Love, Life and LillyAnna: Waiting for Normal

Sunday, June 28, 2015

Waiting for Normal

Dr. R told us we just had to wait and boy he was not kidding. Lil's pain is just hanging on and not letting go. What frustrates me the most is watching her act so normal for a majority of the day, or listening to her sitter tell me she had a great day, and then watching her fall completely apart when her and I are alone. Just last week was a great example of that. She told me she had pain in the morning (pain in her mouth and in her side and belly), she had not been eating so I know it must have been significant. All day she hung in there with the twins playing and running around but when it came to bedtime she just lost it. I finally had to force oxycodone down her throat and it was not until then that she gave in and fell asleep.

The medicine issue is big right now. Her iron is low but she refuses to take any supplement. We really need to get more blood work to see what direction this is all going in but I am so reluctant to do it. I see glimmers of her getting better and doubt myself. Also, I literally have not seen her in about two weeks. How can I judge where she is when I am not with her? I spoke to a very knowledgeable friend about this very thing. Why is she "ok" when she is with other people or out and about but then she sees me or we are home and all hell breaks loose? Why do I see such a different child than everyone else does?

This friend just became a Nurse Practitioner (Congratulations Amanda!) and knows her stuff. We talked about quality of life. I could see her point. If Lil feels good enough to be able to play and run all day she has a good quality of life even if she is losing it when she gets home. I do reserve my mommy 'know how' though (and I know Amanda respects that) to say that she does not see how bad it can be when Lil loses it at home. If a three year old feels they have to hold in their pain all day to that extent is that any quality of life? To see her cry and thrash and scream, to see her skin and body react to the pain by breaking out in pustules and fever, to know I have to give her an opiate in order to calm her down.... Would any adult live like that or would they go to their doctor and say fix this! Something is not right! I am her advocate. This is my job. And to make it worse, I have been working so much the past two weeks I have no read on her. None. I love the work. We NEED the money. Having no pulse on her feels like being trapped in the middle of a pitch black room, arms stretched out feeling for the walls, a chair, anything. No idea what is happening or where anything is; ears straining for sound, skin beginning to break out in sweat, fear sweeping my body in waves.

Thank God I have a wonderful husband who is an amazing father. We have our rough times, living this life is just so hard. Everyone tells us to take time for 'us'. There is just simply not time for it and we do not have the money. The house needs improvements, the laundry keeps piling up around us. You all know how it is. We have this beautiful, hurting little baby in the midst of it all. I was talking to my uncle who is now the grandfather of a beautiful baby girl with Down Syndrome. We were talking about this very thing and he just looked at me as Lil was putting her tired head on my shoulder and said, "How can she not come first?" He gets it.

Chris and I find moments for ourselves. I will say I am the reining queen of falling off the high dive at the pool. We eat dinner alone almost every night (try not to judge). And Chris makes one hell of a banana split which we enjoy together each night (again, try not to judge :). But on top of all the rest of life's normal curves, we have to protect Lil from unforeseen dangers; from the unknown. Everyday we have to guess what is going to be a threat. Is the threat the pain in her side that is making her grunt and double over or is it the mosquitoes because too many of those can put us in the hospital. Is it the guy next to us coughing or is it her mouth and that she has not eaten lunch or breakfast that day? The list just goes on and on.

There is my girl though, running around having fun! Taking all kinds of risks she should not be taking: getting too close to the grill, running in her flip flops, running with a smore stick, etc, etc.

She told me getting in the pool makes her owies feel better. Our pool and local swim team allowed her to join the swim team. She tells everyone, "I am a GATOR!" The other day she woke up and had pain in her legs. I asked if she wanted medicine. She told me she did not need it because she was going to the pool and that would make her feel better. Since she cannot go to school, being a part of a community like a swim team is even more important for her.

So how is Lil? As I write this I can hear her grunting in pain from the living room. She told me she cannot eat a hard boiled egg because her mouth hurts but she is also smiling and talking to me like she is the happiest child to ever walk the face of this earth. This is the dichotomy with which we live... waiting for normal.

Lil with one of her best friends and swim team mates, Spencer!

No comments:

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, June 28, 2015

Waiting for Normal

No comments:

Post a Comment

Down the Shore

LillyAnna in her Favorite Place