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.

Steady Progress (D+250)

"My favorite animal is steak." - Fran Lebowitz

Elise and I made a quick trip out to NIH in Bethesda, MD for a follow-up appointment. The medical team had contacted us and asked to see Elise since our "6 month" follow-up was done a little early. We managed to sneak into and out of the east coast between storms so we missed the intense cold (aka "the Siberian Express") they've been experiencing this winter.

Elise had the routine battery of blood tests and exams. Everything is looking really good, with her neutrophils and all the blood components rising as anticipated/hoped. What's remarkable to me is that some of her counts are better than they've ever been. For example her platlet count is well above 200 - I've never seen it that high in the "normal" range. It's all pretty exciting even if the progress seems glacially slow.

In addition to the standard exams, she also saw a pediatric endocrinologist and also an opthamologist. The pediatric endocrinologist confirmed what the Dr at UCLA had found ("preliminary ovarian insufficiency") but stressed that they really have no way to gauge who's ovaries will recover normal function and whose wouldn't, so she said we shouldn't count out a full recovery. I don't think Elise is concerned one way or another.

What the endocrinologist did confirm for us was that Elise probably won't grow much taller. She's about 4' 8.5" which is less than the 1st percentile (less than 1 in 100 people are shorter). She may gain another inch or two, but the interruption in her growth (probably from colitis and meds) is most likely permanent. I guess we'll need to start looking for cushions to sit on while driving...

Elise had a consult and several tests with an opthamologist. She has been complaining about sensitivity to light and the eyes can be effected by GVHD so they wanted to take a look. It's all good news: no GVHD and her eyesight is essentially 20/20. The sensitivity is probably just due to allergies (the warm and relatively wet winter has led to lots of early wildflower blooms in the desert where we live).

Elise was a little disappointed with news from Dr. Kang, the main transplant Dr.: no cats until later. Elise has been jonesing for a cat and was hoping that she'd been allowed to get one, but they can spread toxoplasmosis from their litter boxes so no joy. On the plus side, Dr. Kang said she could start horseback riding which is something that Elise has wanted to do.

Elise's weight is back where it was before transplant and so Becky and I are starting to pull back from the "eat, eat" mantra. I'm sure Elise is a little relieved by that. Her hair is filling in and she's occasionally going without her bandana, although she still seems self conscious. I'll bet she will lose the bandana in the next couple months as her hair fills in. It's much darker and thinner than before, but I'm told the color and consistency change in the first couple years.

We're schedule to go back to NIH in June for the 12 month follow-up. They've be doing a bone marrow aspiration (ie taking a sample) and also a colonoscopy so we'll be in Bethesda for a few days.

A couple small but good things are that Elise no longer needs to wear the mask at the hospital. She also has changed to monthly vs biweekly visits to CHLA for follow-up monitoring. Yeah!

Elise was initially bummed when she found that Harry's Tavern at Dullas had closed. We typically had stopped there when flying in for NIH visits while we waiting for the shuttle bus. But the disappointment was short lived: the restaurant that replaced Harry's, District Chophouse, had steak and so we split a New York cut that was awesome. I recommend it if you're stuck at Dullas.

Reduced Mileage Ahead (D+211)

"Childhood is a short season." - Helen Hayes

There's been lots of progress since my last post. Elise's persistent cough resolved, the stye she had in her eye resolved, and we got in to see a pediatric endocrinologist to address the suspected premature ovarian failure (POF).

The cough and stye seemed to resolve pretty quickly once she stared a 5-day Z-pack antibiotic. It could be coincidence, but I don't think so. Her eye was looking pretty bad and it took quite a while but it seems to be gone now. The cough she'd had for a month just seemed to turn off like a light switch about 2 days into the 5-day Z-pack treatment.

