Love, Life and LillyAnna: February 2014

Saturday, February 8, 2014

Good Night

Do not go gentle into that good night,
Old age should burn and rave at close of day;
Rage, rage against the dying of the light.

Though wise men at their end know dark is right,
Because their words had forked no lightning they
Do not go gentle into that good night.

Good men, the last wave by, crying how bright
Their frail deeds might have danced in a green bay,
Rage, rage against the dying of the light.

Wild men who caught and sang the sun in flight,
And learn, too late, they grieved it on its way,
Do not go gentle into that good night.

Grave men, near death, who see with blinding sight
Blind eyes could blaze like meteors and be gay,
Rage, rage against the dying of the light.

And you, my father, there on the sad height,
Curse, bless, me now with your fierce tears, I pray.
Do not go gentle into that good night.
Rage, rage against the dying of the light.

Good Night Sweet Lucy. You raged and you fought until your last breath...you and your beautiful family. Sleep tight. You joined the angels in heaven tonight in your Dragon Mother's arms.

Sunday, February 2, 2014

A La Orilla Del Viento/at the Edge of the Wind

In this post I am going to ask you to keep your head about you. I am going to ask you to do it two times. This is the first.

I have been thinking about something. People say as long as you are trying your hardest or as long as you love your children you are a good mother. That is bullshit. There are times I am just not a good mother. Example: this morning I was trying to get all of the kids in the car, a bag packed and we were late. The baby was crying, Nettie was screaming "Mommy, Mommy, Mommy..." at the top of her lungs (think Stewie but much louder and very high pitched) and Chris was refusing to put on his coat. I leaned in the car and screamed shut up as loud and uncontrolled as could be at my kids. Does this make me the epitome of a bad mom? No. Have I scarred my children for life? No. Was I a good Mom? NO! Is that ok? No but it happened and I will do my best not to let it happen again. I could sit here and list stories like this and I could list hundreds of stories I believe describe me as the kind of mother I dream of being. I won't do it now because I am secretly afraid you will not agree and then where will I be! This goes for every relationship in our lives; sometimes we are not good friends, daughters, nieces, employees and even wives and that is the bottom line. But sometimes we hit the nail right on the head and we are amazing. Sometimes we are just somewhere in between. Does this make it ok when we are no good? I guess it depends on how bad it gets. Obviously there are things that are inexcusable. Maybe it depends on if we allow the same mistakes to happen over and over. I am sure we have made some mistakes with LillyAnna but one thing the past two weeks has made very clear to me is that taking her off her current course of medications is not an option.

One discussion I have with family, friends and doctors at least once a week is if we should take her off all medications and see what happens. The question is are the medications causing more problems than they are solving. So far, we have agreed with Dr. R and our team that this course of action is just far too dangerous. It took too long and too much medication for us to get Lil's disease under control. Especially after last Monday, we are even more confident that this is the right course. It is not perfect but it is all we have right now.

Tuesday I spent an hour meeting with Dr. R. Lil's antics the past couple of weeks have caught the attention of all her friends:) The meeting was extremely validating for me and frightening as well. We first talked about the MRI. Nothing new there. As I mentioned Lil's brain scan was normal, the lymph nodes in her neck are not cancerous, her abdomen showed no inflammation, and there was bursitis in her knees. Although the MRI did not show arthritis Dr. R and Dr. B believe she does have it. This is not surprising as it is just so obvious although the methotrexate injection seems to be helping it a little bit especially in her feet, not so much in her hands. Dr. R has sent Lil's paperwork to the NIH personally so hopefully this time there will be no mistakes and he is going to check on it this week. We are going to start her on folic acid for her mouth sores. If the mouth sores are due to the methotrexate folic acid should help. If not... we will decide what to do next but they are bothering her more and more and I am finding more of them. They come towards the end of the methotrexate week. The lymph nodes have not changed as far as we can tell.

I was very surprised to see that Lil has lost weight because she has been eating more and throwing up less. She has once again grown taller but has lost weight and not a tiny bit either. Seeing that line on the graph go down felt like a punch in the gut. And the cherry on top is that I had thought the injections were making a difference in her eating and vomiting but once again about six weeks out her old trends are coming back. Her vomiting has slowly started to pick up and her eating is slowly starting to decline. Where two weeks ago she was running to the table yelling for dinner (which we have never seen her do) now she is eating only one meal a day again. Dr. R mentioned that even though the MRI did not show anything, he is considering another scope. As he put it, with frustration in his voice, he knows there is something going on in her GI tract he just cannot prove it.

This is going to be the second time I ask you to keep your head about you. Dr. R mentioned in this meeting that one of the doctors on his team suggested doing a bone marrow transplant on LillyAnna. Let me be perfectly clear: we are not going through with this plan. However, the idea that a doctor I respect would suggest it, they would discuss it and my doctor could clearly outline why he believes it is not a valid option (showing the thought he put into it) was heart stopping. We do not even think it will be worth it to do a bone marrow biopsy because her blood work is not showing us enough evidence at this point. As Dr. R put it, we are teetering on the edge of having control of this disease. He also reminded me that she is always discussed and never forgotten. This is something I need to hear. There is just so little doctors can do for her, I am in charge of so much of her care.

In the meantime LillyAnna is living her beautiful little life. Her new bed has arrived and she slept in it for the first time! As of this post it has not been christened with puke but it has only been one night. I took the time to iron her new sheets and pillow cases for the first night. If you know me at all you are laughing out loud right now. Her new favorite thing to do is dance and make you watch. She likes to paint and color then hang her artwork next to Nettie's. And she loves to sing. She also likes to play Star Wars and Batman and put her babies to sleep. LillyAnna is all two year old:)

Update: bed has been christened!! LOL!

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, February 8, 2014

Good Night

Sunday, February 2, 2014

A La Orilla Del Viento/at the Edge of the Wind

Down the Shore

LillyAnna in her Favorite Place