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| 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:
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| 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.
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| During the paracentesis on Tuesday. His buddy Pablo right there with him as always. |
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| The playroom has a new touch screen game table since we were there last month. Eli enjoyed many of the games offered on this. |
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| Eli took this picture (and many others) of his dinosaur themed room. |
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| 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! |
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| Another picture of his cool dinosaur room |
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| Walking the halls like a boss! |
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| 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!) |
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| Sent this picture to Bryan and Zachary as a way of saying 'Good luck!' before Z's tourney game that night. |
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| If you look at the little square window above the trash can, you'll see a goofy little guy! |
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| Thursday afternoon waiting to go home. Here he's in the playroom shopping for fat free foods! |
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| Walking out of the hospital! |
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| Bye, PMCH! See you next time! |
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| 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!
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| This should be a clear, somewhat yellow fluid. Instead it's thick, tan, and definitely not clear. |
