Friday, February 28, 2014

Rare Disease Day

Happy Rare Disease Day!  We're all wearing our specially-made Rare Disease Day shirts and would love to see you in your blue as well!  Email me at or tag me on FB with your pictures and I'll do a Rare Disease Day recap!

Yesterday, Caleb got on the morning broadcast at his school and shared about Rare Disease Day and why it is important to our family.  He encouraged everybody to wear blue, so we're excited to see how many of his friends are "blue" tomorrow!  The broadcast sparked a conversation in his class about what rare diseases are, what kind of disease Abby has, and how to talk to kids with special needs.  Caleb's teacher shared with me how cool it was to see a first grader sharing and teaching his classmates about diversity in a way that adults never could.

I was bursting with pride for my boy!  He truly does have a unique perspective as "the brother," and I'm glad he views it as a great opportunity to share with others, rather than something of which to be ashamed.

Rare Disease Day has always been important to us, but it's doubly-so now that Abby is doubly-rare!  While Thoracic Insufficiency Syndrome isn't quite as rare as Cerebrocostomandibular Syndrome, the chances of sitting next to someone at the movie theatre with it is pretty slim.  The VEPTR is FDA approved as a humanitarian device that benefits patients by treating less than 4,000 people per year.  At this point, the VEPTR has been used on "hundreds" of patients.  

Soooo...Happy Rare Disease Days, and share those pictures!

Tuesday, February 25, 2014

Rare Disease Day THIS Friday!

Rare Disease Day is THIS Friday, February 28th!  I can count Abby as double-rare, now that she's also a VEPTR kid!

I would absolutely love it if you would honor Abby by wearing blue on Friday.   We got tshirts made this year that are super cute, but I'm going to save them for the grand unveiling on Friday.  :)

If you wear blue, take a picture, either post it on Facebook or email it to me, and I will spread the Rare Love on my blog!  Thanks for supporting Abby and all of the other Rare kids!

Monday, February 24, 2014

Medical Update and lots of prayer requests!

So much has happened and changed in the last week...where to begin?  

We saw our feeding therapist last Tuesday and decided to end therapy until after surgery.  This made me SO sad because Abby has made so much progress in the last eight months, but we really have to focus on getting as many calories as possible into Abby without burning more chewing higher textures.  We have met with a nutritionist several times last week and she gave us some good ideas.  Our goal now is 1,800-2,000 calories a day...typically kids eat 1,000-1,400.  The feeding GI (whom we love) met with us to discuss putting Abby on an appetite stimulant.  They had always been reluctant in the past, but now everyone agreed that this was the best thing for her.  She's been on it for 3 days now and we haven't seen much of a difference, but we are still working her up to the full dose.  

Her weight was down at that appointment, and I was so concerned.  How could she be losing weight when she's getting so many calories?!  Thankfully, we weighed her again on the same scale at our pulmonology appointment on Thursday, and it was up.  (Then we weighed her 2 more times just to make sure!!)

When we met with our pulmonologist, she had Abby do a walking pulse-ox test.  We took a brisk walk, holding her hands to keep her moving.  We saw that her oxygen levels were great in the beginning, then dropped as she lost her stamina.  We were able to get them back up by encouraging her to take deep breaths, but it is still concerning that a brisk walk causes her to drop.

Our pulm is typically pretty conservative, but after reading the reports from CHOP and talking with us, she agreed that this sounded like our only option.  She did give us some great questions to ask and things to keep an eye on during post-op.

On Friday, we officially scheduled Abby's surgery for July 29th.  Matt gets home from Momentum on Sunday night, we leave Monday morning, and surgery will be Tuesday.  The good thing is that Matt will be able to stay in Philly through the weekend before heading home for Absolutely Awesome (a middle school program).  He'll hopefully be with us through the "worst" of it and there is always the possibility of coming back if it's truly necessary. It would be much more difficult for him to get back if it were one of the weeks he was away on a trip.  

It looks like we will be back in Philly in May for the dynamic MRI, scans, xrays, and lots of meetings with various doctors.  A few weeks before that, we will have to meet with the anesthesia team to make sure that everyone is on the same page as far as Abby's airway is concerned.  While it's inconvenient to have to go up there a separate time, we appreciate that they are concerned and want to make sure they have everything carefully orchestrated before she goes under anesthesia for the MRI.  We don't mind the drive!

