Day +85

Today Raul got his G tube switched out for a GJ tube. We are hoping to be able to get him off TPN sooner rather than later, as his liver is still not happy. His bilirubin had jumped back to over 10 this morning, so he also had a repeat ultrasound. The ultrasound only showed minor changes in the size of his kidneys/liver, and did not give us any straightforward answers for his increased bilirubin.

Other than that, Raul got platelets today after two days without, and he has not needed GCSF for several days now as his ANC has stayed above 1. Raul's CSA was switched from a continuous drip to regular IV doses, which is a step needed before he can get out of the hospital. We had tried g-tube doses unsuccessfully before, but hopefully the IV version will work better for him!

Day +83

Things have mostly been the same the past few days. Raul is getting dialysis four days a week still; usually three days of four hour runs and one day of a three hour run. He is still peeing, though it hasn't increased. We are hoping his BUN won't jump as high between dialysis days once we get him to take more formula vs. TPN. 

On Wednesday Raul will be switching his G tube to a GJ tube. His vomiting has increased recently, and we want him to be able to tolerate more formula as it will help his liver and kidneys. Raul's bilirubin has been high again recently, which is why he looks jaundiced in photos. It was up to 10 at the end of last week, but this morning was back down to 6.7, which was a relief!

Raul is still getting the Soliris medication every Friday. It is hard to tell if it is helping yet, but he did go 4 days between blood transfusions and did not need platelets today. If he has aHUS, infection can worsen symptoms, so we may have seen some of that with his positive blood culture and worsening symptoms around that. 

He has not had any more positive blood cultures, though they are still working on some antibiotic sensitivities for the one he did have as it was an unusual type of staph. His fevers have improved with vanco, but he is still having them quite often. They also sent off a new blood culture today for some rare bacteria. 

Tonight Raul got a short pass for us to go for a drive and look at Christmas lights. Raul has been asking to go on a drive for a few days, so was pretty excited when they said yes! The best part was that we got to see a fire truck back into a fire station, then pulled up to talk to the firemen for a few minutes. They even turned on the flashing lights for Raul! We are hoping to be able to go back another time to go inside the fire station and see their trucks (it was snowing pretty hard and we needed to get him back).

Checking out the lights on the firetruck

 

Day +80

Merry Christmas! Today we got a four hour pass to spend part of Christmas with our family at their rental house. We had a great time, and it was very nice to get a break from the hospital! Raul was up a lot overnight, but then slept in slightly this morning. When he woke up there were Santa presents from the hospital on our counter, which he was quite excited about! We opened those, and he got some great vehicles. Then it was time to get him dressed and unhooked from his pumps!

My mom and dad came to pick us up at 10am for our four hour pass. We headed over to the rental house and were surprised that Raul wanted to sit on the floor not lie on the couch. He stayed sitting on the floor through opening stockings and his first Lego set from under the tree. Then he got to play iPad on the couch for a little bit while we had brunch. He also played a bit with a Lego car on the couch with my brother in law.

Then he was excited to open the rest of his presents. He still isn't playing much, but he wanted everything opened and did smile about his gifts. He had picked out garbage truck shirts for some of us, and did laugh as we all opened our shirts one after the other! After we were done opening presents he wanted us to build a few of his Legos, and he requested to lie on the floor under the Christmas tree. He stayed awake until just before we had to leave to come back to the hospital, and then was pretty unhappy to have to come back. It still seems to be good for him to get these breaks, though, and he acts much more like himself when we are out and having fun!

Days +78/+79

Tomorrow will be our 100th day in the hospital, and things are largely at a standstill. His fevers just won't quit, and they have been looking everywhere but we have not identified a source for his long term high fevers. The past few days they had been getting worse again; almost constant, which also causes gagging 24/7. Yesterday one of his blood cultures from Monday came back positive for Staph, so we are assuming that was the cause of his worsening fevers. We restarted two more antibiotics yesterday, and today he went about 8 hours between doses of IV tylenol! 

Inflammatory issues like infection can also worsen aHUS, so we think that may be happening. This may be the reason his bilirubin has been going up again causing him to be more jaundiced. He is still needing platelets most days, and blood every few days. We are hoping that this will improve over time with the medication for the aHUS. 

Raul is still peeing, which is great! And we were surprised today when his kidneys had actually cleared some of his Vanco from yesterday, so he even had to get another small dose today (last time he was on it they only gave it after dialysis, as his kidneys weren't filtering it). He will have dialysis tomorrow, then get Friday off for Christmas. 

