Today I'll Be a Princess

Where do I even begin with what has been happening with LillyAnna lately?  It is hard to know. Should I start with how she was so miserable from being on steroids and being in pain that she refused to perform in her Christmas dance performance?  And how the two of us stood backstage and cried for the full hour long show? 
Or do I tell you all how she has been in such pain that she has not wanted to do anything but cry and sit on the couch?
Or do I tell you how we left the hospital from seeing my dad and she puked in the parking lot in the freezing cold?  Because, yes, she has been vomiting every night. 
Or do I start by telling you how we have taken to calling her Dr. Jekyll and Mr. Hyde because her mood swings just got that bad from the steroids.  If you have ever been on steroids, you know how crazy they can make you feel.  Try that on a five year old, during the holidays....
I could keep going but I won't.  Let me tell you why.
I also ran into old friends who I call Dragon Mothers.  If you follow my blog, you know that Dragon Mothers are those who have watched their children die.  I am not going to sugar coat it.  They have held their babies while they took their last breath or worse, did not have that privilege.  Recently Lil has been talking A LOT about her friend Lucy whose Dragon Mother was a close friend of mine.  Every time in the past month we pass a cemetery close to our house she says, "I wish Lucy was buried there so we could go visit her every day."  And so I contacted her to let her know her baby, who died at 5 years old, is not forgotten.
Another Dragon Mother I ran into in the grocery store the day before Christmas Eve.  I have not seen her since her son Dan's funeral.  We talked for about an hour and made everyone around us annoyed but I hope they saw us crying and tried to understand.
And yet one more, her son died a horrible death from Mitochondrial Disease, like Lucy, and now her daughter has it.  There is no cure.  I reached out to her to come back to our group.  I cannot imagine what that family is going through.

Why has this been happening?  I believe it is to remind me that no matter how bad it gets, I still get to hold my baby at night.  I still get to smell her and feel her small, soft hand in mine and listen to her sweet voice, even when she sounds like she is a character in The Exorcist. 
There has been a lot of pain with this flair.  Much more than the last it seems.  She is getting much better at telling us about her pain, which meds she needs and when, but she has been in so much pain.  She can tell us when she needs Tylenol /Advil or oxycodone and I can tell her what she is allowed to have.   I don't know how to help her.  I would take it all if I could.  And worse, I don't know where it is coming from. 
She very much enjoyed Christmas morning and Christmas Day but even with pain meds we did not have things under control.  Yesterday all she had was Tylenol and her normal meds so that is an improvement.  But we had a couple of days like that mixed in so I am not holding my breath. 
My baby is suffering, it is getting worse and I don't know why. 
To top it all off, we have been denied Medicaid.  It is some kind of mistake but I am praying it can be fixed even though I have called a number of times and gotten no response.  Julie and I are going to go down there and just sit and wait.  I am starting to have to buy more medical supplies and now have to pay for them out of pocket.  What a mess. 
It is so hard because she looks so good and normal to everyone else but they have no idea what goes into making her look that way or what things look like at home... Happy New Year Everyone.  I am so grateful for this life, my little girl, my other two beautiful babies, my husband, my family, all of you; I could just go on and on.  I hope 2017 brings more beauty and light.  And for today, she will be a Princess.

The Hunger Games

Does the title of this post seem like too much?  Well, it is exactly how I feel.  I feel like a force too big to be crushed is constantly, quietly taking too much from our world... unfathomable things. There are glimmers of hope and we cling to them but it is so hard. 

I have found a career that I never would have found if not for Lil's illness and I love it.  I can honestly say I wake up each morning so excited to go to work and do my job.  I never mind working outside of my work day.  It is all so invigorating.  How lucky am I to have found this work in the midst of all of this?  So many people I know who have medically fragile children either stay at their jobs and struggle each day trying to balance everything or quit their jobs and give up their careers completely.  I was in that second category for awhile and now I have found a career that allows me to do great work and balance my life as well.  I am one of the lucky ones.  It has not been an easy road and we are still healing financially and emotionally from it but we are on the right path.  I am still suffering from chronic migraines.  My hospital stay last December did not do much to improve my situation.  However, my issues are merely a side note.  I am doing ok.

