Thursday, November 14, 2019

Peace


This is my new shirt and it has never been more true.  The last few days have been filled with phone calls, paper work, scanning documents, and faxes.  We have prayed about our decision, talked to Abby's pulmonologist, weighed the options, and cried.  There's no great answer, and our pulmonologist recognized that.  She was quick to acknowledge that we have had to make hard decisions for Abby before and we have always made the right one.  She did echo a lot of our concerns and validated our feelings on the options, which helped us to make a decision.

1.  We are going to meet with Dr. Lenke at Columbia University Hospital in New York City.  There was a 14 page application (yes, I said application) and I had to request all of Abby's medical history related to her spine, as well as her most recent CTs, MRIs, and Xrays be sent to them.  Once all of that is received and reviewed by Dr. Lenke's team, they will decide whether or not they will accept Abby.  If we are able to get an appointment, we will discuss the possibility of a VCR in the future and what he feels will be the best long-term approach for Abby.

2.  In the meantime, we are going to have Dr. Anari put the rib to rib VEPTR back in.  We are hoping for sooner, rather than later, because our pulm is not thrilled about her being in the hospital during flu season but feels we shouldn't wait until after flu season is over.  CHOP has told us that they won't schedule her for surgery until after they meet with the Center for Thoracic Insufficiency Syndrome team (CTIS) at the end of November.  I'm not sure why that really matters, but they assured me that they can get her in sooner than the next available if it is needed. 

We have talked to Abby about the 3 options and she actually said that waiting hasn't worked for us so far and she's not ready for the VCR, so there's really only one option.  Let's get that VEPTR back in!!  I love that girl.  She knows she needs it.

For now, we are waiting on a surgery date and looking forward to our upcoming trip to Disney World!!  There was never a better time for a vacation. 

Yes, I Will



I love this song.  This is what I strive to do.  I'm far from perfect and the scratches on my hand from a panic attack are a visible reminder that sometimes my anxiety gets the best of me.  The last few days have been tough, but today is a new day. 


I count on one thing
The same God that never fails
Will not fail me now
You won't fail me now
In the waiting
The same God who's never late
Is working all things out
Is working all things out

Yes I will, lift You high in the lowest valley
Yes I will, bless Your name
Oh, yes I will, sing for joy when my heart is heavy
For all my days, oh yes I will

I count on one thing
The same God that never fails
Will not fail me now
You won't fail me now
In the waiting
The same God who's never late
Is working all things out
Is working all things out

Oh, yes I will, lift You high in the lowest valley
Yes I will, bless Your name
Oh, yes I will, sing for joy when my heart is heavy
For all my days, oh yes I will
For all my days, oh yes, I will

And I choose to praise
To glorify, to glorify
The Name of all names
That nothing can stand against
And I choose to praise
To glorify, to glorify
The Name of all names
That nothing can stand against
And I choose to praise
To glorify, to glorify
The Name of all names
That nothing can stand against
And I choose to praise
To glorify, to glorify
The Name of all names
That nothing can stand against

Oh, yes I will, lift You high in the lowest valley
Yes I will, bless Your name
Oh, yes I will, sing for joy when my heart is heavy
For all my days, oh yes, I will
For all my days, oh yes, I will
For all my days, yes, I will

Monday, November 11, 2019

Decisions

We went to CHOP orthopedics today and are still reeling from all of the information.  I'll do my best to explain everything, but I'm still processing myself. 

When Dr. Anari came in, he didn't beat around the bush.  He told us he had spoken with the pulmonologist we saw last week and that it's obvious we are seeing a decline.  While the kyphosis looks to be about the same on the xray, there could be subtle changes that could be pushing Abby over the edge, so to speak. 

Then he proceeded to tell us that Abby was one of his top two or three patients in terms of difficulty, complexity of the chest wall, and high risk.  Everyone has to be good at something, right?  😒

He laid out 3 options for us to consider.

