Monday, September 18, 2017

Hopkins

I brought Abby to the Hopkins ER this evening at the request of her pulmonologist.  I called her this morning to fill her in on the weekend and to tell her it was continuing.  Then, I let her know that even on bipap, her respiratory rate is still very high, which was new to us.  Usually, bipap does the trick.   She told us to come here, so here we are.  Hopkins has great expedited ER time when the doctor calls ahead for you, so we went almost immediately to a room, and then she got a chest X-ray shortly after.  

The X-ray showed that the Right lung is diminished, which isn't surprising.  "Noticeable deformity of spine and ribs."  Oh my gosh!  Seriously?  We had no idea! 🤣. That one made me chuckle.  I really hope the radiologist didn't think that was new information for us!

An IV was finally put in on the 3rd try and her blood gas (CO2 level) was good, so that was reassuring.  But her respiratory rates and heart rate are still very high (RR is 66 on bipap and HR is 142 asleep as we speak.). The decision they are making is PICU versus floor, so I'm not sure how long it will be.  "Soon" is a relative term in the hospital.

Sunday, September 17, 2017

Plugging Along

Our CHOP appointments last week were productive.  Her plastic surgeon was thrilled with the progress of her wound.  It should be closed by the end of the month!  We will see him in mid-October and if everything goes well, Abby will be cleared to proceed with the VEPTR reinsertion!!

We had some fun at the aquarium after her appointment.  Abby really loved being out and it was the perfect scenario--the place was empty!!



She had her MRI the next day and recovered well from that.  We came home Thursday night to a very happy boy and dog!





This weekend has been kind of rough, sadly.  Abby's respiratory rates have been very high and her saturation levels have been low...we want them the other way around!  She's been on her bipap a lot this weekend and we have had to stay inside.  Even then, her respiratory rates have been in the 70s.  She has been SO out of breath and has been really frustrated with herself. She is literally gasping for breath while lying on the couch.  She's fine on the bipap, which is why we didn't take her in, but it's still a little concerning. I'm calling our pulmonologist tomorrow to see what she thinks.

Tuesday, September 12, 2017

Simple Joys


Abby loves to water the flowers with her watering can!


We visited the puppies one last time before they go to their new homes.  She is going to miss these dogs!  They have been a bright spot in her summer.


Abs hasn't taken a "real" bath since December because of her open wound.  Just think about that for a second....sponge baths and hair washing that takes 2 people for 9 months. Lying down on a bath bench to wash your hair to avoid getting the wound wet for 9 months.  (Well, actually, she had 4 months in the halo when she couldn't bathe or wash her hair at all!!!) Crazy.  This girl LOVES her bath and has so missed playing.  She asked if we could put a little water in the tub tonight so she could walk around in it.  She had the BEST time stomping, sitting on her bath bench kicking her feet, and playing.  💜

Tomorrow morning we head to CHOP for a few days.  Abby has an appointment tomorrow with plastics.  We know he is going to be excited about her wound!!


I'm so thrilled we are almost there!! (Remember, all of the pinkish skin was open at one point!)

On Thursday, Abby will have a dynamic MRI of her full spine.  This will give us good information about her curvature and the degree of her kyphosis (which we know has gotten worse since hardware removal.) We will also learn more about her lung volumes and what her chest walk really looks like these days.  It will be excellent info for Dr. C to have in preparation for the rod reinsertion.  

The MRI will take about 4 hours, so she will be under general anesthesia.  We are hopeful that she will wake up fine without any additional breathing issues, but we are in I chartered territory these days!  The anesthesia team is booking her a room just in case, and we are praying we won't need to stay.  But we have learned to pack as if we are!

Saturday, August 26, 2017

Today, We Were Almost Normal!

What an awesome day!  The weather was beautiful with low temps and humidity, which meant Abby could be outside!  We took full advantage of this today!  Her cousins were staying here, so they played in the water table, laid out on the hammock, and played with the fairy houses on the deck.  







