Sleepless in Seattle

Last night, Josh and I returned home from a WHIRLWIND trip to Seattle. We were able to check out three different housing options, tour Seattle Children's Hospital, and have a lengthy consultation with the doctor overseeing the trial, all in one day. I think it's going to take me a couple of days to recover from the exhaustion! After speaking with the doctor, we gave the authorization to initiate a search for a new bone marrow match and to proceed forth with whatever testing needs to be done. She said she could see this getting started as early as January. I'm glad we are going with this regimen. The doctor said Jacob is a high risk transplant patient since this will be his second time around being harmed by the effects of the chemotherapy and other medications, plus the fact that he has some unidentified swollen nodules on his lungs. They have transplanted high risk patients on this regimen with good results, so we are trying to stay hopeful.

A Way Overdue Update

I apologize for the delay of communication lately. It's not for lack of action around here. In fact, it's quite the opposite. Jacob has been doing well since our last hospitalization in September, but we've had a lot of major decisions to make: whether we want Jacob do go through a second bone marrow transplant, and if so, when, where, and how would we have it done. We have made some progress in some of those areas.

First, after much inner conflict on my part, we have decided that we should try again. It was a very difficult decision to come to seeing Jacob eating well again and watching his counts creep back toward normalcy. I hate to start from square one and watch him suffer and face complication after complication all over again. I feel grateful that he is almost completely recovered from his transplant in April. He even got the okay to join everyone for our Thanksgiving dinner.

The second question we face is when would we want to go through transplant again. My initial decision was to wait a year or so to enjoy having him healthy and at home again. However, part of the complication that presents, is with CGD, there is a window of good health that is ideal for transplant. The patient must be free from infections, namely fungal (e.g. aspergillus) infections, otherwise, he can no longer be a candidate for transplant because of the increase in risk. Once a patient gets a fungal infection, the doctors are never convinced that it is completely gone, even if labs and procedures say it is gone. It is just too sneaky of an infection and could prove fatal if found in an immunosuppressed (BMT) patient.

So, thinking through that logic, we decided the sooner the better, as much as it pains me to say it. Jacob has never had a major fungal infection that has been detected. He's had some abnormalities on his lungs, swollen lymphnodes, that came back benign after a biopsy was performed pre (first) transplant. After Jacob underwent chemotherapy at Children's Hospital Los Angeles, there was a test that indicated that Jacob may have had a nonspecific fungal infection, but it was never determined what it was and was never detected again after that. The other minor issue that pushed us to sooner rather than later on a second transplant was that his doctor wanted to have his central line removed. It might sound silly, but I'd rather have his line removal be a sign of a successful BMT and recovery, not just a postponement of more dreadful things to come. So there you have it.

That brings us to where and how we'd like to have it done. After being in communication with a couple of other CGD moms, I really liked the sound of going to Texas Children's. They have transplanted 15 CGD patients, 10 of which were unrelated donors, with a 100% success rate. Those are amazing numbers! After I brought it up to Jacob's doctor, he suggested we also investigate Seattle Children's, as well as do a followup with the team at Children's L.A.

The team at CHLA admitted that they didn't know why Jacob rejected his graft. They had a few possibilities to explain it, but nothing for certain. They also mentioned that if he went through another reduced intensity transplant, he'd have a 25% chance of rejecting the graft again. However, if we did a full myeloid ablative protocol, which they were recommending, he'd only have a 10% chance of rejecting the graft, but a 25% chance of mortality. Neither option sounded very hopeful to me, and I left not feeling any better than before we consulted with them.

Incidentally, the BMT doctor and CGD specialist doctor we met with at CHLA noticed the rash that Jacob has had on his face recently (see the picture above). The CGD specialist wanted to rule out the possibility of a fungal rash and suggested getting it cultured. The BMT doctor thought it looked like graft versus host disease (GVHD) and got our hopes up slightly about the possibility that the donor cell count might have increased. Neither seems to have been the case. I had just figured it was the same sort of eczema rash common amongst GCD carriers. A blood sample was sent off to check Jacob's chimerism (the percentage of donor cells vs. his old cells), and unfortunately there was no evidence of improvement in donor cell engraftment. A dermatologist assessed Jacob and said the rash didn't look fungal.