1.  This is the most aggressive option.  Abby would be put in a halo for several months, then she would go into surgery for a removal of a section of her vertebrae.  It is called a Vertebral Column Resection and you can read about it here. She would have to be in the hospital for the entire process.This is a HUGE surgery and very risky.  It is by far the biggest she has done, and none of them have been a walk in the park.  There's a high risk of both paralysis and infection.  Dr. Anari encouraged us to go to a doctor in New York City who specializes in this kind of surgery, since he said he only does one or two every few years.  I'd heard of this surgeon, but I knew he only took the most complex pediatric patients.  Dr. Anari assured us that Abby fell into that category...not sure how I feel about that one.  Having this kind of surgery would also mean that Abby's spine would be fused once and for all and she would stop growing.  She is currently 45 inches tall.  😭

2.  The "middle of the road" option.  Dr. Anari would put the VEPTR back in on the right side rib-to-rib to pull her chest wall back out on this side.  It would be a bigger surgery than her previous VEPTR insertions because he would be dealing with scar tissue and stiffening of the ribs.  There is an additional risk of infection because Abby is prone to them--he gave us a 25% chance of having an infection.  His biggest concern with this option is that he isn't sure Abby's lungs can handle any kind of major surgery right now.  She may not be able to come off of the vent after surgery because her lungs will be too weak.

3.  The conservative route.  We could wait and see how Abby does through flu season and hold off on making any decisions until March.  This would give Abby's body 6 months to recover from her hospital stay and see if her lungs improve.  The risk here is that her lungs don't get better and in fact worsen.  Then we will have lost our opportunity to operate. 

Dr. Anari knows how much we adored Dr. Campbell and is always quick to acknowledge his brilliance and expertise.  When we asked him what he thought Dr. Campbell would have done, he said that he thinks he would have put the VEPTR back in.

So....yeah.  There's so much to consider here and it's up to us to decide what is right.  We aren't anxious for Abby to have a VCR because it's such a risky surgery and if Dr. Anari isn't sure she can handle a VEPTR reinsertion, the VCR is even harder on the body.  It is probably something that needs to be done in the future, but we would love for Abby to grow a bit more before it does.  Our hope was always to allow her to finish growing before we did anything that would fuse her entire spine.  (She already has 2 short fusions that have caused 8 of her vertebrae to stop growing.)  The wait-and-see approach hasn't worked so well for us so far, but the risk of her not being able to handle a VEPTR surgery is kind of nerve-wracking too. 

We left the appointment rather in shock and having no idea what we were going to do.  The shock wore off on the ride home and I had a sudden and fierce panic attack that I managed to hide from the kids. 

Matt and I have talked on and off about it and we know that we want to talk to our pulmonologist about her thoughts on Abby being able to withstand a surgery (and the likelihood of her lungs improving in the next few months).  We would also like to meet with the specialist in New York City to get his thoughts on how Abby looks.  This is really more to get a feel for future surgeries--we have pretty much decided to wait on the VCR until we really have to do it.  We think it's important for him to consult with us now so that we will have a plan for the future.

CHOP has its interdisciplinary meeting with all of the key doctors in the Center for Thoracic Insufficiency at the end of the month.  Abby's case is on the schedule to be discussed and Dr. Anari is hoping others can weigh in on the best course of treatment.  There just isn't a great answer to this one.  We are, however, thankful that Dr. Anari recognized Abby's decline, listened to the pulmonologists' concerns, and came to us with a plan.  This is what we were hoping would happen, because we really didn't want to travel to another hospital.

For now, we are praying, consulting, and processing.  We will be talking to CHOP soon with what we decide and calling to schedule an appointment with the NYC specialist.  Will you please specifically pray:

*  That it will be clear which option we need to take.
*  That Abby will be safe and healthy, no matter which option we choose.
*  That her lungs will improve!!!
*  That Abby will trust that we are making the best decision we can for her.  She HATES the idea of more surgeries and we don't want her to resent us.  We really are trying to do what is best for her.

Tuesday, November 5, 2019

Double the Pulmonary, Double the Fun!

Oh CHOP...

We have had an ongoing disagreement with CHOP orthopedics over which pulmonologist we should see.  We adore our pulm at Hopkins.  She has been with Abby since the beginning and really gets the whole picture.  She truly cares about Abby and we feel like she always has her best interests in mind.  CHOP ortho really only seems to value the opinion of their own pulmonologist with the Center for Thoracic Insufficiency team.  We have nothing against him!  He is a very nice guy and very knowledgeable, but we see no need to switch pulmonologists when ours is wonderful and 2 hours closer.  We don't care for the attitude of superiority we sometimes feel at CHOP and feel like all of her doctors should work together to give her the best care possible, no matter where they work! (Not to mention, Hopkins is not a two bit hospital!!)

