Day +146

After another long day of waiting around, we were able to move back up to the BMT floor at around 5:30! Raul is having a very hard time with what we think might be a combination of ICU delirium and withdrawl from his sedation medications. It is worst in the evening, and very hard to watch. He is getting medications, but sometimes they take a long time to work or don't work at all. 

Raul did get to have story time with his teacher this morning, which he seemed to like. And he actually also seemed to like his PT today; the stretching looked like it felt good. He slides himself down when we try to prop him up in bed, so we got a tumbleform chair to use with him until he is able to sit up on his own again. 

Some of his HLH labs were actually improved today, though the soluble IL2 won't be back until Wednesday. He did have a fever of 101.8 tonight, but we don't know the cause. It certainly didn't help his agitation! His BUN/Creatinine are a bit improved, and the nephrologist is hopeful that maybe his kidneys are starting to wake up a little.

We are really hoping that this delirium/withdrawl won't last too much longer!

Day +145

We had a long day waiting for it, but Raul was finally extubated around 6pm! Early this morning he passed a test on the vent with just pressure support, and then at about 11am we turned him to just pressure support for the rest of the day before he was extubated. 

Yesterday they had said morning extubation, then we had rounds mid-morning and they said early afternoon, but then it took him much longer than expected to be awake enough for it. He had his eyes open all day starting from about 8am, and he was moving, but he was not following commands enough to satisfy them until the evening. 

Unfortunately, we are now dealing with delirium from him being sedated for the past week. It is terrible to watch. He is inconsolable and in his own little world. We are going to give melatonin (part of the issue is the lack of night/day and sleep cycle), and then they are discussing another medication that might help. They said in the long run giving him more benzos now would not help. He has been moaning and writhing for hours, and is finally calming down a little.

Day +144

The continuous dialysis was stopped around 10pm last night as the circuit was starting to need to be replaced and they thought they had gotten enough fluid off. He got a little fever once they took him off that, and since then has had a good temp. It looks like it was the continuous dialysis making him so cold before. Today he got four hours of regular dialysis, and will have his next run Monday. Thankfully he had no blood pressure issues or anything during dialysis.

Our dialysis excitement today was that the drain in the wall wasn't working and we flooded the room next to us and out into the hall! Luckily no one was using that room! They had to disconnect it and drain into the toilet for the rest of the time. So even though our room was dry, maintenance made them move us to a different room so that they could make sure the wall dried out properly.

Last night they also weaned the peep on his vent to 8, and then today down to 6. His lung looked much better on this morning's xray, so the current plan is to try to extubate him tomorrow morning as long as things continue to go well!

We are increasing his formula every 6 hours, and are at 13 ml/hr right now. We stopped his TPN/lipids after dialysis to lower his fluid intake since he will not be getting dialysis tomorrow and we want him in a good place to try to extubate him.

Raul seemed more sleepy today, but we think that might be because before he was on the continuous dialysis which would have been dialyzing off some of his sedatives. Tonight though he had his eyes open for a while and actually seemed a bit more aware even with the heavy sedation. We are not going to wean his sedation until right before they try to extubate him (they are short acting meds.). 

 

 

Day +143

Last night we were able to wean Raul's oxygen back to 25%. Today his lung looked better on x-ray, so we decreased his PEEP back down to 9. We attempted to get an EKG, but were not very successful. Last night we started TPN/Lipids, and today started formula through his J tube at just 3 ml/hr. 

We started him on a new antibiotic as the bacteria from his ankle was resistant to what he had been on. He was also started on a medication to help him produce his own platelets, which will hopefully help keep his level higher and keep him from requiring so many transfusions. His current parameter is 50, and he only got platelets once in the past 24 hours, which is good! 

His BUN/Creatinine have been happy with the continuous dialysis, and are within normal ranges. Today he did not look puffy, so they slowed down how much fluid they were pulling. The current plan is to stop the continuous dialysis tomorrow, and go back to intermittent hemodialysis on Monday. His liver enzymes were improved today from the last check as well.

