Thursday, April 28, 2016

Day +205

Today was Super Hero Day at the hospital (and also mom's birthday)! It was fun to see all the staff dressed up, and we took Raul down to the super hero party in the afternoon. It was a little overwhelming, but he didn't get upset. We get to see our super hero every day, but it was fun to have a special event to go to today!

Monday, April 25, 2016

Day +203

Today Raul got his third dose of Ruxolitinib; he only gets it on dialysis days. We drew his normal Sunday/Monday labs, but the IL2 and the cytokines take a few days to get results. His other inflammatory markers were still incredibly high, but we would not expect to see a change in his labs if the medicine is successful for about a week. So far we have not seen the even bigger drop in platelets that we thought we might with this medication, which is good. He has just gotten them once a day the past few days. We were able to go outside Saturday without Raul's IV pole! Unfortunately now it is supposed to rain for a week, but we are looking forward to getting back out.

I would say Raul's fevers are happening less frequently, though still daily. Unfortunately, he has now had his second positive blood culture from his PICC line. The first grew staph, and he has been getting vanco for that, so we added Linezolid now too. We have also started getting the reports back from where a spot on his head was biopsied, and so far it has several types of bacteria. He is already on so many antibiotics, that it doesn't really sound like we will do much different for it. Raul did also have another episode of severe bleeding from his arm, about 200ml, so they are re-checking one of his clotting factors to make sure it is okay. We noticed it a bit quicker this time, I think, and we held pressure and put on an absorbable hemostat layer under his bandage.

We began weaning Raul's CSA a couple weeks ago, and should be done on 5/13. This is the immunosuppressant he has been on since transplant. We first started weaning it around day +100, but then he was diagnosed with HLH so they put it back on for a while. It will be nice to get him off that so he has a better immune system!

Neurology was supposed to come by last week, but ended up only stopping by the doctors' room as they did not have anything to add at this point. He is still having fevers and still on a fentantyl drip, so he couldn't be fully assessed. Despite his fairly normal MRI, he is currently presenting as being severely affected by his cardiac arrest. We are happy though that he is no longer miserable and agitated! He has also been more alert since we have been weaning the sedation. He should be off his remaining sedative drip by the end of the week. There is still a chance of improvement, and we are hoping we will start getting smiles again soon if the new medication helps him feel better overall!

Due to some of his labs and symptoms endocrinology had recommended the team check Raul's thyroid. They expected the level to be high, but it actually came back slightly low. They will give him just a small dose of a medication to help this. Raul has also been having some GI trouble, which may have contributed to some dehydration, but since putting him back on scheduled immodium and adding fiber back to his formula that has started to improve.

Friday, April 22, 2016

Day +199

I have been pretty sick this week, so my mom spent the past couple nights at the hospital with Raul so that I could recuperate. I guess I have been lucky to make it 7 months without catching anything, but I got hit pretty hard this time. Thankfully after some rest, and now some antibiotics for my sinuses, I am starting to feel better!

A lot of this week was spent getting approval for Raul's new medication, Ruxolitinib, and then actually acquiring it from the company. It arrived yesterday, but then we had to wait to give it today as it is to be given on dialysis days. He also had to get some extra lab work and an EKG as part of the protocol for this medication. Earlier in the week we got back the results for his cytokines, and two were high, so they will monitor those to see if the medicine is helping. The medicine is a chemotherapy, but a long term one, so if it helps he would continue taking it. We had to apply to the company for compassionate use, as we are using it for an off label diagnosis. His diagnosis is still not straightforward, it may be HLH or it may be something similar.

As Dr. Tolar said today, nothing is straightforward with Raul, "his EB is a little different, his VOD was a little different, his HLH is a little different", etc. We are discussing the possibility of whole exome sequencing to look for genetic causes for his issues, as we did not find an HLH specific mutation. Raul did have unexplained fevers and inflammation on and off over the past several years, so it is possible he had this inflammatory condition before transplant.

Today Raul also got two punch biopsies and cultures of a spot behind his head. The doctors weren't too concerned, but it has been looking really off, and no one knew what it was so we wanted to make sure there wasn't anything else we should be treating! As always, he was tough, and it really didn't seem to bother him. We have also been having issues with wounds bleeding through his bandages here and there, but nothing overly concerning since the big bleed from his arm last week.

