Monday was Xavier’s last day of Gleevec (TKI)! The very drug that he has been taking for more than a year now to bring his Chronic Myelogenous Leukemia (CML) down to almost undetectable! He was dancing around the room when we told him he did not have to swallow the yucky tasting pills anymore! What a love-hate relationship we all have had with this drug. The love part strongly out-ways the hate because it got our baby to the point we are today. The hate part just comes more or less with the side effects it had produced. It is still unknown by doctors if they are going to put us back on Gleevec after transplant as a maintenance chemo, but for right now, we are celebrating!
After a three day “break” from the doctor’s office, Tuesday started at SCCA for a long day of appointments. First a blood draw and then we met with the Child-Life Specialist who wanted to speak with John and I about talking to both kids about the big “life events” that are about to take place.
Xavier entertaining himself while Mommy and Daddy have their meeting!
John and I both were a little hesitant about the meeting because while we have the upmost respect for this woman’s educated and experienced insight into speaking with kids, it’s something we needed to do on our own, and at the right time. Throughout the meeting she gave us a few very good talking points and was very understanding that we felt it best to talk to the boys at our own pace and in our own way. She agreed that because of Xavier’s age and how comfortable he is around doctors thus far, we really don’t need to say much. Just keep doing what we have been doing thus far… it’s been working. For Xavier’s big brother W who is 5 years old, that needed to be a conversation that must be handled more delicately.
We took some pointers from the specialist and in turn had a very productive conversation with Westin later on in the week. W is such a bright, kind, and very compassionate little boy. He truly is one of a kind. We sat down and I told him that Xavier’s blood is sick and that Mommy and Daddy are doing to be at the doctor with him for a while so they can get his blood all better. The first thing out of W’s mouth was, “I was thinking that his blood may be sick.” He was just so in tune with the whole conversation. He asked a few more questions like if Xavier was going to be getting a lot of chocolate milk and if he was going to be able to sit on the doctors round rolling chair. We have taught this boy well… We then let him know that he would have friends here staying with him while we were away. He quickly adapted to the idea but then as the message truly sunk in some more, he got a little sad. He then laid his head on my arm with tears in his eyes and said he was going to miss sleeping next to Xavier…
We ended the conversation with W on a positive note, I mean, he is going to be able to sleep in Mommy and Daddy’s bed AND be the Man of the house! We asked him casually just not to mention this to his brother right now. We don’t want to scare or confuse Xavier. Westin agreed. Then Xavier came outside and the first thing W said was, “Xavier, you are NOT going to the doctor.” LOVE LOVE LOVE my W!
So circling back to Tuesday’s meetings, after the Child-Life Specialist we met with the fantastic nurses on our team so we could go over more details of what to expect with transplant. We went through books of information and asked questions here and there. I’m just a tad jealous because John always gets praised by the doctors and nurses for his insightful questions… Well, I guess I would say I am very fortunate to have him there by my side asking the questions. Love you, babe!
We started to talk about the central line insertion and how Xavier would adapt. They then brought in a little baby doll with a central line in its chest and a feeding tube to explain it to Xavier. He asked some great questions and then started pretend administering medication with the syringes. It gave me mixed emotions seeing him playing with the doll. Happy that he thought it was pretty neat, but bummed that he would even have to know what a central line is. They then brought him in his own stuffed doggie with a line that he could take home and play with. The nurses are fantastic!
After all of the chit-chat, the meeting turned into a check-up for Xavier. He did great, per usual. The nurses then started in on cultures which are test that tell us if Xavier has anything weird or infections that would keep him in isolation at the hospital. It also just looks at things like the common cold. The way they test for this is swabs of the mouth and the rear end. Also, saline sprayed in the nose and swabs. All of this very uncomfortable testing… not a peep from Xavier. Nothing. He was completely compliant. Let’s be honest, way more than I would have been!
Wednesday brought us to Children’s Hospital for a quick dental exam. No sweat, he did great. Teeth look fantastic. We have been taking Xavier in for more frequent visits to his dentist in preparation for transplant. We talked with the dentist a bit about importance of mouth care throughout transplant. He was great which I am so glad because there is a lot of follow-up with the dentist post-transplant.
