New Skillz

So now that Clara has had her anti-nausea + hyper drugs, she has decided to demonstrate a new skill that she has developed. She has previously had some pretty advanced developmental milestones and will now obviously be lacking at others now that she is in the hospital. For example, she doesn't really get much of a chance to roll over anymore. But her fabulous new milestone is passing an object from hand to hand!

Hi everyone, watch what I can do!

Left hand!

Right hand!

Sick Baby

We have a nutritionist who is very interested in Clara's food intake. A few of the doctors (not all of them) are also very interested. Obviously it would be a bad situation for Clara to start losing weight. When she had her four month well visit, she weighed 12 lbs 15 oz. There isn't much weight for her to lose, so any weight loss is something to worry about. During her chemo and subsequent recovery period, Cara had not lost any weight, but instead fluctuated right around 13 lbs.

Now for the doctors to know what her food intake is, they would need a way to measure it. For some reason this seemed like a problem that they had never encountered before. Brooke would write down the approximate time that Clara was eating and the nurses would weigh Clara's "outputs" but this was difficult to interpret. To do nutrition you need hard numbers, right? This started talk of giving Clara a NG tube so that they could keep track of how much she ate. We compromised with pre-feed and post-feed weighing (which never really happened because she was asleep after eating and who wants to wake a sleeping baby?).

When it was time for Clara to prep for this round of chemotherapy and load up her body on valproic acid, we wanted to make sure that she actually got the correct dosing. She was really starting to reject oral medications and was developing an oral aversion. So we gave her a NG tube to help with the dosing. This was good because at this point she dropped off in her feedings (none during the day really) so we needed to supplement her food intake.

The day that we were going to start chemo Clara started feeling pretty sick. She threw up three times (the first was before she had even started the chemo). I wonder if the valproic acid wasn't making her sick? She really didn't get enough to eat that day, especially with the vomiting.

Yesterday and today she has really dropped off in her feedings so we are feeding almost exclusively by NG. She will eat in the early morning hours and maybe a snack at night. The way the NG feeding works is that we basically inject milk down a tube that goes from her nose to her stomach. We started by giving her 50-60mL per feeding and have gradually upped that to a max of 122mL/feeding today. We were only able to give her 497mL yesterday and 788mL today (plus a little extra from some breast feedings) That still is keeping her below what the nutritionist would want at 828-1035mL/day.  She is still not losing weight though and seems to be trending up the past few days.

Unfortunately, tonight she was sick again and lost most of the last feeding as well as her valproic acid dose and her NG tube. That was quite an experience to have her NG tube come up, but so far we have managed and she actually jumped right to breast for a feeding (after getting cleaned up). We will have to place another NG tonight to give her a replacement dose of valproic acid as well as a dose of magnesium (which was low on today's labs). We gave her some Ativan to help with her nausea (this is the drug that also makes her really hyper). Hopefully the crying that I am sure will happen when she has to get the new NG will counteract the Ativan. Otherwise it will be quite a late night.

More goings on

Hello everyone. There hasn't really been much happening in the last few days so we haven't had anything to post about. There are a few tidbits of stuff that we have found out that I will share with you all.

First of all, Clara's first dose of valproic acid was administered incorrectly. Apparently the IV version does not have the desired effect and this was unknown to the fellow. This set Clara back a day where she had to receive oral valproic acid. Valproic acid does have some effect on the liver and Clara initially had a negative effect by having high levels of liver enzymes and increased ammonia in her blood. We were worried that this would result in having to cancel the valproic acid part of the regimen, but after her second and subsequent doses, her liver seems to be fine. This is great because hopefully the valproic acid is really helping the chemotherapy agents.

Clara did decide that she didn't like the valproic acid (the taste of it maybe) or any of her other meds for that matter and has taught herself to spit out medicine. For the longest time she did not like to take her oral meds, but we were able to give her small doses toward the back of her left cheek and she would eventually swallow it. Now she just spits it out. She will even wait a bit before spitting it out so that we think that she has actually swallowed it. Tricky girl... To remedy this (particularly so that she will get full doses of valproic acid) she now has a NG tube (feeding tube through her nose to her stomach). It is good that she got this because she has started getting bad at eating again (perhaps the chemo). The doctors would rather her keep having breast milk, so we just give her breast milk through the tube. She will still eat at the breast a few times a day (and is doing so as I write this), but to keep her caloric intake high enough we are complimenting with pumped milk a lot. This really makes the nutritionist and some of the residents happy because they were having a fuss about how much intake she was getting because "20 minutes at breast with ~10 of vigorous eating" was hard for them to interpret.

A lot of people are asking how to donate for the bone marrow transplant. If there is a way to donate specifically for her, it is going to be through a private lab where I am sure that it is going to be pricey. The likelihood of any one person matching her is very low. That is why it is important that there is a national registry of donors. This allows for the random person out there who matches her to be able to give bone marrow or blood to help her. If you want to do something, you can register as a donor with marrow.org and if you match her then they will select you. More likely you will not match her, but you may be able to help someone else. In fact, only about 1 in 200 registered donors are every actually a match. The donation process can be even less invasive than it used to be. Donors are now mostly giving blood as the medium for the donation instead of an actual bone marrow sample. (I think that at Stanford they still use actual bone marrow.) The registry has been greatly improved with the addition of cord blood (from the umbilical cord) donations. I would recommend banking your cord blood with a free cord blood banking service when you have a baby. The cells in this blood will probably prove to be even more useful in the future.

We will be updating soon with the process of the bone marrow transplant as we understand it so far (we still don't know much).

