Day +110

Our flight is booked and we are heading home on Tuesday. Hooray!

Jacob has been doing well. He had his adenovirus treatment on Monday and the doctor wants to do one more next Monday before we leave. Even though the latest adeno count is 3200, she thinks since his kidneys are handling the treatment, it would be the safest thing to do. She told me she has had patients die from adenovirus, and since it was found in all of Jacob's systems, blood, urine, gi tract, and respiratory, she wants to give him as much protection as possible. Jacob also got some gcsf this week to boost his ANC, which has been slowly declining the past couple of weeks. Tuesday he got an ivig infusion and had his final review conference. Monday will be his last appointment here and then we will go home the following day.

He is a long way from having a functioning immune system, but he is stable enough to go home and continue treatment there.

Day +105 Homeward Bound

We successfully made it out of the hospital on Tuesday. It wasn't easy though. J's ng tube clogged at 3am and I worked on trying to unclog it until he woke up at 8am. We finally gave up and just had to pull it out. That tube had lasted about 2 months. The nurses tried getting a new one in 3 different times, one of the attempts lasting for 45 minutes, but he was clamping down his throat and gagging so it kept getting stuck in his throat. It was heartbreaking. Jacob even was saying he would just take his medicine in his mouth, which I was willing to let him try, but after another 45 minutes he had only been able to swallow 3-4 of his 15 morning meds. It just wasn't going to work. Before the third attempt, they gave him a dose of adivan to relax him, but it didn't help. They tossed around the idea of doing it under sedation, but that would mean we'd have to stay another night because he'd have to be npo. Around 1:00pm, the two nurses who had been trying finally gave up and called in a third. She was able to get it in on her first try. It was a huge relief to get it in. Hopefully by the time this ng tube wears out or clogs, he will only be on a few meds and be able to move on without one.

It will be six months, almost to the day, when we will be heading home to be reunited as a family. Our last appointment up here should be July 30th. We are making preparations to fly home on July 31st. We already have an appointment scheduled with Jacob's home doctor for August 1st.

Jacob's Epstein Barr Virus load has come back undetectable the last couple of times, so it seems he's got that under control. I'm so glad he didn't have to get treated for that. His latest run on the adenovirus came back at 8,300. Can you believe it was 9.4 million just a few weeks ago? The infectious disease doctors say we can stop treating it when it drops under 10,000, but his out patient dr wants to do one more cidofovir treatment on Monday, then hopefully he will be done with that. I worry about his kidneys.

Considering everything that has happened in the past month, I consider it a miracle that he is well enough to go home at this point. The doctors here have been incredible. Their expertise and experience is beyond anything we ever could've hoped for. I'm so grateful we came here for Jacob's transplant. I am excited to go home, but sad to have Jacob leave their care.

Happy Day +100 Dearest Donor...

Today is another huge milestone for Jacob's recovery.  It's usually evident by this point whether or not a transplant was successful.  Each day we can breathe a teensy bit easier hoping that Jacob is actually successfully cured.  Of course, it can still take a year, some times up to two years, for his new immune system to mature enough to fight off germs properly.  But each day from here on out makes it more likely that he will hold onto his new marrow.  For now, we have to keep him away from crowds and other potentially germy places.

We aren't allowed to meet our donor until one year post transplant, and only if she is interested.  However, we can send her a note as long as it doesn't reveal personal information about us.  This is what we are sending to her:

Dearest Donor,

It has been 100 days since our precious little boy was infused with your life-saving bone marrow.  Your cells are happily engrafted and already working miracles for him.  Our gratitude is beyond expression for your generous donation and sacrifice.  There is no greater gift that a parent can imagine than to give their little child a chance at a rich and full life, and now thanks to you, he will have that gift.  My husband, other son, nor myself, were matches.  We are filled to the brim with appreciation and love for you for giving our son what we couldn't give him.  My husband and I are registered bone marrow donors, and we hope that we will one day be able to pay forward this beautiful, heroic gift of life that you have given to our family.  You are our hero!  We'd love to get in touch with you as soon as it is allowed.

Love,

Our Grateful Family

We are still scheduled to be discharged tomorrow.  Jacob's adenovirus result from Friday came back at 40,000, down from 450,000 last Monday.  We will get another viral load update this evening.  He gets another dose of cidofovir for the adeno tomorrow morning before discharge.  Details of our return to California are still being worked out.  Jacob's doctor here (Dr. Carpenter) needs to contact our doctor at home (Dr. Cheng) to see if he will be comfortable taking on everything that Jacob is still being treated and monitored for (adenvo, hypertension, ebv, c diff, gvhd, etc...)  Then he will contact the out patient doctor (Dr. Delaney) to give her his recommendation based on what Dr. Cheng wants to do.  So, there are still a lot of details to work out, but it will work out.  

