A Lot Of Images

Bubble Boy.

One of many images of Nonno where I would have a hard time coming up with a caption. This was on our way to San Ambroggio.

San Ambroggio church. This is one of the first Roman Catholic churches. Previously this religion was practiced in secret.

The Duomo. I can't describe this and to be honest not even images due it justice.

This image is of us after we had ice cream. Anything here giving it away?

Another one of many images my father took of the Duomo.

This was the last image I saw Nonno take before we spent the next half hour looking for him. Would you believe he was hiding in the Duomo?

The Gelateria. Parker absolutely loves all kinds of ice cream. Especially if it is coming from Nonno.

Lunch at a pizzeria in the Duomo. My wife ponders what kind of pizza she has. It is a formality because she always has a margharita with pomodorini on them.

Breakfast near Loretto. My father is trying to have every pizza in Milan before he leaves. I am not sure if he understands he will have to stay here more than a month to do that.

Nonno, behind us. Again.

His biggest beef with Italy. Graffiti.

Nonno said that there was no way he was going to guy a pastry from McDonald's.

A very happy little boy.

My wife did not believe that it was meant for cleaning feet. They showed her.

The ducks in the river by Cernusco. A couple were feeding them.

He was so excited to get on the ride.

Parker getting a little taken off around the eyes and ears while flipping through his favourite magazine.

Parker's face at one of its worst stages.

The pizza from the first night Nonna and Nonno arrived.

Fun Weekend

A couple of days have passed since we spent a day in the hospital with Parker. We decided to use this time doing something fun and exciting for everyone. So, with that in mind we made back to back day trips around Milano.

On Saturday, we decided that everyone would take a subway ride to Cernusco and walk and explore the area surrounding the station. My wife and I were a little familiar with the area but the five of us spent over five hours walking through the streets and paths taking in everything it had to offer.

First, we began by walking north past a river and walkway through a park to a little village populated with small stores along the roadside. The buildings were extremely colourful and the store fronts were very inviting. As Nonna and my wife made there way from one store to the next, Nonno, Parker and I walked around exploring the rest of the village. On our journey we found two things of interest. The first was a gelateria, and the second was a small roller coaster for children.

When I first saw the ride from a distance, I knew right away that Parker would absolutely love it. In Canada there would be no way that Parker would be allowed on the ride at 18 months old but here in Italy the rules are very different. We decided that Nonno would be the best person to accompany Parker on the roller coaster; so, after purchasing two tickets and sanitizing the car very thoroughly the two of them got into the last two seats at the back of the ride.

Everyone but me all had a plan to tell the man in charge of the ride to stop the roller coaster if Parker was not enjoying himself. Me, I knew my son and that stopping the ride would not be a problem. The whole time Parker laughed and smiled as the roller coaster went around the track again and again. There was nothing frightening for Parker and the little monkey is definitely going to love going to Canada's Wonderland when he is old enough.

I had also mentioned that we found a gelateria on our exploration. To be honest, it is very easy to find them. Next to a caffe there is probably not an establishment that is more abundant here in Italy. Parker has tasted gelato previously but he absolutely loved sharing Nonno's chocolate ice cream with him. One spoon for Parker and one for Nonno. First Parker, then Nonno.

Yesterday, we decided that Sunday would be a great day for everyone to go to the Duomo together. The subway should not be as busy and if we left early in the morning then the heat on the subway should be bearable for Parker if we needed to put his plastic shield down.

As always, we began our exploration at Loretto. A street similar to Yonge Street in Toronto but with some beautiful architecture. It is normally a 45 minute walk to the Duomo from here but we took our time as we did some extra site seeing with Nonna and Nonno.

We decided to give my father the camera which may or may not have been a good idea depending on how you look at it. Nonno is not the fastest walker on the best of days so giving him a camera so he can take snap shots of everything from an espresso maker in a store window to the gold statue on top of the Duomo slowed him down even more. One of the good thing about it was that Nonno grabbed over 175 images to add to our collection of over 2500 images we already have of our time in Milan.

