Saturday, March 26, 2016


It might be hard to see as the videos don't upload with good quality, but if you look closely you can see Raul smile/smirk occasionally in these two videos from last weekend!

Friday, March 25, 2016

Day +171

On Wednesday Raul had his GJ tube placed and had his 6 month post-BMT tests. During his blister test, the blister started at 15 seconds, but took over two minutes to completely blister! In the past they have all only taken around 40 seconds, so hopefully this means his skin is getting stronger! It will take a bit longer to hear about the percentage of donor cells in his skin and how much collagen 7 there is. His GJ is working well, and we are slowly increasing his feeds.

Unfortunately, the last few days have been rough, and Raul has been pretty unhappy. Along with everything else, we think he is likely just bored and frustrated, so we are trying to think of different things to do. This afternoon we took a short walk, and stood outside for a couple minutes of fresh air. He did seem calmer when we were outside. Hopefully soon we can cut down on the number of IV pumps he has to make it easier to walk around!

Raul's soluble IL2, an HLH marker, this week was about double what it was last week. It went from the mid 3,000s to low 7,000s. Some fluctuation is normal, but this was quite a bit higher. They will not officially make any conclusions based off this one result, so we will have to see what it is next week before making any big decisions. For now we went back up slightly on his steroid and will keep his CSA dose therapeutic (we were considering weaning it again). He has had a few fevers, but not constant at least! It is possible that some of his fussiness is just feeling overall crummy. 


Tuesday, March 22, 2016

Day +168

Raul's video EEG was somewhat abnormal (as they expected), but he did not have any seizures! Neurology seemed more optimistic since his MRI did not show a change in the brain shrinkage. We may potentially do another a few months down the line, but most important will be his clinical symptoms. Right now there is no permanent damage evident from the cardiac arrest on the imaging, but that doesn't mean he won't have any.

Since Saturday he has been much more alert, and been staying awake all day/sleeping all night. On Saturday he screamed once at bedtime, not at all Sunday, and only once yesterday at bedtime. Today he has been more unhappy, unfortunately, but still alert. This morning his belly was bothering him and he threw up his meds., which upset him. I am looking forward to getting a GJ back so we don't have to keep putting things in his stomach that isn't working properly!

Yesterday he had a nosebleed, which was very scary after the last one. Unfortunately, along with him being more alert, he was also scared even though we all tried to stay calm. There were a couple times where he started to choke but thankfully was able to recover on his own. We did suction his mouth and caught a couple clots, as well as drained his g-tube. It was actually a pretty short nosebleed, but felt long since we were so worried about him choking! We gave him extra platelets, did two days of Amicar, and used a little oxygen until he calmed back down. He was most mad about the fact that we had to make him sit up until we were sure it had stopped!

After much discussion, we have decided to put off the PD cath surgery for now. The BMT team has always been somewhat concerned about infection and whether he would even tolerate PD, but those risks seemed doable and worth at least trying it. However, when the surgeon came by last night he was very concerned about the surgery itself and if Raul could tolerate it. He did end up putting Raul on the schedule for tomorrow despite his reservations, but he scared us enough that we decided to wait for now.

He may be more stable in the future, and we could do the surgery then, otherwise we will do 4ish days of HD a week in Boston when we go home. Hopefully once his CSA is weaned his kidney function will improve a bit and maybe we can go to only 3 days a week of dialysis. We will still be going to the OR tomorrow morning to switch back to his GJ tube and do his 6 month post-BMT biopsies.

Saturday, March 19, 2016

Day +165

Raul's comfort level has gone up and down a bit. He was very comfortable Wednesday night/Thursday, then more uncomfortable again Friday. Today has probably been his best day yet! He only screamed once, and it was when he was going to sleep, which can be a hard transition. The rest of the day he was very alert/awake, and gave us smiles on multiple occasions! He also did really well both sitting in my lab on the chair and sitting/stretching with PT. It was so nice to see him like this! Of course he wouldn't let me get any good pictures or videos of it, but I got a couple photos where you can tell he just had finished smiling.

Last night we got a repeat unsedated brain MRI, which was not significantly different than his one from a few weeks ago. This is good news, as we could have seen increased shrinkage at this point from the cardiac arrest. Today we started a 24 hour video EEG to rule out seizures.

