2 years post

So May 26 marked two years since Katlyn received her gene therapy. We marked the occasion with a routine visit to NIH. At first we were a little hesitant going because of the whole swine flu thing, but her doctors reassured us things should be fine. Plus the amount of precautions we take just in our everyday lives, it would probably be unlikely she would catch it. The airport isn't as scarey for us as it used to be. Most of the time, Katlyn wears a mask, especially when the area around her is more occupied. The rest is really simple. We just make sure she doesn't put her hands at her face or anything else in that area, since that's where germs enter your body. I'm really glad we are a lot more comfortable with travelling now, because Johnathan will be joining the Canadian Forces and it's possible I will have to take Katlyn by myself.
This trip was very enjoyable. It was nice to see the team of doctors that have got Katlyn this far. We were able to catch up with a lot of other families that we haven't talked to in a long time, as well as meet some very nice and interesting new families (including a family that is going through the protocol as we speak). I can really feel for them. Having your little one in isolation is not easy. I introduced the child's mother to my Katlyn so that she could see that although Katlyn's immune system isn't perfect, things do get easier. The 15 months Katlyn was in isolation was not very easy, but when we did finally come home, it was no biggie anymore.
Katlyn's immune system is about where it has been for awhile now. Her neutrophils are normal, which used to be our biggest focus. She is around 1600. Her lymphocytes are right around 200 and her cell function is normal. So that means that Katlyn does have a small number of cells, but what she does have is working just right. It was far from normal in the past, so this is very encouraging. Also we been given the green light to start some vacinations if we want. There is no way to check and see if they are taking or not because she is still on IVIG. But once she is done with that, we'll be able to see. Also we are now allowed to spend the night at the campground.
Besides that, it looks like Katlyn's next surgery will probably take place at NIH. They are talking about taking a 1 1/2 cm border this time of one, maybe two of the tumors. That will probably take place in about a month or so time. Also we are planning a much needed visit to the IWK. I cannot even remember the last time we were there, but it will be nice to meet with Katlyn's amazing team of doctor's there as well as catch up with some of our favorite nurses. It's hard to believe the IWK used to be like our home.
We have so much more freedom now. We are slowly but surely starting to live our lives outside of the bubble. We don't hesitate to take Katlyn somewhere now. She literally experiences new things and places on a daily basis. She has been borrowing books from our library as well as going fishing with her father. She spends pretty much her whole day outside enjoying every second of it. We were happy to finally have the yard to be able to get Katlyn a swing set. She loves it. Also we plan on putting Katlyn in swimming lessons this summer and maybe gymnastics in the fall.
We are truely loving every second of life. Yeah we have been through a lot, and still have a lot more to go through...but to be honest, we are truely truely happy. Life just feels so incredibly wonderful. We don't dwell on the negative things. Instead we focus our energy on the positives...and it seems like there have been more and more lately. Hopefully much more to look foward to.

Sorry I have neglected Katlyn's blog for so long. We have just been so busy with life and have not had much time. Katlyn is doing extremely well. She has been sick twice now and both times, she was able to fight it all on her own...with no complications. The biggest thing I noticed is that it takes her longer to fight something off, but she has showed us that she is capable of fighting at least a little bit. Her counts are still low. We used to worry a lot about neautrophils, but Katlyn's neautrophils are more in the normal range. Her lymphocytes however, remain low for now. The good news is that Katlyn's cells, although a low number of them, function normally.
So as I mentioned in my last entry, Katlyn had her first surgery. The surgery was a success. According to two pathologist reports, the whole tumor is gone!!!!! The good news is we got away with taken a smaller border with the advice of a Moh's surgeon here. Because the tumors are so small, he wanted to see if we could get it all with a smaller border, and it worked. So we are looking at removing 1 or 2 about every six months. The surgery itself didn't really seem to bother Katlyn a bit. She never complained of the pain. If you asked her about it, she would say that it hurt but that she was okay. MY LITTLE TROOPER!!! So we have an appointment next week with Katlyn's doctors in Saint John to discuss what our next plan is. After that I imagine we'll book her next surgery.
Katlyn has been really enjoying life. We have been letting her do more and more things lately. She has also been playing a lot more with children her own age. We have however, put some stuff on hold temporarily while we watch this swine flu thing. Before if I needed to go somewhere and Katlyn was with me, she would come too. She was in Wal-Mart just the other day picking out her very first big girl bike...which I must add she's seems to have mastered already. But this swine flu thing has forced us to take a step back. Hopefully it will just blow over and is something that we don't have to worry about much, but we don't want to take any chances.
In other news Johnathan and I got hitched on April 27. It's something we have been wanting to do for a long time now, but we were just waiting for the right time. We have been together for more than seven years, and stuff just kept coming up. Most importantly we wanted Katlyn to be able to be there with us for such a special day. So we decided to have a very small, but very special wedding. That way our girl could be there too!
Other than that we are just enjoying the sun and the new house. Johnathan is currently outside this morning building a deck on the front of our house. I already have him booked afterward to build me a garden box, a garbage box and maybe more! We'll see! We are trying to get our yard in order so that we can have some grass. It's a huge yard though, and it's going to take lots of time and patience, but we are loving it!!! Especially Katlyn!!!!

