I know. I know. I know. It has been ages since I have wrote anything about Katlyn. I think it is real time I got started at it again since it has worked so well in the past at allowing me to get all of these feelings off my chest. Things have been just a roller coaster over the past here. Here's hoping that 2012 is a great year for Katlyn. Here's hoping that she will finally get healthy.

So recently we had an appointment at NIH and things went pretty good. The much dreaded appointment was really going to be all about discussing how Katlyn's gene therapy is not working how it should, and what we are going to do as a plan B. Katlyn caught norovirus two years ago and she has been struggling with it ever since. Its at bay, but it just won't go away. It has really started to take its toll on her little body. She had been up to 57 lbs, and dropped down to 41 lbs. She is now anemic and does not absorb vitamin E. So Katlyn's parents, we decided it was time to do something about it, before the virus gets her very ill and its too late. Also, Katlyn has not been able to go to school this year. She is supposed to be in kindergarten, but it is just to risky to have her around so many children, so she stays home with me.

So Katlyn has been receiving IVIG orally. At first I thought it was outrageous!!! But it honestly has worked in making her feel better. Every time we put her on, she actually gains weight and doesn't feel sick all the time. Unfortunately it hasn't been enough to rid the virus from her gut, and she continues to live with it. So, our next plan of action is to put Katlyn back on Adagen. Hopefully that will be enough to rid her body of the virus. In the past we have always been told that giving Katlyn the Adagen would be deeming her gene therapy a failure. However, there is some new data that may suggest that there is a chance it may actually help the gene therapy to work. This makes us so hopeful!!!!

The problem with Adagen is that it is not available commercially and it is so so so expensive. Because it is not available in Canada, our insurance company said that they would not cover the cost. So our next approach was to ask the New Brunswick government for help. I was really shocked when they also said that they would not help with the cost. Typically this drug is covered under a provinces catrostrphic drug plan, but NB does not have one. So determined as we were, we appealed the insurance companies decision to not cover the drug. In the end they did indeed decide to cover the cost at 80%. At about $350,000/year, this would still be too difficult, but luckily there is a clause that says once our annual copay reaches $3000, the policy kicks in 100%. This is awesome awesome news!!!! Apparently we are supposed to come up with the upfront cost of the drug and then be reimbursed. LOL!!! Yeah, who has $30,000 just sitting around. Also, the $3000 copay would be on the first shipment. We still haven't figured out those little details yet, which stresses me out beyond belief. However, we did get most of it covered so far. I know its going to work out. I just keep praying!!!! We don't have that kind of money, even if it is $3000.

So once Katlyn gets this Adagen, she should be strong enough to get rid of the virus. This drug is a treatment for her condition, but not a cure. As her doctor recently told me, we need to look at it as a way to get her healthy enough to discuss the plan B. I don't even want to think about it. Every time I do, it just makes me so incredibly sad.

A bone marrow transplant.

For now, I am only going to focus on getting my baby healthy. Why she has to go through so much, I will never ever know. It seems so incredibly unfair. I am trying to just have faith in God. Its a challenge because sometimes I just feel so helpless, angry and sad. I have seen in the past though, that prayer is very very powerful. Now, she needs prayers more than ever. Happy new year everyone. May your best day of 2011 be your worst day of 2012.

It Sucks!!!!

