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.

Major Updates!

Well, don't get too excited because I have no news to report on the shutterfly contest or extreme makeover, but I still have some major updates! Thank you to all who have already "liked" our "ABC Extreme Makeover - Pick The Jones Family" fan page and written comments on how you would be willing to get involved. If you haven't done so yet and you're on facebook, make sure you like our fan page and write if you'd be willing to help in some way... the link is to the right!


This is Skylar's new knit hat made by a good friend of ours. Sometimes I feel like I get a glimpse of what Skylar might look like as a young lady - this was one of those moments. Simply stunning.

Okay, on to the updates :)

First off - we had some family come into town all the way from Maryland to visit/meet Skylar which was very exciting! Uncle Tad had been here before with our cousins, but this was Aunt Kim's first time meeting Skylar and it was such a treat! We were so happy to have them and enjoyed some great meals together. We didn't have Skylar's room quite put together by then and they really wanted to buy her bedding for us. I had already picked it out, but the Target near us only had the picture of what I wanted - not the actual quilt for her big girl bed. They didn't have it online either. Uncle Tad and Aunt Kim found it in the suburbs along with some other super cute things and surprised us with some awesome goodies!!!


Not only did they find the bedding, but they found a matching rug and the canvas print to match that we had seen in the store picture. I was so excited to have her room finally come together. We also got some additional fun pillows for her bed and it's just the cutest little girl's room now :) We have some ideas of what we want to do with the walls in her room, but I am not sure if we'll get around to it or not... Reese has completely adopted the rug in Skylar's room as his own and could lay there forever I think.


Second big update - we're letting go of Hospice for now. Skylar has been so stable the past few months that we don't really feel like we need their services (especially since Nurse Natalie is here). We love everyone on Hospice Atlanta's pediatric team and will miss them for now, but it's nice that we don't need them. Now it's just a matter of getting insurance stuff taken care of so we don't have to switch out all of our equipment again (a royal pain). I'm not too sure why we don't have a case manager still but we're working on it. As long as we get the supplies we need on a regular basis still, we should be okay!

Third - we're in the planning of another family outing! We ordered a battery and inverter for her stroller and hope to test it out this week or next with walks around the neighborhood before we go far from home. The aquarium was so much fun that we are hoping to do it again. I am not sure if we can get to the aquarium again, but we're working on a trip to the zoo since the weather is starting to warm up! We even got her a dvd about zoo animals so she could learn about them before our trip :) A wonderful woman from Children's in Atlanta is helping us get a special visit worked out and she's also going to help me try to get some awareness for SMA - such a blessing!

Most of you know that I, along with three other awesome ladies, started the GA Chapter of Families of SMA last fall. We are excited to announce that we're planning our first big fundraiser - a Walk-n-Roll event for early Summer (hopefully June). I wanted to give you a heads up in case you would like to come out and walk (and/or roll a stroller or wheelchair) or be a sponsor or donate money - more details will be coming soon with the exact date, location, etc. so stay tuned.

Health Updates - we've had some random health issues come up between me and Kyle. Last month, my extremities would get wicked cold and my toes would turn purple and my fingers would turn white (Raynaud's syndrome). My toes would actually break out in hives due to an allergic reaction to the cold - completely random! In addition to the circulation issues, my heart was fluttering and I was getting concerned that something was wrong with my heart. After some blood work, I found out that my thyroid is hyperactive and all of these things could be related. Still working on getting that resolved, but at least my heart should be okay. The weirdest part about it all was my blood work in November showed everything to be completely normal so this all was very random. A few months ago, I pulled some tendons in my wrist and those are not healing because I can't give them the rest they need caring for Skylar. Also, Kyle found out that a nerve in his tooth is dying even though his tooth doesn't have a cavity and he'll have to get a root canal. This may seem trivial, but for us, it's annoying and one more thing to add to our plate with more doctors appointments and things to think about. However, I will say, we're extremely thankful that we've been able to stay healthy from illnesses and not come down with anything that is contagious!!!

Skylar's teeth are still fighting their way through. I actually can feel the edges of her four front teeth and can feel a molar trying to poke through, but nothing visible to the eye yet... Natalie and I really thought we would've seen them by now, but now we're guessing by the end of March! Sometimes she's really uncomfortable and I can't tell if it is because of her teeth or something else. With SMA kiddos, it could be a number of things. Lately though, I am guessing it's her teeth because when she's off bi-pap, I can give her a gum massage (sometimes for over an hour) and she's happy as a clam.


Here is a picture of Skylar with her "sister" Anna. They've been sharing breast milk for months and months now. I definitely think the breast milk has been a huge part of Skylar doing as well as she has been and am so grateful for this family and their willingness to share. For Christmas, I got Anna a matching sleep sack so they could be twins for a day. They're the cutest girls ever!

One last thing - Skylar has moved up to size 3 diapers!!! I don't know if any of you use diapers.com to order stuff, but we are going to start ordering all of our diapers and formula from them! Not only do they give you free shipping on orders of $49 or more (easy to do) but they deliver in two days and there's no tax! The Alimentum formula that we use for Skylar is pretty expensive and so far, diapers.com has the best deal on it. If you're ordering diapers for the first time, you can use our code SKYLAR10 to get $10 off. If you're ordering for the first time period, you can usually save an additional $10 with promo codes.

This week I have some family coming into town - my aunt and uncle and 92 year old grandma! She's awesome - you would seriously guess she was in her 60s... I think the secret is doughnuts and miller lite, hahaha. Anyway, they were all here when we thought we were going to lose Skylar over labor day (we actually had to ask them all to leave through our tears), so I am super excited they have a chance to come back to visit and meet Skylar again when she's doing well and we're all in good spirits. I'll update you next post on our visit together.

I hope you all have enjoyed the warmer weather. We're excited for spring. I haven't seen the sun in over 1.5 years and got my first sunburn Thursday when I sat outside on our back patio for lunch with my mother-in-law. Pretty hilarious farmer's tan... I should know better. Now we're back to winter weather, but it was a nice teaser to remind us that Spring is coming soon!

That's all for now. Peace.