Day -6 The Start of Chemo: Treosulfan & Fludarabine with some Sandbox Therapy

Ryan and I are both pretty tired today (I think me more so than him).  Between getting up at 4AM yesterday, then getting to bed late last night, followed by getting woken up every so often after that for the nurse to check vitals, give meds, take blood for labs and weigh him, I feel like I have a hangover (without having anything to drink).  All the side effects without any of the fun! (Note: Ryan has to be weighed every morning between midnight and 4AM. It’s a crazy time to get a kid out of bed, but they need the data before the doctors do rounds in the morning.)

Ryan received two chemotherapy drugs today by IV, and so far he hasn’t had any adverse reactions.  They told me that he may start feeling sick in the next day or two, but that kids normally do fairly well the first few days.

What did surprise me today was that he needed a red blood transfusion. His hematocrit has been fairly stable (but still low) for the last three months (around 25-27), which was unusually high for him, and nobody had an explanation for it.  However, it dropped to 21 suddenly.  There is no explanation for the drop either, because he had not yet started chemo.

The highlight of the day for everyone (hospital staff included), is the indoor sandbox I made for Ryan.  It has kinetic sand in it, which is a type of sand that is mixed with polymers.  It feels a little bit like dry cookie dough and sticks to itself like moist sand.  It has been keeping Ryan busy most of the day and everyone that comes in the room is really intrigued by it.  In fact, his nurse put on clean gloves and played with him in it for a little while earlier.

The other thing that seems to have created a buzz among the staff is Ryan’s ability to easily take pills at his young age, along with my giant cache of flavored capsules that I brought in to assist him in taking any pills that might taste bitter (I put the pill inside a capsule).  The nurse told me that they even looked them up online this morning to check out pricing, etc.  I have now ordered them from “Capsule Depot” twice, with this last order consisting of several thousand, because we are sure to need them when Ryan is discharged and on many oral meds.  I keep wondering if I have now made it onto the DEA “Watch List.”

Day -7 We Are Checked In and Ready to Go

We were up by 4:00 AM this morning and checked into Seattle Children's at 6:00. (This is after I stayed up until almost midnight packing and then spent an additional hour or so trying to shut off my brain and go to sleep.)

Ryan had surgery to remove the port in his chest and replace it with a different type of central line called a Hickman. One of the main differences is that there are two access points, which is necessary because of the amount of simultaneous things that will be going in and out of his body all at once (chemo, blood draws, IV fluids, pain meds, etc.)

A second surgeon performed a procedure to remove a tiny piece of testicular tissue. Half of the sample is being sent to Children's Hospital of Philadelphia (CHOP) as part of a clinical trial to experiment with multiplying the stem cells. In primates, researchers have seen some very encourage results in restoring fertility when they have done this same process and then re-implanted the stem cells later. The other half of the sample is Ryan's to keep. It will be cryopreserved and stored in a freezer at the University of Washington for his future use, if he desires (or even needs it; there is no way to know whether the chemo will affect his fertility, but it is a risk.) By the time he is an adult, the hope is that the research will have progressed to successful re-implantation in humans, resulting in live births.

I managed to squeeze in a fair amount of work during Ryan's surgery and again later in the afternoon. I am trying to average working at least half of the time so that I don't burn through my PTO so fast. Some days I get in a half day, sometimes I am able to work a full day, and then other days I have to take a day of PTO. It's a challenge, but I am trying really hard to juggle everything and make it all work. I am really thankful for the support of my boss and co-workers in being understanding and flexible. Hopefully by this time next year, things will be mostly back to normal.

Tomorrow, chemo starts to destroy what remains of Ryan's bone marrow. Once chemo starts, there is no going back. I was forewarned today that the team will be showing up in blue hazmat suits in the morning.

Consent for Transplant

Tomorrow will conclude Ryan’s pre-transplant work-up with SCCA.  In the past two weeks Ryan has had an EKG, Echo-cardiogram, CT scan, MRI with Anesthesia and has been poked, prodded and swabbed in unmentionable places to test for everything from simple blood counts to every virus known to man.

Tomorrow he has one last clinic visit, where they will likely re-swab and double check to make sure he hasn’t picked up a virus in the last few days.  We will also have what is called a “Data Review Conference” with the doctors, where I will be asked to sign consent forms for the transplant.

I think back to the first few years of Ryan’s life.  I fed him organic food, plenty of fruits and vegetables, interviewed countless daycares until I found the best, and spent two weeks researching the safest car seat.  Now, I am consenting for him to be part of a clinical trial that includes administration of a chemotherapy drug that is not even yet approved by the FDA. What a contrast. However, I know that out of all of the transplant protocols I reviewed, I am doing what I have always done for him and am selecting what I think the best option is.  From there, I just have to remind myself that the rest is out of my control.

“No More Delays; We’re Going”

Those were the words from Ryan’s transplant doctor today.  His bone marrow transplant will be May 29^th.  She stated that “His continued dependence on platelet transfusions and his dangerously low ANC are not compatible with life.”

I actually half expected another delay.  I recently noticed some differences in his labs in the past month.  He has had a lot of unusual red cells noted on his labs: his MCV (mean cell volume) has been steadily climbing, he has had teardrop and elliptical shaped cells, along with polychromasia (an abnormally high number of red blood cells found in the bloodstream as a result of being prematurely released from the bone marrow during blood formation.)  All of this strange red blood cell activity has resulted in his hematocrit staying fairly level (although still low), and he has gone almost three months without a red blood transfusion (he normally only goes about a month in between transfusions).