With Abby's recent lung decline and us feeling that a surgical intervention is needed, Abby's pulm has been communicating with CHOP to share her thoughts.  CHOP ortho has been very reluctant to do any sort of surgery on Abby since she became septic back in 2017 and had to have the right side hardware removed.  He is rightfully concerned about the risk of repeat infections.  He said we would really have to convince him that it is needed and that Abby would really have to "deteriorate" in order for him to agree to do it. 

Since he said that in July, Abby has had suspected pseudomonas, double lung pnemonia, a hospital stay, is requiring oxygen with any kind of activity, and is still not being about to keep her oxygen levels up with exertion!  So....how much more deterioration do we need here?!?!  I've called and emailed the team MANY times, but have been really frustrated.  Bottom line:  CHOP ortho wanted her evaluated by their CTIS pulm before they would see her again.  😒  So we played the game and made an appointment, which ended up being less than a week after our Hopkins pulmonary appointment!

Our Hopkins pulm felt like Abby's lung functioning was the same, based on pulmonary functioning tests.  (The same does not mean good....I mean, there's nothing good about any of it.)  But Abby has been continuing to struggle maintaining her oxygen levels in PT and PE when she was exerting more energy, even though she is currently on 1 liter of oxygen.  She gave us the flexibility to bump her up to 2 liters as needed if she can't keep her oxygen levels up.  As I have said before, oxygen is a band-aid for Abby that doesn't actually fix the problem, but it is what she needs right now until the real problem is addressed.

We also looked at her lung CT from her hospital stay in August and our pulm went into great detail about the issues.  Abby has some deflation of the lung in her lower lobes (called atelectasis) and some scar tissue from recurrent infections.  It's not fabulous.  Our pulm feels like we are doing everything we need to do to clear Abby's lungs (SO many daily treatments...) but encouraged me to ask the CHOP pulm if he had anything else he thought we should add.  As we have been told before, although Abby's lung functioning is well below the level where they would begin discussing a lung transplant, she is not a candidate for transplant because hers is a structural issue that wouldn't support new lungs properly.  Our pulm did make sure to say that while all of this isn't so great and Abby is heading down the path of needing constant mechanical ventilation again, she is also something of a wonder who has surprised us in the past.  She described her as amazingly resilient, and I agree!

So....yesterday after school, we traveled to CHOP and spent the night at a hotel to be there for a 9 a.m. appointment.  It was a total of 2 hours and 40 minutes that we were there, although we did have to wait a bit to see the doctor. We did pulmonary functioning tests again, which naturally annoyed Abby because she had just done them last week!  😆  The respiratory therapists also did some extra tests to assess the strength of her respiratory muscles to be able to inhale and exhale properly.  Those weren't good either, of course!

The CHOP pulm agreed with everything our Hopkins pulm is doing, which is affirming (not that we needed it!)  He also feels pretty strongly that she needs some kind of surgical intervention even with the risk of infection.  The main issues affecting her lungs are her scoliosis and rib gaps, not the kyphosis.  That's a separate issue, but it's not as pressing right now as far as the lungs go.  He also noted the partial lung collapse and scarring from repeat infections, which he said will only continue to get worse.  Her ribs are collapsed on the right side without any hardware and the airway is being pulled up and over the spine because of the spinal rotation.  He thinks getting a rib to rib VEPTR back in on the right side will really help and wants it done sooner than later.  He agrees with our Hopkins pulm that Abby is heading toward continuous ventilation (ie: trach and vent) if something isn't done.

The CHOP pulm commented that pulmonary functioning tests are not a valuable assessment tool for Abby because they aren't so valid when the numbers are "this low."  He also said in his notes, "At this status, her pulmonary functioning is not a helpful outcome measure and that lack of worsening over the last two years is because her lung volume is effectively as low as it physically can be."  That was telling!  This was really good information to send to ortho because in the past, they have been basing decisions on her pulmonary functioning tests and not as much on her actual every day function.

