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.

0.1 WBC = Living in a Bubble!

After an intense treatment plan last week we were starting to feel like we might get a little bit of normalcy back in our lives for a brief moment thanks to 10 days of no chemo. We went to the clinic today for labs, which we generally do at least once a week, and Sinatra seemed like she was doing great. She hadn't needed nausea medication for a few days, was eating pretty well, has been happy and using more and more words everyday. Our only concern was that she looked a little pale and lost some of her energy, so we thought Sinatra might need to get a blood transfusion. Once we left clinic and we had a feeling that the phone would ring telling us to come back later today or tomorrow to get blood.

Playing with her utensils at the clinic today

Then the nurse from the clinic called and said that Sinatra's hemoglobin was 7.7 and she needed blood so I packed things up and we headed over. When we got to OPI Dr. Horvath came in and shared the news that in addition to low Hgb her ANC was 79 and her white blood count was 0.1. This essentially means she has 0 white blood cells or no immune system to fight of any sort of infection whatsoever. This means that the Cornelius household is in full lock down mode trying to keep Sinatra isolated from anything or anyone that could have any sort of sickness. The doctor said we should even avoid food that has any potential bacteria or could cause illness, like fruit from the ground. It's gonna be a lot of canned fruits, veggies, and processed foods for the next few days.

Playing outside on 3/16

We head back to the clinic on Monday to see how her blood counts look  and have chemo planned for Tuesday regardless of any changes in her levels. In the meantime we are just trying to keep the girls separate for a bit, so if any of Siena's friends are healthy and want to have a play date we will be game for making that happen. Siena is doing great by the way.  She is such a sassy little three year old and she is really funny except for the poopy and pee pee references she makes to everything. For example, mom: "Siena, do you want a PB and J sandwich". Siena: "No mom, I want a poopy sandwich,  ha ha ha!!" It was funny like two months ago but we can't believe she is sticking with that joke. Link keeps trying to get her to come up with something new but she still thinks its so funny.

More to come next week, fingers crossed her counts go up so we can keep going with chemo this round and get to that all important MRD test scheduled for April 7th.

When high blood pressure is normal for parents...

Sinatra helps pump a syringe in her port

It has been a busy week since our last post.  In our last post we had mentioned that Sinatra had a low ANC count so her chemotherapy was postponed.  Well at the end of that week we went in to the clinic on Friday hoping for an ANC over 750 (minimum required to continue) but it was down to 300.  We thought there was no way that her ANC would rebound over the weekend and be above a 750 on her Monday lab appointment.  You can imagine how surprised we were when we learned that Sinatra's ANC was above 900 at her Monday labs so we were approved to start her treatment again on Tuesday March 11th.

The next 30 days we Sinatra will participate in a round of treatment called Consolidation Part II and this is the first of the two "study" arms of treatment Sinatra will have. What that means practically is that treatment will have different medications and frequency than whats typical during this phase. Because Sinatra is considered in the Very High risk category of A.L.L. this could really help increase her chances of avoiding relapse. The treatment that we started on this week was pretty intense for Sinatra and consists of 5 straight days of chemotherapy. During the sessions Sinatra began with a drug to help her battle nausea (Zofran), then would have a two hour IV drip of a very intense chemo therapy (Etoposide) mixed with saline solution for dilution followed by a second 30 minute IV drip of a different chemo therapy (Cytoxan) and finally two hours of IV hydration after all of that is complete.  Needless to say the days are extremely long. 

Here is Sinatra on her first day of this chemo

The first day of new treatment is always a scary adventure and this time was no different.  When we started the chemo on Tuesday our nurse was responsible for checking Sinatra's vital signs every 15 minutes while Link was responsible for making sure Sinatra wasn't doing or acting out of the ordinary which could be an early indicator of an allergic reaction.  The chemo started and within five minutes Sinatra started coughing heavily, panting for air and dry heaving.  We called the nurses instantly and they rushed over to immediately stop the chemo and observe Sinatra.  After 10 minutes of observing and Sinatra calming down we started again and luckily everything was okay with the exception of daddy's heart rate, blood pressure and over worked sweat glands.  During the first two hours Sinatra also broke out in a rash but that went away after a few minutes and didn't cause any issues.  

Day two FaceTime with Daddy at work.

These days feel so long to all of us. We get to the infusion center at 8:30 am and don't get home until 3 or 4pm each day. By the time we get home we are so mentally and emotionally exhausted because the entire day we are on edge trying to ensure that everything is ok with Sinatra. Not to mention we are trying to keep her happy and busy while stuck at the clinic or hospital. It is clear that Sinatra is feeling some of the side effects of the chemo because she is a little more grumpy than usual.  But remarkably, the hardest part for her is removing the bandages from her port on her chest.  Because she has had so many bandages on, and she is being accessed every day, it has made her chest area raw.  It especially hurts when they have to do an alcohol swab to ensure the area is clean before they put the needle in.

Getting some good snuggle time with Mommy

The week is finally over and now we have a 10 day break from chemo (however we will still be drawing blood quite regularly).  Essentially this phase will be done in 24 days from today and that is when we have an extremely important marrow and blood test again. This test will determine how well Sinatra is reacting to the drugs and also determine our next steps for treatment. 

Do not pass go, do not collect $200.

This week we were gearing up for Sinatra's next round, Phase 2 of Consolidation, and our first "study" arm of treatment. Sinatra's blood counts from labs done last week looked good and we went back this morning to do labs once more to make sure she was ready to begin treatment again. This next round is what they call "count dependent" which means that her blood work has to return counts that are above a certain number in order for her body to be ready to take on more chemo. Specifically her ANC has to be 750 or greater to begin getting more chemo.

Unfortunately she went from an ANC of 1000+ on Thursday to an ANC of 420 today which means we won't be able to start treatment this week. So no meds this week while Sinatra's body recovers and we will be back at the clinic on Friday 3/7 and Monday 3/10 to see where she lands. If all is well then we will be starting the next round on 3/11. 

The first week of this phase consists of 5 straight days of chemo with multiple drugs and hydration through an IV that will require 8 hour long visits each day to the OPI. This round is planned to be her most intense schedule yet. We will give you more details on the plan, the meds and the next 30 days once we get started. We are literally just taking things one day at a time right now.