A couple of weeks after our CHLA appointment, our doctor called saying he spoke to Seattle Children's and would have them give us a call because they are running a trial up there for which Jacob would qualify. Basically, they replace busulfan, which is an extremely toxic chemotherapy drug used in bone marrow transplantation, with a trial drug called treosulfan. Treosulfan does the same job of aggressively killing the immune system in preparation for transplantation, without the same toxicity to the liver and lungs, and is still a myeloid ablative chemotherapy, which increases the chance for engraftment. It is a preferred drug to high risk patients like Jacob and other children with immune deficiencies, because it does less damage to the body. They are in phase two of the trial and have tranplanted 28 patients in the past two years, 3 of which were CGD patients, who are all doing well. The mortality rate is 10%, versus 25% with busulfan. The one patient that they lost came in for a second transplant and was already suffering from severe GVHD. We are hopeful that Jacob is in good enough health from the start that he would pull through successfully. 100% of their patients engrafted. After speaking directly with the doctor conducting the trial, both Josh and I feel good about traveling there for a consultation and to meet with the transplant and immunology teams. We are waiting to hear about travel arrangements and such.

The other bonus about Seattle (and this may seem trivial), is that Kaiser has a contract with them so we would not have to file appeals to have our insurance cover the transplant, like we'd have to if we ended up going to Texas Children's. It's just one less concern to have to stress over, however unimportant it may sound.

On another note, here are my two super handsome Spideys on Halloween:

We had a fun time doing the trunk-or-treat, minus the face mask and the panic attacks I get any time Jacob's hands touch ANYTHING or anyone outside our house; you just don't know what kinds of germs people and things carry. Josh even sanitized the candy wrappers before we let the kids sort through their stash.

Cultures & Medicine

We ended up moving back to peds, which means the doctors feel he is heading in the right direction. The bacterial culture from this morning came back negative for the first time since we were admitted. That means the treatment seems to be working. Also, Jacob’s c. diff infection came back negative. While that is good news, I’m not holding my breath on that one since we’ve had negative results before only to come back positive again. The para influenza virus that Jacob was admitted for a couple weeks ago is still coming back positive, so they started him on an antiviral to help him. The positive reading could just be from proteins that the virus made in his blood, but they want to treat him for it just to be cautious. If the treatments go well, we could be home in 7-10 days. They are also treating Jacob’s central line by letting an antibiotic sit in there for 12 hours. They don’t want to take any chances of the bacteria sticking to the line and coming back. I’m glad they are being very thorough. Jacob seems to be feeling better. He can sit up on his own to play for a few minutes and he is happy, which makes me happy too.

Smile

I missed that smile!

Jacob didn’t have any fevers last night and slept well. I was even able to snap a few happy pictures of him before bed. He got blood yesterday which also helped to perk him up. It sounds like we might be moved back over to the peds floor today. Their concern right now is that Jacob's temperature is actually running low, which the doctor said could be a sign of infection.

On another note...

The view of the nurses strike from our window

PICU

My brave little man

Jacob was transferred to PICU early this morning after one of his blood cultures came back as gram negative rods, most likely e. coli. Needless to say, this is a very dangerous bacteria, and even more so for CGD patients. Jacob’s cells are helpless against this type of bacteria and he can only be cured through antibiotics. I’m glad we immediately brought Jacob in yesterday. He slept the entire day and night and didn’t break his fever until later this morning. It’s starting to rise again and we are waiting for a dose of Tylenol to come. He’s been awake today, albeit, miserable. His body must just ache because he cannot identify where it hurts, but he just cries at the slightest little thing. The fact that he’s awake is a step in the right direction according to his doctor. If he continues to improve, we will move back over to peds in another day or two to finish his treatment and recovery. In the picture, he is undressed down to his diaper to try to help bring his temp back down. This is Jacob's first hospital stay where he has slept in a big boy bed and it's so nice to be able to lie down next to him to comfort him. He seems to really like it, too.

