Neutropenia blues!

Here is Sinatra at ER with her fever. She would get extremely
mad if we remove her hat!

It has been a while since we updated everyone, and in our case no news usually means good news. We started a new round of treatment, 'Delayed Intensification Part II' two weeks ago. It began with a week of very intense chemo, one of the "study" arms of treatment we are on, which consisted of five straight days of 6-8 hour long infusions at the Outpatient Center. She made it through the week like champ and seemed like she was feeling pretty good considering how intense the treatment was, and then 3 days later it all went down hill.

We had a rude awakening on 8/20 Wednesday night, when she started doing her whimpering cry which usually means she is about to spike a fever. This will happen when she has had chemo that will take her counts down to zero (AKA Neutropenic, is what they call it medically). She did this off an on the entire night but never actually got a fever. We were shocked, but relieved that she made it through. We had an appointment at the clinic for labs the next day in the morning at 9am and we told our nurse about what happened. The labs confirmed that Sinatra needed blood and were supposed to come back at 3pm the same day for a blood transfusion (number 10 or so since we have been in treatment).  At noon we got a call from Grandma Gloria that she was running a fever of 102 degrees and it was going up fast. After a chat with our Oncologist we were headed the the ER once again.

Here is Sinatra at the hospital.  The only thing she wants to
eat is Fruit Roll Ups. Literally 20 of them in two days!

Now here we are 4 days into being admitted in the hospital and Sinatra is still Neutropenic. The good news is the chemo clearly worked and knocked everything out, meaning her blood making factory was completely shut down. The problem now is that it's taking a while for it to start producing again. Until we start to see her counts come back up or "recover", we aren't going anywhere. So now we wait each day for labs that are taken in the early morning to tell us if we have made any progress. What's hard about this is that we have no idea when things will turn around. Could be another day, 5 more or 10 more.

The hospital stay this time has been a little trickier for us because Link started his new job at Amazon last week so things aren't quite as flexible yet since he's brand new. We also just moved into the new house just barely a week ago and things are still an absolute mess. We have decided to focus on spending time with the girls and not worry to much about all of the other chaos.

On the bright side, hopefully after this recovery, Sinatra's counts shouldn't get this low again. We are about 60 days from starting the final phase of treatment called Maintenance. We are literally counting the days, well sort of, because its certainly possible that we might encounter another delay. Thanks for taking the time to read this. We will let you all know when we come home.

Peaches and Eggs!

Sinatra is not herself, she only wants to
cuddle and lay down.

We are currently in the throws of Sinatra's Delayed Intensification round of treatment and it has not been easy. This has been one of the hardest phases so far for all of us.  Sinatra just seems like she is not herself. She doesn't have all the energy she usually does and seems to be much more 'ill'. Its also become more difficult to give her the many medicines at home she takes each day because she often says "no thank you" when you approach her to give it. It's sweet that she's polite about it and it breaks our hearts when she nearly vomits trying to get it down or cries. We have experienced much more vomiting, sleepiness, anger and weight loss in this phase. Sinatra has lost almost all of her weight again. She weighed 10.3kg recently which is less than 1kg more than when she was diagnosed in December 2013. If you recall at that point in time they were concerned with her weight being so low.  It is sad to see Sinatra this skinny, her spine sticks out very far and even the port in her chest looks like a stack of 10 quarters under her skin. 

This will satisfy Sinatra for maybe 1 day!

We have also started steroids again. Now compared to the first time she was on steroids this has been a breeze but we can see hints of Chulk coming back.  The good thing is she will be done with the steroids  on 7/22/2014.  One thing that definitely makes it easier is that she can talk now, so when that insatiable appetite starts taking control of Sinatra she can tell us what she wants.  We assumed it would be more buttered toast (similar to the thousand of slices she had during her first run on steroids), but not even close.  Chulk's new steroid food is scrambled eggs and Dole diced peaches in the plastic cup.  We literally have seen her eat 8 eggs and 8 cups of Dole peaches in one day.  She is so addicted to these right now that we can offer her ice cream, whip cream, pudding, cereal, buttered toast, money, toys, candy and cake frosting but she will always reply with 'Peaches' or 'Eggs'. There are even some nights where she demands that the peaches and/or the eggs are placed in Tupperware or on a plate or in a cup. Of course we comply with her requests to avoid the wrath.