We had a wonderful time yesterday, walking to the Duomo from Loretto, then walking to the Castle and then to a church several subway stops from there. In total, we spent eight and a half hours on our trip yesterday. You would think that an 18 month toddler would complain a little bit during that time having to spend that much time in his stroller, but he didn't. Between his trips of being carried up and down the stairs in his stroller or the ice cream he shared with Nonno yesterday Parker was very happy.

When we got home, Parker was absolutely exhausted by the time bedtime came around. You know that “tired” you get when you fall asleep in a particular position and you wake up in the same position the next day? That was Parker both nights.

Wednesday is Parker's next trip to the San Raffaelle, for which I believe is only for blood work. Tomorrow, there should be an article containing only a plethora of images from the past few days. My wife will give me a hard time if I don't do it, so again I shall humour her.

Busy Bees**

I have to say that having Nonna and Nonno here has made life a lot easier for us. Very much like it did when Nana and Gaga were here.

It has been an extremely busy week with all of the trips to the hospital we have done so far. Add to that that my wife and I have been feeling a little under the weather and are lacking our normal amounts energy has made things difficult for us to do everything we would normally do.

On Monday, Parker went to the hospital for a series of blood tests and an ultra sound of his organs. Tuesday brought more blood tests and a red blood cell transfusion to help decrease the number of toxic metabolites in Parker's blood. Wednesday, again some more blood to check for antibodies prior to beginning his first of three IgG transfusions. Today, Parker had his second transfusion of IgG and tomorrow will be his third. We are hoping that the IgG is enough to combat the antibodies in his blood causing his neutrophils to die off. If there are any other antibodies that we are unsure of yet, we hope that they too will be removed by the IgG (immunoglobulins – Gamma).

Parker's face has been clearing up very nicely over the past week. The size of the spots have yet to decrease but the deep red colour has faded dramatically. Since changing his blood pressure medication and putting a special cream on his face we have seen quite a bit of an improvement.

The last few nights Parker has been sleeping in Nonna and Nonno's room. He sleeps through the night and for that reason he is a great roommate. It has allowed my wife and I to sleep a little longer in the morning since my parents take him for a walk if he wakes up earlier then they do.

I think the little monkey has been sleeping better because all of the walk and fresh air he has been getting lately. I thought it was hard keeping up with all the walks my wife wants to go on but my mother is impossible to keep up with.

I have to say that is also nice having her here because when I don't feel like cooking she takes over. Between my mother and I everyone has definitely been eating well over here.

I am not sure when we will get some more results about Parker as our doctor is on holidays and he normally gives us any results that we need, in addition to answering all of our questions. However, when I do have some results, I will make sure to let everyone know about them.

**I have placed a note hear about today's title because I thought it warranted one. The original title was "Bust Week" but after telling my wife the title she began to laugh hysterically as she watched "The Young and the Restless". I asked her what was wrong and she replied as she normally does when she finds something very funny. Very quickly she blurted out, "You should have called it Busy Bees". I normally discard my wife's strange sense of humour but I thought I would humour her this one time.

Information

I have been meaning to publish a new article the past week but for different reasons I have been unable to. The Internet had been down for several days which kept me from writing initially. Secondly, I have been busy with the arrival of my parents here in Italy. With so much happening lately I thought it would be easiest to write large article with subtitles to keep it organised. That said, I shall begin with the first and probably most important topic, what is going on with Parker and gene therapy.

The Meeting
On Friday we had a meeting with Dr. Aiuti along with the doctor overseeing him. Several tests had been conducted earlier in the week including tests to check his blood for the different types of cells, the amount of toxic metabolites in his blood along with the another bone marrow aspiration.

Parker's most recent blood tests have shown extremely low levels of engrafted white blood cells compared to the amount he had when he was first transplanted. In addition, his toxic metabolites were extremely high, most likely due to his particular mutation. That said, his metabolites did decreases significantly one week ago. The most recent test showed that although they did decrease slightly again it was not dearly as dramatic as previously and Parker still has more metabolites than any other patient at this point in the study.