Thursday was the BIG day. All of the testing we have done over the past two weeks would tell us if Xavier is cleared for transplant. Simply said….
It’s a go!
The doctor went through all of the testing results. I was most anxious to hear about the heart because long-term use of Gleevec could cause cardiac effects. Heart looks fantastic, all primary organ functions all look great!! All of the cultures came back completely negative for anything including CMV. Neither of the boys carry CMV which is a huge win going into transplant. The doctors were amazed how both boys were both so “clean” of things that a typical child would probably already carry. The doctor then went over last week’s bone marrow aspiration results.
Everything came back good, nothing to worry about. Gleevec had still been doing its job the last couple of months. We are not at undetectable, but that ok. The results do however have the doctors a bit baffled. The percentage of leukemia cells in his peripheral blood are higher than what is in his bone marrow where the cells are produced. They have never seen this before and the doctor admits that both she and other CML experts she has reached out to do not know why. But, the good news is that with all this said, this information is not going to steer us off course. In fact, she said that after consulting with other doctors, it basically confirms that we are moving in the best direction for our situation. There is no doubt that Xavier’s unique and rare case will be the topic of many CML discussion.
For us, bottom line, transplant is still the way to go.
Friday we went to the Physical and occupational therapist. It went great. Xavier had a blast doing all of the activities like throwing the ball, shooting hoops and jumping around. W was with us and was a bit envious of the fun his little brother was having. He got to join in on a few activities at the end and of course got a prize too. The therapist was surprised by Xavier’s core strength and said you just don’t see 3 year old with this much endurance, balance and strength. Yet another positive for us going into transplant. We of course will be followed by the physical therapist for years after transplant. No problem, it’s a great time!
Looking ahead, Monday we go into SCCA as the doctor just wants to get her eyes on Xavier one more time before being admitted to the hospital on Tuesday. If he has any signs of a cold, they would postpone the transplant. She has him on what she calls “lock-down” so he won’t catch anything. She knows how good we have been with keeping both boys some-what quarantined the past couple of weeks and she thanked us for being so diligent about doing so. While it may have been difficult to keep our distance, for those that understood how important this is and respected it completely and did not complain knowing that it would make it harder on John and myself, we are grateful.
I guess this would be a good time to thank, from the bottom of our hearts, all of our family, friends, friends of friends, wonderful neighbors and of course John’s work who truly jumped to action in order to help us find a resolution after losing the childcare we had lined up for Westin during this process. The selflessness of so many people is so truly inspiring. It is so comforting to know that we are surrounded by more love and support than we ever thought possible.
Having such a huge set-back like this at the beginning and very critical part of our journey was something we tried our very best to avoid. It has broken my heart this past week that so much of our energy has been going into anything other than focusing on our boys. Despite all this we are strong, focused, and taking one things at a time. The past is exactly that, the past. We are moving forward.
I can’t believe the next update will be from the hospital….
It has been an overwhelming week. But together, we made it!
The pre-transplant testing is now well underway. In fact, we are already half way there. Things are really starting to move along quickly. It is a completely different pace then we have been used to, but we are fine with it. We are SO done with “waiting mode”. We know that this road ahead is only going to get more difficult on all of us, but we are ready to move forward and take things as they come.
We arrived at Seattle Cancer Care Alliance (SCCA) on Tuesday with our boys. Right out of the gate SCCA wants to see both donor and recipient. They need to make sure that Xavier is at the right point in treatment to undergo such an intense chemo regimen and they also need to make sure that W is a viable donor (completely healthy).
From the moment we arrived, we were welcomed in a fashion that really helped decrease the anxiety of the whole situation. I was most concerned about Xavier’s big brother “W” as he typically does not like going to the doctor. The pediatric team at SCCA were fantastic with both boys and made them feel very comfortable.
The first thing we did was go over our schedule for the next two weeks. Oh, it was exhausting just to look at! We also went over an insane amount of paperwork. It’s funny because after the day was complete, I was thinking to myself that I probably only really retained about 20% of all the information given to us. However the rest I wasn’t too worried about because I knew we would get to it again throughout the next two weeks.