Bad News

Well folks, we didn't get what we were hoping for. The MRD results came back very quickly and we now know that she is at 12% MRD. As a reminder we were hoping for < 0.1% MRD. This means that her leukemia did not respond well to the chemotherapy. To be clear, the total amount of leukemic cells dropped dramatically, but they did not drop significantly quicker than her non-leukemic stem cells.

The high MRD in combination with her high risk karyotype causes a change in her treatment plan. The goal now is bone marrow transplant as quickly as possible. To do the bone marrow transplant they need to get rid of all of the existing bone marrow. In babies they do that with chemotherapy. They are actually going to be using the same chemotherapy treatment that Clara just had called induction II.

The plan for those interested is this:
Cytarabine: 100mg/m^2 IV over 30 minutes every 12 hours on days 1-8 (16 doses)
Daunorubicin: 1.67mg/kg IV over 6 hours on days 2, 4, 6 (3 doses)
Etoposide: 100mg/m^2 IV over 4 hours on days 1-5 (5 doses)

In addition to chemotherapy she will get valproic acid:
Day -2: 30 mg/kg once
Day -1: 22.5mg/kg/dose twice
Day 0: 10 mg/kg/dose three times
Days 1-6: 10 mg/kg/dose three times per day

Valproic acid is a new method that they are trying which has been shown to increase the effect of cytarabine by 30x. It words as a histone deacetylase inhibitor which means that it keeps the DNA in a less compact state (this is a very loose explanation). I imagine that the mechanism of action has something to do with keeping important genes on that the cancer would otherwise try to shut down to prevent action of the chemotherapy. The actual mechanism of action is unknown and this is still experimental, but has shown some good results so far. Valproic acid has been used to treat neurological diseases in children so the risks are pretty well known. The main risk is to the liver which will be monitored closely.

The goal is to start this treatment today with stem cell transplant in a week. This is highly dependent on being able to find a donor. Because of our background, the doctors are pretty confident that she will find a match. I will make a post about the stem cell transplant when we have more information.

What is happening today

As a little further explaination of Brooke's previous post I will talk about what exactly we are hoping for. We previously stated that the survival for children with AML was around 65% which is what the doctors told us. In fact, survival is measured in two different ways, and for this cancer just counts out to three years. First, there is overall survival which means that you are still alive at three years. The chance of overall survival at three years is actually 71.1% with half occurring by 292 days. The other measure of survival is called event-free survival. This means that no disease-related event occurs within the three year time frame. An event would be something like recurrence of the disease (this is bad). The chance of event-free survival (EFS) is 63.0% and half of these occurred within 191 days.

Now this isn't really the end of the story. Her survival very much depends on the specific mutations causing her AML. She is missing some of the dangerous ones, but also has some of the dangerous ones. She is also young which has a higher success rate that kids over 10 years old. However, it is generally hard to combine all of this information into a better prediction of her survival. This is where the test that she is having today comes into play.

The study that I am getting all of these numbers from showed that the most predictive measure of her survival is something called minimal residual disease (MRD)*. MRD represents the percent of leukemia cells remaining after the chemotherapy is over in a sample of mostly normal (hopefully) cells. In this study it was shown that bone marrow MRD at day 22 after induction I (her first round of chemo) can significantly separate between a high risk and low risk group (p < 0.0001). So today they are taking a sample of Clara's bone marrow and looking to see what percent are still leukemic and what percent are normal.

In an ideal world, the chemotherapy would have worked perfectly and there would be no leukemia cells left. In fact, it used to be that the tools used to measure MRD were so crude that they wouldn't be able to measure the number of cells left. In the study I am referring to, they were able to measure as small as 1 out of 1,000 cells (so a minimum measure of 0.1%). In the current study they are able to be more accurate and measure as small as 1 out of 10,000 cells. The improved accuracy will help stratify the treatments more in the future but may not really help us now.

Like I said, the previous study showed that MRD was highly predictive of survival. They showed that if the MRD was less that 0.1% (the minimum of their measurement range) then event-free survival was actually 73.6% and overall survival was 80.5%. If the MRD was between 0.1% and 1% the event-free survival was 65.9% and overall survival was 74.3%. If the MRD was greater than 1% then the event-free survival was only 32.1% with overall survival at 44.5%.

Obviously we want the smallest amount of residual leukemia cells in her bone marrow. The sample is taken today but is then sent to St. Jude for testing. We should have the results early next week. If the MRD is high they may skip chemo and move straight to bone marrow transplant. If it is low, we have more rounds of chemo ahead to make sure that every single leukemia cell is dead.

* Minimal residual disease-directed therapy for childhood acute myeloid leukaemia: results of the AML02 multicentre trial. Rubnitz JE, Inaba H, Dahl G, Ribeiro RC, Bowman WP, Taub J, Pounds S, Razzouk BI, Lacayo NJ, Cao X, Meshinchi S, Degar B, Airewele G, Raimondi SC, Onciu M, Coustan-Smith E, Downing JR, Leung W, Pui CH, Campana D. Lancet Oncol. 2010 Jun;11(6):543-52. Epub 2010 May 5.

Stay Tuned...but Pray Now.

We have been promised a conversation with the Fellow who will be assigned to Clara once she is an outpatient.  (It is one of the problems with their system that, because we have a long stay at the hospital, we haven't been "assigned" anyone as Clara's primary doctors.)

