I think we're done (D + 62 mo)

Elise & Dr Elizabeth Kang, the transplant team lead

"The future rewards those who press on. I don't have time to feel sorry for myself. I don't have time to complain. I'm going to press on." - Barack Obama

It's amazing how time has flown by. Elise hit the 5 year post-transplant mark and she had her last formal visit to NIH this week. As part of the research study Elise was in we committed to returning to NIH for periodic follow-up visits for 5 years post-transplant. This trip marked the last of those visits.

Elise has done really well over the past year. Health issues have been at a minimum. She still has the premature ovarian failure/insufficiency that is caused by the pre-transplant conditioning. Dr. Kang said that some other patients eventually have their ovaries kick back on, but it's managed with hormone replacement therapy for now and we keep our fingers crossed. And the atopic eczema Elise has had since the transplant seems to be even more diminished than it was last year; it's pretty minimal, nothing even close to what it was a year after transplant, and hopefully it will continue to fade as time goes on.

They've asked Elise to continue to return every couple of years so that they can keep track of her (and all the other patients) progress, but at this point it's voluntary.

Elise finished her senior year of high school in May. She had an outstanding year. The tennis team made it to the 2nd round of CIF playoffs, she was awarded the Girl Scout Gold and Trifecta awards, and her grades were the best ever. Next week she heads off to college at CSU Channel Islands. Even though we'll be sad to have an empty bedroom in the house and miss the tennis matches and band performances, we're so happy to see her take the next steps in her life.

Elise with her coach and doubles partner after winning their first round at the league finals

Elise's first sold work of art

Girl Scout Gold Award ceremony in Visalia

High school graduation!

Now she's a CSU Channel Island Dolphin

Four years on (D + 48 mo)

"Faithless is he that says farewell when the road darkens." - J. R. R. Tolkien

Elise is four years post-transplant and doing great. She just finished her Junior year in high school and we visited NIH for the her fourth annual follow-up exam. She's had some continued skin issues, basically some sort of allergic reaction on her forearms, but it seems to be reducing over time. Other than that and the anticipated "premature ovarian insufficiency" caused by the pretransplant  conditioning, she's doing great.

In addition to her Junior year academics, this year she lettered in Tennis and ran distance events (800m and 1600m) on the track team. She was also part of the band colorguard during marching season and played flute when she wasn't doing colorguard. We also managed a few vacations including a trip to London at Thanksgiving. It sounds like a busy year and it was.

This summer she's helping at Becky's PT clinic, weight lifting and taking tennis lessons. The next year will be a big one. She'll finish her last year of high school and have her last visit to NIH, both markers of the start of the next stage of life.

Second Birthday (D+24 months)

"Difficult roads often lead to beautiful destinations." - unknown

This week was Elise's "Second Birthday" - the 2nd anniversary of receiving her new stem cells in the transplant process. She is doing great and we're all quite happy about it.

We all (Elise, Becky and myself) went out to NIH this week for her 24 month follow-up checkup. This is a big deal because it marks the point that she transitions to only needing checkups annually. Basically she's done with the transplant protocol. It's really hard to comprehend. They'll continue the annual checkups till her 5th anniversary.

At NIH they drew 19 viles of blood, did a chest CT scan, did a series of pulmonary function tests (PFT), and performed a once-over ("breath deep"). Dr. Elizabeth Kang, her transplant lead, said everything looked good. Her immune system was "coming in" the way they'd anticipated/planned and she was pleased with the results. Of note, Elise's PFT scores had improved, probably due to her running distance this year. And I've never seen her blood work look so good (after so many years you get good at reading blood test results). In general, things are looking pretty darn good.

Since my last post 6 months ago, there has been at least one health issue that's come up. Elise decided to run distance events with the High School track team. I was very proud of her since she decided to do this all on her own - none of her friends were doing it and in fact she ended up being the only girl running distance on the team. She wan't fast but I could see she was getting better every week. About a month into practices she started getting a rash on the backs of her legs. It spread to the fronts and eventually also her arms and a little on her torso.

We looked at what had changed in her environment at the time the rash started and decided the most likely culprits were: 1) the grass field the track team stretches on; 2) the cats she'd gotten several months earlier; and 3) minor medication changes her non-transplant physicians had made.

We tried to mitigate the grass issue by having her stretch on a towel and even asking the coach if she could strech elsewhere. This didn't seem to work at all so we decided to pull Elise out of track a few meets into the season. That seemed to help a little, but didn't totally solve the issue.

