Love, Life and LillyAnna: September 2017

Monday, September 4, 2017

If a T Rex Crashes Your Birthday Party

My Dearest Lilly,

You are turning 6. I might say this every time you have a birthday. I am not sure but since this is my letter I am going to say it again. There was a time in your life we were not sure you would make it to this point. Every birthday of yours is a true celebration of life. The way you soak up every minute of it; the cards, the phone calls, the Happy Birthdays, the parties, the gifts, the family and friends that surround you shows your true spirit. Having people around you that you love and having a good time are what this year has been all about for you. When your pain or disease gets in the way of this, you get pissed. This is new for you. In past years you would lay on the couch quietly when you did not feel good. However, the older you get it is like you are angry that you are sick while everyone around you gets to play.
There are a lot of people playing around you. This year you went to school for the first time. It was preschool and Montessori Learning Center. You only made it to about 1/3 of the year. You were in the hospital three times for various illnesses. I don't know if it was because we started getting the vaccines we could or if it is because you went to school or if it is because you just had a bad year.

We will see what this year brings but we have Julie so I know we will be okay.
Julie... what to say about Julie. She is your best friend. She keeps our household together. She makes it possible for me to work. She comes in every day with a smile no matter what is going on in her life (that is true professionalism by the way, learn from that).

This year you also made some friends at school. But one of the best things to happen, and it happened recently, is that we got some new neighbors. Maggie is becoming one of your best friends and it is so much fun to finally see you have a friend that is your age.

This year you tried to learn how to ride your bike without training wheels but when that did not go so well and you realized you could not keep up with everyone else you asked for your training wheels back! This sums up your year. You want to keep up with everyone else. Your spunk continues to amaze us but the temper tantrums are an issue. Dr. R says they go hand in hand. He said once that you are the most stubborn kid he has ever met. That is saying a lot. But he also said that stubbornness serves you well.
You love playing with your dolls, you LOVE your hatcnhimal, and you really love Peppa Pig. We have watched every episode of Peppa Pig so many times that Julie and I could repeat every line. You also have probably every Peppa Pig toy there is (not really!). You actually play with them a lot though. You also really love your American Girl dolls. I watch you be a doctor to them and do all of the things that

We also found another baby this year who has Idiopathic Infantile Pyoderma Gangrenosum. The 19th case we know of in the world. I am beyond excited. You had a strange look in your eye when I told you. I don't know how much you understand about your disease. I worry that we, and in turn you, define you by this disease instead of by who you are: an energetic, smart, loving, six year old who loves her friends and family with all her heart. Of course, that baby is on remicade and getting better just like the rest and unlike you.
You love to go to school but are so cautious about it. It makes me sad. I know you are afraid you will not feel good during the day and are not sure how that will look or what to do. Your teachers are amazing. I need you to know that but you need to know that too. They even set up a rest area in your classroom.

LillyAnna, watching you grow up has been one of the most amazing things I have gotten to experience in my life. You are constantly underestimated by doctors and therapists. This is new territory to us. We are used to Dr. R who knows you, nurses who know you... when we are confronted with someone who says, "yes, I looked at her file." with a straight face, something is wrong. No one does that. They have not seen the pictures. They were not there for the dressing changes. They do not know that this disease rears its ugly head on you constantly and how rare that is. They have not seen the pictures of adults with lesions, lesions that cause bones to show, the ones that have lost their lives or limbs to this disease- literally.

I want you to live this life to the fullest every single day my little girl because I do not know what the future holds. Right now you are cuddled up next to me and I can hear you making that noise in your throat that tells me it hurts. You have been doing it for a couple of days. It did not stop you from having a great time at a party yesterday but it has stopped you from sleeping through the night. That is how I know. As I slide out of your bed at night you always say (in your sleepy little voice), "Mommy, where are you going?" As I leave I say in your ear, as I smell your clean hair and milky breath, "I will be here if you need me. Just call me." I mean that always my sweet six year old girl. I will always be here when you need me.
Happiest of Birthdays to you.
Mom

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

Monday, September 4, 2017

If a T Rex Crashes Your Birthday Party

Down the Shore

LillyAnna in her Favorite Place