Tomorrow is a BIG day.  Clara will be getting a bone marrow biopsy and aspiration at noon PDT to determine how successful this first round of treatment was.  This is the BIGGEST indicator of whether or not Clara is likely to survive.  We have NO idea what happens after tomorrow - because no one has had an updated consult with us since we got the cytogenetics back.  We assume chemo will be started before we even get the biopsy and aspiration results back...but again...we don't know.  There is a possibility that we may go straight to stem cell/bone marrow transplant...but again...we don't know.

Since it is almost 6pm here and we still haven't seen the Fellow today, we may not have the update I was hoping for.  As a result, we may not have an update for you.  As such, we may not be able to give you the specifics of what outcome we are hoping for or specifics in our prayer requests.

One thing is clear.  Tomorrow is the most important day.  I believe God knows no time.  Tomorrow only tells us if the completed chemo did its job (for this phase).  So, tomorrow is the day that I ask you to pray as hard as you possibly can that we get good results.  He hears those prayers as we ask Him for what has already been.  I believe that He wants us to love one another.  And your prayers show Him that you love Clara.  How can He not answer with Clara's miraculous cure?

Today's Play Milestones

• She can sit up as long as I support her at her hips - unless her legs give a mighty kick which sent her straight onto her back a couple of times (she thinks it is funny) or unless she leans too far forward as she hasn't figured out how to use her legs to "tripod" and she was too distracted by toys to support herself with her arms.  
• She rolled over to chase her mirror on wheels as I pulled it away from her.
• She began scooting herself across the crib (with the help of my hand against her feet so she can get used to the sensation of using her legs to scoot across).  
• She is beginning to accidentally get her knees up underneath her when on her belly (which just sends her face into the bed) so there is some coincidental scooching happening. 
• She is coordinating the use of both hands to manipulate larger objects and to get the edge of the wheels on the mirror into her mouth.  (I'm so afraid she is going to cut her gums on it!)

A trying few days.

Since my last post, things have been a bit trying.  Clara continued to be awake more hours of the day, and I began requesting less and less pain medication - to see if she was actually getting better.

Unfortunately, Monday night consisted of Clara being at the breast CONSTANTLY.  Literally. Constantly.  I had to bring her into the bed with me - which was particularly concerning because she had had a few nights in a row of sleeping in her crib without ANY problem. At some point in the early morning hours, I finally asked the nurse to bring her some dilaudid. It was the first time Clara slept (for 30 minutes) without being at the breast.  But, Dilaudid supposedly has a sedative affect, and since her relief was so short lived, I assumed it only sedated her but didn't find the true source of the problem.

Tuesday day was incredibly difficult as she was just so tired yet not sleeping for more than 20 minutes at a time.  I found myself carrying Clara in her sling to go for a walk around the unit over and over again throughout the day.  She was cuddled up in the cradle position as I have found to be most comfortable for her lately.  Fortunately, the nurses cooing over her and the fish tank and the lights and sounds of the unit were a great distraction for her.  She even was able to fall asleep in it for a little while.

I knew something was wrong.  But, normally, I have some sort of feeling that some particular thing is wrong.  But for the first time, I didn't have any idea.  I had lots of worries but none of them seemed to really fit.  Her pain even seemed to be going away.  She has had mouth sores that were actually gone; so, I thought her throat pain was minimal at the worst. At around 5 pm, I finally asked for some dilaudid again - realizing that I was too tired during the night to have really observed how she responded to the two doses earlier that morning.

She was a new baby!  She was KICKING! JUMPING!  I've not seen her use her legs independently; yet, here she was jumping out of my arms - smiling and laughing all the while!  I immediately told the nurse that we were going on a dilaudid every 3 hours overnight.  I wanted her to be able to sleep pain free.  And she did.  At 2 am, the nurse woke me to let me know that Clara's blood pressure had decreased from hypertension of 110s/70s to 80s/50s.  I couldn't believe it.  The high blood pressure we had been medicating in an attempt to lower it was actually a symptom of her pain (Again! (we had seen this a few days previous)).

Clara continued to sleep well until she woke to eat at 6 o'clock this morning.  She was alert, smiling, babbling.  It was amazing.  She played for about an hour and a half and then she and I fell asleep in my bed for another couple of hours (me) and 4 hours (her).

She didn't eat again all morning - which turned out to be due to some nausea as she threw up around 12:30.  We restarted her Zofran to help with nausea and by, 2pm, I asked for some more dilaudid.  She had a fair-sized feeding and a great period of play before taking what will probably be a 3 hour nap.

A Good Day!

Clara had an AWESOME day.

After over 24 hours of sleep, she was awake and progressively more and more alert throughout the morning. And we only gave her pain meds once this afternoon - just cause I felt like she must surely need them despite her seemingly obvious comfort!

Last night, we assembled her ExerSaucer and, after a few minutes of being in it this morning, she was less overwhelmed and really had a blast checking out the spinning aquarium.  (I'll post pictures soon - didn't get any today as I was too busy gawking at her accomplishments and telling her how great she was doing!)  After about 15 minutes, I realized she was about to fall asleep in the ExerSaucer and quickly rescued her...instead she ended up falling asleep HEAD UP in my arms!

After one of her quick naps and a GREAT, full feeding, she spent some time on the couch/bed with Daddy.

She even put Daddy down for a nap while she watched some football.

We took another walk around the hospital late this afternoon while she was cradled in the Baby K'tan.  She was still awake and curious but it was later in the day, and she was showing more signs of fatigue. So, I didn't put her in the more typical "explore" position like she would have been in if I were wearing her forward facing in a Baby Bjorn or other similar carrier.

She went down for a great 3 hour nap this evening - and since that is what Alan and I were anticipating she might do - we actually escaped to PF Changs for Happy Hour appetizers and drinks for a couple of hours.  I had TWO margaritas!

