Thursday, January 30, 2014

Seven Months Post Diagnosis: Ryan is just a few days away from being admitted for a bone marrow transplant, but things have changed…

I haven’t updated Ryan’s blog for awhile because I have been posting more frequent updates to his Facebook Page (  However, I know there are some people that are not on Facebook (or are not aware of Ryan’s page), so I will try to update here more frequently.  To catch up, I am copying the last several Facebook posts below (most recent first):

January 29, 2014
SCCA Day 7 – Not Aplastic Anemia???

The results of Ryan’s double bone marrow biopsy came back right before our appointments at SCCA started today.

We were in the exam room waiting for the transplant team. I was browsing Facebook and read that a little girl named Gabby lost her battle with Aplastic Anemia today. Bone Marrow Failure diseases in young children are very rare, so as far as numbers, there are not many families out there in the world going through this. However, we have slowly been finding each other through social media and we share in both victories and tragedies. I think we all take it personally when one of our AA families is suffering. I was already teary-eyed by the beautiful post written by Gabby’s mom about her final moments, when Ryan’s doctor entered the room today and delivered another blow to our family’s battle.

Ryan’s bone marrow cellularity is normal and Aplastic Anemia is not his current primary diagnosis. What??? It sounds like great news, right? It’s not. Because of his continued low blood counts and dependency on transfusions, it just means there is something else going on. Even more scary – they don’t know what that is!

One theory is that Ryan’s condition has progressed to a Myelodysplastic syndrome (MDS). MDS is not a single disease but a group of diseases that affect blood-cell formation. About 80 to 85 percent of people with MDS have more cells in their marrow than healthy people do (hypercellular marrow). But these cells do not live long enough to make it out of the marrow into the bloodstream, or they are not in circulation long before they die. As a result, people with MDS have low levels of one or more types of blood cells in their bloodstream (cytopenia). MDS used to be called “Smoldering Leukemia” or “Pre-Leukemia”, because in a third of patients, it progresses into a fast-growing cancer of the bone marrow cells called acute myeloid leukemia (AML). It is usually a disease of older adults, not children. When children are diagnosed with it, it is normally due to something genetic.

None of the bone marrow biopsies done so far have shown any signs of MDS, including the one done this week. In fact, the bone marrow does not show signs of dysplasia (cells of abnormal shape or size), which is normally one of the factors used to diagnose MDS. (Although, after I got home, I remembered a few of Ryan’s past blood tests noting teardrop shaped red blood cells and cells of differing sizes. I plan to mention this to the team tomorrow.)

The team is consulting with Dr. Akiko Shimamura, one of the leading bone marrow failure experts in the world, for potential answers. They have decided to do some more in-depth testing for MDS. The tests should take about 2 weeks, which means that Ryan’s transplant is delayed.

The transplant team believes that, regardless of the cause of Ryan’s illness, it is still clear that he needs a bone marrow transplant. If it is MDS, there is a high risk of it going on to a deadly form of leukemia without a transplant. I asked “What if the additional tests come back negative for MDS?” The team stated that it would be a really unsatisfying situation in which they do not know exactly what is going on, but they would still think it is somewhere on the MDS spectrum and they would treat it as MDS. This means that Ryan would no longer be treated with the lower dose pre-conditioning regimen that is used for Aplastic Anemia patients. He would be treated with much stronger chemotherapy and may even require high-dose total body irradiation instead of the low dose.

In the meantime, I also learned today that Ryan’s donor has asked to have his marrow harvested earlier because he couldn’t be available on the specific date they had originally requested. He wanted to move the transplant up a few days, but now the team is going to have to ask him to delay it by two weeks. I hope the donor will still be available.

I managed not to cry in front of Ryan. For the first time, I almost did, but I managed to hold it together. He was watching a movie with headphones on and had no idea about the conversation going on around him. I have yet to cry at any of his appointments. I have teared up, but I always remind myself that it probably would scare him to see me so worried. Halfway through the drive home, I glanced in the rearview mirror and he was asleep. I burst into tears.

January 28, 2014
SCCA Day 6 – Radiology Oncologist, Platelets, & Dentist

Today was kind of a bummer. The day started at the University of Washington Medical Center for a consultation with Radiation Oncology. This is where Ryan will receive total body irradiation (TBI). The doctor went over all of the possible side effects, which include, among other things, an increased risk of developing tumors, mouth sores, skin sensitivity, dehydration and infertility. I was already aware of the risk of infertility, but I thought it was a 50/50 chance. Maybe that was wishful thinking on my part. The doctor stated that we should assume there is a 100% chance of it. That was hard to hear. Boys post-puberty have options to bank sperm. Adult patients are able to understand the risks and make decisions about treatment. At age 5, Ryan doesn't understand and he doesn't have a choice. It’s another thing this illness is taking away from him, and it makes me angry and sad. Also, something the doctor said about possible gland and cheek pain upset Ryan and he jumped off the exam table and buried his face against my shoulder for the rest of the appointment.

