Where the Sidewalk Ends

 

My Dearest LillyAnna,
I am a horrible mother.  I just want you to know that right off the bat.  I am savoring you and this moment today because you do not feel good.  You are in so much pain today.  Today is a bad day.  It happens like this for you some days.  We are supposed to go to the zoo.  Just you, no other kids and I am so excited to be able to focus on just you but I do not even know if we are going to be able to go.  Do you know what I am able to do?  I am able to sit in our old rocking chair.  You are curled on my chest and your sweet head is on my shoulder and I can smell your breath.  I can feel your breathing and you are so calm at this moment I can rub your soft baby feet as I rock back and forth. 

You are such a big girl, you never let me do this anymore and we will not be having anymore babies in this house.  It is so appropriate that you are deigning me honorable enough this morning, of all days, your third birthday to hold you this way.  This year you have been so busy!  You learned how to use the potty although it was not easy for you.  On the days that you had a lot of pain (which was usually 2-3 days a week) it was harder for you to remember.  But you have mastered it now and this past week you have even been waking up with a dry diaper every morning!  That is pretty good for a three year old. 

Your Monsters Inc. obsession has been replaced by Star Wars this year and your favorite characters are Chewbacca and Yoda.  Why?  Who knows?  Except that you have always been your own person baby.  You love to play with dolls and Barbies but you are just as happy playing with batman and light sabers.  Reading books is a major infatuation lately, especially poetry.  You are loving Shel Silverstein.  I read you two of his poems before bed.  Sometimes you look at me like I am crazy.  Let's face it, you are not too far off the mark.  And sometimes you laugh hysterically. 

We took you skiing this year and although I was too afraid of the wrath of Dr. R in the event of an injury to actually put you on skis I did take you snow tubing.  Your older brother was terrified but in true LillyAnna fashion you had no fear.  You grabbed the sides of that tube and screamed with passion and joy all the way down the mountain.  It was wonderful and made me cry. 

Speaking of Dr. R, he tried to make me take your bottle away this year.  I told him I knew how to do it, I mean I took the twins bottles away a week after their first birthdays.  He said to do it.  I warned him that I was worried you would dehydrate yourself.  He said do it.  I called him three days later.  You were dehydrated.  He said give her the stupid bottle.  When we talked about it later he said you are stubborn.  It is that stubbornness that has gotten you through this horribleness and you need it but it also sucks.  I like your stubbornness. 

You broke my heart about a month ago when you told me you do not want me to lay with you at night anymore.  You want Daddy.  You have stuck to it too.  My heart stays broken.  I bask in my days alone with you though.  The twins are in school all day now and I get to be alone with you most days for now. 

You also lost a friend.  Lucy and her family took us under their wing and showed us the way of this chronically ill child world and then they lost Lucy this February.  There are just no words for saying goodbye to a five year, especially one as magical as Lucy.  You truly enjoyed Lucy and so did I.  You always wanted to go to see her and sit in her bed.  Her mother Nicole has been a true friend to me and we miss them all. 

You are a three year old who still drinks a bottle but I do not care.  I remind myself that I have never seen a thirty year old who drinks a bottle and you will give it up when you are ready.  You know you own pain and ask for medicine when you need it.  You are good at that.  What other three year old can do that?  You also know the difference between a pustule and a mosquito bite. Impressive.  You really love lip gloss mostly as a result of the blisters you got on your lips for a few months this winter although you have not gotten them in a few months.  You really love to paint your nails.  You are particularly attached to your blankies; especially one made by Michele for your brother when he was a baby.  Like your other favorite blankets it has a satin border. 

On the days you do not feel well, like today, you sit in your pint sized lounge chair with blanket and bottle and watch movies.  It is really bad when we go through numerous movies, better when you are up and moving after one movie.  A great day is one where we do not need a movie and you are ready to go first thing like most three year olds... it happens. 

This summer we took you to NYC to see some docs.  It did not go well.  They did not know as much as we had hoped, we were the bugs under their microscopes.  But boy did you LOVE the city!  We did learn a big lesson.  It is time to move on:  we are to stop treating you and our lives like you are very sick and start treating you and our lives like you have a chronic illness.  As our docs said, this is as good as it is going to get.  Although this has encouraged forward movement and positive change in our lives, my chest is constricted in a vice... tighter... tighter.  This is your life.  There is nothing else we can do for your pain.  There is nothing else we can do but give you these horrible medications.  There is nothing to do but wait and see what happens next. 