I was hoping to give her a bath tonight before bed as I recently found that her neck fold has once again been collecting gunk and broken down skin cells.  She was too tired - so I gave her a quick bed bath in all the essential areas and hope to give her a full-on tub bath in the morning when Daddy gets here.

For now, she is in bed.  And, while I hope we will have another full night's rest, I think I'd better join her just in case sleep doesn't come so easily tonight.

Clara needs Clothes!

Okay, folks, I need your help! Our sweet Bug has been in the hospital for 3 weeks now and has been naked or wrapped in a blanket from home at best.  Mommy is ready to get this girl some clothes.

She may look like her Daddy but she gets that look of "Daddy, you crazy!" from me!

Lots of laughs and giggles last night during her only awake period for the day (~3 hours).

Here is the problem: I need help finding clothes that she can be put into feet first or that we can put her arms in and then either zip up the front or are wrap-style and fasten on the side.  Her PICC line is on her left upper arm, and in order for the nurses and doctors to have full access to her lines, it needs to come up and out of the neck hole.  Onesies and outfits that go over the head prevent that from happening.

SO!  Your assignment - should you choose to accept it:

Send me links to outfits that I can purchase online and that you think would accomplish this goal! She is 13 lbs and wears size 3m outfits.

We need to put this girl in some clothes that compliment her naturally cute self!

Cytogenetics Report

Today we got the cytogenetic information from Clara's leukemia cells. They were only able to grow 8 cells (this seems strange to me). On these 8 cells they performed GTW banding and FISH to identify abnormalities. From what I can tell they only used a few FISH probes instead of anti-chromosomal FISH that I've seen in seminars. I don't really follow much cytogenetic work so maybe they can accomplish the same thing with banding (So please make corrections all you genome biologists reading this). I have included a couple of pictures of the types of analysis below. Banding looks at the dark and light bands on the chromosomes based on chromatin density and FISH uses colored probes to identify specific stretches of DNA. In the example below each chromosome has its own color but in the analysis that was done on Clara's cells only specific (small) regions have probes. They were only looking for specific known rearrangements.

 

To help facilitate understanding of the changes in Clara, I will briefly go over chromosome structure (use figure below as a reference). Chromosomes have a center called the centromere which is where the two copies of the same chromosome come together prior to cell division. On each side of the centromere are the two arms of the chromosome. The shorter of the arms is called the p arm and the longer arm is called the q arm. These arms are segmented based on density of the DNA/protein (as observed through light/dark bands). The bands are labeled with the number increasing as one moves away from the centromere.

So her leukemia karyotype:

45, XX, -5, -7, add(10)(p), -11, add(11)(q23), +2mar[cp8]

What should it be? 46, XX

So what does all that mean? Her leukemia cells have 45 chromosomes (instead of 46). She is a normal XX (female). She is missing copies of 5, 7, and 11. **She has additional DNA on the p arm (the short arm) of chromosome 10 (I guess they don't know where). She also has additional DNA on part of the q arm of chromosome 11.** There are also two chromosomes that don't resemble known chromosomes (probably a mix of stuff) called marker chromosomes.

The FISH analysis indicated that there was a break at 11q23. This is a common break in leukemia in the middle of the MLL gene. They see that part of this gene (3' end) is with the additional DNA on chromosome 10 (add(10p)). This may indicate a translocation between chromosome 10 and 11 (t(10;11)) but I guess that they aren't sure or they would have labeled it so. t(10;11) translocations tend to happen in AML, but the exact location seems to matter. We know it is 11q23 somewhere in the MLL gene, but we don't know where on chromosome 10.** FISH also showed that EGR1 (a gene on chromosome 5 at a common break point) is normal and in two copies. This indicated that there isn't really a deletion of chromosome 5 but that it is probably part of the add() and/or marker chromosomes.

Missing chromosome 5 and 7 are usually linked with poorer prognosis potentially making her high risk. We have yet to hear from the doctors regarding her risk level and the last doctor seemed to indicate that she was standard risk. We will be talking to the AML expert to get a better explanation of these results in due time. If she is high risk then she is most likely going to need a bone marrow transplant.

EDIT: I made some changes between the **'s. I misunderstood what add() indicated and have corrected this.

5 Months Old

Clara turned 5 months old today.  I put up a sign on her door so that everyone would know and bought her a balloon at the hospital gift shop.

She had a hard day.  She had her PICC line cleaned and redressed which wasn't fun as they pin her down.  And, she had an ultrasound because of concern about her kidneys.  Her pain seems to be worse, and she is showing more sources of discomfort.  Her nose is running, eyes are watering, stomach hurts before she poops, and she is now being given ~7 oral meds over the course of the day.  I was literally traumatized after her night nurse gave her two of the meds.  She made Clara spontaneously throw up at the first one and scream and struggle more than I have ever seen with the second.  I took over after that.  She looked like she was shoving it down her throat not into the side of her cheek. We have started giving her her pain meds every 5 hours instead of 6 due to her overall discomfort today, and, while she has had more awake periods today, they haven't been very happy.  Alan did take one snapshot, however, that made me cry.

My Aunt Shelly Schutze sent me this necklace which I got today.  I have no words for how small, delicate, simple, and powerfully beautiful this piece is.  It is the Miraculous Medal on top of a heart with "peace" engraved on the top and "Clara" on the back.  The thought of the blessed mother looking over me and the peace that we want for Clara

Our New Digs

Lucile Packard Children's Hospital

Alan and Brooke Boyle

Attn: 1 North Heme/Onc

725 Welch Road

Palo Alto, CA 94304

Front Desk in 1 North (The Hemetology/Oncology Unit). The portholes on the right looks into a salt water aquarium.  Really fun for Clara to look at when we go on our walks around the hospital.

