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:)

Thanksgiving is...

I created this blog for two reasons.  The first was to be able to tell about Lil's disease in detail and what it is like to be her mom without the pressures of facebook.  The other was to spread the word about her symptoms in the hopes of helping people who may be experiencing something similar or possibly even help Lil.  Unfortunately for all of you, the side effect of that is you hear an awful lot of negative things about our lives.  You do not often hear about how grateful I am for all that we have. 

I know how very lucky we are that LillyAnna can run and play, that she is smart and can think on her own.  The physical therapists we worked with at AI were absolutely astonished she began walking during her time there with the excruciating pain she experienced in her legs but she did and she can still walk.  She can eat, drink, walk, and breath.  My favorite is that she can play.  She loves to play.  She pays for it the next day or like today she paid for it tonight either by vomiting or having a lot of pain.  But this girl can play.  And we would never live this life without our support system.  We would not be here without our doctors and nurses and hospital staff.  We have a house, we have heat, we have money to buy food.  We have all of you. 

Then there are the little things.  The smell of sautéing garlic, my kids hair when they wake up in the morning and roses.  The feeling of jumping into a pool on a hot day, sand in your toes, the memory of my grandmother.  Reading.  Cucumbers.  Cuddles.  Laughing with your best friend. 

I know how lucky we are, how lucky Lil is.  Sometimes I need reminding of how good we have it and sometimes I need reminding of how hard we work.  Depends on the day.  Today I enjoyed the day, enjoyed the cuddles and my family... oh and turkey too:) 

Absolutely Almost

It took the nurses and social workers at AI a few months of kind cajoling to get Chris and I to attend our first support group meeting.  I kept thinking , and telling them, that we did not belong there.  I knew that so many people who went to that group had kids who are in wheelchairs or were as medically complex as our beautiful friend Lucy.  But the more groups we attended the more I realized that we did belong there. I realized that Lil's pain, her medical issues and how it affects our family put us right in the mix.  Last night at our meeting, a wonderful new family to our group leaned over to me at the end and whispered, "I feel like maybe we don't belong here.  She is not as complex as kids like yours."  I started with a smile and proceeded to tell her why she too belongs here. 

I truly believe we, our little family, is here in this place for a reason. 

So how is Lil?  We are a little perplexed to be honest.  She is not coming out of this flare as quickly as we thought she would, as quickly as she has come out of flares before.  This is especially strange because we just increased her Methotrexate.  If you remember, the docs at the NIH noted that the Methotrexate had more of an effect than the Humira.  So has the methotrexate hit its plateau? 

The NIH also asked us to get more blood work in their concern for her kidney and liver numbers.  Those numbers were still elevated.  They are not very high but elevated and staying elevated.  My concern is that she is saying she has pain in her belly and side and those numbers are elevated. 

She is limping when she wakes up telling me she has pain in her legs and hips.  This usually works itself out in an hour or so but it should be controlled with the increase in the methotrexate.  In reading the Facebook page on Pyoderma Gangrenosum I am learning so much about the pain that comes along with this disease.  The adults that have the disease say that even loud noises and vibrations can cause the "PG" pain to act up.  Lil has been having these crazy temper tantrums, it takes almost nothing to set her off.  I am wondering if she is just walking the line of keeping herself under control between having some level of pain and because she is three that when she loses it, she really loses it.  Little Chris had some serious temper tantrums at three but Lil is putting him to shame.  She does not have them often but they come out of nowhere and they are major and then she falls right to sleep. 

The other piece of the puzzle is that she is out and out refusing, and I mean refusing to take her medications.  It started about two months ago and I remember we were in New York visiting my friend Alana.  I was fighting with Lil to take her meds and Alana asked why I thought she was fighting so hard. My response was that I think she is just tired of it all.  I mean think about how you feel after being sick for a week or two.  For over two years, every night she has taken 4-8 vials of oral medication some of which tastes terrible no matter how they flavor it.  There is nothing she eats consistently enough that I can put it in food.  There are still days she just simply does not eat.  I reached out to the docs, our wonderful nurse friends... We are spending 2 hours a night trying to convince her to take the meds only to have to sit on her and squeeze her already ulcerated lips and cheeks so I can get the syringe to the back of her cheek which leaves me and her crying.  The best part?  She throws it all up in the end.  So I have stopped.  Thursday night we had a breakthrough!  I filled a couple of syringes with Gatorade and gave them to the twins and they all took 'medicine' together.  She took 3 of 5 with no fussing.  I was all smiles.  Before bed, she threw up anyway.  I just CAN NOT WIN!  My only consolation is that maybe she would have been throwing up every night anyway. 

