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!

So Long, Farewell, Auf wiedersehen, Goodbye (D+95)

The "Day 100" Blood Draw

“Nothing behind me, everything ahead of me, as is ever so on the road.” - Jack Kerouac, On the Road

Elise had her "last" visit and tests at NIH yesterday and she's been released to leave the local area. We're packing up today and hope to hit the road tomorrow for the long drive back to Ridgecrest. We plan to stop several places along the way and visit family and friends.

Of course there are some lingering things that will bear watching in the coming months. Elise will have weekly visits with the transplant Drs at Childrens Hospital LA who will be our "local" transplant team. They will be closely monitoring the recovery of her immune system. And we'll return to NIH for short visits at 6, 12 and 24 month post-transplant. I'm a bit nervous because her blood test results look "odd" to me, but the medical team tried real hard yesterday to make me comfortable that "it's all normal". Her WBC counts were a little low so they gave her a GCSF injection and gave us some doses to take along with us "just in case".

We had the "release" training and it made Elise upset. Since she'll continue to take Sirolimus for many months to help prevent GVHD, her immune system will continue to be somewhat suppressed. As a result she has to continue to be careful about many things. For starters she is more susceptible to viral infections, which she never was before with CGD. The one that she wasn't prepared for was "no contact with farmyard animals including horses". She had plans to start horseback riding when she got home. This is a long running dream of hers: once when she was little she asked me if I could "call up the workers" so that they could come build a stable in our back yard. In any case, all these precautions will go away some time in the next 9 mo when she stops taking Sirolimus.

We've had many talks about the return to school and I'm not sure how this is going to go. She doesn't want to be singled out in any way (good or bad), but we've tried to tell her that people will be naturally curious about what's up. After all she's lost her hair, eyebrows, and quite a bit of weight. And without a doubt there will be someone who will say something inappropriate (she's in middle school - not exactly the bastion of carefully considered comments).  We've tried to convince her that the best approach is to hit it head on (e.g. "I had a bone marrow transplant, but I'm well now and my hair will grow back"), but she just wants to be a normal kid. She's tough though and I'm sure she'll get through it.

So, assuming the movers show up today and NIH doesn't call us with a "we have some bad news" call, we'll hit the road in the morning and be back in Ridgecrest around 1 Oct after seeing many parts of the US we've never visited.

A few members of Elise's medical team at her release visit.

Nearing the End of the Beginning (D+90)

"There will come a time when you believe everything is finished. Yet that will be the beginning." - Louis L'Amour

Wow time is flying by. It's day 90 after transplant and all seems to be going very well. We've been told that if everything remains stable then Elise will be released to return home early next week which is a few days early! We're all real excited at the prospect of getting home.

There isn't much medically to report. They've tweaked some medication dosages based on the weekly tests she has, but everything seems to be going as the medical team would anticipate. Elise did start having the sniffles and a dry cough just about the same time as the D68 entrovirus hit the news, so they ran some tests and decided she just had a normal head cold. She's still a bit congested and coughing a little, but it only seemed to slow her down for a day.

One thing you might notice in the picture is that Elise started to lose her eyebrows. Chemo and radiation affect the fastest growing cells first. I guess the eyebrows grow more slowly. She hasn't completely lost them, but they are definitely much thinner. But they'll grow back just like her hair. The hair on her scalp is now light colored and about 1/4+ inch long. She's finally able to kid me about being bald without me being able to turn it around. I do still rub her head "for luck".

We've made arrangements with Children's Hospital LA for follow-on transplant care once we get home. Elise will need to see a transplant Dr weekly for a while and then it will scale back until we hit the magic 1 year mark. At 6 and 12 mo she'll need to come back to NIH for short visits. After that it's once per year until she hits the 5 year mark.

We're trying to make all the arrangements required to move out of our apartment. Most things are easy, but getting rid of the furniture has turned out to be more of a headache than I'd anticipated. Who knew that it would be so hard to get furniture picked up? But we couldn't expect everything to go smoothly.

Elise has been visiting with the teacher daily. They've been trying to get a little ahead on the math coursework so that she starts back to school even with her classmates. I'm not sure all the other classes will go as well (she hasn't been practicing the flute much at all), but then we asked them to concentrate on math since its so hard to catch up once you get behind.

Around the edges we've been trying to take in the local sites. We finally made it to the Bureau of Engraving and Printing and we hit an Art Fair in Alexandria last weekend. The weather has gotten really nice. Hopefully it's cooled off in Ridgecrest when we finally get there.

We're considering hosting a Welcome Home for Elise after we return, so if you're interested please keep watch for an announcement.

Time Passes Quickly (D+80)

"Time is a sort of river of passing events, and strong is its current; no sooner is a thing brought to sight than it is swept by and another takes its place, and this too will be swept away." - Marcus Aurelius

I realized I hadn't posted an update for a while. Guess I've been negligent...sorry.

It's already 80 days post transplant! I can hardly believe it. The bottom line is everything is going well. Elise's blood counts are good, but don't look as good as I'd expect, but I'm not the medical team. They assure me everything is going well. So I ask questions and get reassurances. At least it gives us something to talk about. Elise does have a rhinovirus (i.e. headcold) that she probably picked up on our trip, but the Drs have told us not to worry. It's not the EV-D68 virus in the news or influenza (they ran a test today), just a normal head cold that will pass.

Elise is down to once a week tests and visits with the medical team and gradually the list of medications she has to take are reducing as well. They have started tapering her prednisone dose although we don't think it'll get to zero for quite a while since her underlying colitis will take a while to heal (maybe a year). She'll likely be on several of the medications for a full year post transplant, but we'll see.

Today we actually discussed coming home with the team. They have a protocol for the 100-day release and we talked a few more details of what that would encompass. Mostly we talked about timing as we're actually at the point of making some plans. Elise is within 3 weeks of being released to come home. We've contacted Childrens Hospital LA to arrange follow-on care with their BMT team and I signed releases today to send all the medical records to Elise's Dr's at home.

Last week was a fun filled week. It was the anniversary of Becky's 29th birthday so we decided to go to Ocean City, MD for a couple days after her Tuesday medical visits. After Ocean City, we drove to Assateague Island to see the wild ponies (of "Misty of Chincoteague" fame). After that we went on to Williamsburg, VA to visit Colonial Williamsburg (Elise's 8th grade history covers early American history). We started the week with a visit to the White House that I'd arranged earlier.

I don't know how we're going to pack up everything we want to bring home, but I guess we got most of it here in suitcases on an airplane so it's all gotta fit in the Toyota - right? We're going to donate the furniture and housewares we bought to a charity that I contacted today. We also bought a rooftop cargo box for the car. And I've been telling Becky that she has to throw away something every time she buys something. I don't think this latter tactic is working.