This is what Piper Jane’s condition is called. I decided to write about it even though many of you already know a lot about it. I chose to make the name of it the title of this post because I’m hoping it will come up if googled, creating a way to get in touch with other families who are experiencing it. It is an extremely rare syndrome, making it a sort of isolating experience. I would just die at the opportunity to meet a family going through what Jake, myself and especially Piper have gone through. With less than 50 recorded cases and most of them not surviving birth, it’s trick-freakin-y. I google it every once in a while hoping to find out more information, or recent discoveries. Who knows, maybe some other hot, young mother is doing the same!!

I’m going to write some important details in Piper’s history, just to fill everyone in…

When I was 17 weeks pregnant, I had an ultrasound resulting in two important finds: a sack of fluid on the back of pip’s neck and a very small, underdeveloped chin. Both big, red flags that there were probably many more problems not able to be seen while in utero. The rest of my pregnancy was blood tests, an amnio, millions of ultrasounds, fetal echos, MRIs and such to try to figure out how to save this special baby and treat her properly. She was of course born 8 weeks early through emergency c-section.

They diagnosed her shortly after she was born with CCMS. The two most common factors in CCMS is the Micrognathia (underdeveloped chin) and the rib/ chest abnormalities. Piper is missing half of her rib cage, which is the suspected reason she has so many breathing issues. She has six on one side and seven on the other. Since the structure of her chest is so abnormal, her rib cage is also too small, so her lungs are prevented from expanding all the way, resulting in quick/short breaths….resulting in burning pretty much all of her calories on breathing so hard, resulting in the teensiness. Along with many of her ribs, one of her scapula and clavicles is still cartilege, so positioning and therapy is vital for her comfort. Her scoliosis and kyphosis (curved and “humped” spine) is taking a negative turn, so I expect we’ll be working on that soon.

I have spoken with a surgeon in Philadelphia specializing in rib surgery and while he thinks Piper is a great candidate for rib expansion, he doesn’t know that she would survive such a major prcedure at this time. He thinks we should wait. I concur!!!!!

Piper has pulmonary hypertension which used to be a really serious thing, but thanks to her incredible doctors, they have found the perfect cocktail of medications to keep it under control and stop scaring the pee out of us. She has mostly been cured of cross-eyedness and has full hearing in only her left ear. Her right nostril isn’t an open airway and the right side of her heart works harder than the left. Making a thick muscle around it. I think that’s it!!

This is a small post compared to the procedures and episodes she has endured. Really, microscopic in comparison. Writing and reading about her makes me love her, apreciate her, adore her and completely stand in amazement at her fighting power! Piper, why do you want to be here? Why are you so awesome? Why do you hate getting your nose wiped? Why did you choose to be a Breinholt? I know I’m going to be answered all of my millions of questions some day. I know a lot more than I did before Piper. How does a baby do that?

I feel it is very necessary for me to be open about Piper, mostly because her condition is so rare and she is such an inspiration. I do try to be respectful to her in the way that I write and hope that I’m doing a good job of it. I love her to the depth of my soul and the tips of my toes. I’m proud of her every day for her progress, strong will and just for being her unique little self.

Sorry I brag so much! Don’t be jeal!