Life as a Leach

Today is Rare Disease Day, where all of the orphan diseases come together to raise awareness for Rare Disease.  While these individual diseases are quite uncommon, one in ten diseases is actually considered rare!  Abby's is one of the rarest, with Cerebrocostomandibular Syndrome only affecting approximately 10 people in the world! But what is Rare Disease for us? Rare disease is a toy zebra hiding among feeding bags, syringes, and cans of Pediasure. It means fighting with insurance to get essential services deemed medically necessary.  Being rare means having to become the expert on your daughter's condition, because no one else is. It is feeding pumps, ventilators, pulse oximeters, and oxygen tanks crowding a little girl's room. Rare disease means frequent phone calls to doctors, nurses, case managers, and supply companies. It is scars that tell the story of battles won. Having a rare disease means isolation and loneliness because of the threat of germs....

We enjoyed some sunshine yesterday with some unseasonably warm temperatures.  It took a little convincing to get Abby outside for some real vitamin D (we have actually started giving her a supplement because she is outside so infrequently and her blood work showed her body wasn’t making it!).  Once she got out there, she loved it! Since the tree house hadn’t been used in a while, I spent some time sweeping it out and cleaning it up.  I let out a little scream when I discovered a DEAD SQUIRREL behind the door!  Gross!  I was able to remove it with sticks without actually touching it, and then I lysoled the area really well.  Abby was very sad he died. My guess is that he got in there somehow and couldn’t get out.  Poor little guy. Today is even warmer, and we are all looking forward to playing outside again after school.  We may even eat dinner on the deck!  I’m going to savor these warm days while I can!

My friend Katie is an amazing couponer and she gifted us 30 beautiful containers of Lysol wipes!  So much germ-free goodness!  We are so thankful for this blessing and will be sharing some with our friends at RMH when we take soda tabs in a few weeks!  

Nothing has changed, and yet everything has changed.  Abby is still healthy and able to do a full day of school at home with frequent breaks.  We are still washing are hands constantly, have hand sanitizer mounted on the wall at every entrance and at the door of her bedroom, and change our clothes the second we come home when we have been in close contact with people.  She is still skyping with her class most every day and able to interact some with the other students.  I am still dealing with the day to day insurance issues, medical phone calls, and specialists' appointments.  And yet, it seemed like everything changed in that moment after Dr. C. said he felt the risk of putting the rod back in was too great.  I cried most of the way home and felt like I'd been sucker punched.  Caleb, who understands the magnitude of this decision, shed his share of tears as well.  Dr. C. didn't directly say that he would never do it, but it was clear that un...

Today did not go well. Dr. Campbell thinks putting the rod back in could disrupt everything and be too much of a risk of infection.  Her last 2 CTs show that her lung volumes are basically the same, so he feels she is stable.  We voiced our concern that she is not functionally the same, but he thinks the risks outweigh the benefits.  He wants to do another ct in May to measure lung volumes then. This is all we know and we were completely blindsided.  The nurse practitioner was just as shocked as we were because she thought we were getting a date today too.  Turns out, we are going to be waiting even longer and she may not even have the surgery at all.   We are devastated, to say the least.  This left us reeling.  We will pick up the pieces and keep going, but today we are heartbroken to hear this news.

Today did not go well. Dr. Campbell thinks putting the rod back in could disrupt everything and be too much of a risk of infection.  Her last 2 CTs show that her lung volumes are basically the same, so he feels she is stable.  We voiced our concern that she is not functionally the same, but he thinks the risks outweigh the benefits.  He wants to do another ct in May to measure lung volumes then. This is all we know and we were completely blindsided.  The nurse practitioner was just as shocked as we were because she thought we were getting a date today too.  Turns out, we are going to be waiting even longer and she may not even have the surgery at all.   We are devastated, to say the least.  This left us reeling.  We will pick up the pieces and keep going, but today we are heartbroken to hear this news.

Tomorrow, Abby has two appointments at CHOP.  We will see plastics first, then orthopedics.  If all goes well and her wound is officially cleared, we will get her next surgery date to put the VEPTR rod back in.  We are hopeful that this will be sooner than later!!  We would appreciate your prayers for wisdom of our surgical team, as this surgery will be even more complicated than usual.  The combination of Abby’s lung failure, scar tissue, and kyphosis make this surgery a challenge and we are very thankful that Dr. Campbell is willing to do it.  He doesn’t give up on kids and for that, we are so grateful.