Thursday, July 31, 2014

Fears & Tears

It's less than 5 days now until Olivia's little belly will forever look different. It's such a short amount of time, for one minute I feel all too rushed and at the same time, I knew this was coming, I've had months, and years really to somewhat prepare, having this brought up at every single visit when the scale didn't tip in Olivia's favor. It doesn't make it any easier. As with most things, the closer something gets the more panic, anxiety and uncertainties about it all set in.

I still don't know that we made the right choice to go forward with the g-tube and when I say choice, even though I know it was ours to make, I still don't feel as if we really were given a choice to make. I don't think we will ever know if we made the right decision even after surgery is all said and done with. Should we have waited, insisted they give her more time to gain weight? I don't know, but as Jeff and I have been hashing it over we know that right now she has some wiggle room, her weight is okay and it's not a dire need and we would rather have the tube placed now than wait and have her weight really falter where the g-tube is really a last resort. As with so many things with CF, there are so many uncertainties, difficult decisions to make, battles to fight . . . days where you feel like you rob Peter to pay Paul.

Jeff and I get it, we know that placing the g-tube now while Olivia's weight is "okay", gives us options and flexibility. We have time to get her to an ideal weight before she goes to kindergarten so that she can thrive at school. We could go to Disney World, on a vacation or camping without having to lug all the tube feed supplies along and just not do night time feeds for awhile. We've been told over and over that CF is to fit into our lives, not the other way around. Some days I chuckle at that statement, as there are days that are so CF consuming that it couldn't be more untrue.

Yesterday, Darci brought over some G-tube pads and a tummy belt. The pads take the place of gauze and tape around the g-tube, they are absorbent, softer on the skin and are said to help with granulation tissue and the belt is for routing and securing the tubing during night time feeds. One of Darci's friend and co-workers bought them as a gift for Olivia. How sweet is that? I cried when Darci told me about them, what an amazing act of kindness, something to make a difficult thing better. The pads have Anna and Elsa on them. As sad as it is for me to know why they are needed and the changes that are coming, Olivia loves them, she clutched all 4 pads in her little hand this morning while doing her vest/nebulizer treatment. So each day she can pick a different pad to place around her g-tube, it'll help decorate her belly, help with the g-tube sight itself and give Olivia a choice and something to maybe look forward to in a situation that isn't so fun and in a cf life where she doesn't always get so many choices.  It was with a heavy heart that I ordered up some more this morning, one that has a kitty face that Olivia will enjoy I am sure. Thanks Ryan for the gift and also for bringing these to light for us, I had no idea there was such a thing and these will be great. They'll give Olivia a choice involving the care of her g-tube and Anna and Elsa from Frozen - seriously, doesn't get better than that.
Extremely Limited quantity!  FROZEN G-tube mic-key button feeding tube protective belt
Fashiontubies on Etsy - Frozen Protective Belt & G-tube Pads

So it's that time, time for the anxiety to set in, the mixed emotions, fears and tears. Time for me to think and ponder those things that will be different in just a few short days. The sweet little belly that I blow on that will look so different. The little girls bedroom that will look a little bit more like a hospital room during the night. The realization that I won't have a little lady trying to crawl into bed with me when she's scared because she'll be hooked up to a g-tube during the night and confined to her bed. Little things, silly things, but none-the-less still a difficult bite to chew.

As I feel sorry for myself, frustrated at the future, mad as all heck at CF right now. . . I still know it could be worse, so very much worse. I know that this doesn't have to be a forever thing, I know that when we take the tube out someday the scar will be so minimal compared to the one that already adorns her belly from her NICU stay and surgery during the first few weeks of her life. Change stinks, it's hard to adjust too at first but we will get there. I'm sure there will be challenges, I'm sure there will be things I hate at first but, all in due time. As if I ever need to be reminded of what this disease is and does, this is yet another reminder, a slap in the face of all the havoc CF wreaks on one's body.

And then I have my sweet little best bud Landen, wondering, questioning, worrying . . . asking why he can't come with to the hospital, telling me repeatedly that he'll miss me while tearing up. He's such a trooper. We are a tight knit family of 4, Landen has been to most everyone of Olivia's CF appointments with us, so he just doesn't quite understand why he can't come to this one . . . it's hard to explain to a 7 year old, even harder for him to grasp the changes that are to occur. Very rarely are we ever away from the kids, especially over night so tears are sure to be shed by all. I thank my sister Darci and niece Braelyn for agreeing to move in to the house while we are away. They will take care of Landen, our home and trusty old Shelby who is miraculously still kicking. It'll be great for Landen to have Braelyn to play with as he is so used to having a sister around to play with everyday. Thanks also to Grandma Pam, Grandma Dianne and Aunt Jennie for offering to help out, Landen is sure to enjoy spending time with everyone of you. Since we can't take our best bud with us, it's great to know that Landen will be in the comforts of home with people near and dear to us. Thanks to everyone for your thoughts, words and prayers, we are blessed to have such wonderful family and friends in our lives.

