The Gift of the Magi

MRI showed that the nodes are not cancerous:) More results to come.

Thank you for your continued support. We could not do this without you.

Merry Christmas!

Quick Update 8

So we are going to get some blood work to find out if the lumps could be caused by a virus. There are two different viruses Dr R is suspecting Lil may have. He is not ruling out that the lumps are signs of cancer. The good news is cancer lumps do not usually move and hers are moving. But there are too many other risks and signs for us to rule it out completely. He also found more lumps in her groin area. He is going to add her neck to the MRI for Thursday, do some blood work and we will keep and eye on things, wait and see.

How the Grinch Stole Christmas

I have ALWAYS been one of those ridiculously annoying people who starts listening to Christmas music the day after Thanksgiving without shame, gets excited when the red and green bows show up on store shelves in October and has most of her gifts bought by September although the list seems to grow throughout the fall so that the chance to keep buying and giving grows with it.  It is pathetic and has elicited groans and moans from those who know me best most of my life.  Even last year when we spent most of December in the hospital I was hanging Christmas decorations on our non-opening windows. This year I can hardly even muster a smile for poor little Charlie Brown; but I am working on it.  I know how much we have to be thankful for.

LillyAnna is still suffering in much the same way with her arthritis.  She has her good days and bad days.  The doctors have decided to switch her Methotrexate to an injection due to the fact that even when we can get her take the medication (which is rare), she usually vomits it right up or vomits at some point that evening.  We are questioning whether or not these medications are even effective so before we do that we have to make sure she is actually getting the full dose.  We did the first injection Friday night.  We will do the injection once a week.  She still gets Humira injections every other week.  Thanks to a special person we got our hands on a Buzzy Bee and it has become Lil's new best friend.  Seriously, she wears it everywhere when we let her and I do think it helps with injections, we will see if it helps with IVs on Thursday.  She is sleeping better because on top of giving her Meloxican each night (a 24 hour pain killer similar to Advil but more gentle on the stomach) we are also giving her Oxycodone.  She is sleeping better because Mommy has been laying down the law a bit... more on that later. 

Unfortunately Lil lost almost two ounces in the last two weeks. That is significant when you having been holding fast at 10 kg for over a year. There is not much we are going to do about it until after the MRI which is scheduled for this week.  I do not have a good feeling about this MRI.  Historically, Lil is not easy to 'put to sleep' and she definitely does not do well with IVs as we all know.  The good thing is I am one of those people whose bad feelings are NEVER right (if you are pregnant and I guess you are going to have a girl, it is pretty much guaranteed you are going to have a boy.... get it?)  The other piece of this puzzle is that the day of LillyAnna's MRI is the anniversary of my mother's death.  My mother died on December 19th three weeks before my twins were born.  Whatever... I know LillyAnna is in good hands.  AI DuPont knows her issues well and there are so many people there who care about her.  When we go there (which still ends up being at least once a week) I feel like we are visiting old friends.  It is so amazing to me how much the people there really care.  I try to walk away from them because I feel badly that they are on their lunch break or whatever and they are still asking me questions about how Lil is doing.

The big battle that has been raging in this house lately is the War of the Crib.  LillyAnna is still a two year old a very smart one at that.  It is so hard for me because when she does not feel good, she wants me to sleep in her room with her.  I know she is two, I know she is smart but she is still my baby and she is in pain. 

 

So this week she and I have battled.  We had a long talk about her needing to sleep in her crib and me needing to sleep in my bed and that if she cries when I leave the room, she will throw up.  Sometimes, many times, she throws up anyway and then gets really upset like she did something wrong.  I hope I am not damaging her mentally.  She is doing well and has learned that she needs to go to sleep by herself.  I don't know how much of that has to do with her pain being controlled by the Oxycodone which is a pretty hard core narcotic.  Or is it just knocking her on her ass because it is a pretty hard core narcotic?   These are the kinds of arguments that happen in my brain all day. 

 

It is one of the reasons I enjoy my job so much.  When I am working at the restaurant, I cannot think about Lil or the hospital or these questions, I am just too busy.  I get to be normal and it feels so good.  It helps that I work in a great restaurant with people who have a real passion for what they do.