That appointment set the tone for the rest of the day. Ryan needed platelets, so we returned to SCCA. Since his porta cath has been accessed so much lately, the dressing (big plastic tape) they put over it has created a large rash on his chest. It hasn’t bothered him until today, when they used cleaner on his chest before accessing it. It must have burned! Ryan’s face got bright red and he was clearly upset and on the verge of tears. Then he told us it was hurting. The nurse was very kind and worked to reschedule some lab work that was supposed to occur tomorrow, so that he doesn’t have to have his port accessed again until Friday. Hopefully his skin will heal up quickly.

We then went to Seattle Children’s Hospital for a dental consult, but Ryan was ready to be done for the day. He was not a happy guy and initially refused to sit in the dental chair. He just went to the dentist a few weeks ago, so all they did today was look at his X-rays, confirm there are no cavities, and talk to us about mouth care during transplant.

The icing on the cake today was when we left the University of Washington and headed back to SCCA. I stopped to get gas, which put me coming from the opposite direction of what I am used to. I was still pre-occupied thinking about the radiation side effects and accidentally got on the 520 floating bridge over Lake Washington, going AWAY from Seattle. I had to go all the way across the bridge, exit, turn around and go back over the bridge heading toward Seattle. This only cost us about 5-10 minutes total, but they recently made this a toll bridge! That little mistake cost me $8.00!

January 27, 2014
SCCA Days 3 & 4 – A Day at Home, Followed by PT, EKG & Echocardiogram

Since Ryan’s bone marrow biopsy was scheduled for last Thursday, but was postponed until Monday because of the cough that his virus caused, we stayed home and I caught up on reading the sea of paperwork I have been handed the last few days. There is a 3-ring binder that is an inch thick with nothing but authorizations that we need to read and sign. There are also a couple of binders full of information regarding the transplant process, infection control, medication, nutrition, graft verses host disease, etc.

Friday, Ryan met with a Physical Therapist to assess where he is currently with strength, balance and coordination with fine motor skills. The PT will follow him over the next year to make sure he doesn't lose any of his current abilities.

He then went for an EKG and echocardiogram of his heart. He was ready to be done for the day by the time the echocardiogram appointment rolled around (and so was I)! The technician informed us that it would take 60 minutes, if Ryan didn't move, and a lot longer if he couldn't sit still. I was worried that we would be there for three hours with me having to remind him every 5 minutes to keep still. Instead, he was such a good sport. He asked me to lay in the bed with him and we watched The Incredibles while the technician did her thing. I only had to remind him to be still twice and it was during times when the technician got the ultrasound wand to close to his porta cath, which he did not like (I can’t blame him). Ryan had a ton of questions about the ultrasound equipment, where the pictures go, and if the doctor could see the pictures while she was taking them. She said she normally doesn’t get such detailed questions about the equipment from someone his age and asked if anyone at home is really into computers. Yep, that would be his Dad, who is in the IT business.

January 21, 2014
SCCA Day 1 – Ryan’s “Arrival”

Ryan had his “arrival” at Seattle Cancer Care Alliance (SCCA) today. From now until the first week of February, when he gets admitted to the hospital, he will have appointments with SCCA just about every day. He will have various physical exams, continued transfusions, new medications to start, an EKG, a Lung CT scan, another bone marrow biopsy, a dental exam and regular blood draws/labs to check for all sorts of things. We will also have conferences with the transplant team, a consultation with a radiation oncologist, and meetings with social workers, nutritionists, and physical therapists. There will also be several classes to learn about things such as food safety for transplant patients and preparing our home for Ryan’s return after transplant.

We will be bouncing between the SCCA facility, Seattle Children’s Hospital, and the University of Washington Medical Center for all of this. We received a printed schedule with each appointment, the time, and where we need to be. I felt like it was the first day of high school or college and I was racing between classes trying to figure out where I needed to be!

Holy cow – they took so much blood today for testing! There were 14 full tubes, plus a huge syringe that they filled to the top with 20 mls of blood! As his reward at the end, the tech let Ryan squirt him in the face with two full saline syringes. Ryan loved that! We managed to squeeze 20 minutes in for lunch today. Tomorrow, Ryan will be getting a red blood transfusion first thing in the morning and it will be an even longer day!

