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.

BIG decisions

Her appetite has slowed down

Four days post steroids and Sinatra is doing great. Her 'roid rage' appears to slowly disappearing, the cravings have subsided, she is eating less, and she is becoming more active every day. Just like our doctor told us, we are beginning to get our happy baby back. Since the medicine takes time to fully work its way out of her system we are still experiencing the night time wake ups and the skin sensitivity which makes the diaper rash tough, but she is truly improving every day. Its so nice to hear her laughing again and playing with her sister.

Whats on our minds now is our next big decision. When Sinatra was initially diagnosed with A.L.L. we were asked by the Oncology team at CHOC if we would be willing to have Sinatra be 'on study' during the first phase of her treatment. At the time when we were asked, we learned that the risks associated with being 'on study' would be minimal as they would only need to take an additional sample of her bone marrow and sharing her personal information (age, ethnicity, etc) for research purposes. Given all of this we read through and asked questions about the consent forms, and agreed. We both are supportive of research and felt like if we could contribute to Leukemia research with no risk for Sinatra, that it was an easy choice.

Now that Sinatra has completed the first phase of treatment, called Induction, we are faced with the decision of determining if she should stay on study. We shared with you a week or two ago that the doctors discovered a genetic abnormality called MLL. Because of this, Sinatra's chances of successful cure rate go from the typical 90% for standard risk A.L.L, to roughly 50% because of the MLL abnormality.

So now comes the tough choice if we decide to stay on study. Assuming Sinatra's passes induction and has a positive MRD result (we still haven't heard yet), we will have 3 options for treatment, two experimental treatment arms and the standard treatment that we spoke of before.  Essentially being on study and having the option of receiving experimental treatment could increase Sinatra's cure rate significantly but because it is a study those results aren't available yet. Basically what happens is if we opt in, Sinatra gets put into a pool of patients and will randomly get assigned to one of three options for treatment. 

1. Standard treatment used for all ALL patients. Known, tested, but in her case only 50% cure rate with the MLL abnormality.
2. Experimental Arm 1.  More intense than the Standard treatment and introduces 2 additional chemo medications that may help minimize the risk of relapse but have increased and potentially serious side effects. 
3. Experimental Arm 2. Most intense of the three treatment options and adds the two chemo drugs from Arm 2 plus an additional medication with such serious side effects that it makes receiving the chemo an inpatient procedure because the patient needs to be monitored 24/7 during that period.

*Both of the experimental treatment plans are currently used as medication for patients when they relapse so the thought is that if you hit the cancer hard with these meds now, perhaps the relapse won't happen.

This decision is weighing very heavy on us because we want to do whatever we can to give Sinatra the best treatment we can but also worry about her ability and ours to manage the side effects.  There is also the fear of the unknown because we don't really know what  treatment is truly the best for her.  We are reading and learning so much that it feels like we are cramming for the most important exam ever, to have as much information as possible to try and make an informed decision.  The good thing is that if we opt in we can change our mind and be removed from the study at any time, no questions asked. The other worry is whether or not our insurance will cover the cost of these experimental treatments. There is just no way to know what is the right decision when your dealing with your daughter's life. 

We have our next family meeting with our assigned team of doctors, nurses and case workers on Wednesday 1/24.  This meeting is where we will learn what Sinatra's MRD is and where we will need to share our decision about being on study.  Keep sending your good vibes and prayers our way because your support means the world to us.

D Day

D Day has come and gone and we would be willing to pay any amount of money to never have a night like that again. Lets walk through the events leading up to the meltdown.

We went into Thursday night somewhat confident, because half of the nights during the week leading up to D Day, Sinatra was able to sleep from 1:00 am to 4:00 or 5:00 am.  If she could do this on D Day it would be great because then we would only have to keep her calm for 4 hours leading up to the procedure.

Thursday night was going great. Sinatra had a great dinner, nice bath, was happy, and went to sleep easily.  As is the new standard, Sinatra woke up at 10:30 pm to be fed and have her diaper changed.  Proactively, we woke Sinatra at 12:30am to feed her the last big meal before her procedure at 9:30 am the next morning.  She ate a lot of food and went to bed quietly.

Then 2:00 am happened! 

S*!T......... Sinatra woke up with a poop, and D Day is on!!

