Wednesday, May 31, 2006

Chapter One Coming to a Close

My apologies for not writing yesterday. My wife and I were celebrating. Our battle against this disease is a long and draining one but yesterday we got some news to make it a little easier. Parker's cell marker test came back and as a result of the numbers we have been given the okay to go home on Friday.

As a result of being on the ADAGEN for the past month, Parker's B-cells and NK-cells are at a normal level. Although he is still quite low in the T-cell numbers they did increase from 39 to 49 in regards to the CD4+ and 15 to 16 for his CD8+. We always knew that these would take the longest to increase and they still have quite a bit to go but this is something they can do while we are at home. Home is where we will be for the next four and half months as we await the trial in Italy.

To say they that we are happy that we finally get to take our little boy home is an understatement. My wife danced in the room with Parker for over two hours after she found out the news.

I thought to myself yesterday, what was it that I was really looking forward to doing with Parker when we got home? I am looking forward to teaching Parker how to roll over by showing him myself as we do tummy time together.

When I asked my wife the same question, she said that she most looks forward to being able to snuggle him without wearing protective gear. She wants to feel his skin and touch his hair without having to wear sterile green gloves.

We have a lot to do in the next couple of days to get the house ready for his return, like cleaning the carpet so we can do tummy time.

Monday, May 29, 2006

Big Day Tomorrow!

Parker has a big day ahead of him tomorrow as he is scheduled for his next enzyme injection. He used to get the injections on Mondays and Thursdays, but they have recently been changed to Tuesday and Fridays. This will mean one less visit to Sick Kid's for us as since clinic is on on Friday's. Clinic is when Parker would get his CBC done to check his blood counts each week.

In addition to getting his enzyme injection, Parker will be giving 10 ccs of blood so that the flow cytometry test may be repeated. Recall that this test will check to see how many of the different types of T-cells he has. The last test revealed that Parker was normal for his B-cells and NK-cells, but was still below the norm for CD4+ and CD8+ cells. Being on PEG will never restore these to the normal range but we do want to see a trend illustrating that they are increasing. I am unsure if we are only looking for this particular trend or if we need them to reach a specific number before we may go home for the summer, as we await a journey to Italy. The last test revealed that Parker's CD4+ cells had reached 39 from 9 when we were admitted and his CD8+ cells had reached 15 from 1 when we were admitted. The same blood sample will also be used to perform his CBC (complete blood count).

Lastly, Parker will be reintroduced to Septra as a precaution to PCP pneumonia. He was on Septra (orally) but was taken off of it when he became neutropenic. He then began Pentamidine (IV) once every two weeks in its place, and as a result, Parker is no longer neutropenic. Now that we are closer to going home, they would like to try the Septra again since it is much better in acting as a deterant to PCP. Although we are anxious about the switch back, it will be monitored very closely and stopped if his neutrophil counts decrease. The fact that Parker will not need an IV is also reassuring.

I did an interview with the Corriere Canadese, the Canadian version of the Italian newspaper. I am not sure when the article will come out, but it will be in Italian. No problem here. I am working on my Italian each and every day.

Parker is doing very well. He has taken to his regularly concentrated formula and has continued to grow. I had the little guy on his tummy for a good five minutes before I picked him up and gave him a big hug. The occupational therapist also came by today. She was very pleased with his accomplishments. Parker is very close to rolling over on his own (back to tummy) and must now work on pushing up with his arms when he is on his tummy. I know it is a lot of work, but a few push ups never hurt anyone!

Sunday, May 28, 2006

Our New Friends

Parker and I have a new set of friends and they are called the Backyardigans. A friend from school whose daughter loves the Backyardigans bought Parker a DVD with four epsiodes on it. Parker and daddy cannot get enough of them. I think it is meant for children a little older than Parker but the bright colours and catchy music is enough to keep our eyes glued to the television. If you have children and have never heard of the Backyardigans, check them out on Nick, Jr.

The past couple of days, mommy and daddy have tried to do a little work around the house in anticipation of going home some time in June. Yesterday, Parker's Nonna was kind enough to stay most of the day and hang out with Parker while we cleaned up around the house. Today, the nurses on 8B were kind enough to play with our little man until we got were just after noon. Again, we tried to get as much done as we could at the house today as it will make it easier when do get the nod to take him home. Parker sure was happy to see us today and boy were we glad to see him.

I hope that everyone had a great weekend. Please come back tomorrow to see how Parker and his family is doing!

Friday, May 26, 2006

A Lot Can Happen In a Single Day

It has been almost 36 hours since I last wrote and alot has happened since then. I guess I will start with what everyone wants to know. How were the results of the T and B cell markers? It took a while since the results needed to be reviewed by the appropriate doctors but they finally did make their way to us by early afternoon. Parker will have to remain in isolation for at least another week as his T-cell numbers are not high enough to go home. As a result of the PEG-ADA injections his NK-cells and B-cells have increased quite a bit. Unfortunately his T-cells have not. Yes they are higher than they were when we came to Sick Kid's but they are extremely low still. When Parker was admitted he had 9 CD4+ cells and 1 CD8+ cells. He is now at 39 CD4+ and 15 CD8+ cells. These are the TH and TC cells respectively. The normal range is much higher than this. To give you an idea, people who are HIV+ have their CD4+ attacked and depleted. When they finally do get below 200 CD4+ cells they have to take medication for their HIV. Again, Parker currently has 39 CD4+.