During dialysis today we lowered his dry weight and pulled more fluid, as they think he could have lost some weight since he has been back on TPN/not getting formula. They are hoping that as he gets less agitated he will not need as many medicines for high BP, as he had come off of them before his cardiac arrest.

I also met with surgery this morning about the PD catheter, and I think they were relieved to see how good his skin on his abdomen is. His WBC/ANC dropped the other day, but now they are coming back up, so we are still looking at next week for the surgery. We may combine it with his 6 month post-bmt tests, might have derm. place more epifix on his knee, and will probably switch back to a GJ tube as he is not tolerating anything through his g-tube. We will put in a shorter GJ tube so that it won't irritate the spot that got the GJ intussusception before.

Wednesday, March 16, 2016

Day +162

As of today we have been in the hospital six months (admitted 9/16); a little over half a year at 186 days. Crazy to think about, and yet we aren't done yet, unfortunately. But at this point another month or two doesn't even feel that long compared to how long it has already been!

Raul's soluble il2 level was the same this week, and his other HLH markers were more or less the same as well, which is good since we have started weaning the steroids!

Raul has started peeing a tiny bit again, which is very exciting! It isn't enough to make a difference in his weight or labs, but it is a start. Today I met with the PD nurse and got some information on PD and the actual supplies/machine. We are still aiming to get the PD catheter in a week or two as long as he is stable.

Starting last night Raul was a lot more uncomfortable. It seems like it is his stomach, but it is hard to say for sure. He was up every few hours over night screaming, and it was constant for a while this morning. He also started holding his breath right before he would scream, which would drop his oxygen sats, though they recovered quickly. We got another ultrasound that looked okay.

A while back the pain management/palliative care team ran some genetic tests to find which medicines would work on Raul given that we have had so many issues with side effects in the past. They just got those results back, and based on those we are starting him on a low dose ketamine drip (targeted for pain not sedation). The fentanyl, which we thought might be helping, actually showed that it should be less effective. He is apparently lacking what is needed to metabolize certain types of medications. They were still figuring out the side effect piece, as some of the medicines that have given him such bad itching show that they should be effective for his pain as a last resort.

We met with neurology again today, but they did not really have anything new to add. They said we still just need to wait and see, especially with how many pain/sedative medications he has been on. They could not give us a time frame of when we might see improvement, or a time after which improvement would be unlikely. They also said they couldn't even do a reliable neuro. exam on him at this point due to his medications, his temperature (he had a fever this afternoon), and some of his lab abnormalities. But they did say that if the smiles we saw truly were a response, as they seemed to be, that that would be a good sign!

Monday, March 14, 2016

Day +160

Today marks three weeks since Raul's cardiac arrest. Unfortunately, he has yet to regain most abilities he had before, but he has not been off his sedatives long so this could still happen. Now that we are back on the BMT floor, we do get occasional smiles for things he finds funny (like the intro. song to the show Sarah and Duck). Some of his joints have gotten a bit tighter, so we are working with PT and OT on stretching them. 

The ICU psychosis seems to have passed, and he now just gets upset when he is uncomfortable. His abdomen has seemed to be bothering him the past few days so we have stopped his gtube feeds again, and have been venting his gtube. We did get another ultrasound today just to check, but it looked okay. If the symptoms persist we will look into doing a CT scan. 

His heart rate was low for a few days, and now it is back to normal but his blood pressure has been high. Both of these are likely medicine side effects. We got an EKG the other day that was stable. Raul had dialysis today, and we are hoping to be able to stick to our four day a week schedule.

We had a care conference last Friday, and are still planning on placing the PD catheter for dialysis before going home to Boston. It is better to have it placed here where they are more comfortable with EB patients. We do not know yet if we will have to transfer hospital to hospital, or if we might be able to go home outpatient. The time frame right now should be to have the catheter placed in 1-2 weeks, and go home in 1-2 months.


Friday, March 11, 2016

Day +157

Raul was able to come back up to the BMT floor today! We had hoped to yesterday, but he was having some low heart rates so they wanted to watch that and lower his Precedex a bit to hopefully help. He is still having some low heart rates now, but his perfusion and blood pressures have been fine so they are just watching it and lowering the Precedex a bit more.

