The next 'new normal'

Wow what a few weeks it has been.  We have had some exciting times over the past few weeks, here is a list of the highlights:

Sinatras last chemo before maintenance

1. We had our final chemo before we go into maintenance
2. As a family we went to a restaurant for the first time in over a year
3. Sinatra got to go with Siena to see Santa clause

Us at a restaurant (outside and in the far corner
away from all human life, but at a restaurant)

It is almost a year since the day that Sinatra was diagnosed with Leukemia and even though the time has flown by it feels like it has been the longest year ever.  There have been so many ups and downs throughout this year that we are really looking forward to things getting back to somewhat of a normal state. As parents though we realize that our normal will never be easy again. We have two years of chemo left and many years after that of blood tests to see if the cancer has come back.  At every blood test while we wait we will not be able to think of anything else in the world besides the pending results of those tests.  For us every cough, sickness, bruise, vomit, minute of dizziness, fever or just acting weird will instantly put us on the defensive wondering of Sinatra has relapsed.  

Sinatra and Siena with Santa

To lighten this blog post up a little. Even though we will forever have the stresses listed above, this entire experience has really helped us change our perspective on things.  For example, last year Christmas was a horrible. We were in the hospital over christmas with Sinatra getting chemo. She didn't get to see Santa and Siena was surly confused because all of her friends were talking about all of the fun gifts they had gotten. We didn't celebrate Christmas until mid January. This year we realize that we need to make up for lost time and enjoy Christmas more than we have any other year. We realize that we need to make every day count. We realize that we need to be happy and thankful for 'today'.  We may get tired and grumpy sometimes but we find it much easier to get out of any funk we are in, because what is important is, making sure that every minute we are all together and healthy, needs to count!

Watching water boil!

Sinatra getting a transfusion at OPI
enjoying some Frozen

We are so excited that Sinatra made it through her Delayed Intensification phase and got some well deserved time off from the chemo.  Originally we were supposed to have about 14 days off in between phases which seemed like a perfect break and enough time for Sinatra to build her counts back. At the end of the hospital stay the other week Sinatra's ANC went up to 100 which is extremely low and risky but it was enough to get us released from the hospital.  To our surprise when we went back for labs the next week to see if we had a 750 ANC which is required to start our next round, she was still below 100 and required a transfusion. To us as parents it worries us because Sinatra was originally diagnosed when her body couldn't produce blood anymore, and now we are seeing the same trend. In fact we have come to the conclusion that for the rest of our lives every sickness, fever, bruise or lethargic moment will have us worried about a relapse.  Luckily the Doctors talked us off the ledge and made us realize that her low count was expected and the chemo worked.  It was the chemos job to get rid of the 'Blood Factory' in Sinatra's body, because that is where the Leukemia is produced as well. So after the low count Sinatra was  delayed and we would get labs again the next week. This time she was up just over a 100 and  after a few more weeks of the counts not going up we would be delayed for a full  month before her counts went up to 750.  We actually had one lab result where Sinatra had an ANC of 738.....uggghhhhhh. All that we can say is that it is nice to have a break and we know Sinatra needs it, but when you are focused on continuing treatment and getting to the 2 year promise land of maintenance, this time off is like watching paint dry.

Siena reading to Sinatra on the long
break from treatment

Sinatra has gained some weight but is still pretty inconsistent when it comes to her eating patterns. All we can tell you is that she loves mini pancakes. She will not eat anything for lunch and dinner and then when she wakes up in the morning she will down about 15 eggo mini pancakes.  Its like cookie monster but with mini pancakes.  

It is official though we are starting our interim Maintenance II next week. Then it is 56 more days until maintenance which means that we are scheduled to do enter day 1 of maintenance on Thanksgiving Day.

Sinatra and Siena getting ready for
football

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.

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.

Heimlich or not?

