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...

Home Again

It is 5am and I cannot sleep, but I am home! They told me this is a side effect of the hospital stay but I have to admit insomnia is something I have been lucky enough to have avoided my whole life so it is throwing me through a loop.  I also have to say that although the nurses at the hospital were some of the best I have ever dealt with (baring those at AI of course:) the hospital stay itself was awful.  I am not good at being helpless.  The first thing they did was insert a PICC line which pretty much rendered my right arm helpless and then hooked me up to an exorbitant number of IV drips which made any movement pretty much impossible.  Does it sound like I am complaining?  Let's just say I have a new appreciation for anyone who has spent any amount of time in the hospital and my respect for them and my own daughter has increased ten fold.  I think I had a healthy respect for that in the first place.   
It would all have been worth it but I came home to a headache that rocked the house.  This is also something they said is normal, "it happens all the time" were the nurse's exact words when I called the hospital. 
I am not giving up though.  Not yet.  If I will not give up on Lil I cannot give up on myself.  So I will continue to follow doctor's orders, try to sleep and hope the headaches stay away so I can enjoy my favorite time of year.  The good news?  Lil did great while I was away... no vomiting!  Thank you God!  The twins missed me a lot.  I am so happy to be home.  Those words are not even enough to express my gratitude for being home.  Thank you all for sticking with us...

Wemberly Worried

I thought Lil had a normal stomach bug.  Why I would think she had anything normal is beyond me.  She had been vomiting in the mornings and at night (which is not her pattern of just nighttime)  and having diarrhea which is very unusual.  This lasted about 24 hours and I thought we were over it.  For once I thought it was a normal stomach virus.  Then last night she woke up with severe pain in her stomach (side), it was so bad I gave her a dose of oxycodone.  There was vomiting and that same diarrhea.  This went on for about an hour before the medicine kicked in and she finally fell asleep.  I know she was in a lot of pain if she took medicine!  She seems fine this morning.  That is my biggest concern.  If it was a stomach bug she would be down and out, not completely fine and eating well for hours or even days and then the symtoms starting up again.  I am afraid this is a reaction to the increased Humira.  Dr. B told us any side effects would not be felt until about 3 months into the increase.  That is about where we are. 

My biggest concern is that I am going into the hospital today and I am supposed to be 'resting'.  Right.  Chris is going to have to deal with all of this by himself and work full time.  I am so worried about this baby.  I know Chris and Julie can handle it but when it comes to disease stuff I need to be involved.  Maybe it is just a stomach bug that is coming and going but what if it is not?  The pattern is just strange.  It is so hard to tell....

I am all ready for my trip to the hospital.  I have my bags packed, I am hydrating myself for my own IV.  I have written notes to the kids for each day I am in, what else can I do?  I told a friend I am going to try REALLY hard to rest so I can get out of there in 4 days.  We had a good laugh about that. She said, "I think that defeats the purpose."  I have a stack of books and they give you free WIFI but it costs 5$ a day for TV.  That is just ridiculous.  And I have my Christmas cards:)  I refuse to lose my Christmas spirit.  I thank God for my wonderful husband, amazing nanny and family and friends who have reached out to support me.  Thank you. 

 

The New Small Person

 

 

LillyAnna has been growing in leaps and bounds.  She looks more and more like a little girl and less like a baby every day.  However, with this recent illness that she cannot seem to kick she is losing weight. I just do not understand why she stops eating like this when she does not feel good.  Lil has congestion in her nose that I do not think has gone into her chest but it is making her cough so hard she is throwing up.  Then again she throws up so often it is hard to tell exactly why she is throwing up.  Whenever she runs she coughs but that has been happening for years as well.  I will be contacting the infamous Dr. R to see what he would like to do.  She is not running any fevers which is why I have not done anything so far but I want to figure this out before I go into the hospital.

Lil's first cannoli.

 

We went to our support group meeting the other night and there was a mom there who was, what I call, 'deep down in it'.  Listening to her share about the ups and downs she is experiencing and how hard it is was hard for me.  She shared that every time she thinks everything is ok something else happens.  I remember writing about that very thing here.  We are now in a place where everything seems to be going well.  It made me feel like I should be waiting for the other shoe to drop.  Every time I relax something happens.  I do not want to do that, just wait for something bad to happen but I also do not want to be blindsided.  This illness is a perfect example.  It is not normal to have a cold for 2 months, one that is bad enough you cough until you throw up and lose weight.  Her little clothes are starting to fall off of her. 