Our family is here for Christmas, and Raul actually demanded to get dressed today before his visitors came! This is big for him, as lately he has refused to get dressed at all! He is also asking more frequently to get out of the hospital after our break the other night, and we are all happy that he is looking forward to that. The plan is still to go out for about four hours on Christmas Day, which I think he will love! He is quite deconditioned, so he will need to rest a lot, but just getting out of the hospital is such a nice change of scenery.

Day +77

Tonight we got the special treat of being able to take Raul out on a pass for about an hour and a half! Santa was making an appearance at the Ronald McDonald House, so we got permission to take Raul over to see him. Raul was talking about it all day, though wasn't too happy when we woke him up to change him and go. Once he realized we were on the move without the IV pole he cheered up again, though! He was quite talkative riding in the car looking at vehicles and lights. He hasn't been in the car in quite a long time!

We made it to the RMH just in time to see Santa before he left! Raul talked to him a bit, and told him what he wanted for Christmas. Santa was very nice and took plenty of time with him. Then Raul briefly got to see Gabe, a boy just a little older than him with EB. Raul was fading, and asked to go up to the room there to rest a bit. He was so comfy on the big bed and it was perfect for snuggling! He really wanted to sleep there, and said he didn't want to go back to the hospital, but we told him that once he got a bit better/stronger he would be able to go there more often. 

 

Then we headed out to look at Christmas lights for a few minutes before going back to the hospital. Even though it was a short break, it definitely gave us all a boost to be out of the hospital together! We are hoping to get out again on Christmas, and I think Raul will do great with a longer break as long as he has a spot to rest. 

Days +72-+76

This morning we put on Christmas music and did some more decorating for Christmas; our room is now very festive! Raul is still not wanting to sit up or get out of bed, but he is more talkative, which is great! And we even got some snowplow pajamas and Santa socks on him today (he has been rejecting clothes lately). Yesterday, while my mom stayed with Raul, I got to go back to the Ronald McDonald House to "shop" for Raul's Christmas presents. I think he will be very happy on Christmas! I also got to spend some time with an EB family who has just arrived for bone marrow transplant. They will hopefully be admitted for transplant later this week.

Medically, things are more or less the same. Still having mysterious fevers, getting dialysis four days a week, needing lots of platelets/blood. The good news is he has not needed the blow by oxygen in several days now, not even while sleeping. His chest CT looked better on Friday than it had the past few times. He is also continuing to pee. Who knew wet diapers would be so exciting!

Unfortunately, he is also peeing some blood. After an ultrasound Friday they determined that he has hemorrhagic cystitis in his bladder, which is causing blood and small clots, and is most likely caused by his BK virus (his viral level has gone down though, thankfully). At this time we are just watching it, as the clots are not blocking the flow of his urine. 

We are all a bit disappointed/frustrated that we are not seeing a quicker resolution to his symptoms of aHUS/TTP with the Soliris, but we will give it a few more weeks before looking into other options. The length it takes to see a change can really vary, and it seems that we are just not on the quick end of the spectrum! There are some positive changes with his labs related to the complement levels, but we are just not seeing much change in his actual symptoms yet.

Days +68-+71

We have now been in the hospital 92 days, and are 71 days out from transplant. The past several days have mostly just been more of the same for Raul. It has now been a week and a half since he needed GCSF, which is great. He continues to have his fevers on and off each day, but his fever curve has gotten a bit better. He got a blood transfusion Saturday, then again Tuesday. Platelets on Sunday, Tuesday, and Wednesday (I think). He has not had another dose of the Soliris since last Friday, they are working on figuring out a schedule for him. He has been following his regular dialysis schedule, but the good news is that he has continued to pee more. After having not peed a measurable amount for several weeks, this is very exciting! Over the past several days I have been trying to get Raul out of bed more often. We told him he needs to practice so he can sit up to open his Christmas presents! We are also trying to get him out of the room more often to practice, as we are hoping to get a pass for a few hours on Christmas. Last night I got him to sit on the floor with me to do a craft of making homemade bouncy balls. 

