Eli and Family Update

Hi, everyone!  Here's a quick update of what's going on with the Veales.

I'll start with Eli.  Just yesterday we got some news about Eli's referral to Children's Hospital of Philadelphia (CHOP).  Our cardiology team received an email from CHOP requesting a conference call with us and Dr. Steinberg (one of Eli's cardiologists in Indianapolis, the one who made the referral).  The conference call is set for this coming Friday, October 12.

In the meantime, we continue to go to PMCH every 2-3 weeks for a paracentesis.  His next one is coming up on Tuesday.  The kids are on Fall Break next week so instead of a family vacation or anything super exciting, Eli will be hospitalized for a couple days.  I'm sure we'll work in some fun things with the kids during their time off as well!  This will be his 10th paracentesis since May.  Since my last blog update, Eli has stayed relatively stable.  He did have a seizure early one Sunday morning in August.  It was nothing like the two times he had seizures in the past.  This was much shorter, but there's very little doubt it was a seizure.  We don't know exactly why he had it though.  It could be from all the stress his body is under.  One of his cardiologists also said it could possibly be due to his low heart rate.  He had been sleeping and if the rate gets low enough it could have caused a seizure.  Cardiology had already decided to decrease the dose of one of Eli's meds in hopes it would allow his heart rate to come up a little bit and that has made a difference.  Although by decreasing the dose, Eli's heart is more likely to go into SVT again (the extremely fast rate >200/minute).  So far he's tolerating that change well along with the increase in his anti seizure medication.

We've also had a meeting about his IEP (Individual Education Program) at school.  Eli is struggling (although I don't think he realizes it).  He's always had a shortened spelling list, varied testing methods, and extra help, but adjusting to the increasing requirements is tough.  He is very far behind his peers in reading, spelling, and writing.  Math is a foreign language to him.  He will be tested again by the school psychologists (he was due for it at this age anyway) and we will meet as a group again after that.  This will help us determine more specifically what learning disabilities he has and how we can better help him at school and at home with schoolwork.  We are lucky to have so many people on his team!

He's as cute as ever!

Mommy and Eli just before paracentesis #9 in mid September

Zachary, Eli, and Charlotte in front of the helicopter that Eli rode in back in May.  There was an event recently that involved local law enforcement, firefighters, and other first responders.  There were two helicopters there and this is the one that Eli flew in.  The other one may have been the one he flew in when he was two and had his first seizure.  It was nice for him to see those and know he didn't have to get on them.  He was shy talking to the pilots but he really did enjoy checking them out.  

These two fight all the time.  Truly, All. The. Time.  But they love each other as fiercely as they fight.  They have to snuggle together most nights.  Many times, I have to unwind them from each other to be able to do Eli's tube feeding or nighttime meds.  It's adorable!

Our sweet guys walking into school together on the first day

A group hug after school.  Charlotte missed her brothers so much their first day back.  They had their group hug and then were fighting before we got home.  Typical!

Eli is a big second grader now!  This is him with his teacher, Mrs. Elsey.  He loves school and enjoys going.  Despite his troubles, he is making advancements and learning.  We are so lucky to have such a great system with wonderful people who want the best for him.

Zachary has been busy studying after school three days a week in hopes of making the Spell Bowl team.  He has a list of 700+ words to study.  I had no idea what all goes into preparing for this and it is quite impressive!  It takes a lot of dedication from the students and the teachers who lead this.  I hope he makes it after all the hard work he's put in.  Zachary's birthday was in mid-September.  He's eleven now!  Standing at 5'7" and 160lbs, he's often assumed to be and treated as much older than eleven.  A couple weeks ago he got glasses.  It isn't a strong prescription, but the doctor recommended glasses now.  We knew last year at his appointment this was a possibility.  With the glasses this fall and likely getting braces after the new year, his looks are changing!  In addition to Spell Bowl and doing great in school, he is getting ready for elementary basketball and is still working out in preparation for travel baseball.  He is a great kid and I love getting to hang out with him.  He's very smart, caring, and funny.  He still loves dinosaurs and is planning to be a paleontologist when he grows up.

Zachary in his new glasses and all dressed up for Dress for Success Day at school

Family picture in front of the Jurassic World photo scene for Zachary's birthday

Zachary with his homeroom teacher for fifth grade.  Yes, she is wearing heels and he is still taller than her.