While there is still a lot going on with Raul, I thought I would post some good news tonight:
1) Raul's engraftment from the beginning of the week came back and it is considered to be stable! They were last drawn on day +99 (I think), and these were drawn on day +154. His CD3 was down from 97% to 82%, and CD15 up to 31% from 24%. They will be checked again in about 1 month for his 6 month post-bmt work up.

2) It might be hard to tell in photos, but Raul's skin has improved. In person you can tell that even areas that looked okay before, now look much more "normal". His mild chronic areas have healed or shrunken significantly. His moderate chronic areas are starting to improve, and we have not yet seen significant improvement of his worst wounds but those will take time. And new areas, which there aren't many of, heal MUCH faster! His entire chest was open after CPR, a pretty significant trauma, yet all but the area around his dialysis catheter healed within 10 days!

3) Raul remains in kidney failure, but his kidneys are still enlarged. This is good news, as once they shrink is when you know you most likely will not get function back. We have actually been seeing tiny amount of pee too, which is very exciting! We didn't believe it at first yesterday, but sure enough, there were 19g! We lowered his CSA dose the other day, which I am guessing is helping, as last time we went down on his CSA he began to pee again too.

4) And finally, what might be the biggest news, is that this morning they told us that they consider his HLH to be in remission right now! He has had several weeks of good HLH labs, and we have started weaning his steroids (which will be a fairly lengthy process since he has been on them so long). Since he did seem to respond to the Campath, we have the option of doing another course of that should his HLH activate again. 


Wednesday, March 9, 2016

Day +155

No intussusception on ultrasound today, removing his GJ tube was successful! We didn't start feeds today, but did start giving meds. through his g tube as he has some that do not have IV versions. We had been trying a dissolvable tablet of one, but his mouth is too dry and they were not actually dissolving.

Last night was another really hard night. He would sleep for 15-30 minutes at a time maximum, and then wake up agitated. It seemed to be more delirium than pain. We played around with his meds., and then he finally really fell asleep in the morning. We did try to get him up on an off during the day though as we don't want him to reverse his days and nights. 

Now that we can give g-tube meds. again, we seem to be back in a better place, and hopefully tonight will be better! He ended up being a little overly sedated during the day today and had some periods of low heart rates, so they did wean back down a little and we are going to try all non-medication things we can for his agitation tonight too.

Raul's weight was stable and we were able to skip dialysis today. He will have a dialysis run tomorrow, and if his blood pressure doesn't drop too much he will hopefully be able to go back to the BMT floor. They also were hoping for him to have at least one good night and have his medication regimen worked out before he goes back up.

The other good news from today was that his HLH markers are still improved! This makes two or three weeks in a row now, so it seems promising. We are going to start weaning his steroids, and after that go back to weaning his CSA. For now we are going to keep his CSA level lower, which will hopefully make his kidneys happy!

Tuesday, March 8, 2016

Day +153/+154

We have had a crazy few days! On Sunday night Raul didn't sleep, literally. He was agitated the whole night despite MANY medications. He finally fell asleep around 7/8am. So we decided later that morning to do a planned move to the ICU for a day to start him on precedex and then start weaning some of the other sedatives he was on. We thought it was best to do it in a planned way rather than in the middle of the night while he was freaking out if we had another bad night.

We have trouble getting accurate blood pressures while he is agitated as he stiffens his body, so that morning he got medicine for a high blood pressure that might not really have been quite as high. Then he calmed down, and we started dialysis, and his blood pressure dropped a lot. He was able to get a blood transfusion and run for three hours with the aide of Dopamine, but they were not really able to pull any fluid without lowering his BP too much. We finished dialysis before heading to the PICU, but it seemed good that we had already had that planned! The precedex he was starting can lower blood pressure, so we waited until later in the evening to start that.

When we got down here they put him on high flow oxygen instead of the mask to help him breathe a bit easier (he is back on the mask today), and did a chest xray and EKG that looked okay. He had some labs that might have been concerning for infection and/or increased oxygen needs, but those improved later in the day. He stayed on the dopamine for his blood pressure until around midnight, and has been off it since. His blood pressure dropped a bit during dialysis today too so they only pulled a little fluid and again just did three hours. He did not receive any blood pressure medications today so we are hoping that will level out a bit.

