The Paper Bag Princess

I think I have told you I am the kind of person who likes to watch movies over and over and over.  Well, I am stuck on one right now.  A line from that movie struck me the other day.  The main character, talking about his estranged wife, says, "Yeah, she wants to change me. And I want to change her.  People want to change each other.  That's normal."  I turned that idea around in my mind for days and then I started to feel very guilty.

I do not want to change anyone in my life.  I do not want to change anything about my life, not one blessed thing.  Let me be blunt.  It is excruciating to watch my beautiful, innocent baby live in constant pain.  It brings me to my knees and literally breaks my heart. Just to let her enjoy the holidays I had to give her extra pain medication.  When people told me she looked good, I did not even argue, I just smiled. My love then meant putting myself aside and making sure she lived life.  True love comes in so many forms. When you have to watch someone you are in love with suffer there are just no words for it.  And I am so in love with my kids.

But here is the thing: this is her story.  The people in our lives have their own stories and hers is still being written.  An insightful friend reminded me of that this holiday season.  I have no power to take that away from her and I have no desire to take that away from her.  Now you see where the guilt comes in; how can I not want my daughter to be pain free, to be free of this disease?  But I don't.  I feel no anger and I feel no regret.  I have my moments, don't get me wrong.  There are days when I just want to hit things or scream at the top of my lungs when she needs me to color for her because she cannot hold her crayons or when she asks when she will be able to go to school. Most days though it is just not there.  We are shaped by our experiences, good and bad; gorgeous and horrific.  We all have them because all pain is relative to all of our other experiences.  When people compare their lives to mine and say things like 'but you, you really have it hard.' I just shake my head and say no, it is all relative and I mean it.

Talk about true love

Same thing goes for my husband.  I love him exactly the way he is.  We do not have much in common.  Nowadays we do not spend much time together. My grandmother was dying from mesothelioma when I met my now husband.  I had only been dating him a few weeks but she was certain he was "the one".  She was a practical woman when it came to romance not having gotten married herself until she was 26 years old.  Her marriage advice?  Whatever you do not like about him now you will hate about him in 10 years so make sure you can live with it.  Chris, sorry, but she was right.  But guess what?  I do not want him to change one bit.  Nothing.  I love him just as he is but I also accept that loving him means he will grow and change as he wants to. 

Today a wonderful writer published a beautiful story about me and my family.  Chris and I were brought to tears by her ability to succinctly and warmly put our story to print.  Reflecting on our story in this way on New Years Eve with my dad's ruminations in the background has made me more pensive than I would like to be right now.  LillyAnna is not good.  The pustules in her mouth are worse.  Her top lip is swollen, both lips are bleeding.  It does not help that she is picking at them constantly.  You can imagine that eating is not going well.  She is running fevers and has a terrible rash on her bottom.  There is a nasty pustule on her cheek.  My biggest concern is her blood work right now.  CRP and Sed Rate are higher than they have been since March.  This concerns me mostly because now we have PG symptoms on the tissue inside her body.  I will be contacting the NIH Friday morning.  Why not go to the ED?  Why not call Dr. R?  What can they do?  The answer is nothing.  That is the scariest part of my life.  I am in charge most of the time.  The only thing we may do is put her on some steroids and we cannot do that until we get a set of blood work that the NIH requested with her next flare in which I am fairly confident we are safely ensconced.

In this New Year I pray I can guide us all through this journey safely and with confidence and in love.  We are all writing our own stories.  Write them well.

Since being inpatient she generally dislikes the visiting dogs but she LOVED Chase today.  He actually sat next to her while they took her blood.  MORE guilt since the kids REALLY want a pet after loosing our black lab this summer:(

Plus... Do not miss us in the News Journal!  www.delawareonline.com

Moxie and the Art of Rule Breaking

 

Since my last post Lil has been feeling much better.  I had written a post to that effect but it was erased by the great computer gods.  Since then she has had four amazing days in a row.  We could not ask for a more amazing Christmas gift.   She was practically bouncing off the walls with excitement.  To watch her have virtually no pain, not vomit, eat like a normal kid and wake up being able to walk for four whole days was just... well to be honest fun!  And a little exhausting because I just wanted to soak it all up.  The last time I saw her have that many days pain free was this summer. 

The only blot on these days was that she had a spot on the inside of her lip that looked like a full blown lesion.  It was only there for a few days.  It did not seem to bother her too much.  And then a few days ago I found a pustule on the roof of her mouth.  This one hurts and is causing her not to eat.  It was so bad yesterday that she drank almost a full gallon of milk in place of eating.  There are a lot of problems with this substitution. It will cause her iron numbers to drop drastically and they are already low.  But I cannot force her to eat, the pustule looks increasingly worse.  It is becoming ulcerated on one side.  Today it is actually bleeding on the side closest to her teeth.  It is very hard to get a picture of, I am sure you can imagine. 