Three Years Old and Surgery

Katlyn had her fist tumor removed yesterday on her third birthday. What a day! We had spent the previous night in a hotel and because it was a strange place, Katlyn would not go to sleep. She asked us if the hotel room was our new house? lol. She can really crack me up. Anyway so after only getting a few hours of sleep we awoke at 5am and set off for the hospital. Everyone was so nice to us and Katlyn. I think they were especially nice because they felt so bad because it was Katlyn's birthday. Before if Katlyn had to go for any sort of surgical procedure, we would have to bring her in the OR and stay until she was asleep. This time however, she (being so grown up now) walked on her own with the nurses. When she was on her way she neared the corner away from us and all the nurses were singing happy birthday to her. She never even looked back at her very distraught parents. My stomach tightened and I could feel tears welling in my eyes. I looked over at John and I could sense he was feeling somewhat the same as me because I could see the pain in his eyes. Our little girl is growing up. It sort of gives me this empty nest feeling. She doesn't need as like she once had. She is brave and she can do a lot of this all on her own.
My baby girl was going for surgery. She wasn't scared to be going down the hall with a bunch of strangers away from her father and I. She never even looked back.
So the surgery was about 2 hours. Johnathan and I sat in the hall amongst other family member anxiously awaiting their loved ones to also be in the recovery room. I fell asleep on John's shoulder and I think he also fell asleep leaning on me. A few times I was was awoken by what I thought may be someone coming to tell us our little angel was all done and everything went well. Each time becoming more and more frustrated and worried because the procedure seemed to be taking forever. Finally, I hear "good morning," and open my eyes to the surgeon. He told us everything went very well and what to expect from Katlyn. he also gave us directions on how to care for her cuts.
Awhile later we finally got to see our little girl. Usually waking from an anesthetic, Katlyn usually does very well. She says crazy things and really the best way to describe her is a little mini drunken sailor. This time was different though. She was very upset. She was screaming and screaming. I could once again feel my entire body fill with tears. I was so scared. It seemed as though she was in so much pain. While the nurse was getting a look at her incision sites, it really took me by surprise how large the one was that they took a tumor. I guess I didn't expect it to look so long.
About a half an hour later the anesthetic started to wear off and we soon realized that the cries from Katlyn were not pain, but just the anesthetic. What a relief. I thought we were in for a lot of terrible pain. Pain I cannot really describe.
So Katlyn was quickly released from the hospital and so far I have not had to give her anything for pain. I wonder if it's just because of nerve damage and things may seem fine now, but pain will come. Where they took the tumour it is so bruised and swollen. It makes me want to scream and cry. I am really having a hard time with the fact we took something that seemed so innocent and subtle away from her and traded it for a big gash, all swollen and bruised.
She seems fine though. This morning she said her boo boo hurt. She wasn't crying or anything. She just told me matter-of-factly. I gave her a little bit of codeine a she hasn't said anything again. I just don't want her to be in any pain at all.
So we go to NIH at the end of this month. Hopefully, by that time the tests from this tumor are back and we're told they got the whole thing. At that point I will get excited because Katlyn will no longer have 13 tumors, she'll have 12.