Last night he started having blood in his stool around 9pm. This had not been happening the past few days, so they ran a few tests. First they checked his G and J tubes for blood, but they only showed a bit of old brown blood. Nothing fresh, and not a lot. Then they did abdominal x-rays, which didn't really show anything. He had several diapers with blood, so then they also did an abdominal ultrasound. There they found a little small bowel intussusception as well as a small intussusception at his J tube. Surgery was consulted, but at that time just recommended putting his g tube to suction and venting his J tube, and discussed switching out the tube.

He had a repeat ultrasound at 7am that did not show the small bowel intussusception, but did still show the GJ tube one. So at rounds we decided to pull the GJ tube (which they did bedside) and replace with a regular G tube. This should get rid of the intussusception there as there will be nothing for the intestine to telescope around. It could reoccur if we replaced the GJ tube, so we are hoping he will just tolerate G feedings. GJ tube intussusception is quite common, and is a risk the GI doctor had discussed with us before we switched to the GJ tube a few months ago. Hopefully now his belly has had more time to heal since transplant and he will tolerate formula through the regular g-tube.

He had bloody stool through the night to varying degrees, which GI thought might be a lot for only a small intussusception so they were concerned about a Meckel's diverticulum. If he had this, it would need to be fixed surgically, which would be risky for him due to his EB and long term steroid use causing healing problems. This afternoon he had a Meckel scan done, and thankfully it was negative! We don't know for sure where the blood was from, but it isn't too unusual for EB patients to have some bleeding at times. He has not had blood in his diaper today, so we are hoping that has resolved. If his diapers continue to be okay and he stays otherwise stable, we are hoping to go back to the BMT floor tomorrow!

We have now accomplished what we originally planned to come down for and then some! Thankfully the precedex does seem to be helping, as he slept well last night and through most of the day today. He has a lot of missed sleep to make up for! When he is awake he has been looking pretty alert, even with all the sedatives, which is good. 

Sunday, March 6, 2016

Day +152

Yesterday Raul had a pretty good day. He got fussy during dialysis, but just on and off. In the evening he had a CT scan of his lungs, which was just a regular follow up based on the ones he has had before. It actually looked a bit better, which was good news! He managed to sleep through it, and sleep right through the night! 

Raul had a good morning today, and even sat in my lap on the chair for a while. After that he was tired out and took a good nap. Since his nap he has been a little fussy, but he enjoyed going on a stroller ride for a while. He also had a short period of seeming quite connected and interactive when we were joking around. I was pretending to eat one of his toys and he was smiling, then I put it up to his mouth and he opened it like he was pretending too. 

We did his bandages this evening as well, which went pretty well. Tonight he did have a bit of a freak out at his usual time, but I would say the worst part only lasted about half an hour. Right now he is awake but quieter. I am hoping that once he falls asleep we will have an okay night!


Friday, March 4, 2016

Day +150

Last night wasn't too bad, though still a little freaking out in the evening and a couple short ones overnight. He was pretty sleepy today, but did see his teacher and had dialysis. We stopped his dialysis at 3 hours instead of doing the full four as we thought he might be having belly cramping and a few other issues from pulling a lot of fluid. He did calm down after dialysis, which was good. 

In other medical news, we stopped two of his antibiotics today and may stop the last one tomorrow if he stays fever free. We are slowing working on weaning some of his pain/sedative medications. We weaned the fentanyl drip a little yesterday, and today weaned his ativan dose a little (but left it on the same schedule for now). It is hard with the delirium/agitation, because the medications help the symptoms, but at times can actually cause the confusion to last longer. 

The neurologist came by this morning and said basically the same thing the BMT team told us yesterday about the MRI. He did say the shrinkage is not surprising, and that he would suspect that possibly half the patients on our floor would have some due to how sick they are and the medications they are on. And nephrology said the same thing about their patients, so it sounds like there was little chance he WOULDN'T have that issue. He agreed that we will just have to follow how he does clinically and wait and see. He did say again that the good news is that due to the lack of focal damage, at this time there are no functions that we know for sure cannot recover. 