Day +142

Today was a quieter day thankfully! They did have to increase his PEEP on his vent back from 8 to 10 due to his collapsed left lung, and had to increase his oxygen a little as it sometimes drops a bit when he wakes up more and gets agitated. Neither were major changes though, and we should be able to wean again tomorrow. We have been using the warming blanket as his temperature has been a little low on and off, but the dialysis seems to lower it a little too. 

Raul opened his eyes a lot today, which was so nice to see! He is still heavily sedated, so he wasn't really "awake", but it still made me happy to see. We got the official CT results and there were no bleeds. He did have a little overall shrinkage, but not necessarily even related to this event.

Later on when he is more stable we will do an MRI to check for any specific damage, and mostly we will just see how he does once we wean his vent and sedation. At this point we really don't know what the level of damage will be. Things could be the same, things could be substantially different, or most likely somewhere in between. 

We have gotten around 1.5 liters of fluid off with the dialysis so far, and he is looking so much less puffy already! Yesterday he was unbelievably puffy! Hopefully he will only need another day or two of the continuous dialysis. Other than the dialysis, today was just a low key day to let Raul rest and let his body heal.

Day +141

Raul had a good night last night, and a busy day today. Last night they were able to wean his Epi fully off, wean his oxygen down to 30%, and wean the PEEP on his vent from 10 to 8. His x-ray this morning did show that his upper left lung had collapsed, likely because his breathing tube was initially misplaced a bit to the right side. This will improve over time. He also has some elevated liver enzymes related to his cardiac arrest and resuscitation, but they actually said they imagined they would be even higher.

This morning he had a head CT at about 8:30. It took 5 people to get him down there, and we briefly had to stop in the PICU hallway while they decided if his heart rate was too low (it ended up being fine). Switching him over to the CT machine and back to his bed was quite a project too! We have not heard the official radiology read of the results yet, but the regular doctors did not see any major bleeds on the CT. The CT does not tell us as much in terms of possible damage, so eventually he will likely get an MRI too once he is a bit more stable.

When we came back up he got pretty agitated about all the movement and activity, so needed some extra sedation and oxygen (but we have since weaned back down). We got him comfortable again, but then had to go down a few hours later to have his extra PICC line placed. This was done in the OR, so anesthesia transported him and gave him extra sedation before leaving the room. He was only gone about an hour, and they were able to find a good vein to use in his other arm. 

He had to get a second line placed as we had been using his dialysis cather since we came down but now he needed dialysis. We have gone to continuous dialysis for a few days as he is just so swollen and fragile enough that we could not pull the fluid all at once with regular dialysis.  Hopefully in a few days we will get down to his dry weight and then we can restart with our regular schedule. The continuous dialysis can only be done while he is sedated, so if for some reason he is ready to be extubated sooner they will switch back his dialysis sooner. With the continuous dialysis we now have to have two nurses in the room at all times. His regular nurse, along with a separate nurse to manage the dialysis machine.

In terms of his HLH they are seeing clinical signs that are promising. Today he got his last dose of Campath, but it could take a few weeks before we really know if it is working or not. His soluble IL2 from Monday was super high, about 27k, but they are still reassured by his clinical presentation. His labs next week will likely be off in relation to his cardiac arrest, so it may take longer for us to see a change. 

Day +139/+140

Day +139
We are currently in the PICU, and Raul is intubated. This afternoon he stopped breathing after choking on a blood clot from a nose bleed, and went into cardiac arrest. They did CPR for about six minutes (with four rounds of Epi), but believe he was without oxygen for at least some time. He did not respond to being bagged, likely because his airway was blocked by the clot. And they had a hard time intubating him because of all the blood from his nose. It was horrible to watch everything he had to go through, and I am thankful for his sake that he wasn't conscious for it. 

Once he was intubated they moved him to the PICU, and continued to stabilize him there. They placed an arterial line to monitor his BP. At first he was not sedated, but pretty quickly they had to do bolus doses of sedatives, and then switch to continuous drips. He is now also getting a paralytic as he was fighting the vent too much and getting agitated. He has not opened his eyes since coding, but he has moved all his limbs while more awake. But for now they want him to rest and let the vent do its job. We did have to adjust his breathing tube once, as his oxygen sats were dropping, and we found that his breathing tube was a little too far down so his left lung was not inflating properly. 

