Cookie's Week

Things have not gotten much better for Lil since my last update. She has been in a lot of pain and just generally under the weather. Some days have been worse than others. Saturday was a beautiful day for her and I thought maybe we had turned a corner but she woke up the next day logy and uncomfortable once again. Her lips continue to grow large open sores that bleed off and on. Today she has been vomiting all day. Her blood work is consistent with what I am seeing. All of her inflammatory markers at up, not very high but up. We will get blood work again Friday if all of this continues. My heart hurts for her. She is telling us things hurt but as is usual for toddlers, she is not very accurate and so it is very hard for is to help her.

As I have shared with you, we have been asked to speak at AI DuPont's Rare Disease Day. This is a huge deal for us. We need people, as many people as possible, to hear LillyAnna's story. We need to find another child with the same symptoms so we can compare them and their treatments. Being the first of only 4 families to speak on May 19th is a big honor and will gain us some of this much needed attention.
Everyone is always asking us how they can help.  We have decided to sell t-shirts in honor of Lil so you can show your support for her and us but more so we can spread the word about her and her disease. Soon, you will see a link on this site were you can purchase a shirt right through this site! (Hopefully!) For now, if you know us or someone in our immediate family personally, the shirts are $16 each. Our computer is having problems again but should be fixed tomorrow. I am going to add pictures if the shirts, Lil's recent wounds (remember this is for documentation sake also:), and I will proofread it! Lol! Until then... Thank you all so much. We could never live this life without your love and support. When I am at my weakest, I really do think of all of you and it gives me strength.

Quick Update 9

Dr R requested that I take LillyAnna to the pediatrician today because he was working out of town.  The frustrating part of this is that although my pediatrician is absolutely phenomenal, he knows even less about Lil's disease than I do.  So after Dr R gave me some specific instructions for him I packed everyone up and off we went.

It turns out that Lil has an ear infection (our faces lit up as we said in unison "oooh... something to treat!"- PATHETIC!), her tonsils are very swollen and covered in puss but she does not have strep.  This was notable to me because since the mouth sores started about two months ago Chris and I have been examining Lil's mouth and throat each day but we do not really know what to look for.  What Dr G saw today is what we have been seeing every day.  I do not see this as a good sign.  This is also the other reason I am ready to go to Medical School.  Sign. Me. Up.  I am sure they will not mind if my homework is covered in vomit.

It has been determined that the mouth sore looked "minimally abraded" which basically means that it looks like a scrape.  My issue (and frustration) with this is that is the explanation for all of her mouth sores.  It is just not possible that all of a sudden she has gotten random scrapes on the inside of her mouth in the last two months when she never had them before and that she has gotten this many of them.

The other piece of this puzzle that gnaws at me is that when Lil was diagnosed with Pyoderma Gangrenosum there was a doctor dealing with a case of it at AI in a child but it was attacking the child's internal organs instead of the skin.  We determined at the time that this was not happening to Lil because her interleukin profile was different than that case and the drug Anakinra actually created lesions instead of making her current lesions better.  My case right now is that maybe her body is now attacking her internal tissue.  Dr R says this is not likely because they "don't switch lanes of the highway".  (Very cute Dr R).  My problem with that is when does Lil EVER follow the rules?  So Dr R and I will have to have a little chat on Monday.  It is a good thing we respect each other- a lot.  It is one of the reasons I stick with him and one of the reasons I think he is such a phenomenal doctor.

No energy to proofread tonight- I was right about last night:)

Boa Constrictor

Boa Constrictor by Shel Silverstein

Oh, I'm being eaten
By a boa constrictor,
A boa constrictor,
A boa constrictor,
I'm being eaten by a boa constrictor,
And I don't like it--one bit.
Well, what do you know?
It's nibblin' my toe.
Oh, gee,
It's up to my knee.
Oh my,
It's up to my thigh.
Oh, fiddle,
It's up to my middle.
Oh, heck,
It's up to my neck.
Oh, dread,
It's upmmmmmmmmmmffffffffff . . .

Today it seemed as if Lil's little body was being eaten by a Boa Constrictor.  This morning she woke up very uncomfortable and crying.  I was not surprised to find that she had a fairly high fever to start the day and then she promptly fell asleep as soon as we dropped the twins off at school.  As she does not normally even nap during the day anymore ( I think this has something to do with her meds), it was highly unusual for me to walk into the living room and find this scene (look to the left) at 9:15am although not unusual these days was that Frozen was on TV!  When she woke up she was shaky and saying she had a lot of pain and that is basically how the rest of the day went.  As it goes with Lil, the best thing to do when she does not feel well is to distract her so I packed the kids into the family truckster and we drove to Aunt Tina's for lunch.  Lil was mostly ok but continued to spike low fevers throughout the day, complain of pain and have trouble controlling her bladder.  I am not sure if this is new or not because she is so new to being fully potty trained but after having seven days of no accidents to having 6 accidents in a five hour period today I would say that is something to note.  Small pustules popped up randomly on her skin but would go away just as quickly.

Most concerning at this point (besides her increased discomfort) are her mouth sores.  As you know, mouth sores have been coming and going in Lil's mouth looking like small pustules.  Sometimes they seem to bother her very much and other times not so much.  At this point there is a sore that has been growing on her lip that has been bothering her a lot.  As Dr R likes to remind me "we cannot have breakdown of her tissue" as we have learned her body responds by basically attacking it.  So I will be contacting him tomorrow.  This sore is really bothering her to the point where she does not want to eat because it hurts.  