He is going to talk to ortho between now and next Monday when we have our ortho team appointment.  Hopefully, he will convince the team that a surgical intervention needs to occur.  While we hated having to go to CHOP for pulm when we already have a wonderful one, I think ortho will listen to their own pulm much more because he is "one of them." 

This isn't the first time we have been in a difficult situation like this with doctors disagreeing on treatment.  It is never a fun place to be and you just hope and pray that you are making the right decision for your child.  I cannot tell you how much research I have done or how many sleepless nights I have had. 

Now that we have two pulmonologists both loudly proclaiming the same concerns and needs, we are ready to take Abby to another orthopedic surgeon for a second opinion if needed.  We are hoping that everyone will be on the same page next Monday and we can move forward with a surgical plan.  If not, we have two other surgeons in mind.  One will require a plane ride, but we will do what is best for our girl!  We have found ourselves really missing Dr. Campbell's wisdom and experience during this situation.  He was such a brilliant man who truly looked at the whole picture.

So....we would covet your prayers this week as the doctors share opinions and concerns.  We truly feel that Abby needs hardware put back in on the right side in order to improve her breathing.  We are also concerned about this being done before she gets any worse and isn't able to withstand a big surgery.  Ultimately, we know that God holds Abby in the palm of His hand and He has brought us through so much already. 

Tuesday, October 22, 2019

A Letter to My Daughter

Dear Abby Joy,

Once upon a time, your mom was a blogger who wrote almost every day...life has gotten away from me, but I figured what better day than your birthday to start blogging again?!

Nine. Years. Old.  Where did the time go??  I still remember every vivid detail of your birth day.  After only being released from the hospital two days before, I started having contractions that didn't stop after I took my medication.  Daddy was at a meeting at the church, so I called Uncle Bruce to take me there and pick up Caleb.  He was worried about my water breaking in his police car, so he made me sit on towels!  We met Daddy, then he raced up to Baltimore to the hospital where you were to be born.  You were determined to come fast, and the doctors scrambled to get everything set up that you were going to need.  We thought you were going to be born on October 21st, the way things were going, but you waited until just 12:12 a.m. to be born.

You flew out like you were on a water slide, and the doctors whisked you away to the room across the hall to start taking care of you.  I never even got to see you!  It was hard not to hear crying when you were born, but I knew they were going to get you breathing.  Daddy kept bouncing between the two rooms to take care of his girls.  A sweet anesthesiologist would come in and give me updates of how you were doing. When they were ready to take you down to the NICU, that same anesthesiologist brought you in for a "fly by" so that I could see you.  You had a bright orange tube to help you breathe and lots of tape holding it on so I couldn't see your face very well, but I could see your gorgeous salt and pepper hair!

I was determined to get down to the NICU as soon as possible and did everything the nurses told me so that I could.  When Daddy and I went to visit you, we didn't know which one was you because we had only seen you for a second!  The NICU nurse showed us your crib and I sat down next to you.



 The thing that struck me most was your HUGE black eyes!  We used to say they stared deep into your soul.  Combined with that beautiful hair of yours, you were a gorgeous baby. 

We got your diagnosis in the morning and I have never been so terrified.  That conversation was horrible and your dad and I were left wondering if you were going to make it.  Little did we know that God had BIG plans for your life!



Since October 22, 2010, you have been proving people wrong and crossing off the "Abby Will Nevers" left and right!  God gave you some amazing personality traits to help you to flourish in the life you have been given.  Your feistiness, determination, and yes, even your sassiness have helped you to fight through the hard times.  You are SO brave and strong.






I'm so proud of the girl you have become. You absolutely love school and do so well.  Your favorite subjects are reading and science, and you are often found around the house or in the car with a nose in a book (just like your brother!)  You want to be a veterinarian when you grow up and you and Anna have already drawn up some plans for your vet clinic!  I'm saving them and will frame them for you to hang on the wall when you open it. 



Your love for people and life shines through everything you do.  You are such a social little girl and you are energized by being around people.  That's the hardest part for you when you aren't able to go to school and it breaks my heart.  You find joy in the smallest things and I just love it.  

You are a wonder, Abigail Elizabeth Joy Leach.  I'm so thankful that God made me your momma.  Here's to many more birthdays, sweet girl!