 

And then there is so much to do.  We can watch you grow.  You are so much fun.  Taking you to the zoo and seeing your personality shine through when it is just you is beautiful!  I am enthralled and in awe and my heart beats fast.  Your independence is at a peak.  You are not big on dogs but you love birds.  This winter you really want to learn how to swim.  Strawberries, chicken nuggets without the breading, spaghetti and meatballs and cucumbers are among your favorite foods but you are willing to try just about anything.  Straight chocolate is just about the only thing you will eat when I cannot get you to eat anything else, but it has to be good chocolate.  You are simply amazing.  You are so smart and so kind and so intuitive and I love you so much. 

You told Nonni that your scars are beautiful.  You came up with that all on your own.  You have no fear and so much faith in yourself.  I think that is what people fall in love with when they meet you.  Because they do, people just fall in love with you wherever you go.  Happy Birthday my beautiful scarred baby girl... Happy Birthday.  I will just sit here and rock you until you decide you are done and get down.   When that happens my heart will break a little more because this moment will be over and there are so few left and you are so precious.  But I will also rejoice because watching you grow is a wonder and I am the luckiest person alive to be here to witness it. 

I love you,
Mommy
2014

Nothing Sticks Like a Shadow

I feel like we are living in the shadow of a circus elephant perched on one of those round yellow stands.  I am standing with my arms around my happy, unsuspecting family.  The elephant keeps looking back at me.  Every time our eyes meet, his seem to say, "I would watch out if I were you.  I do not feel too steady up here." like I do not know that... Guess what?  I do not feel very safe down here either. 

Living this new life has been interesting to say the least.  I am a piece of silly putty being pulled between being completely normal and being in crisis.  And then I can be pressed on any emotion and it is stamped on my face.  Poof.  Just like that.  For a little while I thought maybe it was working, we just ignore all these problems with Lil!  That's it!  She has a little breakout for a few days before her shots, we give her a shot and she gets better.  We can live like that.  Yes!  That is it!  Oh wait.  Then there is that elephant.  And then her birthday.

Her birthday...
LillyAnna was so excited for her birthday party.  It was all she could talk about for weeks.  I am not kidding.  She would hold up three fingers.  Those chubby little fingers.  In the wake of my first babies going to Kindergarten those chubby little fingers were so beautiful it hurt. She would look up at me with those huge brown eyes and tell me she wanted a minions cake.  The next day, while coloring I would hear the most adorable voice yell "Mommy!  Mommy!  I want Star Wars on my cake!"  We went over the list of who was coming to her party I don't even know how many times.  She kept asking me, is ___ coming to my party?  So if you think it did not matter to her if you were there... think again.  And if you wore her t-shirt, she noticed. 

 

 

Between all the fun, thunderstorms, humidity, lots of family and friends, and food LillyAnna had a great time.  My favorite part was when we came back to the house and opened the gifts (thanks to the thunderstorms).  Almost every gift we opened Mommom and I exclaimed, "Well, she knows you well!" or "He sure knows you!" because you all do.  She loved everything.  And we got enough money to pay for her swim lessons for the year.  Thank you all. 

But the next morning the elephant lost his footing.  Lil woke up in so much pain. Was it the weather?  Remember she has arthritis like a 90 year old so the rain and humidity could be the culprit but with her PG it could be just that the excitement was too much.  She also has a bunch of mosquito bites which sends her system into a frenzy giving her a bunch of pustules.  She now points them out herself.  Mom, I have a pustule and points them out with those heart achingly beautiful chubby fingers. This has continued for the past couple of days.  Her actual birthday was not a good day for her but more on that later today.  She is not awake yet today so we will see what it brings. 

The bright side?  There is a pile of birthday gifts and love propping up the elephant so he has not fallen over completely.  We are safe for now.  You are still our saviors and I am still weaving my way through this life. 