Tuesday, July 29, 2014

G-tube & CFRD

Yesterday Olivia had her follow-up appointment at the CF Clinic. It was such a long day but at least we were able to get everything done in one day that needed to be done. We started out the morning meeting with the pediatric surgeon to go over the g-tube surgery and placement. We already knew a bit going into it so most things were expected. We learned that the g-tube surgery itself only takes about 30 minutes. The hospital stay they said for a child with cf who uses the Vest machine for airway clearance is more typical of the 3-5 day range. The reason for this is because the g-tube placement site will be painful and we have to be able to do the Vest machine while managing pain before we are released as the vest will cover the g-tube area during treatments. Obviously Olivia does her Vest machine now 2 to 3 times per day to keep her lungs clear and as healthy as possible. If she can't do the Vest treatment after surgery, because of the surgery itself as well as having a g-tube the potential for pneumonia to develop exists and that will just be so not good. We can't "fix" one problem and have her lungs falter because of it. Therefore, 2 to 4 days before the surgery, we have been instructed to do Vest/nebulizer treatments for 3 to 4 times/day to make sure her lungs are in the best shape possible before going in for surgery and the hospital stay.

After this visit we hopped over to another clinic for the Oral Glucose Tolerance Test (OGTT), the Center for Children with Cancer and Blood Diseases and infusion services. Let me tell you, being in this clinic really put things into perspective for me, again realizing that things could be so very much worse. Tears were shed on my part for those kids that were there for reasons so very much worse than us. As I stood there looking at all the hats that were donated for the peds cancer patients, I realized that it is a must for me to knit up some fun, modern, brightly colored hats to hang on the donation rack. Bless all those who donate these items along with their time for these kids, but as I noticed one little girl with brightly painted nails and toes look them over and pass, I thought maybe she'd like something brighter and I realized that when I get a spare moment, that will be my next "to-do".

By this time it was already 9:30, Olivia was still fasting with nothing to eat or drink. We were set up in a room and Olivia was prepped for the OGTT. They first wrapped her arms in warm blankets, awhile later our practitioner Lauren came in to find a vein to place the IV for the repeated blood draws for the test. She located one in the crook of Olivia's left arm, then used a j-tip (needle free injection system) to immediately numb the area and place the IV. Olivia didn't feel any pain and it was inserted and over with in no time. Thankfully, the IV was in her left arm so she was free to color and color and color some more for the remaining 2 hours, this girl loves to color! She had a total of 5 blood draws, an initial one to establish baseline and then she drank the glucose drink and after she had downed that, she had 4 more blood draws every 1/2 hour to measure her glucose level. She was a trooper, like she always is. By noon and after 5 blood draws and nothing to eat or drink since the previous night besides the glucose drink, she was looking pretty rough. We gave her a snack before we left to make sure she was okay and then I carried her out. We had a 2 hour break until our last visit of the day so we grabbed some much needed lunch and almost immediately after getting some food in her belly Olivia was getting back to her energetic self. We made a quick stop at Target to get both of the kids a special toy for being so good and then headed back to the CF clinic for our routine follow up visit.

During our 8:30 visit with the surgeon, our favorite nurse Emily was there so she did Olivia's sputum culture at that time so Olivia didn't have to fret about it for the rest of the day and they also grabbed all of her vitals. She grew about an inch and gained a little over 1 lb so she is still growing. We also found out the results of the OGTT that we had just a few hours prior at this visit and thankfully, Olivia does NOT have CFRD. That was a huge relief to hear. Olivia is still growing that is for certain, her BMI continues to go up and down but pretty much averages out around the 25th percentile. The goal is and always has been to have her at the 50th percentile. She will turn 5 in October, she will go to kindergarten next year, Landen will be in 1st grade this year and is sure to bring home plenty of sicknesses that they will share. As has been the pattern for us, the winters are hard and terrible for Olivia. She gets sick, she fights them off like a trooper but her weight suffers greatly. When she loses so much weight during bouts of repeated illnesses, it's nearly impossible to make it back up. So much of our days are spent worrying and fretting over food and calories and "eat this, drink that", it's just not fair. Not fair to Olivia to ask us, "Did I do good" after her meals, not fair to Jeff and I to stress out at every meal and snack time over calories and how well she eats. CF in general isn't fair, neither is life.

So it is of course with heavy hearts that we will proceed with the g-tube but also knowing that as Olivia approaches kindergarten she needs to be at an ideal weight, at an ideal BMI so that when she does get sick, she can fight off those sicknesses with the extra calories she needs to do so. She can't do this now because she doesn't have extra calories and pounds to spare, so Jeff and I along with our entire CF team have decided that a g-tube will be the next step in continuing with Olivia's CF care.

