Micrognathia (severely small jaw), obstructed airway, tracheostomy, cleft palate, absent thumb, underdeveloped thumb, underdeveloped radii, fused elbows, small lungs, heart anomalies, asymmetrical ventricles of the brain, imperforated anus, conductive hearing loss, and eyes that make your heart melt. Phew…that was a mouthful. My name is Ella Rose Monaco and my mommy and daddy tell me that I am the best thing that has ever happened to them.
Though I’ve come a long way, my entrance into this world was a rocky one. I was heavily sedated, catheterized, trached, and had many tubes and wires coming out of me my first few days of life so I will try my best to recall the chain of events J. When I was born I was breathing on my side, but whenever they turned me on my back my tongue would fall back, obstruct my airway and prevent me from getting the oxygen I needed. They tried to intubate me, but that didn’t work so they had to put a hole in my neck, straight to my trachea, so that I could get air through a tube. Still my pulmonary veins were so tiny that they weren’t doing a good job of getting oxygen to the rest of my body. The docs weren’t too sure I would survive, but I’ve been proving them wrong ever since.
You see, I’m a fighter; I don’t give up that easily. I’m also a feisty little one. Small, but feisty - just like my mom. Even though my mom thinks it is the saddest thing to see me cry, but not hear sounds coming out of my mouth, I have plenty of other ways of showing her I’m angry. I kick (mainly the bicycle), I turn bright red, tears roll down my cheeks, oh yeah and my heart rate goes above 200. Like any other baby, this usually resolves when either a) I get picked up (which my mom is a sucker for) b) I get fed or c) I get patted firmly on my hiney (I know it is a little odd, but I like it, what can I say).
I have overcome many obstacles for being less than 2 months old. I no longer need to be sedated (only when I have a big test or procedure), my catheter was removed (I do an excellent job of peeing and pooping on my own in case you were wondering), and I don’t need any IVs or special lights anymore. In the beginning I was not able to eat for a couple weeks. Now, they feed me through an OG tube that goes from my mouth down my esophagus to my belly. That silly tape that covers my upper lip (or white moustache as mommy likes to call it) secures my feeding tube in place so I don’t pull it out. I take 50mls of my mom’s breast milk every three hours. I don’t know what she puts in that stuff, but whatever it is it’s certainly working. When I came out I was a peanut, weighing only 4lbs 5oz. Now I’m filling out quite nicely; in fact I just broke the 6lb mark.
When I was first born, I relied heavily on the ventilator. Right now I am getting minimal support from the ventilator. They are allowing me to “sprint”, meaning for four hours in the morning and four hours in the evening I go off the vent and breathe on my own. Sometimes I do incredible and impress everybody, and other times I start to get a little tired. They can sense this because I start to breathe fast and my nostrils begin to flare. When that happens they put me back on the vent to let me rest. I’m still very tiny and they don’t want me to overwork my lungs.
As for my diagnosis, the docs think I am showing characteristics of a very rare genetic disorder, Nager’s Syndrome. There have only been 90 documented cases in the whole world, so I am very special. Thankfully, they ruled out Fanconi’s Anemia and my chromosome tests all came back normal. Unfortunately, there is no test yet to confirm that I have Nager’s and that is because they haven’t found the gene for it yet. Mommy and daddy donated a little bit of my blood to a research study with the hope that we can help them find the gene that causes Nager’s. Frankly, what they call my diagnosis means less to me than the fact that I am getting bigger and better each and everyday and that I will hopefully have a good quality life.
As for my mom and dad, they are doing well. My mom spends her days and nights at my bedside, holding me (she cherishes this since she wasn’t allowed to pick me up until a few weeks after I was born), changing my diaper, pumping breast milk, setting up my feeds, and taking care of my trach. My dad has to work during the week, but always comes down to spend time with me on the weekends. He even comes to say goodbye to me at 5:30 in the morning on Mondays before he leaves for work. He likes to do all my care on the weekends since he doesn’t get to see me during the week. Mommy and daddy often argue about who gets to hold me, while I’m just sitting there thinking, “someone, anyone, just pick me up already!” Mommy and Daddy are also taking a trach class on the weekends. They are learning all about how to suction my trach, change my trach gauze and strings, and change the trach itself. They seem a little scared about all the things that can go wrong, but I am confident that they will rise to the occasion.
So all in all I’ve made amazing progress (you can see this from the pictures-top being most recent). Your prayers and messages of love and support have definitely been working…so we thank you from the bottom of our hearts. God is listening and is working a miracle through my little body one day at a time. Mommy truly believes He has a parade of angels watching over me and that I myself am a little angel sent to touch the lives of many. Please continue to ask Him to heal me. I have made great strides, but I still have a long journey and many challenging roads ahead of me. I especially ask for your prayers next Friday, October 2nd, when I will be undergoing 2-3 surgeries. They will be repairing my rectum, basically rerouting it to where it is suppose to go. The docs will also be putting in a G-tube, a feeding tube that goes directly into my belly so that I can’t pull it out like the one in my mouth. So the next few weeks will be a little scary for my mom and dad and likely uncomfortable for me, but hopefully worth it in the long run.
Love and gratitude,