What does this mean? It means we are not going home next week. The test will be repeated next week to see if his numbers have continued to increase or they remain similar to what the test revealed today. Apparently his results were expected and are not concerning. Let's just pray that his numbers do rise when they do the next flow cytometry test. That said, we are pleased that his NK and B cell numbers did have a much more dramatic rise.

I have mentioned quite a few times in the past that Parker's weight has increased quite dramatically since coming to isolation. What I have failed to mention is that his head circumference has been doing the same. Parker was born at the 50th percentile for his head circumference and when his measurements were done yesterday we were told that he is now in the 90th percentile. I figured it is better to have a big head then one that is too small. Seen the movie Beetlejuice? Well, since his head has grown so quickly and not remained at the 50th percentile an ultra sound of his brain was done to see if there was something abnormal about it. Parker slept right through the ultra sound like a good boy. The test came back by the end of the day and displayed no abnormalities in his brain. To be honest, I have not been charting head circumferences but I did just recently take a look at where Parker is on the chart and it reads between the 55th and 60th percentile for boys. If you look at where his head circumference is for girls it is the 90th percentile.

Parker has also switched formulas again. I had mentioned that he had moved to the 3300 KJ/L formula a couple of days ago. Yesterday, he moved to regularly concentrated formular (2800 KJ/L) with sugar added to increase the calories to 3300 KJ/L. By being on concentrated formula, he is receiving increased amounts of the different vitamins and minerals as well as calories. Parker's CBCs have been coming back with elevated values for calcium and magnesium and most recently potassium. By using the normally concentrated formula with sugar, Parker is receiving the regular amount of vitamins and minerals and we hope it will bring his electrolytes (calcium, magnesium, and potassium) into the normal range. Parker spent Thursday on this new formula. Recently, I have spoken with the dietician and we have decided that Parker does not need to continue to grow at 35+ grams/day and that normal formula without glucose will suffice. Parker started normally concentrated formula today.

So, alot has happened in the past 36 hours. Maybe I need to write more often? Actually, once a day should be fine. Thank you again to everyone who has been visiting this site and supporting our little boy in his fight against ADA deficiency causing SCID. Come back tomorrow to see what the next 24 hours bring.

Thursday, May 25, 2006

A Good Day Had By All!

We are still waiting for the results of the T and B cell marker test Parker had yesterday to come back. The test does not take a long time to complete but Wednesday is an extremely busy day in the lab so it will most likely be later today that we receive the results for this test.

Parker's CBC (complete blood count) did come back yesterday and for the first time in his life, Parker's lymphocytes are in the normal range. Depending on what source I look at I get a different value for the range. Regardless, all the sources place Parker lymphocyte count in the normal range.

Now, although we are hoping for some good news in regards to his marker test there was a SCID (X-linked) family that did have something absolutely wondeful happen to them. They went home after a successful bone marrow transplant. I can only imagine what it feels like to go through all of this for 325 days and then be able to finally go home with your healthy child. We are so happy for this family.

Parker's occupational therapist is scheduled to see him this afternoon. I am hoping that they are going to be impressed with everything he has accomplished in the past week. Parker now, will try and swipe at things he wants. I feel sorry for his little friends on his mobile because it will not be long before he can actually reach out and grab them with two hands. Parker also now will hold his rattle whenever I touch it against the back of his hands. He will even look at it and shake it. Usually after about 30 seconds he throws it and if I am lucky I catch it before it hits the ground. Otherwise he does not get to play with it until it gets sanitized.

What I am most proud about is how well he is doing with his 'tummy time'. Yesterday he stayed on his tummy for a good five minutes before I tipped him over to his back. He then tried to get back to his tummy almost instantaneously. With a little help from dad, he was there. He proceeded to stay there for another five minutes. This game went on for about 20 minutes. Parker does not throw his hands to his sides anymore either. If one does back, he puts his in front of himself almost right away. Way to go little boy!

Wednesday, May 24, 2006

T and B Cell Marker Test To Be Done Today

Parker has now received six injections of ADAGEN (PEG-ADA). We were all very surprised at how quickly Parker's numbers increased once he started the enzyme injections. His first complete blood count (CBC) after starting the PEG-ADA came back with an increase in his neutrophils, total white blood cells and lymphocytes. Each subsequent test displayed a continued rise in these numbers. I had mentioned earlier that once Parker's numbers reach a specific level he will be able to come home until we go to Italy. Unfortunately, his numbers will not be that high forever if we choose to stay on the ADAGEN and not go to Italy to take part in the Gene Therapy trial. As I have mentioned before, to be accepted into the study in Italy, the patient must remain on PEG-ADA for six months prior to taking part in the study.

The lymphocytes consist mainly of T-cells and B-cells and are made in the bone marrow. For Parker's form of SCID both the T-cells and B-cells as well as the NK cells are killed off by the toxins in his body. The T-cells are named that because they are matured in the thymus gland and then become one of three different types of T-cells. The B-cell mature in the bone marrow and then eventually secrete different antibodies in the presense of specific antigens (something that stimulates an immune response).

Now that Parker has started the PEG-ADA his lymphocytes have increased in numbers. Since the different lymphocytes look very similar under a microscope it is close to impossible to distinguish between the different T-cells and B-cells in a blood smear. To find out how many of the different lymphocytes Parker does have they do a test using flow cytometry. Parker is having this test completed today. If Parker has a substantial number of T-cells in his blood stream we will be able to take him shortly.