We spent the days prior to the meeting quite worried that Parker's bone marrow aspiration was going to show something very similar to what was present in his peripheral blood. Parker did not have very many engrafted cells left at all. Why did we think this? Pretty much because everyone thought this was a very good possibility. We heard during the meeting on Friday that they were positively surprised that this was not the case. After seeing Parker's actual numbers my wife and I were happy to see that they had actually increased slightly. One type of the cell had actually increased dramatically. At this time we also found out that although Parker did not lose his engraftment, he is at the lower level of engrafted cells compared to most patients in the study. That said, they do believe that Parker can be cured with the low number of engrafted cells if they do increase in number over the next months.

Remember that the blood cells are produced in the bone marrow so if there are no cells at the source to produce blood cells then there will never be any cells in the peripheral blood. So, that means there must be something that is causing the cells to not be present in the blood. Tests revealed that Parker has anti-bodies to neutrophils and as a result he is attacking the neutrophils (all of them – ones containing the ADA gene and ones that do not) and as a result he does not have nearly as many ADA producing neutrophils as most people.

In hopes to remove the antibodies Parker will undergo a high level dosage of IVIG (gamma globulins). They do not believe that Parker has a lot of antibodies since his neutrophils count is still relatively good (> 1000). They will test this week to confirm that there are no other antibodies in Parkers blood getting in the way of the cells in Parker's blood.

They also feel that is necessary to help Parker remove some of the toxic metabolites so they do not cause any damage to his organs in the long term. They will do this by giving him a red blood cell transfusion. This will allow the metabolites to decrease at a steady rate in his blood as the transfused cells contain working ADA genes.

Of course this is not exactly what we had hoped when we came to Italy but it is pretty much the best news we could have gotten from the meeting. Although it is not guaranteed that the gene therapy will still work they are still optimistic. Parker will have another bone marrow aspiration in one month to see what is happening to his engrafted cells.

What are he hoping for now? That Parker has no other antibodies present. That the IVIG does its job and is sufficient at killing the antibodies. That after this, Parker begins to produce significant numbers of neutrophils and that he can keep his toxic metabolites under control. And finally, that the bone marrow aspiration he will have in a month will show an even higher number of engrafted cells compared to this week.

We have always said that Italy was the place we needed to come to find Parker a cure for his disease. We continue to believe this. We have a very special team of doctors here and we trust them to do what they feel needs to be done so that our dream will become reality. Dr. Aiuti has put an enormous amount of time and effort into helping our son and we are extremely appreciative of him.

Parker's Face
That last time that I wrote about Parker's face we were under the impression that they were caused by mosquito bites that had become infected. The result was for Parker to begin Augmentine in hopes to help fight the infection and remove the blemishes from his face.

Unfortunately, after the next few days we did not see any improvement in his face. The red circles actually got bigger and brighter. As a result, we took Parker to the dermatologist on Monday morning. He felt that there was a good possibility that it was caused my a reaction to one of his medicines.

Over a week and a half ago I had thought that may had been the case but never mentioned it to anyone. See, Parker started his blood pressure medication (Norvasc) at relatively the same time he go the little red dots on his face. As the days passed and Parker took more Norvasc at an increased dosage the red circles increased in colour and diameter.

On Monday, they decided for Parker to stop taking Norvasc and to begin a different blood pressure medication, Triatec. Tuesday was the first day he did not take Norvasc and now, after some peeling the redness is starting to go away and the size of the circles are starting to decrease.

We were told that if this is a reaction to Norvasc that there should not be any scarring and it should take a good two weeks for the redness to go away.

Nonna and Nonno
I have to say that the best part of this week has been having my parents here to help us deal with all of the stress surrounding us.

My parents arrived in Milan on Wednesday evening at the Milan Central Train Station. They flew into Rome originally and after a five our train ride I met up with them at Milan Central.