We really hope that we get through this phase soon because the mental strain on all of us worrying about her have really weighed on us this month.  We had a night where Siena started vomiting and her response was that she was sick like Sinatra. So sad that she recognizes the hard time that Sinatra is having.  We also hear the girls say, 'When Sinatra is better maybe we can go to Legoland, or the beach, or Las Vegas (don't ask), and Disney etc.?'

Speaking of Siena. We noticed a few small bumps on her cheek when she got home from preschool one day. We started thinking about what it was and of course thought, hand foot and mouth, or chicken pox. We definitely were nervous because chicken pox are on the list for us to head to the hospital with Sinatra, even if it is only being exposed to them. We quarantined Sinatra in her room right away and made an appointment for Siena to see the doctor the next morning. Luckily it was only a minor infection and we got it under control.

This has been a tough 28 days for us and we are ready to move on and start our next phase  around Aug 4th. We will keep you posted on how Sinatra feels in the upcoming weeks.

Delayed Intensification begins!

It was very weird to go back to the OPI to begin treatment again. We were discharged from the hospital on June 16th and gave Sinatra her last Oral chemo on June 22nd. During this break Sinatra lost all of her hair and it literally started growing back within days of its disappearance.  Now she has a very soft peach fuzzy head!! It was nice to have such a long break from reality but as of July 2nd we are back in treatment full speed ahead.  We began our delayed intensification treatment on the 2nd with a lumbar puncture and two types of chemo.  The one chemo (Doxorubicin) was pink in color and weirdly enough Sinatra's urine made her diaper pink for the next day. We must warn anyone that comes near Sinatra...... she has started her steroids again. We began steroids the night of the 2nd.  Sinatra's steroid dose is almost double what it was in January but she wont be taking it for a full month.  She will essentially take it for 7 days then break for 7 days and then back on for 7 days.  So hopefully the side effects wont be as prominent this time.  We are hoping that Chulk does not enter the building again.

One amazing piece of information is that on July 4th Sinatra got to go with the family to a 4th of July party.  It was amazing to have her at a social event and we know she loved it. She was so happy walking around and saying hello to everyone.  We even brought her to see the fireworks at the beach.  We know these wont be regular occurrences but we are happy she got to enjoy this day.  We are looking forward to Sinatra entering her maintenance phase which should be around mid October. Once we get there many of these restrictions should be lifted and Sinatra can be much more social.  

In patient treatment, check!

This weekend we finished our fourth and final inpatient treatment for the 'Interim Maintenance' phase that we are in.  This is a 63 day phase that had us in the hospital every other week for about 5 days of treatment.  We can't say enough about how well Sinatra is doing on her chemo. She is so active when we are at the hospital and wants to be out in the hallways all day walking around. All the nurses and Doctors get so excited when she is out walking and she loves 'hamming' it up for them

Sinatra loves walking around the outdoor terrace at the hospital.

.  Sinatra did get sick a few times during her treatment but we continue to give her the anti nausea medicine and it helps out tremendously.  We have about two weeks left in this phase which Sinatra will get an Echo-cardiogram and also oral chemotherapy for half of this time.  We will actually have 10 full days of NOTHING!! Wow, what a crazy thought of not having anything for Sinatra to do.

After this phase we will start our 'Delayed Intensification part 1' phase which is 28 days and followed by another 28 day phase called 'Delayed Intensification part 2'.  We have had all of the chemo that will be given in these phases so are semi familiar with how Sinatra should respond.  The good news is that after these two phases there is only one more 56 day phase before we enter into our 'Maintenance' phase.  We wont get too far ahead of our selves so we will describe Maintenance in a future blog post.

During this phase Sinatra has lost essentially all of her hair. We went through a week where we would be pulling large handfuls of hair  out of her head.  She now has very little left on her head and we expect either the rest to fall out very soon or we may shave it.

Sinatra's Doctor  (Dr. Huynh) came back from maternity leave which was great. We missed Dr. Huynh and are happy to have her back.  All of the Dr's are incredible at CHOC but once you are assigned a Dr and go through some of the initial weeks/months of this treatment you build a bond that is hard to be replaced.  

"Choc Nannies!!!"