Love,
Momma

Monday, April 22, 2019

The House That Built Me



After months of cleaning and selling household items, my grandmother's house is for sale.  While we are thankful to still have her with us, she can no longer live by herself.  It was time to sell it, but that doesn't make saying goodbye any easier.

Next to my own house, I spent most of my time at "Mommom's House."  I stayed there on inservice days when my mom had to work.  My sick days were usually spent there.  When my parents went somewhere on a date, I went to Mommom's.  I loved every second I spent there.  I have SO many memories in that house, and I thought I might share a few with you.


The tree has long-since been cut down, but a huge pine tree used to be here.  You can see the stump in the ground.  It was the perfect climbing tree, and I would spend hours in it.  I had "rooms," would make up stories, and would read books in it.  It was the best!


This stoop was the location of so many mud pie concoctions!  She would give me a dish pan, a shovel, and a cup of water, and I would add dirt and play to my heart's content.  I was a tomboy, so being dirty didn't both me at all!


Even though her house in on a very busy road, she has a huge front yard that I turned many cartwheels on.  I also loved to sit on the porch and count the cars.  We had lots of yard sales and lemonade stands in her front yard too!

 

A low branch that used to be on this tree was the perfect height and size for a swing.  My grandfather hung it for us when I was really little, and I would swing out here for hours.


This field used to be a lovely home garden.  My favorite produce out of it were the tomatoes and strawberries.  I used to love to pick them.  There's nothing like a freshly picked tomato!  Yum!


You wouldn't think there would be a memory tied to the kitchen sink, but I spent many hours playing in soapy water, pouring water from cup to cup, and dunking little toys under the water.  This is also a place where Mommom would often stand washing her dishes.  While she could have had a dishwasher, she never did.  She always said she liked washing her own dishes.


This is where the kitchen table stood.  It was just a little drop leaf round table, but there was always room.  We often joke now that Mommom's love language is food, because she absolutely loved cooking for people.  She would make me yummy salads (I loved them even as a little girl) or sandwiches with chips and we would sit here and talk.  This was always the "kid table" at holiday gatherings.  Even though there were five grandchildren, we all always managed to fit!


Right here sat a red oval-shaped Tupperware container and a white rectangular Tupperware container for as long as I can remember.  In them were almost always chocolate chip cookies, brownies, yellow cake with chocolate icing, or some other yummy treat.


Also in the kitchen right next to the refrigerator sat three clear glass cookie jars that were always filled with pretzels, ginger snaps, and usually some other kind of cookie.  The pretzel jar was always in front and was never empty.  We always knew where we could go for a snack!  Pretzels and orange juice were my favorite snack/drink combo at Mommom's house.


In the dining room, most of the floor space was filled with the dining room table.  When I was growing up, many heated games of Pinochle were played at this table with Mommom, Pop Pop, and Aunt Daisy!  This was also where the food would be spread out buffet-style for family gatherings.  Mommom always had shrimp cocktail and a yummy spinach dip for the veggies that I loved.


This is the living room, which had a couch, 2 recliners, a chair, and 2 rocking chairs.  It's amazing how much seating you can fit into a small space!  Sick days were spent on the couch watching Price is Right with Pop Pop and yelling at the people on the TV when they were too high or too low.  Many, many Christmas Eves were spent piling into this room to exchange presents.


See the mark on the floor there?  This is where the bar used to stand in the basement.  I really don't know why they had it because I never saw them use it, but I LOVED playing at it!  I would serve up all kinds of drinks using the cups from Hawaii and pretend that I was at some kind of tropical place.


I always loved playing in the basement.  I would ride around on my bike or little push toys, play hide and seek,or just pretend.  In more recent years, this area housed all of the teaching materials I couldn't bear to part with.

There are SO many more memories I could share.  As I walked through the house taking pictures and mentally saying goodbye, I couldn't help but tear up a little.  It's the closing of a huge chapter of our lives, and this house was a central point for our family for 60+ years.

Goodbye, little brick house.  Thanks for so many great memories!


Thursday, February 28, 2019

It's Rare Disease Day!