First day of fifth grade and second grade.  Eli was happy about going to school, just not about having his picture taken.

He was in a much better mood and asked to have his picture taken outside school.  Love my boys and their matching shirts!

Zachary came to me and Bryan this afternoon asking what we wanted for supper.  He said he was cooking for us since I didn't feel well and Bryan had been busy.  He made baked chicken, macaroni and cheese, and biscuits.  All of this is within Eli's fat free diet too.  He did it all himself and it was very good.  He also made pink lemonade.  Such a sweet kid!

Charlotte has started preschool.  In fact, picture day was this week.  She is loving school.  She was very reluctant at first, saying she didn't want to go to her preschool but wanted to go to the same school her brothers go to.  She sees them walking in and out of their elementary together every day so it just made sense to her that she should go there too!  She had her first day and did fine.  Some nerves, but no tears.  When she came out, she ran straight into my arms with a big smile on her face and lots of great things to say about her morning.  After she was done talking I asked her if she had fun and she said yes!  I asked if she was excited to go back and with a smile on her face she immediately replied "No.  Never going back to preschool.  Never, ever, ever."  She did go back and so far has not missed a day.  She loves it and it is so good to see her getting to play with kids her own age.  It's bittersweet to see my baby transitioning from being a baby, to a toddler, to a preschooler, but it is so good to see her flourish.  She's also started a baseball class for kids age 3 & 4.  She's had two classes so far and loves it.  We're thinking of enrolling her in dance class because she is ALWAYS dancing around the house.  Dance class is new territory for us!  When we asked her she said she doesn't want to do it, but she also didn't want to do baseball or go back to preschool.

Our baby is growing up!

A rare occasion of them awake and not fighting.  They took turns walking on the ledge and always held hands so they could help each other.  When they get along it is amazing, sweet, and beautiful....but when they don't it's loud, rude, and exhausting!

At her first baseball practice.  This drill had them pointing with their glove where they wanted to throw the ball.  She did pretty well!

Being silly on Picture Day at preschool!

Speaking of transitions, we had a big one over the summer.  Charlotte was not too into potty training.  She liked wearing a diaper, didn't like the toilet, and really didn't care if she was potty trained.  Over the summer we told her to go to school she had to wear underwear and put all her pee pees in the potty.  She's a pretty smart little thing and once she was consistently going in the potty she refused to wear underwear & wanted her diapers back.  After questioning her, she simply said "I want my diapers now and save my underwear for school."  (sigh)  Anyway, that's all straightened out now and she has been fully potty trained since mid-summer.  So for the first time in almost 11 years....we are a diaper free house!!!!  Hopefully we can stay that way for a few more years before Bryan or I need them.  It blows my mind a little to think I've only had a handful of days in the last eleven years that I didn't change a diaper.  I'm slowly making the transition from carrying a diaper bag to carrying a purse.  I still carry wipes because, well because we'll probably never stop needing those!  I also usually carry some equipment to assess Eli if we need to (stethoscope, blood pressure cuff, pulse ox monitor, and bandaids because the boy has a serious picking problem).  But it's not a diaper bag!

I think that's most of what's been happening with the kids.  Bryan and I are busy as usual as we keep up with their schedules, homework, and make lots of good memories!  Bryan has been busy taking care of me the last couple days  I've recently had a flare up of my eye issues (inflammation of the iris and fluids within the eye).  Bryan took me to my ophthalmologist yesterday and I got a steroid injection inside my lower lid.  I've had many of those before and although they're not fun, they do help.  Today I feel better, but still have a lot of vision disturbances and headache.  I'll keep taking my steroid drops multiple times a day, keep my eye dilated, and go back in two weeks.  Hopefully then I can be well enough to start tapering off my meds.  This is nothing new to me, I've dealt with this condition for over 20 years and probably had 10 injections in each eye over the years, but each flare up reminds me how precious vision is.

I do have a list of prayer requests:
- that Eli's procedure Tuesday goes well.  If you can, please wear your Eli shirts and/or bracelets on Tuesday October 9.
- that we get good information from our phone conference with CHOP next week
- we've accepted an offer on the old house so PLEASE pray that it goes through.  We need the buyer's financing and all the inspections and appraisals to happen without much trouble because we would love to have this stress off our minds
- for my eye to get better.
- for us to handle everything going on
- that all of you know how much we appreciate every bit of prayer, support, encouragement, and love we've received from all of you.  It helps us more than you can imagine!