So we are now officially done with our consolidation phase part 1.  This phase seemed to have a focus on protecting the spinal column/fluid and brain from the possibility of any leukemia entering these areas.  During this phase, which lasted days 36 days (28 days normally but we added 8 because of our hospital stay), Sinatra had 4 spinal procedures which means she was under heavy anesthesia and had to fast for a minimum of eight hours before each time. In addition to the LP's (lumbar punctures) Sinatra also had 6 different types of chemo therapy, approximately 7 hours of chemo being administered by IV,  4 hours worth of blood transfusions, 9 doses of chemo therapy taken orally, 1 virus, 5 nights in the hospital,  and too many blood labs and hydration by IV to count.  We are all worn out right now.

Enjoying a snack after her last LP

Now that this phase is over we have a 7 day break and then it is on to Consolidation Phase Part 2.  This is the final 28 days of consolidation and is the 'study' portion of the chemotherapy treatment plan.  This being a study doesn't mean that this is untested drugs or new operations, it means that the Children's Oncology Network (COG) is testing a more aggressive approach to the chemo therapy for very high risk ALL patients.  In this study the doctors are using the same aggressive chemo therapy drugs on Sinatra that they would normally give to a child who has relapsed.  The goal is to see if giving this strong chemo the first time around will better the success rate and  reduce the chance for relapse in the future.

Even though we are glad that this phase is complete, and we haven't posted to the blog in a couple of weeks there has not been a lack of action in terms of side effects for Sinatra. We had quite a scary night this weekend.  When Sinatra was diagnosed we decided that at the end of each successful phase we would go out (date night) to celebrate another step closer to Sinatra being cured.  At the end of this phase we had planned to go out on Friday and had just finished cooking the girls dinner.  The baby sitter was on the way and we were getting ready and while the girls were at the table eating Sinatra started to vomit.  The first two times weren't to bad but then she started screaming and panicking like she was in pain and then stopped making any sound at all, but continued flailing around like she was choking on something.  This was so scary because we weren't sure if we needed to start giving her the Heimlich maneuver or just let her get through the vomiting.  We plucked her out of her seat and luckily she started calming down but then continued to vomit for another 20 minutes.  Since she didn't have a fever or other signs we believe this was a side affect of the chemo which the doctors said would happen.

Outside of the nausea which is starting to become a more frequent occurrence, Sinatra is a bit more wobbly on her feet than she has been. More of her hair is starting to fall out one strand at a time and we are learning that the side effects of her chemo are actually more likely to show up once she has had three doses or more. Then if they do happen its not immediately after treatments but generally 3+ days later right when you would think its out of her system. Its a real reminder of how intense the chemo really is. 

The other fun thing that is happening is that Siena is starting to have what we are discovering might be typical three year old, big girl bed, issues. She has us up tending to all sorts of things to make her happy and waking up randomly at night. There have been snakes in her bed, shadows on the wall, and friends that she must have in order to go to sleep which apparently she can't grab on her own. Needless to say sleep is still not what it used to be. On the bright side, the girls are becoming closer than ever, playing together and imitating each other and our parenting partnership just gets stronger and stronger everyday. We realize how fortunate we are to have each other to manage through each day. We are both managing to still be really engaged in our day jobs thanks to our amazing family helping us out. We are positive and optimistic as we approach each day and try to take care of ourselves and each other to stay strong for Sinatra. Wish us luck on our next round!

Time out

Sinatra enjoying the toys at the OPI center

After our long week off when Sinatra was sick, we completed our first week back on chemotherapy. That meant a lumbar picture and chemo on Tuesday, and then chemo Wed- Fri. Because the RSV knocked out Sinatra's ANC, we had to begin to withhold the MP which is normally an at home, orally administered chemo.  Even though reducing her chemo will reduce her side effects and make her feel better in the short term, as parents we don't love not giving it to her and would almost rather have her have it in hopes that it has a better chance of killing the cancer.  Regardless, we will continue to listen to the doctor and do as we are told.

The parenting dilemmas continued this weekend. We had to give Sinatra her first "time out".  We have been avoiding this but unfortunately Sinatra is a typical 17 month old in every way but her cancer and she is beginning to test her limits. We have probably been a little more than passive when it comes her but discipline is such a hard thing to be consistent on when your kid has cancer. Our doctor has encouraged us to treat her normally and we have to be fair in having the same expectations for both of the girls, but it was awful to put her in time out and watch her cry.