Day +97

Jacob's EBV count dropped from 2800 on Monday to 87 yesterday.  His immune system did that all on its own.  This is fantastic news.  They will not need to treat with Rituxan at this point.  I'm sure they will continue to monitor him to make sure he can keep the counts in check.

We will have a new adenovirus count this evening.  Monday he was at 450,000.  Hopefully, it continues to drop.

The team is working on having us discharged next Tuesday.  Then we could be heading home to California as early as the following week.  I'm so relieved that it looks like we will be back in time for Ethan to start school.  I missed the second half of his kindergarten year and that was hard for me... and for him, too, I imagine.

Thank you for your continued prayers and support.  They mean so much to us.

Day +95

Sadly, Josh and Ethan had to go home yesterday.  It's just the two of us again.  Having Ethan here did wonders for Jacob.  It helped him feel like a normal kid again, laughing, playing, bossing his brother around, and even walking a little.  Who would've thought how therapeutic a big brother could be.  I'm already missing my spunky Ethan, too.  We'd keep him in Seattle if we had another adult up here to help me.

We are still in the hospital.  The good news first: Jacob finished with his inhaled ribaviron treatment on Sunday.  On Monday, we found out that the adenovirus dropped from 2.2 million to 450,000.  It feels good to finally be on top of that.  I don't know if I mentioned earlier, but his c diff infection came back negative last week, which was also nice.  His tummy seems settled and he is eating wonderfully.  He is completely off the pumps, except for night time hydration.  If that were the end of the story, we'd probably be getting discharged soon, but...

Now for the bad news.  Jacob has another virus called Epstein Barr Virus, or EBV, coming back positive.  So far, he doesn't have any symptoms.  This is another virus that can reactivate due to a low T cell count and it was most likely passed on to him from his donor.  Last Monday it was 300, Friday it was 800, then this Monday it was 2800.  They haven't done anything yet, hoping that his body can keep it in check, but I'm not expecting much based on his history.  If Thursday's result comes back at 5800 or higher, they will have to treat with Rituxan.  EPV is found in the B cells and Rituxan will wipe out all his B cells for 6 to 9 months, giving his T cells more time to grow and mature.  Of course there are more risks involved with not having any B cells.  For now, they are keeping him on a low level of immunosuppression, hoping to keep the gvhd in check, while at the same time, giving his T cells enough of a chance to do their job.  It's a delicate balancing act that may or may not work.

We were supposed to be moving back home next week with Day +100 right around the corner, but it just wasn't meant to be.  I wonder if any patients ever actually get to go home on their 100 day mark.

Day +91

We got encouraging news yesterday.  Jacob's adenovirus count is finally dropping again.  It went from 9.4 million copies on Monday to 2.2 million copies from Thursday's sample.  He will be tested again on Monday.  For now, he continues on the weekly Cydofovir treatment and he also had to start on a respiratory Ribaviron treatment three times a day.  That has been something of a hassle, but glad that they are pulling out all the big guns.  In order to administer Ribaviron, he has to breathe through a mask hooked up to a ventilator and then have a clear plastic tent draped over his bed with a fan on top of it to clear out any extra medicine in the air.  He has to sit under the tent with his mask on for 2 hour intervals.  He then gets a 6 hour break, then has to do it again.  The middle of the night treatments have been the hardest.  He can't suck his thumb with his mask on and so has a very hard time sleeping through the treatments, even if it's 3am.  Thankfully, the treatment is only 5 days, so he should be done early Monday morning.

The highlight of our week has definitely been having visitors.  First, my parents and Ethan came up.  Then on the 4th of July, Josh flew up and is still here with Ethan.  Having family here has certainly perked up Jacob and me.  I have enjoyed getting breaks from the hospital room and spending some one on one time with Ethan.  

I came across this picture from Ethan and Dan's visit in May when Jacob was out of the hospital.  We were racing them into Target and it had me cracking up.  They were just too cute for words. 

Day +88

J's biopsy came back positive for mild gvhd. They have to keep him on some steroids to hopefully prevent it from making his viral infection worse. Jacob received a blood transfusion and ivig yesterday. He had a fever last night, but it broke on its own and he woke up happy this morning, asking to play with Ethan. Having them here has lifted his spirits so much!