Being that we have both the donor and the recipient in our family it really makes it more challenging. It’s a bit of a juggling act through the whole process. Oh, but please know that I am so very, very thankful that we do have this challenge. I am SO very thankful that my boys are a complete 10/10 match. In fact, the compatibility report the doctor showed us actually says they are a 12/12 match. That said though, for HLA typing they only look at 10 marks.
W was one cool dude while getting his physical
With having both boys as donor and recipient, they had to split us up a lot of the day because legally the boys can’t be seen by the same doctor. I went with W, and John stayed with Xavier. They both did great on their own but together they were amazing too. Xavier was so excited to show W how cool going to the doctor really can be. You get to push buttons and meet new people. OH and don’t forget the cool prizes!
Throughout the first day they both had full physicals, a full blood workup, tour of SCCA, and numerous meetings. We were emotionally exhausted but that didn’t overshadow how very proud we were of both boys. W is now done with his appointments until 2 days before marrow donation.
How we all felt after day one…
All in a day’s work, right…
Day two went very smoothly. We met with the nutritionist first. She measured Xavier’s muscle mass and height/weight index. They will need to track Xavier’s growth for quite some time as transplant related toxicities can effect things such as longitude growth and puberty down the road. The nutritionist also went over what a safe diet will look like over the course of the next few months. It wasn’t too far off from the precautions we already take, however we will be taking a food prep class before coming home from the hospital which will be good information I’m sure. In a situation in which you have little control, I think it is important to be very thoughtful in the things in which you do control. That said, I want to be certain that the food we give Xavier is prepared with complete precautions.
After the nutritionist, we had a meeting with the social worker who was very kind. We didn’t have much at this time to speak with her about so it was somewhat uneventful. She did however talk about getting Xavier signed up for the Make A Wish foundation. This actually kind of made things more real… She made sure that we knew that while it is for children with life threatening illnesses, it can also be for after the child has beaten their illness or disease. It was still a little hard to talk about though…
Anyway, we then went to Xavier’s arrival conference with the doctor to go through the ins and outs of transplant for Xavier. It was basically a re-run of the meeting we initially had with them months prior. Yes, it’s hard to hear potential things that can go array during transplant, but it was something we have already heard so thankfully there were no surprises. This is not anything that I find the need to go into depth with because while they give you all of the potential risks, that doesn’t mean they will happen. We will take things as they come. It’s the only way.
Thursday we started at SCCA for a blood draw and IV insert. They took the maximum amount of blood they can for his body weight which seemed like so much! Xavier was fantastic as usual. He just let the nurses do what they needed to do. Then we headed to Children’s Hospital for a chest x-ray which Xavier handled ike a complete champ. He even smiled and said, “Cheese!”.
Ahhh! The dolphin got me!!!
Our last stop of the day was to the Oncology clinic for Xavier’s Bone Marrow Aspirate. It is always hard for me to see him go under anesthesia. He is so darn brave it’s ridiculous. He did great. He didn’t come out of the anesthesia very happy though. Typically, this is not a problem. He recovered pretty quickly though.
Friday Xavier had an EKG and an Echocardiogram at Children’s. He has had these before and in true Xavier fashion, he did wonderful. After these appointments we headed over to SCCA for our meeting with the teams pharmacist. We went over a lot of the medications that Xavier will be taking. He has already started a few that are supposed to help protect some of his organs from the high intensity chemo. The pharmacist was so helpful in answering questions and he let us know that we can always call him if there is any question about Xavier’s regimen. That makes me feel good to know this considering when we are released from impatient stay he will be on about 15-20 different medications. Yikes! We will have questions!
After the great meeting with the pharmacist we met with the doctor who will be doing W’s surgery. We went through everything we will need to know about how the procedure is done. He also reiterated how important it is that Westin not get sick before donation. The doctor then reviewed all of Westin’s blood tests. He said the beautiful words… “Westin is a healthy boy and is cleared for donation”. I knew this in my heart but it just feels so good to hear. We have been through hell and back the past year and… well, it was good to hear.
