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

Getting in the Rhythm (D+121)

"The most effective way to do it, is to do it." - Amelia Earhart

We're all getting back into the swing of things and this week should sort of be the last step for the new normal as Becky joins the salaried workforce again. Till now she's been off work which has made getting Elise started back to school much easier since she can run errands, visit the school to give Elise meds, etc. I started back a couple weeks ago and it was almost like I woke up and the clock had advanced 5 months. Weird, but then my coworkers have been great and everyone has been patient while I try to dust off the cobwebs.

Elise's new normal is a school day without PE. There are at least 3 things to worry about: exposure to the sun might trigger GVHD; some of her meds make her sensitive to sunlight; and her platelet count is low so she'll bruise badly if she has a accident.

The transplant protocol Elise went through is meant to reduce the chances of GVHD, but it also means that she's exposed to the threat for a longer period. Most BMT patients are mostly out of the woods for "acute GVHD" by day 100. Elise will be watched for 6+ months. Fore Elise, the pretransplant conditioning drugs will stay in her system for about 6 months, so she needs to take sirolimus at least that long and probably longer to prevent "acute GVHD". After that, we'll be watching out for "chronic GVHD" which is a rarer and different beast.

We've started going to Children's Hospital Los Angeles (CHLA) once a week on Friday's for follow up care. It's a strange transition from NIH. First CHLA is targeted at children so the noise and activity level when you get off the elevator in the lobby is quite a bit higher than NIH. Secondly, CHLA does do research, but it's not a research hospital first. This means there are a lot more patients wandering about. Young patients. And generally mom and/or dad have more than the one in tow. The medical team and other staff are all nice and seem to know their stuff, but it's different and requires some mental adjustments on our part.

Another adjustment for us is the lab test rhythm. Sirolimus requires careful monitoring of the level in her blood and frequent adjustments in dosage. The blood tests are a problem for two reasons: 1) they must occur 24 hours after the last dose; 2) they can't be processed at most hospitals (including CHLA and NIH). We've had to plan a week ahead and try to give Elise her daily meds when we think her weekly blood test will be. Of course this means she needs them in the middle of the work and school day which is a huge hassle for all of us. The second problem means that we are limited in what labs we can use. CHLA (and NIH) send their tests out but they get them back in a few hours. Here in Ridgecrest (where our insurance will pay 100%) they send them to Outer Mongolia and it takes 3-5 days for results. Not exactly timely. We haven't quite got this part of the new routine worked out yet.

As for Elise's health, outwardly she's doing great. She asked to start dancing again this past week so we're letting her attend class once a week for now. And she's been doing things with friends (movies, etc). Her weight is very slowly beginning to rise. Her appetite still isn't huge, but weight gain, even if it is slow, is better than weight loss. And the hair is starting to be noticeable, at least to me.

Internally, many of Elise's blood levels are still very low. I'm unclear on exactly what we should be seeing but the doctors don't seem alarmed. A week ago it looked like she might need a transfusion because her hemoglobin level appeared low, but the next test came back and it had recovered enough to ward off the transfusion. Transfusions are somewhat normal for post-BMT patients and they've been surprised that Elise hasn't needed any since right after the transplant. But CHLA has different protocols and based on discussions with the staff it seems they are more likely to order a transfusion than NIH was, but so far Elise hasn't had one.

It's hard to believe but we've already scheduled Elise's 6 month checkup at NIH. It'll be more like 5 months but it's inside their acceptable window. Elise and I will travel back the weekend before Veteran's Day and visit the medical team for a 1 day checkup. Veterans Day is a holiday for Elise and I so that will limit the amount of time we're away from school/work.

As for me, I'm mostly back in the swing of things. I love being back in the desert and having access to the trails in the foothills around our house. I managed to get in a day hike in the Sierras after we got home and hope to squeeze in an overnighter before the winter weather makes the mountains inaccessible. On top of that I'm eyeing all the wood laying around my garage waiting to be turned into something.

Kearsarge Peak from Mount Gould, Golden Trout Lake in foreground

Party at the Bechtel's Saturday (D+104)

"We can only be said to be alive in those moments when our hearts are conscious of our treasures." - Thornton Wilder

We're hosting a "Welcome Back Elise" party at our house in Ridgecrest so you can see Elise and also to say Thank You to everyone that has been so supportive over the past many months.

What: Welcome Back Elise (very low key) Party

Where: The Bechtel's in RC (call or send me a message if you need the address) 

When: Saturday, 4 Oct 2014, 4pm to 6pm

All of Elise's friends, family and wide circle of supporters and well wishers are welcome. We'll have soda, beer and white wine on ice along with some light hors d'oeuvres. The pool will be open (weatherman says "Sunny and Hot") but the AC will be running indoors too. If you'd like something in particular feel free to bring it, but please no gifts as we ran out of Thank You notes a long time ago (you guys are great!) Oh, and our fridge broke while we were gone so don't bring anything that needs refrigeration (the new one arrives next Tuesday).

Now on to my blog update:

After driving 3080 miles of driving through 11 states in 7 days, we finally made it home to Ridgecrest on Tuesday (9/30/14). We saw a lot of the beauty of this great country and some really cool things along the way, but we're enormously thankful to be home.

Elise is feeling great. The minute we got into town she ran across the street to visit her friend Gina and then they went to a volleyball game! She's been very anxious to get back to school ("I feel like I'm ditching") so she started today instead of our plan to have her go back next Monday. The picture above is her "First Day of School" photo this year. She's kinda brought new meaning to "skinny jeans", but the weight should gradually come back along with the hair and eyebrows.

We are visiting Children's Hospital LA for the first of many weekly follow-up visits this Friday. Elise isn't completely out of the woods yet. Because of the type of transplant protocol she underwent, she's still open to "acute" graft vs host disease and will be for a few more months. This means that she's taking an immunosupressant to prevent GVHD, and this med means she's still more likely get opportunistic infections than the general population. So they need to watch her closely for a few more months. Hopefully by Christmas time (when the effects of the Campath wear off) she'll get to taper back on the weekly visits and the meds.

So we're trying to work our way back into the routine here at home. Elise is back to school, I'll start work again on Monday, and Becky goes back in a couple weeks. I'll try to provide some periodic updates but they will be few an far between unless something comes up wrt Elise's transplant.

Thank you all for your support, whether it was a smile, kind word, one of a seemingly endless stream of cheerful cards, mowing the lawn, or the truly nasty task of cleaning the rotting food out of our freezer when it died while we were gone. All these were heart felt gifts that made this an easier process for us to go through and we are truly grateful!