17 Months Old!!!

Today we are celebrating 17 months with Skylar! It's crazy to think we've been blessed with a year and five months with her... I hope there are many more months to come!

So when I had Skylar 17 months ago, my mom gave us a pink Christmas cactus to celebrate the November birth. Around Skylar's one year birthday, there was one pink bloom, which I thought was appropriate since she was one year old. I wanted to share a picture of the cactus now :)

So to balance my past posts, I wanted this one to be full of pictures and more light and fun! I don't think I've shared Skylar's play time on the iPad with you yet... One of our best friends from college bought Skylar an iPad as a gift a while back and it's been amazing! Since Skylar doesn't have the muscle to move a whole lot, she is not able to play with normal toys. The iPad has made a huge difference because with the lightest touch of her fingertips, she is able to see a cause and effect. She is actually able to do something on her own and see it happen!

With a tap of her fingers, she can blow up balloons, create bubbles and pop them, play a piano, learn her alphabet and numbers, etc. We're always looking for new apps that would be fun for her. I actually downloaded Rock Band for the iPad (we don't have the Wii so the app is the next best thing) and Skylar is actually pretty good on the drums with my help :) Nurse Natalie and I have a good time playing with her.

Some good updates to share - Nurse Natalie is back and I am feeling better too! Skylar's belly issues seem to be almost gone and we're still working on getting in to see the pulmonologist. The good news is that I've found a home health care company able to supply the Trilogy Bi-pap for us and insurance should cover it. I'll keep you posted.

We've also been extremely blessed with three women who have stepped up and are consistently willing to pump for Skylar. That has been such a huge answer to prayer and we are very thankful for them (pumping is not a fun task).

We've also been very humbled with people willing to help our family in addition to the meals and breast milk donations. The 10K team for Skylar was started by a close friend and I just talked with an old friend from high school who wants to have a Chick-fil-a night for Skylar where 10% goes back to our family from that location (more details to come soon). A church in Dunwoody has been so wonderful with donating to us as well. We've also seen on our facebook fan page where companies have been finding out about us and voluntarily donating things in the event that we're chosen for the Extreme Makeover! It's so neat to see the word about our family and SMA spreading across the country. If nothing else, I hope we're chosen so that more awareness can be brought to this horrific disease and more research money can be raised.

That's about it :) Peace to you throughout the Holy week, Good Friday and Easter Sunday! I hope you are able to enjoy the weekend and spend quality time with the people who mean the most to you!!!

A Crazy Week

I don't even know what to say about this past week...

Monday, I was sitting with Skylar watching a movie in the afternoon like we usually do. I had just finished massaging her gums hoping to encourage those teeth to poke through. She usually swallows a lot of saliva when I do that so I "cough" her and pull up anything that might have gotten into her lungs. Afterwards, she's usually pretty stable so we just sit, watch movies and I will stretch her legs, ankles and feet suctioning her saliva out when needed.

Around 3 or so I noticed that her pulse-ox machine was running on battery power. I didn't think much about it because sometimes the cord wiggles loose so I just assumed that is what happened and would fix it later. Five minutes later, I went to turn on the suction machine... nothing. You've got to be joking. I flipped the switch frantically. I was home alone with no help this time, but I remembered I had back-up power and managed to remain calm. I ran to get the battery and inverter that we had used for our attempted trip to the doctor's office. I plugged in her bi-pap and the bi-pap was alarming and had a warning light flashing, but no error message or explanation as to why. Regardless, it wasn't working and I was on limited time where seconds count so I tossed that option aside. The back-up battery for the bi-pap didn't work a few days ago so I didn't even try that and went straight for the generator. Meanwhile, Skylar is happy watching her movie, but it only takes a second for her to choke. At the moment, the portable suction machine that has about 5-10 minutes of battery was my only defense.

I grabbed the key to the shed, ran outside, grabbed the 30 pound generator, ran not so fast back to the house and set it by the back door. I read the instruction manual once before, but I didn't want to mess anything up so I took a few seconds to flip through the pages to make sure I had everything right. Step 1: for a cold start, leave the choke closed and slowly open it up as the generator warms up - check! Step 2: check, Step 3: check and by step 4 or 5 the generator was on and running great. I plugged an extension cord in, green light - power is on! I ran in as soon as it started to get Skylar on bi-pap. As soon as I had the mask on her face, the generator slowed and the power on her bi-pap went out. You've got to be freaking kidding me!!! I ran out, green light is off. I tried to re-start the generator - nothing.

