Bone Marrow Aspiration

Not much new to report. On Wednesday Sebastian had the 1st of 5 bone marrow aspirations (post gene therapy) that will be required over the next 3 years. Everything went smoothly and he recovered quickly, with only minor irritability from the anesthesia. He was his usual self and in great spirits when the clowns visited him later that afternoon.

It'll take about two weeks before we have all of the results, but we did learn that one test showed some initial new cell engraftment, which the doctor said is good news at this early stage. Since Sebastian doesn't have many cells at this time, we will have to wait until he has the next aspiration in two months from now to gather more information and to learn if his engraftment is progressing incrementally which is the ideal scenario.

A few weeks ago, we were waiting for his immune system counts to go down and now we are waiting for them to rise. Specifically his neutrophils, because as long as he is neutropenic, we will not be able to leave the hospital. Sebastian's content here, but I'm looking forward to our family being back at the apartment together.

The weather here continues to heat up and we are told it will get much hotter. We continue to enjoy learning the language and interacting with the hospital staff. They are a wonderful group of people and it is a pleasure to get to know them and learn a bit about their lives and share a bit about ours.

The rest of the week has been uneventful - thankfully.

29 Days...

We have been in the box for 29 days now and hope to be over half way towards our goal. Sebastian is having diarrhea from the antibiotics he is on (which is normal), but other than that he is getting along fine. His immune system has continued to decline. This makes him more vulnerable to infections, so we are happy that he is doing well and that the doctors are not concerned at this point. We have our fingers crossed that in the next week his immune system counts will begin to rise. He should also be having a bone marrow aspiration test in the next week or two to see where he stands with the number of corrected ADA gene cells. Nervous is a good way to describe how we feel at this point. Soon the first marrow test will be completed and analyzed and we should have a basic idea what Sebastian's fate from the gene therapy procedure will be. Please keep Sebastian in your thoughts this week.

Last week was not a fun one. I woke up on Tuesday after a nap with a severe runny nose. Not something you want to deal with when your child has basically no immune system and needs your help. So I went to the hospital, put on a couple of masks, and called upon the doctor to get an opinion. She told me to go home and see what happens. After two days of my nose running like a faucet and no sleep I was thinking I needed to try something new. I called Lynette and asked if she had any allergy medicine. She had a couple of bottles of nasal spray (I will never again tease her for bringing miscellaneous meds when we travel). I took a few doses of it and magically a day later I was cleared by the doctor and back in the game. At first I thought I was just sick and exhausted from spending 16 hours a day in the box, but it was just good old fashioned allergies. Where we are staying, in the outskirts of Milano, there are a lot of trees and grass and my body must not be used to these species. I usually have no allergy problems at home, so it was a surprise to me that I would have allergies elsewhere. Lynette was a true champ and covered my shifts as my nose went hay wire — thank you. It was terrible to know that I could not lend a hand and was basically banished from the isolation area. I am happy to be back where I belong.

Have a great week!

Neutropenic

Happy Fathers Day!

We are thrilled to report that according to today's blood test, Sebastian is neutropenic. His neutrophils are 500, which is exactly what we were hoping for. This means that the chemo has done its job and has made room in his bone marrow for his new cells. The doctors estimated that it would take 10 - 14 days after re-infusion for him to become neutropenic, and today is day 14!

Aside from this great news, there's not much else to report. Life in the box is going smoothly. Once a week, two clowns come to visit Sebastian, which he really enjoys. They come in with their gowns, hats, and red noses over their masks. They do not go into Sebastian's area, just in the outer area of the room. They do silly things that make Sebastian laugh and laugh. It's really great to watch. What cool people to do this.

The European Soccer Cup is going on right now and there are typically two matches on per night between various countries. Soccer (calcio in Italian) is THE sport here — it's a fast-paced, fun to watch, and we are all enjoying the games. We don't normally watch sports at home, but when we're back, Chris wants to get the cable channel that will allow us to tune into the European matches.