On the other hand, his platelets and ANC have tanked.  He has been requiring more frequent platelet transfusions and is considered neutropenic due to his low ANC.  This puts him at great risk for hemorrhage and infection.  His doctor said that even if his red cell line / hematocrit was completely normal, she would still recommend transplant due to the other two cell lines being continually low.

As a result of the recent changes noticed in his cells and counts, the transplant team coordinated an early bone marrow transplant last week rather than waiting for our “re-arrival” at SCCA this week. Preliminary results show his cellularity at around 40%, which is lower, but there still are no clues as to the exact cause of his bone marrow failure.

The pathologist who has reviewed all of Ryan’s biopsies over the last year said that his marrow looks “a little less normal” in this most recent biopsy than it has in the past, and it does have some “pre-dysplastic” characteristics.  With MDS the marrow becomes dysplastic (“dysplastic” refers to abnormal growth or development.)  It seems that maybe Ryan is moving toward MDS, but there still is no solid evidence.  SCCA is currently doing another round of cytogenetic testing on this sample.  Results should be back in a few days, so if a clear sign of MDS is discovered his diagnosis could change, but as of 1:00 this afternoon, his official diagnosis is “Underlying Bone Marrow Failure.”

Two Weeks Until We Resume the Pre-Transplant Workup

When we go back to Seattle Cancer Care Alliance (SCCA) on May 6^th, we will basically pick up where we left off.  I am assuming there will be some activities that we will not have to repeat (such as the dental consult, lung CT, heart echocardiogram, and classes about how to care for a transplant patient).  We will likely have to repeat things like the extensive virus screen and a massive amount of blood tests.

Ryan will also have a new central line placed, some reproductive tissue cryopreserved and banked at the University of Washington for his future use, and one final bone marrow biopsy before transplant.  I really don’t know what to expect from the bone marrow biopsy at this point.  There could be proof in his marrow this time of the Myelodysplastic Syndrome (MDS) that the doctors suspect, there could be a re-confirmation of his original Aplastic Anemia diagnosis, or he could remain a medical mystery.  Whatever it is, I suspect his bone marrow will look different than it did two months ago at the last biopsy, and I am very anxious for the results.

When we left SCCA in February, the doctors had us stop Ryan’s immunosuppressant medication, Prograf (tacrolimus).  They said that nothing in the many tests Ryan has had indicates his bone marrow failure is due to an immune process.  So, in theory, the medication should not have been helping him.  What’s interesting is that once his medication was stopped, his neutrophil/ANC (type of white blood cell) and platelet counts tanked.  He has been burning through platelets almost as fast as when he was first diagnosed, and his ANC has been hovering just over 200, which puts him at high risk for infection.

Here is the very strange part… He hasn’t needed a red blood transfusion since early February.  That is nearly three times as long as he normally goes in between transfusions.  His red blood cell count is still low, but his hematocrit has been bouncing up and down in the 23 to 25 range. (20-22 is his normal threshold for transfusions.) I am not sure what to make of this and will be curious to hear any new theories that the transplant doctors may have.

Even though his red cell count has remained somewhat stable, there are components of his blood tests that have been steadily increasing and are now outside the normal range.  For example, his MCV is now 100.8 (normal is 75-87) and his MCH is 36.3 (normal is 25-31). His RDW is now above normal as well. I have found information that indicates these elevated counts may be consistent with MDS.

If all of this sounds like a foreign language, it really is.  Before Ryan’s diagnosis, I never realized how complex our blood is.  Look at your lab results the next time you get a complete blood count (CBC) at your annual physical.  These numbers are all there, but most of us never have to figure out what the heck they mean.  I include these mind-bending acronyms and counts in my posts mainly to keep a record of them for myself.  I also know there are some fellow Aplastic Anemia families following my posts that, like me, have had a crash course in hematology/oncology and most likely know exactly what I am talking about.  I think all of us that have gone through this deserve honorary medical degrees.  I have definitely put in my time studying this topic!

Ryan at Clinic Yesterday Waiting for His Lab Results

A relative of one of the nurses comes in as each season/holiday changes and paints scenes on the glass doors of each patient room.

This Easter is Different

Every year, Ryan’s Grandparents host Easter.  The highlight for all of the kids is a garage filled with hay that is laden with treasures.  The kids pile in and dig for eggs full of candy, money, and toys.

Ryan can’t participate this year.  In fact, he isn’t even supposed to go in the garage tomorrow because there is a fungus in hay that can be very dangerous for immune compromised people.  I feel so bad for him.  We are constantly having to tell him “no.”  He can’t go to school, can’t see his friends right now, can’t go to birthday parties, can’t go out in public, isn’t supposed to dig in the dirt outside (same reason as the hay), can’t climb up in his fort (because a fall could cause him to hemorrhage), the list goes on.

He is disappointed and even asked if he could stay home tomorrow.  However, I am going to try to make the day fun for him.  I have wrapped up treasures in orange tissue paper (his favorite color) and I plan to stash them around Grandma and Grandpa’s house so that he can have a scavenger hunt indoors while the other kids are out in the hay.  Hopefully he will enjoy that.

The picture of him above is from last year’s hay pile – about 3 months before he was diagnosed.  I have noticed that last year’s Easter pictures are the last pictures we have before he started to look pale and get bruises and petechiae (red dots on the skin from low platelets).