On the other hand we have Lil.  Dr. Raab says he believes her disease is progressing.  Just writing that gives me pause.  We continue to increase her medication and her disease continues to fight back.  September 15th Lil had surgery to remove her adenoids and tonsils, and place ear tubes.  We also had an endoscopy done while she was sedated to see if we could find out why she vomits so much.  The tubes were meant to help with the significant hearing loss she has been experiencing. 

We did the tonsillectomy and adenoidectomy because the Pyoderma has started to attack the tissue not only on the inside of her mouth (which started last December) but her tonsils.  She was getting full blown lesions on her tonsils.  You can imagine how painful this could be.  Our thinking was that if we remove the tonsils, we would remove that which is often inflamed and calm down the PG.  The fear was making the PG "mad" so to speak. We used steroids to help us on that front and only ended up with about 4-5 mouth sores as a result.  Unfortunately, it seems as if the PG is just choosing to attack the tissue around her tonsils; although it is not as often.  She is also still getting mouth lesions.  Now they seem to be coming at that spot where your tongue attaches to the bottom of your mouth.  They are very hard to see in pictures.  She also has lesions on the inside corners of her mouth right now but they often come on the insides of her cheeks. 

The doctor who performed the tonsillectomy and adenoidectomy was amazing!  We loved him and he had actually done our cousin's tube placement.  He told us he found some strange pustules on her tonsils and biopsied them for us.  Dr. R and I knew they were not going to grow anything because PG does not but it was good to have confirmation and to actually see these things up close.

Yes, I am using this as a way to shamelessly add a picture of my cousin who had tubes placed by said doctor but who is the cutest and sweetest thing ever!

The scope showed nothing.  No damage which is good, but also no reason for the vomiting.  The tubes worked and she now has perfect hearing!

So what is going on now?  Now the PG is finding its way or I should say her inflammatory system is.  Her fingers have been swollen and sore so her arthritis is acting up for some unknown reason and her arms look a little swollen to us as well.  She has actually been complaining of pain which is quite unusual for her so that tells us she is very uncomfortable.  She has a large pustule on her thigh.  This one is significant because pustules come and go for her these days but that is just it, they come and go.  This one is big, full of pus and it is not going.  I have to say it is making me cautious. 

 

She has also been getting severe headaches.  They come and go.  Dr. R and I are trying to figure out what they are.  We are going to see ophthalmology.  However, I have a history of migraines and so does Chris.  Another point is that one side effect of Humira and methotrexate is headaches.  She has been getting them for awhile.  A few years ago we saw ophthalmology for the same thing and even did a CT scan for it.  These headaches are so bad, they are preventing her from doing things.  She missed her little Thanksgiving Feast at school.  She said, "Mom, I don't feel good!"  and that was the end of that. 

On top of all of that, we were getting blood work a few weeks ago and some of the numbers came back showing us that her liver might not be too happy.  Dr. R (ever calm) says he wants me to wait a month which will be next week, and get it tested again before we jump to any conclusions because it could have been a virus or something.  Love that man.  But the numbers were pretty high and one of the side effects of both Humira and Methotrexate are liver damage.  Also, remember Lil is young to be on Humira.  They do not really know how it is going to affect her system.

When I say "love that man" I am dead serious.  In our life you cannot freak out if liver numbers are supposed to between 5 and 25 and they are 195.  Also, for some of my friends that is nothing.  The other reason I love him is that he is always there for us.  I cannot say enough about that...

And yes, Lil has started school! She goes to preschool 3 days a week for only an hour a day.  We are seeing how that goes (this was the advice of the NIH) and we can increase from there.   She is going to Montesossori Learning Center and they have been phenomenal in working with us and taking care of her.  They even managed to fill out a report card on her! The school and Lil are dying for more.

Lil's other big news is that she started getting caught up on her vaccines.  We gave her two last week.  I am hoping her current situation has nothing to do with the vaccines, it really should not. 

Poppop took us out to dinner after vaccines and this is what she ordered!

