Love, Life and LillyAnna: The Phantom Tollbooth

Saturday, January 4, 2014

The Phantom Tollbooth

I have to apologize ahead of time. This is a bit of a long post. Our computer is taking a slow walk down the Green Mile and it has taken me hours to write this post.

As Lil got ready for her MRI/MRE the amazing Child Life team gave her a Build a Bear bunny and allowed her to attach heart monitors, IVs and such to it in preparation. Not surprisingly LillyAnna was able to reinact the entire thing afterwards even though she was asleep during the actual IV placement. She has been awake during so many of them however that I think, or I hope, that this was some way of her processing what she has been through in the past.

The results are in and my second biggest fear has been realized. My first biggest fear of course was that she would have some kind of inoperable malignant tumor or something equally as horrifying but as it turns out the tests showed absolutely nothing... my second biggest fear. Does that make me a horrible mother or does it give you insight into how maddening it is to live with Lil and her symptoms day in and day out?

There is some swelling in the bursas in her knees. The MRI itself did not even show arthritis. Arthritis is one of those things that can flair up and calm down without notice. We were warned ahead of time that there was a possibility we would not see arthritis in the MRI. As sometimes happens when you spend a lot of time at AI we had an impromptu conversation with Lil's rheumatologist in the gift shop the other day. She always has interesting insights and she also has a daughter about LillyAnna's age. Dr. B says she is sticking with her original diagnosis that Lil does in fact have arthritis. She said everything she has seen and everything I have told her says arthritis. She also asked if the Methotrexate injection was helping LillyAnna's symptoms. I thought it was helping at first but now I am not too sure. The doctor did not seem surprised to hear this news. There is just no rhyme or reason to her swelling, pain, vomiting, eating or lack thereof. Dr. B would like to have a sit down with us and Dr R. I think that is a great idea.
As far as I know there is nowhere to go from here. We are still waiting to hear from the NIH but as I have said before they told us quite plainly that we should not expect much from them, they will be learning from us.
Little Chris has some competition when it comes to his toys these days but let's be honest so does Nettie. Lil is one of those girls who like tiaras and superheroes, swords and ribboned wands, baby dolls and action figures and boy does that girl love her nail polish. Having to remove her blue sparkly nail polish was probably the most traumatic part of MRI day. And so, when we ended our day by attending the Chronically Cool Families holiday party Lil had her own little nail polishing station at which the therapists and some of her friends let her polish their nails! They are crazy if you ask me...

Aunt Coco and Cousin Bobby had to have theirs done too!

It was at this little shindig that I took a moment to look around and reflect. It so happened that the leader of the group was standing next to me and noticed my quiet. I just could not believe we were spending another Christmas at AI. This has been our lives for over a year and it is just so unbelievable. I know we are not in patient and I know Lil does not have big gaping wounds but it just blew me away that we were still in this place and J said it best: You are not any closer or any further from where you were one year ago. That is just it. Lil's symptoms are different but we are still in basically the same place. Maybe that is just what I have to learn to live with, the fact that I am not going to have any answers and that this is going to be our lives until something happens and it is not anymore. It could be so much worse.

Overall Lil had a difficult Christmas. Chris and I had more than one conversation surrounding the idea of taking her to the Emergency Department. She had fevers spiking up to 103.6 but most of them were going up to 101.5 and then back down again. Between fevers like that and her normal vomiting, she dehydrates quickly. She has also been quite fussy. Why? Who knows? She says her legs hurt, her head hurts, her nose hurts.... toddlers are not very good at describing their pain. I have been trying to do some reading on this but it is difficult to find quality research on chronic pain in toddlers. Someone told me that American children are the most under medicated for pain in the world. I have no idea what their source is and it sounds a little unbelievable but we did joke that it is easier to get people to believe that my dog is in pain than that my baby is in pain!

The cold weather does not help and Lil really did not have much interest with her current mood to put on snow clothes so we brought some snow in to her.

I do think that when we weigh her next we will find that she has lost more weight. When I rub her back now I can feel her little spinal cord sticking out and I can see her ribs even when her arms are at her sides. I am worried again about getting an NG tube. This will honestly be hell for us. I will not go into details because I am refusing to worry about it right now. The part that drives me absolutely batty is when I took her for blood work it looked AWESOME. Seriously. It looked great. I kept looking at it over and over again with my mouth hanging open because I just simply could not believe it. Dr. B (the rheumatologist) did tell Dr. R and I not to hang our hats on the blood work. She said they do not even really use it to monitor disease progress. Dr. R looked like a little boy from whom you just took his favorite toy... he said "but how else will I monitor her?" Poor Dr. R. The best email I have gotten from him said simply... I agree. She IS a mess. Just that little bit of reassurance made me feel so much better. How crazy is it that hearing your doctor call your kid a mess makes you feel better? Ahh life... you just have to laugh.

This weekend Lil has developed some mouth sores. This is a side effect of the Methotrexate and not a total surprise. They are coming and going so I am not totally sure what that means but I am waiting to hear from the good doc on what he would like to do. It may be as simple as supplementing her with folic acid. Of course, supplementing Lil with anything is not simple because not much stays down.

The twins met the New Year fully awake for the first time in their lives eight days before their 5th birthday. In true Lil form, she stayed out dancing in the street the longest (Mommom finally had to call it and drag her in... well actually she was being carried). Nettie quickly fell asleep on the couch. I am pretty sure we have a future Mummer on our hands though. Lil was too sick to go to "the big show" last year but this year she was able to watch Poppop live and she loved it even with a fever of 103! And yes, the twins are celebrating their fifth birthday this Wednesday. Another unbelievable fact of life.

One of our dear friends is dying. She is five years old. Lil has taken such a liking to this little girl and I have fallen in love with her family. Her mother has helped me through this world of having a chronically ill child even though my child's illness does not come close to the diagnosis of hers (and if any of you are reading this... I know we are not supposed to compare but I get a free pass on this one!) This is a true Dragon Mother who has watched her baby's life drain from her beautiful little body over the last five years. Five years of pain and suffering and five years of seeing her three siblings through it with love, understanding, kindness and support. Five years of doctors and medicines and surgeries and blogs and wheelchairs and nurses and hospital stays more numerous than most of us can imagine. Five years of a strength that we can all only hope to have if faced with the same. I have always felt that the women of this world have a connection through time and space. The only way I know how to help this Dragon Mother is to ask all of you to garner your strength and send it her way. I do not know how long it will take but she will most likely
be watching her baby die in the coming days and she needs us.
As I was rocking Lil before bed last night, we were looking at pictures of her and Lil remembered where each came from. "We played in the water that day!" "We saw Santa that day!" "I sat in her bed!" "___ smelled the gum!" Lil named her new baby after this little girl. This family is living their journey and whether this is the end or close to the end or not, this is all part of their story just like the life we are living is part of our story. So as we go through that Phantom Tollbooth, I am looking at being more present, accepting our reality and using this Dragon Mother as a model for how to navigate it all.

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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

Saturday, January 4, 2014

The Phantom Tollbooth

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Down the Shore

LillyAnna in her Favorite Place