C is for Cookie (D+9)

"My mother's mother is a very tough cookie. She buried three husbands. Two of them were just napping." - Rita Rudner

I don't have a new picture of Elise for this post, so I pulled one out that's about a month old. I can't get pictures of her because she's spending most of her day and night in the bathroom due to diarrhea from the mucositis she's got as a result of the transplant conditioning regimen. It's fairly common, but still tough to watch. I can only try to imagine how Elise feels.

She is in pain from the mucositis, but I think the worst part is the lack of sleep. Getting up every 30 minutes, its tough to wedge in any sleep. But she has become an expert in unplugging her monitors (and silencing the annoying beeping when the alarms go off). I don't think Elise's has more than a few hours in the past two nights. She seems sleepy but not like you'd expect. Could be the meds or something else.

Becky and I have been trading off so we've been able to catch up on the Z's every other night. Last night it was my turn to stay at the apartment and I slept 8 hours straight. And I mean straight - no midnight pee, no wake up to look at the clock, nothing. I can't remember the last time I didn't that. I can't imagine what single caregivers do.

She got an infusion of platlets yesterday as expected. The timing was good because she's started getting slight nosebleeds, probably from the dry air in the hospital (she gets these at home in the winter). The problem is if you have no platlets then the most minor nosebleed can't stop. But with the added platlets the nosebleeds are annoying but workable.

They've been adjusting her pain meds and decided today to give her a baseline does and then allow her to bump it up if necessary via the PCA. This seems to be working better as her pain level has stayed down around "4" vs the "7" or "8" it had consistently been the past few days. I guess the theory is that giving her a base makes it so she doesn't have to "chase" the pain. They said they started her at a low base dose and will adjust from there. She seems reasonably alert even with the pain med.

Her fever continues to cycle up and down. She got pretty hot earlier today, but it responded to tylenol pretty well. They still haven't cultured anything out so they've still got her on the broad spectrum IV antibiotics.

She showed me that she's beginning to lose her hair. That's new and exciting for her in a strange sort of way. I guess you take what you can get. I think she's looking forward to sporting the hats we got her before we left.

I'm hoping the mucositis passes sooner rather than later, but I don't see any signs of it diminishing yet. I know I should be more worried about the fever, but that's not what's causing Elise the most grief, and being the parent I just want to see my daughter comfortable.

She's been a real champ through it all. The nurses have all commented on how well she's taking it. I guess not everyone responds the way she has. She is one tough cookie.

The More Things Change (D+7)

 "plus ça change, plus c'est la même chose" - Jean-Baptiste Alphonse Karr

Mucositis is a real bummer and Elise is suffering through it. I spent the night at the apartment last night, but Becky said Elise was up most of the night either with throat pain or diarrhea. We're still counting on the palifermin to reduce the severity and intensity she would have normally experienced if she didn't have that med, but even then the reduced symptoms are pretty bad.

The pain was bad enough that she couldn't eat, and worse, taking her oral meds was a real struggle. So they have transitioned her to IV meds where possible. They've also given her a "patient controlled analgesic" or "PCA". It's essentially a computer controlled morphine dispenser. Elise can press a button that will meter out a dose of pain medication. She can press it all she wants but there's a maximum dose that will be dispensed over a period of time. They say this is the best because they record how much she presses and so they can better judge her level of pain over time. It also gives her some amount of control in a situation where she has little control. The added plus is we don't have to wait 45 minutes from the onset of pain until the Dr approves the dose and the pharmacy dispenses and delivers it. All around it should be better.

Her fever seems to be on a roller coaster, probably due to the Tylenol they are giving her. They haven't cultured out anything in the samples they took yesterday (I guess that's good) so she's still on some broad spectrum IV antibiotics.

Her red blood cell count fell below the protocol targets so she got a bag of red cells. The PA said her platlet count looks like it will drop below the protocol targets by tomorrow, so she'll probably get a bag of platlets in the morning.

Elise said she found some hair falling out today, so that might be just around the corner. But Becky washed her hair this afternoon and didn't notice any hair loss. It would be real unusual if she didn't lose her hair, so we're all anticipating that to hit sometime soon.

It's back to the weekend here, so the hospital has largely cleared out for the next couple days. The weekend residents came by and checked in, but I'm hoping that we won't be seeing them too often. I'm also hoping that by then end of the weekend the mucositis will be easing off or maybe even gone. More importantly, I'm hoping this first fever will have run its course by then as well.

It's hard to believe its been a week since the stem cell infusion. By this time next week we'll have the first indications whether the donors cells are beginning to engraft in Elise's marrow. Now that's something to look forward to.

A Long Day (D+6)

"You're going to go through tough times - that's life. But I say, 'Nothing happens to you, it happens for you.' See the positive in negative events." - Joel Osteen

Elise had a tough night and day today. We'd hoped that she would avoid the mucositis  as it's the reportedly the most painful side effect of the conditioning for BMT. It's also common so they'd premedicated her with palifermin which can reduce or eliminate the symptoms. We we're hoping for eliminate, now we're hoping for reduce.

She had some minor swallowing soreness the past couple days, but it either subsided on its own or went away with Tylenol. Last night though she got the full effect. She had painful mouth sores and diarrhea as mucositis can affect mucosal tissue through the body. Between the pain and the visits by the nurse and the Dr on duty, it kept her mostly awake from 12 am till about 5 am.

Besides the pain factor, mucositis can result in increased weight loss (from not eating), infections (from the mucosal barrier breaking down) and longer recovery periods in general.

Tylenol was able to knock the pain down enough that she got some sleep around 5am, but it was back by about 8am. They decided to switch pain relievers since Tylenol might mask a fever from infection, so they gave her the lowest dose of oxycodone. It took about 1.5 hours to kick in but when it did - wow did she get loopy. She looked like me after my first beer. A little too loopy they decided so they will be dropping the dose back to 1/2 of the smallest dose. Hopefully that will allow her to eat, sleep and function somewhat normally until the mucositis passes.

As if that wasn't enough for one day, once they sorted out the pain meds, her temperature spiked above 38C. That's the magic threshold they use to decide if they should be concerned. The protocol is to draw blood cultures in every possible way and then treat immediately with a broad spectrum IV antibiotic ("Zosyn") and then do a chest xray. The blood cultures can take days to come back with results (which we learned on a vacation to Florida a few years back), so they need to treat without knowing exactly what they're treating. Zosyn is designed specifically for this purpose.

We've known it was a matter of when, not if she'd get some sort of infection. Everything we'd read had warned us of that, so we haven't gotten too worried yet. Her neutrophil count dropped to "effectively zero" today, meaning she has no immunity right now to anything, so it was only a matter of time. We're crossing our fingers that it not something difficult to treat. I've also read that they rarely ever find out what the initial infection was caused by - it's knocked out with the prompt treatment before they can figured it out.

We did get some good news today. She'd had the sniffles the past couple of evenings and so they did a swab and culture and then put her on "respiratory isolation" (masks for all my friends). That culture turned up negative so she came off isolation today. And they ran a PCR of a poop sample and it came up negative for the various GI infections that might be common (e.g. c.diff) so they're pretty sure the diarrhea is from the mucositis rather than something else.

