The Giving Key

 

 

 

Red: Hope is a dangerous thing my friend, it can kill a man...
         Quote from The Shawshank Redemption by Stephen King

Who starts a blog post about a three year old with a quote from a book by Stephen King?  Well, me I guess.  And why?  Because when you live our life, hope can be a dangerous thing.  Since LillyAnna's diagnosis two and a half years ago my husband Chris used to very often say, "One day we are going to wake up and LillyAnna is just going to be better."  He does not say that anymore.  Our daughter LillyAnna has an extremely rare disease.  She is going to have it for her whole life.  Every day of our lives are full of uncertainty.  Each moment is pregnant with the anxiety of her illness, the fear of what is coming next and the desire to keep her safe.  The caregiving comes from me, her mom, who takes her temperature, calls doctors, makes appointments, and countless other tasks. But it also comes from her six year old brother and sister who run to get her bottle when she is crying or scream at me that I am hurting her when I have to give her an injection or hold her hand when she is lying on the couch in pain.  Of course it comes from her loving Daddy when he sits with her and watches TV on days that she is too tired to play, or holds her bin so she can vomit or lays with her in bed to calm her to sleep. 

 

LillyAnna was diagnosed with a disease called Idiopathic Infantile Pyoderma Gangrenosum at 9 months of age.  It causes large, very painful lesions on her skin.  She is the 17th reported case in the world of this disease.  There are adults who suffer from Pyoderma Gangrenosum.  It is still quite rare but more common than in infants.  Usually it focuses on the limbs or trunk.  Lil is unusual in that she developed lesions all over her body (approximately 30 in all) over the course of the next 2 months.  We could practically watch them grow, that is how rapidly it happened.  Once, in 36 hours she grew 6 lesions the size of quarters.  At this point we have the large lesions under control but have found out that her particular pattern of healing and lesion growth is unusual, in fact only one other patient (an adult) has looked even remotely like her.  The medications are not working the way they are supposed to in two ways.  One, the medications that should be helping the most are not and the ones and the ones that should be supporting are helping more.  Second, her disease is not being controlled as it should be.  She is having breakthrough PG attacks.  One of them being lesions in her mouth which as far as we can tell has only happened in eleven PG sufferers, adults, never an infant case.  LillyAnna also has Juvenile Inflammatory Arthritis but this is not associated with her disease.  It does cause her quite a bit of pain.  She is now three and a half years old.  I quit my full time teaching job to care for her and work part time when I can.  Her dad works full time.  Lil has twin siblings, Nettie and Chris who are 6 years old.  Taking care of her is something only I can do.  I truly know more about her disease than anyone because she is so unique even out of the pool of people who suffer from the same disease. 

So how can we have hope? How can we have hope when countless doctors, doctors who are supposed to be famous in their fields, have never even treated this disease and cannot offer us any advice?  How can we have hope when most doctors we talk to have never even heard of her disease?  How can we have hope when our little girl, who is just a baby in so many ways with her still chubby feet and her so soft forearms and her bottle drinking lips is being pumped full of drugs that cause cancer and who knows what else.  What is worse is that they are not even really working.  She is still an anomaly.  At the National Institute of Health she is an outlier.  How can we have HOPE?  I want to scream! 

People tell me she looks so good.  People tell me she looks so normal.  They even look at me skeptically at times.  They are right.  My little girl, my LillyAnna is a strong, brilliant girl.  She knows how to LOVE life.  I want to rip their faces off.  Because I know that her joy, her dancing, her normal three year old bounding around is going to cause hours of excruciating pain, vomiting and most likely she is going to catch some awful illness.  SO GO AWAY!  SHE IS NOT NORMAL! COME BACK TOMORROW!  You come soothe her at 3am when she is writhing around in her bed and will not let me give her any pain medication.  You  come hold the bin in a few hours when she is throwing her guts up because she actually ate.  Give her the credit she deserves. 