If you are near a radio today tune in to just about any Delaware radio station and listen to the WSTW radiothon.  Chris and I will be telling our story to help AI DuPont raise money to help them do the amazing things they do.  They have saved our family... saved our lives and continue to just that.  Check out www.nemours.org/radiothon for the stations that will be broadcasting our interviews.  We will be interviewed live so I cannot tell you exactly what time or how long each interview will be but the less popular stations usually interview for longer times and we will be there from 12:30 to 3:00pm. 

 

 

Giraffes Can't Dance

My mind right now feels like the end of a hurricane.. or maybe I am in the eye and I don't even know it.  At the present moment Lil is laying on the couch not crying for the first time in the last 24 hours.  Nettie is lying on my back and it feels so nice that I do not want to get up to get my coffee (but I really want my coffee).  All of us are watching Aladdin on the Disney channel and for once they are not showing a commercial every five minutes.



Life is changing around here.  I am leaving my job at the restaurant and I cannot tell you how sad it makes me.  There are a thousand adjectives for sad:  unhappy, dejected, despondent, blue, cheerless, dismal, down, lugubrious, melancholy, out of sorts, sick at heart, mournful, morose.... but sad, sad is just the word that describes it best.  I walked into Redfire one year ago to talk to the General Manager of Platinum Dining about a job.  He and I had know each other 13 years before when I was a server at Dome and he was a manager.  In between I had been teaching first, second and third graders and raising a family.  A very good friend of mine and manager at another Platinum Dining restaurant, Eclipse, told Kelly that I was looking for a restaurant job again because of LillyAnna and our situation so Kelly agreed to interview me.  When I sat down with him at the high tops he asked me the general interview questions and then said something you would never hear in the public school system (I am sorry but you would not) 'Gina, you did an amazing job when you worked for me before.  If you need a job, I am going to make sure you have one.'  I started to cry.  If you know Kelly, it made him pretty uncomfortable!  But it was something I will never forget. 

And then it got even better.  The group of managers, servers, cooks, customers and busboys (most of whom do not have kids of their own) took me and my family under their wing and just cared for us.  They understood when I needed to check in at home, they understood when I needed to change my shifts or when I was a little distracted.  They knew when I needed help cleaning up at the end of the night because I was working on my third night of two hours sleep and my third day of a migraine.  They listened to my stories when they had stories of their own.  They became my friends.  I have heard them say that I became their friends too by listening to their stories, by giving advice, by smiling and being excited to see them.  I have heard them say that I gave them excellent service or just gave them the space to take a deep breath with a good drink and good food which was exactly what they needed. 

I have had intelligent conversations with them about God, food, wine, liquor, relationships, love, motherhood, sickness, fatherhood, marriage, war, death, suicide, and being drunk. I have laughed until my stomach hurt and I have cried with these people.  Some of them proofread papers for my work with the House of Representatives, put me in position to be in places with Lil that we got real help, gave us exorbitant amounts of money in tips, gave us as much as they could, gave us a listening ear, bought our t-shirts, encouraged me to make smart moves in my life, gave my kids candy:)...

I was born to be a teacher.  I love teaching.  As my husband says, it is in my blood.  Teaching teachers this summer has been amazing and as I have read my evaluations and heard the things experienced, inspiring, passionate teachers have to say about what I taught them I know that I am making the right move.  But to tell you the truth, I love bartending too.  I love seeing people sit down with an expectant look or sometimes a frazzled look, mixing them a drink that is just right and maybe listening to how their day was and seeing that look of pleasure and relief come across their face as they take that first sip.  Or talking a customer through the menu and helping them pick just the right thing.  I know it is stupid but it makes me so happy.  It is just like cooking for people.  I still get to feed them, I still get to fill them up. 

Now listen carefully: I do not want you to do anything about this, but this blog is and was created as a place for me to be able to share the reality of living with a chronically ill child.  Money is one of those realities.  The one thing I am very worried about is money.  The University of Delaware job is a dream.  I just love it so far and I do not see that changing.  More than that, I am hoping that working during the day will be better for me physically.  Bartending was not good for my migraines and I was taking more and more pain medication.  If you do not know, my mother died from a prescription drug overdose so this is not something I want to mess around with.  When I bartend I only get a couple hours of sleep and that is a migraine trigger for me.  The U of D job will be better for the kids starting Kindergarten; yes, my babies are starting kindergarten.  They need me to be around to get them ready for school especially when Lil is not having a good time.  It has such an effect on them (especially Chris).  This way I will be around to do homework every night, take Nettie to dance, pack lunch and most of all, not be exhausted and have a migraine all day for three or four days in a row. 

