALD.  Dallas has never done a FALD transplant.  And an MRI and biopsy are not on the radar for Kaston through Dallas.

I have had multiple conversations with Houston cardiology via phone and email. The one doctor assigned to his case to review actually was trained under his current cardiologist in Dallas and he was her mentor in medical school. She feels he is most definitely on the right care plan and trusts what they are doing in Dallas.  But, is she being objective? There is another doctor in Houston that is going to review his case  and that wants to consult with cardio in Boston. And they are supposed to get us more info about entering him into the clinical trial.

So, for now we have nothing scheduled. However, we are going to be in the Houston area for the Hunt with Heart picnic over Memorial Week-end. So we are trying to coordinate any consult appointments, additional testing they may want to do, or clinical trial stuff while we are down there.

We are also going to reach out to the heart transplant director, Dr. Dreyer, who happens to be a part of Hunt with Heart and he was a part of Kaston’s camp when he went hunting with in September. So he and Bob have a personal connection with Dr. Dreyer, and that always helps.

But before we really can do anything at all we’ve got to get our hands on all of Kaston’s medical records and live imaging of the Elastography, his stress test, his cardiac CT and his last echo they recorded. CHOP won’t even look at him until they have access to those.  And TCH wanted his last heart cath report….well, he was like 3. And she was quite surprised that he hadn’t had a cath since then.  Apparently alot of centers do routine caths on their Fontan so many years out for diagnostic purposes.  Dallas…not so much.

So, we really are just in a holding pattern until we can access his medical records and get them to the other centers.  Hopefully we can get some appointments scheduled for when we are in Houston and just sorta get a better idea of where Kaston truly is at health wise, because right now Dallas thinks he is peachy keen.

I guess what we are wanting is to get a better baseline of where Kaston is actually at. There’s much more simple testing we can do, like a liver MRI and more bloodwork that can give us a more accurate picture.  And then we can go from there and do further testing if we need to….such as a biopsy or heart cath, etc. It just appears that we really just don’t have enough information yet to know exactly his status, according to the doctor in Boston.  As a sonogram Elastography isn’t super reliable, even the results from different machines can be drastically different.  But a biospy is invasive, although can be somewhat accurate, but not always.  And, can we go in via cath and tweak something to lessen the pressures? Why are we not exploring that option if he is as bad as Dallas says he is?

Our opinion is that it doesn’t hurt to get a second opinion, especially when we’re going to be in Houston anyways and there are other doctors out there that might have a more aggressive approach. We just want to make sure we aren’t over looking anything and are getting the best possible care for our boy.  Not that he’s getting bad care, but just that we aren’t overlooking anything that we could be doing now instead of later.

So that’s where we’re at.  Thanks to all who have been checking in!  We’ll update as soon as we get any more info.

 

Liver Update #2

I promise I won’t be bugging everyone so frequently during this journey, but we’ve been overwhelmed by the response we have gotten! Thank you to all who have reached out, asked questions and have put him on your church prayer lists.  It means the world to us. 

We for sure will keep everyone posted just as soon as we know more details, as all of this news was just the preliminary reports. We anticipate more testing and that will give us a much more accurate idea of what Kaston’s up against.

We have tons of questions ready for GI, as I have been doing pretty extensive research (imagine that! 🙄)…reading medical journals and articles, research articles, clinical trials, hospital & doctor reviews, and reaching out to others that are going through this.  Sadly 95% of them are in their 30s & 40s.  Have come across several that have had successful heart transplants and are reversing liver damage at a younger age.  

We have a team already in place in Amarillo to help facilitate his care here and Dallas has already shared his records with them.  Sure don’t wanna be driving to Dallas (or Houston) every time we need to do blood draws.  I am not sure Amarillo has a pediatric hepatologist (liver doctor), but even if so, we already have who we need in his corner. 

Hoping to hear back from cardio/GI tomorrow to get an accurate idea of what exactly they want him there for next month.

He seems to be handling everything ok emotionally so far.  He just wishes some of the side affects would go away, as it’s pretty frustrating. Sadly, I don’t anticipate that happening any time soon, but maybe we can start treating the symptoms.

How are Bob and I?  We’re good.  I think our friends and family are more upset than we are? Is that weird? 99% of the people in our lives now (besides family of course) were not in our lives during the surgery days.  So all they have ever known is that Kaston is this really awesome kid that has a scar on his chest from when he was a baby and has a funky heart.  They really see him in such a normal light, that I think it’s just harder for them.  Bob and I have been through the worst of the worst with this kid, all the while knowing we were never “finished”. (You heart families know what I mean!) So no it’s not ideal, and we hate it for him, but we have always known stuff like this comes with the territory since day one.  We have been facing these giants for 16 years.  It is not something we aren’t accustomed to.  Heart wrenching as parents…absolutely.  But it is our job to educate ourselves, make sure Kaston is good and is taken care of, and walk through the motions while keeping our focus on God.  Let’s evaluate today, make notes and plan for tomorrow.  It is what it is.

We are never finished with Congenital Heart Defects.  And this is the perfect example.  It’s a storm that’s been brewing inside of him for awhile, and it blew in just like the hurricane force winds we had today.

Thanks for jumping on this freight train with us…we’re full steam ahead!

The precarious state of the boy’s liver.

I have been waiting to post about Kaston’s doctor visit to Dallas this past week, as I am still trying to wrap my brain around it all.  I wanted to talk to my people before I shared it with the world, or at least those of you who decide to read this.

So on Tuesday at Children’s Dallas he had a sonogram of his guts and an Elastography that tests the stiffness of his liver.