So, I can’t say this was very eloquently written, but I wanted to get our whole weeks re-cap out there. We have another full schedule next week and we are prepared to take it on with the strength we had this past week. It’s funny because people always think it’s the parent’s strength that gets a child through a very difficult time. And I believe that. But in our situation, it has truly been the strength of Xavier that has helped John and I be as tough as we are. I can’t even put into words how his bravery has been contagious for the whole family. I feel so incredibly blessed to have all three of my boys.
Over the course of this last week the boys have been thrown curve ball after curve ball and they continue to have so much courage. Their lives have been different, and they are adapting. We had to take Westin out of school. He LOVES school. But he knows that he will be back next year. We have had to keep both Xavier and Westin from their friends and their Cousins. They love their Cousins more than anything. Change is difficult, especially when it happens during a difficult time in life. It is almost funny to me because as of right now, the things that are stressing me out the most have nothing to do with Xavier. I make a point though, to let those outside stresses go. Especially the ones you can’t control. Let go of that, and focus on what is important. My 3 handsome boys. I am blessed.
Below is a compellation of unpublished blog entries outlining some of the details of this last year. From diagnosis, to where we are now headed to transplant.
AND SO IT BEGINS
I must be completely honest and admit that this is rather difficult. Re-living the worst year of your life can’t be easy for anyone, right? Facing your harsh reality. All of the hurt, anger, tears, heartbreak… Yes, I could go on and on. But I know that I have the strength to do this and I also know that I have much more to tell then just the waves crashing down on us. We have moments of light. Many, many special moments that are guiding us to our beautiful sunrise. And I can’t wait…
Looking back now, there were signs that may have suggested there could be something going on with Xavier that was a little more serious. But, in the moment, that did not crossed our minds. Not Ever.
In the month leading up to Xavier’s diagnosis I took him to the pediatrician on three separate occasions. For the first two visits, our pediatrician was not available so we were seen by other doctors. I expressed to these doctors that I wasn’t really sure what was wrong. He was just not himself. Extra clingy, somewhat pale. Maybe an ear infection? Both times I was told it probably was nothing. In fact, something that really gets me every time is that the first doctor even said the words leukemia. She said if they would do additional tests that is something that they would look for. She basically then told me she never sees this and it was not really necessary to put Xavier through some extra testing. So unlikely. Well, what does she have to say now?
The third and final time we went to the pediatrician we saw our doctor. Finally, right? The only thing he had to do was look under Xavier’s bottom eyelid to see that he must be quite anemic. Ok, that is something we can deal with. At least it would explain his low energy and pale skin. So off we went to get his blood test. Not once every expecting to hear what we heard the next morning.
The call came the next morning. To put it into some-what mild terms, it was the kind of phone call that makes your heart sink, completely paralyzing you. When you just know something isn’t right even before the conversation starts. The doctor informed me that Xavier’s platelet counts were normal, but his red blood cell counts were low and his white blood cell counts were very high. I’ll be the first to admit, I am no doctor. I had no clue what this meant. But, you know when a doctor tells you that it is a difficult phone call for them to make, something is potentially very wrong. It was obvious that she didn’t want to come out and tell me what was going on, but after a little more prying on my part, she informed me that these blood counts point to leukemia. I still remember how uncomfortable she sounded during the call. Like it was the first time she had delivered such a horrible message to a parent. She then told us to go to Seattle Children’s Hospital immediately.
CRASHING WAVES
I really can’t remember every detail from the day we got “the call”, but there is no way I will ever forget all of the emotions running through my body. John had rushed home immediately. Thank God he had returned from a business trip the day before. I could not ever imagine going through this day alone.
On the way to Seattle Children’s, the car was silent. The feelings of confusion and disbelief running through our veins. What did this all mean? Little did we know the doctors at Seattle Children’s wouldn’t be able to determine this right away either.