So we know it has been a really long time since we have updated the blog and many of you have asked how Sinatra is doing and have some sense of how crazy things have been for us the last few weeks. Here's a little more on whats happening now and what is coming.

She had just eaten chocolate covered frozen bananas, aka "Choc nannies"!

We have completed 3 of 4 rounds of inpatient chemo, which is 4-5 days long in the hospital, for this phase of Sinatra's treatment called Interim Maintenance. This phase ends in early June. We will check back into the hospital this Wednesday, 6/4 if her labs are good at the beginning of the week and will finish the fourth round. This phase of treatment has been tough for a lot of reasons. Having to stay in the hospital every other week is chaotic for Sinatra and for us and it has been pretty disruptive to the sort of routine we have had in treatment so far. It's hard for her to be away from her sister and without either dad or mom each night, and its hard for us because we don't get to talk in person for more than a few minutes a day. We are truly trying to divide and conquer to keep things running as smoothly as we can for both of the girls.  The chemo this time has also been intense for Sinatra and we have had many sleepness nights both in the hospital and at home as she has the tendency to get sick and vomit at night. The endless medications at home and the hospital are supposed to help her and I am sure they do but there are times when its just so hard to give her one more.

She talks now, which is the cutest thing ever. She responds to questions and says "sure" instead of "yes" and when you ask her to do something she says "of course". Literally makes our hearts melt every time. She loves to sing, especially songs from the movie Frozen, and is truly just such a sweet, cuddly, soul. She still loves to drink lots of milk and eats an abnormal quantity of toast everyday, and has found a new favorite food. The chocolate covered frozen bananas from Trader Joe's are always a slam dunk no matter how bad she feels. She looks really good and healthy and if she hadn't lost so much of her hair in the front of her head you might not even guess she is treatment. She is super active around the house and we can't wait until we can take her back to the park again, or to a gym class, or other activities that the other 1-2 year old children are doing right now.

So here is a bit about what's next. I created a little treatment cheat sheet for us to share what we have ahead of us for the next part of treatment.

Round 2 of 4

Sinatra showing her displeasure with the
'CHOC Bear' waffle. It clearly doesn't fit
into the Toast or Whip Cream category.

We have now finished the second of 4 rounds for the 'Interim Maintenance' phase of Sinatra's treatment.  We were able to start this round much sooner because we didn't need to have a lumbar puncture procedure to start, this enabled us to start the required 6 hour IV hydration right away when we arrived at the hospital.  

One things that was surprising but exciting was the weight gain that Sinatra had shown.  In the previous round Sinatra was ~9.7 kg and when she weighed in this round she was up to 10.7 kg. This is great because it means that she is eating.  Even though all she wants is Whip Cream, Toast and Milk, she is eating!!!!  Now the tough part of this weight gain is we had to increase all dosages of her medication and chemo.

Sinatra showing a big 'smile'
while drawing at the CHOC playroom

As the treatment went on for the first 24 hours around dinner time each night Sinatra started sweating and vomiting.  Sinatra didn't react this way to the High Dose Methotrexate in the first round so we were surprised by this reaction.  We talked to the nurses and doctors regarding this and they say that this is expected.  They mentioned that in the 4 rounds of this treatment plan Sinatra will react worse each time of the treatment.  That is scary because now we can only imagine what round 3 and 4 will be like.

During this stay at the hospital we only saw Sinatra's Blood Sugar drop to a low level one time, which is very good news. It was again at a time when she didn't eat anything for the prior 24 hours so we still think it is during times of fasting.

We finally discharged on Sunday at 10:00 pm and it was nice to get home again.  We are scheduled to begin round 3 of this treatment plan on Wednesday 5/21/14. 

Racking up frequent stay points at the hospital!

Sinatra riding the halls with IV stand in tow.

We have started our first week of the new phase of treatment called Interim Maintenance, which will take place in the hospital.  The expectation was set that this could be anywhere from a 3 day stay to a week.  We of course never plan on a 3 day stay and always assume a full week.  