Thank you all for reading and keeping up with our craziness!  There are a few more pictures at the end of this post.  They are from the fundraiser that was had for Eli at the start of the school year.  Several teachers at the boys' school worked really hard to put together a concession stand, pool party, and raffle.  It was an amazing evening.  Eli said several times "Are all these people here for me?"  Physically seeing the support we have was overwhelming for him and us.  Thank you to everyone who organized, worked, and donated to this event.  About $2500 was raised for Eli's expenses!  We were shocked!  Again, thank you!

What was on the sidewalk outside the concession area

Zachary and Eli working concessions as Charlotte tries to decide what to order

Some of the people enjoying hot dogs or walking tacos

Zachary LOVED working the concession stand

Some of the family who came to the fundraiser

The guest of honor exiting the pool after having played for a long time!

Charlotte loved the slide!

They had Eli autograph a baseball and it was raffled off.  

Mamaw Linda won the autographed baseball!  What's so funny about it, is she didn't buy the ticket.  Our realtor was there and he put Linda's name on the tickets he bought for the baseball.  

A family photo at the end of the night.  We were all wet, tired, and so happy!

We Owe his Liver an Apology

On Tuesday, June 26, Eli and I left for Indy about 4am.  He had to be checked in really early for a paracentesis.  Off topic, but that day was our 19th wedding anniversary (awwww!)

We were so young!  Happy Anniversary!

So we arrive and check in.  He did great getting the IV started and we went to the procedure room. He was given Ketamine for sedation and they got started.  Because we don't want to remove a lot of fluid at once and risk throwing his blood pressure off, the doctor only wanted to remove 2 liters from Eli.  That was easily done and he was stable throughout.  He was transported up to the third floor (our home at PMCH) and got settled in his room, all by 8am.  Eli did great and was slowly coming out of his sedation trance.  Soon he was asking for food and drink, then was up playing.  His blood pressures were great all day long.  Overall, he was doing wonderfully.  So well, in fact, that by late afternoon, the doctors said he could go home instead of spending the night which was our original plan.  So the suitcase went back to the van, the IV came out, and we were waiting for discharge paperwork.  Then the GI doc came in.  Plans had changed.  All because of a picture I took while Eli was getting his paracentesis done.  I took a picture of the fluid that was drained out of him.  It didn't look like it should (should be clear, kind of yellow).  Eli's looked like a thick, tan formula was poured into the container (there's a picture at the end of this post if anyone is curious what it looked like).  No one had mentioned that it looked that way before.  No tests were ordered on this drainage since it was his fourth in a six weeks.  When we got up to his room, I started asking questions about the fluid color and consistency from his previous paracentesis procedures.  It did not look like that.  My picture circulated from the Infectious Disease and Cardiac NPs, to one of Eli's cardiac doctors, to his GI doctor.  They all thought the same thing:  chylous (I'll explain more about that further down).

Because no samples were taken, everyone agreed Eli needed another paracentesis to obtain a sample so they can confirm.  So Eli was scheduled for a paracentesis the next day.  We were not going home, but that was okay because we had originally planned to stay the night anyway.  Knowing there's a specific diet that goes with the diagnosis of chylous, I asked the GI doc if he had any dietary restrictions for supper that night because his aunts were coming and brining food.  She looked at him as he absorbed the news that he was not going home until the next day at the earliest, knowing he'd have to have another IV and more sedation.  She looked right at me and said he could have whatever he wanted that night!  So he ordered a Happy Meal...then proceeded to eat not only that, but half of my sister's Quarter Pounder as well!  His aunts also taught him a new trick with his French fries:

Sticking a French fry out of the hole left by his latest lost tooth!  Goofy kid!

