Love, Life and LillyAnna: February 2017

Saturday, February 11, 2017

The Wonderful Things You Will Be

I am the type of person who likes the TV on for background noise. However, I will only watch movies and shows that are happy or comedic... I don't need more sadness or drama in my life! As I was getting ready for work the other day I had on an episode of I Love Lucy. In this episode Little Ricky had to have some kind of surgery. In those days the family was not allowed to stay overnight (or even after the surgery!) with the patient. In classic Lucy fashion, mom finds a way to sneak into the hospital, create havoc, and when Ricky comes to find her late that evening she is curled up asleep next to Little Ricky in his hospital bed.

I share this with you for a couple of reasons. One is that it reminds me of what a mother will do to be with her baby when she is needed and wanted. The second is to remind us of how far hospitals have come.
When we look at the whole picture of Lil over the last year, there has been a steady increase in her pain, a decrease in what she has been able to do, and a lot of frustration for those of us who care for her (and I would say a lot of frustration for her!). We have tried multiple courses of steroids, pain meds and all the other things you do to take care of your sick babies.
Our solution (Dr. R and I) was to pull her main docs together and some family members, we just could not fit them all, and discuss a new path forward. At the last minute Dr. R and I had the forethought to invite Palliative Care... this was genius. When most people think of Palliative Care they think of end of life care. Now it means so much more. As they self described, they are a team who can help with quality of life, communication between the family and the doctors (which is not really an issue for us), and they can help coordinate everything that is going to make that quality of life the best it can be.

They helped us to understand that while Lil has real pain from her arthritis and headaches, nausea, itching and other things she cannot yet tell us about, she also has a kind of pain called AMPS. AMPS is Amplified Pain Syndrome. After looking at pictures of Lil's wounds and her scars the doctor determined that she has a lot of nerve damage. He said that the nerve damage sends a signal to the brain that there is pain even if there is no longer pain. I asked if that means the pain is not real... he said that no, the pain is absolutely real. The brain is still perceiving pain. However, the problem with AMPS is that pain medicine does virtually nothing to help. This explains so much! Lately we have been having to give her oxycodone and Tylenol just to get her close to comfortable enough to go to school. She missed two months in a row this winter.
Thanks to the hard work of Dr. B, Dr. R and Palliative Care, we now have a new plan that is very exciting. With that new plan comes some new levels of acceptance. I went into the meeting hoping for a new medication regimen. It took a loving push from my dad (thanks Dad) and about 24 hours for me to realize that this is as good as it is going to get for her and that is what the doctors were trying to tell me. As moms, we always want more for our kids. However, the acceptance comes in that this is as good as it is going to get for Lil. She has had breakthrough lesions. Even now as I write this I just walked away from examining her mouth and throat and found a big lesion on the roof of her mouth and the back of her throat. It is good sign when she asks for a lot of things to eat and eats none of them that something is going on in there....
It is not a good idea to change her current course of meds for many reasons. The most prominent being that we are using the safest ones and when we look at the fact that we are going to be attempting to control this 'orphan disease' for the rest of her life we have to be conservative with how many biologiques we go through at such a young age.
That brings us to Palliative Care. Our plan is to get her into psych, PT (specific to AMPS and other PT), try some new pain meds, get us involved with a social worker there and Child Life (on a new level), we are going to get a handicap placard (Dr. R's idea), get Lil an IEP or 504, and get Nettie into therapy. Deep breath. All of this would not be possible without Julie. As a family they want us to change the focus from anxiety (which I have been trying to do but by myself I guess). They also talked about how in pain management they are getting away from those pain scales and focusing more on the function of the patient. I totally get that; people always ask me how I can possibly have a migraine but still be standing there talking to them. It is because I live with migraine 24 hours a day, 7 days a week. Palliative Care said the same for Lil. She will probably never be at a zero, they said her best will probably be a 4 but to look at her function, what she is able to do. I love this way of looking at things.

They would like us to push her to do more, go to school 5 days a week, play more without meds, etc. The mommy in me who has lived with this disease is scared and excited. I have seen what happens when she pushes herself and she pushes too far (so much pain, vomiting, wiped out for days....) but they said if we don't let her do this she will not learn how to gauge for herself what her limits are. The mom in me says, "She is only 5!" The mom in me also says, "She can do this!"
There are a few things we are still "talking about" (AKA disagreeing on) but this is why I love my docs at AI. I can talk to them, it is a discussion. I want a medical stroller. She can play more if I can get her to the playground. It is the same idea as the Handicap Placard. It is a support. I also want to try medical cannibus. We are still 'discussing' this idea but they did not shut me down right away on it. Dr. R had a good point on the stroller. He said, "I just don't want to make it medical." I get that but I also have to live life with her and she does not fit in a traditional stroller anymore. I can't carry her around, that is worse! She is almost 6.
So Lucy, you were ahead of your time. Good care for kids is a team effort. I am excited and scared about our path forward. Our girl looks healthy so much of the time but underneath all of that is a very sick little girl and behind her is a family who has been struggling to support her. Hopefully we will now have to struggle a little less. Our Palliative Care doctor shared a group with me called Invisible Diseases. It is so hard when she looks normal but is just not. The dynamics are very interesting...

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

Saturday, February 11, 2017

The Wonderful Things You Will Be

Down the Shore

LillyAnna in her Favorite Place