The flow cytometry test works as follows. The different cells in your body have specific markers or receptors on them. This test will check to see what receptors are present on a cell and record the number of cells with a particular receptor. How will be explained in the upcoming paragraphs. The next part may be a little scientific but a little bit of science never hurt anyone.

The three types of T-cells are identified by the type of receptor they contain. Tc are incharge of destroying infected cells and contain the receptor CD8. TH are called helper T-cells and aid the rest of the immune system in fighting infection and contains the receptor CD4. Finally, regulatory T-cells aid in the supression of the immune system. These T-cells contain two receptors (surface proteins), CD4 and CD25. B-cells contain the surface protein CD20. These receptors allow for specific cells to bind to them.

The sample of cells that they take from Parker will then be mixed with specific antigens that bind to the different receptors. The antigens are coloured with specific dyes so that the receptors can be identified by the colours of the antigens that have bound to the receptors.

These cells (Parker's blood sample mixed with coloured antigens) are then places in a fluid which moves with the fluid past a light source of a specific frequency. Each cell in the sample is hit by the light source and absorbs some of the light of the controlled frequency. Almost spontaneously the cell re-admits lights of a different frequency. The frequency of light that is absorbed depends on the colour of the dye used on the antigens that are bound to the receptors.

The flow cytometry test keeps track of the number of times each specific wave of light for a particular frequency was emmitted by a cell. By looking at the number of each admitted light wave, you can determine the number of cells of a particular type that are present in the sample. We are hoping to find quite a few T-cells as well as B-cells in Parker's blood sample.

Tuesday, May 23, 2006

Parker Takes It Like a Man!

Parker had his sixth injection of ADAGEN this morning. Recall that the purpose of receiving ADAGEN (PEG-ADA) is to give Parker the enzyme that he is missing. Parker must be on enzyme therapy for six months prior to being part of the Italian study. The injection takes place every Tuesday and Friday morning in the muscle in his inner thigh. He normally cries for about 10 minutes afterwards but once he realises his mommy is there he feels so much better. Mommy usually waits in the anti-room while the injection is being done and once it is complete she runs in to console her little boy. Parker is receiving a test tomorrow to see how well his lymphocytes are working in his body since starting the enzyme replacement therapy.

My wife and I are so overwhelmed by the response we have gotten from people that read the article in the star or saw the news on CityPulse at 6. A majority of the visitors we have had in the past few days have come from or We have received emails from friends whom we have not spoken with in quite some time expressing their concern with what Parker and his parents have been going through. My wife and I can not thank everyone enough for their thoughts and prayers.

Thank you again for everyone who has been visiting Parker's site. Please come back daily to see how our little boy is doing and to learn more about his disease caused by a single faulty gene stored on chromosome 20.

Monday, May 22, 2006

Fourteen Pounds!

Since moving to his isolation room in March, Parker has remained infection free. As a result of feeling better, he has been eating up a storm. When Parker was born, he was in the 25th percentile for his weight. This means that at birth he weighs more than 25% of all babies born. Babies weights are charted on a standard graph against average weights of babies for specific ages. By looking at the chart you will notice that there are lines that display the average weight for babies through the first three years at a specific percentile. Parker was born at 6 pounds and 13 ounces. That places him around the 25th percentile. Generally, a baby should gain weight at a rate that keeps them at the percentile in which they were born, assuming everything else is normal.

Generally, SCIDs have difficulty gaining weight since they spend a good portion of their time dealing with an infection and not feeling very well. As you can imagine, when a baby does not feel well, they do not want to eat very much. For Parker's form of SCID, ADA deficiency, the primary problem is an enzyme not functioning and as a result a toxin dATP accumulates in the body. Those of you that remember high school biology will remember that ATP has to do with energy. The toxins in Parker's body cause his metabolism to increase. This means that he burns calories at a greater rate than the average baby. A test was done on Parker when he first came to isolation that showed he burned calories at a rate of 1.3 times greater than the average child his age. That said, he needs more calories in his diet to gain weight at a rate that would keep him at the 25th percentile that he was born at.

Without knowing that Parker had ADA deficiency for the first six weeks of his life, he was not getting enough calories to gain weight. When we came to isolation, Parker had dropped to the from the 25th percentile to the 3rd percentile for his weight, a drop of 22%.

After helping Parker fight the bacteria they found in his lungs after completing his lavage in early March, he began to feel and eat better. We then changed Parker's formula to Nestle Good Start which seemed to look alot more like milk and as a result, he began to devour his formula. Lastly, his dietician changed the concentration of his formula to reflect the fact that he was burning calories at a much quicker rate. His formula was changed from 2900 KJ/L to 3800 KJ/L. The formula was slightly thicker but Parker did not seem to mind one bit.

Since coming to isolation Parker's daily weigh-ins have been recorded. Parker has been gaining weight at just over an ounce each day. I have been plotting the data in Excel so that I could obtain a linear regression since we began keeping track of his weight.