My stone walls are her illness, my confinement is in my own fear. Someone once told me you can live your life in two ways, through love or through fear.  If you think through each decision you make, it can come down to these two emotions and it is true.  I know this but the memories of our experience paralyze me.  My knowledge of the possible future freezes my heart.  There are things I know that I do not tell anyone because it is just too awful and because it is all so uncertain.  We make the best decisions we can and still we are judged.  No one knows what it is to walk in our shoes.  I know that for a fact.  With Lil's newest developments, we are even more rare than ever before and it all rests on my shoulders.  These walls are tall, cold and thick.  My sentence is undetermined.  There are so many guards preventing escape.

And then one day I got an envelope in the mail.  Inside was a handwritten letter and a key on a chain.  On the key is one word: HOPE.  I have worn the key just about every hour of every day since that one.  Someone who understands sent me The Giving Key.   I cannot emphasize enough the word understands.  So many people in our lives try to understand, so many support us.  Without them the walls would close in and I would suffocate.  However, the people who live this life know the air we breathe.  They have felt these walls and walked these halls.  That is a different kind of knowing.  That letter touched something inside of me that has been long been stiff and still, unmoving. 
         
Andy Dufresne: That's the beauty of music. They can't get that from you... Haven't you ever felt that way about music?
Red: I played a mean harmonica as a younger man. Lost interest in it though. Didn't make much sense in here.
Andy Dufresne: Here's where it makes the most sense. You need it so you don't forget.
Red: Forget?
Andy Dufresne: Forget that... there are places in this world that aren't made out of stone. That there's something inside... that they can't get to, that they can't touch. That's yours.
Red: What're you talking about?
Andy Dufresne: Hope.
          From The Shawshank Redemption by Stephen King

My music is LillyAnna.  She is my song.  Sitting at her princess table in the living room putting together a puzzle with such concentration even when I know her fingers are swollen.  She fights back when her sister and brother do what big sisters and brothers do.. try to control things!  Perched on Poppop's knee wearing his hat and laughing hysterically.  Rolling meatballs, stuffing peppers, asking me to carry her because her legs 'are getting tired'.  Playing with her cousin Antonella.  Engaging with pictures on my phone and talking about each picture; this is one of her favorite activities when she does not feel good.  She remembers everything and everyone, maybe not by name but by something they have done for her:  'That is the guy that drew the picture or my shirt.' 'That is my hospital.' 'Those are my nurses' 'I drew that with Dr. R' 'That is Poppop's Club' 'MeeMaw did my nails that day' and on and on... and then laying next to her at bedtime she reaches out to hold my hand as she is falling asleep even after I have had to hold her down for meds and she screamed and cried for over half an hour and vomited and her lips bled, she still wants me. She is what protects that something inside.  The strength of LillyAnna's fight, the  beauty of Nettie's kindness, the depth of little Chris' intuitiveness and the unwavering, steadfast love and care of my husband.

There is music in my heart, thank you Megan Kelley and The Giving Key and, yes, even Stephen King for reminding me how magnificent it is.

Sun Up, Sun Down

With LillyAnna you never know what is coming next.  It is like riding a roller coaster while wearing a blindfold.  I hate roller coasters, always have.  LillyAnna will be perfectly fine, temp of 98.6 and then within 2 hours her temp will go up to 104.5 and everywhere in between.  Her lips become ulcerated and start bleeding.  Only for all of this to calm back down again within the next couple of hours.  The lip sores are always there, they just get worse and open up and then get a little better.  It is the same with the sore in her mouth (the pustule we were concerned about when she was hospitalized after New Year's Day), it will become ulcerated, red and swollen and then calm down.  Due to all of this, she is not eating and drinking consistently and is in a lot of pain but it is all very inconsistent.  She is sticking to her stand off with the medications.  She will not even take anything for her pain.  I can put the Meloxican in her bottle; this is a long acting pain med that acts like Advil but is kinder to her stomach and helps with arthritis.  This is only helpful when she drinks the whole bottle which does not always happen especially when her mouth really hurts.  She has also started vomiting again.  We have an appointment with the behavioral psychologist on Tuesday. 

