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.