I am also hoping to do more work for the other company that teaches Responsive Classroom, the Northeast Foundation for Children.  They have offered me more work, I am just waiting for the actually work to come in.  This is similar to U of D.  I have been told work is coming, I am just waiting for it to come in.  The problem is, most of our savings is drained from our wonderful (hear the sarcasm?) trip to NYC and other Lil things that have come up, so until I see those jobs on my calendar I will not be able to breath. 

So what are we going to do?  Well... Redfire comes through again.  They told me that I could come back to pick up shifts if I need them.  What a wonderful safety net to have! It is not perfect but it is something.  We also need to re-negotiate our budget since everything is changing AGAIN.  It is just so hard.  It is not that you feel angry with Lil.... I mean, how can you?  But there is an anger and frustration there that is big and ferocious.  If it were not for this horrible disease not only making my little baby girl have so much pain but on top of it turning our world upside down over and over and over again we would be in such a different place.  There is a big part of me that likes the place we are in, I have learned and have grown so much.

 Humira shot so I am hoping that will make things better.  Methotrexate is Monday and I am not looking forward to it.  It has been making her very sick lately.  We have started premedicating her for it but it is not helping much.  She wakes up in the middle of the night with a very high fever and vomiting. 
She has been doing so well until this week.   Quite a few pustules popped up and she just had a lot of pain, and I mean a lot, swollen fingers, swollen toes and she has not really eaten in about 4 days.  But she was due for her









Tomorrow is my last bartending shift at Redfire.  If you would like to come see me off, I would love to see you!  I think Chris is going to come by with the kids around 5pm.  It is best to come a little early on Sundays. 

Redfire Grill and Steakhouse
400 Lantana Drive
Hockessin, Delaware 19707
302.235.2600
 Sundays hours 4-9

An angel descended on us this week just when we needed it.  I have actually been talking to God a lot lately.  Asking him to guide me. My religious beliefs are considered odd by some but they are mine and I do pray.  The gift we were given this week, I believe, is a sign that I am on the right path.  I guess only time will tell.  I am choosing to "take a leap of faith" as my friend Brittany put it.  A leap of faith is scary any time but even more so when you have a group of little ones counting on you.  But here we are, counting on a leap of faith.

Mending

A friend of mine recently described to me what it felt like for him to experience arthritis pain as a twenty something.  This person is a strong man, the kind of man who played sports.  The kind of man who you look at and it seems they can just do anything.... the ones who are so attractive and are just at that point in their lives where they are so full of life and energy that it is practically bursting out of them.  He told me that he had to crawl to his bathroom and was crying like a baby because the pain was so bad.  He called his mother and had to have her come and help him up.  He could not lift himself.  That is my baby.  That is her pain.  She looks so healthy.  I know.  But she is hurting.   

It was two years ago this month that a usually easy going me got her first lesson in how to stand up for a chronically ill LillyAnna.  We had been watching lesions grow on my then 9 month old baby's beautifully soft and until then perfect skin every time the nurses tried to put in an IV.  Finally a tech came up from somewhere and said they where going to try to put one in her skull.  They wanted to shave a spot on her head and attempt an IV there which I knew would cause a lesion.  Lesions at that time were growing so fast we were watching them and we had no idea how to stop them.  To top it off, someone finally told me this was all happening because what my baby really needed was a PICC line but it was a holiday weekend and the people who do that were not working. Lesson 2 of having a chronically ill kid.  I walked out of the room and down the hall, screamed at the top of my lungs and ripped my shirt off Hulk style.  Lucky for me a tech who has since become a very special friend but at the time I did not know walked up to me and wrapped me in her arms.  Thank you Darci.  I walked calmly back into the room and said No.  No you cannot put an IV in her skull.  She asked me if I was refusing medical care for my daughter.  What a horrible thing to say.  I looked her in the eye and said yes.  They called the necessary people in from their fourth of July bar-b-ques and we got a PICC line and Lil did not die from dehydration that weekend.  This is one of only two negative experiences I have had at AI out of hundreds... literally HUNDREDS of experiences we have had there.  I have learned so much in the past two years about myself, my mom-ness, my daughter, my marriage, my friends, my enemies...