The last few weeks have been stressful, but we continue to meet wonderful people here and we greatly appreciate the hospitality we've received in Italy. We have a lot of respect for the families who have done this before us, and we can only imagine the burden that Dr. Aiuti and his team place upon their shoulders when they take on a new patient.

We are thankful to those of you who have taken an interest in Sebastian's health and well being. We know that he is in the thoughts and prayers of people around the world and this knowledge helps us through each day.

Box Life

The first week in the box was very difficult. Thankfully, this second week has been much better. Now that Sebastian's off of the medication that gave him mood swings, and we have a bit of a routine, we are all adjusting to our temporary new way of life.

A typical day goes something like this:

It starts with my drive to the hospital. It's only about a 10 minute drive, with no stop signs or lights. Instead there are 8 round-abouts; these are common through-out Europe and possibly other parts of the world—they are not common in California. There's only one that's very busy, with 4 or 5 different intersecting roads and heavy morning traffic. I'm sometimes amazed that I make it here in one piece! Chris thinks they are very smart and wishes America would adopt this system because you never are waiting at a stop light. He loves driving in Italy and I am getting along just fine.

I arrive around 8:30 a.m. and Chris tells me how their night went. I change my shoes, put on my hair net and get my mask ready. The nurse brings in meds and takes vitals and I get Sebastian's breakfast ready. Food is a whole other matter, which I will talk about later. Everyday someone comes to clean Sebastian's room. He sits in the bed, while everything inside the box gets cleaned. When it's all done, we put his shoes on and he stands near the wall of clean air flow and waits for his bed to get cleaned and his sheets changed.

Bath-time follows, so that he doesn't go into his clean bed until he's been cleaned too. The nurse brings in warm, sterile water and I get everything prepared to enter the box (my gloves, his clothes and towel, and the wash cloths with soap in them). It's probably a good time to mention that last Sunday, I watched as a nurse shaved each beautiful lock of Sebastian's hair. We hadn't been able to wash his hair, because they didn't want him to have wet hair and he had too much for it to dry quickly. We all agreed that for hygiene reasons, it would be best to shave it. He wondered if he still looked like himself and he does. We are all used to his new look and actually like it. Chris tells him that he could use a bit of sun though.

One of the handful of pediatric doctors visits him each day to keep track of his condition and I'm pleased to announce that he's clinically doing well. Other than the nurses coming in to give meds and take vitals, we have the day to ourselves. I thought this was going to be a great bonding time for the two of us, but in reality, he wants to do his own thing - play his video game, watch a movie, color, etc. When he is out of the box school work will continue.

On Friday Dr. Aiuti came by with an update on some of Sebastian's blood work, which seems to be fine and in order for this stage of the procedure. He had a package from his daughters, a bunch of small cars, which Sebastian was very excited about — Grazie! We were able to meet his family very briefly in California when they were on holiday last summer and they are very, very nice. It would be great to see them again after things calm down a bit.

The food is pretty good, and we're getting Sebastian to eat, but it can be a struggle at times. There are limited choices for a picky eater (this would be the case at any hospital). Understandably, he cannot eat any fresh foods, everything needs to be well cooked for germ safety. He either eats rice and oil or pasta and oil and a bit of the vegetables. I've tried different things and discovered that he does like one of the cheeses he can have and will eat it on a roll. I just hope that he doesn't get bored with the same food.

Everyone here at the hospital has been very helpful and the care has been excellent. Our Italian vocabulary is growing daily and to fill in the blanks we have a dictionary to help us communicate with the nurses, although many of them speak English. They are also very helpful at teaching us words in Italian and in return we teach them words in English. This exchange is great and helpful to all.

At this point we are most grateful for the fact that Sebastian has not asked to come out of the box. As a matter of fact, he refers to this room as the hotel. We are very proud of him and tell him so all the time. He understands that this is what is keeping him healthy at this point.

I hope this paints a bit of a picture of what life has been like lately. Maybe this will explain to other families seeking gene therapy what goes on day to day at this stage, as well as letting our friends, families and the curious know what we're up to. Have a great week!