Overall she is not as bad as she was before the NIH but she is not 100%.  It is just so frustrating to see this continue to go on and on and on.  The twins know she is better than before but they also hear us fight with her every night, they lay in their beds in the dark and hear her throw up.  They watch her run around and cough and us grab a bin.  I am so thankful for their school though.  Chris flat out told me he feels very safe with his teachers.  Both of their teachers told me how the twins are able to talk about Lil in school.  This is so huge for Chris.  Nettie is much better at talking about her feelings. 

We also had a talk about how the Congressional Visit went while I was at the NIH.  Here is what I have learned.  Families with kids with rare chronic illnesses have to do crazy things to take care of their kids.  They sell their homes and buy smaller ones.  They get divorced and sometimes actually have to live apart for a time to prove it.  When we applied for SSI the social worker was so touched by our story she was in tears.  Very kindly she told us if Chris could figure out how to lose his job she could help us because he makes too much money.  Too much money is over $25,000 a year.  We are supposed to support a family of 5, one who is medically complex, on $25,000 a year?!?!  I am glad Congress wants to help but it is going to take some serious legislation to help and I am worried that there are just not enough of us to make a change.  Someone mentioned they heard that there was going to be a change in that we would be allowed to have a savings account for our medically complex kids that would not affect their Medicaid.  Wahoo!  Thanks a lot (insert sarcasm).  One of the new things I have been reading about on the PG Facebook page is adults having to get amputations.  Once again, I have no idea what the future holds for Lil. 

The reason Chris and I have been able to keep living the life we live is you.  I am not blowing smoke here.  I am being totally serious.  We have actually talked about having to sell the house and downsize.  Or move to an area where houses cost less.  I just cannot work as much when she is very sick.  Is this fair?  I don't know.  Should we have a donation page?  I don't know.  It is something Chris and I talk about all the time.  How we spend money, how careful we are is a constant conversation in our house. I do not know what is going to happen but I can tell you we are doing the best we can to be responsible and we appreciate you so much.  You have kept life stable and normal for our kids, you have given our kids the little things that make their lives better, healthier.  I see how the government helps other people.  Should they be helping us? The conversation we had last night with others in our position was wonderful.  All we can do is keep pushing. 

I love how she is just chillin' talking to her aunt in this picture!  Aunt CoCo is trying to convince her to take her meds and Lil is calmly telling her all the reasons she does not want to!

All About Me

I love music, all kinds.  I love opera (although I have not been able to go in years), I love classical, I love blues, and I love old school rap. Finding this new radio station that plays all old school rap that I listened to in high school has been a goldmine.  The other day I was driving alone in the car and a song came on that brought out my inner Snoop and before I knew it was bee bopping along singing my heart out.  And then it was like someone threw a cold bucket of ice water on my head and I stopped. 


Chris has been really worried about me.  He says I am miserable.  I just stare off into space and I cannot relax.  He is right.  We have been blaming it on my headaches but actually my headaches have been slightly better.  So what is it?  What has me crying in parking lots and unable to sleep?  Why can't I enjoy a song in the car? Why am I at a kids' birthday party staring out the window?  I do nothing for myself.  I do not go out with my friends...ever.  I don't go to yoga or even spend time with my husband.  If Lil is starting to feel better, then why?