The next day Eli was busy all morning in the playroom.  First they had play doh time.  He mixed play doh and glitter and used hospital equipment {medicine cups, oral syringes, emesis (puke) buckets} to play with the play doh.  He had a blast.  Then the Civic Theatre came for interactive storytime.  He really enjoyed that as well.  It helped pass the time until 12:30 when he went for the paracentesis.  He got another IV started and did great again with the sedation medication.  The doctor drew off a large syringe to be sent to the lab.  Then he drew more fluid off Eli.  His blood pressure was dropping some so the sedation nurse said it was time to stop.  Another 1.8 liters was off his belly.  When we left the procedure room Eli's BP was 90/40.  It was taken as soon as he got back in his room which was less than five minutes later.  In those few minutes it had dropped to 70/32.  Fluids were immediately started.  Fluids were going, but they had not had time to bump his pressure up and he dropped as low as 40/20 before coming back up.  Of course I was concerned, but I really wasn't worried.  He had just had 4 liters of fluid taken off in just over 24 hours, it made sense that his body was redistributing  and adjusting to not having the extra pressure of fluid on his belly.  As his blood pressure was going down, the sedation nurse practitioner, her nurse, the unit nurse practitioner, the hospitalist, and Eli's nurse were all present.  We just had to keep monitoring him and wait for the IV fluids to do their job.  And they did.  Soon he was back up to 80s/40s where he stayed most of the evening.  He woke up well from the sedation and was ready to eat.  The art cart came by and Eli received a bunch of new goodies (colorworks pens and book, glitter glue, a sketch pad, colored pencils, and modeling clay).  Soon after he was visited by the book cart and selected two sticker activity books.   We didn't have any visitors Wednesday night which was fine with me.  I was exhausted and wanted to get him to sleep early so I could sleep.  There was a real chance we were going home the next day.  We did wind up getting discharged, but it was late afternoon when we left.  Throughout the day, his blood pressures were hanging out in the 80s/40s.  He was very active, playing, eating well, being silly, but his numbers were lower than his normal.  They came up some in the afternoon and cardiology cleared him for discharge.  In hindsight, I realize his medication schedule in the hospital was different than the one we use at home and the way some of his meds were given affected his BP.  Now we know better for next time!


Now for some questions:

What is chylous?
Chyle (pronounced like the name Kyle) is something we all have in our lymphatic system.  It is a milky bodily fluid consisting of lymph and fats.  The lymphatic system carries the fluid to our veins where it returns to the bloodstream.  This fluid helps with immune function and fat and protein transport.

Why is it in Eli's abdomen?
Apparently there is a leak in Eli's lymphatic system.  It is draining chyle in his abdominal cavity which then must be drained off every so often.

What do we do about it?
Immediately what we do is test the fluid and wait for confirmation that the fluid drained off his belly is chyle.  We also start Eli on a fat free diet.  Yes, fat free.  Not low fat, not low saturated fat, NO FAT diet.  He is allowed 3-5 grams of fat per day.  That is not much.  The goal with the fat free diet is to slow the leak down so he doesn't have to have a paracentesis as often.

The next step is to consult with a doctor who specializes in surgical repair of the lymphatic system.  That is not a common specialty.  No one at PMCH does that.  We are waiting for a referral to one of the top children's hospitals in the nation.  Eli will be referred to Children's Hospital of Philadelphia.  We don't know how long it will take to gather his pertinent records or for the team at CHOP to review them.  We don't know if we will be going in the next couple weeks or if it will be a month or two.  We don't know how long we will be there when we do go.  All that will be figured out soon.  Bryan and I haven't talked a lot about what we'll do.  All we've decided is that we will opt to drive rather than fly.  Having our own vehicle while there and not worrying about having to change flight plans due to a change in discharge will be much easier.  I have several heart momma friends on FB whose kids have been treated there so I will be reaching out to them for guidance.  I also have an aunt and uncle who live close to Philadelphia so I can reach out to them for support as needed when we are there.

From looking at CHOP's webpage, I have gained a little knowledge of what they may do for Eli.  Of course each case is different and we all know Eli's body does things its own way, but to have a general idea is helpful to me.  It looks like the procedures they do to repair a leak in the lymphatic system are interventional procedures, which means they aren't big huge operations.  That is comforting.  It also looks like (again, in most cases!) the long-term outlook is good.  I'll type exactly what the website has in that section:

"The long-term outlook for many patients with chylothorax is very good.  Nearly all patients with thoracic duct trauma can be cured.  Experts at CHOP have also seen encouraging results when treating patients with other types of chylothorax.  Treatment often provides a long-term cure."

***clarification:  Eli does not technically have chylothorax which is the accumulation of chyle in the chest cavity, but from what I've read, the accumulation in the abdominal cavity is treated the same.  The accumulation of lymph can be in the chest, abdomen, or other body cavities.