The week started on Tuesday when we arrived at the hospital around 8:00 for admissions.  Once we got up to our room we then waited until about 11:30 for our lumbar puncture procedure.  Once that was done we started the seemingly forever wait for our high dose methotrexate treatment to start.  To start the high dose methotrexate Sinatra first has to hydrate over IV for 6 hours prior and then her urine has to show certain criteria in a test before she can start.  It seemed like forever but we finally started the high dose methotrexate at 10:00pm Tuesday night.  This chemo  runs for 24 hours and this first dose went very smooth. Once the chemo has completed the nurses start giving a follow up medicine (Leucovorin) at specific intervals after the start time of the methotrexate to help the body get rid of the chemo.  We can't leave the hospital until her methotrexate level is below .01 which is on average around the 72 hour mark. We also must monitor the methotrexate level and be very mindful of when the Leucovorin is given because if the methotrexate levels don't go down in a steady pace it could cause some serious side effects.  This is the purpose of us being in the hospital the entire time.

As always things were going smooth and then we are dealt another blow.Thursday afternoon the Dr's noticed that Sinatra's labs came back with a low blood sugar.  After research they saw that her blood sugar had been low multiple times over the past few months.  This was significantly low and we were surprised that she showed no side effects (tired, confused, seizures).  Sinatra's blood was 40 and below many times and the normal level should be above 60 (however they want to see above 80).  Now we had to start testing to see if there is organ issues that are causing this low blood sugar.  It also would make for an interesting night because we now had to wake up every 4 hours to prick her finger (the same as someone with diabetes) to test her blood sugar.  As long as she stayed above 60 throughout the night we would be considered ok.  Luckily she would stay around the 90 mark which means she did great.However to be sure we are being sent home with a bold sugar test kit and a emergency glucose pen in case she goes into a seizure. 

Well Saturday morning as we were getting discharged (literally signing discharge papers) we got more roller coaster news, the Dr. said that Sinatra needed a transfusion which means we need to stay there another 4 hours minimum.  In the grand scheme it is not a long time but when you are preparing to go home and you start the process to be told you need to stay longer it is pretty deflating.

Anyway we should be back in the hospital in 10 days  or so for round number 2 out of 4.

The results are in!!!

Wow what a long weekend that was. After our procedure on Friday we just had to wait until we got word of Sinatra's MRD. It was a gut wrenching wait. While we were waiting we would start talking about scenarios and plans of how we would deal with certain results knowing that as a very high risk ALL patient, we probably wouldn't be a 0 MRD.  On Tuesday we finally got in touch with our case coordinator who had the results.  We wish we could express the overwhelming joy when she said '0'!!!!!  We couldn't believe it that Sinatra got a 0. This means that Sinatra is in remission and there are less than 1 out of 10,000 bone marrow cells that are leukemic.

This was so amazing and we decided that it was time for a night out to celebrate. We truly needed the stress reliever of a good night out with plenty of cocktails!!

Having our family meeting with Dr. Kirov,
Amanda our case coordinator and a cake
'Celebrating Sinatra's 0 MRD, Thanks CHOC'

On Thursday we had our family meeting with Dr. Kirov at the clinic to discuss the results and talk about next steps. We decided that it was time to celebrate with the Dr's and nurses that helped Sinatra hit this incredible mark so we brought in a cake to celebrate. Our meeting also helped us enjoy the rest of the day Thursday which happened to be our 4 year anniversary.

Even though this treatment and struggle is not over and we are far from being out of the woods, this was a huge milestone for all of us.

We are now on to the next phase of chemotherapy called Interim Maintenance. This phase will be 63 days and consists of a 3-5 day visit to the hospital every 14 days for chemo.  The chemo that Sinatra will be getting is call High Dose Methotrexate and takes 24 hours to infuse over IV.  There is also special protocol that needs to be followed while taking this drug due to the  toxicity of it.  Sinatra's stays could be longer or shorter and all depend on how quickly the blood tests show that the drug is out of her body.

We get started next week on Tuesday and we will keep you posted on how it goes.

Now we wait...

Hanging out at OPI

On Friday, 4/11 Sinatra had one of the most important  and anticipated milestones in her treatment plan, her Day 56 Bone Marrow Aspiration. Everything with the procedure went well and when we had labs drawn that day we also learned that Sinatra has low hemoglobin and would need a blood transfusion. We also learned that she also has a super low, zero, ANC again so she is neutropenic which means we are in isolation once again until her counts rebound. The doctor believes that this is normal because of the intense chemo she had this last round but its just so frustrating because we keep hoping that things will just start going up. She seems so normal right now; happy, eating well, sleeping great, sometimes it just doesn't make any sense at all.