Of course since Sinatra was up, she was starving, and the odds of her falling asleep were worse than the lottery.  We tried everything to keep her calm. We tried clear liquids but she only wanted milk, we tried playing but she wanted to go to the kitchen, we tried watching Mickey Mouse but she wanted the food network.  We couldn't win. We put her in her crib hoping that she would get tired from crying so aggressively that she would fall asleep, but that didn't work.  We tried everything but Sinatra was on fire all night. Imagine dealing with a child that can't talk, is extremely hungry, on steroids, very hungry, has a diaper rash that almost had her admitted to wound care, did I say hungry, and two parents that are so tired and emotional that they look like Eeyore walking around the house trying to cheer Sinatra up.  

Finally, after what seemed like four days, 6:00 am arrived and we decided to drive her around Huntington Beach. It actually kept her calm until we had to stop the car at a red light, then she would go crazy.  We decided to create a route around Huntington that was all right turns to keep from having to stop the car.  At 7:30 am we decided to head to the clinic a little early for our appointment.  When we got there Sinatra was so mad we couldn't get a good blood pressure reading or take her temperature. Some good news was that Sinatra didn't gain any weight since her visit earlier this week. We also got the news that her ANC was above 3400 which is always good to hear.

At the clinic prior to the procedure. Wow does Sinatra look angry!

When it was time to start her procedure, it seemed very odd for us because we were used to having these procedures performed in the hospital. Today and for the future procedures we will be at the outpatient clinic which doesn't 'feel' like a hospital for some reason. It has all of the equipment, doctors and nurses but there is a weird feeling that it isn't an actual hospital, its hard to explain.  Sinatra finally got her happy drug around 9:35 pm and started to calm down. The next part was unexpected and probably one of the hardest things we have had to experience so far. The doctors wanted us to stay in the room and hold Sinatra while they sedated her.  It doesn't sound to extreme but to watch your daughter slip into an unconscious state is a very hard thing to stomach. This was extremely emotional for us to be a part of and we will never forget these two minutes and how painful it was to watch. 

After about an hour the procedure was done and we were allowed to go see Sinatra in recovery.  The procedure was a success and they got the bone marrow sample they needed to test the MRD and were able to put the chemo therapy into her spinal column.  

When we were debriefing with the doctor about the procedure and what comes next we asked to confirm if Sinatra is done with the steroids that have been causing such havoc on her. We had told Sinatra the night before that it was her last day of steroids, but the doctor told us we had one more day to go........uggggghhhhhh.  We are looking forward to the 'non-roid rage' Sinatra to be back with us.

As for the results of the MRD test, our doctor would normally wait for our next parents meeting, which is on Wednesday January 22nd, to tell us the results, but she knows that we will be a nervous wreck until we hear the results so she said she will call us the second the results are in. 

We will post the MRD results as soon as we get them. Stay tuned...

The Battle of the Diaper Rash!

Sinatra pant-less letting her rash air out and waiting for her blood transfusion

This week will forever be known as 'The battle of the diaper rash'. The week started when Grandma Gloria joined us for what was supposed to be short routine labs visit on Monday morning, it was her first time visiting the clinic with us. This visit was supposed to be a quick and easy visit;  check height, weight, blood pressure and take some blood to get her CBC for this week. Little did Grandma, or any of us know we would be there for almost 6 hours! During this visit we learned that Sinatra weighed 29 lbs, a gain of another 2 lbs over the weekend and totaling almost 10 lbs since December 17th (we are trending at nearly a lb. a day).  and as we waited for our labs we had the doctor check Sinatra's bottom.

While waiting for all of the scheduled checks at the clinic, we had the doctor look at Sinatra's booty for the terrible diaper rash that developed last week.  We shared with the doctor that the rash started suddenly and grew rapidly early last week and while visiting the clinic then, we were able to score some hospital grade goodies from one of the nurses. We told the doctor that we had been diligent in obsessivly changing her and applying every cream and treatment known to man but the second Sinatra poops, all progress is lost and the rash is worse and has open sores again. Since it takes nearly five minutes to do a simple diaper change, because of the many things we need to do, we are changing her diaper nearly every hour and all we can think about is 'the diaper rash'! Oh, and to add to the fun, because Sinatra has gotten so big we had to buy new diapers and we are into a size 6 and may be buying adult Depends within a week. We truly didn't know that they make children's diapers as big as a size 6!!

Because of the rash we had to schedule additional visits at the clinic on Monday and Wednesday of this week and will be back Friday for her procedure. There was even a discussion of actually admitting her to the hospital to have round the clock wound care to try to make it better. Thankfully we aren't there yet.