I know it have been ages since I have shared anything. Really, we were very busy just living our lives. Just doing our best to be as normal as possible. However now as I sit here, writing this post, I know that we always be anything but "normal".
When Katlyn's doctor first diagnosed Katlyn with ADA SCID, he told us that there are many many other things that can go with the condition, most unknown. I really needed to know what he meant, and he went on to describe behavioral issues among other things. At this point, I remembered Katlyn had never actually passed a hearing test, so I asked him if it was possible that she be hearing impaired. We immediately preceded in getting her properly and fully tested, and sure enough Katlyn was hearing impaired.
Since then, I've always known that other things may come along, but I don't dwell on any of it cause there's no point. I just have a "we'll deal it with it when it comes" mentality. I must admit though, the last few years have been very very frustrating to say the least. I have never met anyone as special as Katlyn. EVER! This little girl loves life and just looking at her makes me so incredibly happy. Yet she is constantly faced with new challenges in life. It angers me that she was born with the condition in the first place,but here she is 5 years old, and we are still faced with the fact that she cannot just go to a child's birthday party for fear she will get sick.
We recently learned that Katlyn tested positive for norovirus, and it is apparent that she is having a very difficult time ridding the infection. For sure, she has had this infection since December. This is a very very scary realization for us. It is possible that Katlyn could get sick, and not get better. So while we were trying our best to live our lives as normal as possible and allow her to live outside the bubble, we had to put the brakes on. This year Katlyn was able to enter pre-school. Her class is very small, only eight kids, but kindergarten doesn't look so promising. We attempted to vaccinate Katlyn, but we were unsuccessful.
Because of this virus, Katlyn has dropped a lot of weight and lost her appetite. She went from a chubby little 57 lb baby, to a skinny 41 lb little girl. After all her weight loss, her medicine was not properly adjusted (there is a lot of it too). I believe that because of this, one of her medications has caused damage to Katlyn's liver. She was recently diagnosed with non-alcoholic fatty liver disease.
So on top of her having a poor immune system, being hearing impaired, and having cancer; she now has fatty liver disease.
I was warned in the beginning that other things would accompany Katlyn's condition of SCID, but nothing could prepare for how I would feel now. It saddens me to no end that she cannot just go to school with all the other kids. I thought we'd be past all of this by now. Katlyn is five years old, and her immune system is still a terrible threat to her.


AND IT SUCKS!!!!!

Off to NIH

I know that it have been way too long since I have been able to write on here. A lot has been going on since my last write up, which has made us busy busy busy. Well, for starters, I just started a new job, after not working for more than 4 years. I have to say. I really do love my job. However, i find it a challenge to be away from home all day. Since Katlyn and I have nver been separated since she's been born, it's torture to only see her for a couple of hours in the evening.
Katlyn is doing very well though. Her immune system is still not where we want it to be, but I can say that we have definitely started to live our lives outside the bubble. Katlyn has been sick twice, and both times she was able to fight it off. To be honest, it was very veery scarey for Johnathan and I. She was fine though, which shows us that she does have somewhat an immune system. And what Katlyn does have, works great! I don't really think twice nowadays if I need to go into a store, Katlyn comes with me.
On the other side of things, Katlyn recently had one of her tumors removed. We were not really expecting to hear anything other than we were successful in getting the whole thing. Unfortunately, that was not the case. We suspect that this tumor is probably bigger and deeper than any of her others. We went quite a bit deeper than we had with the last one, but still it was not deep enough. All borders of the part they took, are still positive for DFSP. So we go to NIH tomorrow so we can meet and discuss what our next step will be. Johnathan and I are still in favour of surgery if possible, as anything else could have a negative effect on her immune system. The way we see it, is that her immune system is the most important thing. Without it Katlyn would probably be very sick, and she may not get better. That is not a place we want to go. When you think about the possibility that everything we have worked so hard to achieve, could possibly be reversed because of a stupid chemo drug, it's very hard for us to think any other waay.
So off we go once again. To be honest, I have no idea what we are going to conclude. Is surgery even possible? Johnathan and I have discussed the fact that her first surgery was done in Canada by a Moh's surgeon and a plastic surgeon, and the scar at this point is barely visible. Unfortunately the second scar is not the same. She has a scoop in her skin from where they took the tumor. I think we're both in agreement that it's definitely beneficial having a plastic surgeon as well as a surgeon. I think they understand scars and how they will lok in the future, which gives us an advantage.
Katlyn is well though. She is definitely growing up. I cannot believe she is almost four years old! Wow! Where does the time go?
So I will do my best to get on here a little more often, to update Katlyn's story. Katlyn, you're my little angel, and Mommy knows everything is going to be fine!!!

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!!!!