Day +12–ANC count up, details on Ian’s care

Ginger writes:

Ian update: The least amount of sleep on record last night for poor Joey and Ian.

Ian was up until 11 pm last night, and then woke up before 2 am and was up until 5 am when he received more benadryl. We think it is a combination of the steroids and cyclosporine, which make him agitated (therefore hard to sleep) and the fact that he had received benadryl yesterday morning for his cyclosporine rash, which made him take a long 3 hour nap yesterday during the day. Joey said he had a hard time getting him to sleep tonight too, they were up until 10 tonight.

our fighter!

In better news, Ian’s ANC count was 2100 today (yes that is right, no typo)!!! Obviously over 500 for today. That means that if his counts are still over 500 for tomorrow, that he can get out of his room. He can’t really go anywhere public because he is immune suppressed, but it would really help to help pass the hours and get him/us some change of scenery. I realize now that I haven’t really described what the quarantine fully entails, for anyone who is interested. Feel free to skip all this….

All his stuffed animals, blanket, bumper pad have to go, or be laundered every 24 hours. (We chose to send them home). He has a special bath, scheduled every day. During the bath, cleaning services does a special BMT cleaning of the room, where they wipe every surface possible, (including the ceiling!) to disinfect everything, change the linens, and we have to stay in the bathroom until they are finished. It takes about 40 minutes, but it is so worth it. It makes me feel so good to know everything is clean.

how much longer do we have to wait?

tired of this.

We do a special oil in the bath to help care for his skin and no baby soap allowed, just Cetaphil. We pat him dry (no rubbing) and then slather him with lotion and lots of A&D in the diaper area. All this because chemo kills the quick reproducing cells – like blood cells, but also causes breakdown of skin, slower healing time, and hair loss (which has been happening the last few days, his sheets and our shirts are covered with baby hair and baby eyelashes). There is also a special diaper change procedure. We have two bottles, one with sterile water and one with Cetaphil soap/water mixture and we alternate these and then pat dry again.

So… please pray with us that his ANC will continue to be over 500 tomorrow, but more importantly, that he will continue to do well on the long road to full recovery.

quick update on Ian’s day

Ginger writes:

Today Ian has been much better. He has only eaten a few bites for the entire day and he still looks pretty sick and is wiped out, but all that is to be expected.

His ANC, which is an important blood count they watch, dropped from 800 yesterday to 200 today. Anything under 500 means we are not allowed out of the room, but we would have been quarantined starting tomorrow anyway. The drop means the chemo is working to wipe out his bone marrow and blood system in preparation for transplant. So that is good results for the goal we are working toward, and also explains why he feels so bad.

Caid’s surgery is scheduled for 7:30 am tomorrow, and Ian’s infusion of the cells will probably be around 11 or 11:30 am tomorrow.

Difficult Day

Ginger writes:

Ian just fell asleep now after a pretty miserable day.

He tossed and turned all night last night and I woke up to him wretching at 4 am. He wasn’t throwing up much, since he hadn’t eaten much, but he kept having waves of the nausea. He got back to sleep from 6 until 8 am, but then woke up the same way. We gave him a dose of Anti-nausea medication zofran a little early to help, but it didn’t really work. So next we gave him Ativan, which made him super hyper, but he ate and drank a little. Next they gave him a much stronger anti nausea med which i was told is more specific for chemo patients. He wouldn’t nap until late in the afternoon, but really crashed.

After two hours, I tried to get him to wake up, and he really wouldn’t rouse out of sleep. I waited a half hour and tried again, and called the nurse to ask her opinion and find out if any of the medications would be causing it, which they shouldn’t have been at that point. She checked him and spent a lot of time talking to me and reassuring me that he was probably just really wiped out from the chemo.

I waited another hour and then tried again and then really started to get scared that he was so lethargic. He eventually came around and perked up a bit, but then became really nauseated and inconsolable again. As much as I didn’t want him to have more Ativan, I really felt like it would be better than him being miserable. So we did another dose and he got crazy hyper again (which is actually the opposite of what it is supposed to do.)