Jeff and I knew going into yesterday's appointment that the probability of Olivia having CFRD was very low and that the likelihood of proceeding with the g-tube was very high. We've had 3 months to mull it over, be mad at the world and eventually come to accept it. We know that this isn't a failure on our part. We know that this is to help Olivia and we know that our team wants what is best for Olivia and us. So, Olivia will be undergoing g-tube placement surgery at the University of Minnesota Amplatz Children's Hospital on Monday, August 4th.

The surgery itself will be about 30 minutes. Both Jeff and I are able to stay with Olivia until she is fully asleep, right before surgery. We will then wait in a waiting room during the procedure and after she is done and out of the 1st stage of recovery, the 3 of us will be admitted to our room for the remainder of our stay. Jeff and I will be educated during our entire stay on how to do all things that are necessary for the g-tube care and feedings.

Each night, Olivia will be hooked up to a feeding pump via her g-tube and will be given formula over about an 8 to 9 hour time period. The formula she will receive during these nightly feeds will be about 1/2 of the total caloric intake she needs daily. Therefore, during the day, she will be able to eat freely when she feels like it without feeling pressured to always eat more. On her belly she will have a g-tube button, it's bigger than one would hope but all things considered, it could be worse. It's similar to a pool inflatable air plug. It opens and closes and has a valve that when it's hooked up to the feeding pump, food will flow in and when it's closed, she will be just like any other kid, free to do whatever, whenever without it causing any problems or limitations. Once the surgical site is healed, she'll be able to swim and play just like she has been. The button itself will be changed out every 3 months and both Jeff and I will learn how to do that on our own at home.

This is similar to the g-tube Olivia will have.

This photo is courtesy of the CFF Supporting Nutrition brochure to show the size of the button that will be placed on Olivia's abdomen and what you will see on the exterior of her body. 

So, that is that. A lot to take in, in one day but Jeff and I both knew that this was the road our journey was heading down. We have accepted this path of our journey and always want what is best for Olivia and know that this g-tube will help Olivia grow to be the best her that she can be. We have much to do in less than a weeks time to prepare for the surgery and our hospital stay so that is all for now.

Tuesday, July 1, 2014

Vertex Clinical Trial Results - My 2 Cents

Last week, as I was scanning my inbox I clicked on the letter from Robert J. Beall regarding the results of the phase 3 clinical trials of ivacaftor and lumacaftor in cf patients with 2 copies of DF508 mutation, which are the mutations Olivia has. I read through it and was filled with mixed emotions really. The news is beyond hopeful, super amazing, something to celebrate without a doubt. I feel as if prayers are being answered, I feel like wishes and dreams are coming true. I know this news isn't 'the cure'. I know this drug combo for those people with cf who have 2 copies of the DF508 mutation is not even close to what kayldeco is for the 4% of the cf population that it serves. I know that even though this news won't impact or have an effect on Olivia's care now, the promise of a better future is real.

I know that . . .

over 4 years ago as I walked out of the NICU for the last time, phrases such as "boys seemingly do better than girls at managing this disease", "18 years of age", "the first 5 years are critical" . . . so many other daunting phrases were ringing in my ears.

I know that weeks later, I sat in a small, dark clinic room with tears rolling down my face, clinging to my baby girl, my precious daughter who I had just recently discovered had a disease . . . a rare, genetic disease - cystic fibrosis and the outlook was not so hopeful. As I sat there with our complete team of individuals all trying to explain cf to me, what it would mean for Olivia, for Landen, for our family, it was all so new to us, there were so many unknowns . . . and there was no mention of a cure. I was told that Olivia's mutations, while being the most common, are also the hardest to "fix". I was told that trials were occurring for other mutations, ones that were easier to "fix", but that again, didn't seem to offer much in the way of hope for my daughter.

Now, almost 5 years later, I see things differently. In just 5 short years, things have changed so much with cf.

I know this . . . to have anything at all with the potential to treat the underlying cause of cf and to alter the way the DF508 mutations work, this is so amazing.

To have lived, for over 4 years now, in a life impacted by cf, day in and day out, seeing what this disease does to my daughter, to our family, feeling at so many times that we are fighting a battle that can't be won, feeling like this disease is getting the best of us - this news to us feels huge.

This news is hope.
This news is so much more hopeful than anything I heard just 4 short years ago when Olivia was diagnosed.
These results are a great start.
This is progress . . . we are gaining ground . . .

 . . . we are closer to a cure now than ever before.  

These are the reasons we support the CFF 100%, why we fundraise, why we donate, why we beg and plead and ask others to help us by fundraising and donating as well. This is proof that money buys research, and research will produce the cure. I hope every single person out there who has donated to the CFF, feels the power that our donations have in helping those who are inflicted with this disease.

Any amount of progress, no matter how big or small, gets us one step closer to the day Olivia and so many others can be alleviated from the havoc this disease puts on their bodies.

Better things are here, even better things are coming. I anxiously await the results of the VX-661 and ivacaftor combo study for those with two copies of the DF508 mutation.

We are making progress,

a cure is lurking,

I have faith that my dream will come true.