I wanted to keep you all updated.  There is more I want to tell you.... our greatest supporters. But I am very very tired and I have a feeling tonight is going to be a long one for me.  It took our baby girl a long time  (and some hard core drugs) to get comfortable enough to get to sleep tonight.  Those kinds of drugs do not last long and we have to be conservative using them or they make the vomiting worse which is hard to imagine but possible all the same.  I do want to say that I had to call out of work for the first time tonight because of LillyAnna's illness.  I know family comes first but this is not something I am ever comfortable doing.  My Redfire family rallied around me today and for that I am so grateful.  Thank you guys:)

***I also have to give a shout out to my Aunt Sue who used to sing the Boa Constrictor Song to me when I was a little girl... fun memories Aunt Sue!   And now I sing it to my little ones... so much fun.

Love, Stargirl

It has been awhile... I know!  Our computer has been having so many problems and I am learning that getting your computer fixed is like getting your car fixed; if you do not know what you are talking about you get taken for a ride!  We have spent an obscene amount of money (ok maybe not obscene but a lot) trying to fix our computer only to find out that the problems were not being fixed at all.  Finally a very very kind friend offered to fix the computer for us and did it over one night.  We are going to try in vain to repay him and his family with food and baked goods:)



And now I have so much to share with you, it is hard to know where to start so for this morning I am going to update you on Lil and leave some of the deep stuff for a late night and a glass of wine.

I could not leave this out, it was too cute:)

Are you the type of person that like good news first or bad news first?  I have always been the type that likes to get the bad news out of the way and end on a good note so here it goes:

Lil is having a very difficult week.  She has not been feeling well and the very sad part is that it hardly even affects us anymore.  When people ask how she is I can say, it is her normal stuff and they know exactly what I mean.  I wonder at what point that will stop bothering me.  She has been in a lot of pain and as usual it is very difficult to figure out the source of her pain and with her demeanor it is hard to determine the extent of her pain.  We can usually distract her from it pretty easily but this week it has even been hard to do that.  She has developed some papules and pustules but nothing extreme.  The pustules did surprise me as she just had her doses of Humira and Methotrexate so this should have been a good time for her.  She tells us that her boo boos hurt and points to small cuts or papules that cannot possibly be that painful.  However from what I have been reading and discussing with the doctors, this is how toddlers are notoriously bad at describing their pain.  She has also been saying that her belly hurts but this is new.  With all of her vomiting and not eating, she has never said her belly hurt before.  This week she would actually grab her belly and scream that her belly was hurting.  After a week of sleepless nights for her and I (she was just too uncomfortable to sleep) I gave in and started giving her doses of oxycodone on top of her other pain meds at night and if I time it just right I can get it to settle in her stomach before the vomiting begins.  At least I think that is what is happening and I have my reasons for thinking that but they are too gruesome to share even here!  And then there are the mom instincts... it is in the eyes.  I can just see it in her eyes.  She does not look right.

Going to AI is like Christmas!

Her blood work confirms my gut feelings.  White Blood Cell count is slightly elevated, CRP is 1.6 which is decently high for her right now, Sed Rate is 8 which is not too bad but high for her at this time.  All of this shows us that something is going on and as usual we just do not know what.  I am most concerned that some of her other numbers are elevated and these are related to her liver.  They are higher than I have ever seen them (double than the top of the normal range).  Dr R says they are high but not high enough that he is concerned.  This is the rub for me. I feel like we fall into this grey area in the medical field where a typical pediatrician would have one opinion and a hospitalist (especially one like Dr R who we like to call House because his group sees some of the most rare and severe cases in the hospital) has a completely different perspective and with it a different opinion.  So as a mom in this grey area I am worried.

Hands red and swollen

But then we go to the mall and she carries the bags and eats ice cream and plays and well... there you have it.  We stay out of the hospital.  If we can control her pain at home and she stays hydrated we stay home.

Now good news.... and there are two things.

One: I have learned a lesson.  You will probably think I am slow but this whole thing has been a bit overwhelming and I am going to take the excuse that trauma has significantly slowed my learning curve!  Lucy's funeral was horrible and beautiful all at once.  It was the first funeral I have been to for a child.  Her mother honored her life, death, suffering and family so gorgeously.    And on a personal level it put some things in perspective for me.  One of those things is that I cannot let Lil's disease stop us from living life.  With LillyAnna that can be tough because if she does too much or pushes too hard she pays for it with pain.  Chris and I always have to make the impossible decision of guessing whether or not some activity is going to affect her (and believe me it is a GUESS) and how badly and whether or not it is worth it.  Impossible decisions.  One decision we made was to take her tubing.  All I could think was ' if she gets hurt Dr R is going to say... You did WHAT?' but we did it and she had sooo much fun!  Did she pay for it?  Yes.  But I would say this one was worth it.

Two: the other piece of good news is that we have been asked to present LillyAnna's case at AI DuPont Hospital for Children's Rare Disease Day on May 19th.  There are only four families being highlighted so I feel this is quite an honor and hopefully it will bring us some much needed recognition.  In honor of LillyAnna for this event we will be selling t-shirts and sweatshirts.  Our hopes are to continue to spread the word about Lil and her disease.  We will be ready to begin to sell hopefully by the end of next week.  I will keep you all updated and let you know how to purchase a t-shirt if you are interested.  We are so excited!!!! And thankful to our friends from AI for this wonderful opportunity!

Someone learned how to take their own medicine!