This hadn’t been our first trip to Seattle Children’s as Xavier and his brother W had been there once before on separate occasions. Xavier for an arm fracture and W for mouth trauma. As scared as we were on our previous visits, this was terrifying. For me, I think it was the combination of being scared and not knowing exactly what or why that really got me. We checked in and they directed us to oncology. A nurse came and said she was going to set Xavier up with an IV. We didn’t understand why and we told her we were just there to get his blood looked at. She kindly said she would look into it and then came back a few minutes later with a doctor who explained to us that no matter what they find, Xavier’s red blood cell count was dangerously low and he needed a blood transfusion.
They took us back and attempted to get Xavier setup with his IV and get his blood taken. Simply said, almost impossible to do! Xavier fought them. He fought them so hard. It was completely heartbreaking. At one point there were three nurses plus John trying to keep Xavier still. I was trying to calm him down but that was also impossible to do. After about 20 long minutes, they got the IV in, but it wasn’t without numerous pokes in attempt to get the vein. Then we waited. And waited.
We were finally called back and questioned extensively by what I believe was one of the fellows. Every question she asked I was trying to figure out, “what does that mean? Why is she asking that? What do they think this is?” Finally, the oncologist came in. And honestly, I can’t recall much from that conversation. I just wanted her to cut to the chase. The lead up is the worst. Just tell me what is going on! Tell me that we came here as a precaution and now we know my baby is ok. The previous tests were wrong! This is not what she said.
The normal white blood cell count is 5,000 to 12,000. Xavier’s was over 50,000. They had eliminated all of the “typical” childhood leukemia’s. They had numerous doctors and pathology examining his results. They could not give us an answer. They had a strong suspicion that it could be a leukemia called CML, but that is ultra-rare in children, let alone a 2 year old. Without a diagnosis in that moment, I held on to hope that it was going to turn out to be just a blip in his blood counts and he would be ok. At the end of our conversation the doctor told us that we were to go home, get an overnight bag and come back to the hospital. I couldn’t believe what was happening. I asked the doctor strait up if she is saying that my son definitely has leukemia. She said, “I would be surprised if this was not leukemia.”
OUR FIRST NIGHT
Xavier before waking up from anesthesia. Bone marrow aspiration and spinal tap.
We made it back to the hospital that evening. Still numb, still confused and still very much in denial. We didn’t want to put Xavier through any more. He still had his IV in from earlier that day, but unfortunately it was completely tapped out. They had to put in a new one which again was near impossible. Xavier was fighting so hard. They actual could not believe how much strength he had considering his red blood cells were so low. It was a very painful struggle that involved many nurses, many tears, multiple rooms, and multiple un-usable pokes. He ended up with the IV in his hand.
All of the nurses and doctors were compassionate, but at the same time very matter-of-fact. I do have to say at that moment, I didn’t like them. I mean, none of these people were telling me what we wanted to hear. I hated that they were treating us like a cancer patient before he was even diagnosed.
Xavier received his blood transfusion throughout the night. There was no sitting still. We needed answers that no one could give us. There was one doctor, an older woman, who spent a lot of her time talking with us. She knew what a parent in our shoes needed to hear and I feel as though she was the only one there that gave us hope. She told us to look at Xavier and see that he does not look like a sick child. That he is going to be ok and nothing at this point is for sure. To pray for him. Miracles happen.
They checked Xavier’s blood levels in the morning and all of his counts had improved. His white blood cells had dropped to just over 34,000 and his hematocrit, which measures the oxygen-carrying cells, increased to 25. It was at a 16 before the transfusion. Right away you could really see that this transfusion had a positive impact on Xavier. It wasn’t just in the numbers. It was in Xavier’s energy level and attitude. The color in his face was starting to come back.
Xavier was having a great time riding around the halls of the cancer unit, giving each doctor and nurse a big smile. It didn’t bother him that he was hooked up to an IV. It didn’t matter that the night before he was poked and paraded every 15 minutes. It didn’t matter that he was in a strange place. None of this mattered to Xavier. He was just a brave little 2 year old boy making the best of a situation that must have been so very scary and confusing.
