Sebastian's Space

School Days

2009-08-26T11:07:00.001-07:00

We have had good intentions of keeping this blog updated, however, it’s much harder to manage now that we’re back home. We certainly had a lot more free time while we were in Italy to write updates. Also, there hasn’t been much to report until now.

First, let me start with this very exciting news – Sebastian started his first day of 1st grade today! My husband and I walked him to school this morning and waited for him to go into his classroom with his new classmates. Sebastian commented on all of the kids and said that he had never seen so many. I must admit that I got a bit emotional.

Now I’ll back up to what’s happened since our last update. We were scheduled to return to Italy in June for Sebastian’s 1-year post gene therapy follow up visit. We decided not to go, because we felt that the combination of the H1N1 flu virus and Sebastian’s low immune system numbers would make it too risky for him to travel. The doctors were understanding and supportive and the Italian team worked with the UCSF team so that the tests that would have been performed in Italy could be done here. These were the same tests that were done in Italy prior to the gene therapy treatment, and except for the bone marrow biopsy, were not invasive or painful for Sebastian. The biopsy was done on June 30 and a nurse (our friend, Miriam) from Italy came to San Francisco to bring the bone marrow sample back to Italy. Although it would have been nice to go back to Italy and see the doctors and nurses again, we were relieved that we did not have to risk Sebastian’s health to travel there.

We knew it would take about one month before we learned the results from the bone marrow biopsy, so we waited and hoped for the best. When we received the email with the results, we were elated to learn that the gene therapy was working. His body is producing gene corrected cells! We had a phone conversation with Dr. Aiuti to discuss the results and the possibility of Sebastian starting school in a classroom setting, and he said that we should give it a try. We also had a meeting with the Immunologists at UCSF and they agreed that Sebastian should be allowed to start school. His immune system is still compromised and we have to take precautions, but it’s time to start letting him have a “normal” life.

We were able to take a mini-vacation – our first family vacation with no medical ties. We took a 4-day road trip down to Southern California and took Sebastian to Legoland. It was a nice trip for all of us.

Sebastian will continue to get his IVIG treatment at UCSF to give him the antibodies to help protect him from infections. We will take it a day at a time and hope that he will be able to continue in the classroom setting. This has all happened very quickly and we are grateful that Sebastian can attend the school near our home. It’s a darling little school and the staff have been wonderful and accommodating. It helps to know that he will be in good hands, with caring people.

This new freedom is both exciting and scary. Realistically we won’t update the blog for another 6 months, and we hope to report more good news at that time. We would like to thank all of you for your support to make this happen. All of your kind thoughts and prayers for Sebastian have been greatly appreciated.

Finally an Update from Italy

2009-03-01T13:52:00.001-08:00

Sebastian continues to do well clinically and has been off Peg-ADA since his therapy. He is taught by a teacher who comes to the house several times a week and is doing well in his lessons. Generally he is happy and really enjoys being outside and exercising on his bike and scooter.

After many long months of waiting for test results we finally have some word as to what is going on with Sebastian's immune system. We held off updating the blog until we received these results. It appears his immune system is slowly getting stronger and his t-cell counts are improving. Unfortunately, his numbers are not increasing as well as the younger patients in the study. We hope that in the coming months his "numbers" continue to increase. If they stay at the current levels we will have to entertain other options.

We thank you for keeping Sebastian in your thoughts.

Happy To Be Home

2008-11-05T20:35:00.000-08:00

It's hard to believe that we've been home for 3 weeks now. We are all happy to be home and we are all adjusting (yet again) to our new routine. Sebastian did well on our flight home. He wore his mask the entire time, only lifting it up briefly when he needed to eat and drink. The flight was full and there were people coughing, so we are thrilled that Sebastian made it home safely and continues to do clinically well.