It is very hard to see in this pic but the lesion is right behind the front teeth.  You can see the dark spot.  It hurt very much for her to open her mouth so this was the best picture I could get.  You can also see the sores on her lip.

I was also very concerned about this spot on her lip near my thumb.  It was actually filled with pus and surrounded by red, sore tissue which is what happens right before a lesion opens.  It did not open.  However, although her lips get very sore and ulcerated, I have never seen this on her lips before.  This cleared up after about 6 hours. 

The scariest thing that happened so far is at one point the pustule on the roof of her mouth completely opened up into a full blown lesion.  I could not get a picture of it, it was just too painful for her to open her mouth.  In the hospital we (the doctors and I) thought we saw two other lesions on the insides of her cheeks.  Because this is so rare, PG is rare but PG in the mouth is so rare I could only find 3 pictures of it, we were questioning what was a lesion and what was not.  It turns out those two spots on the insides of her cheeks were lesions because when the one on the roof of her mouth ulcerated so did those two spots.  They developed black spots in them and opened up slightly in the middle.  For my fellow PG sufferers, you know that once a spot opens, it is much more likely to open again.  And so, the spot on the roof of her mouth has been sore and surrounded by puss since then. 

These pustules showed up the morning of the ear infection/ ear draining.

Then yesterday the bomb dropped.  It was not a huge bomb... maybe just a firecracker. I came home from work and about an hour later found that Lil's left ear was draining, she was complaining about her right ear hurting and her nose was once again running like a faucet which caused her face to look almost as bad as it did when we were admitted to the hospital.  So today we will be going to the pediatrician.  The trick will be getting her to take the meds and then keep them down.  That is the only thing that will keep us out of the hospital.  Dr. R and I did talk about doing a round of steroids... in his words "suck it up and give her a round of steroids" (love him).  We save this as a last resort.  I have put this out to the NIH and am waiting to hear back from them.

If you look closely you can see the redness on her face. 

It has just been constant worrying about her, for all of us.  We are all on watch.  Little Chris got into the car the other day from the bus stop, looked at her smiling and said to me, "well someone is feeling better!"  It just made me realize once more how the twins look for her ups and downs just as much as we do.  They run and get her a tissue when her nose starts to run or offer to grab the thermometer.  I do not even think about it as our daily lives go on but when your just turned 6 year old asks what medicines your 3 year old took or runs to grab her a bin because even they know the signs of her upcoming vomit, most of the time it is just normal life.  But every once in awhile it hits you like a slap in the face how abnormal it really is.   I try to shield them from it but it is who we are as a family.  They have been talking about it more and more which is good but so hard to hear.  Nettie said she wished Lil did not come out of my belly because then maybe she would not be sick.  Then she quickly said she still wanted her to be her sister.  Then quickly said she still wanted me to be her mom... You could clearly see how she was struggling to find words for her feelings.  Finally she landed on, "Mom, I just wish Lil was not so sick."  I told her we would not be who we are as a family if Lil was not sick, this is our journey.  I know she does not understand that now but I want that to resonate with her and someday she will.

To top off all of this loveliness, the icy roads got the best of me yesterday.  Driving through my neighborhood, I could not stop and neither could the other car and he hit me.  We were both being very cautious, going very slow, so it was not serious and everyone is ok.  Thank God the kids were not with me.  But it is one more thing to deal with.  I told a very good friend of mine I feel like I have a black cloud attached to my back. She replied with one of the best things I have heard in a long time: yes but most people mistake it for a cape and you wear it well.  I love that.  I have big girl boots and now a cape.  All I need to complete the set is a Super Hero name. 