 

 

About two months ago my very observant father found two small lumps on the side of LillyAnna's neck.  They were hard and about the size of a pea.  We showed Dr. R and he said they were probably lymph nodes and to keep an eye on them.  Friday afternoon Chris was giving Lil a "big hug" and felt the lumps again.  They have grown in size.  One is now the size of a marble and there are two more on the other side of her neck.  Dr. R is going to see us tomorrow and check them out.  I am trying not to worry.  It is most likely nothing; the problem is that the idea that lymphoma is a side effect of Humira is always in the back of my mind but I also know that she gets bloodwork so often and signs of cancer would have shown up there. 

 

So I am working on keeping that Grinch at bay. Chris has been wonderful.  He keeps putting the Christmas music channels on for me because he knows I like them and he has taken all the decorations out without me asking.  We have so much to be thankful for.  Last night we went to one of my favorite events of the year, my friend Jason's Christmas party.  We had awesome babysitters who braved the snow, came early, stayed late and then told us how good the kids were!  The party ended with my one of my most important friends sitting in a chair crying quietly and me kneeling at his feet, head in his lap crying quietly too.  He just kept saying 'this could only happen to you because you are the only one who has the strength to handle this, the only one'.  I know that is not true but to know he feels that way meant something.  

 

Lil is not dying and she does look pretty happy sometimes.  She enjoys her life and this is what her life is going to be, it could be so much worse.  But I am tired.  I am learning to live with this life but it is so hard.  It is so hard to watch your baby throw up constantly, to be on constant vigil of wounds and weight and lumps and swelling and the unknown, it is just so hard to see your baby in pain.  It is hard to watch how all of this affects the rest of your family emotionally and financially.  It is hard when it goes on and on and you see no end.  It is hard when there are so many unknowns and you want to fight and fight to help her but you do not even know what you are fighting against.  I do know what I am fighting for so that is what gets me out of bed in the morning and keeps me moving forward.

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. 

To Make You Feel My Love

 At work on Friday night I was talking to two customers and found out that we both have experience being the parents of chronically ill children. The gentleman started telling me a story about a doctor who would visit a very poor village in the heart of Africa. I will not in anyway do this story justice but I am going to try... I cannot be held accountable for details gone awry! There was a family this doctor saw on his visits who had a young boy that was just not growing. Every time the doctor went to the village, the child was more and more weak. Finally, on one visit, the doctor told the family that he believed he had found the source of the child's illness and unfortunately the child would die. The family was distraught and asked if anything could be done. The doctor told them that the only way to save the boy was to go to the nearest hospital and have surgery. The nearest hospital was hundreds of miles away and the family had no car. After a few days the mother created a sling, put her son on her back, used her family's life savings and walked for three weeks to get to the  hospital. Along the way she was robbed, had no where to sleep, nothing to eat. When she got there the hospital told her she had to have an appointment and that the next available one was in three months but when they found out what she did to get there, they did what needed to do, completed the surgery and the boy lived. 

This story has been haunting me.  The strength of a mother's love is just astounding.  I am reading a book about a mother who finds out her one year old has Ty Sachs disease.  This is a disease for which there is no cure, your baby is going to die and it is not going to be pretty.  There is nothing you can do about it.  She calls mothers who have to watch their children die Dragon Mothers.  She says the strength they need to smile through their child's pain and comfort others when their soul is shattering is something only a dragon can do, a mythical creature known for its sheer power.  I know some of these Dragon Mothers and I can tell you it is accurate. 

Lately LillyAnna has been having a lot of pain in her feet and legs.  It is, as always, so difficult with her because I do not know if this is new pain or she is just better able to describe it as her baby ramblings form themselves into real sentences and discernible words.  The swelling we have been seeing in her hands and feet is also confusing because her body is changing so much.  My baby is losing her baby chub which is normal.  But hers is falling off at an alarming (at least alarming to me) rate as it is exacerbated by her lack of eating and constant vomiting.  Yes, she still vomits multiple times a day.   So when her hands look swollen it is hard to remember if they looks chubbier than the day before or not.  However, some things have gotten more clear over the last week.  I had to buy her crocks because her shoes will fit in the morning and then not fit later in the day, that is more substantial evidence.  Yesterday was just awful.  Everywhere I touched her she told me it hurt and she cried most of the day saying owie.

I had a wonderful moment with the Rheumatologist when I saw her two weeks ago.  She showed me how to tell if Lil is swollen in her knees and ankles with her practiced eye.  It is hard to tell because of the extensive scarring on her legs.  She also showed me how LillyAnna manages her own pain by compensating with the way she walks and moves her body.  She was astounded by how happy and active Lil was.  Then she prescribed me pain medicine.  I just started to cry.  Finally someone saw what I was seeing.  So often, I feel like I have to prove to people that this baby is in pain.  She is just SO happy almost all of the time.  But that is LillyAnna.  She has a little baby dragon heart. 