A few months ago, I talked with a friend whose husband was dealing with cancer.  We chatted about how it is easier to write about medical stuff than our reaction to it all.  That's about where I'm at right now.  I can give facts, I can explain to people about how the surgery will be done, I can talk about the subsequent surgeries...but I lose it when someone asks about how I'm doing. (Or when someone prays for my mother's heart, apparently, since I had to make a quick exit from Sunday school this morning before they saw my ugly cry!)

Please, please pray for us.  This has been really hard on our whole family.  Committing to this surgery is committing to 30, and it's a major life change.  Caleb is worried for his sister, and he's had several conversations with us about how she is going to recover.  While yes, we are typically pretty strong and have learned to roll with Abby's medical stuff, this has really thrown us.  There have been lots of tears without warning this past week, and we could really use your prayers....and hugs, if you see us!  

That being said, I don't mind talking about long as you don't mind a few tears...or maybe an ugly cry!  :)

Specific prayer requests:

*  Abby needs to gain about 5 pounds in 5 months.  This is a HUGE task, as she gained just under 5 pounds in the last YEAR.

*  The scoliosis and lung functioning will not get worse.  If that happens, surgery will be moved up.

*  The airway issues will not be a problem during the MRI or the actual surgery.  

*  Caleb will continue to voice concerns and talk to us about how he is feeling.

*  We will feel at peace with our decision.  There's a lot of anxiety.

Thank you so much for praying!!

Sunday, February 23, 2014

Pups of the Spirit, by Deborah Melmon

Pups of the Spirit is a book written for preschool-aged children, and my kids loved it!

  Through rhyme and a pack of lovable dogs, kids can learn about the nine fruits of the spirit:  love, joy, peace, patience, kindness, goodness, faithfulness, gentleness, and self-control.  I read this to the kids before bedtime and it is now a new favorite!  In fact, Abby was very disappointed that I took it out of her room to write my review.  :)  Both kids had their favorite dog...Abby's was Joy, of course!

The illustrations were bright and cheery, and the details included in each picture were noticeable.  I especially loved how this book sparked conversation about the fruits of the spirit.  It was a really good way to introduce the concept, and I'm sure that I will be able to refer back to the book in future conversations.  The explanations of each "fruit" were simple enough for young ones to understand, so I even plan to bring this book the next time I am in the church nursery 2s and 3s.  I think they are the perfect age to enjoy the illustrations and at least have some basic conversations about each "fruit."  I'm sure they will love it as much as my kids did!

Disclaimer:  I'm a BookLook Blogger.  A complimentary copy of this book has been given to me in exchange for an honest review.  As always, all opinions are mine! 

Sunday, February 16, 2014

What is a VEPTR?

If you are interested in learning more about what the VEPTR is, you can visit here and watch the videos below.

What is early onset scoliosis?  Why is it different than "regular" scoliosis?  What is Thoracic Insufficiency Syndrome?

This video explains some of the testing Abby has had or will be having.

Here, you can see what the VEPTR looks like and how it is implanted and expanded.  (This is not from CHOP, but it is done the same way)

We have some other videos we were sent on a disc, but these are the ones I could find on You Tube!

Saturday, February 15, 2014

I Will Praise You in This Storm by Danny Deaube

Given the most recent "storm" we are in, I Will Praise You in the Storm was a very timely book for me to read.  I found myself really identifying with the author, Danny Deaube.  He is the father of two children who had rare liver diseases, and this book is all about his experience with raising two medically fragile children.

It speaks a bit of the frustration and stress that is a part of the daily life a special needs family, but most of the book is a recount of the medical experiences and surgeries his children had.  Both children underwent liver transplants at young ages, and while Deaube's son survived the transplant and lived to be in his thirties, his daughter died from complications.

As I said, I really could relate to a lot of what the author said.  Like him, we live a life full of hospitals and doctors, while trying to keep our daily routines as normal as possible.  But what I feel was lacking in the book was the raw emotion that comes along with watching his children suffer and ultimately succumb to their disease.  Writers who honestly share their heart, including frustrations and grief, validate the feelings of their readers who may be in a similar situation.  I missed that in this book.  A reader could mistake the lack of emotion for a lack of longing for the two children he lost.  While I certainly don't believe that to be true, I feel that including more of the rawness (that I'm sure the author felt) would have only strengthened the book's message.  That being said, it was a good read...especially for someone who could relate to the medical terminology and "that" life.

Disclaimer:  I'm a BookLook Blogger.  A complimentary copy of this book has been given to me in exchange for an honest review.  As always, all opinions are mine!  For more information about the BookLook Blogger program, click here!