Later, some friends came to visit and we sat on the deck and ate had popsicles and played with light sabers.  There was a lot more hammock time too!  They all had a great time running around.



In the late afternoon, we took a rare trip out of the house to our local beach!!  There were only a few families as far as we could see, the weather was gorgeous, and the kids had a great time playing in the sand, collecting shells, and getting their feet wet.  (Getting truly wet is a big no-no for Abby for several reasons, so we just told everyone not to go in any farther than mid-calf.)









After a while, sweet Abs was so tired that she laid on the beach blanket!  She actually went upstairs and crawled into bed early!  I'm pretty sure this was the biggest day she has had in a long time, but she loved every second of it.  The sweet giggles of those little girls is music to my ears, and their special "Best Friends Forever" cheer they made up made my heart melt.  I hope they really will stay this close forever!

Matt and I declared today "normal," which we definitely haven't had in a while.  It's true that most normal people don't do respiratory treatments every 4 hours or hook their kid up to a ventilator, but we are slowly realizing that life can resemble some sort of its previous normalcy.  The difficulty of the last year has made us even more aware of just how precious days like today are.  



Friday, August 18, 2017

Ortho Meeting






We saw Dr. Campbell this morning.  He took one look at Abby and said, "Well, we've gotta get that VEPTR back in!"  We wholeheartedly agreed!  When we asked about the opposing opinion we had before, he noted that in this case, being old equals having more experience in these situations.  He will talk to the other surgeon to let him know the plan.

So what is the plan?  Well first, we have to get this wound healed up.  Nothing surgical can be done until it's closed.  When it is closed, we can schedule surgery.  The good news is that the wound shrunk a centimeter in both length and width in the last week!!!!  This is fantastic news!  

The kyphosis still needs to be dealt with, but the ribs (well, the lungs) are the primary concern right now.  We may still go to Boston for the kyphosis, but we are going to take care of this first.  

He also LOVED her shirt and took a picture of her to use in a slide for a presentation he is doing for the FDA next week!  😍

We are so, so thankful for Dr. Campbell.  He is a wonderful man who will never give up on a kid.  Abby is complicated and this surgery is even more challenging than the others due to scarring, but he said he wouldn't suggest it if he didn't think it would work.  We have hope, and that's the best possible outcome of this meeting!

We are now waiting for a CT so that he can get a little additional information about her lungs.  It won't change his approach, but does give him more information before he goes in.  She will also have a full spine MRI when we come back in September.  Dr. Campbell really likes to have as much information as possible.

Thank you all for your prayers!!  We would ask that you pray that Abby stays healthy and that her wound heals quickly and without further complications.  Then we can get that VEPTR back in as soon as possible.

For those interested: (X-rays below)



The picture on the right is when she got both VEOTRS in.  Note how open her ribs are and how relatively full her lungs are.  (40% lung capacity is awesome compared to what she's getting by on right now!). The picture on the left side is her most recent X-ray.  If you zoom in, you can see that her ribs have collapsed.  The loops were anchors for the VEPTR that stayed in and have drastically moved position.  Her shoulder blade has become tucked under her spine, so he will have to pull that back out as well.  It will be a bigger, more painful surgery, but it will be worth it to help her breathe better.


Thursday, August 17, 2017

Pulmonary

We had a pulmonary appointment yesterday where our Pulmonologist stressed the need for a plan for Abby from CHOP.  She echoed my frustrations from a medical standpoint.  We are really hoping that tomorrow will give us answers, but we have also gotten an appointment for Boston and are pursuing those plans.  

We keep detailed data about her oxygen levels, respiratory rates, and necessary interventions.  I took it in to our pulm and she was very happy to have it.  After examining the data and the trends, she decided that it is best for Abby to be on bipap twice a day during the day.  She reiterated the big concern for germs and the danger of her catching even a slight cold.  We have decided that Caleb will change his clothes from school as soon as he walks in the door to hopefully keep those school germs out of our house.  

So, as we already knew, no school until there are some big improvements.  Our pulm also helped us to create a plan for Abby flying to Boston, which is a new issue we didn't think we would face.  Changes in altitude and pressure can wreak havoc on kids with respiratory illnesses, but we have a plan to keep her safe.  

