Transplant Day report

Colleen here writing up this portion…

So yesterday was the big day.

I’m getting new bone marrow today!!

Caid went into surgery at 7:30 am to have his bone marrow removed. He had it extracted from the sides of his hip bones. They took 280 ccs–nearly a pint of liquid marrow–which is quite a lot as we were unsurprised to hear.  When he woke up the foremost concern on his mind was that he needed to play Wii…which made everyone happy!

post-surgery popsicle

run henry, run

Wren, Julie (Ginger’s sister), Henry and i arrived at CHOC yesterday around 11:30. We hoped Henry would nap on the way down but he insisted on talking to himself the whole time. First stop in the hospital was, of course, the gift shop…where we bought Caid a big stuffed shark, and Henry got his beloved balloon (his favorite word to say right now).

We had arrived just in time for Ian’s infusion with Caid’s marrow, and so once Henry and Julie were settled in the cafeteria Wren and i went upstairs to see Ian.

We walked into his room in the PICU to find Joey sitting and holding Ian. Also in the room were Papa Steve and 2 nurses, checking one of 2 bags of Caid’s bone marrow that was being infused straight into his body.

sweetest, most courageous one

He had vomited just before we arrived…from any number of things (chemo, exhaustion, etc) and was now fast asleep on Joey’s chest. We only stayed for a few minutes, as we knew how tired and immune-compromised he was…but we got to see his darling face and i was able to take some pictures.

snoozing through infusion

bag of marrow

checking the bag

…

A couple of hours later, after trying to find a place the family could all settle that night and finally getting poor Henry down for a nap, we went to see Caid. He was in a little room with his mom & dad, fresh from a long nap.

happy to have visitors!

time for pizza

i think this picture sums up pretty well why Caid is one of the braves, most awesome-est kids ever.

eating ice cream and watching dad play Wii fishing: “reel it faster, dada!!”

He loved his stuffed shark. Ginger asked him if he wanted to see pictures of how Ian is doing, and he nodded, yes. So i showed him the pictures i took in Ian’s room, including the one of the marrow drip.

“That’s your marrow, Caid!” i said. His eyes widened for a moment, and he nodded again. “I’m the one who had to give it to him, because he’s the only one that matches me,” he stated. Ginger smiled “and you’re the only one that matches him,” she said. “Because you’re brothers.” Caid nodded a third time.

More coming very, very soon~~cnp

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.

BMT Delayed

Ginger writes:

Wednesday we were at CHOC all day for our consent conference, surgery consultation for the broviac line placement and a clinic exam. The plan had been to admit Ian this week on Thursday, but with having a runny nose, it has been delayed.

Our consent conference was a final chance for us to talk with Dr. N, the director of BMT at CHOC, before signing the consents for Ian and Caid. Auntie Alice met us for the consent conference, since she knows the doctor and we always like to have her along to participate in the meetings when it works for her schedule. We feel very good about CHOC and our doctors there. The plan is to move forward with the Reduced Intensity Conditioning, with the day 14 campath, per Dr. F’s recommendation. Campath is a drug that they vary the timing of, depending on the situation for the reduced intensity conditioning – things like whether you have an unrelated donor, or a sibling. Something that is extra nice about Caid being the donor is that he has the same blood type as Ian, so there will less need to prepare the “harvest” (remove red blood cells, etc.)  All this means that Ian will be admitted 15 days prior to his transplant.

There is a countdown to the bone marrow transplant day, which means Ian’s countdown begins -15 until transplant day, which is day 0. From there, the days are +1, +2, etc. The first day he is admitted he will have a surgery to place his broviac line, and he will begin the preparative regimen (chemo drugs) for the two weeks leading up to the transplant day. From there it will takes time, usually at least several weeks, for “engraftment” which means that (ideally) Caid’s stem cells start to work and start making blood. That time period is usually very risky, because the patient doesn’t really have an immune system, and has extreme risk for bleeding, infection, and will need many transfusions of blood until the new blood system, or transplant begins taking over.

If the transplant is completely successful, Ian will have two DNAs – his tissues will still have his own DNA, but his blood will be Caid’s DNA. It is very possible that Ian will have something called “mixed chimerism” where he has some of his own blood cells along with Caid’s new blood cells, and the hope would be that Caid’s blood would be the higher percentage and maintain the majority in Ian’s blood system indefinitely.

Since Ian has a runny nose (along with Henry and Caid now) we had to delay transplant. At our clinic visit, they did a respiratory viral panel which would test for every major virus possible. The results take at least 48-72 hours, so depending on those results and how quickly Ian recovers, we may just delay a week until next Thursday. Basically, even the mildest bug can become opportunistic and dangerous if they wipe out his immune system, so he really needs to be completely healthy to go to transplant.

More soon…

Pre-Transplant Workup Done!