We tried all the conventional treatments and her pediatrician even gave her two courses of 3-day steroid burst to break the cycle. Nothing seemed to have a lasting result. Eventually the dermatologist put her on a two week course of stronger topical steroids. That seems to have helped immensely. NIH said it wasn't graft vs host disease (GVHD) and probably wasn't related to the transplant, but they also said her lyphocyte levels aren't 100% yet and it could be some (hopefully temporary) reaction as they come in. Apparently transplant recipients have occasional transitory weirdness with their health in the early years following transplant. Hopefully this is the last of these for Elise.

Truthfully I'm still just a bit on edge every time a health issue comes up. When I hear her cough or get up in the middle of the night to pee my little alarm bells start ringing and I have to remind myself that things have changed. I guess you can't go for so many years knowing that minor infections can turn life threatening without some residual effect. Some sort of conditioned response I guess. In any case, Elise just wants to move on with life and put this all behind her and I can't say that I blame her.

Just a week before we visited NIH Elise was finally able to put her hair in a ponytail. She says she's never cutting her hair again. I can't believe how strong she's been through all this stuff like losing her hair, eyebrows and eye lashes. I know it's left some lasting effects, but she's become such a strong person that I sometimes forget how I would have responded under similar situations when I was 15 years old.

So we're all moving forward. Elise is looking forward to learning to drive and getting her license. She's got a part time summer job at the child development center. And she's planning to play tennis and run track again next school year. She's excited about the color guard starting again and starting to talk about the types of things she might do in college. All perfect things for a 15 year old to be doing and talking about. It's a good place to be.

Present for Christmas (D+18 months)

"Maybe Christmas, the Grinch thought, doesn't come from a store." - Dr. Seuss

Well we made it to 18 months post-transplant with no recent issues. The transplant team wanted to see Elise for an 18 month follow-up visit so we made a quick trip to Bethesda, MD. The day started with 18 viles of blood tests followed up by a CT scan of her chest and abdomen. Then we did the standard visit with the medical team.

Her blood test results all look excellent. The chimerism test will take a week or two to come back, but the last one was excellent and there's no reason to think this one will be different. Dr. Kang was out of town so we met with Dr. Harry Malech, Chief of NIH's Genetic Immunotherapy Section. He said he was happy with Elise's progress. They seemed particularly pleased that she continued to have no GI problems. Apparently this has become a key reason to undergo a BMT for those with CGD although they aren't 100% sure why the BMT seems to cure this particular problem.

We asked about getting off the Acyclovir and blood pressure medicine but the medical team wanted to defer that decision to Dr. Kang. Hopefully when she returns Elise will be able to go off those. That will be the last of the transplant medications. She'll likely need to continue the hormone therapy until she's 50 since the radiation and/or chemo seems to have shut down her ovaries (a near-universal side effect). We'll see the pediatric endocrinologist again in January, but I doubt there will be a change to that verdict. There's a slight chance the ovaries will kick in again but won't know unless/until Elise goes off the hormone therapy to try to get pregnant later in life (no time soon!)

The day finished with Elise getting 6 (!) vaccinations. With a new immune system she's forced to do all the vaccinations over again. They did the first round at 12 months but she was under anesthesia for a colonoscopy when they did it. This time she just had to endure 6 separate needle sticks. But as usual she was a trooper.

Our visit with the medical professionals was quick. They didn't find anything that made them schedule additional tests so we rescheduled our flight home a day earlier than planned. That made both Elise and I happy as it's Winter school break and neither of us really wanted to be away from home. So we caught an early flight and were home less than 48 hours after we left.

Our next scheduled visit will be at the 24 month post-transplant point (~ June '16). After that I think they transition to a once-per-year schedule for the following 3 years. And then she's done at NIH.

I can't think of a better Christmas gift than to know my daughter is healthy and happy.

Out and Back and No GVHD (D+480ish)

"The woods are lovely, dark and deep. But I have promises to keep, and miles to go before I sleep." - 

Robert Frost

Elise and I took a quick trip back to NIH in Bethesda, MD this week. We left the house about 3:15 AM on Tuesday and returned about 1:45 AM on Thursday. Can you say jet lagged sleep deprivation?

Elise has been having some patches of eczema on her shoulders and upper arms that seem to appear, resolve and then move to a new spot. It's leaving a pattern of light colored patches on her shoulders and upper arms. I'm told the light colored patches are a normal skin reaction and that the color returns to normal over time. This has been going on since last spring and the Drs at CHLA have been monitoring it during our monthly visits.

About 3 weeks ago she started complaining about a burning sensation in her eyes. It seemed to come on suddenly and didn't seem to respond well to eye drops or any of the normal things you might do.

