Don't Let the Pigeon Drive the Bus

Ten years ago today I was drinking Lambic and Mimosas with my bridal party as we started to pull out dresses (for the girls at least) and curling irons and breakfast items.  I was surrounded by friends and family and so was Chris.  We got married at the beach in October so we filled an entire hotel ourselves and we filled it save for one room (those poor fishermen) with our guests.  And although this week is so much different, I feel surrounded by friends and family in much the same way.   It has taken two years of being on Facebook but I have finally reconnected with some very old friends.  There is just something deeply comforting about talking to people who have known you a long time; especially when they are letting you know they are there to support you. 

Being married ten years means something to Chris and I and we had actually saved up a very small amount of money.  We were going to use it on a dreaded Living Social deal to go to Maryland, can you believe the irony of that!  Just for one night away but we have not really been able to celebrate an anniversary in a long time.  I believe it is important to celebrate anniversaries.  Milestones in relationships are important, marriage is work especially with a sick baby or even just little kids! Then this trip came up on the week of our anniversary and we just looked at each other and rolled our eyes.  We told ourselves that we will do something to celebrate and we will.  I know we will...someday. 

We could not live this life without a strong marriage.  I could not do all that I do without him.  He fills in all of my gaps, gets me ice creams after a hard day, understands that I need to do these things for LillyAnna and the twins even though they are not the things he would do and he supports me along the way.  Is he my best friend?  Yes and no.  He is a best friend.  But he is so much more than that, to say best friend seems too small.  When you have spent two years watching your baby wretch into a bin, your eyes meeting over the top and feeling each other's pain; best friend does not seem to cover it.  You all know what I mean from your own situations.  My friend who nursed her father through his death with her mother; there is more there after you have lived that. 

So today on our anniversary I hope to be able to hold his hand like we did when we spoke our vows and made our promises.  I hope that I remember as I do the love I had for him that day because it is different than the love I have for him now but no smaller.  I hope we get some stolen moments, maybe a kiss outside of a hospital.  That would be nice:)  Maybe tonight we will be able to go out to dinner or even to have dinner here.  Dinner here is A LOT of fun.  Last night there was a balloon artist and a ton of give-aways.  Spending an anniversary dinner with Lil is a good time.  I will definitely take that! 

In a Dark Dark Room

If you have followed LillyAnna's story, you know the single most difficult and traumatic part of the history of her disease was dealing with her lesions.  When things were at their worst, she had about thirty of them and the dressings had to be changed every other day.  These dressing changes were excruciatingly painful for her and for me although in a much different way. The memory of these dressing changes is something that still sits in the back of my head and heart.  If you are interested in hearing more, I wrote a post about it all.  It is called Whoever You Are.  Follow the links at the right side of this page, it was written in March of 2013. 

We found out yesterday that the angel doc would like to do a skin biopsy that is usually reserved for a time when patients are sedated.  However, we are not sedating Lil for anything on this trip.  So to do this biopsy we will have to papoose her, just like we did during her dressing changes, hold her down, just like we did during her dressing changes and scrape skin from her.  The team here is concerned about doing this for a number of reasons.  They are concerned about causing a lesion and they are concerned about doing it while she is awake.  I repeated what I have said from the time we walked through these doors, this is why we are here.  So if I have to go down that dark dark hall to that dark dark room in my dark dark heart I can find the strength to wrap my baby once again in a hospital blanket and help hold her down while trying to calm her by whispering in her ear so we can get the samples we need.  I believe that getting these samples is important.  I want to know why her lesions are so "extraordinary".  Even here, in this place where they have actually treated cases like this in infants before. 

In the meantime, we are trying to do what we can to make this place enjoyable for Lil.  It is important that she sees this as a place that is good overall.  It sounds like we will be followed by the angel doc and possibly could come back in six months.  LillyAnna adores the zoo.  My Aunt AnnaLouise lovingly bought us a year long pass to the Philadelphia Zoo and I take her up there to walk around often.  It is great because if she is having a bad day she just has to sit in the stroller and we can leave after only an hour or so.  Lil really enjoys the reptile house and the big cats.  So we attempted to use the Metro yesterday and after getting turned around a bit, we found the National Zoo and Lil was able to see animals she does not get to see at our zoo which was exciting.  Plus, the National Zoo is something to see!  She saw pandas and elephants and a bunch of reptiles that I could not begin to list. 