The Great And Powerful Dr. R (oh if only he could see that!!! Hahaha!) changed one of her stomach medications.  Whenever we do that we see a small increase in her eating, unfortunately, it does not generally last very long.  For now though it is nice to see her actually swallow bites of food even if it is only one or two.  Although it is done nothing to slow down the vomiting.  In fact, the other night, after she threw up she said "eww Mom, chicken."  I am a little worried that will be the end of roasted chicken!  Dr. R also wants LillyAnna to have another scope since it has been a year and a CAT scan of her head.

Without Chris, I would not have the strength to do what I do every day.  He literally keeps me going, he is my comfort at the end of every long and exhausting day.  He is the one I know will always be at my side.  Nine years ago today when I promised him the rest of my life I had no way of knowing what this life would look like.  It is beautiful and horrible and he is there at every turn to hold me and to pick me up. The ways in which he supports me are not obvious but they are strong and they are real. 


I do not mean to take away from those Dragon Mothers but we have all had our Dragon Mother moments.  I do not need to list them.  Sometimes I feel my strength falter and I feel oh so weak. I repeat my mantras "one foot in front of the other, Gina, just put one foot in front of the other" and Dory "just keep swimming" and, although I do not want to admit it Katy Perry's "Roar".  I refuse to let this destroy me.  I have to be strong, I have to find the good in it because LillyAnna, Chris and Nettie and even my husband need me to.  I am a fighter. A champion.  I will not bow down and let this thing that has happened take me down.  I will be the phoenix who rises from the ashes and I will bring my family with me.  We WILL make Lil's illness work for us.  We WILL rise above the pain and the confusion.  I WILL NOT let us fall. 

"Make You Feel My Love"

When the rain is blowin' in your face
And the whole world is on your case
I could offer you a warm embrace
To make you feel my love.

When the evening shadows and the stars appear
And there is no one there to dry your tears
I could hold you for a million years
To make you feel my love.

I know you haven't made your mind up yet
But I would never do you wrong
I've known it from the moment that we met
No doubt in my mind where you belong.

I'd go hungry, I'd go black and blue
I'd go crawlin' down the avenue
No, there's nothin' that I wouldn't do
To make you feel my love.
The storms are raging on the rollin' sea
And on the highway of regrets
The winds of change are blowing wild and free
You ain't seen nothin' like me yet.

I could make you happy, make your dreams come true
There's nothing that I would not do
Go to the ends of the Earth for you
To make you feel my love.

Harold and the Purple Crayon

There are moments in life that just simply freeze themselves like snapshots.   Some  are happy and some are not but all of them are important and unforgettable. 
The moment in sixth grade when I first saw the girl who would end up being my life long friend.  She had permed hair, coke bottle glasses, a big smile and she was absolutely beautiful. 
The single clump of dirt sitting on top of my grandmother's coffin.
The leafless tree outlined by the sun on the side of the road when my father told me to pull over so he could tell me my mother had just died. 
Chris and my hands intertwined and how his freckles stood out. 
The look on my very best friends' faces as they gave me a champagne toast before we walked down the aisle on my wedding day. 
The tip of a needle slipping into LillyAnna's skin.
The pure pleasure in my father's eyes when he is dressed as a mummer. 
My mother and father standing at the kitchen sink doing dishes.
My small hands in a bowl of ground meat mixing meatballs with a bowl of warm water next to it (which my grandmother did because I complained that the meat was too cold).
A black and white fuzzy ultrasound picture of a baby sucking its thumb while its twin sister unknowingly kicks that thumb right out of that mouth! 

So many things have happened in the past year and a half that have forever changed the landscape of my life in ways I never imagined possible.  In the blink of an eye I am become a new person.  Today I wrote to Ellen DeGeneres to share my story with her in the hopes that she will have me on her show.  Who am I?  This weekend I spoke to a woman who wants me to do baking on a large scale for her corporation.  She thinks I make the best cake she has ever tasted and she is in the business.  I am the girl who fifteen years ago was the butt of many jokes because although I could cook, I could not even make brownies out of a box.  Where have I gone?  I have a master's degree in Educational Leadership and student loans coming out of my #@$ but I am bartending.  What is my name? 
No one would call me religious but I heard a preacher on the way home from work tonight quoting Job. 
Job 7:11 Therefore I will not keep silent; I will speak out in the anguish of my spirit, I will complain in the bitterness of my soul.
I appreciate you listening, your support means more than you can possibly imagine.  Some of you I know well and some of you I have never even met yet you follow this blog and find empathy in your heart for me and my family.  Some of you have sent us gifts... very generous gifts.  I have never been very good at accepting gifts or support.  I like to be the one doing the supporting and the one doing the gifting.  But this is part of the new me.  And so I thank you.  In the moments when the only way I can get through the day is to repeat "Gina, just put one foot in front of the other, ok, now the next" until the day is done it is your support in its many forms that gives me the energy to move forward.  Your generosity gives my soul the strength to keep going.