Another good thing is that the rash from the palifermin is nearly gone. A few small red bumps remained on her forehead and cheeks, but looking at her you might just assume it's light acne (she's 13 after all). It's not itching at all and hopefully in a day or two it won't even be a memory.

And the last good thing I'll report is that I got Elise to try a milk shake. For some reason she didn't want to try one (!) but I convinced her and she liked it. She can have one with each meal and it will add some nutrition that also feels good on the throat. There are several flavors to choose from but she seems to like the "cookies and creme". They have some other similar options (slushes made with italian ice, "Boost", etc), so we'll be having her try those. She's a finicky eater though.

So the rough stuff has started, but its clear that the medical team here is all over it. They have a game plan and everyone knows it. I feel Elise is in very good hands.

What Do We Want? Boredom! (D+5)

"When you pay attention to boredom it gets unbelievably interesting." - Jon Kabat-Zinn

I haven't provided an update in a few days because, thankfully, there hasn't been much to report.

As predicted, the conditioning regimen has made Elise's absolute neutrophil count (ANC) drop like a rock. The ANC is a rough measure of the effectiveness of her immune system. In healthy adults the count is 1500 to 8000. Elise's dropped from 1090 on Monday to 510 on Tuesday and was 100 this morning. It should bottom out in the next week or so and then start rising again in the next few weeks. When it drops below 500 they say you are suffering from neutropenia and your risk of infection are significantly increased.

When the ANC goes below 1000 you get put in isolation, so starting on Tuesday Elise has been isolated in her room. Well people can enter if they take precautions (wash hands, no or limited contact, etc) but she can't leave.

Her throat has been a little sore from what seems to be mucositis. It's mild so far and we're hoping it doesn't get too much worse, but they've told us you can't really predict it. She's got several different mouthwash options including one with lidocaine in it, but she doesn't like using them too much. Fortunately Tylenol seems to help.

She's also lost some of her taste sensation so most foods lack their normal appeal (and she's never been a big eater to start with). As a result she's not eating as well as she normally would and has lost some weight. The nutritionist says this is normal and they talked to us about some strategies to compensate. We're trying their suggestions and we'll see how it goes.

On Monday she got a rash from the palifermin which is meant to help prevent mucositis. The rash was mild and it's starting to diminish already.

And this morning Becky said Elise had the sniffles during the night, so they are running a culture and have her on an added "respiratory isolation" until it comes back negative. It doesn't impact Elise, but the medical staff have to put on masks and gloves when they enter the room. Kinda discourages drop-ins which can be good and bad.

She's in good spirits but lethargic. The good spirits are definitely welcomed, the lethargy not so much. In one sense the lethargy is a good thing: it would be difficult to contain a hyper kid in a small room. She's spent most of her time reading, watching TV or surfing the web (ie watching silly Youtube videos), but she did do some crafting yesterday with an art volunteer and she's spent time with her tutor as well.

So I think we're in for more of the same in the near future. We're hoping for little or no excitement. If the next several weeks were boring then that would be exciting for us.

Thanks to Babs O'Neil for the bug pillow cases. They are really cool as Elise loves bugs (but I think Becky was a little creeped out).

Be It Ever So Humble (D+2)

"Home is any four walls that enclose the right person." - Helen Rowland

All is well here. Elise's neutrophil counts are dropping but not so low that she's isolated yet. She's feeling mostly fine, maybe a little fatigued but nothing else to report. They've started a few days of Palifermin to help ensure the typical mouth sores are minimized, fortunately Elise hasn't had problems yet but the peak is from D+3 to D+10 depending on who you talk to.

Because her counts are still up, we've been taking walks around the NIH campus in between bouts of reading, web surfing, TV and boredom. Yesterday Becky and Elise watched a deer for a while (while I spent a few hours at a few of the art museums). The NIH campus is in the middle of residential housing, but there is a healthy deer population that makes an appearance periodically. Surprisingly enough, a black bear showed up on the campus on Friday and climbed up a tree near the metro station until the wildlife management folks could dart it. It was quite the buzz around here.

So I thought I'd take the opportunity to give you some sense of Elise's living environment here. The picture above is the entrance to the Clinical Research Center. During the week its a bustling place as patients, visitors and employees come and go. It's unlike most hospital entrances because there's no emergency room here and it's not open to the general public. Rather it's on a federal installation surrounded by a (nice looking) fence and armed guards. Also, most of the employees come in through another entrance that's more convenient to parking and public transit options (metro, buses, etc).

The lobby area is spacious and nice to sit in and have a coffee. But only during the week. This is primarily a research clinic and so most patients are outpatient and most employees work M-F. On Saturday and Sunday almost everything shuts down and the place has the atmosphere of a bank on the weekend. This picture is on a Sunday, so there is no one here. In fact the kids wing (which is just off to the right in the photo) has only 5 kids this weekend including Elise.

There are nice seating areas on each floor around the atrium so you can find someplace cozy and/ore secluded and just hangout. There is a patient library on the 7th floor as well as a small gym and chapel. The gym is used for patient rehab but its also open a few hours each day for patient visitors. The library has the typical books, magazine and newspapers. They also have newer DVDs and CDs.

Dining for patients consists primarily of something very similar to room service in a hotel. There is a menu and you call to order what you'd like. They know what your dietary restrictions are and so they won't let you order things you're not supposed to have. Right now Elise is unrestricted, but soon she'll transition to a "regular immunosupressed" diet. She won't be able to have berries, lettuce, unpasteurized milk and egg products, etc - anything with above average loads of mold and bacteria. The menu is pretty large, but try eating every meal at the same restaurant for 6-8 weeks and even the biggest menu gets boring.

There are 3 eating options for visitors: Au Bon Pain has pastries, soups, sandwiches and most importantly coffee drinks. The main foodatorium is a short walk and offers a foodcourt like setting sort of like a cruise ship. Lots of variety until you've eaten there for a couple weeks. It caters primary to employees and is only open during typical work hours. There is a smaller cafeteria on the 2nd floor that has longer hours and is open on the weekends (but closed on Sundays at 6pm - I know). There's also a small alternate coffee place that opened this past year that serves coffee like "Velvet Hammer", etc but its only open during normal work hours.

The kids wing (called "1 Northwest" because its at the north-west corner of the building on the 1st floor) can accommodate about 20+ inpatient kids and a bunch more outpatient kids who are recovering from procedures, etc. Most of the office visits happen in other parts of this huge complex. It was originally constructed to house about 500 patients! The core of the facility was built in the 1950s and its been added onto ever since. The part Elise is in was opened around 2004 so its fairly new.

The wing has a "play room" with toys and craft materials staffed with recreation therapists, a "family room" with air hockey, Xbox, and a place to sit and watch movies as a group, a classroom staffed with 2 teachers (where Elise is going an hour each day), and they have a patient fridge, microwave, ice & water machine. They also have a linen closet so you can get new linens, towels, etc whenever you want/need.