10 Lipsticks

We saw Dr. R yesterday to discuss Lil's little trip to the "hotel" as she likes to call the brand new AI DuPont hospital.  With so little information available about Pyoderma Gangrenosum in the mouth (even the NIH has to gather their own data there is so little) Dr. R and I could only look once again at Lil's mouth sores and discuss their history.  He agreed with me that not taking her medicine, particularly the folic acid, is probably not helping that situation.  Remember that PG attacks places on the body where there has been trauma.  One of Lil's medicines (methotrexate) causes her lips to ulcerate and we give her this medication as an injection but the medication we give her to prevent the ulcerations is oral and she is refusing to take that one.  Between that and the upper respiratory infection we believe her disease just went into overdrive. 

This theory does not explain that one pustule because it has been there for at least a month and is still going strong.  It really has not changed overall.  At times it gets smaller or bigger, it will get red around the edges and once it got two smaller pustules on the sides but they went away.  She did tell me it hurt when she was eating yesterday.  However she very rarely tells me anything hurts because she does not want to take medicine. 

Like I said, I am in contact with the NIH about this and I am waiting to hear from the 'angel doc' about PG in the mouth.  They have been following Lil's recent illness.  They have requested labs and I have been emailing them updates.  I am assuming we will be going back there in April and have started talking to them about arranging this trip now so that we are not doing it at the last minute like last time.  A little bit of planning goes a long way! 

The big issue right now is that Lil has lost two pounds in the last month.  This weight loss started before the big issues we have seen recently so Dr. R and I are concerned.  If you follow our story you know that Dr. R considers weight gain and loss a major factor in how Lil is thriving.  Two pounds in one month when you only weigh 28 pounds to start with is a problem.  We believe this is due to a combination of her not taking her medication (reflux) and her recent illness.  Our first intervention is going to be to see a behavioral therapist.  We will start there and then see what happens.  With Lil it is one step at a time. 

It is just really hard for me to put clothes on her and see them fall off again.  It brings me back to this summer.  When we looked at her growth plates we could see how it affected her.  As I was snuggling with her last night and she was holding my hand, it got me thinking about her future.  When I tell people about her disease for the first time they always ask me about her prognosis and I have to tell them we just simply have no idea.  I choose not to think about the idea that she may never grow old and have a family of her own or be a teenager.  But last night it settled in my brain a bit.  That is all I want to say about that right now. 

Inside Out and Back Again

This illness and hospital stay has been hard for me.  I am worried about PG in LillyAnna's mouth, I am worried that she has already had three colds this season when she has barely had a cold in the last year and a half (increased methotrexate), I am worried that she is losing weight again albeit only a little bit, and we have to figure out what we are going to do about her medications.  I am always thinking three steps ahead to keep LillyAnna and this family where we need to be... or at least as close to 'where we need to be' as any family.  Generally I love where we are as a family by the way.
 

You, our supporters, our family and our friends have risen up and done so much to make this easier for us.  I finally feel like my head has stopped spinning.  I am embarrassed by how this has all affected me.  I am used to keeping myself better in check.  But it looks like we are going to be waiting to rest of this one out.  There is still a chance she could let herself get dehydrated or something else crazy but my gut tells me this illness is on its way out and she just needs to rest.  She needs mommy.  Thanks to all of you that is what she is going to get this week.  Thank you thank you thank you.

Quick Update 14

Complications.... how do I begin to explain? We have managed to avoid being inpatient for a year.  My wonderful nurse -friends tell me this is due in part to my good at home care which makes me feel so proud and safe in my decisions. The doctor sent us home with these words to the resident "We can send her home because this mom knows what she is doing and is experienced with home care.  She will bring her back if necessary."  What respect!  It is just amazing.  I am so lucky to have doctors that have such faith in me.  My heart is light and we pack the hospital room up, take my sick baby home with the hopes that she will drink and eat and continue to "do better at home".  So we get home and life just resumes as much as I try to slow it down for her.  The twins have their birthday this week.  I cannot, CANNOT, put that on the backburner AGAIN for them because of LillyAnna's illness.  Every year it happens, every year.  Have I mentioned that every year the twins' birthdays get put on the backburner?  Have you noticed I have some guilt over that?  You will be very kind if you try to make me feel better about it but the bottom line is that it is not ok.  So the kids go back to school, I start planning birthday stuff and try to plan work stuff, do laundry, clean the house, and in the meantime all little Lil wants me to is sit with her.  She is not drinking enough.  She is now running fevers up to 104.8.  So it is off to the regular pediatrician we go, in the snow... look, I just made a rhyme.  Ha Ha...