And now we are at the point were we are saying LillyAnna HAD a terrible sickness.  She WAS very sick.  Now she IS chronically ill.  This will be her life.  This will be our lives.  It is time to pick up the pieces.  Even as I write this I am in awe at how everything has fallen into place.  At this moment in time, this week, this month, right down to this very post.  I started this post three weeks ago on the day of the anniversary of Lil's first lesion and so many things have come full circle.  This past week I taught Responsive Classroom.  The last time I did that was the very first time we found the first pustule which caused the first lesion on Lil's thigh.  How many times have I told that story?  "Well, I found a pimple on her thigh, I popped it and went on a business trip..." hundreds.  I have told that story hundreds and hundreds of times. 

So now our family is once again entering a new phase of life.  A new generation.  I am getting back into a form of teaching.  It is not full time teaching in a classroom but I will be working part time during the day with the University of Delaware to grow new and experienced teachers, such exciting work.  I will be leaving LillyAnna with a nanny (hopefully) for small portions of the day.  The twins are starting Kindergarten and they will be there all day which gives me a little more flexibility.  She will still have bad days, and so we will but nothing is going to change that and life has to go on.  We cannot continue to wait for the next horrible thing to happen.  And unfortunately we are not in a position for me to be able to just stay home and take care of her, I have to earn an income. 

The National Institute of Health is moving forward FINALLY!  This is good news but I will not allow myself to get too excited after New York and we do not have to spend any of our own money for this one. 

We will continue to share our story and continue to hope for a cure for our girl.  We will continue to count on your support and continue to be thankful for everything you all do, for the diversity of support you all offer us that keeps us going.  I am just going to try to have a new perspective.  I am scared, very, very scared.  Two years is a long time but if LillyAnna can embrace life through her pain, so can we.

 

Mending

A giant hand inside my chest
Stretches out and takes
My heart within its mighty graps
And squeezes till it breaks.

A gentle hand inside my chest,
With mending tape and glue,
Patches up my heart until
It's almost good as new.

I ought to know by now that
Broken hearts will heal again.
But while I wait for glue and tape,
The pain!
The pain!
The pain!

By Judith Viorst



Shhh! We're Writing the Constiitution!

The outpouring of support I have gotten since my last post has been just simply phenomenal.  I cannot thank you enough.  A good friend of mine told me once when this all started that I have to be ok with people doing for me because I have always done for them and I will be able to do for them again someday.  I am waiting for someday.  Right now I am still hurting and I am still angry.  Another good friend reminds me that sometimes you just have to feel things until you do not feel them anymore.  I think that is great advice. 

So I am enjoying every minute with my babies.  I am hugging them and kissing them and being a mom.  I am surrounding myself with good friends.  I am taking the extra time to look at the beautiful night sky and the extra second to take a deep breath.  I am feeding people.



As I mentioned in an earlier post, The House of Representatives is asking families of children with rare diseases how they can help us.  How amazing is that?  We were asked to tell our story and respond to some questions they have.  Here is my response.  It is also a nice update on Lil's condition all in one spot if you have just joined us or you are feeling a little lost as to where we are. 


___________________________________________

 

June 10, 2014

 

The Honorable Fred Upton                                          The Honorable Diana DeGette

Chairman                                                                     Ranking Member

Committee on Energy & Commerce                            Subcommittee on Oversight &

 2215 Rayburn                                                             Investigations

Washington, DC 20515                                                           2322 A Rayburn

                                                                                    Washington, DC 20515

 

Dear Chairman Upton and Ranking Member DeGette:

 

Thank you for requesting comments for 21^st Century Cures-Patients. I appreciate the opportunity to share my family’s story, which underscores the need for a greater investment in pediatric research, cures and support groups at children’s hospitals across the country.