The room Elise is in has a special type of air handling. It's a positive pressure system, meaning that air rushes out the door when you open it. That way contagions in the hallway can't get into the room (unless someone brings them in). There's even an alarm if the air pressure in the room drops or the door is held ajar too long. The air coming into the room goes through special filtration to eliminate molds, bacteria, and viruses. As I mentioned in my last post, the filters don't seem to remove odors (e.g. cream corn). There are other types of rooms on the wing that handle air differently, for example there are negative pressure rooms for kids with things like TB that they don't want to escape the room. But most rooms have standard ventilation.

The room has an TV on an articulating arm so you can watch it sitting in the love seat or in bed. They have the standard set of stations including a couple in house movie stations (one grown up one "family" oriented). It's hooked up to and xbox so you can play video games (none of us do) or you can watch DVDs.

The love seat in Elise's room slides open into a single bed that Becky or I occupy each night. One of us is required to remain with Elise 24/7 (well not exactly, they let us get meals at the cafeteria and they encourage giving Elise some "alone time"). We have a REI camp mattress that we use on top of the cushions to even things out. I can sleep pretty well but then I don't normally roll around a bunch either.

Elise's window fills one wall of her room. It faces North and opens out onto some greenery. It doesn't get any direct sun, which is a blessing as that would make things too hot at times or too dark at others. All in all for a hospital room it's nicely arranged/configured.

We've added a few pieces of furniture: a set of shelves and a couple tray tables. The shelves hold books, craft project materials, and in between meal snacks (Elise is a grazer). The tray tables hold our computers. We've also reserved a shelf as the charging station for the obligatory electronic devices. Thank God they have Wifi!

They allow us to decorate as we want and the walls are gradually getting covered with posters, get well cards, and artwork that Elise has created.

Well that's probably more than most of you wanted to know and less than anyone contemplating a stay at NIH needs to know.

Goldilocks (D0)

"A well-spent day brings happy sleep." - Leonardo da Vinci

This was a really big day: Elise received her stem cell infusion today. We don't know a lot about the donor, but we understand the person is male and from Germany. We'll find out more later after Elise recovers.

The medical team said the cell dose that Elise received was not too much, too little, it was just right. So I called it the "Goldilocks" dose. Apparently there is actually a dose size they compute as optimal and the dose Elise received fit in the "optimal" band. 

We're very grateful the donor took the time to register for their bone marrow registry and then also agreed to go through with the donation process. Not everyone does register and this is vitally important especially for those with mixed ethnic origins as the likelihood of finding a match is directly proportional to the number of people in the database that share your ethnic makeup. Low numbers mean low chances.

I'm also glad there is a national registry that coordinates with other nations to produce matches. The National Marrow Donor Program is something our politicians have got right.

If you are under 45 and in good health I urge you to join the national registry at BeTheMatch.org. Joining is free and painless. The chances that you'll be a donor are slight, but if you are matched then you could literally save someone's life.

The transplant was, as they warned us, a bit anticlimactic. They hang a series of bags of strawberry topping looking stuff that is infused via IV. All painless and boring. The bags of cells were thawed one by one from a frozen state and infused. They use a preservative which smells like "creamed corn". They told us this and it was the absolute truth. The nursed brought in peppermint oil, sliced lemons and oranges, and some fruit flavored life savers to cut through the odor. It's not bad for, say, Thanksgiving dinner, but try 4+ hours of that smell. Elise didn't seem to mind although her olfactory senses seem to have gone slightly wacky with the conditioning regimen. But this too shall pass.

They've started a med called Sirolimus. It's an immunosupressive agent used to prevent the rejection of, primarily, organ transplants. She'll be taking this med for quite a while. The levels in her bloodstream have to be monitored and adjusted regularly; apparently the level is important and it can fluctuate a bit.

Of course every med has its side effects and sirolimus can cause hypertension, so Elise has also started taking norvasc which helps address any hypertension she may get.

So now it's mostly a waiting game: we wait until the cells engraft, then multiply, and flourish. There should be some indications that they've engrafted in about 2 weeks. The first test of this is scheduled on 3 July. Once they've multiplied to a certain point then Elise will be released from the hospital inpatient status. This should be around day 30-45. After that she'll be an outpatient but required to stay in the local area until rough day 100 after the transplant.

In the mean time, during the next week her blood counts should continue to drop significantly. Her hair should begin to fall out. And she'll become susceptible to infections since her body won't have a mature immune system to fight them off.

After that she may suffer "acute" graft vs host disease (GVHD) as her new immune system and what's remaining of her old one try to come to a compromise. Eventually, she may also suffer from "chronic" GVHD if this compromise isn't amicable. GVHD can range from minor to severe and is graded on a scale of 1-4 (4 being worst). 

One of the "features" of the particular protocol that Elise is going through is that it's meant to minimize the chance of GVHD. But the tradeoff is an increased risk of the transplant not engrafting properly. We've been told the risk of GVHD is normally pretty high (30%+) and the risk of not engrafting is low (5%), so this is the "tradespace" the Drs are working in.

Becky took the odeur de crème de maïs shift at the hospital tonight, so I get to drink a beer and watch Amazon Prime at the apartment. I'll sleep happy tonight.

Day of Rest (D-1)

"We must free ourselves of the hope that the sea will ever rest. We must learn to sail in high winds." - Aristotle Onassis

Today was a "rest day" meaning no treatments rather than a day to rest.

Elise has an echo cardiogram and got wired up for a 24 hours Holter monitor, which is an iPod sized device that records your heart for 24 hours. This was required because the Campath she was treated with is associated with cardiac problems in some cases. So they ran these tests before and after the Campath treatment.

The person doing the echo cardiogram gave an early conclusion as he examined her heart function (which was pretty cool to watch). He said everything looked fine. I'm assuming the 24-hour monitor will say the same thing.

Other than that and and an hour with her tutor, today was pretty calm.

The pediatrics PA said her blood counts are going down as anticipated from the conditioning, but not so low that she couldn't go out in public areas. So she suggested we go out for dinner as it might be the last chance for a long time. We took her up on the offer and went to dinner where Elise could get her request: "warm chocolate melting cake". We had a good time and stuffed ourselves. It was a nice evening out.

Tomorrow is the big day: she gets the donor stem cells via an IV infusion. It's sort of a 2nd birthday.

The donor's cells have been frozen since he donated a couple weeks ago. They are preserved with something and they've told us that the preservative will have an odd odor, something like "creamed corn" they've told us. They swear it will pass, but apparently it's noticeable enough that they've warned us. Should be interesting. I probably won't be eating corn chowder in the cafeteria anytime soon.

So all is well and we are excited about tomorrow. Keep Elise in your prayers going forward as difficult times are still to come: susceptibility to infections, possible graft vs host disease, and being stuck inside four small walls for a month or longer.

Houston We Have A Go (D-2)

"There are two kinds of people, those who finish what they start and so on." - Robert Byrne

Elise has finished the "conditioning" phase of the stem cell transplant process and is ready for the "transplant" part of the process on Friday. Two different courses of chemotherapy drugs and then a couple session of total body irradiation are behind her.

