Day +100

What has been accomplished in the last 100 days is nothing short of miraculous and the process to get here has been nothing short of terrifying. I think that tumultuous is a word that fairly describes my relationship with the SCCA.. I wanted nothing to do with this organization or the people who worked within it when all of this began, I was scared, angry and really in denial of what needed to happen. Today, gratitude was certainly the message I wanted to communicate. The team that has spent the most time with us outpatient (and some inpatient) were all there today, all commenting on how good Georgia looks and how her wiggles and smiles will be missed. I am so grateful to especially these four people who really care about Georgia and have been thoughtful about her individualized care. The hope is that we will not need to be seen at the SCCA until January for our one year post transplant follow up.
Throughout this process I think I have been seen as far more assertive than I see myself, but in the end I think they all know I am just a mother who loves her daughter. Rare diagnosis and disease aside, I think Georgia has made a mark at the SCCA.

Me, Dr. Carpenter, Nurse Dawn, P.A. Joan & Georgia

Nurse Suni, Dr. Carpenter, Nurse Dawn, P.A. Joan & Georgia

Review Conference

We're here, we have come to the point post transplant where it is time for a review conference and that feels huge.
Yesterday Georgia had an MRI on her left leg, the place where her body first showed us that something was terribly wrong. For the past several weeks I kept looking at her leg, feeling it, analyzing her movements and praying that we would get the results that we were hoping for. Naturally things at Children's were running late so we found ourselves wandering the halls... I was walking Georgia up and down and on our way back stopped in front of the Radiology waiting room.. I looked into this empty room at the very chair Georgia and I sat in back in October, I still have moments where I still can't believe this is our reality.
After her MRI we had a clinic visit at the SCCA, the current attending is not exactly what I would call a cheerleader.. her comments have been, "so it looks like Georgia has finally gained a little bit of weight," or "are you doing speech therapy, I haven't heard her talk much" ( for all of the 10 cumulative minutes you've spent with her that's shocking isn't it?). Yesterday was no exception she said they'd let us know when the MRI results were in because "we're all very curious what they will be"... this coming from the doctor who really wanted us to do radiation on her leg. All evening I kept checking the clock and my phone - it was too late for anyone to call with results.
This afternoon we went to the SCCA and sat in the same room where we had the pre-transplant conference, now, here we were reviewing all of the work that has been done. We met with the same attending who lead that first conference, it felt good to be wrapping things up with someone who knows all of us so well. He took us from the conference room to one with two computers, there on the screens were two MRI's one from October and one from yesterday. As he reviewed the pictures with us I payed attention to everything, the pictures, his tone, his expression and of course the words coming out of his mouth. He said that ultimately there are still abnormalities but that after closely reviewing them and consulting with the orthopedic surgeon who has worked with Georgia since the first biopsy (and who was called out of the OR to review these results) and with our Oncology doctor, all agreed that this was most likely a healing leg. The tumor that was on her leg was so aggressive it's hard to believe it would be there and not be destructive like it had been. This of course is all a theory, we will need to keep a very close eye on her leg so she will have another MRI in one month.
Now our true review of the data could begin, all in all the attending said the late VOD was the only "hiccup" during the transplant... he acknowledged that we of course likely felt differently about the process. All of her Day 80 results show 100% donor and her GVHD seems to be well controlled with steroids. We talked about tapering three of her drugs, both of the steroids and one of the immune suppressants over the next two months. For the first time I asked how do we move our lives closer to normal. Over the next three months as we taper these drugs it will show a lot about her new immune system and if GVHD will continue to be a factor. We were given the ok to play with our (healthy) cousins which will be so good for Georgia, she has missed out on normal kid interaction. We were also told that because she was able to avoid total body irradiation she isn't at an increased risk for skin cancer which is a huge relief. We will still need to be very cautious for a year after she is off of any immune suppressants as a sunburn can activate skin GVHD but overall we should treat her like any light eyed, fair haired toddler in the sun.
We have one more clinic appointment with the SCCA then will transfer back to Children's Hospital where the docs will watch over Georgia's continued healing. We still have a very long way to go in this marathon but it feels like maybe we're hitting our stride.