Jacob

Jacob is back in the hospital for the second time this month. I brought him in a couple of weeks ago because he was spiking a fever and for a BMT patient, that can be very serious. After a couple of days, Jacob seemed to be improving and there were no red flags in his work ups, so he was able to come home. He came back positive for a virus, but the doctor thought his body would be able to fight it off over time. Jacob really did seem to be doing better for the past week. We went in for his check up on Friday and his liver function tests were way off, however. The doctor thought it might’ve been from the virus, but wasn’t sure. He had planned on retesting him today or tomorrow, but Jacob was spiking a fever this morning and had thrown up a couple of times, so the doctor had us bring him back in to the hospital right away. Jacob is sleeping now and getting some iv hydration, along with some antibiotics. He took some Tylenol an hour and a half ago, but his fever has stayed the same. The doctors are considering moving him over to PICU so he can be more closely monitored.

We also recently found out that Jacob’s new bone marrow is almost entirely gone. His body rejected the graft and he has mostly his old cells back now. A lot of people ask where we go from here. The doctors have talked about infusing more cells from the same donor. This idea may have worked a couple months ago when he still had more donor cells, but now it is pretty much just a shot in the dark. Another option could be to do a second BMT. The thought of putting Jacob through that and starting all over again turns my stomach. He is still so fragile and weak from the first time around and from whatever he is fighting now, it just seems like a bad idea. I’m not even certain that his body could handle it. He definitely couldn’t handle it in the condition that he is in right now. I told the doctors we are not ready for that. For now, we just hope that his body heals and that his prophylactic medications protect him from CGD related infections.

First Day of School

My little fireball Ethan had his first day of kindergarten today. Although he won't admit it, I think he had a great time and is in for a fantastic year. What makes it extra special, is that he shares the campus with his Grandma Judy, who is a first grade teacher there. They got a chance to share a hug before school started this morning. It's a comfort to me knowing that the staff already knows about Ethan and has been looking forward to him attending school there.

Change of Plans

Our family heart attacked and decorated our house for Jacob's welcome home.

Ethan's so happy to have his little brother home again.

Jacob is NG tube-free!

In our last post, Josh mentioned that the doctors had requested Jacob's donor in order to give Jacob a boost of cells to help strengthen his graft. Last Friday, Jacob's NBT test (the test that indicates how protected he is against CGD related infections) came back at 35%. This is great news! His past three NBT test results have gone from 29% to 30% to 35%. We haven't heard what yesterday's results are yet, but we are hoping that his score is still going up or at the very least, holding steady. According to the doctors, a person only needs about 12% functionality to be healthy, so 35% would be great! Because of this encouraging news, the doctors have decided to put a hold on readmitting Jacob for his boost of donor cells for now, as long as his NBT score holds steady, despite his extremely low 1.55% donor myeloid cells from his last chimerism test. Apparently, the NBT test result is more significant to Jacob's disease than the chimerism results. So, that is our biggest news.

I also wanted to mention that on Thursday, Jacob's NG tube clogged so badly that we couldn't push anything through it. Since it was useless and knowing Jacob had a checkup the next day and could get another tube, we went ahead and pulled it out. Since Jacob did such a good job taking his medicines orally on Thursday, the doctor said to go ahead and give Jacob a chance to start eating and drinking again. If he can eat, drink, and take medicine, then he no longer needs the nose tube. I'm happy to say that he has been doing wonderfully with these three tasks. Whenever he gives me a hard time with his medicine, I offer to put his nose tube back in, and that quickly resolves the problem. :) Ethan has also been a great helper when Jacob chooses him to administer medicine. Who would have thought!

Ethan starts kindergarten in two days! Last year we kept him in preschool for a second year to give him a chance to mature a little more, and now I feel like he is more than ready for his school experience. He turns 6 in October, so he will probably be the oldest kid in his class, which will hopefully give him a little bit of an advantage throughout his school years.

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.

Day +75 Two Steps Forward, Three Steps Back

When all of this began, I never imagined we'd see July in the hospital, but here we are with July right around the corner and no end in sight. The past 10 days have been filled with a lot of ups and downs. Last Monday Jacob threw up his NJ tube so he had another one placed on Wednesday. He almost made it a week with that one, but threw it up this morning. He has been more willing to eat and take his oral medicines today since the tube came out. He understands that if he doesn't eat, drink, and take his medicines, then we have to put in another tube and for now that seems to be all the encouragement that he needs. He certainly looks a lot more comfortable without that tube taped across his face. I just hope he can get all the nutrition, fluids, and medication that he needs to be healthy.