Sinatra also received a blood transfusion today in order to get her as healthy as possible for her procedure this coming Friday. She will have a lumbar puncture to again check her spinal fluid for leukemia  and add more chemo therapy to her spinal column. She will also have a very critical bone marrow aspiration (she has had this procedure twice so far) to learn if Sinatra's cancer is in remission or not.  Our hair has much more grey in it this week just waiting for the results of the report. Fingers and toes are crossed!

Hard To Function During Induction!

Today we had Sinatra's second to last Chemo appointment in the 'Induction Phase'. We had blood drawn today and her ANC was up to 3900 which is the best its been since she was diagnosed, we were thrilled! The hope is that in one week when she returns for chemo and for her next procedure, Sinatra will be have completed the induction phase and will be in remission. We are looking for a .01 MRD; fingers crossed! We do have to remind ourselves that even though she may be in remission, the treatment that she will continue to have for the next 8-9 months will be much more difficult than these first 29 days.

Daddy and Sinatra enjoying some fruit at Chemo

During our chemo appointment today we were desperate to get some answers on two key issues this week. The first is Sinatra's horrific diaper rash that has developed in the last few days, and the second issue is her incredible weight gain.  

We have found that it is nearly impossible to get ahead of this rash because Sinatra's constant eating and drinking causes her to go to the bathroom almost every 30 minutes and that just makes the rash worse. We have tried everything to try and fix it including, corn starch, A&D cream, Desitin, Petroleum Jelly, bare bottom and now the hospital grade ointment and bandages.  Fingers crossed this heals soon, it has to be so painful and we feel so bad for her.

No one in the Cornelius house is sleeping. Because of  Sinatra's steroids she wakes up a minimum of 2 times a night, but has woken up every hour on some nights.  We have to get up when Sinatra does because the Dr. says that the steroids cause her to have an insatiable appetite. We are trying to be responsible parents by both giving in to try and keep her satisfied and trying to give her healthy foods. What's difficult is that she has gained soooo much weight so fast and it makes us really worried about her overall health. She has literally gained 1 lb over a two day period and a total of 5 or so in the last two weeks. Every time we ask the Dr's if there is a way to keep her full or suppress her appetite they say "no way" and "keep feeding her".

We are predicting a very bad night coming up very soon, we are calling this D Day!!  Next weeks appointment is a procedure to test her spinal fluid, add chemo to her spinal fluid and test her bone marrow for any remaining leukemia. Since Sinatra will be under anesthesia for this procedure she can not eat for 8 hours prior, which will be so hard since she has a hard time going 2 hours without eating. This will be bad news for the Cornelius household Friday morning from 1:00am-9:00am (procedure is scheduled at 9:00am Friday).  Everyone please be sending thoughts of or actual Nyquil and Ambien to our house starting Thursday after dinner!!! We are going to need it.

Santa Came To Town

Santa came this year for us on Saturday Jan 4, 2014, better late than never. We opted to wait to until we could all be together as a family and we are so glad we did. We will say it was hard to get into the Christmas spirit and for both of us we would have loved to just put this holiday season behind us but we knew we couldn't do that to the girls. So on Christmas eve (Jan 3), we stayed up late putting together Siena's princess car and made sure to eat the cookies (leaving some crumbs) that Siena and Jenn made earlier that day.

When the girls got up Saturday morning they were so excited once they saw there gifts from santa they didn't care about anything else. We spent the beautiful morning outside watching them drive around and got wistful about how they will probably do this together when they are teenagers, or at least we hope they will.

Our families were able to join us for brunch and to get into the spirit we put on the holiday music (thanks to Pandora), turned on the Christmas lights on the 'extreme fire hazard' of a dead Christmas tree and exchanged gifts. We felt grateful that our families went along with the delayed Christmas even though we knew it was weird for them too. In the end we were so happy we got to celebrate and capture a photo for our 2013 Christmas ornament to have a fonder memory when we look back. 

First Chemo Outpatient Treatment

"Get in my belly!"

