Nana Upstairs and Nana Downstairs

Next to knowing absolutely nothing about Lil's disease, the second most frustrating part is watching her ups and downs. 
This past weekend we had the honor of being a part of my cousin's wedding. Yes... all three kids and I were in the wedding.  It was insane and insanely fun.  LillyAnna spent her time at the reception running circles around the dance floor.  For almost two hours she waved her arms, danced to the music and did not stop moving; not even for Poppop.  People kept stopping me and asking very politely but also quite skeptically "now that is the sick one?"  I expected her to wake up the next morning swollen and uncomfortable but once again she showed me up.  I cannot say she was all sunshine and roses the next morning but she ate breakfast!  That is saying something. 

 

The week leading up to the wedding was not a great one for Lil but that happens for her sometimes.  Since switching to the Methotrexate injection 6 weeks ago, she has started getting sores in her mouth.  This could be a side effect of the Methotrexate or it could be a side effect of GI issues.  The mouth sores come and go but there is one on the inside of her left cheek that is fairly consistent.  She was having a lot of trouble sleeping, spiking some fevers and being fussy.  Normal Lil stuff. 



 

Next she was Dance Diva.

 

And then this...

I got these pictures while I was at work Monday night.  Apparently these were not the only areas to swell.  They were very painful for her and not itchy.  The areas where red, hot and had pustules in them.  She did not have any fevers and blood work today looked normal except Sed Rate was 2 although that is not really all that high.  Some of the spots were in old lesion areas and some were in new spots.  Although Chris had very appropriately given her oxycodone and meloxican for her pain, Dr R told us to give her Advil to see if that would reduce the swelling.  This was a surprise to me, I was expecting him to say Benadryl.  I was also fully expecting to be taking a trip to the ED.  When I did get home though the swelling had just gone down slightly.  Dr R and I had discussed that unless she got worse, going to the ED was not going to do much because they would have no idea what to do with her.  He said they would basically just freak out.  I had to laugh.  It is true.  The Emergency Room doctors get so worked up when we go because her case is so unknown to them.  This is in no way an insult to ED nurses or doctors... they are AMAZING!!!! It is just that no one knows this disease as well as I do.  That is the bottom line and it is very scary.  Those lesions went from looking completely normal to looking like that in four hours.  I have not seen that happen since all of this first started.  I was so afraid and that fear has not left me even though Lil's skin is looking much better. 

 

 

She is still getting swollen areas and now there are patches of papules. 

 

There is another patch like this on her butt which is actually worse but I did not want to put it on the internet for obvious reasons. 

                                      

We had her regular pediatrician check her this morning just to make me feel better.  Whenever her disease acts up Lil has some trouble breathing and she sounded raspy and congested.  Dr G said her throat looked raw, her ears had some fluid but her lungs sounded clear.  He also said they could be hives which would suggest some kind of allergic reaction but the more we talked and the more details we went over the more convinced he became that it was not an allergic reaction.  Dr R does not think it is an allergic reaction either.  Dr R thinks it is her disease.  This is not what her disease has ever done before.  Scary.

 

I still had to give her the methotrexate injection tonight and I am terrified.  I will probably not be sleeping much.  I have never seen her disease do this and if the docs are wrong and it is an allergy.... allergy to what?  There is a lot of fear here tonight.  For now I will watch the video monitor, enjoy being warm inside while the snow falls outside and wait for something to happen or something not to happen.  We just never know what is coming next.  It was so much fun watching her run around that dance floor though:)

 

 

I Love You Through and Through

Today Chris and Nettie turned five.  They had a blast all day!  We started the day by making treats for their class, they went to school, registered for Kindergarten took a nap and then had a small party.   Although I used to be the kind of person who was prepared and organized for parties, life as it is now does not lend itself well to best laid plans and as it turned out even though I had prepped a lot ahead of time, working the three nights leading up to a party, a husband who works overtime when it snows and a little one who takes up a good chunk of time does not lend itself well to a properly prepped party.  Therefore as our family and a couple friends was walking in the door to celebrate with us (well some family came early and helped to clean the house and cut veggies) the kids were getting haircuts, I was making mac and cheese, Chris was wrapping presents... it was your basic crazy town.  How were the kids? HAPPY!  How was I?  CALM!  Why was I able to be calm?  Because I am surrounded by the most amazing people in the entire world.  I was even icing cakes while our guests were eating dinner.  I feel badly that I did not throw a better party and that my Aunt Tina, Evelyn and Mommom Susan walked in and were put right to work.  I feel badly that people came basically right from work had to wait a bit to eat and then had to leave and get ready to go right to work the next day but I also feel thankful and safe and these people knew Chris and Nettie deserve to celebrate their birthday and that these people were happy to see Chris and Nettie happy.  So thank you family and friends!  Thank you for accepting us for the way our lives are right now and thank you for loving us!

 

The Phantom Tollbooth

 

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

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


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

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

 

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

 

 

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

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

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

 

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

 

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

 

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

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