Love, Life and LillyAnna: April 2014

Saturday, April 5, 2014

Quick Update 10

So Lil did not behave while I was on my business trip! She has been running fevers up to 104.8 that would stubbornly only go down to 102.6 at the lowest with the advil/ tylenol three hour rotation. Dad was a beast and handled it all but the grandparents came through and helped out but coming by for dinner one night and then taking the twins for a sleepover another. I am on my way today to hold my sick baby in my arms but her fever did finally break yesterday afternoon. If it follows the pattern of the last month, I am wondering if it will be back but for now she is calming down. However, the vomiting has increased. Her lips do not look good although for some reason I cannot post pics from here. I will add them from home. I have to say it feels wonderful to be thinking like an educator. As I was driving here I called a colleague and told her I was not sure I could do this anymore. She assured me that I could, that this is who I am. It took about five minutes and then the part of my brain kicked into high gear and I felt whole again.

Wednesday, April 2, 2014

T-shirts

Your response to the t-shirt sale has been overwhelming. I wish you could know how thinking of you all wearing these shirts gives me strength... more on that later. For now do not use the button to buy a shirt! We still have one more step to take to complete the process! My wonderful cousin set it all up and I have one more thing to do. You are all so amazing. I am hoping to be done tomorrow afternoon. Unfortunately if you do order online you will have to pay $6 in shipping. I am sorry about this cost. So if at all possible try to get them from me or one of our family members or close friends. I can post a list of people who have offered to sell shirts if that would help. If you would like to help sell shirts, let me know and I will get you some order forms and envelopes.

LillyAnna's T shirt Sale

Oh The Thinks You Can Think

We decided bad news first right? Well I guess technically I decided and you do not have much choice so I hope that is ok with you too. As you have no doubt noticed, Lil has been on a steady decline for a few weeks now. It is a slow decline and it is not serious enough to hospitalize us but it is a decline nonetheless. She has been in more and more pain, temperatures that usually hover around 99.8 degrees when her disease acts up have now been steady between 100.6 and 101 then sometimes spiking up to 104 and higher. She is coughing her "disease cough" which I describe as a croup type cough and vomiting at unusual times of the day besides her normal night time vomit sessions. Besides the pain, the worst has been the sores on her lips; they just look so uncomfortable, they bleed, they burn and they hurt. Her lips look terrible and then look completely normal the next day only to be covered in sores again in a few hours. We do not know why. I have been hearing the words 'I don't know' out of a lot of doctors' mouths the past couple weeks. It reminds me of Mr. Hand which at least makes me smile.

The good news? She is gaining a little weight finally and growing a bit taller.

But... (there is always a but) if the reason for the mouth sores is that they are a side effect of the methotrexate injections we have a problem. The weight gain is most likely a gift of the methotrexate injections because overall she vomits less since starting the injections. However if the injections are causing these mouth sores then the benefits may not outweigh the cost.

With the other symptoms we are seeing there is a chance that the mouth sores are a result of the disease instead of a side effect of the medicine. Here is the issue: Pyoderma Gangrenosum does not attack the lips. Remember there have only been 16 cases of PG in infants. However there was a case in a child at AI were that child had lesions on the inside of her body. I do not know if it is called PG at that point. My concern is that her PG is now attacking the cells inside her body and not only the cells on the outside of her body which is why we are now seeing sores on her lips. Dr R assures me that PG does not "change lanes of the highway". My response was "when does Lil's body ever follow the rules"?

Did you like how I sandwiched that good news in the middle?
What are we going to do about all of this? Experiment. We are going to give Lil a round of steroids and if her symptoms improve we can assume that she has some type of inflammation in her body. This makes me want to scream, rip out my hair and feel relieved all at once. I am glad we have medications at our disposal in this country that have the ability to make my baby feel better because as Dr R likes to point out, no matter who you are a dose of steroids is going to make you feel pretty darn good. However, it is frustrating to use this as a way to find out if there is inflammation because it is not very accurate and it feels like we are just throwing drugs at her without really knowing why. AGAIN. Steroids are not something we take using lightly.

My wonderful cousin is working on creating a button for you to order Lil's t-shirts right here on the blog! Thank you Ashlie!!! So keep your eyes peeled for that... Thank you all!!

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