We were told she was deaf and nothing could be done about it....so I took her to a few different places and now she hears (well...she heard. That's a different post for a different day). We were told she was completely blind and nothing could be done about it...so I proved she had at least light perception in one eye and, after being completely blown off by an entire team of doctors, took her somewhere else and now she sees some out of one eye. We were told she likely didn't have enough brain matter to even breath independently once she entered the world...so we gave her a chance and she breathed without extraordinary support. We were told she would spend her life in an infantile state...so I ignored the haters and now she scoots across the floor, cruises aimlessly in a gait trainer, sits on occasion, and purposefully plays with toys. Most importantly, she smiles and laughs. She's still severely disabled and it's hard to watch my friend's nine-month-olds fly right past her developmentally but Aria is making progress.
Around the age of one year it became apparent that Aria did have the cognitive capacity to use her arms. I made an appointment to see an orthopaedic surgeon who specializes in radial club hand for when Aria would be 18 months old. It was at that appointment we discussed a plan for Aria's arms. Part of that plan involves moving one of her four fingers on each hand over, rearranging some muscles and tendons and creating a thumb. I was told, in order for the new thumb to remain alive, Aria needed to be THE SIZE of an 18 month old...otherwise her capillaries wouldn't be able to support the newly placed appendage. We were sent home and told to come back when she was the size of an 18 month old.
Well, shit. Aria is good at many things. Growing is not one of them.
|Princess Aria holding a silver rattle during her One Year Old photo shoot.|
He said he'd like to see her be around 30 pounds to do the surgery.
You guys. Daisy is almost five years old and doesn't weigh 30 pounds. I don't know what 18 month olds he's been looking at but they certainly aren't the ones coming from mine and Kevin's gene pool. At this point Aria was wearing 3T shirts and too-long-for-her 24mo pants.
So I really started pushing feeds. I was aggressive in increasing continuous enteral feeds over night in order to get Aria to gain weight. She's so sedated from all of her medications over night that we are able to increase feeds without much trouble. We haven't been able to increase the size or frequency of daytime feeds since Aria left the NICU. She freaks during daytime feeds. She hates it and I hate it for her.
In trying to grow my child to the size of an 18 month old as defined by the hand and arm surgeon I succeeded in growing a 26 inch tall three-and-a-half year old who weighed 27 pounds. She was obese. The same people who had labeled my child as "failure to thrive" earlier in her life were now trying to put her on a diet. The know-it-all in me felt validated. Before I went elsewhere and got Aria her Fanconi Anemia diagnosis I was constantly at odds with the physicians managing Aria's feeds. The doctors told me the reason Aria wasn't growing was because I didn't feed her enough. My assertion was that there was obviously an overlying cause for all of Aria's problems and perhaps the reason she wasn't growing and wasn't able to tolerate increased bolus feeds was because her unknown overlying condition makes her unable to grow and that shoving more food into her distended belly wouldn't make her bigger.
Case and point: I was able to max-out the calories on her renal formula and increase feeds while she's shnockered overnight but it just made her fat...She had gained 25 pounds since birth and 13 inches. That's one messed up height to weight ratio.
Once they started pushing to put Aria on a diet, I told the renal and complex care teams managing Aria's feeds that I was not willing to put her on a diet until the orthopaedic agreed she was big enough for this quality-of-life surgery. Aria's neurodevelopmental pediatrician went to bat for Aria, calling the orthopaedic and explaining that developmentally now is a perfect time to give Aria more functional hands and arms and that, given her diagnosis, it's possible that she might never be the size of the 18 month old he is picturing...and VOILA! Aria is suddenly the perfect size to have the surgery. It's amazing how easy it is to get awesome things accomplished for a child when a physician rather than a parent is doing the advocating.
Aria is scheduled to have her first surgery on her dominant right arm on August 11th. Pins will be placed in her wrist so her hand faces outward rather than turning in toward her forearm (this will give her a further reach) and one of her four fingers will be moved into a different position to become a thumb, allowing her to grasp objects for the first time in her life. She will be in a cast for three to four months and then the other arm will be operated on. All of this will happen if Aria's platelets stay about 40. They are currently resting slightly about that number.
These are the types of procedures we find to be most important for our child. These are the procedures that will make her life better for however long she is on this earth.
|We bought Aria the PERFECT shirt to wear on surgery day!|
|Aria's radial ray deformity on ultrasound.|
And when she was born, Kevin and I were shocked to learn she only had four fingers on each hand. We had sat in a room with a high risk pregnancy doc counting all 10 fingers on ultrasound just weeks before.
The missing thumbs were just an extra shock...a bit hard to get used to. It was the first thing I noticed the first time I was allowed to see my child. I didn't see the tubes or the machines. I saw her twisted arms. I saw simultaneous beauty and dysfunction in them.
Then to learn that she was deafblind on top of these limb deformities was nearly impossible to fathom. There are actual legitimate communication systems for individuals who are deafblind. There is an entire language (TASL) used by the deafblind community...an entire language that interpreters know and use to help give deafblind people access to the seeing and hearing world and the deafblind individual who I love was being excluded from using these tools because of her arm and hand deformities. Every time I went to a conference or watched a video where I saw deafblind individuals communicating so well through the use of their normal hands it stung. And, even now, knowing that Aria's hands and arms will be fixed I know that missing one finger is still going to pose a problem for communication in the mainstream deafblind community. Talk about a twisted fate.
|Baby's first arm braces!|