What caused the leak?
We don't know.  It's most likely a leak of his thoracic duct (the main lymph vessel).  That can happen because of an injury to the thoracic duct (such as being nicked during a surgery), congenital abnormalities (meaning the patient was born with issues of the thoracic duct), or excessively high venous pressures due to heart failure, pulmonary hypertension, or congenital heart surgery.

Hasn't he dealt with this before?
Actually, yes.  Eli had chylothorax after his third heart surgery, the Fontan.  He had several pleural effusions (pockets of fluid) in the lining of his chest wall and the fluid drained off had chyle in it.  It was treated with a very low fat diet and time.  It's not unusual to have it happen after heart surgery, especially in a young child.  There are many, many vessels in a very small space.  He recovered from that and did well.  We have no idea if the two are related.  The fluid drained off Eli's abdomen last month wasn't the same appearance as what it is now.  Many tests were run and I'm pretty sure they tested for fats in it.  If it were positive, his treatment would have been different a month ago.

What about his liver?
Apparently Eli's liver is not the criminal we were making it out to be.  His liver does have some damage.  He does have liver congestion and some fibrosis, but they are not believed to be the cause of all this fluid.  Even when we blamed the liver for it last month, it kind of made sense, but there were still holes in that theory.  Too many of Eli's other test results were normal for it to solely be the liver.  Chylous makes more sense on many levels.

How much fun is a no fat diet?
Well, it's not horrible!  Like I said before, the no fat diet is to slow down the leak, it will not stop it.  Eli is also on a different formula for his nighttime feeds.  That way he can get enough calories without the fat.  Overall, Eli has been adjusting well to the diet.  We're all trying to follow it with him, but we need to make sure we still get our fats, especially Zachary and Charlotte as they need them for healthy growth.  We've scoured our local grocery store and two local Walmarts for options.  We have a lot of turkey and chicken.  Since we can't use any oils or butter for cooking, I've used chicken stock a lot.  There are many things fat free that I didn't realize and chicken stock is one of them.  Last night we had grilled turkey cutlets with bbq sauce for dip, mashed potatoes made with chicken stock and skim milk, gravy, sautéed zucchini, mushrooms, cabbage, and brussels sprouts (sautéed with chicken stock, salt and pepper), and corn on the cob.  The bbq sauce and gravy were the normal ones we use and they're both fat free.  Snacks have been hard.  He loves salsa, but we couldn't find a fat free chip for it.  There were many 'low fat' options, but those still had 5-10 grams per serving which is two or three days of his allowed amount!  We found some soft tortilla shells that are 0.5g each.  After going in the toaster for a few minutes they're crunchy like chips and he is happy with them!  He also eats carrot sticks or fat free pretzels with his salsa.  We're working on more recipes and snack options for him, but we've done well so far.  Did you know you can make a pretty good potato chip in the microwave?  Thinly slice a potato, season it, and put it in the microwave on parchment paper for 6 minutes.  We've all liked those!  So thank you to all you sweet people who have recently sent us gift cards for Walmart!  I just used those to buy a lot of fat free items to get us started on this journey.  Please know your generosity went directly to providing something that would've been an expensive hit to our budget and is necessary for Eli.  Thank you!

What about his pacemaker?
Well, it's cancelled.....  Priorities, you know?  This lymphatic problem has to be resolved before proceeding with the pacemaker so it's on hold indefinitely.  And once it's back on the horizon, the cardiac schedulers get to schedule it for a fourth time.  I hate that for them!  But it is what it is.

So that's where we are now.  It will take some time for Eli's records to be sent to CHOP and for them to be reviewed.  In the meantime, we keep an eye on his belly and watch for more fluid to accumulate.  It's fully expected that he will need another paracentesis (or two?) before we get to Philly.  He's still on five doses of diuretics each day, so there are a lot of bathroom trips.  Meal prep takes more time these days because of dietary restrictions,  Every day he gets weighed, his belly measured, and his blood pressure taken.  Not to mention all the phone calls to and from providers about his status, keeping meds refilled, and having follow up appointments.  He is a busy kid who keeps us very busy as well!  But he is worth all of it!  Thank you for all the prayers and support.  We will update as we can with new information.

During the paracentesis on Tuesday.  His buddy Pablo right there with him as always.

The playroom has a new touch screen game table since we were there last month.  Eli enjoyed many of the games offered on this. 

Eli took this picture (and many others) of his dinosaur themed room.