 

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 happy 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 a couple of days to diagnose her condition as Pyoderma Gangrenosum (PG).  They were the scariest days of my life.  PG is associated with adults who suffer from Crohn’s disease, rheumatoid arthritis and a few other auto-inflammatory diseases.  However, LillyAnna’s was only the 17^th reported case in infants.  Most of these infants responded to oral steroids and then followed up with some other maintenance medication with success.  These other infants were not followed past two years although we did find one who was followed for five years with no recurrences.  LillyAnna would 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.  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.  Yet, 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.  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 soothe her because she had so many lesions. 

After a short trip home, we ultimately  stayed in the hospital for 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 rapid 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.  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 where 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 had to resign from my full time position as a public school teacher; 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.  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.  We began pursuing a second opinion with our doctor’s support; however other children’s hospitals have as yet been unwilling to see us.  It is difficult to contact them due to the “rare-ness” of Lil’s disease.  We do not even have a specialty area we can contact.  I have to try to convince their diagnostic teams to see us.  This is not as easy feat.

At this point I compare LillyAnna’s suffering to that of an eighty year old woman.  She has arthritis and bursitis in her fingers, toes, knees, elbows and hips.  The other day I watched my two year old try to string beads and get frustrated because her little fingers were too swollen and in too much pain to get the job done and she just cried in frustration. She also still vomits so much that it is difficult for her to gain weight and grow appropriately. 

She has her good days and her bad days but the bad outweigh the good unfortunately.  The medications we are currently using to treat her are just plain terrible.  She is getting Humira which is a bi-weekly injection but not even FDA approved for a child her age.  She also gets weekly Methotrexate injections.  My husband and I cannot even think about the carcinogenic effects of these medications.  From my own research and what the doctors have said between their words, it is not a question of whether or not they will cause cancer, it is a question of when they will give her cancer.  Never mind the fact that they are not doing a very good job.  My doctor often tells me we have a tenuous hold, at best, on this disease.  I do not like the fear I see in his eyes when he says this but it is even scarier that he flat out admits how scared he is. 

So the answer to your first question of what is the state of discovery of cures and treatments for our disease is quite clear; there are none, there are none now and there are none on the horizon.  I have worked so hard to be an advocate for this little girl, we have given up so much as a family and yet, I cannot get much attention for her in the medical world or the social world.  Once people meet her they simply fall in love.  They are entranced by her story, by our family and by her…. Her strength, her tenacity, her bravery.  But we have tried to gain the attention of day time talk shows, local news stations, and social media.  The only avenue which has worked has been my blog www.lillyannacastelli.blogspot.com. Finding a way to gain attention for rare cases not only in the medical community but in the government, and social community would open our world enormously.  People just do not understand, the medical community included and maybe even more so than the others.  We need understanding and attention in the medical world more than any other.  It is cases like LillyAnna’s where it seems as if there is only one that there may be others suffering with the same symptoms, we have no idea because no one is listening. 

But we are not alone in this difficult journey.  Other parents of children with rare disease have many of the same struggles.

The single most important piece to any success we have had has been our support group “Chronically Cool Families”.  This group is run by a social worker at the hospital and two Childlife workers who give their time for this group.  They are not paid.  The hospital lets us use the rooms and the Women’s Auxiliary pays for some light snacks if we request them.   The group is composed of families who have a child suffering from a rare disease.  At the risk of sounding overly dramatic, this group has literally saved my life.  It is the sounding board that understands feelings no one else in my world understands. It is the source of medical information to which no one else has access.  It is the group of people who knows the ins and outs of the systems of federal Medicaid and Medicare and how it applies to a child under two who has a disease no one has ever heard of.  It is the group who understand the social etiquette of going to a child’s funeral. We go on trips together so our kids can go places and know what it feels like to be normal.  These types of groups need to be funded in every children’s hospital in the country.  We need money, we need resources, and we need attention.  You can help to give us a platform and to shine a spotlight on the state of research for pediatric rare diseases.