I was also able to take her on a subway which was especially cool for me.  When I was young, I spent a lot of time with my grandmother.  She was my best friend and greatest supporter my whole life.  As a young girl, she always took me into 'town' (Philadelphia) using the trolley and subway.  So taking Lil on was a lot of fun.  Little Chris would have loved it! Before we left my Aunt Tina had us over for dinner, backyard fire and pumpkin carving so I could focus on the twins a bit.  It was a wonderful day.  I took off my wedding rings and left them there.  I hate not having my wedding rings when I meet new people.  It just does not feel right.  Not having enough time to go back and get them before we left for Bethesda, I opened my jewelry box and my grandmother's wedding ring was sitting right on top.  Now that I have lost weight, it fits me perfectly.  I feel very connected to her right now. 

I will continue to keep you all updated.  I know we have a lot of new followers and I am glad to have you all.  Thank you! 

The Unfinished Angel

Best patient ever:)

I remember when the twins were born.  Conceiving them was not exactly the 'fun' people have in mind; we did fertility treatment for four years.  My mother died suddenly three weeks before they were born which caused me to develop pre-eclampsia and they had to come early.  With all of that, I was determined to breastfeed.  Nettie spent the first ten days in the NICU so it was only Chris and I clung to him like he was the rope and I was hanging from a cliff.  I was attempting to breastfeed him the day after he was born.  This being about my sixth failure, I was getting pretty nervous so when there was a knock on the door I jumped sky high afraid it would be the nurse with the dreaded formula bottle.  But it wasn't.  It was the lactation consultant... the real one.  She drifted in, calmly adjusted my pillows while giving me some sage advice, latched Chris on perfectly and for the first time I felt him drink beautifully.  It was like an angel had descended on us.  Just as quickly as she came, she was gone. 

Meeting the lead research doctor today was so much like that moment.  She was ethereally beautiful in a way only some older women can be that made me deeply envious and made me fall in love with her all at the same time.  She knew just the right moments to engage with Lil so that she would trust her.  She told me I was smart and I knew my stuff (not in those words).  She let her grey show.  We brought about 60 pictures of lesions with us and she spent about 2 to 3 minutes pouring over each one like it was gold.  She knew her stuff.   She taught me things and respected my opinion. 

We saw eight doctors today and that is not including the fellows and interns who came in just to take a peek.  I have not told Lil's whole story in so long but today I told it eight times.  Each doctor hung on my every word. 

You have to be humbled to hear the words "extraordinary", "amazing", "unusual for this disease", "fascinating" and a host of others at a place like the National Institute of Health where they see the most fascinating diseases in the world.  These doctors used those words to describe my baby girl.  I am still deciding how I feel about that and I think they are too.  They have not yet told us the plan forward. 

We met each doctor individually today.  Tomorrow Chris and I will get blood work which Lil is going to love to watch! Then all three of us are going to get either punch biopsies of the skin or shave biopsies.  As you know this is a procedure that causes all of us worry when it comes to Lil.  However, we consulted with Dr. R and the new angel Doc and we have all decided that this is why we are here.  We are going to have to take some chances.  The risk is that she will develop a lesion from this more invasive skin trauma.  We have steroids on hand and lots of practiced eyes watching out.  Other than that we have x-rays scheduled for today and some samples to collect.  We do not know what else they have planned for us.  I am hoping the team discussion happens. I hate not knowing what they are thinking.  We are already out of their research group technically, does this "unusual for this disease" amazing-ness put us even further out of that range or does it make us more interesting? 