Hallway to Clara's Room

Approaching Room 1403!

Signs rotate as I choose and as the doctors need to alert all staff when Clara's health changes and may need additional sanitation requirements, etc.

View as you first walk into the room.

Sitting area.  Couch is a futon bed thingy.

Clara's Rocking Chair!  She didn't even have one at home!  She loves to be rocked in it!

White Board on Bathroom Door.  Has areas for the names of the Doctor, Nurse, Nurse's Aid on Service for the day/night.  And a place for me or the nurse to share information - like Clara's breastfeeding times and lengths.  It is also to help us remember what we need or questions we might have of the doctors or nurse.  The best part is that the staff actually, actively use this with us.  So it is as great a tool as it is designed to be!

Desk, Cabinets, TV area as viewed from sitting area.

Our view of Clara from the Sitting Area.  So glad she is sleeping soundly and peacefully!

The sleep dilemma.

After coming off sedation after her visit in the PICU, it seemed that Clara was never going to sleep.  We were told her body had been sending signals to "awake awake awake" during sedation, and it was going to continue for a while even though nothing is suppressing her anymore.  So, the first three days and nights back in oncology left us with a very overtired, awake baby.

Additionally, she has been really jumpy, easily startled, and will scream if you touch her face when she isn't expecting it.  They had been putting prophylactic steroids in her eyes FOUR times a day.  One of our nurses told us that she had used the same eyedrops when she was ill once, and it feels like you are pouring sand in your eyes!  Once I understood that it was worse that the "sting" that we were originally told she would experience, I had a much harder time giving her the eyedrops.  And, I completely understand why she has gotten so easily upset.

So, if you combine those things together, you have a baby that can't sleep and screams when she realizes she isn't in my arms.  After 3 days and nights and a cumulative 5ish hours of sleep for me, I started thinking about co-sleeping with her.  I simply couldn't function well enough to be able to care for her. 

After a nurse that I really love and trust suggested it yesterday morning, I decided to try co-sleeping with Clara.  I NEVER would have considered this before.  In fact, had anyone mentioned it to me previously, I would have quoted all the bad things that can happen to baby when you co-sleep.  But, Clara wasn't sleeping unless she was being held.  Or, if I had been able to successfully put her in bed it would only last about 20-30 minutes.

After a discussion with the head nurse last night and signing a hospital liability waiver regarding Clara's safety due to our decision, we slept.  In fact, I slept from 11pm until 7:30am with only three 15-30 minute interruptions to feed Clara and for the nurse to take her vitals.  It was AMAZING!  I was a new woman today although still a bit fatigued.

There is only one problem.  After Clara got a taste of good sleep, she couldn't stop.  Until an hour ago.  She slept from 11pm to 9pm.  Yup.  22 hours straight and hasn't really eaten since her feeding in the late evening yesterday.  I have a feeling we may be up watching movies, drinking coffee and playing with a - hopefully happy - very awake baby.

Favorite Toy and new Best Friend!

Physical and Occupational Therapy left Clara the perfect toys for her developmental stages right now.  

Her favorite?

 

her mirror on wheels

that baby named Clara!

 

AND

New skills!

Playtime today proved that Clara is really enjoying tummy time now!

Holds her head really high!

Supports with arms...

and plays with toy at the same time!

Clara's First Walk

I had been assuming Clara was not allowed out of her room - which I now know is called "isolation" - since her neutrphils have been just above zero.  Since she has actually been feeling relatively well and chemo is over, I posed the question to "the team" (her doctors) of when she would be able to get out and about!  The answer?  Now! 

So we went for our first walk yesterday and took another today.  Her eyes were quite red because she was overtired.  But it was great to take a peak at the atrium at the center of the Children's hospital and right outside our wing.

They provide wagons so we can take longer walks.  I am looking forward to her being able to sit up or stay propped up a little bit more steadily so we can wheel her around.  Plus, I think she'd like the speed!  :-)  This girl loves acceleration!

Until then, Clara LOVES to be walked around, and I really enjoy being able to hold her and walk without being tied to the bedside.  She has been taken off "continuous monitoring" orders but is still often hooked up depending on which medications she is getting throughout the day and whether or not she needs to be monitored during that infusion.

Again...Like Father like Daughter!

We decided to co-sleep with Clara this morning after only getting 3 hours of sleep last night.  Once the sun came up, I got up to have some coffee and make a few phone calls in the lobby.  When I returned, I was greeted by this beautiful sight!

The Nurse that drove me NUTS!

We've only had one instance thus far where we (not just me - Alan too! which totally validates my insanity over this) had a problem with one of the nurses. 

I have to first say it isn't her fault.  She is nice, offers to do anything to help us, likes working with infants, happy, etc.  BUT, she was a night nurse who talked too much, dropped things, forgot things and had to go in and out to get whatever it was, made WAY more movements to get things done than ANY of our other nurses (and when she is wearing a paper chemo gown it makes extra noise). She always seems behind and a bit frantic, and she uses way too many words.  For those of you who know me, I LOVE to use lots of words, and I love conversation.  Who knows, maybe if she had been a day shift nurse, I might have liked her.  But she KEPT CLARA AWAKE. 