The total body irradiation was done in two parts. The first one had to be precisely targeted to avoid damaging her lungs. Even after all the setup on two different pretreatment sessions, on treatment day this took a bunch of set up and adjustment by the technicians and a verification check by the Radiation Oncologist. It made me feel a little more comfortable that they were being very cautious.

The technicians were great. They were playful with Elise and helped to keep her calm. It's sort of unnerving because they put you in this huge cavernous room with the machine, then tape you to the table so you don't move, and then everyone walks out and leaves you there alone. The technicians let her pick out the music she listened to during the treatment and they made a good game of how she was torturing them with the Frozen soundtrack ("Not Frozen! We have to hear that all the time. Oh you're torturing us!")

Elise walked in and out of both sessions, which was a good sign although she seemed pretty tired after the second one. Her tiredness/crankiness peaked when the nurse told her she needed another IV of the anti-nausea med. She was pretty irritable and I think she wanted a bath to get all the marks off, and the IV prevented that. The anti-nausea drugs must have helped because Elise never felt nausea. That was a huge relief - nausea is the worst.

After a few rounds of crying the nurse made her real happy by telling Elise that there were no more IVs planned for the day. And then she said they'd let Elise sleep through the night (they'd been waking her for vital checks every 4 hours). That made Elise happy and left the day's events on a positive note with her.

The pediatrics PA dropped by in the afternoon and said everything was looking good so far. She said that Elise's conditioning had been "textbook" meaning there had been no unplanned excursion had been necessary (I guess they often are).

So next is a "rest day", meaning no treatment rather than "resting". She'll get an echocardiogram and then be wired up for a 24-hour Holter monitoring of her heart. These tests mirror ones she went through before the chemo. One of the chemo agents (Campath) has been associated with causing heart issues in rare cases so they want to make sure it hasn't done that in Elise.

On Friday Elise will get the infusion of the donor's stem cells. It's a big day but they say its pretty anticlimactic because its basically just another IV. But it's a huge step on the road to a cure!

BTW - all those marks on Elise's skin in the photo are the targeting indexes for the total body irradiation she underwent.

2 Down, 1 To Go (D-3)

"A simple grateful thought turned heavenwards is the most perfect prayer." - Doris Lessing

Today Elise completed chemotherapy conditioning for the bone marrow transplant. That 2 of 3 steps complete. Yeah! 

They woke us up early for some reason and started the premedication at 6:30am. This was after we were up late because they started an IV drip of an anti-seizure med really late. I'm really not sure why the nurse chose to start so early as Elise had very little else scheduled for the remainder of the day. I'm sure she was just trying to comply with what she though the Dr's order where, but it really got Elise out of the routine we'd established. She was stuck on an IV until about noon. She wasn't able to shower or get dressed before they started (try to get a clean shirt on when you're attached to an IV) until after the IV's were complete. Needless to say, she's a bit emotionally fragile right now and the slightest little thing made her break down in tears. But she cheered up pretty quickly when Becky took her to see the guinea pigs again.

Fortunately Becky got her smiling again and the prospect of a cupcake from Au Bon Pain got her through the setup for the total body irradiation tomorrow.

Tomorrow is the last step in the conditioning process: total body irradiation (TBI). She'll get a relatively low dose compared to people with blood cancers who are getting a transplant, but its still a significant dose so they are extremely cautious. They calculate your body mass and do measurements to make sure they don't overdose any separate part of your body. For example, they do regular xray to locate the edges of her lungs and then shield that section (because it has less body mass) partially. They also adjust for how thick you are in the torso, etc.

To make sure they get this all right tomorrow they spent time this afternoon marking up Elise's body with Sharpies and little targets and getting the computer programmed and double checked. With all the body marking I joked that it looked like she was art therapy for the little kids. Fortunately I got a smile and not a glare. She's been a little self conscious about stuff like the catheter and the xray targets stuck to her body, but after the mark up session she walked right into Au Bon Pain and picked out her cupcake.

She'll have two TBI doses tomorrow. One at 8am and then one again at 2pm. We've been told she won't feel anything but may feel tired afterward.

The pediatric PA comes in each morning and checks on Elise and gives us a rundown on her bloodwork. She said Elise's liver function is looking good (a relief because she's had problems with new medications in the past) and her counts are still up but starting to drop as a result of the conditioning process. This is what the conditioning process is all about, but it will make her extremely susceptible to infections in the coming weeks. When the counts go below a certain point she'll be isolated in her special room until they come back up. We're not sure when that will start, but I think it'll be within the next several days.

I'm glad that Becky has been able to join us in the hospital. In fact I'm back at the apartment this evening and Becky is spending the night on the extra special sofa bed they have in the room. If you like an extra, extra firm mattress, then you might like that sofa bed. I'd gotten used to it, but I'm looking forward to a normal bed tonight, but I will miss giving my daughter a kiss goodnight.

Goofy Girl (D-4)

"Mix a little foolishness with your serious plans. It is lovely to be silly at the right moment." - Horace

This was the first of two planned days of chemotherapy with a drug called busulfan. Busulfan suppresses the activity of the bone marrow which produces your blood cells.

They started early by premedicating Elise with Zofran, Dexamethazone and Keppra.

Zofran reduces nausea. Dexamethazone reduces inflammation. And Keppra helps prevent seizures associated with higher doses of busulfan.

After the premeds, they started the busulfan IV. There was really nothing to it. Nothing to report.

Following the busulfan infusion they draw blood every 15 minutes and then 30 minutes then hourly for a while. They are measuring the amount in her bloodstream so that they can tell how fast her body metabolizes it. Apparently kids aren't the same as adults and the exact dosage is really important. If she metabolizes it too fast they may need to do an additional day of infusion to make sure her bone marrow function is suppressed enough for the donor's cells to engraft.

If you read my blog yesterday you may remember that there is another drug that prevents seizures that can cause behavioral issues, but Elise got Keppra that isn't supposed to have this side effect. Well I don't know what did it, but Elise was one goofy girl almost all day. Not way out of control goofy, but on the higher end of her normal goofy spectrum. She was much higher energy than she's been recently. It was nice to see, but a little curious.

Becky was able to join us in the hospital today, and I think that may have contributed to Elise's giddiness. Having someone else around had to be refreshing. I know it was for me.

In between blood draws and various visits by the medical team, Elise went to her daily tutor session and recreation therapy. She was really excited because they had guinea pigs and she's been watching all manor of pet videos on Youtube so something cute and furry was a nice distraction.

Tomorrow is more of the same without all the blood draws since they'll already have measured her ability to metabolize the busulfan. Hopefully it will go smoothly.

Also, tomorrow Becky is planning to spend the night at the hospital so I can sleep at the apartment. That will give Elise someone else's snoring to complain about...

It's late and they just gave Elise another IV dose of Keppra and then Zofran. The goofyness is definitely from the Keppra. She turned into Chatty Cathy almost as soon as they plugged the IV in and she seemed to really perk up. I hope she can get to sleep!

Levetiracetam and Ursodiol and Busulfan Oh My (D-5)

"To a father growing old nothing is dearer than a daughter." - Euripides

I didn't post yesterday because there wasn't really much to say. It was Saturday and the hospital was really quiet. Elise had her 4th of 5 doses of Campath and everything went smoothly. She did have a low grade fever from late Friday until early Saturday morning, but nothing anyone seemed worried about (except me).