Redness on her cheeks is the beginning of the ulcerations on her face we are trying to prevent after last year's hospitalization. 

I have to make sure she is ok before I go into the hospital for my headaches.  I want to thank you for all of your support.  So many of you have told me I need to take care of myself but I hate this, I hate that I have to do this and I hate that I have to leave the kids and my husband.  I know I have to do it and my wonderful husband can handle things at the house so I am letting go of it and accepting it. But I hate it. 

 

Halloween!

 

Young and Old

I just spent a hour reading through old blog posts looking for the title of the one where I talk about Lil's dressing changes.  I could not find it.  What I did find was a lot of tears and a history of not only Lil's disease but also my topic for today.  My own headaches. 

I wish I could tell all of you that Lil is feeling better.  Is she better than she was three years ago in the hospital?  The answer is yes.  However, she has been pretty much the same for the past two years.  As a family we just now know what that will look like and how to handle it. 

This past month she has been vomiting every night.  I am worried she is losing weight.  Our reality is that she has enough weight on her right now that I am not even bothering to tell Dr. R because there is nothing to do about it whereas last year I would have been emailing him a couple of times and probably bringing her in for a check up.  Now I know that, although watching her vomit every night is horrible, we would not do anything about it until she loses enough weight or dehydrates herself. 

She is not eating much except sour cream and onion chips.  I don't even want to know what her blood work looks like.  And I am waiting to hear back from audiology about an appointment for her failed hearing test.  She has a terrible cough right now and is covered in pustules.  Most mornings, with this weather, she has been in pain with swollen fingers and bad hips.  I feel so bad for her.

I truly thank God for our amazing nanny, Julie everyday. 

Here is our new issue.  As most of you know, I have been suffering from chronic migraines for about 15 years.  For most of that time I would have about 1-2 a week which now I know is considered normal. 

When we received Lil's diagnosis and were in the hospital for over two months the first time, I was under a lot of stress.  During that time, we were so worried.  Lil was sick, we were doing dressing changes every other day.  They took two hours and they were excruciatingly painful for my 9 month old baby.  She had over 30 lesions and the bandages were sticky.  We had to peel them off and re-stick them.  It took me half an hour to set up and the aftermath... well you can imagine.  She screamed the whole time and sometimes just passed out from the pain in the middle.  My job was to hold her down and try to soothe her during this ordeal.  Right.  On top of that, we were so scared.  We had no idea how to treat this disease and it just kept spreading.  During all of this, Chris and I were raising the twins, coordinating their lives and I was deciding to quit my job. 

On the outside, everyone was telling me how strong I was but apparently there was a lot going on inside.  My brain was not happy.  I have been waiting 6 months for an appointment at Jefferson Headache Center being told they are the best.  The good thing is that I now have a diagnosis: transfer migraines.  The doctor compared it to getting a concussion.  He said that when you are a migraine sufferer who gets migraines once or twice a week and then something happens that puts your brain under severe stress (like some kind of trauma or stress- for me, Lil's illness) you put yourself into almost a concussion state.  What do you do when you have a concussion?  Rest.  I have never rested my brain.  His recommendation is to go into the hospital for a week.  There I will rest but also get IV medication to fix my brain.  Sounds crazy.  I was flabbergasted.  Here is the thing, I do not have a choice.  I have to do it.  I have a migraine 24 hours a day 7 days a week and it has been like this for about three years.  After his exam the doctor said I am one of the worst cases he has ever seen. It looks like I will be going in the first or second week of December.  I am scared to death.  My kids are going to freak out.  Especially little Chris. I have not yet told them. This has to be done.  My Aunt Tina and I keep talking about it because she was kind enough and smart enough to realize that I needed someone to go with me to this appointment.  She tells me I am so lucky to have so many people in my life that can help.  I am just so tired of asking for it. 

Young and Old
             ~Charles Kingsley
When all the world is young lad,
And all the trees are green;
And every goose a swan, lad,
And every lass a queen;
Then hey for boot and horse, lad,
And round the world away;
Young blood must have its course, lad,
And every dog his day.

When all the world is old, lad,
And all the trees are brown;
When all the sport is stale, lad,
And all the wheels run down;
Creep home, and take your place there,
The spent and maimed among:
God grant you find one face there,
You loved when all was young.