Parker weighed in at 14 pounds yesterday. This places him just over the 25th percentile. We are so happy that all of the work we have been doing with Parker here at the hospital has continued to pay off. The nurses and doctors do an amazing job keeping him healthy. The PSAs (cleaners) do a spectacular job cleaning his room each morning, sanitizing every last inch. Thank you to everone who has helped Parker finally reach his goal weight. As a result of reaching the 25th percentile, and so that he does not continue to gain weight so quickly (babies generally gain weight at a lesser rate once they reach four months) his formula is now concentrated at 3300 KJ/L.

My wife and I would like to thank everyone at CHUM and CityTV for doing an excellent story on Parker last night on CityPulse at 6 and 11. You can check out their website here. There is a link to the actual interview that City did on that page. Thank you to everyone who has visited our site in the past couple of days. Please come back regularly to see how our little boy is doing.

Sunday, May 21, 2006

Who Says You Can't Have Fun In a Bubble?

Yesterday was another excellent day for Parker. Since it was his mommy's birthday yesterday we decided to let mom go out for a little bit and visit with her best friend. Parker and daddy had a great couple of hours playing with his gymnie, sitting in his Bumbo by the window and working on Parker's 'tummy time'.

I decided that the best way for Parker to get used to what the outside world looks like was to have him sit down and take a look at it. So, I cleared all the space by our window, placed his Bumbo chair on the sill and let him stare out at the world. He loved seeing the birds fly by. I don't know what he was thinking as he sat there but he definately seemed to be enjoying himself.

Now, this is the third time that mommy has left for a few hours with daddy taking care of the baby and then return to Parker without his pants and socks on. To be honest, Parker did have one sock on; but, apparently that is not good enough. I just find it easier to do 'tummy time' without his pants on. Well, the rolling over portion of 'tummy time'.

On the topic of 'tummy time', Parker has now decided that when he no longer wants to do his 'tummy time' homework he just places his arms by his sides and does a face plant. Parker has fooled mommy with that one, but he has to be a little more resourceful with his dad. I realised that he does not really like having his hands by his sides and as a result gets angry and then will put one arm back in front and then roll himself over to his back. A little bit of clapping on daddy or mommy's part afterwards lets him know that he has done something special.

Parker had a complete laughing fit yesterday when we were playing peek-a-boo with his oxygen saturation monitor. I have absolutely no idea what he finds so amusing with the monitor (it is turned off) but he was cracking up yesterday. If you check the recent videos on the side there is one were he is laughing at his monitor.

I would like to take the time to thank everyone who visited our site yesterday. We had over 4000 hits which was very exciting. Thank you so much to The Toronto Star and Elaine Carey (medical reporter) who did such an excellent job with the article. Please visit our site often to see how the little monkey is doing. We would also like to thank Dr. Aiutu for speaking with the Toronto Star about his work in gene therapy. He is truly a remarkable man. I am sure that Parker would like to say a special thank you to his nurses on the floor who are truly amazing. He loves playing with them whenever he gets a chance to.

Lastly, my wife and I are doing an interview today with CityTV so check out City for our story. I was told it would air in the 6pm news tonight.

Saturday, May 20, 2006

Thank You to the Toronto Star.

I have just read the article by the Toronto Star at and we are very pleased with the work they did with the article. Elaine Carey, the medical reporter at the star did an excellent job researching the disease and we are so pleased with the number of people that will read the article and visit our site as a result. I had mentioned yesterday at the hospital that the difference between when a doctor writes a medical paper about the disease and when a major newspaper or someone who is experiencing the disease writes about the disease is who reads the work. Doctors write for other doctors to read. The latter write for the general public, and in this case I write for parents to read about the disease. So we are so pleased with the attention that this devestating disease will get today.

With such an in depth article there are only some very small additions that I would like to make. Parker resides on the reverse isolation unit which is isolated from the rest of the hospital. There are very few people that have access to the unit. Parker does stay in a reverse isolation room that is filtered regularly while on 8B; however, not all the rooms on the unit have a bank of filters. When a child undergoes a bone marrow transplant they reside in a reverse isolation room until their counts increase. Once that happens they move to a normal (but very clean) room on the floor. This is only the case for the children without SCID. Children with SCID must remain in their isolation room until they are discharged.

Parker is currently receiving enzyme replacement therapy by injecting the missing enzyme twice a week into his muscle. The idea behind this is to give Parker the enzyme that his body cannot produce so that the dATP does not run rampid in his body killing off his immune system. From my reading, this therapy works very well for two to three years but for some unknown reason the injections become less effective with time. For this reason we do not feel it is a viable final solution to ADA deficiency.

The treatment that we will be involved in in Italy will be covered by the telethon in Italy that is financing the study. While in Italy we will be put up in a residence similar to that of Ronald McDonald House here in Toronto. The expenses we will incur will be the result of not having any income for the next year while we try and help our son through this disease. As you can imagine there will be many expenses that need to be covered at home as well as additional expenses we will incur while living in a foreign country for an average of six months. We have no idea what the result will be with the gene therapy. We do know that we will have to return to Milan, Italy approximately four more times once the study has completed. If the therapy does not work, Parker would have to be placed back on PEG-ADA which is extremely expensive to say the least. We are not paying for the enzyme therapy at the moment but are not guaranteed anything in the future. It is all of the unknowns that make it very difficult to place a dollar amount on how much this battle with SCID will cost. We do know that we will get through it with the love and support we have gotten and continue to get from our families and friends.

Again we would like to thank the Toronto Star for all of their work with this article. We were so pleased after reading it this morning. I don't think we thank the nurses and doctors at Sick Kids in Toronto nearly enough. The work that they do there is truly remarkable.