It was then time to meet with the anesthesiologist. For all of those parents who have had to put their children under for one reason or another, you know its very nerve racking. They walked us to the clinic to get started. To say we were terrified would be an understatement. One minute we are holding a completely awake and aware Xavier. The next, he is out and we are laying him down on the table for his procedure. We were a complete wreck. We were waiting in the hall of the clinic and there was a nurse who walked by and set us up in a room. She was very kind but I’m sure she didn’t need to have any families of patients walking by two noticeably distraught parents. The procedure just took about 7 or 8 minutes and we had our baby back. The nurses made sure all went smoothly and we went back to our room. At that point all we could think about was going home. We had done everything they had asked of us, now, can we please go home.
Earlier that morning the doctors made their rounds. We waited anxiously for our turn. The attending doctor came in with the whole team. Maybe about 8 people. Each person introduced themselves, and then the attending proceeded to explain our case. Every piece of me wanted to cover my ears and walk out of the room. The doctor reviewed Xavier’s case starting with all of his background information and then the blood test results. They then talked briefly about the cancers they still had to eliminate before making a final diagnosis. CML, CLL, or JBBL. All of which are chronic leukemia’s. The doctor also let us know that at this point we were able to go home. I was SO relieved to hear this. All of the other doctors and nurses throughout our stay had been telling us how unlikely this would be. I was SO thankful to be able to take my baby home. I know now that this is not usually the norm. But for us, at that point in time there was nothing that could be done without an official diagnosis and bone marrow aspiration results. The attending asked if we had any other questions, we said no, and they all proceeded to the next patient.
John left our room shortly after the doctors left. I didn’t know what he was doing. Maybe getting something to eat? When he came back, he explained to me that he did actually have a question for the doctor, but couldn’t ask it in front of me. John proceeded to tell me that he needed to know how serious this was. He needed to know if this was something we needed to put a time limit on. This was so hard for me to hear as it was never something that crossed my mind. We both broke down in that moment.
Fortunately, the doctor was very strait forward with us. He even asked John if he was in the medical field which gave me a small chuckle I didn’t know I had in me. The doctor said that they always have a plan for treatment. Each of the possible leukemia’s have a different plan, some treatments more aggressive than the other, but each one is something we will be able to fight.
It was then time to be discharged. All we wanted to do was get out of the hospital as soon as possible. We had yet to process or even begin to comprehend any of the devastating information poured on us over the past 48 hours. The discharge nurse came in and brought me the notebook… “Caring For Your Child”. A book Seattle Children’s puts together for caring for your child with cancer and blood disorders. Again, I almost felt offended that I was being told my child had cancer before there was even a diagnosis. I didn’t want anything to do with this notebook. I think it’s important for me to note that I do understand how vital this book of information is for families with cancer but I mean really… in that moment. I ended up taking the notebook home with us because I knew we had to, but I didn’t take it out of the car for quite some time. It just sat there and I just prayed I would never have to use it.
TRIP TO MD ANDERSON AND FINAL DIAGNOSIS
Three days after being released from the hospital, we returned to get Xavier’s results. This was it. I felt completely paralyzed by the fear. No matter what this doctor said, it would change our lives forever. The doctor sat down, looked at us, and proceeded to tell us exactly what we so desperately did not want to hear…
Xavier has leukemia.
Like they had suspected, this was not a typical childhood leukemia. The doctors believed it most likely to be Chronic Myeloid Leukemia (CML), but they still had to do further testing to eliminate the possibility of Chronic Lymphoma Leukemia (CLL). Both of which are considered ultra-rare in pediatrics, let alone a 2 year old child. The median age being 65 years old. I think in that moment, we knew that this was going to be a unique situation. There are no doctors at Seattle Children’s that specifically deal with CML because frankly, it is so rare to ever see this diagnosis.
When we got home we started doing our research. We could only find 4 pediatric oncologists in the United States that specialized in CML. After all of the research, multiple phone calls and emails with different oncologists across the US, we made an appointment with a doctor at MD Anderson in Huston, TX. They wanted to see us right away so I literally threw some clothes into a suitcase and we headed to the airport.
Xavier and his brother enjoying the sun at a downtown Dallas park.