After her timeout, as our normal procedure, Jenn went down the hall to tell her why she was in time out and to have her say sorry and when Sinatra replied with 'Sorry Mommy' Jenn lost it and started crying.

As far as treatment goes, we have two more weeks left in this phase (called "Consolidation") of chemotherapy and then we start the first experimental section of her treatment.  After the 30 days of the 'study' treatment plan we get our test to see how Sinatra is responding.  Keep your fingers crossed!!

Clicking our heels three times FOR DAYS!!!

Sinatra's Hospital room. 

The good news is that we have finally been discharged and went home today.  After five nights in the hospital it was such a good feeling to have the doctor say we could go home.  It is amazing how stir crazy you can go when you are stuck in a less than 100 square foot hospital room for five days. We literally couldn't leave the room and during flu season we are not allowed to have visitors.  There are only so many times you can look out the window or play with the same toys or read the same books. We are amazed at how well Sinatra did during this past week.

Each day we spent at the hospital we would think that the signs were good and that we would be going home the next day but it never really worked out.  The doctors were waiting for a few things before we could leave. They wanted to make sure Sinatra had no fever, she was breathing with no issues and all of her blood levels start turning around and showing that they are rebuilding.

Sinatra's levels hit some new lows this week and it took until Wednesday night to have any of them show any sign of recovering.  Sinatra's ANC hit an all time low this week at 102 which is a very scary number. This number means that her immune system is at risk. As a guideline you want an ANC higher than 1000. 

Sinatra takes a nap with Daddy while we wait  to be sent home

For now we are scheduled to restart our chemo on Tuesday after a quick check up on Monday at the clinic.  Lets hope that we can get started and try to get a routine back in our lives. This week was a good lesson for us to realize that these weeks really throw a curve ball at our family and we have to be ready for them.  Hopefully we can find a way to continue to be productive when we have these inevitable hospital stays in the future.

On the mend, but..................very...............slowly...

So here we are, night number five, still in the hospital after thinking everyday this week that we might be coming home. Sinatra is on the mend, but her blood counts were hit pretty hard with this RSV and are taking a little while to climb back up. Her cough is better, she seems happy, has negative cultures, and hasn't run a temperature since Monday morning but her white blood count and ANC are still too low to get discharged.

We are going a little stir crazy because we still aren't able to leave our less than 100 square foot hospital room and during flu season we cant have any visitors (until March 31st). We are pretty sure that Sinatra has played with just about every toy that the hospital has on this floor and we spend at least half of the day by the window looking outside. Some days she has resorted to playing with a cup filled with coffee creamer packages and the handles on the hospital crib that make the mattress go up and down. Desperate times.

Sinatra's play space in our room

In thinking about going home, we have spent some time talking about tweaking our strategy to help minimize the spread of illness as best we can by creating some separation for the girls and trying to teach them to wear masks when someone is sick. Seems like a stretch but it could be really helpful if we could get the girls to be okay with it. 

One major tricky part about all of this is that we are both still working (trying) and we are trying to figure out how to get our work done through calls, meetings, etc when things are just so difficult to plan.  This week for example, we both had a very defined plan of who was where, on what days, at what hours, but the fever hit Sunday and our week has been shot since. We both have quite a bit of anxiety about how to manage everything and do the best we can. Thanks to all that are reading this that are impacted by our crazy reschedule requests and inability to plan. XO

Superbowl Sunday!

Super Bowl Sunday started out as a great day. In the morning we dropped off Siena at our amazing friends Carmen and Brandon's to play with their kids Max and Isla, while Sinatra stayed at home with Grandma Gloria. Then we went to downtown Huntington Beach to run the Surf City Half Marathon for the second year in a row. We both had a great race in the morning and achieved our own personal records for the 13.1 miler. As we were celebrating and enjoying a beer in the Surf City Race Beer Garden we got a call from Grandma that Sinatra had started to vomit. We immediately went home as this was the first chemo side effect we had seen this week. 

