Love, Life and LillyAnna: The Wonderful Wizard Of Oz

Friday, October 10, 2014

The Wonderful Wizard Of Oz

2011

It has taken two years but it has finally happened. We have an appointment with the National Institute of Health. If you know anything about the NIH you know what a feat this is... for us it was even more difficult. The NIH works solely on research money. There is no research happening on Idiopathic Infantile Pyoderma Gangrenosum which is the complete title for LillyAnna's disease. Although rare, Pydoerma Gangrenosum does happen in adults that have some underlying auto-inflammatory diseases such as Crohn's disease, Ulcerative Colitis and others.

2011

However, what makes Lil so special is that she is only the 17th
reported case in infants (Infantile). These infants have no underlying disease and we have no idea why they develop the PG (Idiopathic). Bottom line? There is no one is researching this disease. It is just simply not going to help enough people. Most of the kids who we have found reports on are from years ago or the research has many holes in it because it has been translated. The most recent one was 13 years ago and was in England. The other problem is that Lil's disease is presenting differently than most of the other cases. The other cases presented on just the legs or just the arms or just the buttocks. Lil's presents all over her body... ALL over. Also, Lil's is not responding to treatment the way the others did.

The other patients responded to oral steroids alone or possibly needed Remicade. LillyAnna never responded to oral steroids alone and developed antibodies to Remicade within months. In fact, we
feel that under her current treatment (Humira, Methotrexate, meloxicam, lansiprozal, folic acid, and a couple others as needed) she is only barely managed.

Basically this is why it has taken us longer than usual for us to get an appointment. Actually, it is amazing that we have gotten an appointment this quickly. It is thanks to the presentation at AI that I was able to give (thanks to friends and researchers at AI), the connection in Washington, DC that came out of it and of course our hard working and dedicated doctors (one of whom refuses to read blogs but I have to throw the love out there to anyway... Dr. R).

So, as these things go, they have chosen the absolute worst week possible. We are scheduled to go to Bethesda, Maryland from October 26th to the 31st. The 30th is me and Chris' tenth wedding anniversary (we have not been able to celebrate an anniversary properly in quite awhile and were looking forward to at least a very nice dinner at our favorite restaurant). A congressional delegation is visiting AI and I was asked by name to speak based on my before mentioned presentation. I was scheduled for two good paying jobs that week. And I am sure it did not escape your attention that it is the week of Halloween. We cannot bring the twins with us. You do not pay for room and board or co-pays at the NIH but you do pay for food. I am told there are delicious restaurants as we are outside of DC. But if you read between the lines you know that of course money is an issue as usual as it is for most of us who deal with having a chronically ill child. We have worked out child care for the twins but it is just so hard.

It is hard for them to understand LillyAnna's disease and why she has to go see special doctors, how her disease is different from the common cold, why she has pain and how they can take care of her. They worry. Next to Christmas, Halloween is their favorite holiday. We will be back for trick or treating but will miss the week before which is part of the fun.

Don't get me wrong. Although I tried to get the NIH to change the appointment, I have taken the appointment and accepted what we have to handle as a family now. It is all good. This is just all too important. This is stuff that can affect Lil for the rest of her life. I am excited to go and starting to do all of the work to get ready. It takes a lot of research to prepare for this kind of trip when you are dealing with a specialized case like LillyAnna's. I have already met with one of her doctors and am preparing to meet with others. I have to research each department we are going to see and find out their experience with pediatrics. I have to gather the special materials LillyAnna needs for procedures that I am assuming the NIH will not have (AI does not usually use them-we do special things just for Lil). And then the other stuff you do when you are going away with a family of five and leaving two of your kids home. My family has totally risen to the occasion of course.

Dr. B said she has never heard of a patient being seen at the NIH for five days. Are we the exception? I would not be surprised!

I have to admit that I thought our circle of support would start to fade this year. I thought that as we moved on with life and we all began this change of living a more normal life we would also have to live with less of a circle of support. Not because people do not care.... please don't misunderstand. Just because I assumed that would be the natural course of things. I have steeled myself to strengthen my resolve and prepare to be even more powerful. It has not happened though. Those of you who have held us up, who have kept us going have not gone away. You have still offered your support. People have continued to buy t-shirts, send us money (unbelievable!), offered to babysit, send us cards, prayed for us, our nurses keep in touch, you follow the blog, donate clothes, you give me the room to be the person I have become and understand I am not the person I used to be. You understand I am still changing.

Many of you have been asking about my job. I loved Redfire. Bartending served its purpose and Platinum Dining is a wonderful company. The people I met there became my friends and I am still helping out when they need it. It is fun! Let me be honest. However, working nights is not what is best for my family and bartending is not what I was meant to do with my life. I love working in education. Up to now, my job at UD has been great but not very fruitful. The grant I have been hired to work under had not come through. However, that just happened (this past week actually)! So now I can start getting paid. The past couple of months has not been easy financially. I have had some work coming in from other places which has been wonderful but not quite what we expected! The twins started kindergarten at First State Montessori Academy (a new charter school on which I sat the founding board) and have been so happy and engaged. Chris and I have been doing great. We have been in love and happy. He loves that I am home at night again and it has brought a fun spark to our marriage, after ten years I would say that is pretty wonderful!

Which brings us to Lil. She has had a pretty great two months and with me starting what feels like real work again after two years it almost felt like we were leading a normal life. Until about two weeks ago when it all started again... the not eating, vomiting, fevers and general pain. My fears and anxiety all came back with such a crushing blow. And my sadness, my sadness for my little girl. She is growing up and she is so smart. With that comes her ability to talk and her vocabulary is simply phenomenal and so is her intelligence. She figures out when it is an injection night by the kinds of medicines she is getting and does not agree that the shots make her feel better. How can she when they make her feel so awful for the next two days? She can tell us now so plainly when she has pain in her legs, her back, her belly but also knows that if she complains too much she is going to have to go to 'her hospital' so sometimes tries to hide it. Those times she just sits and moans. It is awful. We saw Dr. B, her Rheumatologist and have a plan. Time will tell. Iron is low- not surprising with her not eating. Liver numbers are high again so we will have to go back for blood work right before the NIH to check those. This is especially concerning to me because it is not the first time we have seen these numbers and we are pumping her full of so many medicines; one of which she is not FDA approved for and is a higher dose than she really should be getting.

The docs also did a blood test called ANA. This is a test for a condition that causes inflammation of the eyes in patients that suffer from auto-inflammatory diseases. She came out borderline so we will be seeing ophthalmology every three months to keeps tabs on it. This kind of inflammation can cause permanent eye damage if not detected and there are no other side effects.

I am hoping she starts feeling better soon, she has not eaten in days. I would feel better if she perked up a bit. Overall though, it could be worse and this appointment has finally come through. Who knows, maybe the wizard will have a magic solution and we will not have to commit murder and get the broom to win it.

1 comment:

AnonymousOctober 10, 2014 at 12:26 PM
I am so sad that Lilyanna is not feeling good.Hoping for good results from NIH.Keeping you in my prayers. Maryellenstrohm
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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

Friday, October 10, 2014

The Wonderful Wizard Of Oz

1 comment:

Down the Shore

LillyAnna in her Favorite Place