We were welcomed home to absolutely beautiful weather and got to enjoy a nice walk along the coast and walks and bike rides in our area. Sebastian's been re-connecting with all of his toys. It's like they are all new again. He's also been enjoying visits with his grandparents. He wants all of their attention and doesn't want them to visit with us. We can understand that.

He's already had 2 appointments at UCSF. It's great to see the doctors and nurses who have taken such good care of him over the years. He feels very comfortable there. His numbers are still low and he is still in a vulnerable place, which means we will continue to wait and hope for those numbers to go up. And, of course we'll continue to be germaphobes!

He was so excited to be home for Halloween and he got to go to a few houses for trick-or-treating. At one house, someone was dressed up like a gorilla and Sebastian thought that was the greatest thing. He just laughed and laughed and asked if it was a real gorilla and if he could go back to that house. We're happy that he was able to enjoy himself.

We miss the people we got to know in Italy and hearing their beautiful language. We've found some music videos on YouTube of Italian music that we listened to while we were there, which is something special for us to share together. We also tune in over the internet to listen to Italian radio on the weekends. We will always hold a special place in our hearts for each and every one of the people who took care of Sebastian while we were there.

Returning Home

2008-10-13T06:39:00.001-07:00

We are returning home this week! Ideally, Dr. Aiuti would like us to stay for another 3 months to monitor Sebastian, as the next 3 months are a crucial time; however, we want to get on a plane before flu season gets underway and Lynette's leave of absence is over. The decision to come home was not an easy one to make. We weighed all of the pros and cons and feel that going home now would be best for us. His medications and the use of a mask will help protect him.

After meeting with Dr. Aiuti and getting an assessment of Sebastian's health, it seems things are headed in the right direction; more time is necessary to see if T-cell production will get to better levels in the coming months. Sebastian will continue to be monitored at UCSF, and they will work closely with Dr. Aiuti. We are lucky to have a great team of doctors and nurses at home too.

It's exciting to know that we will be home in a couple of days, although we know that we'll go through a period of culture shock, and it'll take a little time to adjust, as we've experienced from past travels. With Sebastian's situation, we have learned to be cautious and take every care to protect his health. Upon our return, we will need to be even more careful than we've had to be in the past, while we wait for his immune system to improve. He will not be able to attend school or have much interaction with other people. We are in contact with his school and a teacher will be coming to our house to provide Sebastian with his 1st grade education.

Thanks to all for your help, positive thoughts and interest in Sebastian. We would also like to thank Dr. Aiuti and all the scientists, doctors and nurses who have worked hard to help Sebastian. We look forward to being back in touch with family, friends and co-workers soon. Lynette works for Williams-Sonoma and we would like to extend a special thanks to the company and all of the great people there who have been extremely supportive over the years.

No New News

2008-09-21T05:09:00.001-07:00

We wish we had some exciting news to report, but not much has changed since our last update. Sebastian had his 90-day post transplant bone marrow aspiration on Sept. 10. He was put to sleep for about 20 minutes, and as usual he was upset when he woke up. Luckily, he didn't get sick and he recovered quickly. Within an hour after he awoke, we were able to leave the hospital. We were glad that he didn't experience any pain, which was evident by his running and jumping all the way to the car. We should learn the results of that test in another week.

His numbers went up a little, but then they went back down again. They'll take blood again next week—we hope that his numbers will be back up. With his numbers so low, we are staying in the apartment a lot more. We take walks now and then, but not as often as before. Plus the weather is changing, and it's getting quite a bit cooler. After being in the "box", it's not so bad having to stay in the apartment. At least Sebastian moves around (a lot). His new thing is skipping, so he skips around between the 2 rooms—our poor downstairs neighbor!

Parents always think their kids say funny things, and we're no different. As this relates to the picture we're posting, I thought I'd share something we found comical. When I asked Sebastian to get dressed so we could take a picture for the blog, he insisted on wearing his Italian soccer jersey, because he said, "I want to look like a perfect Italian man."

We will update the blog again once we have all the results of the marrow test and a clue as to what the next steps will be and when we might be able to go back home.