 On Monday day we got him to go downstairs to check out the setup for the video chatting with Santa, though he did his chat in the room so he didn't have to wear a mask or use the stroller cover. Raul got all dressed up in his collared shirt and bow tie with Santa faces! He even sat up willingly while talking to Santa! He was very excited, and smiled when the video came into the room, but then got a little overwhelmed as there were a lot of people in the room. Today he got to go downstairs to pick out a few presents, which he was pretty excited about! We went at the end of school, and his teacher came down with us to help him pick the best trucks! 

We are very sad for Anton's family after his passing last night. I am thankful that I got one last chance to see Anton last weekend. Raul does not know, as death is not something he understands right now. But I am sure he will miss seeing Anton in the halls and looking at the pictures on his door! We met their family for the first time almost four years ago, when the boys were both two years old and had been in the U.S. less than a year. We have both been on crazy journeys that have intertwined, and given us the opportunity to see each other, several times over the years. And while at home Anton's mom, Vanessa, was always there to talk or give advice on the issues our boys have had in common!

Days +65/+66/+67

The past few days have gone pretty well! Raul's counts have come back up (ANC/WBC), and he has not needed GCSF since Monday! His platelets and hemoglobin are also improving. He is still dropping, but not as quickly as before. He had to get 25ml of blood drawn yesterday for research labs (along with his regular labs), and his hemoglobin only dropped slightly. He did get blood today though, as he is close to the transfusion threshold and they prefer to give it on a day he has dialysis. He got platelets in the evening on Thursday, and he has been holding on to them much better (his platelets were still 42 this morning and in the 30s this afternoon!). He got his second dose of the Soliris yesterday, and I am cautiously optimistic that it is helping!

Thursday Raul had quite a few fevers during the day, but they did all come down with Tylenol. From Thursday evening to Friday evening he only had ONE fever, which was amazing! Then overnight Friday he had another fever, and one or two during the day, but we are still seeing improvement in how often/how high they are. We have remained on our normal dialysis schedule. Raul's weight has been stable, but his BUN is still getting pretty high between dialysis runs. He also peed once Friday and once Saturday, which was exciting! Raul is still mostly wanting to rest, but has been a bit more perky and talkative. He even told the doctor he could borrow his Christmas blanket because the doctor said he liked it! Today he wore clothes for the first time in a long time, and we got him to go downstairs with us quickly to get a Christmas balloon. We still have some issues to work through, but it has been nice to see improvement! 

Day +64

Today was a pretty low key day, with no major changes. Raul had dialysis in the morning for four hours, then took a long nap. He interacted a bit more with the teacher, which was nice. His blood pressures have been holding up great since we came back from the PICU. So far today he has only had one fever, 103.6, and it came down with Tylenol. Overnight last night he didn't have any high fevers. We are also seeing completely normal temps. now between fevers, which we hadn't seen in a long time! He is needing the blow by oxygen on and off while sleeping, but that is likely mostly due to him not sitting up much recently. 

We learned from the med. company that the Soliris can start making a difference after even just one dose. We are cautiously optimistic that we may be seeing positive changes, as his Platelet count is higher this afternoon than it has been in a long time and his Hemoglobin barely dropped despite not getting any blood today. His next dose will be Friday. Soliris was known as the world's most expensive medication until this year, when it fell to second place. Thankfully our insurance is covering it at this time! 

Dr. Tolar stopped by this evening and said it was a very good sign that we are seeing some areas of Raul's skin look stronger; I will send him photos after his next bandage change. He sounded hopeful that we are headed in the right direction, and reminded me to be patient :)

Days +62/+63

Day +62
Raul's blood pressure remained stable throughout last night, through today's dialysis, and through today's plasmapheresis, so he was able to return to the BMT floor early this evening! It was a long day with four hours of dialysis and about two of plasmapheresis. Every time he gets plasmapheresis they prime the tubing with blood at the start, which gives him a nice boost. He also got platelets twice today, and of course the plasma.

Raul's fever curve has been a bit better, though he did still spike a higher temp. tonight (it did come down with tylenol at least). His diarrhea is still bad even without g-tube feeds, so we sent off another cdiff test, which came back negative. He is also needing the blow by oxygen again while sleeping tonight, but does okay when awake. His most recent viral test was negative for CMV, which is great news, though he still needs to continue the antiviral medication a bit longer.

Day +63
Today Raul slept in a bit, then had school and plasmapheresis. He got platelets again early in the morning, which makes the third time in about 24 hours. After plasmapheresis he actually got out of bed for about half an hour to sit by the window because there were some construction vehicles down below. Then we did his central line and PICC dressings before he took a nice nap. In the late afternoon we got him up again for PT, and he wasn't happy about it, but he did well! He seemed much happier today now that we are back in our normal room, and was a bit more talkative even.