Thank-you for taking the time to visit our site and learn more about this disease. We hope that everyone will continue to visit regularly to track Parker's progress as he beats this life threatening disease.

Friday, May 19, 2006

Stop the Press!

I had mentioned in an earlier post that we had done an interview with the Toronto Star about Parker's disease. After speaking with them the past few days we were told that it is to be slotted on the front page of tomorrow's Saturday Star. In addition, I think there is a related story about the Gene Therapy trial that Parker is going to be taking part in Italy. I was told that they did contact the doctor in Italy for that particular article. My wife and I are elated at the amount of awareness for adenosine deaminase deficiency this will have as well as Severe Combined Immuno Deficiency in general. We definately look forward to reading the story tomorrow morning.

As for my special little buddy? Parker is doing very well. He did have his injection of PEG-ADA this morning. He handled it very well. He usually gets some tylenol about 30 minutes before the injection to ease his pain a little bit. But, to be honest, having his mother hold him after the injection is definately the best pain relief for him.

Parker is definately enjoying his new formula. Actually, I am not sure if he enjoys it anymore than his old formula or if it just doesn't fill him up the same way. It seems to be alot like Chinese Food. He will eat alot and then a couple of hours later he is starving again. To be honest, we knew he was going to be eating more on the new, less concentrated formula. Why wouldn't he? Last time I checked a milk shake is definately more filling than a glass of milk. Parker is now having 180+ mL (6+ ounces) feeds. He is almost polishing off a litre of formula each day (900mL+). If you consider that his formula has 15% more calories than regular formula he is pretty much is having a litre of formula a day. As a result of having a more watery formula, he is receiving more fluid and uninating more frequently. This is something we wanted to see, as we all know the importantce of water in your diet.

It is Parker's mommy's birthday tomorrow so please make sure to wish her a happy birthday. I already know her birthday wish. So make sure to check tomorrow morning for what we hope is a great story about SCID caused by an ADA deficiency.

Wednesday, May 17, 2006

Just the Facts, Mam.

I have decided that today's message will be much more brief then normally. I want to make sure that there is something for everyone to read as well as some pictures for people to see.

Parker has taken well to his new formula. He does drink quite a bit more of it; he drank 850 mL by the time we left for this evening. I am not sure if he is more gassy or he is teething but he did seem a little more cranky when we tried to put him to bed tonight.

Parker worked with his occupational therapist this afternoon. He did a fine job with her and my wife but I he needed a little encouragement over the phone so he would become more interested in the work they were trying to do. It is like I always say, "No SCID of mine is getting out of doing his homework". So, I told him over the phone that daddy said he had to work hard for the therapist. I am told that he did. If you check the recent videos I have added one with his workout today with the therapist.

Parker gained quite a bit of weight yesterday so I was not surprised when he was down 40 grams today. A correction, just like the stock market has done over the past few days.

Tuesday, May 16, 2006

What an Alert Little Boy!

I got to spend a portion of the morning with Parker today. We spent most of the time playing together. We again worked on strengthening his neck muscles by hanging out on his tummy. Parker can now spend a good three minutes on his tummy looking around before he gets cranky and wants to roll himself over on to his back. He does all of this without any support. We used to have to place pressure on his bum to reinforce that he needs to push his bum down to help his head up, but he no longer needs this.

We have now modified his gymni so that he can kick the wheel that spins around and plays music. Yesterday, he actually knocked his lobster right out of the socket on the gymni. No worries though. Daddy's quick reflexes was just quick enough as he caught the falling lobster before it plumetted to its death on the cold floor below.

Parker had his fourth injection of ADAGEN (PEG-ADA) today. Our tough little guy did very well. He was a little hungry afterwards but that is hardly surprising. I had mentioned that his formula was being "deconcentrated". He had his first full day of the new formula. Since five o'clock this morning he has already eaten over 800 mL of the new formula. Parker drinks it like water. Actually, compared to before it pretty much is water. Okay. Not really but he sure is hungry more often now.

My wife and I were a little worried that he would not gain weight on the new formula; however, he gained quite a bit today. Parker gained just shy of five ounces over the last day. I am guessing that alot of it has to do with the increased amount of liquid and will eventually correct itself.

What was also exciting was the Parker slept through the night for the first time last night. We put him down at 6:30 last night and he did not wake up until five this morning. He then ate and then went back to sleep.

My wife and I met with a wonderful reporter from The Toronto Star this morning. We are trying to get as many people familiar with Parker's disease as possible and I am sure The Star will do a wonderful story on him. I am unsure of the date it will appear in the paper but I expect it will be either Monday or Tuesday after the long weekend.

Now, I mentioned in the title that Parker is an alert little boy. In the past few days I have really noticed a difference in him. He is constantly wanting to play with his dad. Whenever I come to the sliding door of the anti-room he stops whatever he is doing (even eating) and smiles at me. Who would have thought that Parker would think all the hard work he does when he is with his dad was fun? Way to go little monkey!

Monday, May 15, 2006

Numbers Are Up Again!