Day +87

Our good news yesterday was short lived. J's adeno jumped from 200,000 copies on fri to 9,400,000 copies today. The cydofovir hasn't done a thing. They think the steroids boosted the virus, so they are putting him back on a tacro drip and getting him off the steroids. He also started on the higher dose, once a week cidofovir last night. I hope his kidneys don't get damaged. We should get his scope results today, but the visual looked good. Still praying for no gvhd so he can come off his other immunosuppressant, too. Being off immunosuppressants might give him a fighting chance.

My parents and Ethan are here for a couple of days. I thought I was holding myself together and staying strong throughout this whole thing, but when I saw my family at the airport, I wept like a little girl. I realized how much I missed Ethan and having my family together, and just the whole gravity of Jacob's condition came crashing down on me at once. I just needed someone to hold me and tell me it's all going to be ok. I didn't realize how alone I was. I know a lot of people pray for us and cheer Jacob on behind the sidelines, but I have just been very isolated in a physical and psychological way for a long time now. Other people my age are busy with chasing dreams, careers, traveling, getting married, being pregnant, and raising healthy children. I'm just fighting to keep my child alive. It's hard not to be envious, but I just try to stay focused on getting Jacob through one day at a time. It was never my desire to participate in the medical community. I never wanted to be a nurse or doctor. I wanted to be a mommy with lots of kids. Yet here I am, placed in this life, and I feel it is the most sacred of privileges to care for, fight for, and advocate for my little boy. You can never understand what it's like unless you've lived it. The only Ones who I know can truly comprehend the weight in my heart are my Heavenly Father and my Savior. It's been a learning process to turn my entire will and Jacob's fate over to Them. I've never cared or wanted something so deeply than to have Jacob made whole, but I have to accept that what I want does not matter if it's not Their will, and that is truly the hardest thing to learn and trust.

Day +86

No fevers last night, so that's good news. Other encouraging news is that they redid Jacobs abdominal X-ray and there is no sign of pneumatosis! That in itself is fantastic. I still need to talk to the doctor, but I'm pretty sure that means he can eat again. Now what I'm hoping for is that since there is no pneumatosis, maybe there is no gvhd and he will be able to get off his steroids. I can only hope and pray. We will have those results back by tomorrow afternoon. Jacob is back getting his endoscopy right now while I am in the waiting area. Being away from him when he goes through any procedure always makes me nervous. We will get another adenovirus count this evening. Praying that it is starting to clear up. Friday's results were worrisome.

Cuddling before his procedure

Day +85

Jacob had a good 36 hours with no fever. Unfortunately, he began spiking again last night.

He is still not allowed to eat or drink, which has been very hard on the both of us. It goes against all instinct to deny him food and drink when he is begging and begging all day long. I have to trust that the team knows what they are doing by putting him on gut rest.

This is all terribly discouraging. His recovery had been going so smoothly and then got hit with adeno, c diff, pneumatosis, and mostly likely gvhd all at once. His endoscopy is tomorrow, so we will get confirmation of the gvhd, but usually pneumatosis is a symptom of gvhd, so they already started him back at square one with the steroids. It's more than his fragile system can bear.

Yesterday I also found out that he has lost bone density due to the long term use of high dose steroids. He is on calcium supplementation, but it just isn't enough. This could lead to bone fractures.

I am feeling very defeated. The bad news just keeps rolling in.

Day +83

This day has gone from bad to worse.  Last night Jacob started spiking fevers and so they switched him over to a stronger antibiotic.

Even though he got GCSF yesterday to boost his anc, this morning's labs showed that it actually dropped ten points.  He got more GCSF today.

After two more doses of treatment, J's adenovirus count actually jumped back up to 200,000 again.  This could explain his fevers.  They will have to increase the dose size, frequency, or both.

All of J's c diff symptoms returned in full force today.  They switched him to a stronger med for that.

After a gut xray, J was diagnosed with pneumatosis.  It causes a lot of stomach pain.  He is getting switched over to everything iv- no food or drink, to give his stomach a rest.

This is why I am always holding my breath.

Day +82

We got Jacob's latest chimerisms today with more reassurance.  Sorry for the redundancy, but given Jacob's graft rejection last year, every time we get any kind of test results showing donor cells are indeed engrafted, helps me to breathe a little easier.  The current results are: nk cells, myeloid cells, and b cells are all 100% donor.  T cells are at 80%.  Jacob's doctor had warned us that they would be the slowest to grow in.  I don't know why it's so hard for me to rejoice in the good news.  I guess I always have my guard up waiting for the next trial with Jacob's health.  For example...