Happy Easter

Day +92

Ah Georgia girl, time is flying so fast, you turned 17 months old yesterday! You are on the move these days.. you no longer want to be carried everywhere, you want to "wa, wa, wa" while holding my fingers. You are fast on your feet with your walker, my fingers or cruising furniture but whenever I ask you if you want to walk on your own you smile and shake your head no. You take a few steps here and there and will stand in the middle of the room dancing, moving your arms and legs so I know you have the balance and strength, just not the interest yet... I think you really may go from crawling to running.

This week we got your final day 80 bone marrow test back.. 100% donor, fantastic news! You also have done a great job in continuing to eat and weighed 10.089kg at our last appointment which means so long TPN!

You don't miss a beat, always mimicking what daddy and I do and you understand what we're trying to teach you... last week you opened the cabinet under the kitchen sink, looked inside and said "me, no, no, no" all while shaking your pointer finger. You are communicating more with "ya" and big head nods for what you want and you call all food "nana". Lately you've been saying "baby" and pointing them out when you see them. Despite having to wrap your lines and tape up your chest at bath time you love the water.. dipping your face in and asking for more after I've dumped water over your head to wash the soap out. You continue to be the best entertainment that daddy and I have ever seen, throwing your head back when you laugh, still waving and making people smile... and of course dancing!

Here is a little video of an ANC dance you did for your buddy Hunter, and wouldn't you know it, the next day his ANC was up and he got to leave the hospital!

Day +87

What a week! We started off with our Tuesday clinic visit - I was sure with Georgia's improved eating that her weight would meet our goal.. her previous weight was 9.87kg.... I didn't take my eyes off the scale as it jumped around.. 9.89kg - what?? Georgia did such a great job eating, I was so disappointed that we didn't achieve bigger results. Weight aside her eating was much improved so they decided to cut her TPN calories in half and reduce the volume to hopefully spur on even more of an appetite.

Throughout the week Georgia's Day 80 test results have trickled in, we're still waiting on the cytogenetics test but what we know so far is:

Peripheral blood chimerism - 100% donor

Bone Marrow aspirate - no blasts, 100% donor

Skin biopsy - inflammation but no signs of subcutaneous GVHD

I was told pre-transplant that it would be pretty easy to check Georgia's blood and bone marrow for what is hers and what is from her donor... why? Georgia's donor is male, she is obviously female so they look for just that.. we want to see male cells. For the rest of her life if Georgia only has blood test done it will appear that she is a male... if she wanted to have genetic testing done she will need to have her skin cells tested - her blood cells are now her own but they will always have originated in someone elses body. Bone marrow aside Georgia is all female, the transplant doesn't make her hormones male. This just scratches the surface of the complexities of transplant but even more so how fascinating the human body is.

This morning we finished our week with a clinic visit, again glued to the scale everyone was very happy to see 9.95kg - we're so close to our goal! In the words of the nurse practitioner.. "Georgia is doing fantastic". With the addition of Sirolimus her ANC and platelet counts have been closely watched as this immune suppressant can effect these levels. Georgia's ANC is solid and stable... her platelet count, although a great number has been dropping slightly since starting this new drug. We are still in wait and see mode but my hope is that her platelet count stabilizes as this really seems to be a better choice on several levels.

Next week we continue to have visits to wrap up our time at the SCCA, the week following is a big one.. Georgia will have an MRI on her left leg, the first since it was determined that we would not do radiation because biopsy results showed dead cells. The docs will watch her leg very closely over the next couple of years, her body needs to rid itself of the dead cells which takes time (possibly up to two years) but also because an AML mass tumor like she had has a high rate of recurrence. We will also have a conference to go over in detail all of the test results and essentially discharge information... come May we'll be back seeing our doctor at Children's Hospital.