Once we got home Sinatra seemed fine. Our friends came over to watch the game and by half time Sinatra was losing steam and wanted nothing more than to cuddle. By the third quarter of the Super Blowout aka Super Bowl, deep mid-cuddle with mommy, she started getting very warm so we took her temperature. Sinatra's temperature started at 100.5 which means we had to start timing it and if it was still between 100.5-101.4 in one hour we would have to call the Oncologist.  We checked the temperature 35 minutes later and it hit 101.6 which means an immediate call to the Oncologist (101.5 and above means pack your bags, start the car and get to the ER). As we suspected, because they had warned us about this, we needed to take Sinatra to the ER immediately to be seen. Within minutes our friends were helping us clean up, Link was getting instructions from the doctor and Jenn was packing to get ready for an overnight at the hospital. 

Sinatra vomited again for the second time today as we waited in line to register in the ER and we were quickly taken into an isolation room (closed off from the general population) where they take oncology patients, since the ER is a germ fest. We waited in the exam room for more than 3 hours and during that time were seen by several nurses and a doctor. By 11:00 pm we were finally admitted to the Oncology floor and Sinatra was beyond exhausted and grumpy after all of the poking and prodding. Who would have thought we would be back on the cancer floor so soon after just having left barely one month ago. After arriving into our fifth floor room Sinatra was examined several more times and was hooked up to receive oxygen, IV for fluids, and some antibiotics. By 5:00 am this morning her fever had broken and we finally started to get some sleep.

We spent the rest of the day in our isolated room with doctors and nurses coming in and out in full protective masks and robes checking her vitals (imagine the scene from ET when the government agents quarantined the house after they found ET and Eliot in the creek ). They ran another VRP and found that Sinatra has a virus called respiratory syncytial virus infection, usually called RSV, which has a lot of the same symptoms as a bad cold.  Like a cold, RSV is very common and very contagious. Most children have had it at least once by age 2. RSV is usually not something to worry about, however it can lead to pneumonia or other serious problems in some people, especially babies. Like a cold virus, RSV attacks your nose, eyes, throat, and lungs and is spread when you cough, sneeze and share food or drinks.There are many kinds of RSV, so your body never becomes immune to it. You can get it again and again throughout your life, sometimes during the same season.

Sinatra is now on antibiotics and we will be hanging out at the hospital for a few days until she is recovered. This also means that our treatment will be paused until the doctor feels that Sinatra is healthy enough to begin chemo again. 

A very long day...

Sinatra began her first day of the 'Consolidation Part One' phase of her treatment today.  Consolidation will be a very difficult phase of Sinatra's treatment.  In this phase Sinatra will be going to get chemo four times a week for the first two weeks and has lumbar punctures (under anesthesia adding chemo to the spinal column) on Tuesdays through 2/18.  The first of the four days in a row she will be getting four different types of chemo.  Needless to say we are already counting the days until this phase is complete.

The first day of treatment started by having Sinatra fast 8 hours before her lumbar puncture procedure at 9:30 am. This fast was much easier since we aren't dealing with the 'roid rage' but it is still hard to have her fast for so long because she gets hungry and doesn't understand why we won't feed her.  The good news was that after Sinatra ate her dinner (only a few bites) she went to bed and slept through the night. This was the first night we have had a full nights sleep in over a month so we were thrilled!

At breakfast time we didn't want to rub it in that she couldn't eat so Jenn and Siena went to breakfast before school and Link didn't eat because he was taking Sinatra to OPI for the procedure.As soon as we arrived at OPI just after 8:00 am the nurses instantly hooked Sinatra up to IV fluids because one of the chemo treatments (Cyclophosphamide) that she was receiving today is extremely hard on the bladder and kidneys so they have to hydrate her two hours prior and four hours after administering the chemo.  

While hydrating we were able to weigh in again and Sinatra has lost another 2.6 lbs since her last weigh in on Wednesday 1/22. She is losing weight almost as quick as she gained it on the steroids, which the doctors prepped us for.