The financial burden we carry is enormous.  As I mentioned, I had to resign from my position as a public school teacher.  We are lucky in that my husband also worked for the school district as a maintenance mechanic so we are able to keep LillyAnna’s state benefits and supplement with Medicaid.  However, we went from a dual income household to basically a single income household.  Currently I have gone back to my college job of bartending two nights a week just to make ends meet but this is extremely difficult for us emotionally.  When LillyAnna has a difficult day, it is almost impossible to leave her and the nights I work I am only getting two to three hours of sleep if I am lucky.  I have learned that most families in our situation sell their homes and downsize.  That is what we are looking at doing next but with the housing market being what it is and the debt we have already acquired before receiving help, it does not even seem that would do anything for us at this time.  The Social Security office told us to let them know if my husband lost his job, then they would be able to help us. 

I wish I had a good answer for how you can help but I am realistic.  I know that no one is going to throw a ton of money into a disease that only one child has no matter how much that child’s family is suffering, no matter how beautiful that child is (inside and out).  But there are some things you can do.  Grow our support group, find money to support it and pay the people who are running ours.  Then give them the resources to make it happen in other hospitals.  You could also work with the National Institutes of Health to allocate additional resources to pediatric diseases as a whole.

The other thing you can do is help us financially.  We fall into a unique niche in the world of disease.  We cannot just hire any old nurse to care for our little ones.  Especially someone in my position.  I have to care for her and she cannot got to daycare or school.  I made more money than my husband and now, suddenly I cannot work.  What would that look like in your house?  If the main bread winner suddenly had to stay home? 

Research into inflammatory diseases could not only help LillyAnna but could help many other children who have similar issues. 

Thank you.  Those words are simple and overused but in this case there are none more appropriate.  You are taking an interest where not many others are and you, who have the power, are asking how to help.  Thank you for listening.  Thank you for caring.  If there is anything else I can do to persuade, assist, inform or educate please do not hesitate to ask. 

Gina Castelli

 

 

Dirty Beasts

It is so unfair but I am going to do it any way because I know for a hard fact that my doctors do not read blogs.  And for my nurse friends who do, please understand that I am angry for my baby girl.  And understand that this does not reflect at all on Dr R and Dr B. And that I know Doctors are WONDERFUL people, every doctor we have met has been so kind, so helpful. 

Doctors can be Dirty Beasts. They are the holders of so much knowledge and they keep it locked away in a vault lost to our lack of understanding.  They are the misunderstanders of our pain and everyday experience but to make it worse they think they do understand and leave us wanting to scream from the bottom our bellies in frustration.  They walk away before you are done.  They leave you with questions and thoughts you did not realize you had until the next morning when you wake up in a strange hotel room next to the softest little baby arms and legs listening to quiet breathing at 5am.  They say things in passing that you hold on to like your last crust of bread and think nothing of it and they diagnose things they have no business diagnosing.  They have everything and I have nothing.  Oh wait, they have my money... and my hopes and dreams.  Too far?  LOL!

We have drained the last of our savings account, we have drained our bank account for this month, I caused myself so much stress I can't even discuss it just to find out that Dr R is an amazing doctor (which I knew but was awesome to hear) and he has done just about everything right. And that she does indeed have Idiopathic Infantile Pyoderma Gangrenosum and that we are doing everything we can to control it and this is what her life is going to look like. I have a few new ideas to bring home but nothing mind-bending.  We had a great time but Chris and I were planning a weekend away which we have not had in a long time and we really need and this took care of that.  Please do not think I am asking you to do anything.  This blog started as a way for me let it all out and here it is friends. 

These doctors were wonderful and knowledgeable but they are not invested in my little girl.  They fell in love with her just like almost everyone does but they did not even take the time to review her records before we walked in the room.   

Basically this dermatologist has treated many cases of PG in adults and even seen a case in an infant.  He said Dr R is a great doctor and gave us his personal phone number to give to Dr R in case he has any questions.  That is a wonderful resource.  He believes she has Juvenile Inflammatory Arthritis and that is the main cause of her PG.  But he is a dermatologist so I need to take that theory to my rheumatologist at home and discuss it with her because if I have learned anything on this trip it is that AI DuPont has some fabulous doctors.  They may not have seen as much as these docs have but they have good instincts, they are smart, and they DO look at records.    And we saw one of the world's best rheumatologists the day before that and he did not diagnose her with it.... see what I mean? 