Turbulence

2008-09-01T08:09:00.001-07:00

We know that this treatment is experimental, and we know from our Canadian friends, who went through this last year, that it can be a very scary ride. Knowing this and actually experiencing it are two very different things. For a period of 3 weeks, Sebastian's numbers were going up, and then to our surprise they went down. Not just a little bit down, but significantly down. His neutrophils went from 1,800 one week down to 500 the following week. We were not just surprised by this; we were shocked. Dr. Aiuti told us there could be fluctuation, because Sebastian's bone marrow has been traumatized, but since his numbers had been continually increasing, we just hoped they would keep going up.

For the past week his neutrophils have been stable at 600. Still low, but the doctors have assured us that they are not worried. We've maintained our position that if the doctors aren't worried, we won't be worried either (easier said than done—but we are trying). We are thankful that Sebastian has been able to stay at the apartment, and has not had to go back into the hospital. As a precaution, he has started a new antibiotic and does need to wear his mask outside again.

Always trying to look on the bright side - Sebastian's skin reactions have all cleared up, he continues to do clinically well, and he has a ton of energy. There's a park near the hospital that we've gone to a couple of times now, and he'll walk there for an hour. There's a river, a lot of trees, grassy areas, and many walking paths to explore. It's a beautiful, peaceful place.

Above is a piece of art made by arranging Sebastian's hands. Throughout Sebastian's life his hands and feet have been sporadically used to make art pieces. This one was made last week after noticing how much his hands have grown since we have been here.

Healthy Progress

2008-08-17T06:34:00.001-07:00

August is the time of year that most Italians take their vacations, and a good percentage head toward the sea or lakes where it is cooler. Many of the shops in town close for the entire month. The supermarkets are still open and there are still people about, but the change is definitely noticeable. Even the hotel near the hospital, where we go to update the blog, is closed. Unimaginable for our North American brains—but very cool.

Last week we had to move to a different apartment. We actually moved back into the same apartment that we stayed in last year. It took us a couple of days to get situated here, but we are settled in now. The two apartments are basically the same, but one good thing about this one is that there are screens on the windows, so we can get some fresh air without worrying about mosquitos. The view faces another direction and Sebastian does miss watching the construction of the building across the street, but he can still see it when we go outside for a walk.

His numbers are continuing to go up, with the exception of his lymphocytes, which we know will take a long time to increase. We are very happy with how well he is responding to the gene therapy treatment up to this point. The lesions that he had in the hospital have more or less disappeared, however, we believe that he was bit by some sort of insect (maybe a spider) and he had another reaction on his face. The doctors gave us some cream to put on, and it seems to be helping. We still don't take him to parks or into stores, but he no longer needs to wear a mask when we go out, except when we go to the hospital.

In the picture, Sebastian is holding an Italian flag that he made out of Lego's. He's standing in front of one of the windows in our apartment. All of the windows have outside window coverings that can be raised up completely, closed partially to let some light through (as shown in the picture) or closed completely to block out all light. It seems that every home has these window coverings, or some version of window coverings. We think they are for temperature control, privacy, and safety. We prefer to keep them open during the day, so that we can look outside and get some natural light, however, most of our neighbors keep them closed the majority of the time.

Sebastian is enjoying doing his school workbooks. Lately, after we wake up, he has a few lessons prepared for us. These are similar to what he has learned in the previous few days. He says, "I have a game for you to play." Then he gets out a pen for each of us and tells us what we need to do and how it should be done. He often changes the rules of the "game" mid-way and makes sure to correct us all the time. Basically, this is his opportunity to tell us what to do and to be the boss. We think it's really cute. With all the rules Sebastian has had to live by we believe this is very healthy for him. On that note, when we go out for a walk he always says, "Can we go where I want to go?" Of course.

