Wednesday, July 19, 2017

The Hard Things

We went into this pulmonology appointment today so nervous and anxious about what would be discussed.  As you all know, Abby's respiratory status has really declined with no indication that it is going to improve.  It's more obvious to us every day that the rib-to-rib VEPTR is the key to Abby's lung volumes improving, but that won't be able to be done for quite some time because her body has to heal from the trauma of the last year.  We discussed that today with our pulmonologist and she is hoping that she can provide support to Abby as a bridge until the rib-to-rib VEPTR is able to be put back in--whenever that may be.

The question is, how much support is Abby going to need?  She's needing additional bipap during the day now and seems to decline after every surgery...and she has another one next week.  What will she be like after that?  The bottom line is that we aren't sure how much more her little body can take, or how long she can continue with such little lung functioning.  At 25% functioning, our pulmonologist said she typically starts talking lung transplant with her cystic fibrosis kids.  Abby is at 15%...but she is not a candidate for a lung transplant because it's not a lung issue--it's her anatomy.  Giving her new lungs won't change that.

Our pulmonologist said she never expected to have this conversation with us because Abby had been doing so well for so long.  And yet, here we are.

But there is hope.  If Abby continues to decline, she could get a trach again and be put on a ventilator.   While life would change for our family, she could be stable and safe with a trach and able to have a relatively good quality of life.  And given the alternative, we will wholeheartedly choose a trach.

For many, a trach would be terrifying.  And in a way, it's still pretty scary for us to think that Abby could be heading that direction again after doing well for so long.  But we've done a trach and vent before, and nothing can squash Abby's zest for life!  She can still be her sassy self with a trach.  It's amazing to think that a tiny tube of plastic could save Abby's life twice.

We aren't there yet.  She is currently managing with bipap support.  Right now, our awesome pulmonary team is working hard to get us a more sustainable machine that can give more support than a basic bipap can, as well as a more portable cough assist machine and oxygen concentrator.  The machines we have now are large, heavy, not very portable, and don't have internal batteries.  They certainly aren't easy to take with us to appointments!  I think the insurance representatives should shadow us for a day before making any decisions to approve or deny any equipment or treatments.

We will continue with bipap at night and as needed during the day, as well as giving her some oxygen support.  Her respiratory treatments will continue 4x a day for the foreseeable future.  After this next hospitalization, Abby will have a sleep study and then another pulmonary appointment to check on her status and discuss the results of the sleep study.  Between now and then, we will be in close contact with pulmonology and were told to go to Hopkins ER with any further decline in respiratory status.

So, while this appointment included a lot of tough discussions, at least we walked away with hope and an option.  A trach and vent might not seem like good news, but it was in a way for us because we weren't sure if we had any options at all.

Thank you all so much for your prayers.  We will continue to fight for Abby and do whatever we need to do for her because we know she's not ready to give up.  There's way too much sass in her!


Susan Shaw said...

Sending love and prayers as you face these extremely daunting decisions.

Rachel Gilman said...

Julie, I've been following on the VEPTR site. I'm praying for you and Abby and for all if your family. I know from my experiences with my own abby that we would all do anything to help our kids.

Anonymous said...

Praying for Abby & all of the family.

Aimee Yoder said...

You all are in our thoughts and prayers!

Judy North said...

OMG I have followed this little angel for some time now. I couldn't believe her lungs are at 15%!!! I will be praying for a miracle and sharing with my friends to lift her up in prayer also.

Dottie said...

Prayers are with you and your.