We ended up giving up on the pediatric endocrinologist at CHLA. We couldn't get in for first visit until late February. And worse, we received a letter saying that we should expect to wait 3-4 months for any necessary follow-up visits. They should have just given us a list of alternate providers. So I looked up who was available at UCLA and called. We had an appointment within days.

The endocrinologist ran several tests including bloodwork, an ultrasound and a x-ray to determine bone age. In the end, she confirmed the POF diagnosis and placed Elise on hormone replacement therapy which is essential a variety of birth control pills. The Dr said Elise should have a period again in a few weeks and things should even out pretty quickly. When we discussed the longer term the Dr said she really couldn't tell. Some women recover function and can go off HRT but others can't. Since the treatment is birth control the Dr. said that most women just take HRT until they decide to try to get pregnant and then take it from there.

We also discussed Elise's overall growth. She had been tracking just above the 10th percentile for height and then her growth stopped a little over a year ago. Now she's well below the 3rd percentile. The DR. couldn't say why this is so (probably has to do with colitis and all the meds she's taken), but the hand x-ray indicated that her "bone age" is about 13.5 years, so she's unlikely to grow much more than another inch. That means that Becky probably won't be the shortest one in the family anymore. On the other hand, she's eating well and there is anecdotal evidence that some people "catch up" so we'll just wait and watch.

The biggest news is pretty exciting for all of us: Elise is moving from biweekly visits to CHLA to monthly visits. Yeah! Her lab results continue to look better and better and so the Dr's feel that she doesn't need to come in as often. Additionally, she had been getting monthly pentamidine breathing treatments to prevent pneumonia; now she is transitioning to a oral antibiotic that she takes only on the weekend. Neither the frequent trips to CHLA nor the pentamidine treatments have been onerous, but it'll sure be nice to save the insurance copay, gas and time.

So all is well. We're looking forward to Spring and then Summer which will mark the 1 year post-transplant point.

Dianniversary (D+186)

"God gave us the gift of life; it is up to us to give ourselves the gift of living well." - Voltaire

Elise made it to 6 months post-transplant this past weekend (her "dianniversary" according to Urban Dictionary). She's almost fully transitioned back into life as it was pretransplant. She's going to school and doing very well (an almost seamless transition thanks to the help she received from Ann at NIH). She's dancing 1 day each week and probably going to ramp back up to 3 days a week. And she's participating in Girl Scouts where she received the Silver Award in early December.

So things are going well but there are a few little nagging things that have required some attention.

She caught a cold around Thanksgiving. It passed quickly but it led to a cough that became "productive". The cough also led to some pain that was difficult to diagnose. The Drs seemed to settle in on soreness from the act of coughing (I'm sure there's a medical term for it). The soreness led to less deep breathing and possibly "atypical" pneumonia (aka walking pneumonia). It was borderline but they gave her a 5-day Zpack and the cough cleared almost immediately.

Following the watery eyes from the cold, she developed a stye in one of her eyes. We weren't sure what it was because the eye became quite swollen. After a visit to the opthamologist it seemed to initially resolve with hot compresses and antibiotic ointment. But then it jumped to the opposite eye and we spent the next couple weeks chasing it around from eye to eye and lower eyelid to upper eyelid. It seemed to get under control with the combination of oral antibiotics and antibiotic eyedrops.

The last thing will present some longer-term challenges. Based on some blood tests, it appears Elise does have Premature Ovarian Insufficiency (POI) or Premature Ovarian Failure (POF). From what I've read this almost always happens to women who undergo a BMT and is caused by the conditioning regimen. It is generally treated with some form of hormone replacement therapy that can be short or long term depending on the individual's response. Simple, but we are still trying to get in to see an Endocrinologist. At this point the earliest appointment we've been able to establish is late February. But I'm on the hunt for an earlier one.