I will start this article with what everyone is waiting to know. Parker's numbers from his CBC came back early this afternoon and they have continued to increase. Last week, Parker's neutrophil count had crept back over 1.5(cut off for neutropenia) to 1.55 making him no longer neutropenic. Today, Parker's neutrophil count reached 3.97 leaving him even more better off than last week. Looking at his white blood cell count last week he had gotten up to 3.5 (4.5 is normal). Today it was 8.3, in the normal range. Now, Parker's biggest hurdle is getting his lymphocyte levels in the normal range of 2 - 17. When he came to Sick Kids he was 0.080. Last week he made is to 0.43 and today he was 1.7. Just shy of being in the normal range. From what I understand, Parker's B-cells are monitored by his gamma globulin levels. Each month he gets a transfusion replenishing these cells. Parker's IGG levels (immuno gamma globulins) have decreased again this week. PEG-ADA does not normally repair the IGG levels and monthly transfusions are normally still needed. That said, there is the possibility that it has just not taken affect yet.

Parker also had a visit from his occupational therapist today. She was very pleased with what he accomplished over the past few days. Daddy and Parker had worked on Parker's tummy exercises as well as rolling over and it definately paid off. Parker came through when it mattered and impressed his therapist. When daddy came by at the end of the day he rewarded Parker with some more play time. He is a strong little boy and daddy is very proud of everything he has accomplished.

Tomorrow, I will get to see Parker in the morning as I will be dropping by to do an interview with the Toronto Star about Severe Combined Immunodeficiency caused by an ADA deficiency and how our little boy is coping with the disease. We will let everyone know when it is going to be in the health secton of the Star.

Sunday, May 14, 2006

Happy Mother's Day!

Today was Parker's mommy's first mother day. Parker and daddy spent Saturday trying to think of what to do for his mommy. We sent mommy off for the afternoon and I in my chair and Parker in his 'aquarium bouncer' sat in front of the computer looking up things to get for a first time mom. We decided that flowers and chocolates were not going to suffice so we had to put our head's together. In the end, we decided that we would tell mommy to get her hair done on us, get her each a card, and have daddy take her out to dinner. Parker wanted to make sure that he could give her the card, so the plan was to place his card under his blanket so she would eventually find it when she made his bed. From the look on Parker's mommy's face, she was more than pleasantly surprised. Parker and his mom had a wonderful day together. Parker even went to bed for mommy at five in the afternoon so we could go to dinner.

Parker is getting his counts done again tomorrow. We are really hoping that his numbers continue to increase. We were so happy to see them go up last week that we would be quite set back if they did not go up tomorrow as well. Parker is also getting his Pentamidine on Monday. It would be nice if they could find a vein in his hand or foot so they did not have give him a another poke in his head.

Happy Mother's Day!

Saturday, May 13, 2006

Message Number Fifty

I would like to start by apologising for not writing the past two days. I have been so busy at work and at the hospital that I have been so exhausted by the time I come home I am unable to write. That said, I was able to sleep in this morning since Nonna is at the hospital for Saturday morning. I am rejuvinated and ready to write another article. Believe it or not, this is actually the fiftieth post I have done since I started. I would like to thank everyone that comes each day to read my articles as it is nice to know so many people care about our family.

Parker received his third injection of PEG-ADA without a hitch on Thursday. He was receiving his rejections on Mondays and Thursdays but the injections are going to be moved to Tuesday and Friday starting next week. This will allow us to make one less trip to Sick Kid's once we get to go home. Once we go home we will have to return for the injections at clinic. Clinic will consist of checking his complete blood counts (CBC) to make sure that everything is going well with the PEG-ADA. The new injection dates will coinside with the clinic date (Friday) so that we may make once less trip downtown.

I was doing my regular research on the disease the past few days. I have spent most of my time focusing on the different types of white blood cells in the body. While doing this I came across some great footage of a neutrophil attacking a bacterium. For those of you who would like to take a look please click here to see what Parker was missing while he was neutropenic.

Parker had an x-ray done yesterday. The doctor was worried that his respitory ratae was creeping up again over the past week. Athough we do feel that there are some variables that may account for the data depicting this we did agree to have the x-ray done. Mostly because we were curious to see what his lungs looked like. Recall that Parker does have some protenosis in his lungs and that caused his respitory rate to be increased an forced him to be on oxygen for quite some time. We were pleased to hear that the x-ray not only showed his lungs not to be worse than his last x-ray but did display some clearing in some portions of his lung. That was good news. Obviously we would like his lungs to be completely clear we are happy that he does not display any worsening symptoms.

I heard Parker laugh yesterday. I mean laugh. I was sitting with him in the chair beside his oxygen saturation monitor (which he no longer uses) and I would bring him to the monitor and then pull him away so he could not see it any more. As a result he would open his mouth with a big smile and then start laughing. I have never seen anything so funny before. My wife and I just looked at each other.

Parker will begin a less concentrated formula on Monday. He currently has his formula concentrated so it is 3800kJ/L but will drop down to 3300 kJ/L. This is still elevated from the normal 2800 kJ/L concentration but will allow him to have more fluid. The dietician is concerned that Parker is not receiving enough fluid since the formula is concentrated so much. She did suggest we could give him water once in a while but we opted for the decreased formula. Parker is continuing to gain weight at a above average rate. We do expect him to be at the 25th percentile very shortly.

For those of you who live in Peterborough. The Brick at Lansdowne had a tent sale and bbq yesterday. I believe the proceeds from the bbq are going to Parker. Thank-you very much to The Brick for doing that. It is truly appreciated.