Sebastian's Typical Day

2008-08-03T06:21:00.001-07:00

Sebastian's doing well and enjoying his time outside of the hospital. He usually wakes early and entertains himself by drawing or coloring. He'll even work on the "fun" pages of his workbooks, like dot-to-dots or mazes. After breakfast he gets to play or watch a movie until lunch. We usually spend about 2 hours on school workbooks and then he can play until dinner. He didn't get to play much with Lego's while he was in the hospital, because it was difficult to keep them clean, but he is making up for lost time and using his imagination to build a lot of different things. He's very creative and visual (like his Dad). Except when we go to the hospital, he rarely plays his video game.

It's hot and humid during the day, so we usually wait until after dinner to take a walk. Sebastian wears a mask whenever he goes outside. He seems to be comfortable wearing his mask and never complains or questions having to wear it. This past week, during one of our walks, we ran into a family from Brazil whose son is also in the study. We met them once last year and it was nice to see them again. Their son is a real cutie. He underwent gene therapy already and they will be heading back home at the end of next week. We wish them the best. After our walk, Sebastian has his shower and then we watch a funny Italian show. It's called La Botola (The Trapdoor) and people do some kind of performance (sing, dance, juggle, etc.) and the audience votes on the best performer. The losers fall through a trapdoor that lands them in a pool. We all get a good laugh from it.

He's still thin, but he'll gain his weight back now that he's eating much better. When we're out for our walks, he runs ahead and isn't having any pain in his legs. I'm surprised that he's bounced back so quickly, especially since we could barely get him to eat or get out of the bed while he was in the hospital. Another nice surprise, after being waited on hand and foot for 53 days, is his enthusiasm in learning to be more independent. Without being asked, he's putting his dirty clothes in his laundry bag, clearing his dishes, and even trying to make his bed. We are proud of him, and even more importantly, he's proud of himself.

Sebastian has to go to the hospital once a week for blood work and a clinical visit. His lesions are practically gone - his ears are taking the longest to heal. We were told that his immune system numbers will go up and down for a period of time, which has been the case. His neutraphil counts were down to 800 two weeks ago and were back up to 1,000 last week. For protection, he takes daily prophylactic medications and has IVIG every 3 weeks (the antibody infusion that he's had to have all of his life). He will continue with these precautions for some time, however, I am thrilled (as is Sebastian) that he no longer needs his Adagen shots. Those were the shots that gave him the ADA gene that he was missing. For 6 years I've given him shots twice a week and it's hard to believe that his body is now producing this on its own!

We will plan to update the blog every two weeks, since there's not much to report on a weekly basis. Our hope is that things will continue to go in the positive direction that they are going now and that we won't have any unexpected surprises ahead. Thanks for keeping Sebastian in your thoughts and prayers and please check back in a couple of weeks.

Out of the Box

2008-07-18T00:54:00.001-07:00

On Wednesday, the 53rd day in the box, Sebastian was discharged. The picture above is of him leaving the box, with one foot in and one out. The first thing he did was give Chris a big hug and then he gave me a big hug. It was strange to be able to touch him again without having the gown, cap, mask and gloves on. Almost scary, but great. As we were leaving the isolation area many of the nurses who cared for Sebastian came out to say bye to him. It was a cheerful send-off, but very quick, because we were anxious to get him to the apartment.

As you can imagine, he was very excited to leave the hospital. When we were walking to the car, he was saying, "look at all the people, look at the planes, look at the clouds", as if he was seeing them for the first time. Now, it's as if he was never in the hospital. All the energy that he wasn't expending is certainly back and coming out. It's unbelievable how well he's doing. We're already starting to set a new routine. We are easing him back into school, which was a bit of a struggle at first. His video game play has been reduced to no more than a hour or so a day. He has fallen in love with building Lego's again.

We are able to interact much more now that surgical preparation is no longer necessary to be close to one another, and he's catching up on hugs and cuddle time. Now we need to work on building his strength and getting his weight up. He is able to walk outside with a mask on and he talks all the time about taking walks. He needs to stay away from people and no parks are allowed, but he is enjoying short jaunts. We will try to go a little further each day. He's been eating great, and couldn't wait to eat some of Chris's garlic bread.