Nonna and Nonno have been a burst of energy for the three of us. To be honest, Parker did not see them too much prior to Milan but he has not stopped laughing for four days.

Of course, everyone goes for there daily walks around Milano Due and no one seems to love it more than Parker and his Nonna. My wife and I do as much as we can, all the time but 18 months of taking every possible precaution has drained us of most of our energy. Yes, we always took Parker out for his daily long walks but Nonna has now added two more walks for Parker. One when she gets up in the morning and another one which she drags Nonno for after dinner. Parker absolutely loves it.

There is so much that I want to write about in this section but to be honest I will just say this. I have never felt so happy to have my parents with me. I did not expect them to come to Italy to help us but I am so glad that they did.

Il Pinocchio, la zanza e un infectione

I decided to write two articles in as many days because I have not been that faithful lately when it comes to updating the blog. Parker is currently down for his nap and my wife is about to go outside to catch a few rays during the next couple of hours. That gives me time to sit down at the computer to write for a bit.

I had mentioned a little while ago that Parker's stroller had broken in several places from over-use here in Italy. My parent's bought it for us before Parker was born. It is a Peg Perego which is an excellent stroller and happens to be Italian made. So, a week this past Friday I took a subway to a baby store that is also a Peg Perego representative. They were able to take the stroller and get it repaired at the factory about an hour away from Milan. To be honest, this was probably the only thing we have ever bought that we actually sent the warranty card in. The result, they repaired the stroller by replacing two main parts and it only cost me the subway ride to the store.

I picked up the stroller Friday afternoon, one week after handing it in to them. While I was there I noticed that they had alot of nice things for babies and toddlers. First, I picked up Parker a set of large Lego type blocks. He absolutely loves playing with them. Secondly, I noticed something that my Nonna had brought me back from Italy over 30 years ago. I saw a wooden Pinocchio. It was almost exactly the same as the one I had when I was a child. I had to get one for the little monkey. I was very surprised how much Parker loved the wooden boy. Parker carries him around everywhere. I actually feel sorry for his monkey because he has taken a backseat to Pinocchio since yesterday.

Now, I am sure you have noticed from the images that Parker received a few mosquito (zanza) bites last week. We are pretty sure that it happened inside the residence while he was sleeping. We did not think too much of it and felt assured that they would go away by themselves. The doctors had seen them when they were small and said to wait a few days and we should see them start to get better. That said, yesterday we woke up and noticed how bad they looked and decided to take him back to the San Raffaella to have them looked at.

Apparently, they have become infected and without an immune system Parker needs some help to rid his skin of the staph bacteria that is most likely the cause of it. Parker restarted Augmentine yesterday and we hope to see an improvement by tomorrow. The last thing we want is for the infection to get any worse than it is. Parker has such a beautiful face that we want to rid him of the penny size welts that he currently has.

Tomorrow we will go to the hospital for them to look at his infection and for them to take some blood. Let's hope that both the infection and the blood test yield some positive news.

I am sorry for not mentioning this sooner but with everything that has been going on I have been preoccupied. We are very thankful that Auntie Natalie spent last week with us here in Milan. It was a very stressful week and having her here made things a little easier for us. Parker really enjoyed playing with her and is sending a million kisses her way! It was also great for my wife to have her best friend here.

More Tests Next Week

I figured that everyone who is not directly related to Parker are probably wondering what has been going on the past few days. Emotions have continued to be all over the place the past few days as results from tests come in.

I had mentioned that Parker's metabolites had decreased significantly recently. On Friday, we found out that Parker's red blood cells that had increased in the amount of ADA activity they had produced had once again decreased. This supported the idea that the therapy may not be working. Since the doctors are very curious to know what is going on with Parker they have requested that Parker have another metabolite test this coming Monday and then have a bone marrow aspirate on Wednesday to see what is happening in his marrow.

