Love, Life and LillyAnna: The Birthday Queen

Sunday, September 6, 2015

The Birthday Queen

My Dearest LillyAnna,
            This week we celebrated your Fourth birthday. This year you have grown in so many ways. I am amazed every day by you. Each day you show us a strength and love of life that is beyond anything I could imagine. You still wake up most days not feeling very well but it never stops you. You live with pain almost every day but you have continued to meet milestones that three years ago I would never have thought you could meet.
            This year you had your first sleep over. Of course it was a family sleepover and not a friend sleepover but you loved the independence just the same. You take your suitcase out and pack it up all by yourself, you fill it with favorite books and some candy. I have to remind you that clothes need room too!
             You also always have to have your green or blue blankie. You call them your "shovey" blankets because they are trimmed in satin material and when you drink a bottle (yes, you still drink a bottle) you rub your little hands on the soft edges. I love it because an old friend of mine is the one who made them (Thank you Michele) for the twins when they were born but you have taken them as yours. They go everywhere you go, or at least one of them. At night when we lay with you as you go to sleep (yes, we still lay with you to go to sleep) you pull that blanket up to your chest and flatten it out so you can "shove" on it. You pick whether you want to lay with me or your dad. For most of this year you had to have daddy at night but just recently you have wanted me. And after your shots, which you still get weekly, you always want me too.

The coaches at Graylyn Crest let you join the swim team this year even though you could not really swim. This was huge for you because you are not allowed to go to school. Being a part of the team made you feel like such a big girl. I will never be able to thank Coach Amber enough for how she included you and made you feel like part of that team. Now it is the end of the summer and you are swimming. Of course, your winter swim coach (Danielle) had a lot to do with that too! You hid your swimming from me for weeks. I could not believe it. You would not let any of us see that you could swim but Melissa caught you swimming on your own one day and then it was all over. You have been all over the pool and I have been right by your side. You have the biggest look of accomplishment on your face each time you swim further. The best part of swimming this summer is that it has helped with your pain. One day I asked if you needed pain medicine (you were obviously hurting) and you told me you did not need it because you were going to go swimming and that makes you feel better. How are you this aware at 4? It blows my mind.

This year you were a Philadelphia Mummer for the first time. We all ended up in the show believe it or not. You were so sick and ended up in the hospital the next day but true to you, you refused to leave the Convention Center and stayed for both shows. Poppop was so proud and Golden Crown even dedicated that year's show to you. We were all in awe of your determination to be in the "Big Show". What we discovered the next day was that your mouth was full of mouth sores that turned out to be Pyoderma lesions. This is particularly scary, you are only the 6th case as far as we know of PG in the mouth. These mouth sores have affected you all year but again, you keep on.
The other big milestone this year is that you have started riding your big girl bike. We are not taking the training wheels off yet but you are determined to keep up with your brother and sister. You speed down the street behind them trying to catch up. Again, your look of accomplishment is priceless.

             Your favorite things to eat have not changed much. You love my meatballs and gravy and can already roll a meatball all by yourself. You can stuff peppers and crack eggs, you can even separate them! It is your favorite thing to stand at the kitchen counter and cook with me or stand at the sink and 'wash dishes' while I am cooking. Chicken and chocolate and pasta are your favorite foods.
            You are a four year old in every sense of the word. I watch you wanting to play and engage with Nettie and her friends even though part of you still wants to hang back and be with me. You are trying to figure out how this whole social structure works and where you fit into it all. My heart hurts for you that you do not have your own group of friends. You not being able to go to school is not easy for any of us. You want to go to school so badly but the doctors are still telling us it is not a possibility. I am hoping that will change for you but who knows what is going to happen. For now, you make friends when you can. You have made two friends at the pool and it is fun to watch you interact with them but it also hurts me to watch sometimes because I want you to have everything.
          I think we have finally found a nanny who will meet your needs wonderfully. It is a hard expense on us but you need someone who is going to stretch your thinking and get you moving when you are able. Each day I come home after you have been with her, you are just so happy. I have not been emotionally available to you and Chris and Nettie and Daddy lately. I lost my sister this year. I know I cannot allow my sadness to affect you but it is so hard.  I am so sad and so anxious. You do not deserve it and you do not need it. You have your own battles to fight. But someday you will understand the love between sisters, its ferocity.

Your party was especially fun for you this year. You were so ridiculously happy to have everyone around. No one could believe you chose a Star Wars theme. The funny thing is, you wanted that last year too! You are definitely your own person girl! You really loved opening all of your presents. I am always excited for Christmas but after watching your excitement when opening gifts this year I am even more excited.

           I love you LillyAnna Rose. I hope I am doing everything I can for you. I want you to have everything in life you deserve. Knowing how much pain you are in some days, knowing how badly you fight to live the biggest life you can, I can only hope I am guiding you the way I should be. There is no road map for you. We are on our own path. I just want it to be amazing for you in its own way.  We all love you so much. Happy Birthday.
                                                                             Love, Mom

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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

Sunday, September 6, 2015

The Birthday Queen

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Down the Shore

LillyAnna in her Favorite Place