Caid’s bravery

Ginger writes:

We had a very long day at CHOC doing all of the pre-transplant workup for Ian and Caid, but they were great through all of the testing and we got everything done in one day. Both boys had exams with the doctor, bloodwork, and urine analysis. Bloodwork was of course the scariest part, but CHOC had a child-life specialist (fancy title for a totally awesome, nice person with cool toys) waiting for us to help keep Caid and Ian busy and distracted during the blood draw with an iPad. Caid did great and barely even noticed the stick and blood draw thanks to the iPad, but they needed a LOT of blood and he got dizzy and pale about halfway through. We took a juice and cookies break and a breather in the lobby outside the clinic while they worked on Ian. Ian also did ok thanks to the iPad, but it took three sticks to get enough blood to fill all of his vials.

All Ian

Caid came back and made it through the next blood draw like a hero. Papa Steve came by to help keep kids happy and entertained for more tests at St. Josephs. Both Caid and Ian had chest x-rays, and Ian also had an echocardiogram, which gave him the opportunity to fall asleep for a little while. They sent us back to CHOC for Ian’s EKG and then we headed back home. Special thanks to Colleen who watched Henry all day so that we could fully support our two guys through the day.

Moving Forward…

Ginger writes:

Things are starting to move forward quickly.

Caid and Ian will be having their pre-transplant workups at CHOC next week. We asked CHOC to have Dr. F, the foremost HLH doctor, consult on our case and we also have an appointment to see her in person at Cincinnati Children’s Hospital the first week of May. This is mostly to give me the peace of mind and reassurance going into this that we have the opinion of the doctor who knows about the latest advances in HLH and bone marrow transplant for HLH.

When I last spoke with our nurse at CHOC, the plan is to go into the hospital the second week of May to place Ian’s broviac line and begin pre-conditioning treatment, with June 1st being the transplant date. This may change, and the doctors are still finalizing plans for timing of one of the drugs, which will affect things too, but we know it won’t be long now either way.

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.

Important Update on Ian’s treatment…

Ginger writes:

looking at animals~

I apologize that I haven’t been keeping updates coming frequently. Here is what has been happening in our HLH fight.

Two weeks ago, we met with the Bone Marrow Transplant team at CHLA for the first time, since we officially got a referral for transplant now that we found our donor, Ian’s brother, Caid. We had many questions, but I felt that I didn’t have a clear answer on something called “Reduced Intensity Conditioning”. In a bone marrow transplant, the patient (Ian) would be given chemotherapy to wipe out the existing bone marrow, which is diseased. This chemotherapy is much stronger than what Ian is currently getting, and Ian will have to admitted and kept completely isolated and closely monitored while it is done, prior to the transplant.

**This chemotherapy is so strong that it has a risk of death (from a number of complications), or at the minimum, long term side effects that run the gamut of infertility, organ damage, learning delays, etc. Basically, there is a lot of toxicity with this chemo, since it has to be strong enough to wipe out his blood making system and blood cells.

In preparation for the meeting with the transplant team, I read a little about two different types that are done with HLH patients. Myeloablative is the harsher preparative regimen and Reduced Intensity Conditioning is a less toxic, newer approach. I found several medical journal articles written from the doctor at Cincinnati Children’s Hospital who is at the forefront of research on HLH. These articles said that in Cincinnati (although it was a small number for a study and a single center study), that they had a much higher survival outcome with the Reduced Intensity.

To us it seemed that if there is something that provides a better chance for Ian to survive the treatment, we had to investigate it. We had another meeting at CHLA last Thursday to discuss these articles and found out that CHLA does not offer Reduced Intensity Conditioning, although they offer a lower dose Myeloablative regimen. There are risks and benefits to each regimen, and basically CHLA as a center isn’t convinced about Reduced Intensity Conditioning. We decided that we would like to get a second opinion from Cincinnati Children’s Hospital, and that is in the works.

In the meantime, our Auntie Alice, who is a MD and has been our personal physician consultant in all of this, called a friend who just happens to be the director of Oncology at CHOC. He put us in touch with a BMT doctor at CHOC who talked with Joey and I about all of this. We came to find out that they offer Reduced Intensity Conditioning at CHOC and plan to have a formal meeting with them sometime this week.

**Please pray for our wisdom as we are making the decisions for which doctors to entrust Ian’s care to, and of course for his treatment. As scary as all of this is, we are in the best possible position since Ian’s HLH is in remission, he has no Central Nervous System involvement, and has a sibling bone marrow donor. We just know that it is essential to expedite this and get him to transplant as soon as possible.

Bathtime screaming smiles!!

~please check back soon for more information~

New month…new update~~

Sorry for the lack of updates…the family has been mostly “maintaining ” for a couple of weeks now since Ginger met with the BMT (Bone Marrow Transplant) team. We know that doctors would like to begin the process within the month…so this could be coming up very quickly.