Something is happening we just do not know what. We have come to grips that there is a very good chance that the therapy has not worked. Something has kept it from working. You can only imagine the stress that we have been under as we tried to figure out how to go on from here.

Later, on Friday afternoon, my wife spoke with the lead doctor and asked if there was any possibility that if it is confirmed that the therapy did not work if they would be able to repeat it. He replied that they would do there best to find out why the therapy did not work, and then if possible they would do something to counter the reason during a second attempt so it would be successful.

The team of doctors here at TIGET are in my opinion the best in the world when it comes to ADA deficient SCID. We believe that in the end they will be successful in finding a way for Parker to live a normal life. Yes, if they have to repeat it we will be in Italy for quite some time but that is something we have no problem doing if Parker can do everything else a little boy can do.

Please continue to keep Parker in your thoughts and prayers. We hope that Monday's and Wednesday's test results bring some encouraging news.

Disappointment, Encouragement and Uncertainty

Before I begin, I have to admit that I have never felt so much stress in the span of one week in my life. Everyone that reads the blog regularly knows that we have been waiting for some results the past few days in hopes that Parker has a chance of making the gene therapy work.

I had mentioned previously that Parker's metabolites had been increasing at a very high rate. The metabolites give his new cells an advantage over the old cells but for some reason we were not seeing the effects of the advantage. We were waiting for Parker's blood test results to arrive on Tuesday in hopes that his toxic metabolites either decreased or stayed relatively the same. What we got instead was our doctor telling us that he was not very optimistic anymore, that the toxic metabolites not only increased but they doubled and Parker had lost most of his engraftment when they looked in the peripheral blood. He was not ready at the point to say that Parker was the only patient who the gene therapy did not work for but he did say that he had the least number of engrafted cells and the highest number of toxic metabolites compared to any other patient so far.

Everyone was dumbfounded. No one could have predicted that Parker, the best patient to enter the study was going to be the only patient that would not display relative success with gene therapy. My wife cried. Me? I just could not understand what was happening.

The only good thing that came from our conversation was that for the first time the red blood cells had shown an increase in ADA activity. When Parker had his bone marrow aspiration he was given a red blood cell transfusion. Of course these are not his cells and display ADA activity. But as the days passed these cells died and the ADA activity from his red blood cells constantly decreased. What was surprising was the fact the Tuesday's results showed a 50% increase in ADA activity from these cells. The only way this could be would be for Parker's bone marrow to have produced them. The team was not convinced that this was the case and that it was not just an error in the test so they decided to repeat the test and put a rush on the results.

So, the past two days we have been miserable. We have been trying to figure out what we would do if the gene therapy did indeed fail for Parker. Yes, we were hoping for some miracle but being realistic we did not expect anything to get better when the results arrived later this week. No one here in Italy was very optimistic.

Last night, the phone rang close to nine in the evening. It was our doctor. He said that he did not want to wait to give us some good news. Parker's metabolites had decreased 30% in the span of one week. He also said that they now feel they can predict what is going to happen with his metabolites. They should slowly decrease over the next weeks. Does this mean that it is working? Does this mean that we are out of the woods? We don't know. We will probably meet with our doctor tomorrow and get a better understanding of what is going on. We do not really know what is happening in Parker's bone marrow. Did his cells proliferate and increase or were they all overstressed and die off. We don't know. We just hope that the metabolites do continue to decrease and his white blood cells begin to increase steadily.

We just want this to work so that Parker, his mother and father can live a normal life eventually.

Thank you to everyone who has been praying for Parker the past while we know that every prayer helps.

Nonna and Nonno Coming!

I received some very good news yesterday unrelated to Parker's treatment. My parents (Parker's Nonna and Nonno) have decided to join us here in Italy for a month. I was so surprised when they told me and I really did not know what to say. I have been so stressed lately wondering what is going on with Parker's numbers and metabolites that to be honest I really did not say too much when I found out. I have just been extremely overwhelmed with emotions the past day knowing that my family is coming here to help.