When you have a sick baby that is having a hard time getting to sleep, that is doubly awful!  After having her two nights in a row, I talked to the head nurse and requested we not get her again.  I was assured we wouldn't.  YET, the very next night...who did we have?  You guessed it!  I was shocked!  And the problems with her were even worse that night.  I ended up loosing my cool and in my attempt to constructively let her know she needed to slow down and quick going in and out so much, I insulted her and put her in a position of feeling like she needed to justify her every move.  It was a horrible position for her to be in (which is why I knew she needed to never be our nurse again!) but I was seriously pissed and couldn't just smile through it and say thank you etc.  It was again the wee hours of the morning and HER fault that we were all in the place that we were.  And her talking about being willing to do whatever I need so that I can get good sleep was driving me nuts!


Ugg.  Anyway, the good thing is that we really shouldn't have her again.  And we at least knew we weren't going to have her last night because she had already worked her 3 nights for the week.  They tell us not to feel bad - that there are always personality clashes and this was just ours.  For her sake, I hope they don't forget and assign her to us.  She was doing the best she was capable of and it was just the wrong thing for us.


Okay.  Done venting.

I LOVE Packard Children's Hospital at Stanford!

For those of you who know me well, I am a huge stickler for customer service, efficiency, organization, etc.  With that in mind, let me say that I am overwhelmed by the AWESOMENESS of our nurses and the teams of people at Stanford hospital.  We have loved every single nurse we have had (except one) and have been floored by how much attention we have gotten from the nurses up through to the attendings.

We've had a resident sit down with Alan to explain Clara's labs so that, when he gets daily print outs, he can interpret them.  We've had one Oncology attending print the full protocol for Clara's treatment plus recently published studies on AML (somewhere around 500 pages) so that Alan could read as much as he wants.  We've had the most senior PICU attending (who was responsible for Clara both times she was in the PICU) personally come to visit yesterday just to follow up and see if she looked as good as the stats in the computer were saying.  We've had nurses who soothed Clara to sleep by bundling her in a pillow and blankets with Clara's swaddler against her nose so it would smell like home and not the hospital.  When Clara was at her worst with tubes, IVs and catheters all over her, that same nurse made a bow with purple tape to put against her head IV.  (I was honestly sad to see the head IV go away because the make-shift bow went with it.)

Roller Coaster

It's hard to liken this journey to much of anything, but as someone said earlier this week and as Mimi continues to talk about - this is a roller coaster!

I'm feeling pretty good today.  The "if Momma ain't happy, ain't nobody happy" saying used to hold true for our household.  Now, it has everything to do with how Clara is doing.  Are her meds keeping her comfortable? Does she have a procedure? Is she retaining fluid? Is her nurse keeping her awake?!?!

Yesterday was a GREAT day.  We finally figured out the best combination of meds and, after sleeping from 3am to 2 pm, she awoke happy, smiling, and eager to interact!

I immediately called the Occupational and Physical Therapy gals who hadn't yet been able to "play" with Clara to see where she is developmentally.  One of my concerns is about her ability to coordinate her latch and suck.  She seems to be making progress, but they are helping make sure that continues.  My other concern has to do with her being previously "advanced" in several development categories, and I want to help support her continued growth despite being in a hospital bed for so long.

It was INCREDIBLE to watch them with her.  I love how they can calmly coax her into grabbing a toy, rolling over, enjoy sitting up and moving about.  She has regressed in some ways and actually progressed in her tummy time strength - which shocked me!

Today, she had two great awake periods and other than some serious discomfort when she yawns which makes her gag and cough, she was a really happy baby.

She came off of her lipids and TPN (which are how she has been getting her nutrition since she hadn't been able to eat).  This should show a marked improvement in the number and duration of her feedings.  We are expecting a good night tonight as she seems to be quickly readjusting to nights being nights and days being days.  But, we will see.  I wouldn't be surprised if she wakes up in an hour or so and wants to be awake for a while.  Two nights ago it was until 3am and last night it was until 1.  Based on her behavior when she went to bed at 9 tonight, I am hoping she stays asleep...until she wants to eat for the first REAL feeding in 2 weeks.  I honestly can't wait for that!  I hate this pumping thing and totally love having her back at my breast.  I completely didn't understand how wonderful it was until I lost it.

So, there is my big post.  I am bad about starting a post and then never finishing it.  So I will try hard to update with smaller, easy to post and read posts each day.  Thank goodness Alan is giving you some updates as well so that we can both remember what has happened over time and keep you all in the loop.

CSF Free of Blasts

That would be Clara's Cerebrospinal fluid is free of the leukemia cells that are circulating through her system. Initially they discovered 2 leukocytes per mul in Clara's spinal fluid. They first discovered this when we first came in for possible meningitis. Here is the treatment plan for this situation:

Patients with < 5 leukocytes per mul of CSF and the presence of leukemic blast cells on CSF cytospin (CNS2)will receive weekly intrathecal therapy until the CSF is free of blast cells. These patients will then receive 4 additional doses of intrathecal therapy at approximately 1-month intervals (generally given with each subsequent course of chemotherapy).

They have currently treated for this twice with methotrexate, hydrocortisone, and cytarabine injected into her spinal fluid. Each time they test to see if there are still blast cells. The test from the second injection showed no more blasts in her CSF, so that means that she is back to the original protocol (1-month interval spinal injections). I hope that it is a good sign that she responded so quickly!

Demon Child (that will eat your soul)

Ok, funny story.