Please pray for our appointments tomorrow.  We are hanging out at the hospital tonight and will head to the hospital first thing in the morning.  The bottom line is that we need a plan.

Monday, August 14, 2017

Appointments, School, and Looking Toward the Future

This week is going to be a busy one around here.  Abby has 2 appointments in Baltimore on Wednesday.  She needs her hearing aid mold redone (it squeals constantly!) and she has a pulmonology appointment.  Not too much has changed with her breathing--she is usually still requiring daytime bipap, and sometimes needs it twice during the day.  There doesn't seem to be a rhyme or reason to it, except that it is always in the afternoon.  We have kept the temperature in our house lower in hopes that heat will not be a factor, but the weather has really been pretty mild anyway.  I don't think our pulmonologist will change much at this appointment, since there really haven't been improvements.  I have a feeling we will just "stay the course" with daytime bipap as needed, respiratory treatments and airway clearance 4x a day, and inhaled steroids 2x a day.

On Friday, we have appointments at CHOP with the infectious disease team and our orthopedic team.  This is the long-awaited discussion about what the plan is for Abby.  We are hoping that they will know where they will go from here and be able to give us a timeline of sorts.  They avoided us for 3 admissions, despite us practically begging them to come, and we are pretty sure it's because they really don't know what to do.  So now we have made an outpatient appointment so that they have to see us and gave them 3 weeks to figure it out!

Abby is complicated and we get that.  I know they have exhausted most surgical options.  But we aren't ready to give up and we don't want them to be either!  I've been researching other methods, doctors, and cases like crazy in the past few weeks.  There has to be something we haven't tried.  Matt and I have decided that if we don't leave this meeting on Friday confident that they have a plan and are willing to keep fighting for Abby, we will pursue a different surgeon--most likely in Boston.  It's not ideal because if we travel to Boston half as much as we travel to CHOP, the airfare will be ridiculous.  Not to mention, there are a lot of factors involved in switching to another hospital.  It's not a simple process.  But we will do whatever we need to do to take care of our girl.

So this week, please pray that the surgeons have a plan and are able to discuss with us where they plan to go from here.  This will determine how our pulmonologist proceeds and whether or not we will continue to be seen at CHOP.

Also please pray for Abby and Caleb as the start of school draws near.  Abby is not going to be able to go to school for the foreseeable future, which is heartbreaking for all of us.  I'm not sure that she quite understands what this means, although we talk about it a lot.  She will be getting home/hospital teaching each week, in addition to what I will do with her, but we don't expect that she will actually go to school for quite some time--if at all.  For a girl that is as social as Abby, this is a tough pill to swallow.  Caleb has also had a hard time with realizing that Abby won't be there with him.  That boy sure does love his sister.

The main reason for her not being able to go is that our pulmonologist has said in no uncertain terms that catching anything would be very dangerous for Abby.  Couple that with her need for intermittent daytime bipap and an unstable respiratory situation and it's just not a good idea.  When I asked our pulmonologist about school, she flat out said no.

This will also mean, of course, that we are extremely careful about who comes to our house. Being healthy is an absolute must--if you think you have a sniffle, don't come. Everyone removes shoes at the door and gets hand sanitizer.  Frequent handwashing is a must.  Naturally, no one who has not had the flu shot will be allowed to come to the house this year.  Since our pulmonologist has already said that Abby really can't go anywhere unnecessarily (meaning basically doctors' appointments), we are hopeful that we will be able to keep Abby's exposure to germs to a minimum.  It will certainly make things interesting around the holidays, but at least family members (who are healthy and who have had the flu shot) can come here to visit.

There have been a lot of plot twists since May, and most of them we would not have chosen. But what I am learning more and more each day is that God gives us grace for the moment and He knows Abby's entire story.  Since we can't skip ahead to see how the story ends, we are simply trusting that He will take care of our sweet girl that He loves more than Matt and I ever could.