We are done with the chemo, today was a rest day and so is tomorrow, but the stuff he had yesterday is really a strong and notorious kind of chemo, plus I have been told that sometimes there is a cumulative effect from having 6 days straight chemo prior.

I tried to think of it today as “working” to get ready for transplant, but it is so hard and exhausting to see Ian suffering and I get so scared now when my kids act super sick. It is especially hard since he is little and can’t tell me what is going on or what he is feeling so I am left to guess whether it is typical BMT related sickness stuff or some scary complication.

I am very thankful for the kind and patient nurse who took my fears seriously and spent a lot of time with us today. More tomorrow…

Transplant day -3

dada’s phone is pretty cool

Ginger writes:

Today Ian got his first and only dose of Melphalan, the last chemo drug before bone marrow transplant on Friday.

His appetite has been slowly waning and tonight he didn’t really even want his bottle (which is usually something that he really loves and a big source of comfort for him). They are just keeping a close eye on his weight and caloric intake. Interestingly, patients going through bone marrow transplant need almost double their daily calories and protein, so they tried getting him to take a high calorie supplement drink today. He took the first one, but when I tried sneaking some in with his milk tonight, he wouldn’t have it. He didn’t do much with his regular milk either, since he’s probably just feeling nauseated.

Caid and the alligator lizard

Last night I talked to Caid about how he is going to help Ian since we had another blood draw, exam and a tour of the pre-op area scheduled for this morning. I told him he will help by giving Ian some of his healthy cells, since Ian has sick cells. Caid’s blood draw today was the third in the last couple of months, so he was pretty savvy about what to expect and he was not happy about it at all.

Papa Steve babysat Ian and Julie stayed with Henry at the house, so that Joey and I were both able to be with Caid for the appointment this morning. He made it through and got his clearance for Friday. He will not be going to school this week to avoid germs so we can go ahead on Friday with the transplant.

Thanks everyone for dinner help, prayers and babysitting!

day of rest

rest day & scooter ride…how much better does it get?

Ginger writes:

Last night Ian had a low heartrate while sleeping, which had been happening the last three nights. Everyone thinks it was likely from being on steroids, which can cause high blood pressure, so the body compensates by slowing down the heartbeat. They kept a close eye on it and did an EKG, which was normal.

As of today, we are done with campath, so we had a day of rest with no chemo, steroids or benadryl. He still had to take a few meds, but nothing that bothered him.

Tomorrow we begin a different chemo/immune suppressive drug, fludarabine.

Henry & fam visits Ian, etc…

Ginger writes:

We just had a very nice visit (which made Caid especially happy) from my Dad and Teri the last two days. My sister Julie also came into town to stay with us to help with Caid and Henry for the next couple of weeks. Joey has been at the hospital with Ian the last couple of nights, but we all went down to the hospital to see Ian while Caid was in school today.

Ian was very happy to see Henry, it was so precious. He was laughing and reaching for him when Henry came into the room. They didn’t get to play together for too long, since Ian had lots of appointments with therapists to attend to, but it was nice to have them together for a few minutes.
Ian continues to be doing well. He is tolerating the chemo with the medications for the side effects and only has one more day of this particular drug, campath. Then we move on to the next one.
Thanks everyone for your love and prayers.

Better Day

Ian’s labs came back showing no signs of infection- so it is definitely just a reaction to the chemo. The medications to help with the reaction are working great and Ian is feeling pretty good again.

~Thank you all who have been praying ceaselessly…we are so grateful!~

update on previous post…

Ian is doing better, they have him on round the clock medications to minimize the symptoms. The doctor thinks it is a reaction to the chemo, she says this one in particular is a nasty drug. Thanks everyone for your prayers.

Ian very sick, hopefully from Chemo

Ginger writes:

Ian began his Chemo yesterday, the first day of the 2-week regiment before the bone marrow transplant.

Sweet guy knocked out with the benadryl and tylenol they gave him to help with any reaction.