Friday, February 14, 2014

The Next Chapter

We are home from our visit at CHOP.  I'm going to apologize in advance if this isn't my usual style of post.  I'm exhausted, both mentally and physically!

Abby ended up having five appointments/tests done:  CT scan, Pulmonary Functioning Test, X-ray, and appointments with the pulmonologist and orthopedic surgeon.  We managed to get to our appointments on time on Thursday.  After debating whether to call a cab, drive, or even walk, we decided to see if we could drive in.  It ended up being fine.

After the CT and PFT, we saw Dr. M, the pulmonologist.  He went over the results and shared his thoughts, but deferred to Dr. C, who would be the one doing the surgery.  So we really had to wait until Friday to get the answers we wanted.

We saw Dr C this morning, who went over the CT and PFT, but then asked why we didn't get an xray.  (We were told during the scheduling process than an xray wasn't necessary...)  So, back to radiology we went!  When the xray was done, we went back to ortho to see Dr. C again.

After gathering lots of information, we have learned that Abby's scoliosis is progressing and has significantly worsened in the last 6 months.  She now has a 28 degree curve (scoliosis) and a 59 degree hump (kyphosis).  Because of this and the narrowness of her chest, her lungs aren't growing the way they should be.  The capacity is only about 1/4 of what it should be in the right lung, and about 1/2 of what it should be in the left lung.  She appears to have about 40% of lung function.  While this hasn't affected her everyday activities too much yet, it is a matter of time.  We are already seeing it in her resting respiratory rate, which is about double of what it should be.

It appears that we have received the "big sign" we were praying for to help us decide about the surgery.  We will be tentatively scheduling surgery for this summer, while closely monitoring her scoliosis and lung functioning.  They want to see her again in 4-6 weeks for a dynamic MRI, as well as repeat scans, xrays, and some blood work.  We will also meet with both Dr. C and Dr. M, as well as a few more on the team.  When all of that data is collected and the staff has discussed her case, we will meet again to make an official decision.  But it is highly likely that she will be having surgery this summer--or earlier, if the lung functioning continues to decrease.

We obviously weren't expecting the lung capacity and functioning issues, so that definitely threw us for a loop.  It's been a very long, emotional day.  But we will continue to rest in the assurance that God has a plan for Abby's life, and this is just the next chapter.  Thank you for praying for us during this journey!

Who's Your Hero?

Mine wears a rainbow tutu!

Tuesday, February 11, 2014

We're On!

I called CHOP today to make sure we were still on for Thursday (I'm not so worried about our Friday appointment--I think everything should be clear by then.), and we are!  I made sure it was noted to call our cell phone if anything changed, but our plan is to make it to all 4 appointments.  We have located all of the hotels within walking distance to the hospital so that we can just walk there if needed.  The snow pants and boots are packed, and we have plenty of hand warmers and a few blankets stashed in the car.

I think we are ready!

I have started praying that God will make it blatantly obvious if we need to do this surgery.  I need to know very clearly that VEPTR is the way to go...rib pieces overlapping on a CT scan, significantly decreased pulmonary functioning, etc.  Otherwise, it will tear me apart to agree to something that is such a life change when Abby is currently doing so well.

Will you pray with me?  (and maybe for a little peace for Momma...this is all quite stressful all around!)

Monday, February 10, 2014

Brave Girl

I have one tough little girl!  She deals with so many doctors' appointments and medical procedures.  Through it all, she has a smile on her face and a hop in her step!  I'm amazed that a child with so many obstacles in her life is still so happy!

We've recently started talking openly about Abby's ribs with her and how Dr. C is going to fix her ribs.  We're obviously not going into detail about the surgery, but we wanted to start an open discussion so that she would get more used to the idea if and when we actually move forward with surgery.  It seems to be helping, because she's been telling everyone that Dr. C is going to fix her ribs! (Say it in a sassy voice with your hands on your hips to get the full effect!)

Today's adventure was ophthalmology, where we discovered that Abby's eyes are still crossing quite a bit.  After discussing giving her bifocals (for a three year old!!!) he finally decided that it would be too hard to explain to her how to use them.  He increased her prescription by quite a bit (it went from +4 in both eyes to +6), and the next step will probably be the bifocals--or patching--or both.  Surgery was also mentioned, but we're really hoping to avoid that.

Our next adventure starts on Wednesday when we head to CHOP.  You've probably heard about "The Beast" that is heading toward the Mid Atlantic Wednesday night and into Thursday.  Yeah. We're not friends.  We are going to try our absolute hardest to make it to these appointments.  They're too hard to schedule to just cancel them!!!

