The Path of the Orphaned Star

If only LillyAnna really did have wings. It has been determined by her doctors with almost total certainty that she now has arthritis. If you follow our blog regularly, you know that her diagnosed disease, of which she is the 17th reported case on the world in infants, is actually a symptom of another disease. Much like a cough is a actually a symptom of a cold, Pyoderma Gangrenosum is a symptom of another disease which is so rare we have no idea what that disease is. In adults, Pyoderma is usually a "symptom" of Crohn's disease, Rhuematoid Arthritis, Ulceritive Colitis and a few other obscure diseases. According to LillyAnna's extensive testing, she does not have any of these conditions.  In the few other cases in infants, they did not have any other underlying disease as far as we know. Remember those other cases were not nearly as severe, did not have the ongoing symptoms Lil has and basically, the disease cured itself with one or two medical interventions. We are well past one or two. One of the head Rhuematologists at AI once told me he believes she may have a brand new disease that has never been diagnosed. Awesome!

You may be thinking as I did that if she has arthritis, it answers the question of what her underlying disease is... Rhuematoid arthritis. I thought maybe we had an answer. Unfortunately, that is not the case. Children LillyAnna's age do not get Rhuematoid arthritis. Dr R and Dr B believe that her confused immune system has decided to stop attacking her skin (her skin looks beautiful!) and started attacking her joints. Why? WHO KNOWS? No one. And actually although her skin has been looking very good, there are still some small pustules that she is saying hurt her.

What does this mean for Lil? A lot of pain. Her feet and hands are very swollen. The swelling goes down and back up. This weekend her feet hurt so badly that she would not let me put socks on her... Forget shoes. Thanks to the generosity of family, we went to Olly... the very knowledgeable (and kind) saleswoman helped us find a pair of slippers and pair of boots. She had lots of advice as they work with special needs kids all the time. Now I just need to find the same in the mattress world. Mommy cannot sleep in a reclining chair with a 2 year old on her night after night anymore (especially after working at the bar all night). I inevitably wake up with a migraine. We are thinking if buying her a big girl bed but that just brings a whole new host of issues. Any suggestions on mattresses that are good for arthritis and affordable would be appreciated!

Good news: Dr R is "happier" with her nutrition. He was careful to empahisize happier and not happy  and then said "I am not threatening you with an NG tube this time." I will take that!  However, Lil has decided to stop drinking pediasure this week. To the angel who anonymously sends us pediasure each month: thank you. Hopefully, this is a phase and she is going to start drinking it again because I am pretty sure the pediasure is the reason we are not getting a NG tube now.

Dr R again suspects that Humira is not working, most likely because she is building antibodies to it.  There is a VERY new test to see if this is true but as Lil is not FDA approved to take Humira in the first place so getting the necessary stuff for the antibody test is proving difficult. The methotrexate is supposed to help prevent her from building antibodies and also suppress her immune system so it stops attacking things it should not like healthy skin and healthy joints, and lord know what else. One guess could be that the methotrexate is not working either. We cannot assume that because very rarely does the methotrexate stay in her stomach - if it actually gets into her stomach. Recently we switched from an oral liquid form to a crushed pill form but she eats so little and so inconsistently that the pills are not working either. Our next step is to use an injection of methotrexate that I will give Lil once a week.

So here is the plan of action: we are going to get an MRE which is like an MRI. She will be sedated for about 5 hours. This test will prevent us from having to get a CAT Scan and a scope. So that is very good! It will show us inflammation throughout her body including her bowels and  the parts of her bowels that we cannot see in a scope simply because she is so small and even a pediatric GI does not have tools small enough to safely go into those parts, especially in Lil, since we try to minimize trauma to her tissue.

So if LillyAnna had wings she could fly. She would be weightless and free of this terrible pain.... This terrible pain that I as her mother know she is in no matter how many times other people say she looks so good or how often they see her happily playing. I see her picking things up without bending her swollen little fingers or telling me her legs hurt and asking of she can have a chair while she plays with play doh. Yes, maybe she does want me to just hold her at night when she is uncomfortable but I also know her hips hurt and I bet that does make it hard to sleep. So if these fairie wings had the power to give her weightlessness I would thank God for a reprieve from her suffering because her suffering is real. LillyAnna chooses life. In no way shape or form does she understand that is what she is doing but again, that does not make it less real.

Take your wings and fly Lil. So many who love you are here to give you wings.

Inside Out and Back Again

If you follow my blog you know that "just keep swimming" first sung by Dorey the fish is a mantra I hold dear to my heart.   Last night I was trying very hard to keep my happy face on but this week has just been too horrible and the surface cracked enough that people could tell I was not myself. Finally I told one of my dear regulars, after having asked if I was ok about 5 times, that I was doing what I always do, chat Dorey says: "just keep swimming" and her response was "Yes, but no one says you have to swim alone."

That has been my lesson this week. I can no longer carry this burden alone.  I know that no one is asking me to and in fact many, many people offer their help and support. I am just going to get better at accepting it.  I have to take better care of myself. When we were in patient last summer one of the amazing staff on 3F would start her shift by stopping in Lil's room and asking when I had last eaten, slept and showered. If I could not answer.... It was taken care of. No one does that at home. At home I expect myself to be Wonder Woman. Perfect in every way and when I fall short, I beat myself up mercilessly and push myself to do better. It is a viscous cycle because it never ends, I can never be perfect and so each time I fail I fall further and further behind and push harder and harder failing more miserably.