When the transplant team at NIH got wind of the eye problems they asked that we visit so that their specialists could take a look. Their worry was that she was experiencing graft vs host disease (GVHD), a common but potentially serious (it can be life threatening) side effect of transplants. About 30% of stem cell transplant patients get some amount GVHD. The transplant protocol Elise went through is designed to minimize the chances of GVHD, and usually someone that has gotten this far post transplant without GVHD wouldn't expect to experience it.

So we scheduled an impromptu visit to NIH. I wanted to minimize Elise's missing class so we crammed everything into a day with the Drs and took a later flight home.

The opthamologist put all sorts of dyes and stuff in her eyes looking for "pitting and cracking" that is characteristic of GVHD. And the dermatologists (yes multiple, they travel in packs at NIH) did a biopsy. The good news is that neither specialist found GVHD. Her eyes are apparently responding to an allergen (grass pollen from the football field she marches on every weekday morning?) and the skin is just normal eczema (possibly triggered by a combination of meds and sun exposure).

So a sigh of relief is in order, but mostly we just wanted to catch up on our sleep.

In general Elise continues to do well. She's on the band color guard and also playing on the JV tennis squad. Along with classes and homework, this keeps her very busy. Last weekend she attended the Homecoming dance.

We probably will have our planned 18 month post-transplant visit to NIH in December or January. I'm hoping she can get off all the transplant meds and then we can eliminate the monthly visits to CHLA. The visits provide some amount of positive reinforcement and comfort, but they also take Elise out of school. It would also be nice to spend some money on something other than healthcare.

Elise with Percy

The Fun of Monthly Visits (D+426ish)

Elise at Hollywood and Highland between appointments at CHLA

"Forget what hurt you but never forget what it taught you." - unknown

Elise is about 14 months out from her bone marrow transplant last summer and things are going really well. She is still seeing the Drs more frequently than most kids (monthly), but compared to a year ago it seems like nothing. When she transitions off all the meds it should taper off to once every six months or so, and then once a year.

She's still on a few medications. She's taking acyclovir to dampen the impact of viral infections. There are a few they worry about, but at this point its more caution than necessity. She'll take this until the 18 month point. She's also taking the antibiotic bactrim to prevent pneumocystis pneumonia which causes problems in people who are immunocompromised. Her Dr at CHLA said she's ready to come off the bactrim now and so if NIH confirms that will be one less med (yeah!) 

Another med we tried to drop recently was the one she takes for high blood pressure (HBP). HBP is a side effect of one of the immunosupressant sirolimus that she was taking for 12 months. The side effects of the sirolimus linger in the system though so when she discontinued it she had an "event". She was at tennis practice and experienced elevated pulse and difficulty breathing. The coaches had me pick her up and pretty quickly we determined her BP was very elevated so we restarted the med and it came back in line within an hour. She hasn't had an event since, but she's going to continue the HBP med for a while longer.

We also had some fun with joint pain. Elise was complaining of pain in her left knee and so the Drs wanted her seen by an orthopedic specialist. Apparently a potential GVHD complication can lead to joint pain. So we met with them this week. Of course the pain subsided right after the consult was scheduled and xrays were completed. The orthopedic Drs didn't see anything of concern. We'd certainly like that outcome rather than the opposite, but it seems frustrating when you're trying to get the appointments all arranged and done.

The last thing she's dealing with (other than generally thin hair which seems to be growing back in slowly) is some patches of skin on her upper arms that have lost pigment. Each pigmentless patch seems to be proceeded by a light rash. The Dr has been watching it for a while but doesn't think it's GVHD and has referred us to a dermatologist.

I mentioned in my last post that Elise had been cleared to have pets again, so she made sure to pester us incessantly until she got a couple kittens. Becky and Elise found them at the local shelter. "Percy" is a "blue" color with Scottish fold features. "Tris" is a burmese color with a very slight fold to the ears. Even though they share some of the fold features they aren't from the same litter. In any case Elise loves her new kittens and we all get a kick out of watching them romp around the house.

Percy and Tris

Elise started her first year at high school a couple weeks ago. She enrolled in summer school and completed the compulsory Health and Geography classes so that she could take an extra elective, German, during the school year. She's practicing after school with the girls JV tennis team and also practicing before school with the marching banch color guard. She's in Cadet band as an elective, but they only join the Symphonic band for a few performances so she can participate in color guard with no conflict. Needless to say she's very busy and I'm happy to report her stamina is very good - you really wouldn't know she had a BMT 14 months ago.