This is the original pustule. 

This new development is terrifying.  To make it simple: the tissue that makes up the inside of your body is different than the tissue that makes up the outside of your body.  If her Pyoderma Gangrenosum is starting to attack the tissue inside of her body that could be significant.  This is also the largest pustule she has had in a long time.  And of course it all started again yesterday... she was limping around the house, she threw up before bed, waking up in the middle of the night.  I contacted the NIH to see what their advice is, we will see what they say.  Dr. R suggested putting Maalox on it.  Her wonderful Aunt CoCo made a cameo appearance yesterday and she was SO excited.  These kinds of visits, seeing friends and family literally keep her going. 

There are so many different ways people support us.  But it is the way people support LillyAnna specifically that I would like to focus on today.  You would think that she is only three so how much can she possibly remember or know.  What you have to remember is that she is no normal three year old.  No chronically ill child has a normal childhood and neither do their siblings.  LillyAnna depends heavily on her support system.  She cannot go to any kind of preschool; her school, her life, is her family and our close friends.  You mean the world to her; you who are kind to her.  You all have to know that she sings about you in the moments before she goes to sleep, she talks about you and laughs about you!  She draws pictures for you and dances for you when she can.  Those of you who are kind to her, who give of yourselves to her, she does not forget you.  When Lil does not remember your name (which she usually does) she gives you little pet names; my friend, the one with the books, the chocolate lady... And she wants to share things with you.  You literally make her days brighter. She remembers everything.  She remembers watching Sara Spicer sing "Something Bad", sings it all the time and when she sees her thinks Sara Spicer is her personal friend.  That is how Lil is.  She thinks Nancy Johnson from WSTW is speaking directly to her when we hear her on the radio because we met her at the Nemuors radiothon. The thing is, these people were also very drawn to Lil.  She is a force. People fall in love with her because she is one special young lady.  I have to, have to believe God put her here in this place for a reason.  She is going to do something great with this disease of hers.  She told her Nonni once that her scars were beautiful.  No one ever said that to her.  She came up with that herself.  You all make her days brighter, I am saying it again.  Do not doubt it.  Your smiles, your hugs, your attempts to make her smile.  She remembers it all and I just simply cannot thank you enough. 

Quick Update 12

 

So Lil is not feeling much better.  I would say she is worse since this weekend.  The general pediatrician says it is just a cold.  Unfortunately for Lil, a small cold can quickly turn into much, much more so Chris and I are on watch.  The past two days all she has done is lay on the couch and sleep.  She is going on 6 days of fever running between 101 and 103.  I suspect it is going much higher but I do not have the heart to take it rectally when she feels this awful and there is no purpose to it.  We are not going to take her to the ED unless she is dehydrated or labored in her breathing, or if I feel like that fever is so high she is at risk of seizure. Do I feel like we are getting close?  Maybe.  I was up most of the night last night with her.  I had two full days of work this week that I just had to do.  It was too much money to miss out on and it was important to the future of my job... this is reality.  My aunt took very good care of her yesterday but Chris is going to stay home with her today.  We need to make sure she is stable.  I am thankful she is drinking.  If not, we would definitely be at AI.  I have off Thursday and Friday and I am thankful for that also.  I am thankful for all of the support we have at this time.  Yesterday was a comedy of errors for me.  Everything that could go wrong did, now I have been up for nights in a row.  Laundry is building up and so is my stress level.  Little Chris is feeling that something is not right and acting out again.  I am watching our friends going in and out of the hospital and I am thankful we are not there.  I feel like 'shouldn't this be easier?' but it is not.  And we talk about that in our group too.  This is when chronic illness gets really hard. 

*No time to proofread... sorry!  I have to get ready to teach today:) 

Nest

 

Lately I have been reading the signs. Not street signs or any other but our kind of signs; grey hospital bins rotating from Lil's room to the dishwasher to be washed back to the steps to be taken upstairs, small bundles of laundry wrapped with a hospital pad moving from the top of the steps to the laundry room, opened alcohol wipes piling up in the trash can...  would I call this a flare?  Not exactly.  One of the things Dr. R and I discussed at our appointment this week was what constitutes a flare but we were interrupted by Lil who was insisting quite fervently that Dr. R color his portion of the snowman picture.  We did manage to agree that between all of Lil's symptoms, for it to be a true flare she should have most of them going on at the same time.  Mostly we agreed on this because I said she has one or two of her symptoms at anytime.  What is really frustrating is that through most of the summer, she was doing so well.  Then Fall hit and so did that huge flare. She is not bouncing back from it the way we expect.  Here blood work backs me up on this point.  The sed rate is just not going back down.  Sed rate is an inflammatory marker that rises and falls more slowly than other inflammatory markers.  Dr. R and I have been frustrated over why blood work has not been showing rises in CRP and WBC when she is symptomatic (these are other inflammatory markers).  The NIH said this is not unusual in Pyoderma Gangrenosum patients. 