I apologize for the lack of posting.  But...no news is good news in the medically fragile world!  Abby has been doing SO well and we are thankful for the many improvements to her health.  She had a touch of pneumonia in the beginning of February and stayed at home!  This is huge because pneumonia can be a beast for diseased lungs.  She did her breathing treatments like a champ and hung in there just fine. 

The last few weeks, her school has been pretty germy.  We kept her out for a week and a half to protect her while the flu made its rounds.  Everything is looking much better now though, so she happily went back on Tuesday.  While she is the one of my kids who I could easily home school, she is also the one of my two who simply loves being around people!

Today is Rare Disease Day, and I wanted to share what I have been posting on Facebook with you all here.

Cerebrocostomandibular Syndrome, Abby’s primary diagnosis, is extremely rare. There are only an estimated 10 children living with it. We have no support organization, no research funding, and no specialists. The parents are the specialists!




Abby’s secondary diagnosis is Thoracic Insufficiency Syndrome. She has a rib-gap anomaly that caused about 1/2 of her ribs to be in multiple pieces. Those pieces are a ribbon consistency, which leaves most of her thoracic cavity unprotected. Her rib cage is unable to expand, keeping her lungs from being able to grow.
Because her ribs are so flimsy, her spine is unable to be supported. She has congenital scoliosis (thoracic spine) and early onset kyphosis (cervical and upper thoracic spine).
Abby has VEPTRS (Vertical Expandable Prosthetic Titanium Ribs) created by Dr. Robert Campbell. These VEPTRS manually expand her rib cage because they don’t do it on their own. This gives her lungs the room they need to grow.
Abby in Summer 2016 with 4 VEPTRS

To date, Abby has had 31 surgeries. Most of them have been to work on reconstructing her chest wall and address her kyphosis. Right now, her biggest chest wall concern is her kyphosis because cervical kyphosis is really difficult to correct. 2 years ago, it began to compromise her spinal cord. A surgery to correct it caused a spinal cord injury that resulted in nerve damage and a whole lot of PT!
You can see the severity of the Kyphosis here, and this was during the time that she was braced

She has been in multiple braces, including a pinless halo for 6 months. She developed a severe spinal infection, resulting in an open wound for 13 months and a severe sepsis that nearly killed her. Her team ended up removing the hardware in the right side to get rid of the infection. Abby has been on long-term antibiotics to keep the infection at bay, and will continue to be for as long as she has hardware.

The pinless halo

We have not found the magical cure, so we are just in a waiting game as long as it doesn’t begin to affect her spinal cord again.
The left picture is Abby in September 2018 and the right is Abby's most recent xray

Abby was diagnosed in utero with Pierre Robin Sequence, which is a tiny jaw and often a cleft palate. It was enough for us to switch my care to a specialist in Baltimore, but it really wasn’t anywhere near the concerns they found once she was born. The NICU did standard preemie xrays and discovered multiple rib gaps. They had no idea what this meant and hung a huge sign on her isolette that said “Fragile! Do not touch!”
Thankfully, a geneticist had seen a case of Cerebrocostomandibular Syndrome 30 years before during her residency. She conferred with another hospital that specializes in skeletal syndromes and Abby was diagnosed in the same day she was born.
We were fortunate to not have to hunt for a diagnosis. Because of research studies, we were also able to have a total genome sequencing done without cost. This gave us quite a bit of information and ruled out other syndromes. Most rare disease families aren’t that lucky.


We have often been asked about Abby’s long-term prognosis. The answer is that we are thankful for every day! Because CCMS is so incredibly rare, studies on long-term health haven’t been done. Most babies die in the NICU if they are not treated with aggressive respiratory care, like a trach and ventilator. We had to fight for that aggressive care and know all too well how a grim diagnosis can affect the doctor’s willingness to treat the child.
I now know of 3 adults with CCMS and that gives me so much hope!!! So while Abby is blazing her own trail, we know that there is hope and we will continue to treat each symptom that pops up. Sometimes I feel like we are playing Whack-A-Mole! Lungs...now palate...now eyes...ribs...lungs again...feeding...scoliosis...ribs...hearing...kyphosis...lungs...lungs...lungs...lungs again....eyes...kyphosis...oops eyes again...🤣
Our girl is a miracle and we are so thankful to God for choosing us to be her parents. We will continue to choose joy and be thankful for every day!