This past Sunday was a really rough day for Jacob. He had tachycardia (an elevated heart rate), and high potassium (which can cause cardiac arrest) for which he had to take a terrible medication to cleanse it from the body, causing him to vomit and have terrible diarrhea all throughout the day and night. All of his numbers were off too, indicating that there were other problems going on. His stool and vomit were coming back positive for blood as well. Doctors decided to give him a blood transfusion since his hemoglobin was trending down anyway. After a restless night Sunday, Jacob didn't wake up until nearly noon on Monday. Luckily, he seemed to be back to his happy self again and the symptoms from the previous day seemed to clear... or so we thought. Although he continued happy throughout Tuesday, a couple of tests came back with bad news. First of all, we found out that he has an infection in one of the lumens of his central line (the lumens are the two tubes hanging down from the central line placed in his chest, and the IV is attached to one of the lumens), so he started on an additional antibiotic for that. Next, we found out that he tested positive for C. diff, a problem with the bad bacteria taking over the good bacteria in the gut. He is taking Flagyl four times a day, and unfortunately, it tastes really gross and without his tube in, he has to get it down on his own. C. diff is contagious so now we have to take extra precautions in and out of his room.

Other news we got last week that was terribly discouraging was that it appeared that he might be losing his graft. All the donor cells that tested so high in his body a month ago had dropped, some of them dramatically. The doctor ordered an NBT (nitroblue tetrazolium) test to see if his CGD was still cured despite the drop in donor cells. We got the results today and thankfully he still has 60% functionality of working neutrophils, which is enough to protect him from CGD-related infections. According to his doctors, he only needs about 10% donor cells to be protected, so 60% is plenty. However, this loss of donor cells did concern the doctors enough that they are weaning Jacob off of his immunosuppressant, afraid that it might be too weakening to the donor cells. As he comes off of that drug, hopefully his chimerism of donor cells will increase, or at least not drop any more. There is a chance of graft vs. host flare-ups while coming off of the immunosuppressant, but it's something that needs to be done to protect the graft. I would feel terrible if we lost the graft and put Jacob and our family through this ordeal for nothing.

There have certainly been more bumps in the road throughout this process than I ever imagined beforehand. We thought for sure that Jacob would be home by now, but with one problem arising after another, the doctors still can't say when he will be safe to leave. Thank you for your continued thoughts and prayers.

Day +65 NJ Tube & Happy Father's Day

Since Jacob's NJ tube placement last week, he has gradually increased through the minimum dose of Pedialyte to the maximum, and then switched over to diluted formula and is now on full strength formula. He has tolerated the nutrition well, which has been a blessing. We struggled a little with medication through the tube at first because he kept vomiting afterwards even though it was being inserted all the way down in his intestines. We have found that if we slightly warm up his medications and water and do his medications very slowly, he doesn't throw up. Jacob is taking all but two medications through his tube now, so once we get those last two switched over and the doctors are happy with the levels of those medications in his body, we will hopefully be discharged. He is getting formula continuously through his tube, but the doctor wants to try increasing the amount of formula so we can take breaks off the tube sometimes. The formula seems to have helped resolve his diarrhea and has even triggered an awareness to eat again. It's not a lot, but at least he will eat a couple of crackers or something each day. We are grateful for everyone's continued prayers for Jacob's complete healing. We still have a long way to go with him, but feel like he is slowly making progress. We will get the results on another chimerism test this week to see how the new marrow is doing. We are hoping that it is thriving!

Josh was able to spend Father's Day at home with Ethan and they got together with extended family for dinner today. I'm grateful for my husband who is a terrific and loving father, for my own incredible dad who has taught me so many things throughout my life, and for my amazing father-in-law who is so kind and helpful. I love these men so much and am grateful to have them in my life.

Other good news:

*Ethan's transfer request to go to school where Josh's mom teaches got approved! It is also where Josh and his younger brother attended elementary school. We are so excited! He starts kindergarten on August 8th, I believe.

*Last week I got to witness the birth of my cute little nephew Colin! It was an amazing experience and I'm thankful that my little sis' invited me to be present for it.

Happy Birthday, Jacob!

Jacob playing with his new toys

Jacob is showing how old he is now.

He says this is harder than two fingers.

He is really concentrating.

Jacob is delighted to frost his cake with Ethan!

(Too bad he wouldn't eat any of it.)

Putting on the finishing touches!