 

Thank you all for your continued support.  We could not live this life without your love and understanding.  If you know me at all you know how excited I am for the holidays.  The holidays were very important to my mom and I love to honor her memory that way.  It is not about the gifts to me but the whole two months of good will and good cheer.  Just think... two months of thinking of other people!  It is awesome.  Anyway, I hope you all enjoy it.  

How Do Dinosaurs Say Happy Birthday?

Dear Miss LillyAnna,
             Happy Fifth Birthday!  How do I begin to describe this year for you?  It has been phenomenal.  You continue to wow us with your full personality, love of life and fierceness!  You keep telling everyone that now you are five then you say, "and I love it!"  I wonder what you love about being five.  Is there a part of you that knows there are things you are doing right now we once thought you might never do? 

              Dr. R says your disease is progressing.  Your lesions have moved to the inside of your body and we have had to continue to increase your medication.  How have you handled this?  Well, like you have handled everything else life has handed you.... by throwing it punches.   You are scheduled to go into the hospital in a week for surgery to have your tonsils and adenoids removed, tubes inserted into your ears and be scoped in an attempt to see just how far your disease has progressed and to try to stop these new lesions from progressing further.  We will see how your body reacts to all of this.
             So what have you done?  You have started dance class, dance class!  You became a ballerina this year.  You do not always make it to class (on your bad days it is hard) but Miss Sue says you do very well when you are there.  The first time I watched you in class I bawled my eyes out.  All I could see was that nine month old baby covered in bandages.  All I could feel was the fear that we would lose you or that you would never walk. And there you were dancing. 

             With this small success, the NIH said we could try short bouts of school and see how you do.  So we are doing 3 hours a week of preschool.  You love it.  This is another milestone I was not always sure we would meet.  You have not been feeling well since we started, we already had to do a course of steroids but I am hoping that will resolve itself after the surgery. 

               You still love your shovey blankets and rub on the satin part to go to sleep (and yes you still drink a bottle!), this year you have dad and I take turns laying with you at night.  You are still throwing up a lot at night.  Plus, to be honest, we love it.  You are so sweet and you tell us all about your day with Julie. 
               Julie... how do I even describe your relationship with Julie.  Julie is your nanny but she is also your best friend and just an integral part of our family.  You absolutely lit up when she got to your birthday party this year.  It was adorable.  She makes you so happy and takes such good care of you... of all of us. 

               This year you have loved going to the zoo.  You love the big cats and the reptile house. 
               You chose a Jurassic World themed birthday party this year.  You insisted!  You have never seen the movie but something about those huge, ferocious dinosaurs intrigued you.  That is how you deal with life; big, bold and sometimes loud!    So we had a Jurassic World birthday party. 
               I read the Pyoderma Gangrenosum facebook page.  It has stories of adults with a disease similar to yours and their treatment.  They talk about how awful their lives are, how horrible the side effects of the medications are, how bad the pain is... Then I think about you and think about how everyone says you act just like a regular kid.  You are so amazing.  I know that when you complain of pain it must be really bad.  You say you have headaches and your legs hurt.  Then you go play like every other five year old I know.  I just don't know how you do but I am so proud of you. You truly are an inspiration.  Happy Birthday sweet girl.  I love you. 

The Uncorker of Ocean Bottles

A big unknown. That is what is ahead of us.  If you remember, Lil has hearing loss.  She also has lesions on her tonsils.  Those are what we can see.  What we suspect is that she also has lesions on her esophagus.  We know that she has significant reflux so there is a question about how much and if any damage that is causing especially with all of the vomiting. The doctors' solution to all of this is to do a couple of things.  They are going to put tubes in her ears, remove her adenoids, remove her tonsils and scope her.  They like to do all of this in one shot so they only have to administer anesthesia once.  All of this is going to happen for Lil on September 15th.  For a normal kid this would not be a big deal and they would most likely not even stay in the hospital.  They are going to admit Lil and keep her for a few days or as long as she needs to stay.  We are going to give her steroids and keep a close eye on her.  We know that Pyoderma Gangrenosum reacts to skin trauma.  When Lil's disease was active, every skim trauma (every time she scratched herself, every needle prick, everything) caused a lesion that grew out of control).  So the question is, what is going to happen when we start to cause trauma to these lesions on her tonsils?  Maybe nothing because we just increased both of her meds.  That is what I am hoping. 
Unfortunately, although we had about three weeks off of the super runny nose and coughing fits, they are back in full force.  And as I said before the grunting she does when her throat hurts is back as well. 
For now, we are planning her birthday party.  She is so excited and we are just going to have a good time.  She absolutely loves her birthday parties.  All she cares about is that we have it at the pool and that she has a piñata!  She has not asked for anything else.  Well, she keeps asking for people to be there...
So we will plan her a great birthday party, enjoy every second and then plan for surgery and hope everything goes well. 