All The Colors of Home

As it turns out, LillyAnna is once again breaking the rules.  From the rudimentary research I have been able to do while here, there are only 11 reported cases of Pyoderma Gangrenosum in the mouth of all the PG cases ever reported.  Remember that this includes all the adults cases as well and there are hundred of those cases.  Although PG is still rare in adults it is not nearly as rare as it is in infants.  I did not find any oral cases in children.  I will of course be checking my research with the NIH when I send them labs and pictures from this flare. 





I am sitting in this beautiful "hotel room" as Lil is calling it writing this
post watching her scarf down a plate of chicken nuggets and mac and cheese having just heard that we are allowed to go home.  The docs do not want her to stay here any longer than necessary and neither do I.  There is just too much she can catch.  Dr. C who works on Dr. R's team and knows our little Lil well told me he trusts that I will call if she does not start drinking when we get home.  I do not know where this change is coming from, yesterday we were talking about an NG tube, but I am happy about it.  She just simply looks SO much better.  We got her the fluids she needed, she has gotten all of her meds by IV and she had a good strong dose of a knock it out of the park antibiotic.  We definitely did the right thing by coming in, it took her over 24 hours to get hydrated.  Her little body needed this. 

We found three mouth lesions here whereas I had only been focusing on that one pustule in the front after doing that research and looking at some PG lesions online.  This is a whole new ballgame and I am not ready to think about what it all means. 



What I do know is we did the right thing, we are now going home and this little trip has been one for the books:)   

 

Quick Update 13

How do I begin to explain?  If you have been reading my posts the past couple of weeks you know that Lil has not been feeling well.  Things have finally caught up with her. This morning I had to make the very difficult decision to call the in the big gun docs at the NIH and AI to get their opinions on what needed to be done.  You see, as I have said before, we do not usually go to the Emergency Department because we will most likely be admitted and that is precisely what happened.  I do believe we did the right thing and I totally trust the doctors opinions. 

Lil has only urinated twice in the last 40 hours which means she needs fluids.  She has pustules in her mouth which you know and the docs found a couple more while we have been here.  There have been pustules popping up all day on her skin as well and then calming back down.  I have seen this disease grow lesions the size of quarters from a small pustule within 36 hours.  It is not something I want to see again.  She has an undescribed rash on her bottom, her lips are swollen and we do not know why.  We have been admitted to AI and that is right where we need to be.  They are giving her fluids, antibiotics and keeping a close eye on her for now.  We are just watching to see what happens.  Hopefully the fluids and antibiotics will give her body the boost it needs so that the PG will calm down.  That is really all I can tell you.

She is pretty miserable and I have to tell you that is hard for me.  She is used to being in pain so usually she just sits around and watches movies when she does not feel good but right now she is fussy and whiney and very uncomfortable.  My heart is breaking for her.  It feels so much like home here at AI, that is scary but beautiful.  We walk into the ED and get hugs and hellos; walk onto our floor and get stopped so many times I had to walk back out into the hallway just to look around.  I talked with old friends, yes, but Lil and Chris and I were also surrounded by people who had lived our horror with us.  Not just heard about it or read about it but lived it.  I do not say that to take away from all of you who have joined our journey since that time but I feel so safe with these women who helped me hold down my baby girl to remove those horrible dressings and watched those lesions grow, these women who watched her take her first steps in this hospital and celebrated her first birthday with me. 

To be most accurate, it was not this hospital.  When Lil was first admitted, they were digging the foundation for this brand new building in which we sit.  We have watched as cement was poured, walls were erected and windows were set.  And now here we are.  As I walked through the ED today I glanced into rooms and thought about whose lives were going to forever change today like mine did two years ago.  I also thought about those who were going to turn and walk right out.  You just never know...