Day +49

Time keeps marching on. I can't believe tomorrow we will be half way to Jacob's 100 day mark when we will hopefully be able to head back to California!

Jacob's hair is growing back and so far it looks like it will still be blonde. It's really short, so it's hard to know for sure.

Jacob has been really tired, some times requesting two naps a day. All he wants to do besides eat and sleep is watch Curious George on Netflix. The doctor said fatigue is a normal part of recovery. J's also been eating a ton because of the steroids. He has been off feeds for about a week and is maintaining his weight on his own! He is still getting extra iv hydration at night because his kidney function has been a little off from all the medications that he's on. Hopefully we will get to start peeling back some of the meds soon. Another issue that has come up this week is a rash on the palms of his hands, classic gvhd. It doesn't seem to bother him and we have been treating it with ointment. In fact, the ointment bothers him more than the rash. :)

Praying for his kidneys and liver to handle all the meds and for his gvhd to clear up so we can continue on his steroid taper schedule. Also, Jacob has another chimerism test on the 31st, so we are hoping and praying that his graft is holding strong and even increasing in its percentage. Maybe the gvhd on his palms is a good sign for the graft? We will see.

Here are pictures of the palm rash and his cute fuzzy hair growing in.

Day +42

We were discharged on Tuesday! I'm happy to be out of the hospital. It's hard to believe that Jacob was hospitalized through his day +100 mark for his first transplant last year. This experience has been so much better all around. Being out patient is certainly better, but it puts all the responsibility of Jacob's care on my shoulders. He takes 15 medications, two of which must go in every 8 hours, 8am, 4pm, and midnight. The midnight one has been waking him up even though it goes through his ng tube. He wakes up asking for food, so then we are up for a while. He wakes up at about 6:30 am because it is light outside. He still looks tired, but won't go back to sleep. I think we are both a little sleep deprived. He's on iv hydration for 10 hours at night and ng feeds for 5 hours during the day. Overall he's been a good sport about having to be hooked up to pumps for so much of the time. They are portable and fit nicely into a little backpack. We had a clinic visit yesterday and Jacob is doing really well! All of his blood work came back looking great. We are starting to taper him off one of his nausea meds because he hasn't thrown up since last Monday and we decreased his feeds because he has been eating really well. It looks like we will decrease his hydration next week because he is drinking a lot. He is also tapering off steroids and will be done with them around the beginning of July. So all in all, we are certainly moving in the right direction. We will repeat Jacob's chimerism test at day +56, to see if his graft is improving.

Here is my brave little warrior:

Day +34

This whole past week we have been able to go out on 4 hour passes to leave the hospital each day. It is therapeutic for Jacob because he has something to look forward to daily. Not to mention he gets some great leg exercise being able to run around the apartment. Jacob has also started on enteral feedings through his ng tube. The steroid treatment has definitely settled his tummy down and increased his appetite, but he still isn't up to his caloric goal, so that's why we have to supplement. His favorite foods are goldfish and hot Cheetos. The best news I have to share is that it looks like we will be discharged on Monday! I have been trained on doing his iv hydration, ng feedings, and medications. I'm not sure how many meds he's on, but just off the top of my head I can think of 12. Thank goodness for his ng tube. Jacob loves it too. When it's time for meds, I ask him if he wants to swallow them or take them down the tube, and he hands me the end of his tube. It takes off so much stress for both of us.

Chimerism Follow-up: Not as bad as we thought

On the morning of Day +31, we got further clarification from the doctor overseeing the treosulfan trial. She said not to worry about the B cell percentage because B cells can take 6 months or more to develop. We were more worried about the T cells which were at 76%; the doctor said that this is within the expected range for the first chimerism result for this chemo protocol, and she expects the percentage of donor T cells to increase by the next sorted chimerism test.

The NK and myeloid cells were at 100% donor, which is very good. Last year's first chimerism test in L.A. was unsorted, meaning the "78%" last year was an overall indication of engraftment and was not broken down by each type of white blood cell. The Seattle doctor overseeing the treosulfan trial said that an overall/unsorted chimerism test is heavily influenced by myeloid cells, so given last year's first test result of an overall 78%, Jacob probably had a much lower percentage of donor myeloid cells shortly after last year's transplant than he does this year.

All things considered, Jacob's initial chimerism result is better than we thought when we first got the numbers. After hearing the doctor's interpretations, we feel better about it than we did the night before.

