Love, Life and LillyAnna: The Giving Key

Friday, January 30, 2015

The Giving Key

Red: Hope is a dangerous thing my friend, it can kill a man...
Quote from The Shawshank Redemption by Stephen King

Who starts a blog post about a three year old with a quote from a book by Stephen King? Well, me I guess. And why? Because when you live our life, hope can be a dangerous thing. Since LillyAnna's diagnosis two and a half years ago my husband Chris used to very often say, "One day we are going to wake up and LillyAnna is just going to be better." He does not say that anymore. Our daughter LillyAnna has an extremely rare disease. She is going to have it for her whole life. Every day of our lives are full of uncertainty. Each moment is pregnant with the anxiety of her illness, the fear of what is coming next and the desire to keep her safe. The caregiving comes from me, her mom, who takes her temperature, calls doctors, makes appointments, and countless other tasks. But it also comes from her six year old brother and sister who run to get her bottle when she is crying or scream at me that I am hurting her when I have to give her an injection or hold her hand when she is lying on the couch in pain. Of course it comes from her loving Daddy when he sits with her and watches TV on days that she is too tired to play, or holds her bin so she can vomit or lays with her in bed to calm her to sleep.

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, yes, even Stephen King for reminding me how magnificent it is.

2 comments:

Paul QuinnFebruary 2, 2015 at 1:31 PM
I ache for what your child is going through. It numbs the mind and sears the eyes to even face that kind of odds. However, the only thing you can do is to soldier on, and provide support and therapy for the various other inflictions it might cause her. At the very least, be prepared and be present. You will all get through that, I'm sure. Here's to good health for Lil! All the best!

Paul Quinn @ MedCare Pediatric
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History

On June 19, 2012 we found a small pustule on our
daughter LillyAnna’s thigh. At the time,
she was nine months old. Until this time, she was a healthy baby who ate well and gained weight appropriately. She was also the happiest baby I have ever seen. Even though she was breastfed, she began sleeping through the night at six weeks and almost never cried. She was an absolute dream come true! Within a week there was a lesion about three inches in diameter that went down to her muscle. It was extremely painful. Later we were told that the pain was much worse than that of a burn but she still almost never cried. As per our regular pediatrician, we were treating her for MRSA. We were told to take her to the
Emergency Department if she developed a fever.
Ten days after the initial pustule, she developed a fever and we were admitted to the hospital. It took 24 hours to diagnose her condition as Pyoderma Gangrenosum. Those initial days were the scariest days of my life. Pyoderma Gangrenosum is
associated with adults who suffer from Crohn’s disease, rheumatoid arthritis and a few other auto-inflammatory diseases.
However, LillyAnna’s is only the 17th reported case in
infants in the world. Most of these infants responded to oral steroids and then are sometimes followed up with another maintenance medication successfully. These other infants were not followed past two years although we did find one who was followed for five years with no recurrences. LillyAnna does not follow that same
path. During the first week in the hospital, she developed another large lesion on the side of her abdomen and a few other small ones on her limbs. In total, she had 30 lesions during the first 2 months of her disease. We were forced to insert a PICC line. The PICC
line was necessary because she was refusing to drink or eat and she was on IV steroids. We also had a lot of trouble
keeping an IV line secure. Every time there was trauma to her skin, she developed a new lesion therefore we could not
use the standard methods for keeping an IV or PICC secure. We could not use any tape or even band-aids. Every time there was trauma to her skin it turned into a lesion. The
lesions were growing out of control and they grew very quickly. Watching our baby’s skin deteriorate so quickly was one of the most terrifying things I have ever experienced.
It was so difficult to control her pain. Because LillyAnna
was always a happy and vivacious baby who almost never cried or fussed, it was difficult to tell when she was in pain.
Even with all of this going on, she was pleasant as long as we controlled her pain. It was evident that she was in
constant pain even with morphine every four hours and oxycodone for breakthrough pain. It was difficult to
hold her and sooth her because she had so many lesions.
Finally the growth started to slow and we were sent home from the hospital about a week after our first admission with a PICC line and oral steroids. Unfortunately, in the thirty six hours we
were home, LillyAnna developed six new lesions that were about the size of a quarter on her limbs. We were readmitted
to the hospital where we stayed for the next two months. LillyAnna celebrated her first birthday in a
hospital conference room. It was
actually awesome. The hospital helped us
organize it and it was a blast. We immediately began using IV steroids again and added some other medications that
are meant to lower the immune system in an attempt to stop the remarkably fast growth of the lesions. In our first week
back, she had developed lesions on her face, scalp, and diaper area and most painfully at the PICC insertion site.
Every other day we had to change her dressings which took
sometimes over an hour. These procedures were extremely painful for her although the hospital did a wonderful job of
trying to make her more comfortable by premedicating her, using very special bandages and trying to distract her as much as possible. Although, even with all of that, it was
horrible for her and she would scream the entire time. In total she had about thirty lesions. This is a time in my life that I have to block out of my memory. No mother can hold down her screaming infant while doctors and nurses peel bandages off of her totally exposed muscle and be a whole person again. Finally we tried a treatment called Remicade. While
receiving this treatment, we were able to begin weaning her from the steroids and by the time we left the hospital she no longer needed the PICC line and was taking all of her medications orally.
Her eating and drinking were not totally were they needed to be but she was improving. It was during this admission that the vomiting began. I started to notice that the days when LillyAnna’s lesions were particularly sore, she would vomit before bed.
We were discharged from the hospital on Remicade treatments
and a plethora of other oral medications in late August of 2012. I was unable to work as her care took up the
majority of our day and we were at the hospital two or three times a week sometimes for a whole day.
Soon, I began to notice that LillyAnna was having some of
the reactions to the Remicade that we had been warned may happen when a child has an adverse reaction the first couple of infusions. She would get extremely fussy, had rashes on
her face and trunk, and was very flushed.When we went home, she seemed ok. However, we were supposed to be lengthening the time between Remicade infusions and we could not. Her symptoms would begin to worsen after two weeks and we would have to infuse again instead of waiting longer. When we would wean the steroids, she was uncomfortable and the symptoms were just getting worse. She began vomiting each night and
not eating or drinking again, she was very fussy all of the time and her lesions looked red, sore and like they were growing larger instead of healing.