I just want to scream and cry and rip their hair out!  Don't tell me not to worry.  My baby is two and a half and has days when she cannot get up and play... DAYS!!!!! Do you understand that?! And you say don't worry about the side effects of the Humira and Methotrexate... well I want to wave my magic wand, reverse, take their two year old, hold that syringe up to their leg and then ask how worried they are.  Oh wait... let me do that twice a week... for YEARS!!! Now how worried are you?  And then weird things happen like softball size, hot, red welts show up all over her body for no reason. Or her lips get PG like lesions on them.  Now how much should I worry? 

Fuck them.

Sorry.

The next post will be better.  Now I am going to leave NY and see my other babies.  We got them some little treats.  My dad and stepmom are going to spend the day with us.  And I am going to breath.  Just breath and thank God that today is NOT a day when she is sitting in that lounge chair all day. 

Not proofread... don't even want to read what I wrote:)

Strega Nona

I am not sure if you have noticed but all of my blog titles are children's books and they are somehow related to the contents of my post.  This one has no connection to the contents of this writing but it has a connection to the contents of my heart.  This book is one that my grandmother, my heart and my soul and best friend who passed away eleven years ago, read to me over and over again then I read it to gads and gads of kids as a school teacher and now I read to my kids.  I need her right now.  I can't have her.  Being here in the city reminds me of her but it is so much more than that.  I want to know what she would say about all this, what she would do, how she would put this all in perspective. 

When I met my husband Chris I was dating someone else (don't think less of me... it was a convoluted situation at best).  At the time my grandmother was dying of cancer.  In those days my family was always around her, soaking her up, eating, being together.  She would come sit with us and then go in her room to rest.  Sometimes her grandchildren would just go lay with her, no TV no phones, no nothing.... just her.  I finally got her alone (my family was wonderful about allowing me to have some time to myself with her) and I laid down next her in her bed.  We faced each other in the quiet dimness of her room and I said "Mommom, some boy asked me out."  Her response: "GO!  You are not married!"  (caveat: the relationship I was in was less than healthy to say the least but my grandmother was one person who actually understood my attraction to this person and enjoyed him as well).  To which I responded, "But Mommom I am with Jim." and the conversation went on from there.  Needless to say, I went out with Chris, I dated Chris.  When my grandmother finally ended up in the hospital where she died a few weeks later I introduced her to Chris.  She sent him out of the room and told me I would marry him which of course I did and I have never regretted it.  I am still in love with him.  So what would she say about this, what would she think?  She knew everything.


We are here in New York City to see doctors who 'have seen it all'.  So far I am not impressed.  The Rheumatologist we saw yesterday is supposed to be one of the best in the world and he was obviously an amazing doctor and a wonderful man.  He came in with quite a crew: a few doctors, some fellows and a medical student.  We are used to this, we never walk into a doctors office without being the star of the show and I support it fully.  I even pulled out my computer so they could look at wound pictures.   I want as many doctors as possible to see this disease so that if any baby ever walks into an office or hospital with it maybe someone can say they have seen it and we could have helped with that.  But the bottom line of what this doc said is that we are doing the right thing.  Stay on the track we are on.  We can increase the Humira if we want.  This breaks my heart.  This is it?  This is what my baby's life has to look like?  Having days where she does nothing but sit in a chair because she is in so much pain?  Vomiting every day?  Getting painful pustules all over her body?  Waiting for the next awful horrible to happen?  However, kudos to Dr. R and Dr. B.  They walked into this disease blind and did all the right things.  We paid $700 to hear from him that we don't need to change anything.  Of course, we did create a relationship that will help us in the future when we need.  Does he take insurance? Of course not.... the best of the best never do!  LOL! Never mind the $265 a night we are spending on the hotel:) Good times my friends, good times. 

Thank you to everyone who helped out to make this trip happen so quickly.  It IS good.  Chris and I are just in a bit of a whirlwind and are using this venue to vent.  Finding this doc is truly a goldmine in so many ways.  My mind is just not there right now.  Maybe it will be later.  Today we see a dermatologist who specializes in auto-immune disorders.  This is something we have never done. 

In the meantime we are enjoying the Big Apple thanks the generosity of some very kind people. 
I did not proofread this, need to get out and see some of the city:)