We nevertheless remain slightly cautious about getting overly optimistic at this point. While we trust this doctor's expertise, last year's experience still haunts our memories.

Because of Jacob's history of losing his graft last year, the team will be extra cautious and will do the second sorted chimerism test on Day +56 (instead of the usual Day +80). This will allow for earlier detection if the graft starts decreasing, and it will provide more time for corrective action if necessary.

Mixed Chimerism

Jacob's first chimerism test result came back mixed. His various white blood cell counts range from 76% to 100% donor cells, which sounds good until you consider that his numbers were very similar last year and within months he had completely lost the graft. We will discuss the chimerism results with the team in the morning and see what they want to do. Please pray for the doctors to be inspired to make decisions that will be best for Jacob's long-term health and lifespan, and please pray for Jacob's body to accept the donor cells so he can finally be cured of CGD once and for all.

Day +28

Yesterday Jacob had his endoscopy and biopsy, and the results just came back that he has grade 1 gvhd in his stomach and colon. It is a 4 point scale, 4 being deadly and 1 being mild. That explains why he has been so nauseous and uninterested in food. He will start treatment for it today and should bounce back within a couple of days. The Dr. said we could start to wean him off his tpn on Monday. Today he finished his morphine drip and replaced his continuous tacrolimus infusion with 3 boluses throughout the day. This will free him off his line more often. We even got permission to take him out of the hospital for a few hours to go for a walk or drive. I think he will really enjoy that if he is awake enough. He's on 3 antimetics staggered so that he gets something every 2 hours. That will hopefully keep his nausea under control, although it also keeps him in a constant state of drowsiness. The chimerism test was sent out yesterday and they said we should hear back on that by Monday. Please continue to pray with us that he is 100% donor cells. That would mean a cure for him and no chance of his old cgd cells taking over the new marrow, like what happened during his first transplant.

Also Jacob had his ng placed yesterday which made him so very sad. We invited a little 8 year old patient who has one to come in to talk to Jacob about it. She said she liked hers because she didn't have to taste the medicine. She also told him she could still play around with it in. Jacob smiled while she talked to him. I think it was very therapeutic.

Here is Jacob enjoying one of his cute new pillow cases.

Day +26

This week has been really busy and stressful.  Jacob developed a rash all over his back earlier this week, which considering the timing post BMT, was probably GVHD.  Luckily it cleared up within a day using a tacrolimus ointment called ProTopic.  Jacob has also completely stopped eating and drinking.  He throws up at least daily and often tells me he feels like he needs to.  He is still getting anti nausea medications, but they are not strong enough to help him.  He also has really bad incontinence.  The doctor suspects GVHD in the GI tract, but will not begin treatment for it until he has an endoscopy and biopsy tomorrow to confirm the diagnosis.  The biopsy results will be ready on Saturday.  I've requested a NG tube be placed during his endoscopy tomorrow since he will already have to undergo sedation.  I figure that will spare him the trauma of having to do it while awake.  I hope it helps.  We have to battle over trying to get any oral medications in him daily.  Another issue that has come up is he is hypertensive, most likely caused by all of the medications that he's on.  He's taking blood pressure medications to try to get it under control, but they don't seem to have found the magical combination yet.  Another stressful thing was that within a week, both lumens of his central line needed to be repaired.  They basically cut off the old one and glue on a new one with a little cuff that connects them together.  The line goes out of commission for 24 hours while the glue dries. Luckily they didn't break at the same time, so he had one working lumen at all times.  They are both up and running again.  The doctor  predicts that once Jacob starts his low dose steroid treatment for his GI GVHD, that he will bounce back quickly with his eating and that we could be discharged as early as next week.  I'm really hoping that is the case.  Hospital lifestyles are grueling and I'm feeling it.  I'm sure Jacob is, too.  The chimerism test to show what percentage of donor cells are Jacob's will be performed tomorrow.  The results will be in some time next week.  We continue to pray for 100% donor cells.  I think I covered all the major things from the week.  Jacob has his good days and bad days.  He enjoys music therapy, speech therapy, physical therapy, his nurses (sometimes), and hospital volunteers.  Thanks for your continued prayers and for the care packages and cards.  I'm sorry I have not thanked everyone individually.  I'm juggling everything on my own right now and it is difficult to even fit in the basics, like showering, sleeping, and eating.  But please know we are grateful for the kind words and fun things to help pass the time.  We feel loved.

Jacob wrestling with his Spiderman balloon

Playing with his dolphin

Cruising the halls