This boy was still a little loopy from the sedation meds after the paracentesis on Wednesday, but looking pretty good for just having a blood pressure of 40/20!

Another picture of his cool dinosaur room

Walking the halls like a boss!

He was always ready to shed the hospital clothes and put on his own.  This was on Wednesday evening as we walked the halls again (it happens many, many times a day!)

Sent this picture to Bryan and Zachary as a way of saying 'Good luck!' before Z's tourney game that night.

If you look at the little square window above the trash can, you'll see a goofy little guy!

Thursday afternoon waiting to go home.  Here he's in the playroom shopping for fat free foods!

Walking out of the hospital!

Bye, PMCH!  See you next time!

Riding the pony at Meijer where we stopped for a few fat free supplies before heading home

Warning!  The next picture is of the fluid drained Tuesday in his paracentesis.  I don't think it's gross, but things like that don't bother me.  Just warning you this is your last chance to stop scrolling if you don't want to see it!

This should be a clear, somewhat yellow fluid.  Instead it's thick, tan, and definitely not clear.

Liver Biopsy Results and Future Plans

When Eli was hospitalized with his heart rhythm issues in May it was also discovered that he had liver problems.  We asked for many prayers during multiple tests including a liver biopsy, but we were reluctant to share what exactly was going on.  At the time, we didn't know anything for sure and what the doctors feared was very bad news.  We just couldn't explain until we knew more.  Outside of our medical team, we didn't talk about it with anyone, even our immediate family.  We had to know more before we could share.  Thank you for all the prayers even without us disclosing exactly why we needed them.  The power of prayer is amazing and Eli's results were much better than anticipated.  First, let me explain what we thought was going on and what Eli was being tested for, then I'll explain what the tests showed and a plan for him.

What we thought:

During his May admission, once his heart rhythm was more stable, the focus shifted to his growing abdomen.  After an abdominal ultrasound he had a liver ultrasound.  Then he had a more specialized liver ultrasound.  Then he had a CT.  Then a liver biopsy.  From what the team had seen on ultrasounds and the CT, they were fairly certain he had an advanced case of liver cirrhosis.  They wanted the biopsy done to confirm that and to show what stage of cirrhosis he was at.  Liver cirrhosis is a disease of liver damage from a variety of causes which lead to scarring and liver failure.  There's no reversal of the damage done from cirrhosis.  Kids with Eli's type of circulation (also known as Fontan circulation) are known to have liver problems later in life, but cirrhosis is rare.  His gastrointestinal team, cardiology team, and the hospitalists had never had experience with a Fontan kid having cirrhosis.  The research they did yielded very little help.  It looked like Eli was once again blazing his own medical trail and doing things no one else has done.

{Let me get off track here just a bit and say something about his heart and treatment so far.  The heart surgeries Eli has had done are still relatively new in medicine.  Twenty five years ago, most babies born with his defects were sent for a transplant right away (which gets rid of the defective heart, but brings about a whole lot of other problems and issues to deal with in addition to the fact that a transplanted heart doesn't last as long as a natural heart....studies estimate a heart transplant averages 13 years).  If the baby wasn't a good transplant candidate, then they were made as comfortable as possible and let nature take its course.  As time passed, less and less baby hearts were available.  Why?  Because of stricter car seat laws.  We were keeping our kids safer in our vehicles (which is a VERY good thing!) which meant fewer infant/child deaths which in turn meant a major decrease in the availability of donor hearts.  Some surgical options had been developed and trialed in heart defect children, but weren't routinely used.  With transplant options becoming less prominent, these surgical options became more prevalent.  My point with all this is, there are not a lot of adults living with Fontan circulation.  Most of the patients are still children.  We don't have a large medical base to study the long term effects of Fontan circulation on other parts of the body.  So although we do know liver disease is common with Fontan kids, there's still a lot of research going on to discover other negative effects or problems the Fontan will bring.  However, we don't regret undergoing the Fontan.  The goal for Eli has always been have this series of surgeries which is believed to help get him to his late teens/early adulthood before needing a heart transplant.}

