Love, Life and LillyAnna: Don't Let the Pigeon Drive The Bus

Wednesday, June 22, 2016

Don't Let the Pigeon Drive The Bus

I feel like a pigeon is driving our bus today. I feel like no one knows what is going on and there is just simply no one to ask. Lil had her little flare last week, if you can call it little, as most of you already know. Running fevers between 102.5 and 104.5 for a week is not what some people would call little. Most of the time they were up in the 104 range. The big question is: did she have any lesions? The answer these days is that I don't know because now this horrid disease has decided to start attacking tissue on the inside of her body instead of the outside. For many reasons Dr. R and I believe this recent spell was disease activity and not an illness. She was showing so many signs of her disease activity like vomiting before bed, small pustules popping up, fevers with no other illness symptoms, no one else in the house was sick or got sick, the list goes on. Also she has started making that grunting noise in her throat that tells us the lesion in her airway may be back. I cannot for the life of me get her to take a steroid. We tried everything under the sun. She can even swallow pills and she will not do it. We had a bag packed for the hospital and Dr. R had a bed waiting for us when the fever finally broke. If it were not for that amazing man we would have been inpatient a thousand times over, any other kid would have been inpatient with those temps, but we try to keep her out of the hospital at all costs and we were able to once again. It was close this time though...very close. And boy does it take a toll on me. Playing nursemaid to a four year old is no easy feat.

I always tell Dr. R that when I have his attention, that is when I get worried. I like when it takes him a couple of hours to return my emails. That means we are not a top priority. Lately he has been answering me within minutes. I don't like that. It means we are a top priority. It means he is worried about her and when he worries I worry because if he is worried that means there is something to be worried about. Today's response was that he was consulting with rheumatology and they think we should increase her Humira again. We just increased the Methotrexate and we increased the Humira in the fall.

It just feels like we are going in the wrong direction. Aren't we supposed to be in a place at some point where we can take her off these meds? The other 17 babies who had PG were on one biologic (if that), short term, went off it and that was it. Why is she different? What does this mean for her future? She is only 4. And I always go back to what the risks are of suppressing her immune system long term.

I would love to say that we have to keep increasing because she is growing but I know how much weight she has lost recently. I just wish there were some other cases we could compare her to, someone we could consult... someone who was living this same life. But of course I am glad no other child is going through this at the same time.

This long term worry thing is tough. There is just no end to it. It never goes away. It is the same worry day in and day out and it is such a big worry. The worry being will she get cancer? Will she get start to get lesions on her skin that eat away her flesh? Are there lesions eating away her flesh right now that I cannot see? Are we making the right decisions? How much pain is she in right now? What should I do about it? Should I do anything about it? It is every day, all day long. And there is just no end in sight. And she is just so little.

I don't want to complain. Life could be so much worse. I have my baby, she is home and not living in the hospital. She runs, she plays (as much as she can), and she does so much more. As I always tell people who say to me, "I don't know how you do it." we each have our own reality and they all are hard in their own way but they are also beautiful in their own way. Right now I am working on holding onto the beauty.

4 comments:

AnonymousJune 22, 2016 at 7:36 PM
I hear you. I feel your pain ,I feel your worry. I have 2 girls ,both have bad anxiety and my oldest also suffers from depression. So I understand your worry,stress and sadness for your child and family. Just remember your doing all your capable of,your loving your babies, your nurturing them. That's what they need the most. You have to take care of yourself. That will help you to stay strong. I will keep sending positive vibes for LillyAnna and your family.
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LynsiJuly 1, 2016 at 1:46 PM
Hi Gina. I'm not sure if you remember me or not.. we met at the children's inn at the NIH two years ago. We chatted about your little girl and my son who is in a study there for neuromuscular diseases and really hit it off. I took your card and poof, it was gone. We're moving this month and somehow in the packing I discovered your card. I was a bit afraid to follow the link, fearing the worse for Lilly.. yet hoping for some miracle! And after reading a few posts, I see that not too much has changed. Anyhow, I just wanted to check in and tell you that I hadn't forgotten about you.. I hope you guys are holding on! (Are you still going to the NIH? We go every October.. I'd love to see you again!)
Lynsi
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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

Wednesday, June 22, 2016

Don't Let the Pigeon Drive The Bus

4 comments:

Down the Shore

LillyAnna in her Favorite Place