Love, Life and LillyAnna: I Am Running In A Circle

Sunday, May 5, 2013

I Am Running In A Circle

In order to attempt to prevent a reaction to Humira this week, we have premedicted LillyAnna with Benadryl and Tylenol. Dr. R told me to give her a dose of each every four hours after the shot. We will see how it goes. Her blood work looked ok this week. Sed rate was up from 8 to 9 but still in the normal range. White blood cell count is down from 9.3 to 8 and CRP is the same at <0.5. I have stopped seeing a clear pattern in her symptoms which Dr R and I agree could be from building up antibodies to the Humira but could also be her body adjusting to Methotrexate.

Dr R consulted with rheumatology on Tuesday about a test that determines if antibodies to Humira are present. They told him one did not exist. He went looking himself just to gather information and found a test. How did this happen? It was approved that day! We should play the lottery! First a disease that is rarer than rare and now this! Lol!

As soon as they can figure out the logistics we are going to test her. It is only a blood test, she gets blood draws each week so this will not be a problem for her. Dr R and I also talked about the fact that her blood work does not really correlate with her symptoms which is very perplexing. He mentioned that he is glad we are going to Cincinnati because if she is building antibodies to Humira, he would like some other opinions on where to go next. Enbrel is the only other drug in this category and he does not think it would be a good option. We did not get to talk about why. One thing I did notice this week is that she was fussy and temps were elevated before her lesions started acting up. She did not eat for two days either and was up at night. She has been pulling at her lesions and crying saying they hurt. She has been limping a little and falling over. Hopefully now that she got the Humira she will start to feel better.

Seeing him and touching base with him did wonders for me. We talked a lot about my options for being able to work next year, my daily life with her and what is happening with her disease. We also discussed Nettie and her stomach issues. Nettie is going to see a GI doctor. After that, we will try to see if there is a connection between the girls and their stomach issues. Lil was supposed to get her shot Friday but we had issues with the pharmacy (not our local pharmacy, our local pharmacy is amazing) so she did not get it until this morning (Sunday). Last night was vomit city. Not much fun when you are on vacation! Luckily we have loving and understanding family who is willing to work through this time of our lives with us.

We are now in Cape Hatteras for vacation. Even with two four year olds and Lil our trip down was wonderful. The kids were great, no one threw up and we stopped for a nice dinner. After the hell of getting the Humira taken care of Friday, the drive was a welcome retreat. Unfortunately, when we got in and settled I attempted to put Lil to bed. Silly me, thinking I could go a day without puke! She vomited three times. And in my relaxed state, I was not prepared. Therefore I got hosed (think The Excorcist), literally. We both ended up getting a shower at midnight.

I am still waiting for Dr. R and the doctor from Cincinnati to connect. They are both very busy and have been calling each other every day this week. Once that happens, we can move forward. Senator Carper's office has been phenomenal. I sent my letter in an email format and paper. Someone got back to me the next day and we are moving forward with that also.

Things are moving forward and I feel good about that but my stress level is through the roof. It is good because it is keeping me energized and I am not eating too much! LOL! Nothing is easy. Everything is taking ten thousand phone calls, I fill out paperwork and have to fill it out again. It is times like this I am so overly grateful for the help we have been given and continue to get. It comes in so many shapes and sizes, it comes from unexpected places and people and it warms my heart every day.

Lil's first ice cream cone... she loved it!

1 comment:

AnonymousMay 5, 2013 at 1:34 PM
I love her eating the ice cream cone.My favorite too.
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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, May 5, 2013

I Am Running In A Circle

1 comment:

Down the Shore

LillyAnna in her Favorite Place