Today was more of the same. She got her last dose of Campath this morning and she slept through most of it. She hasn't had any fever following it (so far). After sleeping for a while she woke up refreshed and seemingly unaffected.

As with yesterday, Becky came down to the Hospital campus and we spent several hours in a picnic area outside the Children's Inn which is right next door to the hospital. The whole NIH campus has nice grounds and the weather was really awesome, so an afternoon outdoors was welcomed by all of us.

Today Becky brought pizza, bread sticks, and (drum roll) key lime cheesecake for me for Fathers Day. My favorite! Yum! (Thanks Doug and Dottie for the latte money!)

Becky is still coughing so she can't come into the hospital. It seems to be worse in the morning, so if her coughing calms down tomorrow later in the day we're going to give her a mask and see if they let her in (more like see if they kick her out).

Today they started Elise on a drug called ursodiol. It's used to help protect against hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome. VOD is a major complication of hematopoietic stem cell transplantation and it carries a relatively high mortality. The risk factors for VOD include just about everything they do for transplant so trying to prevent it is a smart thing to do. From what I can tell the prevention medicine doesn't always work, but some protection is better than none.

They also plan to start Levetiracetam (Keppra) this evening to prevent seizures during the next course of treatment with Busulfan. The dose of busulfan that Elise will get is relatively low, so they wouldn't expect any seizures, but they want to prevent them if they can. I was relieved to hear they were going to use Keppra instead of Lorazepam as adverse effects, including amnesia, depression and effects such as excitement or worsening of seizures, may occur (per wikipedia). Kids are more sensitive to the effects and several people (including the transplant staff and nurses on duty) had warned us that it could be a rough couple of days. The studies show that both drugs have appropriate outcomes, so based on that limited info I have I'm glad they are going with Keppra.

Tomorrow Elise has the first of two sequential days of a drug called busulfan. Busulfan "causes profound myelosuppression at the recommended dosage", ie it significantly suppresses the function of the bone marrow. This is the "big gun" drug in the conditioning regimen for a stem cell transplant. Elise will receive a somewhat reduced dose compared to someone with leukemia. Other than the minor fact that it suppresses the function of your bone marrow where all your blood cells are produced, it has minor side effects that are listed on any medication you buy: headache; diarrhea, constipation, nausea, upset stomach; flushing. There are more severe, but also more rare acute side effects as well but I won't list them here.

We're hoping that the tolerance Elise has had for the conditioning regime so far will continue this week. Friday is the big day, she's scheduled to get the donors stem cell infusion. So keep Elise in your prayers.

I turned 52 on Saturday. While I wish I could have been out hiking, running, woodworking or with Becky at Lelani's drinking a mai tai, I really couldn't think of anyplace better to be on my birthday than helping my daughter progress towards a cure. After all, I want my daughter around when I grow old, which isn't any time soon.

How To Train Your Dragon (D-7)

"Be happy for this moment. This moment is your life." - Omar Khayyam

Today was the 3rd of 5 sequential days of Campath medication. It's old news by now which Elise put an exclamation point on by doing math homework during 2 hour the IV infusion! I was shocked. I guess chemo is just so yesterday... Funny how something like chemotherapy can become mundane, but I'm happy its going well and she's adjusting to it.

Elise has two more days of Campath (Saturday and Sunday), which should be pretty boring since, except for the inpatient wings of the Clinical Center building, NIH mostly shuts down over the weekend. I think there are maybe three other kids on the wing.

So tomorrow and Sunday are doses of Campath and not much else going on.

On Sunday night she'll get a dose of a prophylactic anti-seizure medication in preparation for the Busulfan chemo treatment on Monday and Tuesday. Busulfan can cause seizures at higher doses and while Elise will be getting a relatively low dose (compared to, say, a leukemia patient), they want to make sure she won't have a seizure.

Elise had been bummed that she was going to miss the opening of the movie "How To Train Your Dragon 2" because she'd be in the hospital. She really liked the original movie and was looking forward to the sequel all year. But she's been doing well on the Campath regimen and so when the medical team did rounds today she asked if she could get a pass to go to the movies. She was all smiles when they said "Yes".

So after a session with the counselor and an hour with her tutor, they released her for the evening. We went to the movie (which was really good), went out for dinner, and then dropped by the apartment for a fresh set of clothes and a few odds and ends (paying bills, updating Becky's computer so she can do electronic scrapbooking, etc). It was a nice evening out, but now we're back in the hospital winding down for bed.

It's been a month since we left California for Maryland. I can't believe it. I hope the next 3 months go just as fast!

More of the Same Only Better (D-8)

"There's never one sunrise the same or one sunset the same." - Carlos Santana

Today was more of the same as yesterday only without the fever. This was day 2 of 5 successive days of treatment with Campath as part of the conditioning regime preparing Elise's body for transplant of the donors stem cells on 20 June 2014 - a week from tomorrow.

After waking us up at 5:30am to draw blood and collect her vitals neither of us could really get back to sleep. So we got up and around. The day nurse came on shift and got the ball rolling about an hour earlier than yesterday. She premedicated Elise and then after about 30 minutes started the Campath drip that takes about 2 hours. They ramp up the Campath dose each day so they'd warned me that she might respond more strongly the 2nd day before starting to taper off. So I expected a fever like yesterday.

I was pleasantly surprised when no fever or other side effect happened. Elise was drowsy from the benedryl premed, but that was it. No headache, nausea, etc.

By 1300 she was up and around. She went to recreation therapy for some arts and crafts at 1330 and then for an hour of math tutoring at 1430. Elise likes to draw and they have an art therapist who comes around occasionally. She met with Elise today and Elise drew Poseidon's Trident. The therapist said the art is supposed to signify something, but I was kicked out of the room during the session so I don't know what it is.

So other than being in a hospital, today was a good day. They say the remaining 3 days of Campath should only be easier. Knock on wood, but I'll take more of the same.

Pronounced a-lem-too-zoo-mab, or "Campath" for the Rest of Us (D-9)

“I pass with relief from the tossing sea of cause and theory to the firm ground of result and fact.” - 

 Winston Churchill

After 6 months of planning, hospital visits, middle of the night worry, and soul searching, Elise started the conditioning process for her stem cell transplant today with the first of five doses, one each day for the next five days, of a drug called Alemtuzumab.

Alemtuzumab, or "Campath" is  monoclonal antibody that binds to a protein present on the surface of mature lymphocytes, but not on the stem cells that create these lymphocytes. After treatment with Campath, these antibody-bearing lymphocytes are targeted for destruction by the immune system. Or in layman's terms, this medicine causes your body to kill off its own T-cells which fight infection.

This is the first of three "big gun" conditioning therapies used before the transplant to prepare Elise's immune system to accept the donor's stem cells. The next two are Busulfan and then "total body irradiation", but I'll cover those two in a future post.

Campath is given slowly over a couple hours for each of five consecutive days. The side effects are said to be the worst on the first day and gradually becoming less severe each day after. The official website says: "Campath administration can result in serious, including fatal, infusion reactions. Carefully monitor patients during infusions and withhold Campath for Grade 3 or 4 infusion reactions."