If there's one thing I'd have done differently given 20/20 hindsight, I would have made an appointment date with an reproductive endocrinologist at the same time we were making arrangements for follow-up care at CHLA. As I mention, it seems that POI/POF is a near 100% thing and endocrinologists are in high demand. Waiting too long is not a great idea because from what I've read POI/POF can also impact recovery of weight, height and hair as well as lead to issues such as reduced bone density. So if you're reading this and going through a BMT yourself (or your child) bring this up with your Drs.

While these things may seem like major issues, they really aren't impacting Elise too much. She hasn't complained too much about any of them and just keeps moving forward. We'l get them all resolved eventually and in a years time we'll likely have forgotten them (except for this pesky blog).

We're all looking forward to Christmas Eve tomorrow and then Christmas Day. We're following tradition and having breakfast at Becky's sister's. Then, after digesting and maybe an episode of "The Christmas Story", the family will come over to our house for dinner. We'll follow all that eating with a gift exchange. But I know we've already gotten the best gift possible.

Thankful (D+144)

"There's no happier person than a truly thankful, content person." - Joyce Meyer

Elise and I made a quick visit to NIH for her routine "6 month" checkup. It's really only (!) been about 5 months since the transplant, but the upcoming holiday season made this the best time to visit.

Elise had a battery of tests: about 15 tubes of blood work; chest CT scan (looking for any evidence of infections); echo cardiogram; and a 24-hour Holter monitor. After that we met with the medical team. 

Everything continues to look good. Her blood counts are gradually recovering. She's gaining weight (although she has about 10 lbs to go to get back to where she was last spring). Her hair has started to become noticeable. And no signs of the dreaded GVHD.

The one thing they are going to look into further is whether her ovaries were damaged by the chemo and/or radiation conditioning therapy. We all knew going in that this was a possibility. They can't protect the ovaries during radiation therapy so they are totally exposed. And chemo can do all sorts of bad things to the body.

Elise hasn't had a menstrual cycle since June and she's been having hot flashes. The hot flashes may be caused by one of her meds, and the lack of a period was by design: they gave her a dose of Lupron to inhibit her cycle while her platelet, hemoglobin and red blood cell counts were suppressed post-transplant. At this point it should be kicking back in, although it's still in the range of expectations.

So they'll run some tests and wait and see. It could be a number of different things: a late restart after the Lupron; her low body weight; the ovaries "going to sleep" from the chemo (could take a year or more to "wake up"); or her ovaries could be damaged. If they are damaged then they'll put her on birth control pills to normalize her hormone levels which will restart her period.

Elise is just fine with not having a period and she wasn't real excited to hear discussion of working to restart it. I suppose other women probably feel the same way.

On a real positive note, they've decided that Elise can stop taking 3 of her medications. The first was ursodiol which she took to help prevent VOD, a rare but potentially nasty complication of chemo therapy. The next was metronidazole, an antibiotic that treats gut bacteria like c.diff. She'd been taking it prophylactically for quite a while due to her severe colitis. And lastly is prednisone, a steroid meant to manage the symptoms of colitis. She's been tapering off the prednisone for quite a while. It was a miracle drug when she had colitis, but she hasn't had any symptoms for several months and hopefully it's gone for good (her severe colitis is the main reason that drove the transplant).

Of course she'll need to take some meds for up to a year: sirolimus to help prevent GVHD; and acylovir and fluconazole to help ward off opportunistic infections that might pop up because of the immunosupressive action of the sirolimus. But eventually these too will go away. That will be an amazing day.

It's really hard to believe that a year ago almost to the day I was sitting in a room at NIH realizing that we'd need to go forward with a bone marrow transplant. Elise was having a colitis flare and the GI specialist had just finished a colonoscopy. In the little room they use to have private meetings, his prognosis wasn't good. Up until then we'd avoided discussion of a BMT because of the extreme risks, but we found ourselves boxed into a corner. After talking to Becky on the phone that night we started down the path the very next day. I sure am glad it's a year later and things have gone well. It's going to be an honest Thanksgiving celebration this year.

View of the Children's Inn at NIH