It felt right. We had to do something in this situation where we felt so completely helpless. I am so deeply thankful for my pro-active husband who did not freeze up in this time of confusion and heartache. He kept us busy and feeling like we were doing everything we could in the moment. A second opinion and a few more eyes on Xavier was important to us. On the way to the airport, Seattle Children’s called and finally gave us an absolute diagnosis of CML. We felt a bit of relief to finally have a firm diagnosis. They still had a few more tests they needed to run, but fortunately, they were ok using MD Andersons results.
We spent about 5 days in Texas where they had to yet again do all of the grueling testing Seattle Children’s did including another bone marrow aspiration and biopsy. Xavier was a trooper through it all. The initial results came back and confirmed the diagnosis of CML. The oncologist at MD Anderson mostly agreed with Seattle Children’s thoughts on initial treatment so we decided, for us at this point, we would head back to Seattle to start treatment.
The trip to Texas was very important for our family. Despite having to go to Huston for some long and difficult doctor’s appointments, we spent a lot of our time in Dallas where we were able to re-group. Get our bearings so to speak. It was very much needed time spent as a family. We were able to truly refresh and gather our thoughts before returning home and starting treatment.
TREATMENT BEGINS
Diagnosis: CML
Mission: Doing everything in our power to give our baby boy the best chance at a long, happy, healthy life.
Now that that is cleared up, here is what happened when we returned home from MD Anderson…
We had an appointment with doctors at Seattle Children’s. Now that there was a finalized diagnosis, we were able to discuss in depth the type of leukemia, treatment options, toxicity related side effects and long term goals.
Here is a very brief overview of CML (Please remember that I am writing this as a parent, not as a doctor)
– CML (Chronic Myelogenous Leukemia) is a disease of the blood and marrow.
– CML is associated with an abnormal chromosome known as the Philadelphia Chromosome (PH Chromosome). Chromosomes are structures in cells that contain genes, which give instructions to the cells. The PH chromosome is an abnormality that occurs when a piece of chromosome 22 breaks off and attaches to chromosome 9. The breaks in both chromosomes cause the BCR and ABL genes.
– The BCR-ABL gene contains instructions that tell the abnormal blood cells to produce too much of a protein called tyrosine kinase. Tyrosine kinase promotes cancer by allowing certain blood cells to grow out of control.
– Too many blood stem cells develop into white blood cells that contain the abnormal Philadelphia chromosome crowding out the healthy cells.
– CML is ultra-rare in children and usually occurs at or after middle age. It accounts for less than 3% of all childhood leukemia’s.
– A Bone Marrow Transplant is the only possibility for cure.
After Xavier’s CML was diagnosed, they determined the stage, chronic phase with no blast cells. This is the best possible scenario when diagnosed.
Treatment: Tyrosine Kinase Inhibitor (TKI)
A TKI is a targeted therapy that blocks signals that tell the cells to grow and divide while not disturbing the healthy cells. This drug has been around for a little over a decade and has been proven to be quite effective. It doesn’t however provide a cure. It is a lifelong drug, or until it stops working. Additionally, because it is a newer therapy, there is little to no research on how it effects pediatric patients. What they do know is that in addition to some more mild effects from the toxins (nausea, fatigue, etc.) it has additional side effects for prepubescent kids including possible slowed growth and mild to servers bone pain. That said, before this drug came along, life expectancy for a CML patient without transplant was about 3 years. We are very thankful we have these TKI’s available to us.
Toward the end of our discussion, the doctor talked to us about getting his brother W tested to see if he was a bone marrow match for Xavier. It is about a 25% chance a sibling will be a match. While this is not the front line defense now that we have TKI’s, we have a unique situation with Xavier being so young. The doctor told us that if Westin was a match, we would have major decisions to make.
FURTHER RESEARCH AND DECISIONS
I can’t even begin to express how much Xavier impresses me each and every day. We began taking the oral TKI (Gleevic) immediately. This kid truly inspires the hell out of me. How many two year olds will agree to swallow horrible tasting pills every day that don’t make them feel so good? And for anyone wondering, I did lick my finger one day when there was residue from the medicine on it and it tastes horrible!