Day +82

This last week my sister came with me to Georgia's bone marrow aspirate and other procedures. While we were waiting to been checked in she met several of the families who I have shared this journey with - it felt so good to introduce my sister who is such a huge part of my life to these people who have become my new support system. Since Leslie has two young kids we haven't been able to spend nearly the time together that we're use to.

That evening I got an email from her, among other things it said: "It was interesting to get a glimpse into your world. It seems like there is a bond between the moms, you all know things no one else does." I can't tell you how true this is on every single level. There is a camaraderie for sure but it's more than that - we don't judge.. we listen, we offer opinions when asked, give a shoulder or a pep talk but we don't judge because we know that living this reality means that it's your own story, not one that you would comment on how you'd do it differently.. we all do things differently but we're all writing different stories.

If you are a parent, especially a mother you know about a place inside that hurts because you love your child so much. You want to do your best not only for them but by them. This is where I start each day, not on a wholly conscious level, but this is where as a mother I begin.. I take this with me to each doctors appointment, procedure, conference, lab draw, xray - all of it. I am not writing someone else's story, I am only writing ours.

Day +81

Things here have been busy, aside from the regular visits to the SCCA I am told that I am raising a normal 16 month old... she's testing her limits. Overall I definitely celebrate this, in the moment... well that's another story. I am working on defining how we will do discipline at this young age. Georgia is feisty.. and smart, she knows what she wants and isn't afraid to go after it but figuring out how to communicate what is appropriate while knowing there is a caveat.. she is on low dose steroids is challenging. It's a work in progress but is also exciting to see her learning right before my eyes.

This week Georgia had her Day 80 bone marrow aspirate and biopsy as well as a skin biopsy and chest xray. We are hoping and praying for the same results we had at the Day 28 tests.. 100% donor, no blasts, no abnormal cells. Friday we had our Day 80 clinic check up, from all exterior signs Georgia isn't showing any signs of chronic GVHD. Her eating has picked up and our last two visits have shown weight gain.. we need to get her to 10kg (22lbs) before they will take her off of TPN but we're closing in on that.. she was 9.87kg on Friday.

Next week one of our visits is a follow up dental exam.. they said beforehand that transplant can delay dental development... Georgia has had six new teeth pop through since that visit.. this girl is unstoppable!

Day +76

Ahh... that is the sound of a slight exhale. Today we had a good appointment and Georgia's weight was up! Her calories were up and her weight went from 9.68kg to 9.77kg - not a huge jump but certainly a sign of improvement. I knew she'd been eating a little more but she is moving more too so I was thrilled to see the results. The team was pleased and we are sticking with our current course of treatment, her counts look good and for the first time we talked about moving toward day 100 when we will transition back to Children's Hospital. The doctor said Georgia isn't showing signs of chronic GVHD, something that typically shows up as dry eyes, mouth, or on the skin and nails. We talked about tapering her immune suppressants and that through that process we'll be seen at both Children's and the Continuing Care Clinic as the SCCA at they are the experts in GVHD. It felt so good to looking toward the future. Today I will enjoy this, today I will take a breath, have some wine and squeeze my baby girl.. I will wait until tomorrow to start worrying about the chest xray, bone marrow aspirate and skin biopsy that she has on Thursday.

Day +74

Six months ago today was the last day that I would consider my life normal. Six months ago tomorrow Georgia and I swung by Children's Hospital for a quick xray, we waited in a little room for the go ahead to go home, instead someone came in and said it looked like she had an infection or tumor in her leg then we were walked straight over to the Emergency Department. I mourn the loss of life that was normal, it will get better in time but will never, ever be the same.. my worry will always be a little more than the average mom and I imagine I'll cry a little harder at her pre-school and college graduation. What we know today:

• JMML is a rare form of leukemia and accounts for 1-2% of childhood leukemias each year; this equates to about 3 cases per million children.