I called Kyle (who had ridden his bike to go do work) and told him to come home immediately - the power was out, every back-up option had failed and I needed help. I called my neighbor who was 5 minutes away and said she would come straight into our driveway. Meanwhile, I am about to have a heart attack because I am totally helpless and was tempted to call 911 just to get a vehicle out to our house for power. I kept repeating out loud, "God is in control, God is in control" to keep myself calm and sane. My neighbor pulled in (4 kids with her in her van - the oldest girl comes to read to Skylar every now and then) and helped me get Skylar into her stroller and then into the van. Thank God we have a little dinky inverter with a cigarette adapter that runs the bi-pap. Skylar was upset and crying, but at least stable. Kyle arrived minutes later on his bike, beet red, dripping in sweat, coughing and exhausted from his 10 minute ride home on a bike without working gears.

Minutes after that, the power had kicked back on and Kyle double checked to make sure everything was working. Seconds before we were going to transfer her back to the house, something happened in the car and the bi-pap power went out. I quick pulled her mask off, scooped her up in my arms, had kyle grab the pulse-ox machine attached to her and we ran in to do a cough assist and move the mucous/saliva that she was choking on. Once we got her stable and on bi-pap, she passed out for her nap. Kyle and I were right behind her for the nap, but I had a nice little emotional breakdown first. Poor Kyle had an extremely tough bike ride to get to us and wasn't sure what he was going to come home to. We both were so exhausted. I hate SMA. I hate the fact that we are so dependent on power, the fact that my daughter could die at any moment, and that there is nothing we can do to save her in the end.

So later that night, we found ourselves in the middle of a wicked storm and the power was flashing on and off for a little bit. Thankfully, it never went completely off, but we were ready to go back in the van in the event that it did. In hind sight, I think I flooded the generator. I left the choke closed to start it and as soon as it started I ran in the house to hook Skylar up to it and was going to open the choke when I came back out because I didn't know how long it took to "warm up" as it said in the instructions. Give me a computer, a stereo, a modem or anything electronics and I can set it up, but electrical and power stuff is not my forte. I don't know what was wrong with our battery and inverter. The inverter came with really wimpy wires and I don't think they were connecting securely to the battery because the battery was fully charged.

Lesson learned, we're going to do trial "emergency" test runs once a month to make sure everything is running well and working. We want to know this equipment so we could do everything with our eyes closed. We're also looking into a UPS (uninterrupted power supply) system so that we can keep her bi-pap plugged in at all times.

So after the storm, the next morning, Kyle was on his way to work and noticed a lot of emergency vehicles three streets over. As he got closer, he came upon a vehicle that was smashed completely in half due to a huge tree limb falling on it. The driver had to have been killed instantly because the bough was right across the seats. The driver ended up being a young guy in his early 20s visiting a friend for coffee. There was no warning. He was just driving down the street. It's amazing to us how the timing of things causes events to happen - good or bad.

It's rather mind-boggling to think back through events and ponder, "if only..." and the turn of events could've been completely different. Some attribute it to fate, to coincidence, but I like to think there's purpose to everything - even something as tragic as that incident. Monday, we could've lost Skylar. The driver of the 18-wheeler that knocked out two power poles causing a large chunk of west midtown to lose power had no intention of sending me into a panic that day and doesn't even know the effect of his accident. If that incident would have happened earlier or later in the day, Skylar could've been a lot less stable and we may have lost her. GA Power was awesome yet again and had it fixed within 30 minutes. Had it taken longer, Skylar might not be here. That driver who lost his life from a huge tree branch falling on his car could've survived if he had taken longer to say goodbye to his friend or any other event he did that day. Seconds. It truly blows my mind. Even with our belief in God, it's hard to understand things like this and I don't think anyone ever fully will. We know we live in a broken world where things aren't perfect and thank God that it was not Skylar's time to go to heaven last week.

Below is a picture of Skylar with her bi-pap on with covered in her new spring blanket that was given to us by Blankets 4 SMA and our new owl pillow in the background that was hand-made by a good friend!

Anyway, it's taken a while to process everything. Plus, we've been busy with work, taxes (yes, we wait until the last minute), gardening and other random stuff. Tessa, our awesome photographer, came back to take pictures of our family again (since it's been almost a year since our last photo shoot) and we're so excited to share some of them with you! You can see the rest on her blog. It was a little sad because Skylar didn't smile at all - she was extremely tired and was ready for a nap, but Tessa is so talented and we're really happy with them.