I was hoping to maybe get discharged soon, or at least to take him out of the hospital on a pass today, but last night and today Jacob starting running a low grade fever and complaining of stomach pain.  They ran cultures this morning, but so far, no new infections have been identified.  They also started him on a broad spectrum antibiotic just to be sure.  They will run it for 72 hours and if the cultures are still negative, they will stop it.  Other causes of the fever could be: the adenovirus, c diff, or a flare up of his gut gvhd.  The adeno and c diff are being treated.  He has a scope scheduled for Monday to test for the gvhd.  It will be nice to have some answers, but I really hope it's not gvhd, because I'd like to get him off his immunosuppressants.  This hospital stay is ending up longer than I had planned, but it's nice to have him watched so closely.  He slept all day and just woke up around 5pm, so we could be in for a long night.

Day +79

After a couple of rough days of tummy aches, Jacob turned a huge corner today. He was playing and eating again, which couldn't make me happier. It looks like the medicine is kicking that nasty stomach infection. Now we just have to hope and pray that it didn't flare up his gvhd so that he can continue to taper off the steroids and other accompanying drugs.

Speaking of drugs, Jacob was FINALLY able to come off a medication. His blood pressure was a little low, so they stopped one of his two blood pressure medications. We've only been adding drugs since we got discharged in May, so it's been nice to stop at least one.

Now onto the adenovirus. We got word back today that after just the one dose of medicine on Friday, the viral copies dropped from 230,000 all the way down to 88,000 today. This means that the treatment is working very quickly! They originally said it could take a week to see any effect, so this is wonderful news. He is getting another dose of the treatment as I write this.

The last bit of exciting news from today is that Jacob's neutrophil oxidative burst test came back completely normal. No more CGD. We already suspected this based on his chimerisms, but he hasn't had this test done since pre transplant work ups when he only had 2% functioning neutrophils, so it was just wonderful news to hear! Once Jacob recovers from transplant in about a year, he will have a normal functioning immune system for the first time in his life.

Thank you for your continued prayers for Jacob's complete healing and chance at a normal life. We love him so much!

Day +77

We are back in hospital. Jacob's adenovirus count was up to 230,000 yesterday (up from 37,000 on Monday), so we don't have time to mess around with the trial medication. Jacob is getting Cidofovir 3 times a week for the virus. I wish they would have just started him on it Monday when we first came in instead of wasting so much time letting the virus grow while waiting for the trial to get all set up. They will recheck the virus twice a week to make sure the medicine is effective. Just to clarify, the adenovirus was a virus J already had in his system from before transplant. He actually had it even before his first transplant and had it reactivate during his conditioning last year. Luckily the Cidofovir worked last time and we are hoping that it works again. When the t cells are wiped out during conditioning, old viruses, such as adeno, can be reactivated. Since J's t cells are still young and immature, the virus was able to reactivate without any effective way to be kept under control.

We also found out that he has a c diff infection, which has been causing him a lot of stomach pain. He is on Flagyl for that. Even though I put it through his ng this morning, it made him throw up.

Jacob's blood counts have been steadily declining the past few weeks. Platelets are down to 30, hematocrit 25 (getting blood today), and ANC down to 800. They don't know why. Could be the infections. I'm guessing we could be here as long as a week. They need to make sure the therapies are working before letting us do outpatient. Some of his day 80 work ups that were scheduled for next week can be done inpatient, but others will have to be rescheduled, delaying our return home a bit. Glad we are here where they can keep a close eye on him.

Day +74

Just a quick update:
We were discharged last night after a frustrating and physically and emotionally draining day. Ultimately we decided not to treat with iv Cidofovir because of its potential to permanently damage Jacob's kidneys, which are already being taxed by the tacrolimus. The other options were to do nothing to see if he got worse or remained non symptomatic, or to do a blinded trial oral Cidofovir called CMX001 which is supposed to be non toxic to the kidneys. We decided to do the trial for a week to see if it helps and then reassess from there. Since it's blinded, that means there's a 1 out of 3 chance that he is getting a placebo. Annoying, but we can ask for an open label if Jacob worsens or does not improve with treatment. This could potentially delay our return home depending on how many weeks it takes to clear up the virus. Jacob will also get an early ivig infusion tomorrow for some added protection even though he isn't due until next week.

Jacob is still having leg pain and falling down a lot. We've increased his calcium and he has a bone density scan next Wednesday.