All I want to do is be with her.  Right now she is what makes me happy.  Yes, the twins make me happy.  Chris, when you read this, I am sorry.  I love you.  Although I do not want to compare Lil to a small sweet child who is dying I remember once when Lucy was dying I was sitting in the room talking to Nicole, her mom.  We were talking about how everyone kept telling her to eat.  She just looked in my eyes and said "Eat?" and  we both shared a moment of understanding.  That understanding was not necessarily about a dying child.  For me it was about a child in pain.  When your baby, your baby who you are supposed to protect and care for and advocate for and take care of, that helpless child with her still soft and chubby arms and feet, and kissable cheeks, who likes to cuddles and have blankets is in pain you do not want to do anything for yourself.  You just do not care.  That whole thing about 'if you don't take care of yourself you can't take care of her' is bullshit.  When she can't eat for days at a time because her belly hurts so much you do not want to eat.  When all she wants to do is sit and watch movies because her hips hurt, you want to carry her around.  My little girl can't wear socks some days because it hurts too much.  Socks hurt too much.  As a mother how am I supposed to allow myself to enjoy anything when my baby's feet hurt so much that she cannot wear socks?  Or her mouth, from ulcers, hurts so much she cannot eat? 

How can I feel ok about who I am as a person when I then have to sit on that beautiful baby and squeeze her cheeks while she screams that I am hurting her to get her to take 4-7 vials of medicine every night only to watch to her vomit it all up.  I have tried everything else, I am as gentle as I can be, I have reached out to the doctor and our nurses.  I can't stop thinking about it and I hate myself for not being able to figure it out.

My mother in law has recently helped us find a Pyoderma Gangrenosum Facebook group.  Chris is very concerned about me reading it.  The problem is, and he agrees with this, we are learning so much.  When these adults describe their pain and daily struggles, we know how Lil feels.  For example, she will sometimes say her scars hurt.  I always felt torn by that when there are no open wounds on them.  Why would they hurt when they look so good?  Until the other day when I found a post on this group from a person who described what his scars, which are 4 years old feel like.  Here are some phrases from him and comments from him and his fellow PG sufferers:  intense, insane, life changing pain, exploding from the inside out, pliers ripping my toes off, searing hot knife constantly slashing my flesh, vice grip constantly gripping my foot, red hot ants trying to chew their way out of my flesh, pain never goes away, issues depending on movement and how clothes affect my skin, hurts now as much as it did when it was open.  Many comments just simply agreed with the descriptions and talked about pain killer options.  These terms have been running through my brain like a continual recording. 

And so, is there emotional energy leftover for Chris and Nettie?  How about my husband?  Where do I fall in the hierarchy?  Exactly...you do the math. When people compare their lives to mine mentioning that mine is more difficult I always say everything is relative and I mean it.  However, in this case I have to say only a few moms can understand what I mean.  Moms who have seen their kids in pain know what I mean but only to a certain extent.  Moms who have watched their kids live with pain over long periods of time, who have had people disbelieve their pain, who have had to fight for them day in and day out.  These moms can truly sympathize with the depths of my pain and sorrow right now. 

So why am I sharing this self pity with you?  I am usually pretty good at dealing with things while they are going on; I pull on my big girl boots, lace them up tight and do what has to be done.  But when things start to calm down, that is when my strength wears thin.  It is always after she starts to feel better that I fall apart.  Don't worry.  My pendulum will fall back to middle, it is just a matter of time. 

Have You Seen My Dragon?

It has taken me a few days to process everything we saw and heard.  One thing everyone has to understand is that we are not going to have answers from this trip and we may never have answers.  Another thing is that I know it is hard for those of you who are close to us to get 'the story' from the blog but at least if you read the blog you have the base story and we can discuss the rest with you.  As Chris likes to put it, "Even I read the blog!"  

I am going to give you the details I feel are necessary for you to get the big picture.  If you have questions I am happy to answer them!  There is a comment section on the blog that I am not very good at utilizing but I would like to get better at using.  We can also use Facebook.  Those of you who are close family members, we will talk about it, of course!

The NIH is doing a clinical research study on a group of auto-inflammatory diseases.  Pyoderma Gangrenosum is the process of an auto-inflammatory disease.  It is not actually a stand alone disease.  That is why it is so strange in infants.  In adults it is the process of, or it comes along with, Chrons or Ulcerative Colitis or something like that but in infants it comes without an underlying disease.  There is no prognosis for Lil's disease. 