Sebastian's immune system will hopefully increase in strength over time. He is by no means completely cured yet, but he is surely one step closer and we are so appreciative of Dr. Auiti for his brilliance. We are thankful to him and the other doctors for putting up with all our questions.

Thank you all for your positive thoughts and help in getting us to this point. We are very grateful!

52 Days...

2008-07-15T00:57:00.000-07:00

Good News: Things are really looking up and it's very likely that we will be taking Sebastian back to the apartment this week! On Monday his neutraphils were up to 1,300, and if tomorrow's blood test results are still up he'll be discharged on Thursday. We are very excited about this possibility. He will still be vulnerable to infections and we will basically need to keep him in the apartment, but at least we'll all be together.

Two dermatologists visited Sebastian last week and believe that the lesions are a reaction to a medication. He had been given a new medication to stop the growth of gallstones that were discovered when an ultrasound was done a few weeks ago to check his liver. They've stopped giving him this medication and his lesions seem to be healing. It will take some time for it to clear up. They took a sample of one the lesions to rule out a fungal infection and we should have the results later today. Neither of the doctors think it is a fungal infection and we hope they are correct.

Sebastian is excited that he will be able to leave soon. He has been a real trooper about staying in the box. When we imagined what it would be like in here, we thought that the hardest part would be just keeping Sebastian inside. We couldn't have been more wrong — we are very proud of him.

47 Days of Isolation

2008-07-10T00:31:00.000-07:00

Sorry for the delayed entry, we were waiting for some additional information before posting. The last week was a bit discouraging for us. We had expected to be in the hospital for 45 days, but it's going to be longer. It could be a few more days, a week, or more. It appears that Sebastian's immune system movements are going to be as hard to predict as what level any given stock market will close at next week. The words sporadic, complex and multifaceted come to mind. Just like a stock market, there are many variables that react with one another for advancement or decline.

Sebastian's counts weren't going up as anticipated, so last Wednesday he was given a growth stimulant (which is not uncommon) to help his bone marrow production along. We were told that this would bring his immune system "numbers" up within 6 hours after receiving the shot and then they would proceed downward after 48 hours. Ideally, the numbers wouldn't go too far down and his bone marrow would remember what it needed to do and continue to produce white blood cells. He responded well and his numbers initially went up a lot as anticipated. This was very encouraging because if Sebastian were to get an infection it proves that his immune system would try and fight it. This also shows that the therapy appears to be starting to work, which is very exciting. He currently has 600 neutrophils, which is a lot more than before the stimulant shot. He needs at least 800 before they'll consider discharging him—he is getting pretty close. The results from his blood work on Monday and Wednesday were the same. We hope this means that he's reached a new baseline now from the growth stimulant and his marrow will continue to proliferate on its own in an upward trend. If not another growth stimulant injection will most likely be prescribed.

As you can see in the picture, Sebastian has some lesions on his face. He actually has more spots/bumps on other parts of his body too. A dermatologist will visit him later today. It is now thought to be either a reaction to a food or a medicine. It started out as 2 small bumps on his cheek and over the past week, it proceeded to get worse. It looks like the first 2 initial spots might be starting to dry up. We hope the dermatologist will have an answer as to the cause, and also what needs to be done to get rid of these. We also hope it's nothing serious. It's been distressing to see these lesions on his normally flawless skin.

Although we have written about what appear to be minor problems above, there are a lot of positive things going on - his platelets are going up, his hemoglobin is in the normal range, and aside from the lesions, he continues to be in a good clinical state. As you can see from reading above our bodies are complex and ever changing. This procedure will hopefully save Sebastian's life—he had no other options, as other drugs were starting to become less effective. We are thankful for people like Dr. Aiuti who are experts in this complex field.