This week at home has been fun so far. We are still figuring out how to make our adorable "Chubby Hulk" aka 'Chulk' baby happy when it comes to food. Her routine is totally out the window when it comes to her meal times and sleep schedule because she wants to eat about every 2-3 hours. The tricky part about it is that she is really inconsistent with what she wants and likes. One day she wants grapes, and the next day she hates them. For two days all she wanted to eat was slices of bread, but now wants nothing to do with it. We are not sleeping well either and have gone back to trading nights of who gets up to feed Chulk, which was our newborn schedule for the girls when they were babies.  Picture getting up in the middle of the night, trying to get her not to scream because she is starved and then walking her around the entire kitchen letting her point and nod to what she wants. One of the funniest parts about this is when we debrief in the morning about how the night went and talk about the crazy menu we prepared for her. For example we could say, "At 2:00 am I fed her a slice of wheat bread, a slice of american cheese, 3 saltines, 10 gold fish, pulled pork, a slice of orange, two strawberries, a cranberry muffin, Cherrios, half a gallon of milk and a gerber cereal bar. Its like the World's Fair buffet! 

Today Sinatra had her first outpatient chemo treatment at the Infusion Center at CHOC. During the last three weeks of this round of treatment we will be at CHOC for appointments or procedures at least once a week. We had an opportunity to play a bit before hand, talk to the doctor, and had a nurse make sure that we had properly applied Sinatra's numbing cream prior to her procedure to ensure that she felt no pain while receiving the chemo. They took great care of her and us during our first visit.

We were not completely surprised to see that Sinatra had gained significant weight based on her new crazy eating habits but we were shocked to find out that she had gained nearly 5 lbs in less than 3 weeks! For a 20 lb little person that is a ton of weight! We were slightly concerned about progress in this area but our doctor assured us that at the end of January when she starts her next phase of chemo she will most likely loose this weight plus some. Sinatra's blood counts were overall acceptable but based on the way she is trending we are guessing she might need blood at our next appointment this week. One of the more concerning results was that her ANC was a 287 which is the lowest point we have seen since she was diagnosed. This means that we will definitely be keeping her away from people and germs until we see it come back up.

Raging New Year

Happy New Year!  

Siena and Sinatra partying on New Years Day.

Being at home this week has been great. We are adjusting to giving meds and managing through Sinatra's newly developed "roid rage" and crazy appetite. One of the side effects to her meds is that she may become irritable. Ha! If by irritable they mean screaming at the top of her lungs in a rage and pointing at her high chair because she wants to eat yet again, then I guess that's irritable! It's unbelievable the amount of food this child is putting down. She even bit Jenn during one of these rage sessions most likely because we were out of milk (Sinatra's currently favorite thing in the world). We have dubbed Sinatra 'Baby Hulk' thanks to the rage.  The rage is not only weird but thankfully we find there is a bit of humor when Sinatra rages, (although we feel bad laughing) and when we laugh it helps bring her rage down to a mild simmer.  By day the rage is tolerable, but at night when Sinatra is sleeping it is becoming more difficult. Sinatra is normally great sleeper and would normally sleep from 7:00PM to 6:00 AM.  However now, she wakes up she has trouble falling back asleep because she  realizes that she is starving. Poor Siena who shares a wall with her is not liking it at all. We really hope we can figure out how to help her and get back to our routine because we are sure to be tired after multiple days or weeks of Baby Hulks middle of the night rage sessions!

As expected, the Cornelius household had an uneventful New Year this year. Fortunately, we were thrilled to ring in the new year with some good news. We told you before that Sinatra also has a genetic mismatch (MLL) and this makes her and is considered a very high risk Leukemia patient. We also told you that one of the major milestones is our Day 29 (about two weeks away) Minimum Residual Disease (MRD) test.  This test will tell us the percentage of leukemia cells that are in Sinatra's body. What we are looking for at the end of the first 29 days (which is about two weeks away) of treatment is a number below .01 . So the news is that just 8 days into treatment they did an MRD test and she is already at .071! We don't know what our starting point was but when Dr. Huyhn said it could be a 25 and it turned out to be so low this early, we were excited. Lets hope the trend continues downward. This is great progress and shows that the meds are working so we are thrilled.

In other news, Santa comes to our house tomorrow night, Jan 4th, so we have been scrambling to get caught up on things that we missed while we were at the hospital.  We had to finish some Christmas shopping and wrap everything but we are now ready to go. Keeping the Christmas jig up has been hard but we don't think the girls have noticed. If they were any older we think we would have been in trouble. Even though our tree is brown and an absolute fire hazard we are ecstatic that with all of the recent events, we are still able to celebrate Christmas as a family at home. So to all, Merry Christmas!