My question, and it does not have an answer, is why this is happening when we just increased her Methotrexate yet again.  We should be seeing a decrease in her inflammatory markers, symptoms and flares not an increase.  This morning when she woke up she was limping so badly she could hardly walk.  Her fingers were so swollen yesterday she could not put together a big piece puzzle.  Her lips are ulcerated to the point that it hurts to eat.  I feel like I am waiting for the other shoe to drop.  If we are going through meds at this rate now, at three years old, what does the future hold?  But, she is still Lil.

We went to get our tree and she had a good time singing Christmas Carols.  Aunt CoCo and Aunt LuLu took her to Chuck E Cheese and she had a blast!  But we did one thing per day.  Each day ended with a fever and vomiting.  Today she has not gotten off the couch.

I read a beautiful article describing what it is like to live with a chronic disease.  In this case it was Lupus which Lil has been tested for and does not have but the symptoms are similar.  You all do so much for us but I am going to ask you to do one more thing and that is to read that article.  The link is posted here:  http://www.butyoudontlooksick.com/articles/written-by-christine/the-spoon-theory/  She compares having a chronic illness as a give and take.  You have to make decisions every day of what you are going to give up and what are going to get to do and what the consequences are going to be.  The added dynamic for Chris and I is that we have to make these decisions for Lil and observe the consequences from afar.   When we have  pushed her too far because of our own agendas or for the twins, we watch her pay the price. 

Her blood work looks just terrible.  Iron is low, liver numbers are up, we already went over inflammatory markers and a whole bunch of other stuff was flagged that I have never seen flagged before.  Part of the problem is that Lil is refusing to take her meds.  We are working on it.  Of course now she is throwing up every night anyway so it does not matter much but we are going to try giving meds earlier in the evening... we will see.  All I can think is that the fight will happen earlier and last longer.  But I am going to have a good ATTITUDE!  I WILL!  I am smiling right now!  I promise:)

Dr. R took his accolades from the NIH well.  They were so impressed that he diagnosed Lil as quickly as he did.  He tried to hide how proud he was but I could tell.  He should be proud.  He diagnosed a disease that only a handful of other kids have ever reported in the world.  That is pretty amazing.  And as we are talking about it he is coloring a picture with my baby.  He is just wonderful.

 

Every time I think I have accepted that this is going to be her life I realize that I have not accepted it, that a part of me still hopes and still wishes that she is going to get better.  That she will not have to make choices as simple as 'choosing not to run errands, so that she can eat dinner at night'.  No, as I read that it is not right.  A part of me still expects that she is going to get better.  That is what we do as moms.  We make our kids feel better.  We make them soup, get them a tissue, hold them, sing them a song, give them a band aid, give them a treat... We make it better.  And I can't.  I will never be able to make it better.  It is a pain in me that I just cannot explain. 

The Littlest Elf

LillyAnna very rarely leaves AI DuPont Hospital empty handed.  If she has a procedure, Childlife always visits to make the ouchies less ouchy with a toy or activity.  When we are inpatient, toys always seem to show up in her bed.  Forget being inpatient during the holidays... I don't even want to talk about it.  Even when Lil gets blood work her 'girlfriends' save the ends of the rolls of coflex in bags and give them to her because they know how much she loves to play with them.  This is all in addition to the stacks and stacks of stickers. the whirligigs and, if her grandparents have anything to do with it, balloons.  You would think LillyAnna would be spoiled.  You would think when I ask her what she wants for Christmas she would have a list a mile long. 

In this, as in so many things, this beautiful little creature has just blown me away recently and I could not help but share.  She never asks for anything for herself.  Even if she has candy or a lollipop, she will offer it to her brother or sister first.  Oh wait!  The only thing she does not share is the blue bag of Doritos!   I just cannot get over how generous and kind she is, how she is the happy kid in our house, she is the pleasant one.... even when, like today, she was limping around the house crying because her legs hurt so badly.  I literally cannot get this kid to ask for more than three Christmas gifts.  Now, we are not getting the kids many gifts anyway but as I said, I am just blown away.  She is just amazing and I had to share.  Believe me, I am not bragging.  The other two are screaming, "Mom!  I want that!"  every other second:)