I have a lot of ground to cover. Its becoming more and more difficult to find the time to post though things are quiet, for the moment. I'll start on October 18th and work my way to today. A lot has happened. Spoiler alert, we serve a strong and mighty God who is working miracles in Riley's life. There is a lot to be thankful for in Riley's cancer battle.

Riley was in the hospital for chemotherapy starting Friday, October 18th, and running through Monday, October 21st. His blood counts were good so there was no hesitation to start the chemotherapy. He had also healed really well from the surgery; his scars are very small. Cycle 5 of chemotherapy required the use of the same drugs given to Riley in cycle 3. To recap, these are the two drugs, Cisplatin and Etoposide, that Riley had just before the radiation infusion (I-MIBG). Going in, we anticipated that this cycle was going to be rough. Cisplatin is known to be one of the worst for nausea and Riley had a reaction to the Etoposide previously where he started having trouble breathing. Riley was strong. God was stronger. Riley did vomit frequently though the drugs from a few days. After the 3rd cycle with Cisplatin, we drove to Philadelphia the day we got back from an I-MIBG consult; not one of my best decisions. This time we knew to play it safe. Riley did have a reaction to the Etoposide again. Despite giving him Benadryl ahead of time, he still lost his breath and got red in the face. They slowed down the rate at which he was receiving the drug from over the course of 2 hours to over the course of 4 hours and things were fine. Benadryl and slowing the rate of infusion of the Etoposide were the same things that were done in cycle 3. We recently talked to an allergist who does not believe that Riley had an allergic reaction as slowing down the rate and taking the Benadryl would not resolve the issue if it was a true allergic reaction. Instead the allergist believes that this is Riley's natural reaction to the medication and that it is not uncommon. It took Riley awhile to recover from cycle 5. He didn't go back to school until the following Thursday. He got one day of school in that week and then had to head back to the hospital for blood counts on Friday. While in the hospital for cycle 5, we got a chance to talk with some of the people in BMT (Bone Marrow Transplant) about the upcoming high-dose chemotherapy cycle that Riley would be receiving next in his treatment plan.

The month following cycle 5 was filled with weekly blood counts, tests to re-baseline the status of the disease and to determine if the treatment plan was working, and lots and lots of meetings to prepare us for transplant. With the exception of one blood count check 2 weeks after cycle 5 (platelets), Riley hasn't needed blood products. The terms transplant and BMT might be a little misleading in Riley's situation as he is having a stem cell transplant, not a bone marrow transplant, similar to what he had after the I-MIBG. These are the stem cells that were harvested from him in June. This time, however, things are going to be more intense given the state of his blood counts and the chemotherapy drugs he will be taking. The list of side effects is heavy. A lot of it stems from needing to control his fluids. The chemotherapy drugs for the high dose cycle, BuMel (Busulfan and Melphalan Hydrochloride), are going to put a strain on his body as they will aim to kill off the cancer and everything else as well. His blood cells are going to get to a point where they are considered 'leaky' meaning that they will be leaking water. At the same time, he will be getting IV fluids and likely not eating much. This means that the doctors and nurses will need to balance his weight. If he gains weight, it will be water weight and the increased water from the IV fluids and the 'leaky' blood cells will put strain on his heart, lungs, liver, kidneys, and just about everything else. If his weight drops then he is likely malnourished. He will be on steroids that will increase his blood sugar, an issue we have had since the beginning though one that has gotten better. He will be at risk for VOD (Veno-Occlusive disease), which is an uncommon though serious liver disease that can occur from high-dose chemotherapy. VOD can occur with many BMT treatments but Riley will be at increased risk because it is a more common, though still uncommon, side effect of Busulfan. Another side effect that is common for BMT patients and one that can be caused by Busulfan is mouth sores or mucositis. The mucositis is caused by both the chemotherapy as it is killing off the healthy cells that are designed to fight infection and fungus in the mouth and throat and by the stem cell transplant which can cause graft-versus-host disease (GVHD) which is where the newly transplanted cells attack his normal cells. If Riley develops mucositis, he will likely be put on morphine to help manage the pain.

