Jacob Is Home! ... For Now

Let's start with the good news: Jacob came home from the hospital on Wednesday! He has been enjoying spending time at home with both of his parents and his big brother. In fact, we've all been enjoying spending time together as a whole family, which we hadn't been able to do very much for about 3 ½ months.

Jacob still has his central line and his NG tube in, and he's still on his regimen of medications throughout the day and nutritional supplementation at night via the NG tube. Kylene and I are basically Jacob's nurses now; we have been trained in administering his medicine every four hours during the day and operating his automatic feed machine at night, changing the dressing over his central line site and replacing the tape holding the NG tube to his face as often as needed, flushing his central line daily to keep it functional, replacing the caps on the central line lumens weekly, etc. Of course, we're also responsible for constantly reminding him to be careful not to be too rough or careless with his central line since we don't want it to pull out, and throughout the day we try to encourage him to eat. He's been doing better at eating since he's been home, although he promptly spits out much of the food that he tries—often onto the new carpet.

With all of the medical knowledge and training we've gained in recent months, Kylene and I joke with each other that we could practically be MDs or nurses. Now maybe we could add carpet cleaning specialists to our repertoire. But I digress.

On Friday, Kylene took Jacob to CHLA for a follow-up appointment with a BMT doctor, where we received the bad news: the latest sorted chimerism report shows that while the percentage of certain donor cells is very high (79.2–85.5%), the percentage of certain other donor cells has dropped to almost nothing (1.55–3.25%). According to the BMT doctor, the type of cell that has dropped to 1.55% donor DNA must be higher for Jacob to be effectively cured of CGD. This seems to contradict the 30% coverage against CGD-related infections from the last NBT test, which was done prior to the last sorted chimerism. They drew blood for another NBT test on Friday, but we found out this morning that they made a mistake when doing the test and will have to redo it (this is not the first time this has happened with that test at CHLA). Now we apparently have to wait until Jacob's next CHLA visit on Friday for them to draw blood for another NBT test.

In any case, the BMT doctor indicated that they would be contacting the donor to try to get him to donate stem cells from his blood that can then be infused into Jacob to boost his donor cell count. The donor would just have to agree to take GCSF (the white blood cell boosting drug that Jacob took for one day prior to a test once) for 5 days prior to the stem cell extraction, which should be painless unlike the bone marrow aspiration that the donor went through before. If the donor agrees to donate cells, Jacob would have to be hospitalized for an extended period of time again. If for some reason the donor refused (probably unlikely), Jacob would have to find another bone marrow match and go through the entire bone marrow transplant process all over again, chemo and radiation and all (although I'd guess that they probably wouldn't do the reduced intensity treatment this time). It was very frustrating to find this out just two days after Jacob got home. We already wondered whether they might have been in too much of a hurry to get him home given that the last sorted chimerism results were still pending and there was another C. diff test that needed to be done.

Speaking of which, we also found out this morning that after two negative results for C. diff after finishing his Flagyl treatment, the third test showed that he does currently have C. diff. Both Jacob and Ethan have been having C. diff-like symptoms, and we know that C. diff is highly contagious, so we're trying to figure out some way to get Ethan tested (they won't just prescribe Flagyl based on Ethan's symptoms and the fact that his brother has C. diff, and Kylene can't just bring in Ethan to be tested because she can't take Jacob out in public, and I work during the day so I can't take Ethan in either).

On the bright side, at least we get to have Jacob home for a few weeks or however long it takes before the donor can donate cells.

On the less bright side, my academic leave of absence ends August 14th, which means I have to get back into my Ph.D. studies just three weeks from today, and Jacob could be in back the hospital around then. I have no idea how I'm going to maintain my sanity and fit everything into my life; it's been stressful enough without having school studies and assignments on top of everything else. My next class covers statistics, so if anyone reading this is a statistics expert and would be willing to help me occasionally, please get in touch with me.

We continue to be in need of your faith and prayers as we go through this extremely stressful and trying time in our lives.

Moving to the New Hospital

Everyone wants Jacob's picture

He is the final patient to move

Bye, bye old room!

View from Jacob's new room

Jacob, and the rest of the patients at CHLA, moved from the old hospital to the new one today. Jacob was selected for a press event, so he had a photo shoot with the media. Here he is getting to leave his home from the past 3 1/2 months. His sign says, "They Saved The Best For Last" because he is the very last patient to leave the old hospital. So cute! I love that he's also giving a little shout out to my alma mater with his shirt. I'm sure Josh planned that on purpose. His new room looks out onto Sunset Blvd. Although we will only be here for 3-4 more days, I'm sure he will enjoy looking down on the street. There are a lot of emergency vehicles that pass by that will be intriguing for him to watch. We might even be able to see an emergency helicopter or two land on the helipad across the street.

Day +91 Coming Home Soon!

The doctors have decided that any other testing they are monitoring can be done outpatient. Jacob has held steady at 30% coverage for his CGD while off immunosuppressants, so we will just watch to see if he needs a boost of donor cells or not. His doctors are happy with 30%, so we hope and pray that he holds onto that. He will have a lot of restrictions once he's home, like following a special diet, not having children visit, avoiding the sun, not being in public places. We basically have to avoid germs and conditions which could flare up his graft versus host. We are excited to have him home, but nervous about the extensive care he requires. He is coming home with a double lumen central line which needs to be cleaned and flushed daily. We also have to change the dressing at least weekly. He's coming home with his NG tube and a pump to run his formula, so we will have to learn how to set all of that up. Not to mention his myriad of medications to keep track of: antifungal, antibiotics, antiviral, appetite increaser, folic acid, etc. This is an exciting, yet a little scary, time, as I become his full time nurse.

Day +86

I only ever feel like posting when we have good news, which is why it has taken so long for an update, so I apologize. I know a lot of you are worrying and praying for Jacob and our family, and we do appreciate it and want to keep you informed of the details. The good news on Jacob right now is that he is back up to his caloric goal getting formula through his NG tube. He had lost a bit of weight, so it's good to nurture him back to health. Other good news is that he hasn't thrown up for almost two weeks straight (knock on wood). The latest bad news is that he seems to still be rejecting the graft. The other CGD patient who had the procedure just before Jacob had 100% donor cells when he got discharged (lucky), and Jacob is down to 29% right now. It's depressing how one thing works for one patient, but not for another. The immune system is so complicated, even the doctor admitted they are still learning the intricacies of it. They are going to test Jacob again on Thursday to see if he is still trending down now that he's been off immunosuppressants for a while. If he is, the good news is, they can give him a boost of stem cells from the donor. The bad news about that though, is that this hospital has never done this procedure before AND that Jacob's graft versus host could flare back up, starting us over from almost square one. So as you can see, we have ups and downs almost on a daily basis and it's very emotionally exhausting. I get scared to get hopeful any more because every time I do, we get more bad news. Ethan starts kindergarten on August 8th and I feel so bad I'm missing out on so much of his life lately, that weighs me down with guilt. I see him only 3 days a week and there is no end of this situation in sight. There's really nothing more I can do though. We are managing the situation the best we can with the help and support of loved ones. It's just taking way longer than we ever imagined. A lot of people ask if we are getting any idea of when Jacob will be able to go home, and the answer to that is: nobody knows.