Love, Life and LillyAnna: Where the Sidewalk Ends

Wednesday, September 3, 2014

Where the Sidewalk Ends

My Dearest LillyAnna,
I am a horrible mother. I just want you to know that right off the bat. I am savoring you and this moment today because you do not feel good. You are in so much pain today. Today is a bad day. It happens like this for you some days. We are supposed to go to the zoo. Just you, no other kids and I am so excited to be able to focus on just you but I do not even know if we are going to be able to go. Do you know what I am able to do? I am able to sit in our old rocking chair. You are curled on my chest and your sweet head is on my shoulder and I can smell your breath. I can feel your breathing and you are so calm at this moment I can rub your soft baby feet as I rock back and forth.

You are such a big girl, you never let me do this anymore and we will not be having anymore babies in this house. It is so appropriate that you are deigning me honorable enough this morning, of all days, your third birthday to hold you this way. This year you have been so busy! You learned how to use the potty although it was not easy for you. On the days that you had a lot of pain (which was usually 2-3 days a week) it was harder for you to remember. But you have mastered it now and this past week you have even been waking up with a dry diaper every morning! That is pretty good for a three year old.

Your Monsters Inc. obsession has been replaced by Star Wars this year and your favorite characters are Chewbacca and Yoda. Why? Who knows? Except that you have always been your own person baby. You love to play with dolls and Barbies but you are just as happy playing with batman and light sabers. Reading books is a major infatuation lately, especially poetry. You are loving Shel Silverstein. I read you two of his poems before bed. Sometimes you look at me like I am crazy. Let's face it, you are not too far off the mark. And sometimes you laugh hysterically.

We took you skiing this year and although I was too afraid of the wrath of Dr. R in the event of an injury to actually put you on skis I did take you snow tubing. Your older brother was terrified but in true LillyAnna fashion you had no fear. You grabbed the sides of that tube and screamed with passion and joy all the way down the mountain. It was wonderful and made me cry.

Speaking of Dr. R, he tried to make me take your bottle away this year. I told him I knew how to do it, I mean I took the twins bottles away a week after their first birthdays. He said to do it. I warned him that I was worried you would dehydrate yourself. He said do it. I called him three days later. You were dehydrated. He said give her the stupid bottle. When we talked about it later he said you are stubborn. It is that stubbornness that has gotten you through this horribleness and you need it but it also sucks. I like your stubbornness.

You broke my heart about a month ago when you told me you do not want me to lay with you at night anymore. You want Daddy. You have stuck to it too. My heart stays broken. I bask in my days alone with you though. The twins are in school all day now and I get to be alone with you most days for now.

You also lost a friend. Lucy and her family took us under their wing and showed us the way of this chronically ill child world and then they lost Lucy this February. There are just no words for saying goodbye to a five year, especially one as magical as Lucy. You truly enjoyed Lucy and so did I. You always wanted to go to see her and sit in her bed. Her mother Nicole has been a true friend to me and we miss them all.

You are a three year old who still drinks a bottle but I do not care. I remind myself that I have never seen a thirty year old who drinks a bottle and you will give it up when you are ready. You know you own pain and ask for medicine when you need it. You are good at that. What other three year old can do that? You also know the difference between a pustule and a mosquito bite. Impressive. You really love lip gloss mostly as a result of the blisters you got on your lips for a few months this winter although you have not gotten them in a few months. You really love to paint your nails. You are particularly attached to your blankies; especially one made by Michele for your brother when he was a baby. Like your other favorite blankets it has a satin border.

On the days you do not feel well, like today, you sit in your pint sized lounge chair with blanket and bottle and watch movies. It is really bad when we go through numerous movies, better when you are up and moving after one movie. A great day is one where we do not need a movie and you are ready to go first thing like most three year olds... it happens.

This summer we took you to NYC to see some docs. It did not go well. They did not know as much as we had hoped, we were the bugs under their microscopes. But boy did you LOVE the city! We did learn a big lesson. It is time to move on: we are to stop treating you and our lives like you are very sick and start treating you and our lives like you have a chronic illness. As our docs said, this is as good as it is going to get. Although this has encouraged forward movement and positive change in our lives, my chest is constricted in a vice... tighter... tighter. This is your life. There is nothing else we can do for your pain. There is nothing else we can do but give you these horrible medications. There is nothing to do but wait and see what happens next.

And then there is so much to do. We can watch you grow. You are so much fun. Taking you to the zoo and seeing your personality shine through when it is just you is beautiful! I am enthralled and in awe and my heart beats fast. Your independence is at a peak. You are not big on dogs but you love birds. This winter you really want to learn how to swim. Strawberries, chicken nuggets without the breading, spaghetti and meatballs and cucumbers are among your favorite foods but you are willing to try just about anything. Straight chocolate is just about the only thing you will eat when I cannot get you to eat anything else, but it has to be good chocolate. You are simply amazing. You are so smart and so kind and so intuitive and I love you so much.

You told Nonni that your scars are beautiful. You came up with that all on your own. You have no fear and so much faith in yourself. I think that is what people fall in love with when they meet you. Because they do, people just fall in love with you wherever you go. Happy Birthday my beautiful scarred baby girl... Happy Birthday. I will just sit here and rock you until you decide you are done and get down. When that happens my heart will break a little more because this moment will be over and there are so few left and you are so precious. But I will also rejoice because watching you grow is a wonder and I am the luckiest person alive to be here to witness it.

I love you,
Mommy
2014

3 comments:

Sister Rosie, OPSeptember 3, 2014 at 3:08 PM
This is a beautiful letter to Lillyanna, Gina. Rather than being a horrible mother, you sound just the opposite. Please don't think that. You have done everything possible to make Lilly's and your own life happy. Don't worry, be happy. And once again, happy birthday to our little Lil!
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UnknownSeptember 3, 2014 at 4:42 PM
I agree with Rosie you are far from a horrible mother, and I'm sure that Lillyanna will never think that of you.(well maybe when she is 15 and wants to go out with her friends and you tell her no). :) Happy birthday Lilly!!!
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Allyson StorySeptember 3, 2014 at 7:21 PM
Gina, you are far from a horrible mother. Quite the opposite. You are an amazing mother to LIllyanna and the twins. They are so lucky to have you to call mommy. The strength that you and Lillyanna have are so inspirational. Happy Birthday little lady!
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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

Wednesday, September 3, 2014

Where the Sidewalk Ends

3 comments:

Down the Shore

LillyAnna in her Favorite Place