We celebrated Jacob's 3rd birthday in the hospital and I think he had a fun time. I was in the room with Jacob, and Josh was outside the room with Ethan. Josh wiped off gifts to get ready to be in Jacob's room and Ethan had the job of handing them to Jacob. After gifts, the boys decorated a birthday cake in the doorway. Jacob had a lot of fun and as I was putting him to bed that night, he told me "I want to go home." We really miss being all together as this process continues to drag on.

Yesterday Jacob had the NJ tube placed. It passed the test of not coming up when he vomited, like the NG tube did. After some complications with the tube kinking inside of Jacob, we finally got it working this morning and now Jacob is getting pedialyte through it. He's starting with only 1 teaspoon/hour to see how he tolerates it, and then it will gradually increase. He is still getting hydration through his central line until he is getting the proper amount of nutrition through his tube. Jacob is still getting used to having a tube taped to his face, but at least he hasn't tried pulling it out. He does seem sad about it though, which breaks my heart. He has barely said anything or smiled at all these past two days. I really wish he could have his big brother here with him. Ethan really seems to brighten Jacob's day the most.

Day +49 Possible Virus

The boys send each other a hug since they can't touch.

Say cheeeeeese!

Jacob's CT scan result came back that his lungs are clear! In fact, the problem that was identified 3 weeks ago has been resolved. By process of elimination, the doctor suspects he has a virus and that there is not much we can do for him. He is already taking antiviral medication, so that is about all they can do. Jacob is still coughing so hard that it makes him vomit. His appetite is non existent, so it's really hard to get him to eat anything. It sounds like the doctor is just waiting for him to start eating again before he can go home. She wants to try a different type of feeding tube called an NJ tube since Jacob threw up his NG tube two days ago when we tried that solution. The NJ tube goes down past the stomach and into the intestine a bit in order to keep it down even when he's coughing and throwing up. We will try the NG tube a couple more times next week before we resort to the NJ tube. I'm not comfortable taking him home yet, so I'm glad he is still here under professional care. His ANC has dropped really low as well, down to 440.

Dropping ANC and white blood cells

Sorry for the delay in posting an update. It’s easy to share joyful news, but hard to share our setbacks. Jacob’s GVH rash is pretty much gone, which is our good news. Now onto the bad news. Jacob is learning to take his oral medications. Some times he gets it down and keeps it down, other times we are not so lucky. Jacob is not interested in eating yet and he always falls far below our goal for liquid and caloric intake. Two nights ago Jacob’s cough started getting worse and his anc and white blood cell count have been dropping steadily the past five days. Six days ago his anc was 2580 and today it is 580. These symptoms are concerning to the doctor and ourselves because he seems to be fighting something. He hasn’t had any fever and his lungs sound clear, which could lead us back to the silent and sneaky fungal infection that the doctor suspected a couple of weeks ago. Jacob is getting a repeat CT scan today to see if the lung inflammation has gotten worse. If there are any large areas of inflammation, Jacob may have to get another lung biopsy to see if the doctors can identify the problem and treat it more specifically. He also had another sinus rinse this morning to see if they can identify a problem there.

Day +33 Functioning Neutrophils!

We got some great news yesterday. The cell that was functioning at 0% in Jacob’s old immune system, called a neutrophil, is now functioning at 96%. That means the donor’s cells are working in Jacob’s body! Other news we got was that 78% of Jacob’s cells are donor cells and 22% are his old. The donor’s percentage should continue to grow as it takes over Jacob’s weaker cells. In 6 months or so it could reach 100%. That is what we are hoping for. The doctor sent off a test today to find out what types of cells are left of Jacob’s. That will take some time to come back.

Jacob was supposed to start taking an appetite increaser medication yesterday, but was unable to swallow any. Oral medications are going to be a huge challenge. How do you get a three-year-old to willingly take yucky medication? We are going to try a sticker chart today. We may never get out of here if he can’t learn to swallow his medications. We are not completely opposed to trying an NG tube, so we will see. An appetite increaser would be great because he would then be allowed to come off of iv nutrition; one step closer to coming home. But for now, he might not be getting enough calories and the doctor might order him an actual increase in iv nutrition a couple times a week.