Ian had his Chemo last weekend, and this past Friday had his steroid treatment. As I’ve said before, the steroid weekends are the most difficult because the drugs have such a physical and emotional impact on Ian. We’ve found that singing is a good distraction for him from his agitation…he even tries to “sing” along sometimes!

Walking! And also growling like a tiger.

G has been trying to get physical and speech therapy for Ian since they came home from Phoenix 2 months ago, and of course it’s been like dragging a boulder uphill to get the services set up.  But he’s managed to start walking on his own again for the first time since Christmas…so that’s been really sweet to see! Ian finally has started receiving his speech therapy for feeding and physical therapy should be getting scheduled soon.

We are trying to enjoy and be grateful to see him gain ground as much as he can, because he may regress physically again once the transplant process has begun.

This Thursday Ginger and Joey will be meeting with the Bone Marrow Transplant doctor at CHLA in the morning to discuss the preparative regimen (chemotherapy to wipe out Ian’s Immune System/Bone Marrow) prior to the transplant. More info on that as it approaches.

*Also on Thursday, is the Benefit for the family, which is being wonderfully and generously hosted by Bento Box. Please read about it and pass along the information…it should be an amazing night.

**Lastly, for those that have been sending mail to the PO Box, it’ is getting to us! Thank you so, so much for you blessings!~~cnp

Ginger’s Report from CHLA Doctors, Bone Marrow Transplant Team and more…

This day was made possible by the generous help of auntie Anna (our in hospital babysitter for Ian and Caid) and auntie Carol who babysat Henry and helped me till bedtime so Joey could go back to work! THANK YOU both.

We had a good meeting with the bone marrow transplant team. A flurry of information and I am working to process all of it.

Ian the strongest, fiercest baby in the world!

We met with a BMT doctor, nurse and a social worker. The meeting basically covered the main risks and complications of BMT, how they address those, and how the process works. The good news is that the survival outcome for Ian is greatly improved and all of the risks of BMT are less likely to be as severe by having a sibling donor. Discussion of things today were very theoretical, meaning that the timeline, the preparative regimen, the drugs he will receive, etc, can change depending on so many variables, that we have many steps to take first.

Caid the little puppy (or chameleon, or hedgehog)

There are MANY tests that Ian will need prior to transplant, and all have to be done within a 30 day window prior to the transplant date. Caid will also need his workup to be coordinated and all of his tests will have to be complete within the same window. We found out that Caid will have to have the surgical procedure to collect the bone marrow and that they will do a double aspiration (from both hip bones). Caid will be admitted to the hospital and stay overnight. They will try to have Caid and Ian’s rooms as close as possible, and will do the collection of bone marrow and then Ian will have the transplant within hours.

The swallow study went fine. Ian graduated from “honey-thick” to “nectar-thick” liquids. Sheesh these names sound silly. What this means that he still needs to be on thickened liquids, but he took a step in the right direction. I was pretty disappointed, but the Speech Therapist said that for what he had been through, that he had made a great improvement and that they would need to follow up again in another three months. !!!!! I couldn’t believe we would have to wait that long, but we have a lot of other more pressing matters first, so I am going to focus on just getting through the BMT process.

Our speech therapist wasn’t sure how this outcome works with BMT, but we will get coordinated once we have made our rounds around the hospital. We follow up with urology next week for all of the urinary tract stuff with our wonderful Dr. K.

Thanks everyone for your love, support and prayers.”

Update from Ginger (and some pics)

Ian’s arm (where his PICC line is inserted) is all wrapped up and he’s ready for a bath!

“Here is my belated update…. last Friday we had our chemo and appointment with our doctor at CHLA. Everything went well; the appointment pretty much takes all day, we were there from 10 am until 4:30 pm for vitals, blood draw, doctor exam, PICC line dressing change, chemo infusion which is 2 hours.

The doctor anticipated my asking about the gene test results, he had checked and they are not back yet. He promised to notify us as soon as they are received. I am on pins and needles waiting for them, hoping that the results will identify a gene mutation so that we can test Caid and Henry, then hoping that they will not have HLH, then hoping that Caid is a great match for Ian as a bone marrow donor. The doctor is wary of Henry being a possible donor, since he got so sick with the same virus as Ian and required hospitalization for almost a week.

Today was a big day. Joey went back to work and Ian started “cruising” – for you non-baby speakers, that means standing and taking steps while holding on to the coffee table! Another big physical accomplishment for him. I survived Joey’s return to work since I had help from Colleen in the morning and then Carol this afternoon and evening! I have more help coming from Auntie Robin this week, which is great, since Ian continues to be emotional and easily upset from the steroids. Henry seems to be noticing all the extra holding and attention I am giving Ian and wants to be held and hugged too. (:”

Henry shares a rare seated, quiet moment with Ian (:

Henry runs around pushing things, while Ian waves around a pedialyte bottle.

Henry teases Grandma Tina while she feeds him