Don't Let the Pigeon Drive The Bus

I feel like a pigeon is driving our bus today.  I feel like no one knows what is going on and there is just simply no one to ask.  Lil had her little flare last week, if you can call it little, as most of you already know.  Running fevers between 102.5 and 104.5 for a week is not what some people would call little.  Most of the time they were up in the 104 range.  The big question is: did she have any lesions?  The answer these days is that I don't know because now this horrid disease has decided to start attacking tissue on the inside of her body instead of the outside.  For many reasons Dr. R and I believe this recent spell was disease activity and not an illness. She was showing so many signs of her disease activity like vomiting before bed, small pustules popping up, fevers with no other illness symptoms, no one else in the house was sick or got sick, the list goes on.  Also she has started making that grunting noise in her throat that tells us the lesion in her airway may be back.  I cannot for the life of me get her to take a steroid.  We tried everything under the sun.  She can even swallow pills and she will not do it.  We had a bag packed for the hospital and Dr. R had a bed waiting for us when the fever finally broke.  If it were not for that amazing man we would have been inpatient a thousand times over, any other kid would have been inpatient with those temps, but we try to keep her out of the hospital at all costs and we were able to once again.  It was close this time though...very close.  And boy does it take a toll on me.  Playing nursemaid to a four year old is no easy feat. 

I always tell Dr. R that when I have his attention, that is when I get worried.  I like when it takes him a couple of hours to return my emails.  That means we are not a top priority.  Lately he has been answering me within minutes.  I don't like that.  It means we are a top priority.  It means he is worried about her and when he worries I worry because if he is worried that means there is something to be worried about. Today's response was that he was consulting with rheumatology and they think we should increase her Humira again.  We just increased the Methotrexate and we increased the Humira in the fall. 

It just feels like we are going in the wrong direction.   Aren't we supposed to be in a place at some point where we can take her off these meds?  The other 17 babies who had PG were on one biologic (if that), short term, went off it and that was it.  Why is she different?  What does this mean for her future?  She is only 4.  And I always go back to what the risks are of suppressing her immune system long term.

I would love to say that we have to keep increasing because she is growing but I know how much weight she has lost recently.  I just wish there were some other cases we could compare her to, someone we could consult... someone who was living this same life.  But of course I am glad no other child is going through this at the same time. 

This long term worry thing is tough.  There is just no end to it. It never goes away.  It is the same worry day in and day out and it is such a big worry.  The worry being will she get cancer?  Will she get start to get lesions on her skin that eat away her flesh?  Are there lesions eating away her flesh right now that I cannot see?  Are we making the right decisions?  How much pain is she in right now?  What should I do about it?  Should I do anything about it?  It is every day, all day long.  And there is just no end in sight.  And she is just so little. 

I don't want to complain.  Life could be so much worse. I have my baby, she is home and not living in the hospital.  She runs, she plays (as much as she can), and she does so much more.  As I always tell people who say to me, "I don't know how you do it."  we each have our own reality and they all are hard in their own way but they are also beautiful in their own way.   Right now I am working on holding onto the beauty. 

The Borrowers

 

LillyAnna is not what you would call a planned baby.  We worked for four years with a specialist before we were blessed with the twins and they were awful babies.  They were both colicky.  They cried constantly.  They never slept and by never I mean there was a point at which I was actually hallucinating from lack of sleep.  I was getting 15 minutes at a time.  Of course I insisted on breastfeeding both of them even though Nettie spent a week and a half in the NICU on bottles.  Let's just say the first year was hard... to say the least. 

So that was the first year, the twins were about a year a half when I found out I was pregnant with Lil. 