The mouth sores, nausea, and lack of appetite lead to BMT patients being recommended for a NG-tube (naso-gastric, though the nose) normally or a G-tube (gastrostomy, to the stomach) often. Riley did not do great with the NG-tube during his I-MIBG treatment but it will be ideal for him to get one to ensure we can keep food on his stomach and so that he can continue to take his medication (some of the medication have to be taken PO, per os - by mouth, rather than being able to be administered through his IV). The NG-tube is typically inserted the day before the stem cell transplant. We will likely try it out though if he has any trouble with it, we will remove it. His primary oncology doctor thinks he will be okay without it though BMT is still recommending. We considered a G-tube for him as well but were told it was not necessary and would require another surgery.

During Riley's BMT visit there will be many changes to the care and precautions that are taken by the hospital. We met with the BMT nutritionist, the BMT social worker, the BMT child life specialist, the BMT clinical director, his BMT nurse practitioner, and his BMT doctor. The staffing they have for a unit of normally 6 rooms that can surge to 11 rooms says a lot. The nurses are only assigned 2 patients per shift and Riley will typically get the same nurse for multiple days and nights in a row. The team will perform rounds on him daily that are much more extensive than what we have experienced previously. The list of other changes is extensive:

• All visitors must have had a flu shot.
• No visitors that are sick.
• No visitors under the age of 10 (no Ethan and no Cooper, but Grayson can visit).
• No eating food that was not prepared within the last hour (no fast food and no take out). This will last for 60 days after his stem cell transplant.
• No drinking water that has set out for longer than an hour. Only bottled water.
• No going to school for 60 days after his stem cell transplant.
• No lettuce, mushrooms, sprouts, or berries (no issues here as Riley doesn't eat any of these).
• No walking on the floor without shoes on.
• No sharing of Riley's bathroom (there is a hall bathroom for parents and visitors though we can use his shower as long as it is cleaned after we use it and before he uses it).
• His room will be cleaned twice a day and they typically rotate rooms every 21 days.
• Everything will be measured (what goes and what goes out of Riley).
• Riley cannot leave the room, no exceptions (his teacher at the hospital, the art specialist, the music specialist, the child life specialist, and Spiderman, who Riley's has seen at the hospital on 3 different occasions, will all have to come to see him).
• All visitors, except Ashley and I, have to wear a mask.
• Items coming into the room must be cleaned in advance.

As I mentioned, during the workup to BMT, Riley underwent a bunch of tests. The only test that was conducted that was on the lower end of normal was his kidney function test. This puts him at greater risk of VOD. His heart function is normal as seen by his EKG and Echo. They also ran a bunch of tests to confirm that he was not sick and to see if he had certain illnesses in the past. It turns out he had mono at some point in his life though they don't know when exactly (could have been 3 months ago, could have been 3 years ago). This just means that they will ensure he is taking the right medications to prevent further complications from this during the treatment.

Prior to BMT, they also ran tests to see how well he was progressing. God is amazing. Riley's MIBG, the CT/MRI like scan that specializes in detecting traces of neuroblastoma as it looks for traces of where Iodine is picked up revealed NSD (no sign of disease). This means no cancerous tumors in his chest or abdomen and no signs of cancer on his bones. This is a great results and one that his doctors are very pleased with. It is also the ideal place for his body to be prior to the high risk chemotherapy in BMT. His CT scan was also completely negative. All body parts are normal and no tumors were recorded. Though it seems a little troubling that his scans are showing no signs of the disease and Riley is headed into the most challenging and detrimental to his normal body function phase, it is the reality of high-risk neuroblastoma. Given the state of the cancer at initial diagnosis, we need to ensure the cancer is completely killed off as there is a significant chance of it coming back. Further good news was his bone marrow biopsy results. They ran two stains on his bone marrow. The first looks specifically for neuroblastoma. It was negative. The second looks for abnormalities in the bone marrow. It showed a slight trace of something that could be neuroblastoma but it could also be newly created blood cells. I cannot express how much this result has lifted my spirits. God is performing miracles on Riley and we are beyond thankful for all who are caring for him. The treatment is working.