December 31, 2013

As 2013 winds down, I have been reflecting on Ryan’s diagnosis this year of life-threatening bone marrow failure and the whirlwind of the last 6 months. While this has probably been the most difficult year of our lives, it certainly could have been much worse without all of the support from friends, family, co-workers and, in some cases, complete strangers. We had two months of meals prepared by co-workers, gifts for Ryan, donations that helped us pay our 2013 health insurance deductible, phone calls, texts, cards, and kind words said in passing. When Ryan was at his weakest after chemotherapy and was getting tired walking, we even had a big kid stroller show up on our doorstep. I have continued to work (mostly) full time and am very thankful for the patience my coworkers have had with me as I come and go in between appointments, call into meetings, work remotely, and try to juggle my work responsibilities with my mom responsibilities. Thank you everyone for helping make 2013 a little less horrible for us!

December 20, 2013
The Transplant is a Go

Ryan's doctor is giving the green light today to proceed with the bone marrow transplant. He definitely feels it is time. Although this is a scary next step, I am glad to have an action plan and am filled with hope for this potential cure. We don't yet have an exact date, but it could be as early as mid-January. In the meantime, Ryan burned through all of his transfused platelets last week and is getting another transfusion today.

December 13, 2013
Quick Update

Ryan may receive a bone marrow transplant as early as January and we have some great news about a possible donor.

I am a little behind on updating Ryan’s blog, but wanted to pass along that we have met with the doctors at Seattle Children’s Hospital, and also had our transplant consultation at Seattle Cancer Care Alliance. All of the doctors are recommending proceeding with transplant, subject to a couple of genetic tests that are still outstanding and another bone marrow biopsy. His last biopsy showed some increased cellularity in his bone marrow, but those results just don’t match his continued regular dependence on blood and platelet transfusions. The doctors seem to be of the opinion that the last biopsy likely just hit one of the few “good” spots in his bone marrow (bone marrow can be “patchy” in patients with Aplastic Anemia).

The most desirable unrelated donor for a transplant would have the same 10 HLA tissue types as Ryan. This is called a 10/10 match. Sometimes people have to go to transplant with a 9/10 match or even a “half match” from a parent. However, the best chance of survival, with the lowest rate of complications, generally comes from a 10/10 match.

I have heard of so many patients not having a single viable match in the registry, or only having one or two matches, then having to wait on pins and needles to find out if those individuals will truly donate. This has been a major worry for me over the past few months and, even though Ryan was HLA typed in September, I couldn't seem to get the specific results from anyone. The doctors just kept saying that they didn't think they would have any problem finding a donor. Finally, this week, we were sitting in the transplant consultation and the doctor had a thick file on Ryan and turned to a particular page. She asked “Has anyone told you how many potential donors Ryan has?” “No, I have been trying to find that out…” She proceeded to tell us that three of Ryan’s tissue types are very common in the general population and that he has multiple potential donors. I looked at the page that she was turned to and I saw a computer-generated list that had about 20 entries on it with a hand-written note next to one of the lines that said “10/10”. I asked “Are all of those 10/10 matches?” She confirmed they are all 10/10 matches! What I didn't realize at the time was that the rest of the thick file behind that page was a continuation of the list. In speaking with the donor search coordinator the next day, I learned that Ryan has 3110 10/10 matches in the registry!!!! Yes, that is four digits!!!!

While I am grateful beyond words that we have so many options for donors, my heart aches for the families still searching for just one. Please, please, please register at if you haven’t already!!!

This week, the donor search coordinators started calling some of the potential donors within the continental U.S. to ask them if they are willing to donate, and to set up further testing on them. It is so humbling to know that complete strangers are being called up right now to save Ryan’s life.

November 22, 2013
A Second Bone Marrow Biopsy

Ryan’s bone marrow biopsy yesterday was fairly uneventful. He was such a trooper. Our appointment started at 8:00AM. He couldn’t eat breakfast due to the anesthesia, and his procedure wasn’t until almost 11:00. I went prepared to hear him say “I’m hungry” a million times throughout the morning while we were having labs done and meeting with the doctors, but he didn’t say it once. Then, as he was waking up after the biopsy, he looked cold, so I asked him if he wanted his blanket. His immediate response: “I want some food!”

While we were there, we met again with one of the doctors/researchers that we saw last week. He had discussed Ryan’s case with the other researcher and Ryan’s Hematologist/Oncologist. All three doctors agree that, unless the bone marrow biopsy shows signs of recovery (or Ryan goes into remission all of the sudden), the right thing to do is to move toward transplant. We should have initial biopsy results in about a week and our consultation with the Seattle Cancer Care Alliance transplant team is still set for December 9th.

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