Love, Life and LillyAnna: November 2015

Monday, November 23, 2015

The New Small Person

LillyAnna has been growing in leaps and bounds. She looks more and more like a little girl and less like a baby every day. However, with this recent illness that she cannot seem to kick she is losing weight. I just do not understand why she stops eating like this when she does not feel good. Lil has congestion in her nose that I do not think has gone into her chest but it is making her cough so hard she is throwing up. Then again she throws up so often it is hard to tell exactly why she is throwing up. Whenever she runs she coughs but that has been happening for years as well. I will be contacting the infamous Dr. R to see what he would like to do. She is not running any fevers which is why I have not done anything so far but I want to figure this out before I go into the hospital.

Lil's first cannoli.

We went to our support group meeting the other night and there was a mom there who was, what I call, 'deep down in it'. Listening to her share about the ups and downs she is experiencing and how hard it is was hard for me. She shared that every time she thinks everything is ok something else happens. I remember writing about that very thing here. We are now in a place where everything seems to be going well. It made me feel like I should be waiting for the other shoe to drop. Every time I relax something happens. I do not want to do that, just wait for something bad to happen but I also do not want to be blindsided. This illness is a perfect example. It is not normal to have a cold for 2 months, one that is bad enough you cough until you throw up and lose weight. Her little clothes are starting to fall off of her.

Redness on her cheeks is the beginning of the ulcerations on her face we are trying to prevent after last year's hospitalization.

I have to make sure she is ok before I go into the hospital for my headaches. I want to thank you for all of your support. So many of you have told me I need to take care of myself but I hate this, I hate that I have to do this and I hate that I have to leave the kids and my husband. I know I have to do it and my wonderful husband can handle things at the house so I am letting go of it and accepting it. But I hate it.

Halloween!

Sunday, November 8, 2015

Young and Old

I just spent a hour reading through old blog posts looking for the title of the one where I talk about Lil's dressing changes. I could not find it. What I did find was a lot of tears and a history of not only Lil's disease but also my topic for today. My own headaches.

I wish I could tell all of you that Lil is feeling better. Is she better than she was three years ago in the hospital? The answer is yes. However, she has been pretty much the same for the past two years. As a family we just now know what that will look like and how to handle it.

This past month she has been vomiting every night. I am worried she is losing weight. Our reality is that she has enough weight on her right now that I am not even bothering to tell Dr. R because there is nothing to do about it whereas last year I would have been emailing him a couple of times and probably bringing her in for a check up. Now I know that, although watching her vomit every night is horrible, we would not do anything about it until she loses enough weight or dehydrates herself.

She is not eating much except sour cream and onion chips. I don't even want to know what her blood work looks like. And I am waiting to hear back from audiology about an appointment for her failed hearing test. She has a terrible cough right now and is covered in pustules. Most mornings, with this weather, she has been in pain with swollen fingers and bad hips. I feel so bad for her.

I truly thank God for our amazing nanny, Julie everyday.

Here is our new issue. As most of you know, I have been suffering from chronic migraines for about 15 years. For most of that time I would have about 1-2 a week which now I know is considered normal.

When we received Lil's diagnosis and were in the hospital for over two months the first time, I was under a lot of stress. During that time, we were so worried. Lil was sick, we were doing dressing changes every other day. They took two hours and they were excruciatingly painful for my 9 month old baby. She had over 30 lesions and the bandages were sticky. We had to peel them off and re-stick them. It took me half an hour to set up and the aftermath... well you can imagine. She screamed the whole time and sometimes just passed out from the pain in the middle. My job was to hold her down and try to soothe her during this ordeal. Right. On top of that, we were so scared. We had no idea how to treat this disease and it just kept spreading. During all of this, Chris and I were raising the twins, coordinating their lives and I was deciding to quit my job.

On the outside, everyone was telling me how strong I was but apparently there was a lot going on inside. My brain was not happy. I have been waiting 6 months for an appointment at Jefferson Headache Center being told they are the best. The good thing is that I now have a diagnosis: transfer migraines. The doctor compared it to getting a concussion. He said that when you are a migraine sufferer who gets migraines once or twice a week and then something happens that puts your brain under severe stress (like some kind of trauma or stress- for me, Lil's illness) you put yourself into almost a concussion state. What do you do when you have a concussion? Rest. I have never rested my brain. His recommendation is to go into the hospital for a week. There I will rest but also get IV medication to fix my brain. Sounds crazy. I was flabbergasted. Here is the thing, I do not have a choice. I have to do it. I have a migraine 24 hours a day 7 days a week and it has been like this for about three years. After his exam the doctor said I am one of the worst cases he has ever seen. It looks like I will be going in the first or second week of December. I am scared to death. My kids are going to freak out. Especially little Chris. I have not yet told them. This has to be done. My Aunt Tina and I keep talking about it because she was kind enough and smart enough to realize that I needed someone to go with me to this appointment. She tells me I am so lucky to have so many people in my life that can help. I am just so tired of asking for it.

Young and Old
~Charles Kingsley
When all the world is young lad,
And all the trees are green;
And every goose a swan, lad,
And every lass a queen;
Then hey for boot and horse, lad,
And round the world away;
Young blood must have its course, lad,
And every dog his day.

When all the world is old, lad,
And all the trees are brown;
When all the sport is stale, lad,
And all the wheels run down;
Creep home, and take your place there,
The spent and maimed among:
God grant you find one face there,
You loved when all was young.

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

Monday, November 23, 2015

The New Small Person

Sunday, November 8, 2015

Young and Old

Down the Shore

LillyAnna in her Favorite Place