His high dose chemotherapy and stem cell transplant began in the BMT unit on Wednesday, November 20th. The first day was low key. The team ran some tests to confirm he wasn't sick, his port was accessed (he did amazing), and we got moved into his room. This is the room that Riley will be in for the next 4 to 6 weeks. During this time he will get chemotherapy and a stem cell transplant, his counts will plummet to 0, he will receive multiple blood transfusions for his hemoglobin (likely 2-3 times a week) and platelets (likely every other day), and we will wait for him to recover. During this time, he will be prone to infection since his ANC (white blood cell count) will be at 0. He will stay in the hospital until his ANC is over 500 for 3 days in a row, he is fever free for 48 hours, and he is able to go at least 3 days without needing blood products (red blood cells or platelets). The schedule for the high dose treatment is as follows:

Day -7 (November 20th, 7 days prior to the stem cell transplant): tests and check-in
Day -6: chemotherapy - Busulfan (starting dosage), blood tests
Day -5: chemotherapy - Busulfan (starting dosage)
Day -4: chemotherapy - Busulfan (adjusted dosage)
Day -3: chemotherapy - Busulfan (adjusted dosage)
Day -2: no chemotherapy, Pentamidine (antibiotic that Riley has a reaction to previously)
Day -1: chemotherapy - Melphalan Hydrochloride
Day 0 (November 27th): stem cell transplant
Day 4: likely drop to ANC of 0
Day 10: ideal beginning of ANC recovery
Day 21 (December 18th): best case scenario for going home
Day 60 (January 26th): back to school and back to eating whatever he wants

His first 2 doses of Busulfan were the same, but the dose yesterday and this morning were 50% greater in quantity. During the first dosage of Busulfan on Thursday, they took his blood at the end of the treatment, 15 minutes after the treatment was done, and then every hour for the next 5 hours. All of the blood was then shipped to Seattle to test whether the desired ratio of the drug to non-drug was achieved. In Riley's case it was not, so they increased the dosage by 50%.

He got sick yesterday (Saturday, November 23rd), vomited, during the chemotherapy but has been good since then. He woke up not hungry which likely contributed to him getting sick. He is taking lots of medication to counter some of the side effects and he took them on an empty stomach. For the meds, he is taking mostly pills. He does better swallowing pills than taking liquid medication which is crazy to me for a 5 year old. He is taking medication for the nausea (Zofran, Ativan, Benadryl, phenergan)  and a few other mediations to help him flush water appropriately. He is also taking Keppra to prevent seizures which is a less common side effect of Busulfan. We have some experience with Keppra as Grayson used to take it for his adolescent seizures.

Riley's spirits are high. Despite being restricted to the hospital room and mostly restricted to a bed, he still manages to smile most of the day. He has been catching up on ABC mouse, Roblox, Avengers video games, Mario Kart, and Christmas movies. He is eating okay thus far and has only gotten sick the one time. He has been taking his medication without complaint. He has gotten a chance to Facetime his brothers each day. Ashley has done a great job decorating his room with Christmas emojis, especially Christmas poop emojis, a Christmas tree, and lots and lots of wall art from friends and family.

We are extremely thankful for the team taking care of Riley and even more thankful to God for being exactly what we need, all the time. Lord, thank you for performing miracles on Riley.

Psalm 100:3-4 "Know that the Lord is God. It is he who made us, and we are his; we are his people, the sheep of his pasture. Enter his gates with thanksgiving and his courts with praise; give thanks to him and praise his name."