Day +72 Part 2

I write this from the hospital. We got a call around 6:30, just as I was getting Jacob into the bath, that his adenovirus count went from 300 last week to 22,000 today and that they would have a room ready for him at the hospital at 7:30. He still doesn't have any symptoms, which is good, but they want to run some more tests to see if the virus is found any where else besides his blood. Most likely he will start Cidofovir tomorrow which is a weekly medication to wipe out the virus. I will get a better picture of his treatment plan tomorrow, but we shouldn't have to be here more than a couple of days. The doctor said that this virus is "as common as dirt" in transplant patients, which is why they screen for it weekly. We will still be able to keep our immunology appointment tomorrow since it is here at the hospital. Please pray that the treatment will be effective against the the virus. Jacob is still immunosuppressed and will need the help of this drug.

Day +72

This week we begin all of our work ups to get ready to be released to go back home around Day +100. They call them the Day +80 work ups, even though they span over the next few weeks. This week, in addition to our clinic visit and blood draws, we have a visit with the immunologist. I look forward to talking to him about Jacob's immune health outlook.

Overall, Jacob has been doing well. However there a few issues we are working through. One is that his blood cultures came back positive for adenovirus last week. They said it wasn't very much and we shouldn't be concerned at this point. They cultured him for it again today, so we will see what's going on with it. He hasn't really had any symptoms, so I think his body and medicines are keeping it under control, but we should hear back on the cultures in a couple of days.

Another thing is that Jacob's legs have been bothering him. His right knee hurt so badly on Saturday that he couldn't even walk. A variety of factors could cause this, but in my opinion, it's most likely from how sedentary he is. He doesn't like being active and spends the majority of the day wanting to watch tv. I know I'm guilty for allowing this, so I'm trying to get better at encouraging him to move around more. I also ditched the stroller when we go to clinic and pharmacy.

The last thing is just trying to keep his body chemistry and liver and kidneys happy while on so many medicines. The tacrolimus (immunosuppressant gvhd drug) depletes the body of magnesium. We have been increasing his mg supplement weekly because it keeps coming back low in his blood work. The tacrolimus and prednisolone cause high blood pressure, so he's still on two meds for that and is being monitored. The tacrolimus, voriconzole (anti fungal), and acyclovir (antiviral), are hard on his kidneys and liver, so his bilirubin and creatinine have been slightly elevated. And the prednisolone depletes calcium, so he is taking calcium supplements to counteract that side effect. Luckily, we are almost done tapering off prednisolone. It's due to finish July 3rd as long as his gvhd doesn't flare up. He comes off of tacrolimus 6 weeks after that. I look forward to cutting back on the amount of medications that he's on and hopefully even taking out his ng tube when he's down to 5 meds or so. Right now he takes 17 meds and supplements and receives 500ml of supplemental water through his ng, so it's still rather intensive. I'm happy things are moving in the right direction though. Thank you for your continued prayers!

Day+66 Jacob's Birthday

I can hardly believe my baby is 4! I feel so incredibly blessed to have Jacob in our family. He brings so much joy and love to all who know him. In his four short years on this earth, he has had to endure more pain and suffering than most people experience in a life time, yet he remains happy, kind, courageous, and strong. He has taught me what is really important in life. I love and adore him more than I could possibly express.  Happy Birthday, Jacob, and may the coming year bring healing and a new beginning!

Here's a little trip down memory lane:

Jacob's 4th Birthday (Pete Gross House, Seattle)

Jacob's 3rd Birthday (Children's Hospital Los Angeles)

Jacob's 2nd Birthday (Grandma & Grandpa's House)

Jacob's 1st Birthday (Grandma & Grandpa's House)

Jacob at Birth

Day +56

This week has been so fun having Ethan and Dan here visiting. I love how Jacob and Ethan don't even skip a beat. It's like they haven't been separated at all. I'm so grateful that they are best friends and that Ethan is an amazing big brother.

Yesterday we received some encouraging news on Jacob's latest chimerism. Both his myeloid and nk cells have remained 100% donor. His t cells grew from 76% donor cells last month to 80% donor cells now. And finally, his b cells went from 87% donor last month to 98% donor currently. If I may be so bold to say it, I think Jacob is going to hold onto his new marrow and be cured of cgd. Another chimerism will be done around day 80.

Other good news is that Jacob came off his nightly hydration infusion. The team wants to access his central line as little as possible and even talked about having it removed around day 90.

I am so grateful for our Father in Heaven's tender mercies. He has seen it fit to bless us so abundantly. We have an incredible, vigilant, top notch team of doctors and nurses. I could not imagine a more thorough, knowledgable, and caring team. We have the most amazing and supportive family and friends who have been with us every step of the way.

Here are pictures from our week with Dan and Ethan:

Bananas in Pajamas

Bubbles

Snakes

Best Friends

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.