Love, Life and LillyAnna: Little Miss Birthday

Monday, September 2, 2013

Little Miss Birthday

LillyAnna,
I am not sure how someone who cannot speak in full sentences is able to inspire me but you do. Today is your second birthday. We celebrated with a beautiful birthday party surrounded by the people who love you the most. Last year we were celebrating in the hospital. I have had to make some drastic life changes. In this last year, your strength has inspired me to do and be things I have only ever hoped. Last year you were pretty obsessed with Monsters Inc. It is funny, your brother and sister were pretty much not allowed to even see a TV until they were two but you had already had a favorite movie at one! Of course, you have extenuating circumstances. Throughout this year you have stayed in love with Mike, Sully and Boo but now that the year is ending you are also enjoying Elmo and Mickey. Actually, Mickey is in the lead right now! Which is good because we are supposed to be going to Disney as a treat from your grandparents later this year. I hope that goes through for your sake. You deserve to smile.

You also really enjoy singing the Batman song. For Halloween this year you picked out a pink batgirl costume. I feel like that sums you up pretty well. You are not afraid to love what you love and it is a mixed bag. You will sit and play dolls with your sister and then turn and play trucks with your brother. I will be in the kitchen baking and turn around in response to "Mom!" only to see you smiling up at me with play high heels on your feet (usually mismatched), a Batman cape on and a princess crown holding hair in a birds nest on top of your head and pushing a good amount down into your eyes grinning from ear to ear.

You do not eat very much but when you do it is all about chocolate.... chocolate, chicken (which you call eggy), cucumbers and macaroni and cheese. Unfortunately, you throw most of it up. You also love my buttercream icing. One of my favorite things is when you sit in your high chair (although you boycotted the high hair about a week ago) and I give you spoonfuls of buttercream icing while I bake. You get so excited and happy each time I hand you the spoon and you take your time licking the icing with your eyes trained on Monsters Inc. You have even started potty training and just today you went the whole day in panties. You had three accidents but you are only just 2! When the doctor asked why I was potty training you I told him you insisted and that is exactly what happens with you. You insisted on being potty trained. You insist on continuing to use a bottle. You insisted on giving up the high chair. You insist on enjoying life.

We spend a lot of time together, you and I. For the past year I have been on a leave of absence from teaching to care for you and this summer I resigned my position with the school district. You need me and it is that simple. You cannot go to any kind of daycare because the medicines you are on weaken your immune system and you have not had your vaccinations since six months. But really your disease just keeps surprising us and there is no other case like yours that we can find. So you need me. In order to make things work I am bartending and baking. It has been a hard transition.

It goes against everything I did with the twins but I rock you to sleep each night. You know how to put yourself to sleep and have been able to since about 6 weeks but you throw up so much at night that it does not seem right to make you go through that alone. We are home together all day and while the twins go to school a few hours a day, it is just you and I. I also rock you to sleep at naptime. If I go to the store, you go too. If I am in the bathroom, you are there too. I can't even take a shower without you opening and closing the door every two minutes. If I am being completely honest, I don't mind at all.

Your brother and sister who are now only 4 years old take such good care of you. Whenever they are not around we have roll call. "Where Bubby?" "Where NetNet?" Where DaDa?" They worry about you so much. With all of that worry and all of the attention you get, one would think that they would be jealous or angry with you. I have never seen Nettie get aggressive with you, ever. No matter how mad at you she gets. Your brother gives you a smack with his gaga every once in a while but nothing major. Today I caught him holding his ears when we went to get blood work. I was wondering what he was doing until a few minutes after they stuck you he took his hands away and said, "Is she done crying? Is she ok? " Your sister swears she knows how much you threw up each night because when she tries really hard she can see through the wall to check on you. They make sure you are in your car seat correctly, give you their last piece of candy if you are crying, and always check that you have a space to sit.

I am so sorry. I can't fix this pain for you and I can't figure out how to help you. I am the one who is here with you all of the time and we are so close. When you are in pain I can tell and when you do not feel good I can tell. It is all up to me to stand up for you and to make sure everyone knows what it is like to be you, what you are going through. I don't think I do a very good job. Doubting myself is just something I have always done. I wish I could say I am one of those moms who is like a mother tiger and springs into action when her baby is in need; but at this I fail you. I allow people to talk me out of what I know in my heart to be true. Being over sympathetic to my kids has been a fault for the last four years. I guess there are worst faults in a mother. But with you I know when I am right and most times I am right. You just play it off so well. The distractions of the world invigorate you. You steal energy from being around people and it fulfills you. At night when we are alone and you are squirming in discomfort from who knows what pain or when you are asking for your bin to throw up for the umpteenth time, I know I was right. Your love of life and your strength is what gets me up each day. It is what keeps me going when I feel like the world is collapsing on top of me. Because of your illness I have changed my entire life and my own strength astounds me. But it is all for you and because of you. Your brother and sister deserve me too and their annual birthday letters will show their special connections to me but this is your letter. This is your second birthday. I am so afraid for your future but I am so enjoying your present. Happy Birthday little one. I love you.
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

Monday, September 2, 2013

Little Miss Birthday

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Down the Shore

LillyAnna in her Favorite Place