Raul is almost off his sedation! His Ketamine will be turned off tomorrow, though he will still have lots of PRN doses available if he needs them. His Fentanyl has to be weaned more slowly to avoid withdrawl, but we are making good progress there too. He has definitely been more alert the past few days, and we are hoping that once he starts to feel better he will show us some smiles again!

A few days ago Raul had one positive blood culture from one of the lumens on his PICC line, so got a dose of Vanco. We also narrowly avoided having to have his dialysis line replaced! The regular clamp broke off last Saturday, so they put something else on instead. It worked okay for dialysis Monday, but by Monday night all the TPA was gone from the line and replaced with air. They were concerned that it might be cracked so put him on the OR schedule for Tuesday, and used a big blue clamp overnight. The dialysis nurse came to check it Tuesday morning and thankfully agreed it was a clamp problem and put a second one to make sure it stays tight, so the team was able to cancel his OR slot! We are still aiming to switch to PD eventually, so it would be nice not to have to change out his HD line in the meantime!

It has been great to have Dr. Tolar on service this past week since he knows Raul and his condition the best! He does tend to be very optimistic, but we were still relieved to hear that, from his perspective, we are not at the point of end of life care yet. Raul's issues may not all be curable, but we are not out of treatment options yet. This was a refreshing opinion to hear! The new medicine we started is not just for comfort care, the goal is for it to actually treat his condition. He says he is stubborn, and we know Raul is too, so they make a good team!

Sunday, April 17, 2016

Day +194

Yesterday marked seven months inpatient here in MN! Raul continues to have fevers, though went quite a while during the day yesterday without one. We re-started him on cipro, as his BK viral level had increased. His sodium has been quite high, so Endocrinology had us increase the amount of water he gets with his formula and also recommended checking his thyroid. They said he does not have diabetes insipidus, which had been questioned due to potential neurological damage from his cardiac arrest. Since adding the increased water his sodium has been in normal range. We will be seeing neurology again this week, though I'm not sure how much more they will have to add at this point.

Friday we stopped dialysis at three hours instead of four, as he got really agitated. When he gets agitated his breathing gets labored, and they tend to check lactic acid and blood gasses during that time. His lactic acid has been high, but everything else has been decent. I think the consensus is that when he gets agitated he breathes fast, which raises his lactic acid, then because of kidney failure he can't clear it as quickly as someone else would. Yesterday we only stopped dialysis 13 minutes early for agitation, and he was actually .1kg below his normal dry weight, so it turned out to be good we stopped!

The past few nights Raul has slept better, after being awake most of Thursday night (not agitated, just awake). He has been sleeping in pretty late in the morning too. Last night as we were getting ready for bed he gave us a bit of a scare. The lights were already off, but I noticed his arm bandage was bloody when I had just changed it earlier that day, so I turned them back on to check what was bleeding. With the lights on I could see that he was in a fairly large puddle of blood that had come from his upper arm wound. The nurse and doctor came in and we got the spot to stop bleeding by holding pressure, then putting thrombin powder on it as well before re-bandaging. It was a tiny spot that was bleeding, but bled a ton (5-6 ounces). Thankfully he had just gotten a blood transfusion, and was getting platelets at the time. This morning Dr. Tolar asked me some questions about the spot, and he thinks it is likely a vein, so if it happens again we will do a dye study through his PICC line. For now we will just check that spot frequently to make sure it isn't bleeding again!

We are looking into one specific medication now to hopefully help Raul's "extreme" inflammation in his body. The medication is intended for two different conditions, so a panel at the company will have to decide if it is appropriate for Raul. Dr. Tolar hasn't used it for EB patients before, but successfully used it recently for a patient with a different condition. We would hopefully be able to get it in the next week, but I am not sure how quickly they would expect to see a change. 

Thursday, April 14, 2016

Day +191

The past two afternoons the weather has been great, and Raul has enjoyed spending some time outside! Today we found a good spot where he could watch the road and he actually stayed awake the whole time and seemed to be watching all the vehicles. We also declared him to be the greeter for the hospital, as we were out front and he had lots of people saying hi to him!