Wednesday he got bloodwork drawn and we visited with his cardiologist for awhile.  They of course talked about all of the fun things he does, riding dirt bikes and shooting guns, driving and having a job. From a cardiac standpoint his function looks great, his resting heart rate is lower than we’d like, in the 30s, it seems as if it’s slowing down a little but still functioning good.

We discussed his energy levels and fatigue and how he’s been feeling overall. Then comes the elephant in the room.  Dr. Zs demeanor totally changed and there was definitely a big shift in the conversation.

I won’t bore you with all of the medical details, and quite honestly I don’t understand it all just yet. Simply put, Kaston’s liver is failing.  And it seems quite aggressive for his age.  Now keep in mind we’ve known that possible liver complications would be in his future.  But the way it was explained, Kaston’s liver is twice as stiff as it should be at this stage/age.  Based on what we can tell off of labs and what the Elastography report showed, he has moderate to severe liver fibrosis.  He is on the high end of the scale before it’s considered full blown cirrhosis.  His blood work also indicated some major issues to coincide with the Elastography report and his spleen is enlarged.

Most people with the type of cardiac circulation he has (Fontan) don’t reach this level until 20-30 years out….Kaston is only 13 years out.

So what causes this?  Basically put, his heart is pumping at too high of a pressure and it’s damaging his liver much quicker than expected.

So what does all of this mean? Essentially he is in liver failure and has what’s called Fontan Associated Liver Disease (FALD).  There really is no treatment for this but they are doing clinical trials on some meds that we are possibly interested in.  But they really don’t know enough about how to treat it, only what causes it.

Symptoms range from fluid retention, fatigue (which makes sense now), nausea (which he has been complaining of lately), weight loss (which he hasn’t gained in 6 mos.), loss of appetite (which we have noticed), yellowing of the skin, confusion, and a few other things.  He has a few of the symptoms but we just have been dismissing it and not really making a big deal out of it except for the fatigue.

The only thing they have found that can correct it once it has completely failed is a heart transplant or a heart/liver transplant.  They won’t transplant just the liver because the funky heart will just damage the new liver.  We have absolutely zero idea what that will look like for Kaston as far as a timeline goes. Some people can live a few years with cirrhosis before transplant is required.  And transplant is a whole new ballgame with it’s own challenges.  Of which we know nothing about.

I’m sure there will be more extensive testing in the near future to get a better grip on where exactly he’s at. We will go back to Dallas in 3 weeks to see a GI doctor and go from there.

We are also looking at other options as to where we would like to pursue this next phase.  Texas Children’s Hospital in Houston is the top cardiac center in the country. And they have a strong and experienced FALD program. Dallas does not.  So we definitely will be having that talk with his docs in Dallas about transferring his care there .  We want the best team in the country taking care of our boy.

So, we’ve talked in depth to Kaston what this potentially could mean for him and had some pretty tough conversations this week.  Definitely not something that’s easy for a 16 year old to wrap his brain around.  So please keep him in your prayers, not only for his health but also for his mental stability and peace.

And please pray for Bob and I to guide him and support him in the best way possible and that we’re making the best possible decisions regarding his care.

We will try to keep everyone updated as we start this journey and learn more about FALD ourselves.

The Boy’s Cardiac Status

Heading home from yet another trip to Dallas…our 15th year of appointments at Children’s Dallas.

This time we moved with Dr. Zellers over to the Plano hospital, and I am quite impressed!  Was just really wierd not going to the main hospital after doing this for so long. However, we got to spend alot more personal time with Dr. Z and had virtually no wait time.

So where are we with Kaston’s cardiac status?

He had a 24 hour Holter monitor done Monday/Tuesday but Dr. Z didn’t have time to get the reading done on it. He wants to compare it to his last one and see if he sees anything different. His EKG and Echo looked good and he is in normal sinus rhythm.

He said he may want him to come back and do an exercise, or stress test, to rule anything out.  Basically, we are trying to get to the root of his extreme fatigue.  He sleeps all the time and it’s only getting worse.  (He’s asleep right now and slept 12 hours last night) When he is active, his recovery time is twice as long as it used to be.

We feel it is likely a combination of things.  His heart rate really doesn’t get above 115 or so and his average heart rate is in the 40s.  This in itself will cause anyone to slow down.  Top that with his 60% cardiac output from his Fontan anatomy.  And add the demands of puberty into the mix.

This may be just how his life will be. Could a pacemaker help?  Maybe.  But could that harm him too because of his anotomical design? Maybe.

Is there an underlying cause we’re missing? Maybe.

So we will wait to hear back from him next week and see where we go from here.  We may need to go back and do the stress test.  We may just let him push through it and see if he improves.  We might have to just say, it is what it is.  And let the boy sleep.

I am not sure how I feel about it all. Part of me says if, it is what it is and this is what his life will be like from now on, I knew this day would come, just not quite so early.  We knew he would start slowing down and his heart would not be able to  manage the demands he puts on it. So that’s a little disheartening if that’s what it is.

If he needs a pacemaker, that’s a whole new set of responsibilities and limitations for him to worry about. But if it improves his quality of life, then that’s what we need fo do.

I just have to remind myself it could be worse.  He is in relatively good health, his liver seems to be doing ok, his heart function looks decent, his lungs are junk but nothing we can’t deal with, and he gets to lead an easy and adventurous life. Because we designed it that way for him. Let’s get as many as those peak life experiences in as long as we can, because that opportunity will not always be around for him.

Dr. Z seemed pretty excited for him to have gone scuba diving and was glad to hear he did so well.  He said he only had a couple of Fontan patients that had attempted it.  And he asked what his next adventure was going to be…skydiving?  Of course Kaston said yes, one of these days!

So, we’ll just keep on keepin on until we hear differently!