Upon checking in at the Children's Inn the receptionist told Lil it was the coolest hotel she would ever stay in... I am starting to think she was right.  This place is pretty cool.  It is full of playgrounds, toys and books.  There are activities going on all the time.  There is crappy coffee and breakfast in the morning and dinner served at night.  I have no problem with crappy coffee!  It is odd for me to be on the other side.  I remember a time when, as a teacher, I served dinner at the Ronald McDonald house.  Listening to conversations at dinner I realized that if we continue on this journey I will have to learn and whole new language, again. And an whole new social life.  Is it one I want?  Yes.  And no. 

And then there are my babies at home who are being loved at by family and their school family in ways I cannot even begin to thank them enough for.  I had a chance to put Nettie and Chris to bed Sunday night over Facetime.  They used a book which they connected to a Heartstring when Lil was in the hospital, then when our beautiful friend Lucy died and now  again for this trip.  Their teachers and head of school shared with their classes. And our family, I just do not know what I can say about how our family has come through for us this week. 

I will continue to keep everyone updated.  Thank you for your support.  You have no idea how I feel supported just by seeing how many people have read the blog.  You just have no idea. 

 

GO! GO! GO! STOP!

 

Yesterday my wonderful cousin came to visit with the kids just so that when she comes to stay for the night while Chris and I are away with Lil at the NIH little Chris and Nettie are more comfortable.  I cannot possibly express in words how much this means to me.  Their anxiety is nearly palpable the closer we get to leaving, especially Chris.  I think we are going to have to take him to his pediatrician and figure something out for what we think are migraines brought on by stress before we leave.  He worries about it all so much, but especially me. 

When I got my new job he started to act out a bit.  I finally got out of him that he was worried there might be a fire or emergency while I was at one of my jobs and he would not even know what it looked like and would not be able to get there to help me... his words.  He told me that a son's job is to take care of his mommy.  He is five. 

While my cousin was here yesterday, Lil had a temperature of 101.5 but you would never know.  She went to the park and played.  I don't think she really ate all day but she did not throw up either so that is one small victory.  However, her lips are starting to become ulcerated again and she is still consistently complainingly of belly, leg and back pain.  She is usually active for about a half to one hour and then needs to rest for one to two hours. 

We are going to get blood work to recheck liver and kidney levels on Thursday because they were so elevated  last week and we are also going to meet with Dr. R in preparation for the appointments in Maryland.  There are just going to be so many decisions to make on the spot and I have a lot of research to do so that I am prepared to make them. 

Even the paperwork they have sent me is overwhelming.  This is a whole new category of disease and medical language.  Of course, I am lucky to have Dr. R and I have access to him at all times (and I mean all times) but I do not take advantage of that and because of the true nature of our patient centered care, there are many times when the decision will just lie on our shoulders.   The simple fact is that I know the most about Lil's disease.  That is very very scary. 

I filled in for a friend behind the bar at Redfire.  It was good for me. Besides the money, Chris and I talked about how it gets me out of my own head.  I cannot think about her while I am bartending.  There is just no time.  I serve good friends and it is interesting to me too how I see the people who work there so infrequently but those that I was close to all noticed that I was not myself.  That is how the restaurant business is, friendships there are different than friendships almost anywhere else.  I love my new job and it is beyond a doubt where I am supposed to be but it felt really good to be in that restaurant last night for many reasons. 

Beginning of ulcerations on lips
 

The House at Pooh Corner

I am sitting on the couch right now writing this and choosing a title.  I chose this title for two reasons.  One, Lil has been loving Winnie the Pooh lately and when she feels like shit she wants to watch a lot of movies (over and over and over) so we have been watching a lot of Winnie the Pooh.  The second reason is that it is simple and comforting and soft and I feel awful.  Why do I feel awful?  I feel awful because I am listening to that happy little baby girl vomit her guts up again and again for the third week in a row.  To make it worse, when we are trained in the emotional side of giving injections we are told to tell the child that the shots are going to make them better and that is why we do it.  Well if that is my promise, I am breaking it.