Thank you for your interest in Sebastian and your positive thoughts. Have a good weekend.

Bone Marrow Aspiration

2008-06-29T00:10:00.000-07:00

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...

2008-06-22T00:16:00.000-07:00

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

2008-06-14T00:30:00.001-07:00

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

2008-06-07T00:38:00.000-07:00

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!

Infusion!

2008-05-31T08:27:00.001-07:00

Last week was a very busy one. Sebastian had his bone marrow extracted from his hips. He then had chemotherapy taken through an IV (called Busulfan). He reacted fine to the chemo drug but was a little out of it and easily agitated due to some anti-seizure meds (which is common). The dose was reduced a bit and all was well. The reason he is given chemo is so his immune system, now propped up by Adagen, will be compromised so the gene therapy administration does not have to compete against any naturally occurring immune system reactions to fight the vector (the virus that delivers the corrected gene). While this was going on, the talented scientists were taking his bone marrow, preparing the cells and incorporating the vector for injection. This was all orchestrated like clockwork. The staff of doctors, nurses, etc. have all been wonderful. They are an inspiration to me—there are many ill children around this part of the hospital and they are doing amazing work! I hope Sebastian will go into the medical field, it is the most noble of professions in my opinion.

On Friday at 4:30PM Sebastian received back the corrected gene. Dr. Aiuti said the cell counts, etc. were of good quantity and was optimistic. It was a day of excitement, relief and stress for us. We are excited and relieved that he has had his gene therapy treatment administered. Next, as I think I mentioned in an earlier post, is a thirty day waiting period in order to discern if the treatment is working. This is where the stress comes in. The mind tends to play scenario games in quiet hours thinking of all the variables that could go both right and wrong. Only time will tell.

The fireman drill: When Sebastian has to go pee (he has a potty seat in the box) we have to put on full surgical regalia (hair net, mask, robe and gloves) in order to lend a hand. This is not ideal by any means, but necessary. I joke to everyone it is like being called to go put out a fire the way a fireman must adorn all their protective gear and get on the truck—and if you are not quick there is going to be a big mess to clean. Our bodies, when functioning correctly, are truly amazing machines and this multi-step safety procedure reminds me every time I go into his area.

A few notes of thanks: Two of Lynette's co-workers gave Sebastian a few games for his PSP device. One of the games, after trying to appease him with several others, is a hit! It is a Lego meets Star Wars game in which there are miscellaneous tasks and one can run around in different "worlds". This bit of escapism has kept him entertained for hours when coloring, stories, etc. is of no interest. Thank you! Secondly, we met a Swiss family who has undergone the same procedure in Milano. The boy's grandma gave Sebastian a toffee that he took a liking to, she then gave him the rest of the box. Last week as we entered isolation a package came from them. Inside was a lovely note and beautiful drawing from their son, two boxes of toffees, and a guardian angel ornament. Thank you—Danke! Lastly, when Sebastian was undergoing his first bone marrow extraction procedure our Canadian friends gave Sebastian a cool soccer ball of the Milano team and brought us all some pastries. This was wonderful and much appreciated. We look forward to playing with the ball soon. No matter where you go there are great people in this world!

That's about it for now. Thank you all for your help in getting us here, as well as your thoughts and prayers. It is greatly appreciated. When Lynette and I are going through some of the lower points, we think of all the gestures of kindness.

Isolation Preparation

2008-05-24T05:06:00.000-07:00

After another week of multiple tests, we are preparing to go into isolation tomorrow. We reviewed the rules that we must follow during this period and we visited the room we'll be staying in. Sebastian will be limited to a very small area inside of a bigger room. We can go in his area as long as we wear a gown and mask. Only one parent can be there at a time, so one of us will stay during the day and the other will stay at night. Our understanding is that the first week will be the toughest, with 3 days of chemo treatment and his bone marrow harvest on Monday. By Friday he will have the corrected gene re-infused, which is the big day we've been waiting for! 30 days following re-infusion, he'll have another bone marrow aspiration, which will give us the first indication if the gene therapy has worked. It's hard to imagine what the upcoming weeks have in store for us. We will just have to take each day as it comes and do our best to keep Sebastian entertained, and most importantly, to keep him healthy. Please understand if we do not update blog during this period of isolation.