The other day we did the bone scan, and (somewhat surprisingly) he had no signs of a bone infection in his body. This is good news, though also doesn't give us any more answers. Last night he had another chest x-ray and had some atelectasis on the right side, so we are trying to get him to lie with that side propped up. This morning he gave us a little scare when he threw up, swallowed it back down, and then de-sated to the mid-80s. We briefly turned his oxygen up and suctioned him, but he returned to his baseline pretty quickly.

Raul's soluble IL2 came back almost double what it was last week, so we have held his steroid wean. Dr. Tolar came on service today, and in talking to him it sounds like Raul most likely does not fit any one diagnosis exactly. Raul never likes to follow the norms., so I guess this shouldn't be surprising! He just seems to have his own special inflammatory condition. Dr. Tolar actually said that this is good though, as it opens us up to more treatment options than if it were just HLH or just TMA/etc.. They were looking into a few medications today to see if we could get them for Raul. He also agreed that the kidney biopsy was likely not worth it, as the treatment for that condition would likely be steroids, and he is already on steroids.

Dr. Tolar seemed optimistic that we could get Raul home in an acceptable condition and also that he may perk up a bit more as we continue to wean his sedation. We do not expect that he will be the same as he was before his cardiac arrest, but any improvement would be great! He has already gotten more alert this past week as we have weaned both the Ketamine and Fentanyl a bit. 

Tuesday, April 12, 2016

Day +189

Well my last post was about Raul's improvement in his throat, which has been our best recent news! He is still having some old blood coming out his g-tube, but I think some is from his mouth as he has an area we have seen bleeding at the back of his mouth. We did give a dose of the clotting medication tonight just to be safe with the mouth bleeding, but it is not bleeding heavily. His antibiotic for his throat ulcers finished yesterday as well. 

Yesterday Raul had a CT scan as he has continued to have fairly constant high fevers. We looked at his sinuses, chest, abdomen, and pelvis. They found what looked to be some pneumonia in his right middle lobe, so we added a course of azithromycin for that. This doesn't explain any long term issues, but may help explain some of his most recent fevers/discomfort. Tomorrow we will be doing a bone scan of his whole body to check for bone infections. A PET scan had been recommended to look for infection throughout the body, but was not a good option for him due to his kidney failure, so we did the CTs and bone scan instead. When you have kidney failure, any test that requires contrast could finish off whatever remaining kidney function you have left, so isn't a good option.

We have been switching over many of Raul's IV meds. to J-tube versions, and today were actually able to remove one of his PICC lines! His line from November was no longer central, so they said it would be more risky to leave it in (clots, infection, etc.), than to have to place another line in the future if it were needed. It is so nice to only have him hooked up on one side now! First time in almost two months! Really his only meds. left that could be switched over from IV are his sedative/pain medications. We are also back up to full feeds! He is getting a new renal formula, so his goal amount was lowered and it will be better for his kidneys.

Raul had a long bout of agitation Saturday evening, but has not had a really bad night since. He has had discomfort, especially with the high fevers, but not full on freak outs. He is also coughing a lot, so that keeps us up some, but it is good as he has a lot of junk in his lungs and throat! The past several days Raul has seemed pretty out of it during the day, so today we went down on his ketamine dose, and he did stay more awake. We will be working on weaning both the ketamine and fentanyl as he tolerates.

On the day of his last respiratory arrest Raul had been given a stroller by a wonderful local family with several children with special needs. He finally got to go in it for the first time three days ago, and loves it! He looks really comfortable in it, and has spent long periods of time sitting in it each afternoon. We are hoping to take it outside soon as the weather is supposed to be warming up. It tilts, which is really nice, and also has tie downs so it can be used in the car.

Our most confusing news came last Friday afternoon. We had been asking the team to consult Cincinnati, as they are the top research hospital for HLH. Well that happened Friday, and that team wasn't completely convinced Raul even has HLH! He still may, as he does meet all the criteria, but they wanted some other things looked into as well. They had made suggestions on testing for infections, as that would cause his inflammatory markers to be high like HLH. They also suggested looking into the TMA/TTP/aHUS category of issues again, so we have sent of more blood work. A kidney biopsy was discussed as it can be helpful for some of these diagnoses, but he is too high a bleeding risk for that to be worth it, as we could treat without it. Tomorrow we should get more of his HLH and TMA/TTP/aHUS lab results back. The potential change in diagnosis does not make him any less sick/fragile, but it does mean we could possibly find something more treatable than the HLH.

