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. 

You Are Here for A Reason

 

Having a child with a rare disease makes you feel very helpless.  I feel very helpless.  It does not help that my child refuses, almost always, to take any medication to make herself feel better.  LillyAnna is still in the midst of her flare which means continued vomiting, fevers, pain, headaches and belly pain.  I have been wondering where the lesions are and it looks like we found them.  When I mentioned to Dr. R that she was having pain in her 'throat' I thought it might be reflux.  But oxycodone would not take reflux pain away.  He wondered if she has a lesion inside her airway.  This would make a lot of sense with her current symptoms.  She has a hard time catching her breath lately and the inhalers are not working.  She coughs frequently especially when she does any kind of exercise.  She is also eating less.  So last night I did a mouth and throat check.  Sure enough I found a lesion.  This one was on her tonsil.  It was almost impossible to get a picture of it.  I got one but you can hardly see it.  I also saw what I think is one on her tongue (I see another one like it this morning) and the one in her mouth is enflamed again.  When I told Dr. R, he told me he is not surprised. 

I was. We have had such a nice couple of months.  I thought things were going so well and that the increase in Humira was going to be our ticket but I feel like all it did was fool me.  It fooled me into thinking we were safe.  But what has really been happening is that this horrible disease has done a sneak attack and decided to go incognito on us.  Instead of creating lesions where we can see them, it is creating lesions where we cannot see them... as easily.  But they hurt just the same.  And I am helpless, even more helpless now than I was before to help my baby because I cannot see what is hurting her.  I have to guess.  I have to guess.  I have to guess. 

My four year old, almost 5 year old, drinks bottles.  These bottles are probably the reason we are not in the hospital right now for dehydration or would not be in the next couple of days.  She is sitting in my lap right now drinking one, grunting in pain. It hurts to drink.  It hurts to eat.  I am wondering when she is going to stop doing both.  Or, the flare will stop and we will be ok.  I have no idea.  I have no one to ask because there is no one to ask.  There is no Infantile Idiopathic Pyoderma Gangrnosum group.  Or even one other mom that I can ask.  Lil's symptoms are all her own.  I feel overwhelmed and I feel helpless and I feel scared and my baby hurts. But she looks so healthy and she plays and she pushes through because that is what she does and it is what she always does.  They tell us it is because she has known pain her whole life.  She has known pain her whole life.  She has known pain her whole life. 

You have all heard this before.  You have heard it many times.  If you continue to follow our story you will hear it many more times because this is our life.   It is up and down.  It is in and out.  It will not change and it will not go away.  It will not change and it will not go away.  It will not change and it will not go away.

We are moving in a week.  I am so lucky to have my support group because if I did not know all of the extraordinary things those families do under extraordinary circumstances I do not think I would believe we could pull this off.  But I do know them and I have seen what they do and so I know that we can and we will get it done because that is what we do.  We get it done.  I am going to try to not worry.  We will get it done.  We will get it done.  We will get it done. 

Thank you for listening. 

Twenty-One Elephants and Still Standing

I am calling it.  We are in the middle of a flare.  Last night we gave Lil oxycodone which we have not done in months.  But she is making this grunting noise (I have told you about it) which tells us she is in pain.  I offered her the "red medicine" and at first she refused.  We do not force it.  If she refuses it we assume she does not need it.  Lil knows her pain and she knows what ''red medicine" does for her at this point.  I just put it next to her dinner plate and in 15 minutes she picked it up and took it.  The grunting stopped 20-30 minutes later.  She is still vomiting every night, spiking fevers, showing a little swelling in her fingers and she has not been the happiest child ever as you can imagine.  But she is still Lil; playing when she can, running around when she can.  The coughing is getting a little out of control and she is less likely to sit and rest than she used to be but she will rest when it gets bad.  She is getting little pustules but the band-aids are not causing a problem; it was just that one, so strange.  We start moving in 3 weeks and I am just praying this all goes smoothly.  The last thing I ever thought we would be doing, ever, if not at this point in our lives is moving.   No part of us was planning for this or thinking of it.  It will all work out in so many ways.
The other day a friend dropped off a letter telling us how inspired he and his family still are by us and our story.  They included tickets to baseball game for our family.  I cannot tell you what a pick me up it was for me.  I just cried.  I have been talking to a couple of close friends lately, one from AI, about this journey.  About how you learn to live this life but it is hard and you make it look easy because it just becomes your life.  About how everyone's journey is their own but they follow basically the same pattern.  It is so interesting to me... and empowering.  But right now I sit here listening to that beautiful little girl who 'looks so healthy' grunt in pain and it is hard. I know I have to order her meds today, get her to take them, fight with Medicaid because (once again) they are trying to get us to pay a copay.  It just goes on and on.  So all I can say to all of you is thank you all for your continued support. 