I know that Parker is really going to enjoy his time with his Nonna and Nonno and I can't wait to see his face when he does. My parents are flying in to Rome and will take the Eurorail to Milan where I will eventually meet up with them and bring them back to Milano 2.

We hope that Parker's metabolite results are ready tomorrow and that they show a decrease in number. I apologise that this is not a long article but I still have a lot on my mind with Parker and I find it difficult to write when I am under a great deal of stress. That said, I did want everyone to know how happy I was that my parents were going to be coming to Milan.

When Does Life Get Easy?

I know that it has been a while since the last time that I wrote but I find it very difficult to write when things are not going as smoothly as we like. That said, I am a little less stressed at the moment so I thought it would be a good time to write.

The latest bit of information that we have obtained is that Parker has the most toxic metabolites after transplant compared to the other 11 patients they have had here in the study. What does this mean? No one really knows yet. They toxic metabolites are removed by ADA and for that to happen there needs to be ADA in his system. It can be expressed either by his cells (which we want) or by giving to the body external using PEG-ADA. During the time in which the toxic metabolites are high his new cells have an advantage over the old cells and as a result, they can proliferate and increase in number. This is what we want. The new cells will produce white blood cells that go out into his blood stream, produce ADA and remove the toxic metabolites. For this reason, we remain optimistic. However, Parker's white blood cells did not increase yesterday. They decreased by 100. This is neither considered an increase or a decrease so it does not give us too much information. On Monday, we hope to obtain results from one of Parker's blood tests to see if the toxic metabolites have once again increased in numbers, stabilized or decreased. We are praying that they do not increase again.

As I had said, we do not know why his metabolites are so high. It could be due to the fact that Parker is taking longer to engraft, or maybe it is his particular mutation in his gene that causes him to produce metabolites at a higher rate than most children. It could also mean that the gene therapy did not work. We do not know. We just know that we want his white blood cells to begin to increase significantly to help remove the metabolites and for the result of the metabolite test to come back with a positive result early next week.

Parker wore a blood pressure monitor for 24 hours yesterday to get a better idea of his blood pressure. Apparently it has been quite high over the past weeks and they want to control it. Unfortunately, the monitor did not get very accurate readings over the 24 hours so the results were invalid. That said, Parker will begin taking medication to control his blood pressure while the toxic metabolites are causing his pressure to be elevated. When the metabolites so decrease (I am being optimistic) then his pressure should also decrease and he will not longer need therapy to control his pressure.

My wife's best friend arrived in Italy on Tuesday and we are very happy to have her here. Parker loves playing with her and it is nice to have an extra pair of hands around here to help. Thanks for all your help and support Auntie Natalie.

We did speak with Dr. Aiuti today and his still seemed optimistic with Parker and his progress. We just need to wait for more data to come in so they can have a better picture of what is going on with Parker. The fact that Parker is doing extremely well clinically definitely gives everyone more time before anything must be done to intervene in the natural process of the gene therapy.

As soon as we know more information I promise to update everyone. Parker gives blood on Tuesday of next week and we will be able to get a better idea of his white blood cells (including neutrophils) then. So, we hope to know more then.

Walking with Mom and Dad

Again, I have decided to upload a series of images for today's article. There is nothing really new around here except that now that Parker is walking or running everywhere we are going crazy chasing him. Everyone told us that once he starts walking and has a different perspective that we would spend our time making sure that he does not get into everything. Well, if you check out the newest video you will see what I mean. In the past few days I have added three videos including the two that I just uploaded. Check them out.

Mom and Parker posing for the camera. Parker knows that he has the best looking mom in the whole world.

Parker and daddy love watching soccer together at the pitch. Yesterday it was the over 60 men playing.

Now, that we are out of isolation we go for walks twice a day again. We always make sure to bring the camera with us.

Mom and Parker getting their story time in. Parker loves story time. Check out the video to see why Parker was laughing so hard after story time.

Parker has the coolest set of wheels. No wonder he is smiling.