On top of this, Lil's case is extremely unusual as I understand it.  I told you before, as the doctors were looking at her wounds they used words like extraordinary, amazing, awesome, unusual.  When you are in a place like the NIH where they have seen and treated multiple cases of adult PG and you hear this... it is not good.  Through the fundraiser my mother in law set up (thank you thank you thank you!) we have finally found another case of Infantile PG.  I have not contacted the family yet but I plan to this week.  They just returned from the NIH.  However, Lil's disease does not look like that child's.  In fact, her disease does not look like any other case they have seen.  The angel doc said they have seen one case that looks a little bit like hers but she is their outlier. 

They want to know why the Humira did not have as much of an impact on her disease as the methotrexate.  Methotrexate is definitely effecting her disease more.  We just increased her methotrexate again so they want to see what is going to happen.   If her flares reduce they will see us in one year.  If they do not, they will see us in six months.  There is one caveat to that scenario. 

LillyAnna's original blood work came back with double negative T cells, a very high number of them.  What does this mean?  Well, this is what has taken me so long to research.  The first response would be to assume she has Alps disease which is a horrible disease.  She DOES NOT have Alps disease.  That leaves the question of why the high number of double negative T cells? 

Extremely simplified:  T cells are made in your bone marrow and are an integral part of the inflammatory response.  Starts to make sense.  Big Picture?  I still do not know.  I have made an appointment with Dr. R to discuss the trip and these results.  At one point we had talked about a bone marrow transplant with Lil.  But as Dr. R very bluntly put it "bone marrow transplants can kill kids" and we were not at a point that Lil was that bad.  By the way, I love that Dr. R is that blunt.  I need that.  No bullshit.  The angel doc is not even close to recommending that either.  Before we left she ran more blood work using Chris as the control.  Dr. R also has some data on this from Lil's previous  hospital stays.  Basically we are still looking into it.  But it is the big white elephant in my brain.

Lil's liver levels are still elevated.  This is common immediately after she gets her medication.  However, we took this blood work three days after her methotrexate and Humira so the levels should have gone down.  This is the second time this has happened this month.  There is some evidence here that her liver is showing that it is unable to handle the amount of medication we are giving it.  What happens next?  I do not know.  I could not ask.  It is one of those 'we will cross that bridge when we come to it' issues.  We will get blood work again in a couple of weeks.  Unfortunately we just increased the methotrexate and that is the medication that is having the biggest impact.  It is also the medication that is most likely causing the liver dysfunction.

She does not have Lupus!  Good news!

Iron is consistently low enough that the angel doc wants us to give her iron supplements daily.  Have you ever smelled liquid iron supplements?  They taste and smell like pennies.  I can't even get her to eat some days....

One thing that has been gnawing at me, Dr. R and Dr. Brescia is that her inflammatory markers do not go up when she has a flare.  The angel doc told us this is normal for PG.  It made me feel so much less crazy!!!!!

I met Lucy's mom in the laundry room at AI.  We started talking and she began to lead me along this yellow brick road of having a chronically ill child and raising a family at the same time.  While in the play room at the Children's Inn at the NIH I met a lovely woman who was at the same place I was when I met Lucy's mom.  I was able to begin her walk down that yellow brick road and she thanked me.  She seemed so much more calm when I said good bye.  Her boys where so beautiful.  I hope I did Lucy proud.  Lucy's life had so much meaning for so many people but this is Lucy's legacy for me, to be able to help families who are just beginning this journey the way that Lucy's mom helped me.
We are doing it Lucy:) 

Just Me and My Little Sister

I remember when Lil was born and we used to read the book Just Me and My Little Sister.  My favorite part was when they go to the park and the little girl (the new baby) falls off the slide, she gets muddy and miserable and cries.  Her older brother picks her up, quickly soothes her and takes her home.  Chris always loved this part of the book when Lil was a infant.  He often asked when he would be able to take Lil to the park by himself.  These days I have been watching their relationship grow and grow and become more mature.  They are very similar you see. 