Dark Water Rising

I have not written lately.  I have not written because I felt I have asked enough of all of you over the last three years and things have been pretty quiet so I thought it was a good time to give us all a break.  Unfortunately Lil has decided that break has come to an end.  It has not come to a screeching halt but our blissful little break has ended.
Little LillyAnna has had a relatively quiet winter.  Everyone keeps asking me how she is.  I never know how to answer that question.  What I want to say is "What do you really mean, how is she compared to a normal child? or how is she for LillyAnna?"  Because those are two very different questions.  But I never do say that. I have just been saying, "She is doing pretty well actually!"  To which everyone heaves a big sigh and I can see in their eyes they have moved on to some other worry.  In reality, she is a sick little girl that looks really darn healthy.  She has a cold all winter to the point where our Nanny has created a 'friend' for her named Chompy who sits with her and eats her dirty tissues.  I think Lil actually believes it is a toy, or even more pathetically, her friend! 

She still gets pustules although, since increasing her Humira this fall, I would say she gets less.  She also has less daily pain, but the pain is still there. 
This winter we also found out she has hearing loss in both ears.  It most likely from fluid or ear wax but we need to see an ENT and figure out what to do.  Poor kid can't hear a thing though... And now we think she may have asthma.  When she was first diagnosed with PG, each time her disease acted up her airway would constrict.  For some reason, all winter she has had a strange cough that is getting worse.  The doctor is having us try an inhaler and if it does not work move on to heavier drugs.  The biggest problem is that she coughs so hard she vomits.  Dr. R does believe it is somehow associated with the PG. 
I am working part time and it is going well but I would not be able to do it without the Nanny that we have.  She knows Lil well, she takes care of our family, and she is willing to do what it takes to take care of our family.  She is willing to take 20 minutes to get Lil to take her medicine if that is what it takes.  And Lil will listen to her when she tells her to take it. No small feat with our Lil.  They have dance parties but she knows when Lil needs to rest.  They play dolls but knows when Lil can handle playing with friends. 
So life is going on but it is no normal life, we have just learned how to live it.  I have watched that happen with a few of our friends who have to live this life too.  They have learned how to live it.
Lil uses a special kind of Band-Aid because all the other ones cause sores on her skin.  A very kind couple from Golden Crown NYB ordered a whole case of them for us and we have been using them for months with no issues.  This week, for no known reason, we pulled one off and found this: 

 

 

These are the pustules.  Again, not big but there all the same and right at the injection sites.

 

 

I then noticed that the spots where we had given Lil her last couple of injections had caused small pustules.  Now they are small however this is scary.  It is scary because if you will remember, skin trauma causes lesions with Pyoderma Gangrenosum.  We have not seen an injection site cause a pustule in three years, at a time when we had no control over this disease.  They have not turned into anything but she is running random low grade fevers and not eating dinner.  She is also vomiting just about every night again.  These are not good signs.  The other thing is, the pustules are not going away.  They are not getting any bigger either which is good.  She is also in pain.  She grunts in pain all day.  I know that grunt and I know it well. First she told me it is her belly hurting then she said she was clearing her throat.  I don't think she knows what hurts, just that she is uncomfortable. 
That kid was listening to "Roar" today and said, "Hey mom!  It's our song." Then gave me a smile that just melted my heart, looked at me with eyes wiser than they should be and nodded her head.  She just gets it. 

Move Over Rover!

I have some news to share with all of you.  We will be moving in with my father in law.  Next week we will be putting our house on the market to sell and looking for a house in which to live with him.  This situation will give us more support in many ways and we cannot be more happy to share our family and space with my father in law. 
LillyAnna has been flourishing with her new Nanny.  Since increasing her Humira (and the adjustment period following that) this fall LillyAnna is having less pain.  What comes with it is that she has been sick pretty much ever since.  Dr. R says she is good enough right now that we can get her vaccines.  It is always a give and take. 
My normal fears about Lil are rearing their ugly heads with our upcoming move but I am trying to be optimistic.  I don't know how to explain that although Lil is in less pain, there is still so much of her care that takes over our lives and makes me worry about how a move will affect her and her care.  The biggest difference now is that I have her nanny now and she makes all the difference.  She loves Lilly and loves our family and takes such good care of all of us but most especially Lil.  I never felt alone in all of this because I have all of you and loving family and an amazing husband.  But having someone who is here just to take care of and focus on Lil makes a big difference.  We have always been just on one side of qualifying for a nurse but never quite there.  This way (even though we pay for it out of pocket) I feel like I have that support.  I do not think I could even consider a move like this without her.  She is already offering to help me pack and keep the house in order for showings.  But what I know is that she will always keep Lil's care first.  And that is what is most important to us...