The best part of our day was in the late afternoon, as Raul seemed much more interactive and alert! He actually seemed to be smiling in response to what we were saying when we were trying to tell jokes and make him laugh. He also was intentionally opening his mouth for his mint spray when I showed it to him/asked if he wanted some. And he would open it again if I asked if he wanted more. Of course when I tried to get a photo or video of either of these things he would stop; silly boy!


Thursday, March 3, 2016

Day +149

Last night Raul only had a brief freak out, and then slept pretty well through the night after watching his iPad for a while. He slept in this morning, and then had a pretty good day. Not too much going on for him today other than a bandage change and changing the dressings on all three of his lines. His teacher read to him again, got back in the chair for a while with PT, and tonight the music therapist sang to him.

As far as the MRI results, we are pretty much back in a wait and see mode. The results were essentially the best we could have hoped for! No focal damage anywhere, just the overall shrinkage that had already been seen on the CT scan last week. This may not even be related to his cardiac arrest, as that would be quite quick for it to show up.

This does not mean that he will not end up having neurological defecits, but means that we can be optimistic for at least some improvement and that there are currently no areas that we know of that have no chance of recovering. So now we just need to give him time to show us what he can do!

We have been told that, despite the fact that most of the meds. are processed through the liver, kidney patients take much longer to get the sedatives out of their system. Raul is still pretty well sedated, and is also still having some of the ICU psychosis/delirium, both of which will improve with time. We are seeing small signs of improvement here and there.

The other good news is that all his HLH markers were improved this week! We will have to continue following them and see if they stay down, as with the Etoposide they improved briefly and then worsened again. But right now they are hopeful that the Campath is helping! His Soluble IL2 went from the 27 thousand range last week to the 3 thousands this week!

Below is one of his first small smiles (after a yawn) since his cardiac arrest:

Wednesday, March 2, 2016

Day +148

Raul had a pretty good day today! Last night he only had the one short freak out, and then slept through the night without even moaning in his sleep. He actually slept through quite a bit of the morning too making up for the night before. And during the times he was awake today he was much more comfortable and content, barely any moaning or agitation. 

Raul had dialysis this morning, and then late this afternoon he was a rockstar and did an unsedated brain MRI! They only had to repeat one or two of the sequences for movement, which was great. We will hopefully get those results tomorrow. We feel that there are certain angles and things that he does see okay. Tonight he has been watching his iPad consistently as long as it is in a certain spot. Assuming he really is watching it, he must be so happy that we have figured this one out! As of now he has yet to have an evening freak out tonight!

Tuesday, March 1, 2016

Day +147

Last night was one of our hardest nights. Because of the delirium, Raul was inconsolable for seven hours before going to sleep. He would slow down on and off, and we would think he was asleep, but then he would start right back up. When he is very agitated he breathes very quickly, which was a bit concerning, but once he finally did get to sleep that improved as it had the night before. We did check a new chest x-ray, blood gases, and a nurse stayed in the room through the night just to make sure he was okay. 

Once he went to sleep he slept okay, but was still moaning quite a bit even in his sleep. Morning came quickly, but thankfully during the day he is much less agitated. He got to see some familiar faces, read stories with his teacher, and stretch with PT. We also got him up into a tumbleform chair for several hours, and he seemed more comfortable there than he had in days! He even napped a little.

We met with several doctors today, but I don't have much to report as we just need to wait a bit longer to see how things improve. He likely does have some brain damage from the cardiac arrest, but we do not know to what extent at this time because he also has some withdrawl and the ICU delirium. Based on some of the neurology exams he may have problems with his vision, but he may not as other times he does seem to track with his eyes. Thankfully they did not think he was having seizures. We will do a brain MRI once he is stable enough to be sedated for it.

So far tonight has been much better. At the same time as last night he did start freaking out again, but we were able to calm him fairly quickly and he has only been up a couple times since then. He had a high fever when the agitation started tonight, so we put ice packs on him which seemed to both help his fever and calm him. Watching him go through the delirium is terrible, and I'm sure even more terrible for him, so we are hoping this is the start of that improving!