Recently we were invited to Longwood Gardens with the Kelly Ann Dolan Fund (http://www.kadmf.org/) through our Chronically Cool Families Support Group.  This family had two daughters.  Both fell ill with rare forms of cancer.  One did not survive.  The family started the foundation in order to help support families in their struggles through chronic illness.  Their mother, Peggy was in attendance.  We all gathered after a beautiful outdoor lunch for a picture.  Gathering this particular group is not easy and as we were saying 'cheese' I happened to glance to my left.  In so doing I added another snapshot to my collection when I saw Peggy look back at the gathered families and smile as a tear slowly made its way down her face.  In that tear I saw her pain as a mother who worried, watched her baby suffer, buried that baby and then built a new life for herself on the other side.  

I do not know where all of this is going to take me.  I hope it is somewhere good.  Right now I know that I am going to take care of that baby because I am her voice and I am her advocate.  Now matter who I thought I was, I will do whatever I have to help her and create the best life possible for this family.  Thank you all. 

Mia and the Too Big Tutu

My daughter Antoinette is 4 years old. One of the bright sunny days we were enjoying last week before the downpours began she was wearing a pair of Capri pants and I thought to myself "those fit her very nicely, I need to check the size." Low and behold when checking said size my four year was sporting 12-18 month Capri pants and they fit beautifully. Nettie is thin. She always has been. She was born at four pounds versus her twin brother's 5lbs 11ounces because (as the doctor said) they did not share well in utero. Nettie spent ten days in the NICU and fought me tooth and nail with breast feeding. The battle raged for a good month before she gave in. Although she likes her sweets, her choices at a birthday party usually include the crudités and cheese or grilled chicken if available. Needless to say she is thin. We hear about it all the time and the advice on how to make her less thin has been given in copious amounts since day 1. However, since day 1 every doctor she has ever come in contact with has said the same thing about her weight. Nettie's weight is fine. She is growing appropriately and ALWAYS has. Eating raw vegetables and healthy proteins is a good thing!

This week I put Lil in a onesie that she wore for her first birthday party. It was too big. As the week has gone on I have been putting clothes on her that used to fit and watching as they get too big and fall off of her now thin frame.  I am talking about clothes she wore well over a year ago.  Time and again I see her grow too small for her clothes.  I cannot tell you how this tears at my heart.  There is just some kind of instinct in mothers that makes them want to fatten up their babies.  Or maybe it is just Italian mothers... or maybe it is just me. In any case, LillyAnna loses weight or does not gain any at each appointment and it is a problem. 

 

She continues to grow taller and that is what is saving us from an NG tube right now.  That is a tube that we would insert through her nose and down into her stomach each night.  It would slowly feed her throughout the night and in the morning we would take it out.  There are many issues with this as you can imagine.  The biggest being that she is not going to keep it in and will probably need to be held all night.  We cannot use normal tape on her face as it irritates her and can cause lesions.  A close second is that the "food" is still going into her stomach and therefore still has the potential to cause vomiting.  Third, we could cause damage inserting the tube each evening which could possibly cause lesions inside.  Hopefully it will not come to this but she has to start gaining weight which is not happening right now. 

The plan at this point is to meet with GI again and possibly do another scope as it has been a year and we still cannot determine the cause of her constant vomiting.  Dr. R would also like us to have a CT done of her head.  He is not terribly concerned about there being a tumor there but it is the only thing we have not checked and he wants to cover all of our bases.  We saw rheumatology today.  This appointment filled me with mixed emotions.