Immediately we did see side effects, the bone pain being the hardest for Xavier. But in true Xavier fashion he just fought through it. Over time the bone pain has subsided and just pops up here and there along with nausea and a few other things. It’s truly heartbreaking to see your child curled up in pain saying their “leggies” hurt. Xavier being so young, a lot of times I think he just pushes through the discomfort because it is so difficult to express to us exactly what he is feeling.
We started Xavier’s treatment as an outpatient. We went for blood tests at Seattle Children’s just about every day for the first couple of weeks, then every couple of days and so on. Lots and lots of pokes for my little man… Xavier had to receive yet another blood transfusion which improved his numbers and before we knew it, it was time for the one month bone marrow aspiration. Results came back telling us that the Gleevic was doing its job.
During this first month Xavier’s big brother W went in to be tested to see if he was a bone marrow match to Xavier.
We anxiously awaited the results….
Fast forward about a month and we got AMAZING news…
Xavier’s Big Brother is a PERFECT 10/10 Bone Marrow Match!!
What a truly amazing feeling. John was the one that got the call from the doctor and then called me immediately after to share the great news. I didn’t realize until that moment how much I truly wished him to be a match. I knew the chances were 1 in 4 so I hadn’t gotten my hopes up. I had prepared for either scenario. I was fortunate to be with my family at the time John called to let me in on the potentially life altering news so I could share it with them right away. They were beyond excited which truly confirmed to me what a big moment this was. Now, like our doctor had told us from the get go, we had some big decisions to make.
While our hearts were telling us to go to transplant from the moment we received the news that Xavier had a perfect match in his big brother, it was incredibly important for us to do our research and become as educated as possible. No doctor was willing to give us a straight answer as what to do. We needed to look at this from both angles. The risk of taking a TKI his entire life, and the risk and benefits of undergoing an Allogenic Bone Marrow Transplant.
Seattle Children’s set up an informational meeting with Seattle Cancer Care Alliance. Before that meeting, we decided to meet with Dr. Druker, one of the doctors who developed the targeted therapy (TKI) that Xavier is dependent on. Fortunately, he practices in Portland so it was just a day trip. The doctor was able to truly educate us in a fashion no other doctor had been able to thus far. He was also very honest with us which I completely respect. He did not have an exact answer for us. He absolutely stands by the TKI and wanted Xavier to continue taking it to get his leukemia under control, but after that…
The fact that the man who invented the TKI was not sure if we should continue or move to transplant spoke volumes. The fact is that Xavier is 3 years old, otherwise healthy, and has a perfect sibling match… We were now more anxious than ever to meet with the transplant team at SCCA .
FINAL DECISIONS
A few weeks after traveling to Portland to meet with Dr. Druker, we headed 30 minutes up the road to Seattle Cancer Care Alliance (SCCA). Fortunately, since this was strictly an informational meeting, Xavier did not have to come with us. I love my little man but was so thankful to be able to give the doctors our undivided attention.
We were anxious and scared to find out the risk and benefit of transplant for Xavier. Every situation is different and we knew that they would be extremely honest and forthright with describing how risky the transplant process is. So… they went over everything. It was a lot to take in.
The doctors agreed that because Xavier is very young and otherwise healthy, he should have an excellent prognosis change for cure of CML with and bone marrow transplant from his HLA identical sibling.
In terms of timing, the doctors wanted Xavier to take the TKI’s for 6months in hopes to achieve a complete molecular response. Ideally, they also wanted the transplant to take place when the risk of respiratory virus infection is low.
FAST forward to May 2015 and here we are. Preparing to start the transplant process. There have been a few delays but we are now here. Xavier has responded well to the TKI’s and is close to MMR. He has been on the TKI’s well over 6months and flu season is winding down.
Over the past months, while we continued to endure so very much, we have also taken advantage of our time as a family. From fun family get-ways to basketball games to just enjoying a fun evening at home as a family.
We certainly have a long road ahead, but our beautiful sunrise will soon be in sight.
We are ready. We are strong. We will do this.
Xavier Style: Surfing life's waves with strength and courage
Bye, Bye, TKI!