I have been doing a lot more photography myself and am trying to find a balance of the amount of work I can handle while still getting enough rest and taking care of Skylar. For the first time in two years, I am sick. Our nurse has been out for over a week and a half now which has been difficult since we've been spoiled with her, but we're managing. It couldn't have come at a worse time since I am trying to keep out of the nursery as much as possible so Skylar doesn't get sick. Thankfully, all of Skylar's grandparents are close by and are able to come and at least sit with Skylar so she's not alone, even though she has to stay on bi-pap all day. Please pray that Nurse Natalie will be able to come back and I'll get well soon and no one else will get sick!!!

Hopefully my next update won't contain any emergency situations and will be full of good news :)

One last thing for all you runners out there, a good friend of ours is organizing a 10K team for Skylar for the race on May 21 in Grant Park. It's called "GeneRACEtion" which I thought was really cool because "gene" and "race" are in the title and we're hoping gene-therapy (among other things) will help us win the race for a cure for SMA. Also, the race benefits a senior citizen group and Kate’s Club, which is a non-profit organization that empowers children and teens facing life after the death of a parent or sibling. Who knows, it could be from SMA! Anyway, for more details, check out the awesome website our friend put together.

Peace.

March Madness

So the below quote block was what I had written on March 12 and had every intention of posting it then, but somehow never got around to it. This is a warning that this post is really two or three posts in one so grab a cup of coffee!

March 12: "It's been a rough week. Kyle was out of town on business and as soon as I posted the last summary of how well we were doing, Skylar started struggling. In fact, her mucous was extremely thick and you could tell she was uncomfortable. She was in and out of sleep all day and night and was so miserable. Her heart rate was super elevated, but there weren't any other signs of illness - no fever or anything. After a day or two of that, her stomach started acting up too. It was all very similar to what happened right before Christmas which I summarized in a blog post early January.

Her stomach stopped digesting and her residual (the food in her belly still waiting to move through) was growing in volume. We had to slow down her feed rate so that we didn't overflow her stomach, but then she lost calories, nutrients and fluids. Then after not getting enough nutrients, her body doesn't function as well and it's just a downward spiral. When I noticed her residual backing up again, I immediately called our pediatrician and Dr. Swoboda's team out in Utah and spoke with a nurse to help guide us through this time. We agreed that pedialyte was the best option and started her on it right as her residual started to change colors (bile starting to show in her belly). Before Christmas, it only took 48 hours to "reset" her system with the pedialyte and things were back to normal. This time, not so much.

She definitely started feeling better after two or three days (you could tell by her alertness and lower heart rate), which I was thankful for so my family could see her when she was comfortable despite her belly having trouble digesting. Throughout the week, we had to take her down to such a low rate that we were worried we might end up in the hospital from dehydration. The nurse in Utah has been really encouraging and helpful as well as our pediatrician. Without them (and nurse Natalie), I might have had an emotional breakdown because I was without Kyle all week trying to deal with everything. I've had to call home health care providers to figure out orders that got messed up and get supplies that we needed over a week ago, United Health Care for insurance issues, Pediatria for billing and other issues, and it's just one thing after the next. Thankfully I can do most of that while Natalie sits with Skylar so I am not neglecting her, but it can be overwhelming and definitely frustrating. I was so happy to have Kyle come home safely and have his support - even something as simple as a hug to make me feel better. He surprised me with some down-filled boots to wear around the house to keep my toes warm :)

Anyway, our pediatrician ended up coming over last night to check on Skylar. As much as I can tell him things over the phone, it helps for him to be able to feel and listen to her belly first hand. I have said this before, but our pediatrician is really one of the greatest doctors ever. We've been so blessed by his willingness to help our family and are so grateful to have him in our lives.

For now, I think we're on the road to recovery and are still keeping a close eye on her belly functions. We have a few things to try to help Skylar completely recover, but it is hard because we don't know what is causing this. It could be a stomach bug - most kids would be throwing up and it would be over with, but Skylar can't vomit and it could just be staying in her system. It could also be that her belly is just slowing down due to the progression of the disease (but I don't think so since this happened before Christmas and we pulled through). However, with SMA, it could be a number of things so it's just a big guessing game with trial and error. Preferably trial and no errors. We just continue to take things one day at a time - sometimes an hour at a time - and focus on the positive. I don't know how you could function otherwise.

It's been a rough week for a lot of SMA kids - families in the hospitals, others dealing with sicknesses, etc. It seems that most are on the same schedule that we are and are hoping to be back to normal soon. We're optimistic and hope Skylar is back to her normal self this coming week. It was encouraging to get a little giggle out of her last night. Hoping for many more to come!"