If I had a nickel for each time I heard the words "but she looks so healthy!" I would never have to
work again.  It is hard to believe that Lil vomits multiple times a day, does not eat, has painful pustules that pop up all over her body, gets very uncomfortable, complains of pain and feels lethargic.  She is just so happy and lively most of the time.  However, as the wonderful doctor was examining her today she pointed out to me things I did not notice before.  Like how LillyAnna favors one leg over the other, how she cries out when her leg is bent at the knee, how she does not bend her leg when she walks and a host other small things I never noticed but are signs that the pain I believe she is in is real.  That validation was just priceless.  However, finding out that the doctor believes Lil could have arthritis is terrifying.  It makes sense and we knew it was a possibility but to know that she already has arthritis and it will not go away for the rest of her life makes ME want to vomit.  We took some x-rays today and will talk to the doc on Tuesday to find out more.  I will be better about keeping you updated.  Your support means more than you will ever know.  I am not good at showing it because I feel stunted by the idea that I can never repay you, can never show you how huge your support is no matter what form it takes and I am sorry for that flaw. 

 

I also want to say welcome to the world Antonella Rose Ventura.... our new cousin. 

We love you so much:)

Quick Update 6

We saw Dr R today because of Lil's iron deficiency anemia and the new things I am seeing with her skin.  Her temperatures have continued to fluctuate throughout the day.  She is getting new pustules quickly, they get very red and swollen and then go away almost completely on their own in a matter of a day.  She continues to throw up each night usually more than once and usually a few times a day too.  The biggest problem today was that she has lost more weight.  She did grow in length and that is a good sign that she is getting enough nutrition but losing weight is an issue.  I have also been concerned about dehydration because I now have to limit her milk intake and that is all our stubborn little girl will drink.  I am also trying to get her off the bottle but after three days I had to give in on something and today she drank about 15 ounces of pediasure but she won and drank them from bottle.  If she continues to accept the pediasure (something she has refused to drink in the past) that will hopefully help with her nutrition and weight.  I am afraid once she realizes she is going to have her precious bottle available more often again she will get mad about the pediasure and begin her strike once again. 

Dr. R is going to get his assistant on the NIH since I am not allowed to contact them to find out what is going on and if I need to I have Senator Carper's office still willing to help.  We are going to keep a food diary for three days to see if she is even taking in enough food.  Dr R is wondering whether she is simply not taking enough in or if she is not absorbing the nutrients from the food that is making it to her stomach.  We are also going to get weights from her regular pediatrician and plot them on the growth chart from the hospital to help us determine how dramatic the weight loss really is based on her whole history. 

Dr R did not look happy today.  Her blood work did not look bad from what I could tell except that her sed rate is climbing up little by little.  We will just have to keep an eye on things for now and stay the course.

Little Miss Birthday

LillyAnna,
I am not sure how someone who cannot speak in full sentences is able to inspire me but you do. Today is your second birthday. We celebrated with a beautiful birthday party surrounded by the people who love you the most. Last year we were celebrating in the hospital.  I have had to make some drastic life changes. In this last year, your strength has inspired me to do and be things I have only ever hoped.                                                                                                          Last year you were pretty obsessed with Monsters Inc. It is funny, your brother and sister were pretty much not allowed to even see a TV until they were two but you had already had a favorite movie at one! Of course, you have extenuating circumstances. Throughout this year you have stayed in love with Mike, Sully and Boo but now that the year is ending you are also enjoying Elmo and Mickey. Actually, Mickey is in the lead right now! Which is good because we are supposed to be going to Disney as a treat from your grandparents later this year. I hope that goes through for your sake. You deserve to smile.

You also really enjoy singing the Batman song. For Halloween this year you picked out a pink batgirl costume. I feel like that sums you up pretty well. You are not afraid to love what you love and it is a mixed bag. You will sit and play dolls with your sister and then turn and play trucks with your brother. I will be in the kitchen baking and turn around in response to "Mom!" only to see you smiling up at me with play high heels on your feet (usually mismatched), a Batman cape on and a princess crown holding hair in a birds nest on top of your head and pushing a good amount down into your eyes grinning from ear to ear.

You do not eat very much but when you do it is all about chocolate.... chocolate, chicken (which you call eggy), cucumbers and macaroni and cheese. Unfortunately, you throw most of it up. You also love my buttercream icing. One of my favorite things is when you sit in your high chair (although you  boycotted the high hair about a week ago) and I give you spoonfuls of buttercream icing while I bake. You get so excited and happy each time I hand you the spoon and you take your time licking the icing with your eyes trained on Monsters Inc. You have even started potty training and just today you went the whole day in panties. You had three accidents but you are only just 2! When the doctor asked why I  was potty training you I told him you insisted and that is exactly what happens with you. You insisted on being potty trained. You insist on continuing to use a bottle.  You insisted on giving up the high chair. You insist on enjoying life.