They prepare for the worst. Before they started the drip of Campath, they premedicated her with a max dose of tylenol and benedryl. The tylenol prevents the fever, chills and body aches that are the predominant side effect of Campath. The benedryl reduces the chance of an allergic reaction and also helps to calm the recipient.

I was prepared for what I thought would be the middle of the road reaction: an allergic reaction that caused some breathing issues and a fever and body aches. What Elise had was nothing of the sort. The benedryl made her sleepy and she dozed off and on only to be woken by myself or a nurse to ask her how she was doing or check her vitals. She did get a 102 degree fever about an hour and a half after the dose was complete, but it's resolving itself and she seems mostly herself. We went for a short walk around the hospital and watched some funny cat videos on YouTube. Right now, she's sitting on the couch watching a movie before bed.

I think the medical staff is somewhat surprised. They came in frequently and seemed surprised each time. In a good way.

I'm told days 2-5 of Campath gets progressively easier as the patient's body gets used to the medication. I hope that's true. I'm sure there are rough days ahead, but the roughest thing about today was the anticipation.

Becky is still coughing pretty bad so she wasn't her today but we swapped texts, emails and phone calls so she was kept up to speed as the day progressed, but she didn't miss much. Her presence was felt though: she'd helped Elise pick out some bedding to put on the hospital bed to make it more like home. She also sent along a "How To Train Your Dragon" poster which Elise and I hung up. Elise is crazy about the new movie coming out and she's bummed that she'll miss it while she's stuck in the hospital. So she was happy to get the poster.

As for me, I spent the day working on a short story I started writing last week. It's sort of a scifi genre based on Hugh Howey's "Silo Series". If I'm happy with it then I'll publish it on Amazon under the Silo Kindle Worlds umbrella (it's set up for self publishing fan fiction writers). If even I don't like it, then it will be nothing worse than a good diversion and some exercise for my fingers. We'll see.

Godzilla? Not in Tokyo! (D-10)

“Remember tonight... for it is the beginning of always” -  Dante Alighieri

Today was the last day of preparation for the transplant conditioning regime. Elise received her last dose of Palifermin before the series of 8 days of chemo and radiation conditioning starts tomorrow. She also had an appointment with the Radiation Oncology group for a "Radiation Simulation". They did another CT scan and took a bunch of measurements so that they could compute the correct total body irradiation (TBI) dosages. They also marked indexing targets all over her body and then covered them with some clear protection tape so they don't wash off before her treatments next week.

They radiation oncologists are quite careful and meticulous about this process: the measurements and markings are independently verified and the calculations require a formal review process that takes a week. They even make a special mask for her lungs so that they don't get too large a dose. The day before the TBI they will make sure the custom made lung mask is properly fitted and put more indexing marks on her chest for placement of the mask. They said the indexing takes about 45-60 minutes but the treatment will only be about 3 minutes.

Elise also had another session with the teacher. The teacher she working with at NIH has contacted her math teacher in Ridgecrest to tailor the lesson plan for the summer and they've started off on the plan. While I don't think she's thrilled about having a 1 hour math class each day, Elise said she liked the one-to-one instruction that isn't available in the classroom. I hope it helps keep her engaged and helps her cover what she missed during all our trips to NIH in the past 6 months.

Elise is getting pretty self conscious about things right now. She's sure everyone is staring at her. The catheter bandage and hospital bracelet were more than enough to trigger it, but the large number of radiation targets/indexes that are visible put it over the top. She's trying to be tough but she's clearly only got a thin veneer left holding it all together. Being different when you're 13 years old can really suck.

Eventually they decided to let us go out on pass again for the evening. We have to be back first thing in the morning to do vitals, etc before the first dose of Campath. This is where things start getting physically tough for Elise. They say she'll have a strong reaction to it much like the flu. After tonight there will be no night passes until late July which will be somewhere around 30-45 days after the transplant. This is typically the time that her new immune system should be strong enough to handle limited exposure outside the hospital setting.

So we took advantage of the hospital pass and did what Elise wanted to do: see the new Godzilla movie. We took advantage of the nice weather and walked to and from the theater (about a mile each way) which is probably that last outdoor exercise she'll get for a while. The movie was actually better than I anticipated and Elise liked it. All in all a nice evening.

And A Big Chocolate Dipped Cookie for You (D-11)

“People who live in difficult circumstances need to know that happy endings are possible.” -  Sonia Sotomayor

For a day that I thought would be pretty laid back, we stayed pretty busy.

When we arrived at the hospital we thought that they'd take her vitals, flush her central line, and give her a dose of Palifermin and then we'd have to wait around for a 15 minute class on how to take care of her central line. Mostly it would be a day of cooling our heels. No, no, no. We found out that there was much more on the plate than that. 

Elise had a Pentamidine breathing treatment scheduled at 9 am that we were unaware of. She also was supposed to start a new medication, Acyclovir and get blood work done. The blood work needed to be done before the pentamidine treatment so that it was available during "grand rounds" later in the morning. And the palifermin also needed to get done before the pentamidine because the medication mixture goes bad within an hour of mixing and it was already mixed.

So the nurse hustled and got it all done before we headed off for the breathing treatment. I was impressed by how organized and on top of everything she was given the changing timeline.

The acyclovir is an antiviral that helps prevent thinks like herpes (of different varieties), CMV and other nasty infections from taking hold while Elise's immune system is suppressed.

Pentamidine is used to prevent pneumonia caused by the organism Pneumocystis jiroveci (carinii). The treatment is given by a nebulizer and it takes about 30 minutes once per month. It apparently tastes really bad and can also cause an asthma attack. Even with the warning Elise just about gagged when the treatment started due to the taste. She had a slight drop in her blood oxygen level and so they had to giver her albuterol treatment about halfway through it. Apparently this is fairly common. The respiratory therapist said next time they'd just start with the albuterol. In any case it took about an hour before the taste left Elise's mouth enough that she didn't have a sour look on her face.

Following the pentamidine treatment the grand rounds started for Elise's study. About 10 MDs and PAs crammed into Elise's room, asked some questions, poked and prodded a bit, and then answered questions we had.

My first question was about the switch to fluconazole from posaconazole that Elise has been taking for a couple years to prevent fungal infections. We'd tried a few other triazoles in the past and posaconazole seemed to work for her, so I was curious about the change. They said there were two reasons for the change: 1) fluconazole is better at preventing candida outbreaks which are more common in transplant settings; and 2) they wanted something stronger available (ie posaconazole) to use in case something unusual cropped up.

Elise's blood work also came back and showed that her potassium level was slightly low. I'd never noticed if it was low in the past. In any case, they gave her a potassium supplement and will be watching it along with her extended blood panel on an almost daily level from here on out.

Elise also had "school" today for an hour. The local school system has a classroom in the children's wing that is staffed by two teachers. I'd spoken to them about keeping Elise fresh during the summer and also helping her with math. Elise has done well in school but with all the travel to NIH this past semester (4 different trips), her math understanding suffered. So they are going to try to go back and pull her forward so that she can start the next school year on step. They actually assigned her homework tonight! It's a good thing as she's getting really bored and can get testy when there's nothing to occupy her time.