They were considering doing CT scans tonight, but held off as his BP has been a bit low and they didn't want to have to transport him to the CT room unless they really had to. His blood gasses have gotten to be surprisingly good, and they have started to wean the oxygen on his vent slightly. Because he was already in Boston for work, my dad was able to make a flight out here tonight, and my mom will be coming first thing in the morning. Over the next few days we will get more of an idea of how he will recover.

Day +140
Raul had a good day. His BP was much more stable during the day than it was overnight last night. We have been weaning his Epi, and will hopefully wean it the rest of the way off tonight. We have weaned the oxygen on his vent from 100% to 30%, and will continue to wean towards room air as he tolerates it. The rate has also been dropped to 18 and he is taking his own breaths over that. 

He has been kept sedated so far, though occasionally does wake a bit and move around. He was especially unhappy when dermatology came to place more EpiFix on his knee and kicked his legs a bit. He will also put both arms up like he wants to fight when he gets messed with. He is continuing with his spunk even while sedated! He is getting a Fentanyl drip with his sedation, and is likely having some pain so he gets bumps of it when it seems like he might be in pain. Unfortunately, while it is to be expected, he did have quite a bit of skin loss from his chest as well as "at least one" broken rib due to the CPR. We will continue to give him pain medicine as he needs it to make sure he is comfortable with these issues. 

Tomorrow morning he is having a head CT first thing, and then a little later will be getting another PICC line placed as we have been using his dialysis catheter and that will not be possible tomorrow as he needs dialysis. We will most likely be starting continuous dialysis to be more gentle on his body while he is still intubated. He has gotten VERY puffy due to all the fluids he needed yesterday!

In terms of his labs and blood gasses, Dr. Tolar said this morning that he is basically improving as quickly as he can. Of course he still has a ways to go, but he is making good progress and they were pleased. Hopefully the scan tomorrow will give us more insight into any deficits there may be due to the loss of oxygen. But he is moving all his limbs and has a cough reflex, which are both good things!

Day +137

Yesterday was a really hard day. Raul was totally miserable all day, and nothing really seemed to help, which was hard to watch. There was a lot of screaming, moaning, itching, pain, and just acting out of sorts. We gave him all his itch and pain medications, and also ativan, but nothing really helped. Thankfully he perked up a little in the evening, and then after more fentanyl and ativan, slept pretty well.

Today was much better. He was still tired and complaining of pain, but otherwise acting pretty normal. We even got him to sit up for OT this morning, and he was back to wanting to eat/drink all day! He built a building with suction cup toys with OT, and said he was going to be an architect like his Hapa and help make the plans. He took a good nap this afternoon during dialysis, which seemed much needed after yesterday! It was reassuring that his behavior was more normal today, but we will watch that as well as his headaches to see if we need to do any further imaging/testing related to his HLH.

Luckily he has not had major issues from the Campath so far. Last night he got a bit more gaggy during it, and his fever went up a little. They had warned that he might have high fevers, but I think his highest so far has been 101.5 He does get tylenol, benadryl, and decadron as pre-meds before the Campath. Tonight he slept for the last hour of dialysis, then woke up for a little bit, but went back to sleep at the beginning of his infusion and slept soundly through it.

Day +136

This week we have been able to go back to doing dialysis four days a week, which is nice! His BUN/Creatinine are getting high, but his weight has been pretty stable since we cut back on some of his fluids. On Tuesday Raul had his scopes of his nose and his esophagus. His nose looked okay, and his esophagus just had some inflammation, but no strictures! He also had a product called Epifix placed on his knee with exposed bone to try to help that wound heal up. It will be replaced weekly in his room, they just did it in the OR this time because there was time and they didn't want to make him more anxious while awake.

Unfortunately, it was decided this week that Raul's HLH treatment was not working, and we will move to a second line treatment. They waited until his soluble IL2 level came back Wednesday before deciding for sure, although we all knew it would be worse since all his other labs and symptoms were. His soluble IL2 is up to 11,200; last week it was 4,600 and a month ago before beginning treatment it was 5,700. He is back to being more tired, more jaundiced, having occasional fevers, needing some blow by oxygen, etc. It is also possible he is having some neurological symptoms as he has been more irritable and for the past few days when eating his yogurt he doesn't usually remember to swallow until I remind him to.

