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.