So in addition to praying for Abby's appointments, a joint-decision about the rib surgery from both doctors, and clarity of thought as we ask questions and make a decision about surgery...please pray that we stay safe during this crazy weather!!!

Friday, February 7, 2014

Love You Tightly

I recently found out that the lead singer of Sanctus Real has a medically fragile child.  His son, Bowen, has Hypoplastic Left Heart Syndrome, a rare, serious heart problem.  This is the same syndrome that the infant son of a teacher-friend of mine has, and he is currently fighting hard in the hospital!  

As I was reading Bowen's Story, I learned that many of Sanctus Real's songs were inspired by their story.  When I read the lyrics to this song, they definitely tugged at my heart strings.  He gets it.  He gets it in a way that only the parent of a medically fragile child can.

We're heading to CHOP next week, so this was a very timely reminder as we continue to discover God's plan for Abby's life.

Love You Tightly

It's my reality, you don't belong to me
I know we're living on borrowed time
And when I look at you, I can clearly see
A gift that I may only hope for a while
So while we still have time

I'll love you tightly but hold you loosely,
Cause you were never mine
No, you were never mine
I'll love you tightly but one day I'm gonna have to let you fly
Cause you were never really mine

I've learned the hard way, life is a give and take
You've gotta take it one day at a time
And every minute is a chance to breathe it in
Before you know it it will pass us by

So I'll love you tightly but hold you loosely,
Cause you were never mine
No, you were never mine
I'll love you tightly but one day I'm gonna have to let you fly
Cause you were never really mine
Oh yeah
You were never really mine

Some days I catch myself holding you too tight
Cause I know that I can lose you
I don't wanna lose you
Some days I catch myself staring at the time
Cause I know we're gonna lose it
It's gonna fly by
So in the meantime
I'm gonna love you tightly

I'm gonna love you tightly but hold you loosely,
Cause you were never mine
No, you were never mine
I'll love you tightly but one day I'm gonna have to let you fly
Cause you were never really mine
I'll love you tightly
But hold you loosely
Cause one day I'm gonna have to let you fly
Cause you were never really mine
I'll love you tightly

Wednesday, February 5, 2014

Book Looks!

I am starting a new venture on my blog!  My sweet neighbor (who is also a blog reader!) saw that I was enjoying reading again and she recommended Book Looks.   Basically, I choose books each month to read and write reviews about them on my blog.  In exchange for my honest review, I get to keep the books!  There are some that can be downloaded as e-books and some that can be mailed to you as hard copies.  The company has TONS of books from which to choose, and it is constantly adding new ones.  This is a Christian publishing company, so it has everything from Bibles, to devotionals, to fiction, to biographies, to commentaries.   I will probably be reading a lot of different types of books over the next months!

My first book is I Will Praise You in the Storm.  I just downloaded it, so stay tuned for my review!

Monday, February 3, 2014

Medical Update

Since Abby's tubes were put in her ears January 15th, she has continued to have a lot of ear issues.  Her ears began bleeding again well after they had stopped post-op, so our ENT put her back on Ciprodex.  (If you are looking for a good stock purchase, Ciprodex might be it.  I think Abby is responsible for most of its financial success.)  The nurse practitioner said to call if she wasn't better in a week, and she would see her when we were coming up for Abby's feeding evaluation on Thursday.

Welp, they were no better, despite pouring Ciprodex into her ears twice a day.  In fact, her right one was definitely worse.  So I called ENT on Wednesday to make an appointment for Thursday afternoon.  Dr. T. wouldn't be able to see her because he was in surgery all day, but the NP could.  We went straight there from the feeding clinic at MWPH (a 10 minute drive with an extra 20 minutes of driving through the parking garage trying to find a space!!!)

The nurse practitioner took a look and agreed that her ear needed to be sucked out.  She took us to the procedure room. Abby started crying as soon as she set foot in there.  Nothing good has ever happened in the procedure room, and goodness knows Abby has been in there more than her share of times!  Our awesome ENT came back to the clinic just as we went in, so he came in to take a look.  I like the NP a lot and trust her, but Dr. T. is so protective of Abby that he makes me feel very secure. He knows she isn't your typical tubes kid, so I was glad he was able to take a look.