Within two hours of getting to the OPI Sinatra had already been put under anesthesia, gotten her lumbar puncture (Intrathecal Methotrexate), received a second type of chemo therapy (Cytarabine) via IV  and was prepping for the third type of chemo by continuing her hydration.  After another hour we started the third chemo (Cyclophosphamide) which takes one hour to completely run. While we were running this chemo Sinatra couldn't stand still and was out and about walking the floors of the OPI and playing with whatever toys she could find. This is a great sign but we are told that the effects of the chemo won't be truly visible until 3-4 days after the treatment starts.

By the time the third chemo was done Jenn had come in to the OPI to relieve Link for the rest of the day.  At this point Sinatra was just receiving IV fluids and being monitored. Thankfully she was able to fall asleep in the chemo chair and slept for a couple of hours. 

When Sinatra got home it was after 6:00 pm and we had to feed her dinner quickly because her fourth and final chemo (Mercaptopurine) for the day was to be given to her 1-2 hours after she eats dinner.  The problem is her appetite is clearly waning now and by the time we got home from OPI she barely ate anything at all.  After trying to entice her with a buffet of food we gave up and got her in for her bath and ready for bed. 

Its crazy to watch her run around and seem so happy after she had such an intense day.  Sinatra literally runs around the house hyperactive, yelling, smiling and laughing.  It is a very good thing to see but also very confusing for us as parents. It makes it difficult because it is very easy to slip into a state where you see her acting so healthy that you forget for a moment and think that she can just play and be normal. Tonight and for the last few days Siena has had a really nasty cold with a cough. We have been trying to keep distance between the girls but when you see them so excited to see each other and want to cuddle and play its difficult because we have to try to create more space for Sinatra. We try to stay as aware as we can and keep reminding ourselves that its 8-9 months that we have to be to be very disciplined with how we manage and take care of Sinatra and her Leukemia.

She PASSED, but with a C.

The long awaited results of Sinatra's marrow test are in and Sinatra passed her MRD test!!

Even though she passed the results weren't the .01 that we had hoped for but were higher at a .11. This again is a roller coaster of emotions because we really felt confident that Sinatra would pass this with a .01.  Even though these results aren't ideal, they are good enough to move her ahead to the next round of chemo. However we now have to start the conversation that we were dreading, a possible bone marrow transplant.  The transplant conversation is very early but Dr. Huynh wanted to us to start the process to be prepared in case we need to go that route in the next few months.  We will both get tested and well as Siena to see if we are a match, and our doctor will begin to explore the database to see if there might be a match out there as well.  If we aren't a match we will let you all know in case you ever considered being tested.

We will begin the Consolidation phase on Monday and after 60 days in that treatment plan, with two different rounds of chemo we will have another bone marrow aspiration to test her MRD again. This time she must have a .01 or lower or else the transplant discussion becomes more than just a discussion.  

Today we also found out which treatment plan we will be following. As discussed in our previous post we had the option of staying on study which gave us 3 different plan options (randomly selected).  We decided to stay on study after a lot of thought and a long question and answer session with the doctor today.  Once we decided, she was able to confirm that we were randomized to the experimental arm #1. This is the medium intensity treatment plan that has an additional two medicines introduced into the plan. It is a pretty intense treatment plan which starts next Monday and has 4 days in a row of chemo treatments for the first two weeks. The first treatment of the 4 day sessions will require Sinatra to be at the clinic for the entire day to monitor her vitals and keep her hydrated with IV liquids.

Sinatra reading her MRD results!

For some more upbeat news.  Sinatra is doing so good now that she is off steroids.  She is so happy and is laughing and playing constantly.  She only woke up once last night which was her best night since being on chemo therapy. When she weighed in today she had lost about 1 pound since last Friday which is great.  We also had the wound care specialist look at her rash again and it seems that her horrific diaper rash is getting much better. All of these little wins throughout her treatment feel great and keep us optimistic.

We will be adding a tab in the blog that outlines her treatment to give a more comprehensive view of the whole plan and will be easier to follow. Stay tuned for that.