On Monday Jacob had a chest CT scan to investigate the cause of his persistent cough. Originally, Jacob was supposed to be sedated, but he did so well, they let him stay awake. He squeezed his eyes shut tight and I was able to stay right next to him and touch his legs and talk to him the whole time. I couldn't help but get choked up watching his sweet little face. I’m so proud of my little man for being so brave for something that was scary. Just as the doctor suspected, Jacob had swollen lymphnodes throughout his lungs. Jacob had a lung biopsy before starting his bone marrow transplant, and the biopsy came back normal; however, fungal infections are hard to detect and frequently sneak through undetected. The doctor figures that the swollen lymphnodes found before the bmt were in fact a fungal infection and now it has spread. We are hoping that his new functioning cells, along with the antifungal medication that he is on, will take care of the problem, but it is definitely a concern. The new cells could go overboard attacking the fungus and end up causing pneumonia. We are keeping a close eye on any new symptoms and Jacob will get a followup CT scan down the road to make sure the infection is not getting any worse.

Another Difficult Day

Jacob has just had two rough days in a row. As Kylene mentioned, Jacob threw up 6 times yesterday while I was at the hospital with him. This morning things seemed better at first: very little coughing and no vomiting, and he hadn't vomited at all while he was sleeping last night. Later today, however, the vomiting returned (4 times). He had a fever tonight before I left: 100.2° F (37.9° C), which is a mere 0.1° C (0.2° F) below what they consider to be a fever spike. When I spoke with the doctor on Thursday afternoon she said that if Jacob started spiking a fever it would be cause for concern and they would have to reevaluate his situation, so hopefully the main BMT doctors will take the fever into consideration when they discuss Jacob's situation in the morning. I just called the nurse, and she said that the fever has gone down and that the resident instructed her to only do further tests if his temperature reaches exactly 38.0° C or higher, as per their usual procedure.

Since the CT scan couldn't be done until Monday, Jacob had to get X-rays this morning to see if any problems could be identified before Monday. We don't know whether the doctor had a chance to analyze the X-rays yet, but I'll discuss it with the doctor in the morning.

Since Jacob's hemoglobin and hematocrit were low this morning, he received a blood transfusion.  Thanks again to all those who have donated blood or platelets, or have tried to donate, or have wished they could but live too far away. If you missed Kylene's mention of it a couple blog posts ago, she said that Jacob does not need platelets at this time, but he may still need blood. Little did we know that he would need blood again so soon—today!  If you have O+ blood, you can donate whole blood at Children's Hospital Los Angeles specifically for Jacob.  After scheduling a time to donate, please sign up on our calendar. Here's the link.

We have a lot of questions right now; I literally have over a dozen questions to ask the doctor in the morning. We don't even know for sure why Jacob has started coughing and vomiting. A couple of theories have been floated, but no definitive conclusions have been reached yet. Jacob is on an additional medication today and restarted another medication to hopefully help reduce vomiting, but both medications are more about controlling the symptoms rather than curing an underlying problem. Kylene and I are very concerned about Jacob's health right now, and we hope we get some answers soon (and that the problems are curable).

This is a very difficult time for all of us, so we humbly ask that you please keep Jacob and our family in your prayers. We greatly appreciate your love and support.

Four Weeks Post Transplant

I can't believe how quickly Jacob's condition can change from one day to the next, in this case for the worse. On Wednesday evening, Jacob started coughing and then threw up. It was strange because he hasn't thrown up for a long time, but an isolated incident didn't seem to be much of a concern, although I did notice his appetite was nearly gone that day. He did mention a couple of times that his tummy hurt, but I thought it might've been from not eating for so long and then starting up again. Yesterday, the coughing continued and he threw up six times by the time Josh had to leave last night. His doctor is concerned that it might be a fungal infection, since those are the hardest to detect and clear up. The doctor ordered a sedation and a CT scan to check for a fungal infection which will probably take place Monday. The doctor thinks his enlarged lymphnodes that he had biopsied in February may in fact have been an infection, but slipped through undetected. Please continue to pray for Jacob. My heart is breaking. It seems like just when things start looking hopeful for Jacob we have a major set back. All Jacob's numbers are down today. ANC 1310, platelets 159, white blood cells 1.92, hemoglobin 8.6. Sorry to share depressing news, but we need to rally our faith and prayers in Jacob's behalf. I've seen the power of prayer work in the past.