Love, Life and LillyAnna: Have You Seen My Dragon?

Wednesday, November 5, 2014

Have You Seen My Dragon?

It has taken me a few days to process everything we saw and heard. One thing everyone has to understand is that we are not going to have answers from this trip and we may never have answers. Another thing is that I know it is hard for those of you who are close to us to get 'the story' from the blog but at least if you read the blog you have the base story and we can discuss the rest with you. As Chris likes to put it, "Even I read the blog!"

I am going to give you the details I feel are necessary for you to get the big picture. If you have questions I am happy to answer them! There is a comment section on the blog that I am not very good at utilizing but I would like to get better at using. We can also use Facebook. Those of you who are close family members, we will talk about it, of course!

The NIH is doing a clinical research study on a group of auto-inflammatory diseases. Pyoderma Gangrenosum is the process of an auto-inflammatory disease. It is not actually a stand alone disease. That is why it is so strange in infants. In adults it is the process of, or it comes along with, Chrons or Ulcerative Colitis or something like that but in infants it comes without an underlying disease. There is no prognosis for Lil's disease.

On top of this, Lil's case is extremely unusual as I understand it. I told you before, as the doctors were looking at her wounds they used words like extraordinary, amazing, awesome, unusual. When you are in a place like the NIH where they have seen and treated multiple cases of adult PG and you hear this... it is not good. Through the fundraiser my mother in law set up (thank you thank you thank you!) we have finally found another case of Infantile PG. I have not contacted the family yet but I plan to this week. They just returned from the NIH. However, Lil's disease does not look like that child's. In fact, her disease does not look like any other case they have seen. The angel doc said they have seen one case that looks a little bit like hers but she is their outlier.

They want to know why the Humira did not have as much of an impact on her disease as the methotrexate. Methotrexate is definitely effecting her disease more. We just increased her methotrexate again so they want to see what is going to happen. If her flares reduce they will see us in one year. If they do not, they will see us in six months. There is one caveat to that scenario.

LillyAnna's original blood work came back with double negative T cells, a very high number of them. What does this mean? Well, this is what has taken me so long to research. The first response would be to assume she has Alps disease which is a horrible disease. She DOES NOT have Alps disease. That leaves the question of why the high number of double negative T cells?

Extremely simplified: T cells are made in your bone marrow and are an integral part of the inflammatory response. Starts to make sense. Big Picture? I still do not know. I have made an appointment with Dr. R to discuss the trip and these results. At one point we had talked about a bone marrow transplant with Lil. But as Dr. R very bluntly put it "bone marrow transplants can kill kids" and we were not at a point that Lil was that bad. By the way, I love that Dr. R is that blunt. I need that. No bullshit. The angel doc is not even close to recommending that either. Before we left she ran more blood work using Chris as the control. Dr. R also has some data on this from Lil's previous hospital stays. Basically we are still looking into it. But it is the big white elephant in my brain.

Lil's liver levels are still elevated. This is common immediately after she gets her medication. However, we took this blood work three days after her methotrexate and Humira so the levels should have gone down. This is the second time this has happened this month. There is some evidence here that her liver is showing that it is unable to handle the amount of medication we are giving it. What happens next? I do not know. I could not ask. It is one of those 'we will cross that bridge when we come to it' issues. We will get blood work again in a couple of weeks. Unfortunately we just increased the methotrexate and that is the medication that is having the biggest impact. It is also the medication that is most likely causing the liver dysfunction.

She does not have Lupus! Good news!

Iron is consistently low enough that the angel doc wants us to give her iron supplements daily. Have you ever smelled liquid iron supplements? They taste and smell like pennies. I can't even get her to eat some days....

One thing that has been gnawing at me, Dr. R and Dr. Brescia is that her inflammatory markers do not go up when she has a flare. The angel doc told us this is normal for PG. It made me feel so much less crazy!!!!!

I met Lucy's mom in the laundry room at AI. We started talking and she began to lead me along this yellow brick road of having a chronically ill child and raising a family at the same time. While in the play room at the Children's Inn at the NIH I met a lovely woman who was at the same place I was when I met Lucy's mom. I was able to begin her walk down that yellow brick road and she thanked me. She seemed so much more calm when I said good bye. Her boys where so beautiful. I hope I did Lucy proud. Lucy's life had so much meaning for so many people but this is Lucy's legacy for me, to be able to help families who are just beginning this journey the way that Lucy's mom helped me.
We are doing it Lucy:)

1 comment:

UllisNovember 15, 2014 at 1:17 PM
I'm praying for your sweet angel. She is so brave and strong. I have pg myself so I know she's strong ��
God bless you ��
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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

Wednesday, November 5, 2014

Have You Seen My Dragon?

1 comment:

Down the Shore

LillyAnna in her Favorite Place