So this is a story that relies on a bit of an inside joke (pretty much between Alan and Cooper), but I will try to give a little background (Cooper, feel free to help correct this). While playing Halo shortly before Clara was born there was a discussion with a fellow player about the significance of having children. He stated that he was not particularly impressed to which Cooper replied "but what if she is the demon child that will eat your soul?" Now this seems out of context here, but trust me, it made perfect sense at the time (I'm sure that Cooper was referring to her eating your soul because you would fall in love with her). The guy we were talking with loved this and continued to repeat, with a raspy, surfer-like voice, "the demon child that will eat your soul". Fun was had by all. The thing that made this a classic was that the guy pops up in the next game that Cooper and I are in and starts saying it again. Keep in mind that there were something like 200,000 playing Halo at the time and that we are randomly assigned to other people in the game, so this was highly improbable. Anyway, Cooper and I thought that this was hilarious, and it has become somewhat of a meme while we play.

So fast forward to two nights ago (1:30 am). Brooke is attempting sleep on the couch in Clara's hospital room, and I am standing by Clara's bed trying to get her to sleep. This is after I had already gotten her to sleep at 10:30 and she was awoken by the chemo nurse at 11. The doctors had given her Ativan to help her deal with nausea (and I think also as a sedative) . Right after this she went from being a somewhat sleepy and grumpy baby to a crazy (read high) baby! She was looking around at all the lights and swinging her head around. Her bp was way up so the nurse thought that she was in pain and gave her morphine. That should have had a sedative effect, but instead she went even crazier (like she was a baby on speed). She was thrashing her arms around and started grunting like you wouldn't believe. Finally, we call the doctor in to see what was going on. She was a little surprised as well, because she thought that Clara would have been asleep from the drugs. About this time, while Clara is grunting, I say "I wonder what is going on," and Brooke rolls over from her sleep and mumbles, "She is the demon child that will eat your soul". I chuckle to myself but wasn't even sure that the resident heard her.

So finally we get Clara to sleep around 3am after a dose of benadryl to take away the itchiness, and she sleeps soundly. The resident thought that she was having a reaction to the morphine that was making her hyper and her face itch so recommended in the future to move to a different drug. I think that she was having a reaction to the Ativan making her hyper, but I agree that the morphine was probably making her itch. So yesterday they moved her to a new drug, Dilaudid, as a pain killer which really seems to work well. We are also trying to avoid the Ativan as much as we can. This drug works MUCH better and Clara seems to actually be in a good mood and was able to play yesterday.

So last night the resident comes by to check on Clara. Brooke was telling her how excited she was for the new drug combo and how well Clara seems to be reacting to it. We did indeed have a great day yesterday. The resident then says "Well, I'm relieved, I know things are bad when a mother calls her baby a demon child!!!" Brooke and I broke out into laughter realizing that the resident had not only heard Brooke the night before but that it had clearly struck her and made her think Brooke thought things were REALLY bad!  So we tell her that it is a long story and an inside joke and that Brooke didn't really think that Clara was a demon child.

Clara Update

Hello everyone. We have been really busy but now I have time to catch people up with what has been happening since the previous post.

Starting on Monday (8/30). Clara was scheduled to have a central line put in which would allow for her chemo treatment to start. Apparently the chemicals are harsh enough that they don't want to administer them via traditional IV in the arm or foot. The plan was to either have a PICC (peripherally inserted central line) or a Hickman line which is a more direct and permanent solution. Because she was neutropenic the doctors were worried about infection so they decided on the PICC. Unfortunately she was so swollen that after multiple attempts (2 left arm, 1 right arm, 1 right leg) it was decided to insert a catheter subclavian (under her collar bone). During this procedure she was asleep so they also gave her a lumbar puncture with her first dose of chemotherapy because of some leukemic cells that were in her earlier spinal fluid sample (from then they thought it was meningitis). The standard here is a monthly injection of chemo into her spinal fluid and because they saw some leukemic cells they are adding a weekly dose for the first month.

On Tuesday (as I also mentioned on Monday) Clara was pretty swollen from retaining fluids. She seemed to be in better spirits though and we actually got a smile at her favorite toy, "Happy Man". Today the main regimen of chemo was to start. She is on the study AML08 which is a collaboration between Stanford and St. Jude Children's Hospital. The initial difference from the standard of care was a randomization for a new drug. Unfortunately, her liver function was below the cutoff required by this new drug so she was simply given the standard drug set. This started with a high dose of Cytarabine on Tuesday night. This started day 1 of her chemotherapy.

Tuesday night / Wednesday morning we received a series of scary phone calls about Clara. She was now getting some fluid on her lungs as well as having deteriorated kidney function. The doctors thought that the signs pointed to a dosage effect response to the cytarabine. The fluid buildup was exacerbating any kidney problems because of the added pressure on the kidneys. She was brought back up to the PICU and was put on a kidney dialysis machine and a breathing tube. They also took this opportunity to put in a PICC in her groin and an IV on her wrist and her head. When we arrived in the morning all of our stuff had been removed from our room in Oncology (where we had been slowly moving in with the intention of staying for months. We were both very nervous about seeing her hooked up to all of these machines but finally got to see her. She was incredibly swollen and at this point had gained over 3lbs in fluids (She was admitted at 13lbs and was now over 16lbs).

Thursday she was in a lot better situation in terms of fluid retention. The dialysis machine had been removing fluid slowly overnight and Clara was significantly less swollen. She was completely drugged though and was asleep the whole day.

On Friday when we came in, she was already off of the dialysis machine. She was looking very thin at this point, especially in the face. Throughout the day Friday she was asleep on the drugs they had given her to relax. Her chemo was also restarted today (Day 1 starts today at 5pm). They decided to move her from the original Induction I plan to the plan for Induction II (Which uses lower doses of Cytarabine - low dose is actually the standard dose used in most protocols). Induction is the phase of therapy where they try to kick the disease into remission. This plan now calls for Cytarabine for 8 days, Etoposide for days 1-5, and Daunorubicin on days 2, 4, and 6.