We just back from Katlyn's appointment at NIH yesterday and things went really well. Apperently they have been looking at some of their other patients with ADA SCID for the DFSP. Surprisingly it seems as though they are definetly linked with Katlyn's condition. They checked out 6 of their other patients and 5 of the 6 also have DFSP. The very interesting thing is that these patients also have multiple tumors just like Katlyn. Where it seemed impossible before for Katlyn to have 13 of these tomors because it has never been reported that a patient has had more than one, it is just comfirmation that we have the right diagnosis. Also one of the patients is 10 years old so it lets us know that these tumors are not going to go crazy tomorrow and we do have time to surgically remove them. They are offering their patients three options; they can have Moh's surgery, chemotherapy or they can just be under observation. We feel very comfortable with surgery, which will be happening any day now. Usually with Moh's surgery they will take a 3 cm border around the tumor and deep to ensure they have gotten the whole tumor. because Katlyn's tumors are so small, the doctors are going to try a 1 cm border with the first surgery. For that reason the first surgery involves taking one tumor and then taking a close look at it to see if it will work. The surgery will be any day now and we're pretty anxious to get started.
Besides that Katlyn's lab results to indicate some change. For starters, for a little girl who didn't have any ADA activity before, she is now 2/3 normal. What does this mean. Hopefully it means that the ADA gene will filter her body of toxic matobolites and she won't have anything killing off her cells. At this time her numbers are still low, but we are confident her lymphocytes should take off at anytime now. For a little girl who usede to have a lot of problems with her neautrophils, she no longer does. Everything seems to be taking its time, but overall things are getting better. Someday I believe Katlyn will be deemed normal and healthy.
So her doctor's in the US think she should get her flu shot. It came to a surprise to us, but they say it won't hurt her and there is a possibility it may work. Also we no longer have to take Katlyn to the hospital for regular blood work. They said there is no reason to check her any sooner than her appointment next time in February. So unless something comes up we will no be going to the hospital as much anymore. Lastly, the doctors also feel as though it's time we start to try and get Katlyn off IVIG. She has shown signs she is starting to make her own antibodies. We will not know if she's ready to be off the IVIG unless we try. So we're going to just start going for longer periods of time between each infusion. It's a bad time of the year so stopping completely isn't a good idea but going for six weeks and then longer and longer is what they suggest at NIH.
Katlyn is doing very well. We believe it's possible she got her first cold. All we know is that her nose was runny and she compalained of having a sore throat. That has all got better though....on its own!!!!

I know it's been awhile since I've updated Katlyn's blog. Usually it's because there isn't really much to report. This time however, we have been very busy. Katlyn had a terrible accident involving her feet. She is okay and they look really great now, but it's been a long process. You see, a couple of weeks ago I was getting Katlyn ready for her bath. This involves me first disinfecting her bathtub and then waiting 10 minutes. After that it's all clean and I usually give her a bath. On this day, events took a different spin. After disinfecting Katlyn tub, she was pretty upset because she didn't want to wait. It's not really unusual for her to be upset because she loves the bath so much. So anyway I had 10 minutes until her bath, so I tackled a ripped bag of garbage. I was taking it into the hall and cleaning up the mess. I could hear Katlyn crying, but I just thought she was having a temper tantrum. Then her screams worsened. I realized that usually by this time she would have either stopped crying or come to me. So I went to investigate. I couldn't hear much because our HEPA machines are always going and are quite noisy. As I rounded the corner to the bathroom I realized the water was running. I soon as I got to Katlyn she grabbed on to me and pulled herself out of the tub. Her feet were very red, but I didn't really know how bad they were. I quickly grabbed a towel and wet it in cool water and wrapped Katlyn's feet in it. I proceeded to the tub and stuck my hand in and it was dangerously hot. I looked at Katlyn's feet and the skin was beginning to loosen, automatic blisters.
I had no idea what to do. She was screaming so loudly and I could tell she was in so much pain. John was at work and I'm not very familiar with burns. So I called 911. They came a short time later and brought Katlyn to the hospital. Once there they gave her pain medication and wrapped her feet. She has first degree burns to the bottoms of her feet and second degree to the tops. She was then airlifted to the IWK. She stayed there about a week and we are home now. A nurse comes in everyday to change Katlyn's dressings and they are looking very good again. She will need changes for a bit longer but everyday is progress. She has started to walk again but she still has a limp.
As far as Katlyn's cancer goes. I think it's looking like she will have her tumors surgically removed. I can't say for sure, but it seems like we are leaning in that direction. She will have an appointment at the end of the month is Saint John with the surgeon to see if he can do it and how he will go about doing it.
She is also set to have her IVIG treatment in Fredericton this month as well as a hearing test. Also at the end of next month she will be going back to the US for a checkup. Hopefully soon we will know what form of treatment Katlyn will get, and soon it will be underway.
Her immune system seems about the same. Her neautrophils are around 2000 and it's hard to say where her lymphocytes are. Her doctors think some monocytes are mistakably being counted, when they are actually lymphocytes. The number we get is around 150-200, plus possibly more.
She has been doing very well. She's been talking so well. Actually I don't even know where she learns the things she does. New stuff everyday. We have been baking a lot. I actually find it to be a huge stress reliever, and everyone seems to love the yummy freshly baked goods I make.
We are looking forward to Halloween this year. We will be taking Katlyn trick-or-treating to some of our families houses. Katlyn has never experienced any of that before so it is a new exciting experience.