• About 80% of JMML patients have some sort of genetic abnormality in their leukemia cells that can be identified with laboratory testing - Georgia has tested negative for all genetic tests that have been offered.


• Boys are effected by JMML more often than girls.


• JXG (Georgia's skin condition) Due to its infrequency and misdiagnosis it is hard to get an exact number but it is estimated that skin JXG attacks 1 in a million children.

I think about what a million people looks like - Quest Stadium holds 72,000 so take 14 Quest Stadiums and ask three children to step forward, this is JMML... that's insane.

Georgia has been to hell and back through this process and so have we, we still have a long way to go and still have a lot of unanswered questions but our daughter is simply remarkable.

Day +72

Today was.. exhausting. Brian left early for a trip so Georgia and I were on our own for our clinic appointment. We had our routine blood draw then headed upstairs for our date with the dietitian. I knew the food records that I'd filled out over the past three days weren't enough.. not enough to cut back on TPN, not enough to get us where we need to be going. I was candid with her and said that I'd hoped we would have had several days like we did on Monday with over 600 calories but that just didn't happen. Thankfully Georgia's weight was up by about .04kg but still down .005kg from our first weigh in.

When the team came in we talked about the fact that Georgia needs to be gaining weight, that the current drugs aren't solving the whole problem although they have helped. I guess I went into today's appointment thinking that the next step was clear, we'd start the Sirolmius that we had talked about during our last appointment. Instead I found myself smack dab in the middle of conversation talking about several different "options".. the NG tube, Prednisone, Budesonisde (a topical steroid that treats lower gut GVHD) as well as Sirolimus. We talked about my pros and cons for each.. I said I wasn't sure why we wouldn't do the Sirolimus, it has shown in a small study to be effective against mild GVHD as an alternative to Prednisone and although it isn't protocol there might be a secondary benefit that it would combat JMML.

The current attending doctor is the same one we had going into transplant (yes, former said ass whom now we are thankful to have on our team) so he knows us well. He said that he would not start Georgia on Sirolimus to combat JMML that idea is still a theory and not practice (yet?) but that he would consider it because of her GVHD based on the study. Ultimately he said if the time comes when we need to do Prednisone I will let you know but right now I can honor your request to be a part of this process and ask you what you'd like the next step to be... ummmmm is all I could think. I am an advocate and want to be part of this process but I'm not dealing with the same depth of knowledge that everyone else in the room is... I took a breath and didn't allow myself to hem and haw like I wanted to - Sirolimus. The PA also said why not see if we can get her to take the Budesonide (it's a capsule that has to be broken open, put into food... then hopefully she'll be willing to eat) to see if maybe there is a little bit of lower GVHD... just higher than the sigmoidoscopy could show.

I left with my head spinning.. called Brian, this is what happened, did I make the right decision? Called my sister... did I make the right decision? Called my parents... did I make the right decision? There is a voice in my head that occasionally says don't screw this up (we'll that's the PG version) and I kept wondering... am I screwing this up? What I know is this, first off the doctors aren't going to give me any options that aren't viable, second, yes I did choose the one that isn't standard practice but at the end of the day if Georgia doesn't gain weight by our next appointment she will be put on Prednisone. Lastly, every fiber of my being says that we are going to do this and be done with it... move on with our lives but in the chance that JMML were to come back I would always ask myself what would have happened if we tried the drug that "in theory" combats JMML.

Late this afternoon Georgia and I did some serious playing that brought out squeals of laughter.. the kind that you will continue to do anything to get, it was just the mental break I needed. Thankfully Brian's trip has all Seattle overnights so we'll be able to talk this through in person.

This evening Georgia pulled herself up on our wine rack and pointed to each bottle saying "ba-ba", "ba-ba"... she is now asleep and mama is enjoying some ba-ba.