Tests & Play

2008-05-18T05:12:00.000-07:00

This week Sebastian has had many miscellaneous tests. We hope that the next family that has to go through the SCID-ADA roller coaster ride will benefit from some of the data points that he is able to contribute. Our little guy was able to have some fun play time with his Canadian buddy, and now that he has gone back home, he will surely be missed.

By next weekend Sebastian will be in what they call the "box" and now that he is no longer receiving Adagen shots we will need to be more cautious with him, e.g., having him wear a mask at the hospital, keeping him out of stores, no park play. I never thought that we would say we look forward to spending 40 days or so in "isolation", but I think we are as ready as we will ever be and look forward to getting past this upcoming step in a few weeks.

We are all adjusting well to our life in Italy and Sebastian hasn't shown any signs of feeling homesick. When we aren't at the hospital he enjoys playing with his legos and video game. He's even doing school work, which he'll get a break from in isolation.

Port Placement

2008-05-11T04:59:00.000-07:00

Happy Mothers Day!

Sebastian's port has been surgically placed. It is a small device that sits on his right side in his upper chest. This device enables the medical staff to insert IV's, etc. for up to seven days. This will be beneficial for delivering the gene therapy as well as allowing easier, more predictable access to his major veins.

The procedure itself went well and the hospital followed up with an x-ray and determined that the placement was good. After the procedure, he had a rough afternoon and evening, because the anesthesia made him sick, but he was better by that night and was discharged the next day. A day later the port was flushed out with saline and Sebastian said that he felt less pain than with a traditional IV, especially when the needle was removed.

Aside from spending a couple of days in the hospital, we have been able to meet up with some of the other families that have successfully undergone gene therapy. Dr. Aiuti took a picture of three of the children together as we were chatting in the hall way. What a sense of accomplishment he must feel knowing that two of those children are walking around without the need of Adagen and are on their way to pursuing normal lives. We can only hope and pray at this point that Sebastian will be so lucky. I value the Doctor's abilities, as well as the rest of the staff, beyond words. We understand how lucky we are to be here.

The weather in Milano at this time of year is wonderful. It is really nice to see people (especially the children) out walking, riding bikes or gathering in their local squares. I love the sense of history of downtown Milano, the food and the people. Both Lynette and myself are learning more and more Italian every day. I enjoy the language and hope to teach Sebastian as well. It would be nice for my grandfather, who is no longer with us, to know that I will be able to speak the language and so will future generations. I hope to have a proper conversation with his sister (my Great Aunt) when I return home.

Next week Sebastian will have some more tests and will stop receiving his Adagen shots. We are all very excited at the possibility of this being his last dose.

Glad to be back in Italy

2008-05-05T07:57:00.000-07:00

We arrived in Italy safely and have settled in nicely. We flew Lufthansa (great airline) from San Francisco to Frankfurt, Germany and then to Milano. Considering the length of the trip, it was uneventful, and Sebastian did extremely well. Most importantly, we all stayed healthy, which seems like a miracle given the number of people coughing on the planes. We are staying in the same village and same apartment complex as last year, but we're in a different building and apartment. Famed architect Mario Botta (designer of the SFMOMA) is building two beautiful office towers across the street from us and Sebastian is enjoying watching the workers and the cranes during the day. Although we have been studying Italian, everyone speaks very quickly and it's still difficult for us to understand. Even with the language barrier, it's been an easier transition, since we are now familiar with things here. It feels like our home away from home. With the 9 hour time difference, we all have some jet lag. It'll take about a week before our bodies are completely adjusted. Sebastian will go into the hospital tomorrow for his first procedure. He will have a central line surgically placed on Wednesday, which will be used in the future to access his central vein. This will stay in for 1 - 2 years. We'll spend two nights at the hospital, and depending on how he does, we'll come back to the apartment on Thursday or Friday. We're a bit nervous, but it will give us an idea of what to expect during his time in isolation when he has the gene therapy treatment. We will plan to post another update in a week and will let you know how the surgery went. As always, thanks for sending positive thoughts our way.

