LillyAnna was diagnosed with a disease called Idiopathic Infantile Pyoderma Gangrenosum at 9 months of age. It causes large, very painful lesions on her skin. She is the 17th reported case in the world of this disease. There are adults who suffer from Pyoderma Gangrenosum. It is still quite rare but more common than in infants. Usually it focuses on the limbs or trunk. Lil is unusual in that she developed lesions all over her body (approximately 30 in all) over the course of the next 2 months. We could practically watch them grow, that is how rapidly it happened. Once, in 36 hours she grew 6 lesions the size of quarters. At this point we have the large lesions under control but have found out that her particular pattern of healing and lesion growth is unusual, in fact only one other patient (an adult) has looked even remotely like her. The medications are not working the way they are supposed to in two ways. One, the medications that should be helping the most are not and the ones and the ones that should be supporting are helping more. Second, her disease is not being controlled as it should be. She is having breakthrough PG attacks. One of them being lesions in her mouth which as far as we can tell has only happened in eleven PG sufferers, adults, never an infant case. LillyAnna also has Juvenile Inflammatory Arthritis but this is not associated with her disease. It does cause her quite a bit of pain. She is now three and a half years old. I quit my full time teaching job to care for her and work part time when I can. Her dad works full time. Lil has twin siblings, Nettie and Chris who are 6 years old. Taking care of her is something only I can do. I truly know more about her disease than anyone because she is so unique even out of the pool of people who suffer from the same disease.
So how can we have hope? How can we have hope when countless doctors, doctors who are supposed to be famous in their fields, have never even treated this disease and cannot offer us any advice? How can we have hope when most doctors we talk to have never even heard of her disease? How can we have hope when our little girl, who is just a baby in so many ways with her still chubby feet and her so soft forearms and her bottle drinking lips is being pumped full of drugs that cause cancer and who knows what else. What is worse is that they are not even really working. She is still an anomaly. At the National Institute of Health she is an outlier. How can we have HOPE? I want to scream!
People tell me she looks so good. People tell me she looks so normal. They even look at me skeptically at times. They are right. My little girl, my LillyAnna is a strong, brilliant girl. She knows how to LOVE life. I want to rip their faces off. Because I know that her joy, her dancing, her normal three year old bounding around is going to cause hours of excruciating pain, vomiting and most likely she is going to catch some awful illness. SO GO AWAY! SHE IS NOT NORMAL! COME BACK TOMORROW! You come soothe her at 3am when she is writhing around in her bed and will not let me give her any pain medication. You come hold the bin in a few hours when she is throwing her guts up because she actually ate. Give her the credit she deserves.
My stone walls are her illness, my confinement is in my own fear. Someone once told me you can live your life in two ways, through love or through fear. If you think through each decision you make, it can come down to these two emotions and it is true. I know this but the memories of our experience paralyze me. My knowledge of the possible future freezes my heart. There are things I know that I do not tell anyone because it is just too awful and because it is all so uncertain. We make the best decisions we can and still we are judged. No one knows what it is to walk in our shoes. I know that for a fact. With Lil's newest developments, we are even more rare than ever before and it all rests on my shoulders. These walls are tall, cold and thick. My sentence is undetermined. There are so many guards preventing escape.
And then one day I got an envelope in the mail. Inside was a handwritten letter and a key on a chain. On the key is one word: HOPE. I have worn the key just about every hour of every day since that one. Someone who understands sent me The Giving Key. I cannot emphasize enough the word understands. So many people in our lives try to understand, so many support us. Without them the walls would close in and I would suffocate. However, the people who live this life know the air we breathe. They have felt these walls and walked these halls. That is a different kind of knowing. That letter touched something inside of me that has been long been stiff and still, unmoving.
Andy Dufresne: That's the beauty of music. They can't get that from you... Haven't you ever felt that way about music?
Red: I played a mean harmonica as a younger man. Lost interest in it though. Didn't make much sense in here.
Andy Dufresne: Here's where it makes the most sense. You need it so you don't forget.
Red: Forget?
Andy Dufresne: Forget that... there are places in this world that aren't made out of stone. That there's something inside... that they can't get to, that they can't touch. That's yours.
Red: What're you talking about?
Andy Dufresne: Hope.
From The Shawshank Redemption by Stephen King
My music is LillyAnna. She is my song. Sitting at her princess table in the living room putting together a puzzle with such concentration even when I know her fingers are swollen. She fights back when her sister and brother do what big sisters and brothers do.. try to control things! Perched on Poppop's knee wearing his hat and laughing hysterically. Rolling meatballs, stuffing peppers, asking me to carry her because her legs 'are getting tired'. Playing with her cousin Antonella. Engaging with pictures on my phone and talking about each picture; this is one of her favorite activities when she does not feel good. She remembers everything and everyone, maybe not by name but by something they have done for her: 'That is the guy that drew the picture or my shirt.' 'That is my hospital.' 'Those are my nurses' 'I drew that with Dr. R' 'That is Poppop's Club' 'MeeMaw did my nails that day' and on and on... and then laying next to her at bedtime she reaches out to hold my hand as she is falling asleep even after I have had to hold her down for meds and she screamed and cried for over half an hour and vomited and her lips bled, she still wants me. She is what protects that something inside. The strength of LillyAnna's fight, the beauty of Nettie's kindness, the depth of little Chris' intuitiveness and the unwavering, steadfast love and care of my husband.
There is music in my heart, thank you Megan Kelley and The Giving Key and,Stephen King.
