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!


  1. Oh I do hope we get to see more smiles soon! I also believe that Raul has more life to live on this planet, so God will make a way! Love to you both!

  2. Glad you are feeling better!