The plan changed a bit today, and they are now putting the plasmapheresis on hold and instead going to give him the Eculizumab medication (something they say they have never used on this floor before). They are not sure if he has TTP or aHUS, but one test result that came back led them to believe the medication would be beneficial. The medication is usually used for aHUS, but even if it is TTP, there is a chance the medication would still help, there just isn't documentation on that. They cannot do both the medication and plasmapheresis at the same time as the plasmapheresis would pull out the medication. He is getting his first dose tonight, and the second will be Friday. 

aHUS is incredibly rare, even more so than TTP, I think. The doctors here are consulting with a team in Cincinnati that has more experience with these conditions, and we are waiting for his genetic testing results. It is hard not to know for sure which condition he has yet, and to have to guess a bit with the treatment, but I am hoping we will start to see some improvement! 

Days +60/+61

Unless it was some sort of complication of TTP, the doctors believe Raul had septic shock. We do not know exactly what is infected, but he is on antibiotics to cover just about everything! He had one high fever in the evening yesterday, but was as low as the 97s during the day. It is now 8pm and he only had one fever today of 101.7. These numbers are a huge improvement to his recent almost constant 104+ fevers! It sounded this morning like the doctors think the most likely antibiotic to be working is the Vanco, which is the last one we added. It is very toxic to the kidneys, but of course we need to treat whatever is infected first. 

Raul's breathing is so much better! He was requiring the high flow and still having retractions yesterday, but then overnight he was pulling off his nasal cannula himself and his numbers weren't changing so we just took it completely off! In less than 24 hours he went from needing 60% oxygen with a pressure of 12, to 30% with a pressure of 12, to room air without even blow by oxygen! His respiratory rate and heart rate are much better too.

Earlier this evening we weaned him off his Epi (down from .05 this morning) and tonight off his Vasopressin too (down from .0004)! Yesterday morning they weren't sure if we could do dialysis due to his blood pressure and were discussing either skipping it or going to continuous dialysis. Thankfully the nephrologist was willing to give it a try, and he did perfectly! They gave him blood and plasma during it, and even pulled his weight down to his goal weight (and then his weight went .6kg even lower overnight, possibly due to diarrhea). They decided to skip the plasmapheresis yesterday and just gave an infusion, but we did it today with no issue. If anything his blood pressure got better afterwards.

We got him to sit up in bed today for a bit of PT, and will try again later. Him getting mad just helped his blood pressure :) . We are hoping to be back up to the BMT floor tomorrow if all continues to go well. Most likely after dialysis and plasmapheresis just to make sure he doesn't have any problems with his blood pressure during those. We got a few visits from people from the BMT floor yesterday, which was really nice. Raul is definitely having a bit of a hard time with the change in scenery and staff. After being in the same room for so long it has been hard for him to adjust to somewhere new. I am still getting occasional smiles from him though, which is great! 

Raul asked to smile for a picture and this is the face he made haha

Day +59/+60

Our night did not go as planned, and we ended up in the ICU around 2am because Raul's blood pressure was too low. They had been trying to manage it on the BMT floor, but even with fluids and a lower dose of dopamine it was not coming up enough. 

He started getting dopamine and epinephrine in the PICU, and now they are going to switch the dopamine to something else. He is not acting any different, and actually slept well once we got down here (he had been refusing to go to bed upstairs and we had just fallen asleep when they decided to move him). 

Moving him was a project, with many people along for the walk down. They moved him very quickly after deciding he needed to go to the PICU, but I had just enough time to grab the things we would need overnight/this morning. 

We do not know exactly what is causing his low blood pressure, most likely either infection or his TTP. He is now on about half a dozen antibiotics, so if it is an infection we will hopefully see improvement in a few days. If it is related to his TTP, it is the plasma that will help. But in the meantime he is stable as he is getting the medications to keep his blood pressure in the normal range.

The doctor seemed optimistic that we could only have to be down here a few days. He can go up to the unit on a small dose of dopamine, but not the other "big gun" blood pressure medications. Raul was pretty anxious last night about switching rooms after being in one room this whole time, but was happy to get to keep his same bed! 