I cried.  The pregnancy was awful because I was just so tired and I had loved being pregnant so much the first time.  I only gained 35 pounds with the twins pregnancy.  With Lil, I had gestational diabetes that they could not get under control and I just kept gaining weight, in total I gained 65 pounds with Lil.   I was chasing the twins around all the time, I was exhausted and I was terrified.  I was literally terrified that she was going to just cry all of the time like they did.  At the end of my pregnancy I developed a mild case of pre-eclampsia so they scheduled a C-section and LillyAnna had a birthday. 

That was when I first learned, scientifically, about how children truly affect their mothers.  Pre-eclampsia is when your blood pressure rises in pregnancy.  It is dangerous for the mother.  While I was in "labor" they closely monitored my blood pressure and  wanted me to do things to keep it down.  The doctors had suggested I bring something little that might help keep me calm.  I brought a picture of the twins, not because I thought it might keep me calm but because I thought I should to be honest.  The strange thing was, every time I looked at that picture of the twins, my blood pressure decreased.  We even made a little game out of it.  I am going to be really honest, I was shocked.  I felt like they brought me so much stress, I was always so worried about them.  But in reality they physically calmed me down.

By the way, when she finally came, she was a perfect baby: slept beautifully, ate beautifully, almost never cried. 

I had a similar experience recently.  Little Chris sees a therapist.  The therapist is working on biofeedback with him.  He is being taught meditation while being hooked up to a computer.  The computer monitors his heart rate, moisture on his fingers and something else (I can't remember right now) but basically how relaxed you are.  When you are stressed your little monitor is bright yellow.  When you are relaxed, it is dark blue.  As you are "meditating" it is all the shades in between.  In order to be able to support Chris, I am learning it too.  So they hooked me up.  We start watching this butterfly and all of the things show that I am NOT relaxed. Bright yellow. I am supposed to breath to relax.  Yellow.  It was not working.  So what happens?  I get more anxious, more yellow.  And being me, it was like a test I was failing which made it worse!  Instead of showing that I was calming down it was showing that I was getting more anxious.  I look over at little Chris and he has his eyes closed, he is making a butterfly with his hands and he is breathing.  He opens his eyes, looks up at me and smiles, then nods his head.  I feel myself immediately relax and look at the monitor.  Blue.  Not dark blue but blue.   I take a deep breath, close my eyes and breath out.  Look at the monitor.  Dark blue.  When we were done the therapist was very impressed.  "Wow Chris your mom did so well!" 

I have to be honest, I don't get it.  Kids are stressful.  It is right there though.  I have proof.  The kids calm me down.  There is some kind of connection, a physical connection, between a mother and her children.  It is there all the time.  

LillyAnna is not feeling well.  She is in pain.  She is running fevers.  She won't eat.  She is drinking at least, for now.  But this time I cannot see what is hurting my baby.  I cannot see what is inside that is making her hurt.  Are those awful lesions all over the inside of her?  The steroids helped a bit for a few days.  The lesions on her throat are smaller but that is what I can see.  What is there that I cannot see.  It is what is there that I cannot see that is keeping me up at night.  It is all I can think about.  Something is hurting my baby.  I can't decide what is worse;  lesions I could see and we had to dress and take care of or lesions I cannot see and are just hurting and silent.  If there is a connection between a mother and a child so strong that it can physically bring your blood pressure down just by looking at a picture, what does it do to your body to know that your baby is hurting? 

Yesterday she was running fevers up to 101.4 and that was with Advil.  She keeps asking for food and then when you give it to her she does not eat it.  My dad and I think something is hurting in her throat but when I look all I see are those lesions on her throat I saw before but they are smaller, there is no redness.  She fell asleep on the couch in the middle of the morning and the vomiting picked up again (we had about 5 days reprieve).  So we will see.  I hope she keeps drinking.  So I feel like I am borrowing something, happiness I guess, normalcy?  I don't know.  Something does not feel right.  I hope I am wrong. 

How I Found the Strong

 

Yesterday we bought a house.  That is a big deal right?  Lots of stress.  And it was.  For a family like ours, it is never that easy.  We also ended up at AI with Lil for an appointment.  Don't let me be more dramatic than I need to be, this was planned.  Our doc, Dr. R could only see us that day so that is when we saw him, before settlement.  Nothing is ever easy. 

