Oh The Thinks You Can Think

We decided bad news first right?  Well I guess technically I decided and you do not have much choice so I hope that is ok with you too.  As you have no doubt noticed, Lil has been on a steady decline for a few weeks now.  It is a slow decline and it is not serious enough to hospitalize us but it is a decline nonetheless.  She has been in more and more pain, temperatures that usually hover around 99.8 degrees when her disease acts up have now been steady between 100.6 and 101 then sometimes spiking up to 104 and higher.  She is coughing her "disease cough" which I describe as a croup type cough and vomiting at unusual times of the day besides her normal night time vomit sessions.  Besides the pain, the worst has been the sores on her lips; they just look so uncomfortable, they bleed, they burn and they hurt.  Her lips look terrible and then look completely normal the next day only to be covered in sores again in a few hours.  We do not know why.  I have been hearing the words 'I don't know' out of a lot of doctors' mouths the past couple weeks.  It reminds me of Mr. Hand which at least makes me smile.

The good news?  She is gaining a little weight finally and growing a bit taller.


But... (there is always a but) if the reason for the mouth sores is that they are a side effect of the methotrexate injections we have a problem.  The weight gain is most likely a gift of the methotrexate injections because overall she vomits less since starting the injections.  However if the injections are causing these mouth sores then the benefits may not outweigh the cost.

With the other symptoms we are seeing there is a chance that the mouth sores are a result of the disease instead of a side effect of the medicine.  Here is the issue: Pyoderma Gangrenosum does not attack the lips. Remember there have only been 16 cases of PG in infants.  However there was a case in a child at AI were that child had lesions on the inside of her body.  I do not know if it is called PG at that point.  My concern is that her PG is now attacking the cells inside her body and not only the cells on the outside of her body which is why we are now seeing sores on her lips.  Dr R assures me that PG does not "change lanes of the highway".  My response was "when does Lil's body ever follow the rules"?

Did you like how I sandwiched that good news in the middle?
What are we going to do about all of this?  Experiment.  We are going to give Lil a round of steroids and if her symptoms improve we can assume that she has some type of inflammation in her body.  This makes me want to scream, rip out my hair and feel relieved all at once.  I am glad we have medications at our disposal in this country that have the ability to make my baby feel better because as Dr R likes to point out, no matter who you are a dose of steroids is going to make you feel pretty darn good.  However, it is frustrating to use this as a way to find out if there is inflammation because it is not very accurate and it feels like we are just throwing drugs at her without really knowing why.  AGAIN.  Steroids are not something we take using lightly.

My wonderful cousin is working on creating a button for you to order Lil's t-shirts right here on the blog!   Thank you Ashlie!!!  So keep your eyes peeled for that... Thank you all!!

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!

Good Night

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

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

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

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

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

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

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

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

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

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

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

 

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

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

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

 

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

Update: bed has been christened!! LOL!