Ginger wrote this morning: “Ian is not feeling well. Last night he spiked a high fever at about 9:30, six hours after they gave him the chemo. He had severe chills, rapid breathing and vomited a couple of times. This is not an uncommon reaction to the chemo drug he received, but they cannot rule out infection, so they started a broad spectrum antibiotic, changed all the IV lines and his surgical dressing and took blood to docultures. They gave him Demerol to help with the chills, more Tylenol for the fever and more benadryl, steroids and Prevacid to help with the reaction. He slept most of the night with all the mess and woke up without fever, but everything started again after being awake a little while, probably because the meds wore off. The doctors will now have him on the medications to help with the reaction and fever around the clock, and they are going to be monitoring him more closely with vitals every two hours instead of every four. They also have him hooked up to monitors to watch heart rate, respiratory rate and oxygen saturation.”

Update: new answers from CHOC

Ginger Writes:

Last Friday, we met with the doctor who is the head of Bone Marrow Transplant at Children’s Hospital of Orange County (CHOC) and the rest of the BMT team there. We came away with a much better feeling about the plan. We had clearer communication from the doctor and nurse there in answer to our questions and from what we discussed, we feel more certain about moving forward using Reduced Intensity Conditioning.

That is not to say that there are not risks involved with Reduced Intensity Conditioning or the BMT itself, there most definitely are still side effects, both short and long term and risk of death, but with Reduced Intensity Conditioning, the risks associated with the toxicity of the chemo is minimized, since it is not as strong. *With RIC, Ian’s HLH disease will not be as effectively wiped out by the chemo they give prior to the transplant because the chemo isn’t as strong. However, because it isn’t as strong, the risks of complications related to the chemo are minimized.*

In the articles about RIC vs MAC (Myeloablative) the doctors discuss that kids with HLH are seemingly at a higher risk than most BMT patients for developing veno-occlusive disease (VOD) which affects the liver and pneumonitis (non-infectious), which affects the lungs, and these diseases happen from the chemo drugs given to prepare the patient for transplant. This is doctor speak for saying that the liver and lungs are already more susceptible, since HLH attacks and damages these organs when it flares. So with RIC, we minimize these risks, although these complications are still possible. The drawback with RIC is that it does not wipe out the disease as effectively.

With RIC, we are relying (and hoping) that the donor’s system will replace Ian’s diseased blood system, but because Ian’s system is not completely wiped out, we may get something called “mixed chimerism” which means that Ian has blood made up of some of Caid’s cells and some of his cells. I’ll explain more about that later. Basically, we want the Caid cell numbers in Ian to be high or at least the majority and holding. If they begin to drop, then Ian may need something called a Donor Lymphocyte Infusion. This is not unusual with RIC, and this was the main reason that CHLA told us they are not on-board with RIC. A donor lymphocyte infusion would be a blood draw they would take from Caid to get the lymphocytes from his blood, and they would give them to help boost up Ian’s “Caid/donor” numbers. When they do this, they have to pull back on immune suppressive drugs they give Ian to help prevent the dreaded graft versus host disease, another potential complication of transplant.

All this is obviously pretty complicated and scary stuff which is why we were feeling so unsure about how to proceed previously. That said, it seemed to us that a much higher survival rate with the RIC was the most compelling thing. It certainly hasn’t been exhaustively studied, but it seems like that may not be a possibility since HLH is a rare disease. It was important to us was that we get a recommendation on the treatment from a facility that offers BOTH types of preparative regimens, so we know we are getting a recommendation for what is best for Ian and not for the only thing they offer.

The doctors at CHOC said that the evidence for increased survival rate can’t be ignored and that any complications that come with RIC are ones that you deal with, since you are giving your child the best chance for survival, and minimizing toxicity at the same time. Because Ian doesn’t have active HLH or central nervous system involvement (both of which would exclude him from RIC) and has a sibling donor, he is an ideal candidate for the reduced intensity. We feel very hopeful and are glad that we have found a place that we can get reduced intensity that is close to home.