Good thing, too.  He was pretty miffed at all of the build-up he saw.  In fact, I believe his exact words were, "Gee, I just have no idea what is going on here!"  He cultured it again and cleaned everything out really well.  He uses a camera that projects the image onto a big screen television mounted to the wall, so it's actually kind of cool to get to see it as he sucks out all the gunk. If you've never had your ear vacuumed out using the same suction machine we used to use for her trach, you're not missing much.  Abby was terrified. I can imagine that noise in her ear was really loud and painful!  She's had it done several times before, but this is the first time she remembers!  I don't think she will soon forget!

Then, Dr. T put in what he called a wick.  It was a really skinny piece that went down into her ear canal.  He put a new kind of drops in her ear so that the wick expanded and filled the ear canal, then put a cotton ball on the outside of her ear.  The purpose was to help the medicine stay in her ear rather than drip out.  I asked if she could hear in her right ear, and his reply was, "Oh, NOOOO.  She can't hear a thing!"  The packing filled the ear canal, and it was kind of funny to hear her say, "What?  What?" over and over again.  Thank goodness that is already her bad ear, because her left ear is good enough that she could somewhat compensate for it.

Dr. T said the wick should stay in for several days, but to take it out if it was still in on Monday.  He said we may even have to go to the pediatrician's office to have him take it out, because it was pretty jammed in there.

Yeeeeeah...Abby pulled it out that very night, shortly after I put her to bed.  She called my name and I went in there.  She very dramatically announced, "It allllll came out!"  I knew what she was talking about immediately, so I turned on the light. Sure enough!  "Abby!  That was supposed to stay in there so that it would help your ear feel better!" I lamented.  With her arms out palms up for effect, she asked, "What???  It's just a french fry!!"  I got a good laugh out of that one.  And when I looked at it in its expanded form for the first time, it really did kind of look like a french fry!

Dr. T. is really hoping these drops work.  When we find out the culture results later today , he may change or add medicines.  But if this doesn't work, he may have to take the tube out.  :(  This is doubly bad because she would have to go under anesthesia again and her right ear is already the one that has the most hearing loss.  She really needs the tube to help her hearing.  

So please pray that these drops finally do the trick.  She has been on ear drops for the past 2 months straight, and this is her third kind of drop.  Thankfully, she is really good about getting them!  I think they're just so much of her routine that she's just used to it!  In fact, she asks for ear drops when we aren't having to use them!  Lol, the life of a medically complex child!

Sunday, February 2, 2014

Get Your Tissues Ready!!

Have you seen this commercial about Seattle Seahawk Derrick Coleman?!?

I first heard about it on our local Christian radio station.  What an inspiration to everyone, but especially special needs families!

Well, two little girls who are hearing impaired thought he was pretty inspirational too.  They wrote him a letter and their dad tweeted it to Derrick Coleman.  He wrote back, much to the girls' delight!  Then he surprised them with a visit as they were being interviewed on Good Morning America!!!  The icing on the cake was tickets for the family to go to the Super Bowl!!  What a great guy.

I love this story and the message it sends to all the kids who have to work harder than most to accomplish their dreams.  <3 p="">
As much as I dislike football and only watch the Superbowl for the commercials, I'll be cheering Derrick Coleman on tonight!

Saturday, February 1, 2014

Feeding Update

We went to a feeding team evaluation last Thursday, where a whole group of therapists and nurse practitioners watch her eat, ask a bunch of questions, and develop a new feeding plan.  This was our first eval since exiting the feeding program because Abby got sick for the first one, so these therapists last remembered her reluctantly eating purees and often refusing to drink her milk.

Imagine their surprise when they saw Abby taking bites of a peanut butter and jelly sandwich, drinking her milk out of a open cup and straw, and eating an applesauce pouch independently!! They were very impressed at how far she has come.

Of course, they had a few concerns too.  Abby only gained an average of 3 grams per day, and she was supposed to gain 6-10 grams a day.  She's hanging onto that first percentile, and hasn't gotten any taller since this summer.  (That is a separate issue...)  She's also having a lot of tummy issues, which they believe is from not drinking enough liquids.  With all of the Pediasure 1.5 (which is hard on tummies), she just about refuses to drink anything else throughout the day.  It was much easier to just put some water through the g-tube, but we won't be going back down that road!!  We are now giving her Miralax daily, mixed in with 4 oz of some kind of liquid.  I will probably do juice just for the extra calories.  (Mixing Miralax into Pediasure isn't an option, as it won't dissolve in milk protein!)

The team was also very understanding in that increasing the textures of Abby's food would mean that she wouldn't gain as well.  It's much easier to gain weight by eating all purees that don't require much effort.  It's definitely a process, but we are getting there!