Back to now, we don't have other cases or research to compare Eli to.  But the fact remains he is suspected to have severe liver cirrhosis based on his radiology studies.  The CT and ultrasounds showed that his liver was hardening and had many nodules in it.  The good news is that the ducts going into and out of the liver showed good flow and his liver function tests were good.  But we've always known Eli's body compensates for many defects very well, so the fact they thought he had those good things didn't mean the bad things weren't happening.  One doctor even had a discussion with me about the immediate plan for Eli once the liver biopsy results were in.  This talk was only two days after the biopsy when we had no more information than we did two days before the biopsy, so in hindsight this talk was a very premature discussion that I now believe she did not need to have with me, but I'm sharing here to further explain how serious and worried we were at the time.  She said Eli would need to be listed soon for a liver and heart transplant.  The reason for both was to have both organs come from one donor which would greatly minimize the risk of rejection.  If he were to be listed, it would happen in the next few weeks (as in yet this summer).  PMCH does not do pediatric transplants.  In fact, if Eli needed to have both done, not many places do that.  We were likely looking at going to Cincinnati or Philadelphia for an undetermined amount of time for him to be listed, wait for the perfect donor, then have the surgery, and begin recovery from that.  So not only were we facing a very uncertain medical future for Eli, but we thought our family would be further broken up for a long time as one of us (probably me) would move with Eli and the other stay with Zachary and Charlotte.  All that in addition to having just moved and our old house still being on the market (had an offer 11 days after listing.  After working on that deal for six weeks, it fell through just prior to closing so it is back on the market and we technically still have two homes).

And that's not all that was going on.  When Eli was to have the liver biopsy, he also had a lymph node biopsy. Yep, more concerns about Eli's health.  Through all the imaging that was done, it was discovered that he had several enlarged lymph nodes.  The teams wanted to biopsy one of those to rule out lymphoma.  That's right.  There was a slight concern that Eli could be battling cancer of the lymphatic system.  Overwhelmed doesn't begin to describe the emotions Bryan and I were feeling.  There was too much going on, too many unanswered questions to explain publicly at the time.

What we know:

On Wednesday, May 23, Eli had a big day.  He had a heart cath, liver biopsies, lymph node biopsies, and paracentesis.  So he had a cardiologist, an interventional radiologist, a pathologist, and an anesthesiologist in the procedure room in addition to the staff of nurses and technicians necessary to accomplish everything that needed to be done.  None of his procedures were actually that difficult or dangerous for him, but just scheduling all that together is so complicated.  Major kudos to the hospitalist staff and others involved who made it all happen for our little guy.  He tolerated everything very well.  We had the heart cath info immediately from Dr. Parikh and it was all good.  Eli's Fontan was intact and working just as it should.  Eli has several (many, many!) collateral veins that have grown off of some of his major vessels.  Sometimes those are closed off with coils, but Dr. Parikh said Eli's are too small and too numerous to do.  Also he said if the body grows them and we close them off, the body will grow more.  He doesn't believe these are hurting Eli and saw no reason to spend hours coiling each of them, even if he could.  Any other problems he saw from the cath were things that he thinks can be corrected or at least improved with a pacemaker.  Overall, it was great heart news.  We knew the biopsy results would take many days to come back so we were happy with what we knew.

A few days after the biopsies were done, he was sedated again.  He had more fluid removed from his abdomen for testing as well as drainage of an abscess located deep in his abdomen.  We had to be sure he didn't have any active infection going on before proceeding with a pacemaker in the future.

As answers came in we confirmed Eli has no active infection going on in his body.  At an appointment with Eli's regular GI doctor (not the one who had the transplant talk with me), we got the biopsy results.  We actually had the results before that so I had a good idea of what was or wasn't happening, but we didn't have results as far as how they pertained to Eli and what the next step was, so we didn't share them yet.

The liver biopsy showed liver congestion and mild fibrosis...NO CIRRHOSIS!!!!  Fibrosis is scarring of the liver, but it's an early stage and Eli's is classified as mild.  What damage has been done is not reversible, but if we can alleviate and control some of the liver congestion, then the progression can be significantly slowed.  So he does have liver damage, but honestly the news we got is basically best case scenario.  I made it a point to discuss with her the conversation had with the other doctor about transplant and she confirmed my thoughts that we are no where close to needing to go that route for Eli.  Although it may be in the cards for him one day, it's not anything to worry about at this time or likely even in the near future!  The lymph node biopsy also did not show anything of concern so we were blessed with amazing news on many of our fears!