Finally we had to readmit her to the hospital in November of
2012. There we found out that she was
building antibodies to the Remicade; that is why it was losing its
effectiveness and we were seeing a resurgence of symptoms. She most likely will never be able to use Remicade again. At this time we were faced with many decisions and LillyAnna was taking her first steps. We needed to find out why she was refusing to
eat and drink because it was such a problem that she was dehydrated and losing weight. Along with the advice of her doctor, we decided to scope her entire
digestive tract. This is something we had avoided in the past because of the fear that we would cause lesions to develop on the inside by causing trauma.

However, at this point it was necessary. The scope did not show
us much. There were some parts of her intestines that the doctors could not reach because they were too small even though we were at a reputable children’s hospital. Other than that, they did not see much. There were small signs of what could be Crohn’s Disease but because she was already being treated with the medications that are used to treat Crohn’s, this was non-conclusive. What they saw could also be signs of a
virus. We also did a modified barium swallow study which again was not very helpful. It showed that she had reflux which we already knew and can be caused by some of the medications she is on and some slow stomach emptying. However, she is now on the highest dose of a medication to treat slow stomach emptying and it has not helped. This is one reason we are pursuing a second opinion with our doctor’s support.
Keeping her safe causes us a lot of anxiety; we cannot keep her in a bubble but it is terrifying to think of the
sicknesses she could contract. We work hard to keep the house extra clean which is not easy because LillyAnna shares this space with her twin siblings. We keep hand sanitizer around and remind all visitors to use it often. This also means that she could not
go to daycare or school which had been a problem for us but she has since started Kindergarten. She just stays home when it is a bad day. I had to resign from my full time teaching job. There are so many expenses in a situation
like ours. They are things that Medicaid does not cover, things that you might not even think about. For example, sometimes I have to take LillyAnna’s temperature twice a day.
To keep that sanitary, we need alcohol wipes and Vaseline. The expense for over the counter medications adds up also. We have narcotics for when her pain is very bad but we always use Advil first. She gets a lot of Advil. Other seemingly innocuous stuff are things like the extra laundry detergent we need to properly clean her clothes, sheets, and pads after she vomits and the hot water.
Or the extra cleaning supplies we use to make sure the house is as germ free as possible. These are just a few examples of things that can quickly add up. LillyAnna is being followed by the National Institute of Health and even at that world renowned facility
she is considered extraordinary. There is only one case they have seen in an adult (which is only remotely related to her disease) that even comes close to resembling her disease. LillyAnna has been suffering day in and day out for years with this disease.I worked part time for awhile but now work full time with the help of an amazing Nanny. We are doing the
best we can but the strain of years of this is starting to wear on us. LillyAnn is such a happy little girl. Most days if you met her you would not know anything is wrong with her. People are
always saying, “But she looks so good!” The doctors have told us that she does not know what it is like not to
be in pain because she has lived with pain so much of her life so unless the pain is just excruciating, she is going to act like everything is fine. It is heart-wrenching. And I know this to be true because when we get home she will just sit for hours or limp around the room or on a bad day lay in my lap and cry.

LillyAnna is an inspiration to me. She embraces life to the fullest even with the intense pain she lives in, not being able to go to school and very often feeling far more exhausted than any child should. She loves to go to the zoo and observe the slow movements of the animals, especially the reptiles! She really loves to be read to all snuggled up with me or her dad and her brother and sister. Coloring is one her favorites too when her fingers are not too swollen to grasp the crayon. And when she is eating chocolate and spaghetti are her favorite foods, she is even pretty good at helping me roll meatballs. We have no prognosis for
LillyAnna. We do not know what the future holds for her. We take every day and enjoy it to the fullest. We try to soak up her zest for life and just love her.

Love, Life and LillyAnna

Friday, January 30, 2015

The Giving Key

2 comments:

Down the Shore

LillyAnna in her Favorite Place