On Saturday they began to dial back the breathing machine. She had long period of open eyes! She was still drugged but they were starting to wean her off of the medications. That night she had a ventilator sensor begin malfunctioning. These ventilators provide a puff of air when the patient is breathing in so that it doesn't feel like they are breathing through a straw. Unfortunately, the machine was also giving her a puff of air when she tried to breath out. Clara was not happy with this (who would like to have air blown into their lungs when they are trying to breathe out?). Before they figured this out though, Clara was probably overly medicated to sedate/calm her.  The plan was still to take her off of the ventilator late tomorrow morning. She slept wall that night from the sedation.

On Sunday Clara was still in PICU but finally came off of the ventilator. They also removed her hand IV. She was moved to a different room in the PICU which was more away from the hustle and bustle. She was doing very well and the doctor decided to let Brooke hold her for the first time in quite a while. Clara was having some withdrawal from the drugs and so was awake for over 9 hours, but finally she was able to settle down and with her Benadryl that night (to help with nausea) she slept the whole night.

On Monday she was finally moved back to the Oncology ward. Hopefully she will stay put here for the next month+. She was back to her admission weight today and looked a lot better. We got a good laugh out of her today and she actually was able to nurse from Brooke for the first time in 10 days. She seemed a little confused at first but managed ok in the end. Monday night was a rougher night for her. She was still a little wired from the drug withdrawal and so was not able to get to sleep until 3am. At some point during the night she decided that she no longer needed her feeding tube so she helped the doctors by removing it.

She didn't have any trouble staying asleep though. On Tusday she slept until she had to go into surgery again. She had to have the subclavian line removed due to a bad blood culture (possible infection but probably not). They also wanted to remove her groin line because it is hard to keep clean. They took her down and were able to easily insert a PICC in her left arm as well as give her the weekly lumbar puncture for her chemo. After the surgery she was hungry! (No food allowed since 3am) The nurses were giving her pedialyte (60mL) which she had scarfed down. Brooke ran in and was upset because she wanted to nurse her. Fortunately Clara wasn't done yet and continued to nurse on Brooke and was generally a happy baby. She actually looks like herself (except for some tubes and lots of poke holes all over her body) and was able to be carried and fed well throughout the day. Brooke stayed the night with her now that she isn't under sedatives and had a tough night again with Clara not finally settling down until late. Brooke reports that Clara kept waking up and laughing at the vital signs monitor.

In an instant...

...everything changed.
***This update has been adapted from my best friend and Clara's Godmother's blog.  She posted this a few days ago after getting an update from me and in an effort to get this blog going with updates on Clara's condition, I thank her for writing this so well and amazingly accurately considering all the chaos, details, and confusing course of events.***

Brooke is a new mommy to my precious Goddaughter, Clara Violet Boyle. Clara is 4 1/2 months old, and one of the most loved little babies that ever was.

On Monday, August 23rd, Clara was a healthy little baby, and actually went for her "well check" in California where they moved in May.

On Tuesday, Clara was sleeping heavily and only waking to eat which was getting more difficult due to her congestion that had started a couple of nights before without concern.  When she spiked a fever, they brought her to the doctor's for a sick visit where Clara threw up in the waiting room and was clearly not a well baby. The doctor wasn't sure what was wrong, but thought a virus or cold might be the culprit. Brooke was advised that the fever might return, but not to worry.

Wednesday brought a new fever slightly higher than expected and as such they brought Clara back into the Doctor's office and was still clearly sick but not alarmingly so.

On Thursday, Clara was still ill and the pediatrician suspected a UTI and so had Clara brought in for a urine analysis.  That night, Brooke noticed that Clara's soft spot was swollen, and called the on-call doctor immediately. The doctor on call said that since Clara was not clinically lethargic, she was not overly concerned about the soft spot or the continued cold symptoms. Urine Analysis preliminarily looked negative.

On Friday, Clara was back to sleeping a lot (seemingly regressing to the state that she had been in on Tuesday - which concerned Brooke that Clara wasn't getting better). She wasn't eating very much either as the congestion was making sucking increasingly difficult. Brooke called the doctor's office, not wanting to go into the weekend with a sick baby and still not knowing the cause. The doctor requested that Brooke bring Clara in, just to look over her once more. Once the doc saw Clara (confirming a bulging fontanelle), she was immediately concerned, and had Brooke and Alan take Clara to the ER with concern for meningitis. A spinal tap, brain CT, chest x-ray, and full blood work-up later, meningitis was negative, brain and chest looked fine, but the blood smear showed "immature cells". 

The hematology doctors explained that this meant they were 75% sure that Clara has leukemia. Clara was admitted to the PICU (Pediatric Intensive Care Unit).  On Saturday, she received what was the first of what will be many bone marrow aspiration (fluid) and biopsy (bone).

On Sunday, they received confirmation that Clara does in fact have acute myeloid leukemia and a 65% chance of cure.

In an instant, Brooke and Alan went from being normal first-time parents of an infant to being parents of a very sick child. Clara went from being a happy, healthy baby to a baby who will be in the hospital for at least 6 weeks while receiving her two initial chemotherapy treatments. And her course full course of treatment will take at least 6 months.

**Clara is being treated at Stanford's Lucile Packard Children's Hospital under the current St. Jude's AML Study Protocol which utilizes the current standard of care for this disease.***

Hi, Auntie Kelli!

This was the home video taken in front of the computer a few days before Clara got sick.