What's next:

It is believed that the liver congestion and resulting fibrosis are related to Eli's heart rate and rhythm.  He is still in his slow junctional rhythm and also has some breakthrough SVT.  Everyone has agreed he needs a pacemaker, but cardiology did not want to do surgery to place one until we knew he had no infection and what the status was of his liver disease.  Now that cardiology has clearance (and even encouragement) from Infectious Disease and Gastroenterology, they got to work getting surgery set.  Of course it's not that simple as picking a date on the calendar.  I do not envy the scheduler in their office.  This is the third time this poor person has had to coordinate all this for him.  As of now, he is set for pacemaker placement the week of July 23.  The day before his pacemaker placement, he will undergo a few tests.  One will be another paracentesis to drain fluid from his abdomen.  We'll talk about that more a little later.  The other will be a test by Dr. Steinberg, his pacemaker doctor, to further investigate Eli's heart rhythm.  He wants to see if there's a chance Eli could have a pacemaker with just one lead.  The significance of this is pretty major.  If one lead would work for Eli, then the surgery to place it is a lot easier than if he needs multiple leads.  Of course we want what's easiest for Eli, but what's easiest now may not be easiest in the long run.  I'm curious to see what will happen.

While Eli was in the hospital in May he had three paracentesis procedures done.  Nearly seven liters of fluid was taken from his abdomen.  Because test results were still pending, nothing was done about the cause of the fluid, so we knew it would come back.  And it has.  At his GI appointment last week the doctor said he needed it drained off.  She waited a while to see if we could coordinate with cardiology to have the paracentesis and pacemaker closer together.  Now we know pacemaker can't happen for another month, so he needs to have a paracentesis soon.  Actually she's recommending three between now and pacemaker placement.  He has a significant amount of fluid to remove and he will likely accumulate more as time passes.  When they took four liters off the first time, he did not do well that night.  We don't want to wait and do a large amount the day before pacemaker surgery and risk him crashing and not being stable enough for surgery.   That's why she wants to do three between now and then, so they can remove smaller amounts each time.

Eli is scheduled for tomorrow (June 26) to have a paracentesis.  He has to be checked in at 6:30am so he and I will leave town a little before 4am.  We opted not to spend the night up there somewhere, it just makes more work and Zachary has a ball game we'd like to see tonight.  After the procedure, he will be admitted so they can monitor him closely.  The plan is to just stay the night and come home on Wednesday.  I will pack for multiple nights!  He will have another paracentesis done in early/mid July, then a third one the day before pacemaker surgery.

The thought process now is that once his heart is in a regular rhythm with a higher rate, it can decrease the amount of liver congestion he's experiencing, thereby decreasing the progression of fibrosis.

We would appreciate your prayers tomorrow as he undergoes this procedure, for safe travels, and for Zachary and Charlotte.  Zachary understands what is going on and is doing okay with it, but Charlotte is not pleased to hear about Mommy and Eli leaving again, especially overnight.  She will be fine, but it's a lot for her to take in.

Thank you all for your prayers!  Eli is proof they work!  In closing, I'll add a few pictures of events that have happened since we've been home.  We have been BUSY!  Eli has had baseball, Zachary has had baseball, Bryan's sister got married, we've had a family reunion, and we're still trying to unpack from the move.  Busy times!  But always time to cherish Every Little Beat...

Eli and his little league team after their last game together

Receiving his medal from the league president

Not really sure what these three were up to, but they're so cute!

We went shopping for me to get a dress for my sister-in-law's wedding and these two had to have a ride :)

Eli with my mom and sister at the Summer Social

Playing ball in the yard one day, these two had to have a rest....on Aunt Kendra

Throwing coins in the fountain at St. Vincent the day of his appointment

Playing with kinetic sand in the kitchen.  Not really sure why his foot is in it.

Eli receiving a special blessing from the pastor who performed Kendra's wedding ceremony.  He listened intently but did not put down his bag of chips.

At the rehearsal.  From left to right:  Alyssa, Charlotte, Eli, Gunner, and Zachary.  All five were flower girls and ring bearers for the ceremony.

Taking pictures after the wedding.  All five kids were very good through the ceremony and getting pictures after.  So proud of all of them!  Then they danced the night away and provided lots of entertainment!

Not an official photo, but the Veale family in full force.  We were blessed with a beautiful evening for the wedding.  The temperature was good, the rain held off, and it was gorgeous.  

Bryan, Eli, and family friend Tom.  

Zachary, Ashley, Eli, and Bryan having some fun at the reception