He is getting dialysis now, and we will try to pull some fluid as long as he tolerates it. It sounded like we may wait to do the plasmapheresis tomorrow and just give him regular fresh frozen plasma (FFP) today, but we will see.

Day +59

80 days in the hospital today. Raul got platelets last night after I did my update as his level had fallen to 18 after his nosebleed. Today was a long day, with lots of people in and out of the room. Dialysis and school in the morning. With dialysis they pulled his weight down to about 18kg, which is around where we think is dry weight is now. He will have dialysis again tomorrow and get Sunday off.

Raul was somewhat out of it today, and was harder than usual to understand (which was not helped by the loud blow by oxygen!). They checked both a blood gas and an ammonia level, which were normal. He was awake more of the day today, but not acting like himself.

We had hoped that getting extra fluid off with dialysis would help his breathing, but unfortunately he was still working harder to breathe, so we put him back on high flow oxygen to get his lungs opened up more. He does seem to be breathing more comfortably, and he has been coughing well today to get some of the junk out. They also added another new antibiotic last night.

In the afternoon he had plasmapheresis. It was interesting to watch as they separated out his plasma from his blood and replaced it with donor plasma. The idea is that the donor plasma will have what his plasma is lacking. His ADAMTS13 level was less than 5% when they tested, which is quite low. The nurse told us that his plasma was darker than normal, which is to be expected with his diagnosis (the largest bag hanging in the photos is his plasma that was removed).

It will be several weeks before we get any more of the test results back. They wanted to do the plasma exchange now instead of waiting for the genetic testing results as we did not want to miss the window for it to be effective. They are also ordering the medication we may need as it can take some time to order. He will get plasmapheresis daily until his platelets rise and can be maintained better. 

 

 

In the below photo his plasma is the brown-ish liquid in the big bag next to the monitor

 

Day +58

It looks like Raul may be getting another rare diagnosis. Last weekend they ran some enzyme tests for things that could be keeping his kidney function from improving, and his tests came back with a very low level of ADAMTS13. They are now testing to determine whether he has the acquired/secondary or inherited form of TTP. Due to how low his level was, they believe it could be the congenital form, that was just flared up by transplant. Congenital TTP has a prevalence of less than one in a million. Whether it is secondary or congenital, this is likely affecting his kidney function, as well as possibly causing other symptoms (such as hemolytic anemia, low platelet levels, and even his fevers and nose bleeds).

The good news is that there are ways to improve this condition. Until we get the genetic testing back, in about a month, we will use FFP (fresh frozen plasma). Then the genetic testing results will determine if we do plasma exchange or treat with a specific medication.

We don't know for sure, but really all of Raul's current symptoms could be related to the TTP. Yesterday and today he has slept most of the time, he had a bad nose bleed today, and his fevers are not coming down well with Tylenol. Today his fever got as high as 106, and only came down to 103.5 with Tylenol. He is pretty miserable. He did get both blood and the FFP today, so I am hoping if this is the TTP that we will see some improvement! 

Days +56/+57

Yesterday Raul's temperature was very high, and stayed quite high even with tylenol. He got a chest CT in the afternoon, which did show some differences, so his antibiotics were changed. He is still needing the blow by oxygen intermittently, especially when sleeping. Yesterday we got him on his belly during PT and his oxygen sats went to 100 without blow by, which was great! Today we got him out of bed for a little while for more PT, though he spent most of today sleeping (4 hour naps in the morning and afternoon/evening). The lady who brings by books came today too, and Raul got several new books and a new teddy bear!

The past two days his diarrhea has been awful, and we tried a few creams/ointments with no luck. Yesterday we cut his feeds to 15, and then today we stopped them completely to give his GI tract some time to heal. They also finally agreed to give him a dose of immodium today and it seems to be helping, which is great! His vomiting has been a bit better the past few days, even when he was getting more formula. His bilirubin has been elevated again this week, but thankfully they don't believe it is the VOD returning. I knew last night that his level would be high today as his wound drainage was looking yellow again!

Amazingly, today he has not had as many high fevers, and even went below 100 for the first time in four or five days! I'm hoping that the new antibiotic is helping! He got platelets this morning, and blood during dialysis. Even though he got blood yesterday too, his hemoglobin actually dropped, we think due to hemolysis. They are running a few more tests to investigate that. He also had an echo done this afternoon, but I have not yet heard the results from that. 

Raul sometimes likes to sleep with things over his face...