They both love to build, they love cars, trucks, and they both love Star Wars.  Lil loves 'girly' stuff too but she is the kind of chick who will run around the house in a princess dress and camouflage hunting boots with a battle ax in one hand, light saber in the other on top of being dressed to the nines in princess jewelry.  Their intelligence runs along the same vein as they will attend to a task until its completion no matter what and they are especially in tune to figuring out puzzles or, more so, the way things work. This is a picture of Chris sorting his candy on his first Halloween- self directed!


Since we have returned from our trip, Chris (little Chris that is) has been an absolute mess.  I have not seen him act out this way since he was two years old.  He has been throwing things, screaming, screeching, crying hysterically at the drop of a hat and all day long.  My little boy is in pain.  Something is wrong.  Chris and I have been pulling our hair out all weekend trying to figure out what to do with him.  Chris is a very emotionally intelligent little boy and because of that he needs to know about Lil's disease but we have to walk a fine line between telling him enough and telling him too much.  Having our five year old friend Lucy die had a profound effect on him.  He talks about her all the time.  We do have therapists that the twins work with at the hospital while Chris and I are working with own support group.  We had a special meeting with them before the trip in which they told me they could have video taped Nettie for the perfect therapy session but Chris was a tough nut to crack!  No surprise there!  After many failed attempts at intervention this weekend father and son went for a long walk in the woods but no go.  Finally last night Chris and I were at the end of our proverbial ropes. 

I was in the bathroom with Bubby (AKA little Chris) and he had just gotten out of the shower, a shower which I had screamed at him through when all I wanted to do was let him wash his little body and wrap him in a warm towel and help him get his little boy pjs on and then snuggle him until his pain went away.  But all he was doing was pushing us away and pushing us away and pushing us away.  On top of all this, I have not gotten to the wrap up post of the NIH visit but there are some things hanging over my head that I am running away from. 

After a lot of yelling I finally got him to give in to cuddling in my lap and trying to talk about what is bothering him and it hit me like a ton of bricks.  I finally blurted out, "Are you afraid she is going to die?"  I felt his body go limp and the racking sobs begin.  We both sat on the steamy warm bathroom floor and cried.  I can't take away his pain and his fear.  I can't tell him she will not die.  Although I can reassure him it is not going to happen anytime soon!  We talked about the beautiful Lucy, we talked about the support group, we talked about Lil and her disease.  We talked about our fears and how much we love each other and how much we love our family.  Then we stood up and walked out of the bathroom. 

When I put him to bed we talked some more and this morning even more.  Have I made progress?  Will we keep talking?  I don't know.  One of our new CCF (support group) friends has a son who is losing his fight with a rare disease at 18 years old.  He was not supposed to live past 8 years.  He has an older sister who went away to college this summer.  His mother shared with me that the sister had an awful summer; she described her as a bear!  Mom could not figure out why and it sounded exactly like my weekend!  Finally his sister screamed, "I don't want to leave ___!!  How will I know how he is doing?  How will I know his levels?  What if I miss something?!?!"  Then she just started crying.

If a college student who has been dealing with this her whole life and has an amazing family does not have the skills to deal with these emotions, how can I expect my five year old to?  Having a chronically ill child is not only about taking care of that child, not even close.  It is about fighting off demons and creating celebrations out of thin air for everyone.  It is about making other people understand those demons and celebrations because your support people become your lifeline.  And sometimes it is about letting go of people who break down the delicate fortress you are building no matter how much you used to love them because if you are not with us, you are against us.  Thank you to all of you who are with us. 

My baby boy needed me, he needed all of me this weekend.  I came downstairs after putting him to bed.  It was one of those times when it took every once of my energy to put one foot in front of the other and I even paused a few times because I could not find that energy.  Then I sat on the couch with my husband who made me smile a little.  I ate three ice cream sandwiches which are a big treat for me and my heart swelled with the idea that I was able to afford them through your generosity.  And I thought of you; I thought of the Dragon Mothers from whom I draw strength and I knew I could sleep that night and wake up the next day with new ideas to help this family grow and thrive... not just get through the next day. 
 

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.