Wednesday, May 10, 2006

Work Hard ... Play Hard

Today was another special day. Daddy got to spend the morning with Parker so that mommy could have a bit of a break. The last thing we need is for mommy to get sick. It was also so that I could meet with the dietician as well as the occupational therapists.

I was very excited to work with the occupational therpists today because Parker and I had been working on our rolling over (his rolling over) as well as increasing his strength in his abdominals. Although Parker needs a little bit of coaxing to roll from his back to his front he is improving greatly in this skill. He now knows when it is time to roll over and aticipates it by moving his arms into a position so that they will be under his head like we do for 'tummy time'. Then it is a quick 1...2...3... roll. When he does reach his tummy we make sure that his elephant mirror is in front of him so he may look at himself. By banging on the top of the mirror with his rattle we can make sure he is attentive and keeps his head up for an extended period of time. Every once in a while Parker will move his hands to his side causing him to lose the support he needs to raise his body but he is learning to place them back once he starts to lose control. Think daddy is tough on the little guy? Mommy may be obsessed with making sure he does not have dry skin, but no SCID of mine is getting out of his homework before he is done.

The occupational therapist showed us a new trick today. Well, not really a trick. Parker is now able to roll over from his front (tummy) to his back all by himself. How, you may ask? When he gets angry enough while using his arms to lift his body up he lets out a large cry and pushes really hard that he rolls back over to his stomach. We all cheered when he did it. He looked very satisfied with himself. So you know what his reward was? A trip back to his tummy so he could do it again. Hard work will help our little guy surpass all the goals we have set out for him.

Parker's dietician also came by today. It was nice to meet her and discuss the progress Parker has made with his weight, height (length) and head circumference. We were all in agreement that Parker has made some very positive gains in the past two months. He is almost at the 25th percentile in his weight. His length has also shown improvement. We discussed dropping his caloric intake to the normal level but agreed to revisit that in two weeks after the PEG-ADA has taken more of an effect. The extra concentrated formula does increase his calories to 1.3 times the normal value but it also increases the vitamins and minerals in the formula as well. We were so pleased with his reaction to the PEG-ADA that we do not want to introduce any new variables into the equation. My wife and I both agree that his formula will have to be concentrated less by the time we go home as we don't want our baby to be too much of a chunky monkey.

Tuesday, May 09, 2006

Great News!

Normally, I would write a very large message but I have some information that I want to tell everyone as soon as possible. Parker’s PEG-ADA injections have already begun to work. He had his blood taken this morning and the results have already come back. Parker is no longer neutropenic. His white blood cells have gone up and his lymphocytes have also started to increase. We were not expecting that his numbers would rise so quickly, but they have and we are so thankful for that. We need Parker’s cells at normal levels before we go to Italy. In my opinion, Parker has shown us that he has very good bone marrow and as a result will be an excellent candidate for the gene therapy trial in Italy.

Monday, May 08, 2006

Seriously Though

My wife and I were talking today about Parker and we decided that Parker has developed his own personality. We have noticed that our son is a very serious little man. I am not sure if it is because he is stuck in his little room all day or he is just a very focused little boy; that said, I guess when you do not laugh very much you appear a little on the serious side. That is not to say he does not smile or laugh. He still finds his friends very amusing. His saturation monitor is also quite exciting to him. Just recently I have started tickling his feet with his rattle. He seems to enjoy that as well. I guess it is hard to know when to laugh and smile when your parents' faces are hidden behind a mask all day. That said, we know this little monkey is going to grow up with quite the sense of humour. You have to after going through all of this.

Parker received his second injection of his PEG-ADA today. Again, it was quite painful for him but the little man persevered. Some snuggles from his Nonna was enough to get him through his pain. Parker is scheduled for quite a bit of blood work tomorrow. They will be doing his regular blood counts as well as a special test to check his neutropenia. We are hoping that his neutrophils do return as a result of being on the PEG-ADA. Parker will have his Nanna and his mommy with him tomorrow during the blood work. There is the slight possibility that the neo-natal team (baby specialists) may came by over night to take his blood. I would feel terrible if that is the case; however, this team is absolutely amazing with infants. I would definately feel much better having them take his blood than the regular blood work team.

The occupational therapist came by today. Parker did not really want to have much to do with her as he was definately more interested in eating. I wonder where he gets that from? My son would rather eat then exercise. She was kind enough to leave us with a folder with pictures of him working through his exercises as well as explanations on how to help him. Now, I don't know much about what babies are suppose to be able to do at 15 weeks of age. We try and work with him on as many things as possible so that he does not lag behind. But, apparently he does need some work with his rolling over. Now, I have yet to know of another infant that can roll from their back to their front before 4 months of age but if they say Parker is behind in his rolling over I can guarentee you that he will be ahead of the class in no time at all.

Parker is looking forward to hanging out with his Nanna for the next few days. He missed seeing her last week and can't wait to have some snuggles with her.

Sunday, May 07, 2006

What Did You Say?

Parker has just recognized that he has a voice. Today, after his bath I decided to have Parker work on his rolling over. So while he was on his back, I began to roll him over to his front. Once he made it to his front, he popped his head up and started making some weird noises that we had never heard before. I looked at my wife and we were in shock. We were always told that Parker would definitely be delayed in speech so we never expected to hear him try and make sounds. When I repeated the drill for Parker, he again popped up his head and started making some noises. Afterwards, while he was watching his Baby Einstein he again started making the noises. We are so proud of our little boy. I know that this is hardly speech, but it is a step that needs to be taken so he eventually may.