Day +185

Sorry, but I have to share this amazing progress despite the slightly gross photos! Last week Raul's throat was referred to as having "gruesome" pseudomonas in nickel sized ulcers around the piriform sinuses as well as a pedunculated mass. We were told it couldn't get much worse, and likely wouldn't ever heal. Well today ENT went back in and the ulcers were essentially GONE (he had fully removed the mass before)! There was nothing there for him to even consider cauterizing! 

We will finish out a course of his tobramycin for the pseudomonas. These ulcers were likely caused by infection causing the sites to erode over time, and despite them not being found earlier, amazingly they still healed. It doesn't mean he is out of the woods for bleeding/choking, but these were the sites we were most concerned about.

Thursday, April 7, 2016

Day +184

I'm sure this will end up being a long update since I have not updated in a few days! Yesterday was Raul's 6 month post-BMT date, and in the afternoon Raul was moved back up to the BMT floor. We are happy to be back! He did have a long period of agitation in the evening, but calmed down through the night and has been okay so far today. He breathes strangely when very agitated, so they did a chest x-ray and blood gas just to be safe, and both looked good. Today he has napped on and off, but we will try to get him up for a while later with hopes that he will sleep better tonight.

Raul went back on continuous dialysis Friday after his ENT scopes, and then the machine clotted again around 9am Sunday. He again lost his blood, but still maintained his hemoglobin pretty well. Even though they had slowed what they were pulling, he ended up below his dry weight and was a bit too dry so had to get two fluid boluses to help. After that we were able to skip regular dialysis Saturday and Sunday, which was nice! Monday they got off all they needed to without problem, and again Wednesday. Both days he was just under his dry weight after dialysis, but the bed scales are hard since they aren't 100% accurate. Today his weight was the same as after dialysis yesterday, and he even peed 34g, which made nephrology very happy! Hopefully tomorrow we will only have to run three hours. We have also been running only a very small amount of formula the past week to help with his fluid status, so we are hoping to be able to go back up now that his weight is more under control.

Raul continues to run frequent high fevers. He tends to get more irritable/agitated when he has the fevers. Despite some improved numbers, his HLH is still considered active, so it could be related to that. It may also be related to the pseudomonas that grew both from his ET tube (when it was in) and the ulcers in his throat. We are treating that with an antibiotic it is sensitive too, but it may just take time. Tomorrow ENT will take him back to the OR to scope him again and see if there is any improvement to the ulcers after 1.5 weeks on antibiotics.

The pedunculated lesion pathology showed inflammation and bacteria, so it does NOT appear to be malignant. Hopefully this will improve with the antibiotics as well. We got to see photos of this lesion, which were interesting. We have discussed options of cauterizing the ulcers or injecting steroids into the sites to help healing, but of course both options come with risks and are not guaranteed to work. We will just have to see how things look tomorrow to start. They briefly discussed leaving him intubated until ENT looked again, but at the time it was looking like ENT wouldn't have time until next week, and thankfully we all agreed we did not want to leave him intubated that much longer if we didn't have to, so he was extubated on Tuesday! Being intubated would not prevent him from having bleeding issues anyway, though would likely make it easier to manage if there was a problem.

When checked last week, Raul's engraftment had dropped more. His CD3 was 89% but CD15 was only 9%. However, with the CD3 still high, the CD15 can still rise. It is also more important to look at the clinical presentation, and clinically Raul's skin has improved! His worst wounds have not changed much, but mild/moderate ones have. And especially new ones! His chest healed from CPR within 10 days! That certainly wouldn't have happened before transplant! We are still waiting on the results of the donor cells in his skin. He also has not had esophogeal strictures since before transplant, and has not had any corneal abrasions in that time either. BMT is not supposed to help the eyes, yet many patients find that it does.