It all worked out fine and we made it through but I am still reeling a bit and will be for a while I guess.  The house is beautiful and the neighborhood is amazing.  The kids did not want to leave the new house because the were already in the basement of the house across the street playing.  We hung out in the creek out back for hours while the house was being cleaned, so much fun.  We are VERY lucky, we have a whole week to work on the new house and move things over from the old house until we make settlement on the old house.  This is HUGE.  However, now it looks like we might also be spending a couple of days in AI somewhere in there. 

I like to be right.  It feels good and it feels validating.  But sometimes I would like to be wrong.  Lil does have lesions all over her throat and they are PG lesions.  Dr. R had a good point, She may have always had these when her disease acted up she just was not able to tell us about the pain.  She was always strident when her disease was bad and we could never figure it out.  (Strident is the funny sound kids make when they breath and they are sick.)  We are assuming from this and the pain she is describing that she also has lesions farther down (and the fact that she is actually losing weight- I knew it!) She can still eat because they are in her airway.  We are going to put her under, have her scoped, have her ears looked at by ENT and also give her a colonoscopy because she is still vomiting.  Once they put any child asleep for a procedure, they like to get as much done as possible but especially a kid like Lil.  Just thinking about doing all of this scare the *&%$ out of me.  Doing a colonoscopy means doing a clean out, doing the ear check mean possibly doing tubes.  Tubes for Lil are terrifying.  Any trauma to the skin causes more lesions.  When you see the lesions on her throat and think about causing more of them it is scary.  The fear of doing the scope is the same, if we enflame her throat more we can cause more lesions.  We are going to do a 3 day course of steroids to try to calm things down before we do any of this to see if it will help.  They want to see her at baseline.  What bothers me about this is that they are not going to see the lesions.  The steroids will calm the lesions down and possibly make them go away.  The ENT wants to see them at baseline.  Also, the colonoscopy will be done when everything is calm, again.  I understand why they do this, if we are going to go in it is safest to go in when things are calm but what is the point of going in if they are not going to see anything?!?!  Dr. R sees and feels my frustrations in this.  He calls it the double edge sword.  I get it but it is so damn frustrating.  The other thing is, this is her whole life and she is only four.  And remember there were only four people (people, not people under one but people altogether) with PG I could find that had these lesions in their mouths.  The other morning I went to wake her up and she had blood all over her mouth from one of the lesions in her mouth.  It was upsetting. 

He also talked about the fact that her tonsils are big and they may want to take them out.  He said they do not just cut tonsils out anymore, they shave them down.  I said doesn't that scare you?  He said, "It terrifies me."  I don't like when he says that.  This man does organ transplants in kids.  How can anything in my kid terrify him?

What bothers me the most is that I cannot see any of it.  I cannot see what is attacking her this time.  At least last time I could see the lesions, I could see how big they were.  I knew where, how many, all of that.  This time I just have no idea.  She is in pain and uncomfortable and I have no idea.  It is so frustrating. 

I wore one of our t-shirts the other day and she said, "Mom I don't like me in that picture."  When I asked why she said, "I look mad."  I told her no, you are fierce.  And a huge smile spread across her face.  So now we make the fierce face all of the time and she loves her shirts again.  I asked Dr. R how she can have those horrible looking lesions on her tonsils and eat sour cream and onion chips or sour patch kids. It is so frustrating.  How can she be spiking fevers and have this pain but run around and play?  He said, she has been in pain her whole life.  She has figured out how to do the things she really wants to do even with her pain.  FIERCE. 

So in the midst of moving.  In the craziness of keeping my family safe and happy while getting settled in a new house and still working I have to deal with the fact that this horrible disease is now attacking my baby on the inside and it is hurting her and we have to do something about it and it is scary to do something about it and scary not to do something about it.

In the meantime I am going to play in the creek with her and let her eat green chippies and have fun.  She is loving the new house.  And she is fierce. 

Yes.... I put Rocky in my blog!  LOL!

BTW, I still have t-shirts and sweatshirts for sale if anyone wants one.