So either today or Monday we will begin an antibody medication called Campath, we are just waiting to hear if it arrived in time to be given today. He will only have five days of this, but it could take a few weeks to know if it is helping or not. The most expected side effect is fevers. We are also looking into a trial drug back home at Dana Farber, but him being on dialysis will likely disqualify him from the trial. He will not be allowed out on passes while getting it as it will lower his counts even more and we don't know how he will react to it, so last night he got to go on a pass to the Ronald McDonald House for bingo. He wasn't feeling great, but he did like getting out, and especially liked riding the shuttle!

Day +132

Since late last week Raul has not been feeling quite as well, but he still managed to have a good weekend with his grandparents here. On Thursday and Friday evenings we took short trips to the Ronald McDonald House. Then Saturday we went to the fire museum, and Raul got to check out all the old fire trucks and equipment. Saturday we went to the aquarium, where Raul's favorite animals seemed to be the alligators! 

Raul's weight has been staying much more stable lately, but his BUN/creatinine still get very high in between dialysis runs. He has been getting platelets daily, but went four days in between getting blood. Tomorrow he is scheduled for a possible esophogeal dilatation and a scope of his nose with ENT, so today he will need to get several platelet transfusions in preparation. 

Unfortunately, Raul's HLH-related labs are still not good this week. His Ferritin was down a little from last week, but still higher than when he started treatment. His LDH, Fibrinogen, and Triglycerides were all higher. We should get his soluble IL2 results back on Wednesday, and then will decide if we need to move to the next line treatments. There are several possibilities for treatments if the current regimen is not working.

Day +129

Today marks 150 days in the hospital; just a few days shy of 5 months. Late last week through early this week Raul was acting almost like his normal self, but the past few days he has started to feel worse again. More tired, more pain, and just overall more crummy. We did make it to the Ronald McDonald House on passes the past two evenings, but he didn't want to stay too long.

This week he got to skip dialysis Thursday, and will skip Sunday too. We are hoping next week that he might be able to skip three days. He is eating a ton of yogurt again, so we may be able to cut back a little on his formula, which would be good, and would help his weight not get quite so high between dialysis runs.

Today Raul got his 7th dose of Etoposide (5th week's dose). Unfortunately, on Wednesday we got his soluble IL2 level back and it was elevated. Before treatment it was about 5,700, then last week it was down to 1,800 (I think), and this week back up to about 4,600. For now we are staying the course, and the labs will be checked again at the start of next week. It is possible that we will need to go to the next line of treatments if his HLH does not go back to responding to the current treatment. Raul had a couple fevers this week as well, which may have been HLH related. 

 

This week in PT he had fun trying out a few different "vehicles". He says his favorite was the ride on tractor, though he was the quickest on the little scooter since he could push himself with his arms. When he used the scooter he got to race his PT and nurse! He didn't love the bike, but I think if we could find a better way to hold his feet down he could do it himself.

Day +125

Despite everything going on, Raul has been feeling better and acting much more like himself recently. He has been sitting up all day, in and out bed, and wanting to go on stroller rides every day. We have been exploring the hospital, and also going outside to play in the snow. Raul also started eating yogurt again yesterday, and is wanting to eat it constantly! (probably because of the steroids)

Raul needed dialysis daily for 8 days, and finally got a day off yesterday (but we had to seriously restrict his fluids over the weekend to get that day off). At first his BUN was okay, but his weight was getting super high. Now his weight is a bit better, but his BUN is getting quite high again. So we are hoping not to have to do every day this week, but we will see. It may be that the chemo for the HLH is worsening his kidneys again, but there was not an alternative medicine that could be given in any case. 

We got some of Raul's HLH-related labs back and unfortunately some have gotten elevated again, but in talking to the doctors it sounds like some up and down is normal. His Ferritin is up to 21k, and his triglycerides are back up to the 500s. However, his LDH and Fibrinogen are both still improving, which is good. We hope to get his soluble IL2 level back mid-week. He is now only getting the chemo once a week, which is good, as after the last dose his counts dropped significantly, so we are hoping this will improve when he is not getting the chemo as often. He is now needing GCSF daily for his low counts, and he has gotten some mucositis again. 