Golfing "Fore" Parker

2008-04-30T16:18:00.000-07:00

Last year when we were in Milan, we met a Canadian family whose son has the same condition as Sebastian. They'll be in Italy for a short time for their son's annual follow up and we look forward to seeing them again. They are having a golf tournament to raise funds for their son. Anyone who has had an ill family member knows how costly it can be. If you reside close by Ontario Canada or are in a position to sponsor a hole please read below:

Date: Sunday June 1, 2008 with a 1pm shot gun start
Where: Four Seasons Golf Club
Price: $125 which includes 18 holes of Golf
(scramble format, 4 players per team only)
Dinner only: $55.00 per person

For more information, to buy or sell tickets, help with this event or sponsor a hole, please e-mail: golfingforparker@gmail.com

More information can also be seen on Parker's Blog

Six.

2008-04-06T19:30:00.000-07:00

Today Sebastian turned six years old. We all had a nice day. In the back of my mind I was thinking this is all possible because of talented scientists and doctors who have dedicated their life's work to understanding how our bodies function and coming up with cures for very complex ailments. Thank you—Grazie.

There is a beautiful, two year old Canadian girl with the same condition as Sebastian, who we have been following for some time. Take a look at the video her mom made.

Ritorno!

2008-03-28T17:18:00.000-07:00

After much anticipation we have received word that we will be going to Italy at the beginning of May for Sebastian's treatment. We are very excited to be returning and Sebastian looks forward to seeing his friends. He won't need to repeat the tests that he did last year and the treatment will begin shortly after our arrival.

Since the last blog entry Sebastian has remained pretty much in isolation, and he has stayed healthy. He enjoys his home schooling and is learning all the things a normal five year old would. He is actually now doing math and workbooks at the first grade level and we are very proud of him. His favorite activities are family bike rides and a rigorous play session at a local park, unfortunately when we visit the park we have to make sure other kids are not around.

His mental acuteness and attention to detail is quite amazing and he is very social with the people he gets to see (some family, neighbors, and UCSF staff). We were very concerned that he would not know how to react around people he has never met, but he is quite the little conversationalist.

We are very excited to see everyone at the Hospital San Raffaele again. We feel very privileged to be able to have this opportunity and are very grateful. We will keep everyone posted when we arrive in Italy as to the next steps in Sebastian's treatment. Your thoughts and prayers are greatly appreciated.

Happy New Year!

2008-01-01T20:12:00.000-08:00

We hope everyone enjoyed the holidays and we wish everyone the best for 2008. We don’t have any new news from Italy, but we will update the blog as soon as we do, which we expect this month. Since Sebastian cannot visit Santa at the mall or events that other children go to, Santa made a special visit to Sebastian’s Grandma and Grandpa’s house (of course, he had his suit cleaned first). Thanks Santa! Despite the fact that Sebastian’s immune system is slowly declining, he continues to remain “healthy”. We are thankful for that.

Still Home

2007-10-22T19:17:00.000-07:00

Yes, it's true, we are still home. The most recent batch of medicine didn't pass quality control, which postpones our return trip until next year. At first we were disappointed to learn this news, but we are focusing on the positive. It'll be nice to be home for the holidays. It's also a relief that we won't be putting Sebastian on a plane and then having him spending time in and out of the hospital during the flu season. We expect to find out in February the results of the current batch and hope to return to Italy in March, 2008. In the meantime, our mission will be to keep Sebastian healthy.