You know those friends who are more an extension of your humor, your brain, and even sometimes your body?  I am lucky enough to have a couple of them (I have written about them before).  This past weekend I decided we were going to see one of them.  My friend Alana.  She lives hours away in New York.  I have lost so much weight from these migraines and stress that I need an entire new wardrobe.  As you all know, we are not in a position to purchase said wardrobe but this friend has a fabulous and sophisticated sister who lives in a very posh and fancy section of Manhattan who just happens to be my exact new size (shoes and clothes) and just happens to be getting rid of basically everything in her closets. Awesome right? 

Have you ever seen the movie Stand By Me (a short story originally by Stephen King)?  Remember the scene at the pie eating contest?  Despite the fact that Lil recreated it in my living room half an hour before we were to leave, I left anyway.  She puked most of the way there but we went anyway.  Ironically I puked more times while we were there than she did!  Damn migraines... and possibly food poisoning.  While there, we went to the Chelsea Market, Lil was in a stroller and when she stood up her legs actually buckled under her.  She turned, looked at me and just started crying. I have never seen that happen before.  The thing that really bothers me is that when we were at her Rheumatologist two days before that and she was dancing around the office.  Notes state that she looks great!

Dr. B tells us young children who have had pain most of their lives are like this; they can go from happy go lucky to pain in seconds because they are so used to pain they act like they are fine most of the time.  This is why people always say, "she looks so good!" and she does a lot of the time.

But the doctor did not see the fear, pain and confusion on her innocent face when she turned around and the depth of those big brown eyes when they met mine as she stumbled, tried to catch herself and realized those legs just could not be trusted.  It was quite a moment for both of us.  And I have a witness.  My friend asked why I need that, why I need people to see what I see, why do I not trust myself. 

I do not know the answer to that question.  I don't know if I do not trust myself and I need someone else to see what I see so that I can feel more confident.  Is it because people question me and I feel like I need back up?  I truly do not know but it is good to have people see what I see.  My gorgeous family has graciously offered to spend time with Lil while I work during the day.  As you know, she cannot go to daycare.  Now that I have people caring for her for longer periods of time they can see what I see.  It is good for everyone involved in her care. 


Once again LillyAnna just loved New York City.  She just eats it up.  And my friends in New York take such good care of me and my babies that it is near to impossible for them to not love anything that involves them.  At one point I turned the corner in my Christina's apartment, the kids were standing on the counter and Christina was standing there helping them pick out gourmet chocolate more expensive than I care to think about and then laughed while they ate it.  This being their first time to the city, the twins absolutely loved it. 

On our last night there I was fighting Lil to take her medicine. Alana watching helplessly hoping to do something but having no idea what to do.  There was really nothing to do.  When Lil does not want to take her meds, it is a real fight and not for the faint of heart.  Alana asked me later, why do you think she does that?  My only answer was that I think she is just tired of it all.  Tired of the pain, tired of the medicine that tastes bad, tired of the feeling like crap... tired of being sick. 

This bed is one of the happiest places I know.  This past weekend I had the chance to share it with my babies and my friend embraced them and all the baggage and drama that comes with them and me.  She would not even question this... would not even think about it.  But to me, to us, it means everything.  Family means so many different things in my world.  It means depending on my aunts and uncles to give up their time to watch Lil so that I can work and coming home to a baby who is well cared for and a house that is cleaned up and has fresh flowers on the table and laundry done!  It means depending on my mother in law and close friends to do the same thing.  It means not being able to give what I used to be able to give and having people love me just the same, in some situations even more.  Family is always there. 

One of the most painful parts of Lil's illness for me are Nettie and Chris.  As Lil's illness increases in its anger as it is now, they have their own ways of showing their anxiety and with the trip to the NIH coming up they have even more to worry about.  Chris gets especially worried about his sister but Nettie told me last night that the next house we get needs to have thicker walls so she cannot hear Lil throwing up all night because it makes her sad.  Chris has been building this little tent in the living room the past couple of nights.  Finally I asked my husband Chris what it was and he said Chris told him it is a healing tent.  He is putting a couple of his favorite broken toys in it each night and we are not allowed to take it down.  I am afraid to ask him more about it.  I know him too well.   

The Wonderful Wizard Of Oz

2011

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







2011

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

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

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

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

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

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

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

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


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

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

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

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