In the afternoon Elise and I went to a class on how to take care of her vascular access device (VAD) which was placed last week. It's a common source of infection (which is particularly bad for the immuno suppressed like her) so good "care and feeding" is important. I figured the class would be about 15 minutes, but it went over an hour with hands-on practice followed by a test. Yes a test! Elise and I both scored 100%.

After that we went back to the room where they went over another study assent with Elise. They are really good about going over the details and really giving you the option to opt out. Just about everything requires a consent so this wasn't the first and won't be the last. This particular one allows them to collect data on the donor/recipient pairing and outcomes for future study. Elise's most favorite study (not!) is one where they collect poop and have you fill out a survey about how you feel. They said they are finding out a lot about how the microbiotics in your system affect your overall health in significant ways; think about when they found out that most ulcers are caused by a bacteria rather than stress. Who would have known? In any case Elise thinks that one is embarrassing and weird, and she thinks the Dr. is a little too interested in poop.

The day ended on a high note: the Au Bon Pain in the lobby had the chocolate dipped shortbread cookies that Elise likes. And they've let Elise out on pass for another night. So it wasn't all bad.

One Step At A Time (D-12)

"Sometimes you don’t know when you’re taking the first step through a door until you’re already inside.” - Ann Voskamp

Elise had the first of three pre-chemo doses of Palifermin today. She'll have three more doses right after the stem cell transplant. It's meant to reduce the severity of the oral mucositis that people get from the chemo and total body radiation used as conditioning for a stem cell transplant. As Wikipedia says:

"Palifermin reduces the incidence and duration of severe oral mucositis by protecting those cells and stimulating the growth of new epithelial cells to build up the mucosal barrier."

We were told it is generally well tolerated, but there are some potential side effects that might not be good. Given Elise's "luck" I was worried she might not be able to tolerate it successfully and that would mean she'd get the worst of the mucosal issues (think extremely painful mouth sores and possibly sores throughout your GI tract - not good). But I needn't have worried, the first dosage of Palifermin was pretty uneventful. She became slightly flushed for a little while, but that was it. We stayed at the hospital for a couple hours to make sure she didn't react poorly and then they let her leave on pass again.

The central line has to be flushed every 24 hours to make sure it stays open (I guess blood can clot it closed), so we had to visit NIH each day this weekend even though she had a "pass". On Saturday the flushing wasn't bad but changing the dressing was quite painful for her. She's always been extra sensitive to tape on her skin being removed and the dressing is essentially a water tight seal around the site the catheter exits her chest - its strong glue. Add to that that she is still tender from the procedure and you can guess that changing the dressing on Saturday wasn't pleasant for anyone, least of all Elise. Worse, they had to change it twice because it started bleeding (very minor) shortly after they finished changing it. All that commotion really wore her out, but she still wanted to see the new Tom Cruise movie. So Becky dropped Elise and I off at the theater (Becky was still feeling puny from the respiratory infection) and afterward Elise felt good enough to walk back to our apartment (about a mile).

A funny thing about flushing the line: Elise can taste and smell what gets injected in the line. Somehow it travels through the bloodstream and she has an olfactory sensation. The nurse said this wasn't uncommon.

Tomorrow (Monday) Elise has he 2nd dose of Palifermin, some bloodwork, and then we are scheduled to take a class about how to take care of the catheter (it can be the source of serious infections if not maintained properly). There might be some other tests and consults that pop up, there usually are. We're hoping that Elise can continue to get passes to stay at the apartment until Wednesday when the heavy meds start.

Becky still isn't quite up to snuff yet, but her coughing was diminishing today and if she continues to improve she'll probably join us at the hospital on Tuesday. As for me, I've been able to workout in the apartment complex gym every other day. It's a pretty decent facility with cardio gear and a set of weight machines. I'm not much of a gym rat, but it's good for me because I'm still recovering from the knee surgery in December . Being in the gym (vs the hills or mountains) keeps me from over doing it. Plus it helps me sort out my thoughts and burn off some energy.

It's odd how anticlimactic everything seems. It's just one step at a time and then you find yourself in the middle of it. I told Becky that I hope it continues like this and in a few months we'll just find ourselves on the other side of it with a daughter cured of CGD and wondering where the time went.

Keep Pressing On

“Therefore I tell you, do not be anxious about your life, what you will eat or what you will drink, nor about your body, what you will put on. Is not life more than food, and the body more than clothing?" - Matthew 6:25

Last night we slept a little better than the previous one. I guess sleep deprivation helps, although the nurse shift change brought the midnight vital sign check and attendant bladder attention call. And then, for whatever reason, they decided Elise needed Tylenol at 6 am and proceeded to wake her up to give it to her. Uh OK I appreciate the thought I guess. But that's all just wanking...

Some really good news is that Elise is off "contact isolation". Her PA pushed the issue and convinced the epidemiologists that 3 negative test in a row should be sufficient. That means that she's not locked in a room (yet) and everyone who comes in doesn't need to "gown up" every time they enter and "degown" every time they step out. Being on contact isolation is an incredible hassle and it means that people don't just drop by to see how things are going. But now she's off and that's a relief (not to mention that Elise doesn't feel quite as stigmatized).

She's feeling Okay although the central line placement and bone marrow sample procedure have left her sore. The tylenol seems to help, but I'm sure in a few days she'll be as good as new. Just in time to start chemo next week (Wednesday).

At rounds this morning her medical team brought up the idea of delaying the transplant further in order to resolve what appeared to be constipation. After an xray and a GI consult they decided this wasn't necessary, but there were several hours that I wasn't sure how how things would go. Doing the right thing is paramount, but further delays would just be demoralizing, so I was glad they've decided that a further delay isn't necessary to go forward.

Else got her Lupron shot today so she won't have a period for 3 months. That should help when her platelet counts fall and also keep her general blood counts better. While I think she was happy about this, when the nurse brought in the shot she was taken aback: the needle was about 2 inches long! Turns out it only needed to go in 1/2 inch or so but it did freak her out a little.

She also had a session with the ENT today. They used a long thin camera to look inside her sinus to make sure there wasn't an infection. The CT scan a week ago showed some fluid buildup and they wanted to make absolute sure. It was pretty cool to watch - I told it looked to me like going through Luray caverns without the stalagmites and 'tites (ie boogers). The stuff they used to numb her nose was apparently pretty atrocious tasting, but not as bad as advertised. She did really well with the procedure and checked out 4.0.

The candida test also read positive which was what they hoped for (read the earlier post on the subject). This was a sigh of relief because after an initial positive result it had cleared up and looked negative. It only started looking positive again this morning.

The really good news is they gave her a "pass" to stay at the apartment tonight and Saturday nights. We have to go in to have her catheter lines flushed and apparently they want to start her on a new antifungal tomorrow, but that should take an hour and then we can go do something more enjoyable than sit in a hospital on the weekend.