Now we wait for her Day 56 MRD results...

The MRD will help us understand how much minimum residual disease (cancer) is still in her bone marrow. At this stage if it is more than .01% we are told that we will need to consider a Bone Marrow transplant. We are hoping and praying that it will be .01 or less which will allow us to continue doing chemo and complete our road map over the next 20+ months.

We can honestly say that the waiting for this result has proved to be the most anxious we have ever been in our lives so far. We have thought about the times when we have waited for things before, like waiting to find out if the loan for our house got approved,  or waiting to find out if we were pregnant, or waiting to deliver our daughters in the operating room. Compared to waiting for Sinatra's MRD results, waiting for those things seems easy now and we will never look at these 'life events' the same or with the same level of anticipation.  Nothing has ever had us this stressed, this anxious, killed our concentration, affected our sleep, affected our dreams and had us to tears just because.... This is more than anxious and we aren't quite sure how to describe it.

We hope to get our results Tuesday or Wednesday this week and we have a tentatively planned Family Meeting with our doctor on Thursday to discuss the plan for going forward.

Please continue to think about us and hope that the cancer is gone. We will update you soon.

Hello again CHOC Children's Hospital

Sinatra at the ER early Saturday morning. 

Well we thought we could get through Sinatra having no immunity without a stay at the hospital, but we were wrong.  It was a week since Sinatra's last dose of chemotherapy and we were doing ok but on Friday night Sinatra started looking uncomfortable.  When we put Sinatra to bed she slept maybe 3 hours before she started whining and rustling around. We checked on her and she seemed ok. From that point forward she was up crying every hour and started coughing. We called the hospital at 11:00 pm to see what they suggested and they said as long as she doesn't have a fever and isn't vomiting we should be ok.  At 1:00 am we took her temperature again and it read 99 degrees.  For the next 4 hours Sinatra was awake and clearly uncomfortable. At 5:00 am when we took her fever it registered a 101.6 which means get back to the hospital.  We called the Dr and she said she will get a room ready and to get to the ER ASAP.  

We got to the ER and they started giving Sinatra antibiotics and fluids right away.  By 9:00 am we were up in our room.  Sinatra s temperature spiked at 103.9 that morning but it finally got under control (although wasn't completely gone until Monday) in a few hours.   Sinatra wasn't eating or drinking and just wanted to lay in bed. Little did we know she wouldn't leave that bed until late afternoon Tuesday.

This may have been the hardest few days of our journey so far.  Not only did we find out that Sinatra had a 0 ANC and white blood cells less than 100 but she also started to show severe pain.  From Saturday morning until Tuesday Sinatra was in enough pain that she literally would cry if she wasn't laying flat. We couldn't get her to sit up in bed let alone stand up or walk.  Sinatra would cry in agony when we would change her diapers. This was a painful few days for our poor girl.

During this time the Dr's confirmed that Sinatra had no infection or virus which was good news but they were still worried about the pain.  Over the next two days Sinatra was on three antibiotics, pain medicine, anti-nausea and stool softener.  During these three days they also had done an X-ray, cat-scan and ultrasound to ensure there was no organ failure or other major issues.  The Dr's couldn't find anything.

On Tuesday after some pain medicine Sinatra was able to sit up in bed. We were able to then put her in a chair and even walk for a bit until the pain medicine wore off, but this was a huge step and relief. Over the next few days she was sitting up more (with less visible discomfort) and was walking around the hospital.

We ended up staying a loooooonnnggggg full week at the hospital. These nights are often hard to get any sleep whatsoever. The nurses have to come in every 2 or 4 hours to check Sinatras vitals. Even if the nurses are very quiet we did notice that when they wear crocs, the crocs squish on the floor so loud that it can wake Sinatra up.  Crocs may need to redesign the sole of those shoes!!!!

Standing up after some good pain meds.

We are glad to be home and Sinatra is getting some great sleep in her own bed.  Sinatra still isn't eating great but she remains happy and energetic.   We have started chemo again and will finish our last treatment of this phase on 4/4/14.  After that we will be anxiously waiting for the bone marrow aspirate procedure on 4/11/14 and the results the next week.