So all of that was written on March 12 - and to be honest, all of that has been true for the rest of the month. Kyle was out of town for two more weeks (he normally doesn't travel that much, but this month was unusually busy), Skylar is still not back to 100% with her belly, but things are getting much better with a slow and steady recovery. Nurse Natalie, the doctors, family and friends have been a great support. Unfortunately, several other SMA families around the globe are still fighting and struggling to keep their children alive - one 6 year old boy was lost last week. I've been thinking of these other families so much - sorry for their loss, the struggle, thankful that we're surviving and doing well, dreading the day that we'll be in their place. Hopefully that day is far off, but if we've learned anything, it's that none of us are guaranteed another day on this earth so we take things a day at a time and try to enjoy every moment.


Despite all of the craziness of this month, Skylar managed to smile for a St. Patty's Day picture. I am glad Nurse Natalie and I haven't been betting money on those teeth or we'd be broke by now. They are so right there, ready to poke through!!! You can sort of see in her picture that she's developing a nice mullet. I really don't like mullets but I don't have the heart to cut her hair because it took so long to grow :) I don't know what her hair in the middle and on the sides is waiting on, but Skylar's got double mullet with a party in the front and in the back...

In other news, last Friday, I was able to have the extreme privilege of touring the SMA research lab at Emory last week thanks to the Hope and Light Foundation and was fascinated by what I learned! Emory has a great program - it is right around the corner and it is nice to be able support something local. I am going to be partnering with the Hope and Light Foundation in their fund-raising efforts for the Emory lab and the gene therapy going on at OSU by Dr. Kaspar and will continue to help them with their graphic design needs. Hopefully more updates to come soon with all of that.

While at the Emory tour, I had the honor of meeting Dr. Brooks - pediatric pulmonologist - who took time out of her day to come to the tour to learn more about SMA. I have made it no secret that we've not had great experiences in the pulmonology department throughout our journey, which is so unfortunate because that is where SMA kids need the most support (and parents too). After meeting Dr. Brooks, and realizing how awesome she truly is, I called the office manager and was able to get Skylar switched over as a patient of Dr. Brooks (something they would not let me do in the past). This was a huge success!!! Since last summer, after a horrible experience at the office, I have been calling and canceling Skylar's pulmonology "check-ups" because she's been healthy and we don't get any advice other than, "go home and keep doing what you're doing - she looks good." I've tried calling Dr. Schroth (the SMA pulmonologist specialist in WI) to get advice over the phone, but since we're not a patient, their office won't return my calls. Anyway, we're so excited for our first office visit with Dr. Brooks!!!

Today we attempted a trip out to see Dr. Brooks, but failed miserably. It was right around Skylar's nap time and she was extremely tired so we kept her on bi-pap and planned on using our new portable power. The back up battery that we had for our bi-pap wasn't holding a charge, even though I had charged it two days before... Then, the portable power we got for her stroller worked, but the bi-pap plug did not fight tightly in the outlet and would wiggle loose - which would then cause her bi-pap to power off and alarm, Skylar would be unable to breathe until it kicked back on, and she was getting really stressed out. This happened multiple times, which caused me to get stressed out and say some really not nice words to her machines! I just felt so bad for Skylar because all she wanted was to take a nap and instead, she was having to panic for air randomly and without warning. We finally got loaded up in the car and down the street when I called the doctor to let them know we were running late. Since we were the last appointment of the day, and running over 20 minutes late, the doctor was not going to be able to see us because she had to go pick up her kids (totally understandable). When I explained what happened, she asked why we didn't have a trilogy (the mercedes of bi-paps that has a built in battery) and I told her that our home health care company wouldn't buy one for us because of the cost. She said that's unacceptable and would work on it so that we could have one. It feels so good to have someone else in your corner. Fighting home health care companies, insurance companies and other people who are just looking at you as numbers and dollars is exhausting! Again, a huge and overwhelming good feeling about having Dr. Brooks taking care of us and fighting along side of us!!!

Since we were already in the car and had just worked for over an hour to pack things up and get Skylar stable and comfy in the van, Natalie and I went and treated ourselves to Chick-fil-a milkshakes because that will make most bad days better :) The good news is that we're rescheduling her doctor's appointment soon and since Kyle won't have to travel as much this month, hopefully he can join us to meet Dr. Brooks too!

I'll keep you posted and I'll also leave you with one of my new favorite quotes:

"Although the world is full of suffering, it is full also of the overcoming of it" ~ Hellen Keller

I was reading Kyle my post and he finished the quote above before I could finish saying it and then said, Hellen Keller... and I just looked at him. He said he learned that quote after he had been struck by lightening, which I thought was really cool that I stumbled upon it recently to look to as positive encouragement for our current situation. Anyway, more pictures to come in the next post :)

Peace.