Raul's soluble IL2, an HLH marker, has been trending down. However, other things like his ferritin are still increasing (doubled this week). It is a mixed picture, but shows that at least things are staying fairly stable, not rising uncontrollably. We are considering several options to improve his HLH, as he still has active disease. It may not be curable at this point, but at least should be able to be improved.

Friday, April 1, 2016

Day +178

On Wednesday Raul had an echo in the morning, and dialysis in the afternoon. His echo showed a little left ventricle enlargement, but showed the function to be normal. His dialysis run was a bit complicated so the nephrologist stayed in the room for the full three hours with the dialysis nurse. We ended up getting off 900ml, but did have to give him blood pressure medicine to get through it. He also got a large blood transfusion during dialysis as his hemoglobin had dropped very low.

Raul has been waking up early every morning (like 4am), and watching movies. He has been very awake and alert overall, but doesn't seem too freaked out by being intubated. Since he seems comfortable, we are not going up on his sedation, though he does get extra doses here and there. Hopefully with less sedation and a better sleep pattern we will not end up with as bad ICU psychosis this time!

Yesterday they decided to start continuous dialysis since we had been having so much trouble with regular dialysis lately (no full runs/hadn't gotten him to his dry weight in about 10 days). So we started that in the afternoon, and then had to restart at night because the circuit clotted. Unfortunately, when it clots they can't rinse back his blood that is in the machine, so he lost about 160ml, but amazingly his hemoglobin didn't drop significantly. They had to call back in a dialysis nurse, and the doctor had to stay late. They got him back on around 10pm.

He also had a flexible ENT scope done at the bedside yesterday morning. ENT has been a bit resistant to look as they hadn't been sure how they would treat something even if they found it, but the ICU/BMT/Nephrology attendings were all firm in wanting it done! They didn't see anything major, so the last thing I heard yesterday was that they were not going to do anything further. However, around 8am this morning the ENT attending came in and asked if we knew he was going to the OR (neither the nurses or I did). They had gotten a cancellation so the ICU attending had snagged the slot for Raul for a more invasive scope. So we had to take him off continuous dialysis again and get him ready to go.

Raul is at a point where he could be extubated, but they had wanted to wait to see what ENT said, and we are now waiting to see if any more intervention will be needed. During today's scope they cauterized two spots in his nose, and packed it, but did not feel those were the sources of the major bleeding we have seen. They also found a decent sized ulcertation on one side and a pedunculated lesion (and ulceration?) on the other side of the posterior piriform sinuses. They felt that this ulceration could be the source of the bleeding, though it was not actively bleeding today. They do not know what the lesion is so took a sample for pathology. So now we wait to see if anything else will be done, and when we will be able to extubate him. For now we put him back on the continuous dialysis since it is gentler and he is not moving a lot anyway since he is intubated.

And lastly, amazingly Raul's soluble IL2 was NOT higher this week than last. However, it is still above normal, as are his other labs, so his HLH would be considered active. The new BMT attending is reading through his history and then either he or Dr. Tolar will reach out to Cincinnati again, as they have the top HLH research center. Dr. Tolar had talked to several HLH doctors when Raul was diagnosed, and at that time they all agreed with the treatment protocol we were doing. Raul's case is very complicated though, and he has not responded to first or second line treatments, so it will be nice to have more eyes on his case and maybe they can tailor a treatment to him. 

Day +175

(written on 3/29)
This afternoon Raul was again unable to manage a nosebleed, and ended up intubated during a code. Thankfully this time no CPR was necessary, and he actually did start taking breaths on his own pretty quickly once they started bagging him (before intubation).

He had a couple small nosebleeds in the past week, and he was actually still getting a medicine to help his clotting, but this one must have been worse. It was strange, as no blood came out the front of his nose even when we sat him up, it all went down the back. We only knew it was happening as we started to see some blood draining from his g-tube. Thankfully both his nurse and the hospitalist were in the room with us at the time the code needed to be called! 

We will get his final HLH marker for the week back tomorrow, but at this point I think everyone would be shocked if it had not gone up. We did increase his steroid dose today to hopefully help with some of his symptoms (he has been having fairly constant fevers, and we have been having trouble with his BP during dialysis). Our goal is to get him home, as it has always been, so that is the next step!