I heard back from a center in the States that informed me that they have witnessed other ADA deficient patients with neutropenia. It did eventually correct itself, so that is encouraging.

Parker will be receiving his second injection of PEG-ADA on Monday morning. It will be done in his other leg, alternating legs every injection on Monday and Thursday. Tomorrow, his Nonna will be there to sooth his pain after his injection.

On that note, when his Nonna came to visit him on Saturday morning, Parker let out a big smile when he heard her say that she was coming in for her visit. It is nice to know that he is starting to recognize who different people are by hearing their voice. Now, when my wife and I open the glass door from the anti-room to his isolation room, Parker turns his head and stares at us. Even when we close the door to get changed he continues to stare, hoping that we will return.

I have a couple of new videos going up today. One shows you Parker checking out his mom as she gets ready to enter his room, and the other is of him experimenting with his voice. Make sure to check out the new videos each day. I have limited bandwidth at the moment so please let me know if there are any problems viewing the videos. I can always get more bandwidth if needed.

My apology for not writing an article until now as the Internet at the hospital was a little hit-and-miss today. Unfortunately, I did not get a chance until now, but please check in each day to see how the little monkey is doing.

Friday, May 05, 2006

And The Winner Is ...

Parker Joseph DesLauriers.

We received confirmation today that Parker was the Grand Prize Winner for the April Photo Contest on BabiesOnline. We all knew that he was the cutest, but with everyone's support everyone now knows it. Thank-you so much to everyone for voting for Parker each day during the final round.

A day has passed since Parker had his first injection on PEG-ADA. It went very well. There were no major side affects that we noticed. He was in some pain afterwards as it was a large injecton in his right inner thigh. I can only imagine how much that would have hurt. But my boy is tough. Very tough.

For those of you that are unsure of what PEG-ADA is and why he is taking it I will explain. First, to be considered in the Italian study Parker must be on PEG-ADA for six months. The idea behind getting PEG-ADA injections is that Parker's gene that regulates the amount of ADA his body produces does not work correctly. Well, it does not work at all. So he does not produce any adenosine deaminase (ADA). The purpose of ADA is to remove a specific compound in his body. Deoxyadenosine is a natural substance that is made during the break down and synthesis of DNA in the body; however it is toxic. ADA normally binds to it and converts it to deoxyinosine a non-toxic compound. Obviously, if the body does not produce ADA then it cannot convert the toxic deoxyadenosine to the non-toxic deoxyinosine. As a result, these toxins build up in the body. The toxins kill off his lymphocytes, and we hope have also hindered his neutrophil production.

The idea behind ADA enzyme injection therapy is to give the body the ADA it needs to convert the toxic compound to the non-toxic compound. PEG is just a compound that the ADA is attached to so that it lasts longer in the body than it normally would; as it usually removes itself quite quickly. As a result, the bodies T-cells are able to survive, and in some cases, so do the B-cells. We are hoping to see these effects in the next four to six weeks. If we do we will be able to bring him home before we go to Italy.

Some of you who have not read my previous articles may be wondering why we not just do that forever. Unfortunately, for some unexplained reason, the T-cells production and function decreases dramatically after a few years. As a result, we do not feel that long term PEG-ADA injections is a solution to Severe Combined Immunodeficiency caused by an ADA deficiency.

Thursday, May 04, 2006

PEG-ADA Starts Today

Today is a very special day. Parker is scheduled to have his first injection of PEG-ADA. We do know that there may be some autoimmune problems we may have to deal with as a result of starting the PEG-ADA; however, they should dissipate as his body becomes used to the drug.

I had mentioned yesterday that we were worried that Parker may have to have a bone marrow aspiration done to see why he is neutropenic. I did speak with a woman from California yesterday who informed me that her son who was also ADA deficient prior to starting enzyme replacement therapy is no longer neutropenic after having started the injections. The doctors nor I have read any literature linking the two together but I am definantly pleased to have now found one case of it. Regardless, the haemologists have decided that they would watch his counts over the next week and a half to see if the PEG-ADA does increase his neutrophil counts.

Yes, it was Parker's daddy's birthday yesterday. I had hoped that it would not leak out, as I did not want a big deal made of it. That said, I did have a good birthday. Parker sent his daddy some balloons and a card yesterday. That made me a little teary-eyed to say the least.

Although I do feel much better than I had a few days ago, I am definately not feeling well enough that I can go into the room with my baby boy and hold him. It was definately hard not being able to give him a hug on my birthday; however, he did smile at me through the glass. It was enough to make me smile.

The occupational therapist came by for another visit yesterday. She was pleased with everything he is doing; however, we now must work on his rolling over from his back to his front. I will post a video later today showing my wife and him working on just that.

I know that I have bene a little slow with the pictures on the web site lately. I do apologise for that. I promise that you will be able to see some new pictures for yesterday and today if you check back later this evening.

I have to say that I am completely overwhelmed with all the support my wife and I have received through this whole ordeal. With every kind word and every act of kindness we gain another ounce of strength. I have said it before and I will say it again. I know deep within my heart that Parker has been chosen to have this disease because he can beat it. And in beating this disease he will make people all around the world aware that it exists. We are very proud of our little boy.