On Sunday she starts the conditioning medication buildup. The first medication is Paliferman. The conditioning routine will cause mucositis which is a painful condition where mouth sores (and possibly sores through the GI tract) occur. Some people say this is the worst part of a BMT. Palifermin reduces the incidence and duration of severe oral mucositis by protecting those cells and stimulating the growth of new epithelial cells to build up the mucosal barrier. But of course it has it's own side effects. If these don't hit Elise too bad then we may get some additional nights in the apartment.

So we have a nice weekend ahead. Tonight we went to Rockville Town Square and had a drink and listened to a blues and for a while in an outdoor courtyard area. Tomorrow we'll likely go to see the new Tom Cruise movie or Godzilla. And with the additional time, maybe Becky will be well enough to join Elise by the time the really tough meds start next week.

So all is well, and I'm trying real hard not to be anxious, but it's difficult.

Happy Juice

"Spectacular achievement is always preceded by unspectacular preparation." - Robert H. Schuller

Today was event filled.

The best news first: the donor was able to provide a stem cell donation and preliminary tests indicate that its a "good product". The donor is from Germany so their process was much earlier in the day so we had initial good news by about lunch time. Later I received more definitive information and also that "the product" had been received by NIH already. I guess FedEx delivery is really fast!

They'll be freezing the donated cells until Elise needs them in a couple weeks. I'm told they actually separate them into several individual packets and spread them across multiple freezers to minimize the risk (say if a freezer broke).

We also heard today that the donor has type O blood while Elise is B. She'll gradually change types over the next year after the transplant. Apparently there might normally be some complications from having different types, however one of the meds they are using for the conditioning (Campath) prevent these complications. In any case one of the folks said that if they took a spit swab (say from an envelope or drinking glass) and compared it to a blood sample from a crime scene that the CSI team would be confused because the DNA and other info would be different. I'll bet there's an episode like this.

Now for the "routine" news...

Elise had her vascular access device (VAD, aka "central line" or simply "line") placed this morning. She was sort of looking forward to it because they would give her "happy juice" to put her under (valium followed by some drug cocktail). They insert a catheter into an artery in her neck and then install a line under the skin from the artery to a dual access port that comes out her chest right of the centerline. They can use this to get blood samples, inject medications, do transfusions, etc without a million needle sticks. Elise will have this with her until just before we leave. She can shower with it, etc with a little care. But baths are out.

She seems OK with it. I guess she's known for a long enough time so she got comfortable with the idea before we got here. The only complaint she's had about it is the incision they made to access the artery. It's sore and feels weird (sort of tugs on her skin when she turns her head) because of the closure method they used: super glue. It forms a rigid scab-like covering that will eventually peel off. They say its faster to apply, faster to heal, and minimizes scaring.

While she was still unconscious from the VAD placement, another group went in and took a sample of her bone marrow and a core sample from her hip bone. They run some tests on this but also use it for later on down the road (about a year) when they'll do another one and compare the results.

The puncture for the bone marrow sample came from her posterior and she's a little sore from that. Tylenol seems to help a great deal, but I don't think she feels the trade off for getting "happy juice" was a good one.

We found out that they've decided to give Elise a Lupron injection that will interrupt her periods for a few months. Because her platelet counts will drop as a result of the chemo, they want to reduce any unnecessary bleeding. I don't think Elise was too upset to hear about this.

Tomorrow we get to visit with the ear, nose and throat specialists who are going to get a look at Elise for a baseline. One of Elise's Drs warned her that they'd likely want to spray some stuff up her nose that smells and tastes "like feet". I'm sure Elise is looking forward to that!

Sometime tomorrow we'll get training on how to handle the new line in emergency situations. They gave us a handout that includes the precaution "never use scissors near your catheter". I guess they've seen it all. In any case is all goes Elise will get a "pass" and be able to leave the hospital over night this weekend. Because the full catheter "care and feeding" class isn't available until Monday, we'll need to drop in once every 24 hours to have them flush and inspect the line. That's a good trade for a night in a comfortable bed (for me).

Becky broke down and visited urgent care today, so she's being medicated for a "respiratory infection" and hopefully she'll be allowed in the hospital starting next week. She was on her own and had to find a GEHA approved urgent care and pharmacy (the latter was not too hard because there's more CVS than Starbucks here).

So all is well and most of the groundwork is done.

Off to the Races

"Hope is like the sun, which, as we journey toward it, casts the shadow of our burden behind us." 

- Samuel Smiles

After months of preparation the journey has begun. After eating a big breakfast of chocolate pancakes with chocolate chips and whip cream at IHOP, Becky dropped us off at NIH and then Elise checked into the hospital. Becky couldn't join us because she's still coughing and recovering from the head cold she came down with. Officially, Elise isn't anticipated to be discharged until late July, 30-45 days after "day 0", they day they infuse the donor's stem cells.

First up was the typical intake questions and vitals taking. The same questions are asked about 5 times by 5 different people, on purpose so that they make sure they get a complete an accurate story. Same with the vitals.

Next, Elise got an IV placed. They only needed to draw some more blood today, but the procedure to place the Hickman catheter is real early in the morning tomorrow so they got the IV part out of the way already. I doubt that means we'll get to sleep in any later though.

After the blood draws there was a pre-anesthesia consult. I always wondered why they check your neck flexibility. Apparently they're checking to see if they can easily insert a breathing tube if necessary. They also always ask if you know what procedures you're having done. I'm not sure why they do this, hopefully it's not a double check of their paperwork!

The last thing was a candida test. I was curious about this one, but it's simply a double check of another test. Apparently everyone has been exposed to candida so everyone should test positive for it. If you test negative then it's most likely an indicator that the test methodology doesn't work on you for some reason. So they administer the test in the same way they test for TB (a small bolus under the skin on the forearm). That way, they can double check that a "negative" TB result wasn't a "false negative". Pretty clever, but it also shows the importance of getting these right.

Tonight will be the first night of many spent in the hospital in the coming weeks. Hopefully Becky and I will be able to switch off as planned because I'm not sure how much "Turtleman" on Animal Planet I'll be able to take. Not that I have to watch it, but Elise loves the show for some reason and insists on watching it when we let her watch TV. (Fortunately we don't get Animal Planet at home). An episode is fine, but she'll watch 2 hours of the show if we let her.

Elise's room is a private room with special air handling. They said the air is handled separately and specially filtered for this room. I think the nurse said there were 4 rooms like it on the wing. It's pretty large and they've said we can rearrange it however suits us. They've even said we can decorate the walls short of painting them. There are several chairs and a small single-sleeper love seat where Becky or I will spend the night. Only one caregiver is allowed (and also required) to spend the night, although "spend the night" is apparently loosely defined as we can stay very late and come back very early.

So we're off and running. Tomorrow Elise will have the Hickman catheter placed and they'll also take a bone marrow sample. I suspect she'll have some pain and they've said she'll need to lie reclined for quite a while afterward. We may also hear something about the success of the donor's apheresis as it should be started and possibly finished tomorrow (in Germany a few hours ahead of us).

There isn't anything planned for Saturday so we're hoping Elise will get a "pass" and we'll be able to get out and do something (she wants to see Godzilla) before they start with the medications that require closer monitoring.