Sometimes is never quite enough

If you're flawless, then you'll win my love

Don't forget to win first place

Don't forget to keep that smile on your face

Be a good boy

Try a little harder

You've got to measure up

And make me prouder

How long before you screw it up

How many times do I have to tell you to hurry up

With everything I do for you

The least you can do is keep quiet

Be a good girl

You've gotta try a little harder

That simply wasn't good enough

To make us proud

I'll live through you

I'll make you what I never was

If you're the best, then maybe so am I

Compared to him compared to her

I'm doing this for your own damn good

You'll make up for what I blew

What's the problem...why are you crying

Be a good boy

Push a little farther now

That wasn't fast enough

To make us happy

We'll love you just the way you. are

If you're perfect

I know it is in my head but when people say "but she looks so healthy" and I hear "it must be you, you must be doing something wrong." When people

say things like "maybe you should try feeding her _____" I hear "you are not a good enough mother" and when people say "well I wouldn't give her that medicine" or you should..." I hear "you are a piece of shit".  And when it is dark and she has been vomiting for hours or when we are alone in the morning and she is holding her legs in pain or when I am scanning her body for lesions or when I am watching her put food into her mouth and take it out and put it in and take it out all of those things I hear come traipsing through my brain like a comic clown parade. They mock me. They tell me it is my fault my baby is still in pain and that baby is not growing, or worse, that baby is just fine and I am making all of this up, exaggerating it all somehow.  

Add on top of that my need to be the perfect wife, housekeeper, friend, bartender, school board member, author, mother of four year old twins, niece, daughter, sister, daughter in law, aunt, sister in law, hostess, cake baker, and caretaker and the pressure cooker is going to explode my friends. Let me tell you. I have been trying to do it all and do it all very well. I am not by any means saying I am succeeding.  But I am trying.

I am writing this to you on my phone at midnight because LillyAnna will not go in her crib. She says her legs hurt. She has been vomiting since 9:30 and her hands and feet have been swollen all day. I gave her pain medicine but of course she vomited soon after so who knows if it got into her system or how much. She has a temperature of 100.7. Last week her bloodwork looked her strange, or as Dr R said "it is weird". Her white blood cell count was quite high, sed rate was high but CRP was low...

Makes no sense. However most recent bloodwork looks better. Tomorrow we will get more bloodwork and see Dr R and possibly get to talk to Rhuematology. Dr R is concerned once again that Lil has built antibodies to the Humira which as you know is a major problem for us. There is talk (rumors really) of putting her back on steroids and maybe switching her to an injectable methotrexate at a higher dose which I can't even write about without feeling bile rise in my throat. All of this talk (talk, just talk) makes Dr R and I  very uncomfortable. As he very succinctly put it, we are treating her but we have no idea for what. As you can imagine, putting these drugs into my beautiful little baby girl when I have no idea why but I know full well the awful risks involved does not make me want to tiptoe through the tulips either. But I also cannot watch her suffer anymore. I will know more after our appointment tomorrow. 

You will have to excuse the raw nature of this post and the lack of editing. I am not going to have time to get to my computer as my instincts tell me my bed this evening will once again be the armchair in Lil's room. Thank you for listening dear friends and supporters. So many of you ask what you can do to help. I have to be honest and say that when an anonymous

box of pediasure shows up on our doorstep, I cry. And when you come to see me at the bar and leave a nice tip it is so appreciated. But knowing how many of you out there read this blog regularly, many of you I have never even met, that just simply overwhelms me. All of it gives me strength. Thank you. 

Quick Update 7

LillyAnna had difficult week.  Off and on all week she had a lot of trouble sleeping. She was up all night seemingly very uncomfortable and only fell sleep after receiving her prescription pain medication.  She was fussy during the day and started to show cold symptoms.  This may be the same story many moms out there would be telling if they kept a blog on their own kids as it is the week of daylight savings and the beginning of cold and flu season.  All of this sent us to the pediatrician this week and when her ears turned up clear and a strep test also turned up clear we took a trip to AI for blood work.  With Lil everything is just turned up a notch and comes with the added worry of when it is all going to send her immune system into overdrive and create what the rheumatologist call a cytokine storm. 

She has a cold which explains why her white blood cell count is very high, her CRP was low but her Sed Rate is higher than it has been in months.  To be honest I have no idea why that would be.  Sed Rate is usually the slowest to rise and slowest to drop so I would expect the CRP to be the one to be high with her cold.  We will see what happens.  Her skin looks great.  The rheumatologist increased her Methotrexate noting that she should not still be getting pustules and breakouts on the Humira and Methotrexate.  She also wanted us to try giving it to her in a pill form crushed instead of a liquid form which she usually vomits up immediately.  Like most of the medication LillyAnna is on, it took us quite awhile to get the medication in the dose and form she needed it.  This was the first week LillyAnna received the new dose and in the new form.  She ate it with chocolate ice cream and did not vomit.  I would like to celebrate this as a victory but I have been down this road so many times that it will take more than one dose to convince me. 

Today she has a fever of 100.9 to go along with her cold so we will just wait and see.  In true Lilly form as I write this she is playing "roar" with me and hiding in the curtains.  Not much keeps her down.