One of the things we've been doing recently is going throught he house and cleaning out all the old and discontinued medications. After 14 years of dealing with a primary immune disorder there were lots of meds stuffed in cupboards, drawers and various other nooks and cranneys. We learned early on not to discard them because they often came in handy (colitis flare? better have predinsone on hand in a variety of dosages). It's been a real pleasure to get rid of these.

Today my wife cleared out "the last" drawer. I was looking at the meds and noticed it was simple antibiotic that was filled in Florida. It was the from the first serious medical incident Elise had encountered. We were on vacation in Orlando and she had been suffering with something that couldn't be pinpointed. Finally in Orlando her body just could cope any more and she spiked a fever and we took her to the hospital. Turns out it was c.diff (which we thought it was all along but the tests were negative for). Of course they thought it was something worse based on a false-positive test for a blood infection and she was hospitalized for 4 days with IV meds, etc. When they finally decided it was c.diff they had me go to CVS and get $5 worth of metrnidazole. $14K of hospital bills solved with $5 antibiotic they didn't want to give us in the first place - aarrgghhh). But the longer game had started. The c.diff seemed to trigger the first of a few colitis flares that would keep her sick and ravage her colon. Eventually it became clear that she needed either a ileostomy, colostomy or a BMT.

Throwing away that CVS bottle sort of put a long chunk of history behind us. Gladly.

No More YouTube Cat Videos Please! (D+348)

"Start by doing what's necessary; then do what's possible; and suddenly you are doing the impossible." 

- Francis of Assisi

I haven't written an update recently. Spring has sprung and summer is upon us.

We made it through the Spring with no incidents. There was a lot of sniffling and sneezing going on likely due to the overabundance of wildflowers here in California's high desert. It's a drought year in CA, but our little town got higher than normal rainfall and with it came a lot of wildflowers. And there were some coughs and eczema, but so far no GVHD (knock on wood) or other serious complications. The hormone therapy seems to be doing it's thing and the pediatric endocrinologist doesn't want to see Elise again until January.

As you can see Elise's hair is slowly returning. It's still very fine and not very dense but it seems to be filling in gradually. She shed the bandanas towards the end of the school year after my sister Carla trimmed it up a bit. She told Elise that the bandanas may actually be keeping the hair from growing out by rubbing the delicate hairs off. Elise gets stares from adults and small kids, but most of her peers don't seem to pay attention.

The end of the school year was chock full of activities. She "promoted" from middle school (8th grade) to high school at the end of the school year. She's done well this year grade-wise despite missing the first quarter. She even got several A's including in honors math (although she's quick to point out that it wasn't algebra that many of the better students were taking - that was just a bridge too far this year).

This week she had her 12 month follow-up visit at NIH. They did a bunch of blood work (15 viles), a pulmonary function test, an echocardiogram, a CT scan, a colonoscopy, and a bone marrow biopsy. The Drs said everything was looking good, in fact the GI specialist said her colon looked "pristine". This latter is a relief since the damage she had suffered from several bouts of colitis was the main tipping point for deciding to go forward with the stem cell transplant. Her bloodwork was also impressive - at least to me. I'd never seen her blood counts so high, nearly everything was well into the "normal" range. In the past her WBC, RBC, HGB, and platlets were borderline low at best; now they are all well into the normal range.

They won't get the chimerism test back for about a week. It measures how much of her immune system is the new system vs the old system. In her case they want the new system to ramp up gradually so that she avoids GVHD. This is very different than someone with, say, leukemia where they actually get some benefit from GVHD (it helps kill off any remaining diseased cells). So far it's gone well and we anticipate a good result. If it is good then she can stop taking the immunosuppressant (sirolimus) and other supporting medications. Elise is really looking forward to that.

While she was still sedated following the colonoscopy and the bone marrow biopsy, we'd arranged to get her first round of immunizations. She has to go through the whole series that kids go through because she has a new immune system. So she had SEVEN shots split between her two arms! Between the shots and the bone marrow biopsy (which requires a large needle puncture to the pelvis), she was pretty sore Thursday night and Friday morning.

We made it home from NIH late last night. We're all looking forward to our upcoming vacation at Newport Coast. It will be particularly special for Elise since she'll be able to get in the public jacuzzi pool this time. Because of the CGD we haven't let her go in jacuzzi's until now. It may not seem like a big deal, but when all the other kids are doing it and you can't, well it just highlights that you're somehow different. Well not anymore.

Oh and the best news (from Elise's point of view) is that her doctor agreed that she can now have a cat. While she's been recovering from the transplant we couldn't have a cat in the house (due to germs from the litterbox). She's really excited about the prospect of having a cat.