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