Today we had a care conference to go over longer term plans as well as discuss transitioning home. He will have scopes done soon by ENT to check his nose after the last bad bleed, and GI to check for any strictures as he is starting to have some early signs that he may have one. We will also repeat his chest CT later this month, though he is not symptomatic with any respiratory issues at this point.

We are also planning to switch from hemodialysis to peritoneal dialysis before going home. As of today, the plan is to work towards having the new catheter placed in about three weeks. After that we have to wait several weeks before we can actually use the catheter, so he will continue with HD during that time and I will get training on doing the PD. PD is done every night at home, and would save us from having to spend long days in an outpatient dialysis center at home. He would instead be able to spend his days doing normal kid things, and just have dialysis for about 12 hours each night.

As of now, Raul will stay inpatient at least through getting the PD catheter placed. Then for the weeks following that he could be inpatient or outpatient, before needing to be inpatient for a couple nights when we first start doing PD. He is not quite ready to be outpatient yet, as he is still having some fevers, and still requiring many blood products. The plan is to try being outpatient here first, probably for a couple weeks, and then hopefully transfer home as an outpatient. We are hoping to avoid a hospital to hospital transfer at this point. 

So while there is a lot going on, the best news is that Raul is feeling much more like himself!

Day +120

Raul's HLH-related labs were checked this week and are heading in the right direction! His Ferritin is down from about 17k to about 13k. Triglycerides down from the 500s to 300s. And his Soluble IL2 is back within normal range! Now we are hoping to see his Hemoglobin and platelets rebound, as he is still needing many transfusions of those. He has not had a fever in almost a week!

Unfortunately, he has been needing dialysis daily since Saturday. We will be trying to find fluid somewhere that we can cut down on as far as his intake goes. Daily dialysis is not ideal, especially now that we are now getting closer to being discharged again! We will be having a meeting on Monday to go over plans for longer term care and plans for getting back to Boston. If he keeps doing well we should not have to stay inpatient for the full 8 weeks of initial HLH treatment.

Raul has been doing really well with sitting up lately; he has actually sat up most of the day the past couple days. He had a great week of school this week, too. This afternoon Raul was talking about wanting to throw snowballs, so tonight after dialysis we went outside for a couple minutes to see the snow! He ended up not wanting to touch it himself, but laughed about having snowballs thrown at his stroller!  

Day +118

We had a bit of a scary weekend, but Raul is back to his baseline now! On Saturday when he came off dialysis his blood pressure started getting quite high. We gave a medication to lower it, but it still stayed high. Then he got a bad nosebleed, which hasn't happened in a while, but isn't totally abnormal for him. It bled quite heavily for an hour, despite our attempts to stop it, and he had started to throw up clots of blood that had gone into his stomach. He was acting a bit unusual, freaking out more than usual about the nosebleed and breathing differently. Suddenly he tried to throw up/cough up a clot that he could not get out, and it blocked his airway, causing him to turn blue. He could not get it up, so a code was called since he was not able to breathe. They came amazingly quickly, and he did manage to either get it out or swallow it back down. But then he was still not responding to the doctors, and his eyes were rolling around. They checked some labs and his bicarb and blood PH were very off. They also checked an EKG and chest x-ray, which luckily were okay (we thought he might have aspirated the clot into his lungs).

It took two boluses of sodium bicarb, but he actually started acting more normal pretty quickly. He was sent down to the PICU for one night so that he could be monitored more closely. Down there he also got two bags of platelets and one of blood. After a nap, he was back to acting like himself. That night he had a couple issues with high blood pressure and low heart rate, but they resolved. We do not know exactly what caused his issues with his blood chemistry/blood gas, but think it may have been a combination of pulling a lot of fluid with dialysis and his chemo from the day before, then complicated by the bad nosebleed. It was a perfect storm of issues, that caused a more serious episode, but he thankfully recovered quickly. We are back up on the BMT floor, and he is doing well!