 

We spend a lot of time together, you and I. For the past year I have been on a leave of absence from teaching to care for you and this summer I resigned my position with the school district. You need me and it is that simple. You cannot go to any kind of daycare because the medicines you are on weaken your immune system and you have not had your vaccinations since six months. But really your disease just keeps surprising us and there is no other case like yours that we can find. So you need me. In order to make things work I am bartending and baking. It has been a hard transition. 

It goes against everything I did with the twins but I rock you to sleep each night.  You know how to put yourself to sleep and have been able to since about 6 weeks but you throw up so much at night that it does not seem right to make you go through that alone.  We are home together all day and while the twins go to school a few hours a day, it is just you and I.  I also rock you to sleep at naptime.  If I go to the store, you go too.  If I am in the bathroom, you are there too.  I can't even take a shower without you opening and closing the door every two minutes.  If I am being completely honest, I don't mind at all.

Your brother and sister who are now only 4 years old take such good care of you.  Whenever they are not around we have roll call. "Where Bubby?" "Where NetNet?" Where DaDa?"  They worry about you so much.  With all of that worry and all of the attention you get, one would think that they would be jealous or angry with you.  I have never seen Nettie get aggressive with you, ever.  No matter how mad at you she gets.  Your brother gives you a smack with his gaga every once in a while but nothing major.  Today I caught him holding his ears when we went to get blood work.  I was wondering what he was doing until a few minutes after they stuck you he took his hands away and said, "Is she done crying? Is she ok? "  Your sister swears she knows how much you threw up each night because when she tries really hard she can see through the wall to check on you.  They make sure you are in your car seat correctly, give you their last piece of candy if you are crying, and always check that you have a space to sit. 
I am so sorry.  I can't fix this pain for you and I can't figure out how to help you.  I am the one who is here with you all of the time and we are so close.  When you are in pain I can tell and when you do not feel good I can tell.  It is all up to me to stand up for you and to make sure everyone knows what it is like to be you, what you are going through.  I don't think I do a very good job.  Doubting myself is just something I have always done.  I wish I could say I am one of those moms who is like a mother tiger and springs into action when her baby is in need; but at this I fail you.  I allow people to talk me out of what I know in my heart to be true.  Being over sympathetic to my kids has been a fault for the last four years.  I guess there are worst faults in a mother.  But with you I know when I am right and most times I am right.  You just play it off so well.  The distractions of the world invigorate you.  You steal energy from being around people and it fulfills you.  At night when we are alone and you are squirming in discomfort from who knows what pain or when you are asking  for your bin to throw up for the umpteenth time, I know I was right.  Your love of life and your strength is what gets me up each day.  It is what keeps me going when I feel like the world is collapsing on top of me.  Because of your illness I have changed my entire life and my own strength astounds me.  But it is all for you and because of you.  Your brother and sister deserve me too and their annual birthday letters will show their special connections to me but this is your letter.  This is your second birthday.  I am so afraid for your future but I am so enjoying your present.  Happy Birthday little one.  I love you. 
Mom

Quick Update 5

Symptoms of iron deficiency anemia

By Mayo Clinic staff

Initially, iron deficiency anemia can be so mild that it goes unnoticed. But as the body becomes more deficient in iron and anemia worsens, the signs and symptoms intensify.

Iron deficiency anemia symptoms may include:

·         Extreme fatigue

·         Pale skin

·         Weakness

·         Shortness of breath

·         Headache

·         Dizziness or lightheadedness

·         Cold hands and feet

·         Irritability

·         Inflammation or soreness of your tongue

·         Brittle nails

·         Fast heartbeat

·         Unusual cravings for non-nutritive substances, such as ice, dirt or starch

·         Poor appetite, especially in infants and children with iron deficiency anemia

·         An uncomfortable tingling or crawling feeling in your legs (restless legs syndrome)

·         Shortness of breath

·         Headache

·         Dizziness or lightheadedness

·         Cold hands and feet

·         Irritability

·         Inflammation or soreness of your tongue

·         Brittle nails

·         Fast heartbeat

·         Unusual cravings for non-nutritive substances, such as ice, dirt or starch

·         Poor appetite, especially in infants and children with iron deficiency anemia

·         An uncomfortable tingling or crawling feeling in your legs (restless legs syndrome)

Now I know why she has been grabbing at her legs and feet and saying owie. And her head and mouth and saying owie. Why she has not been letting me put her down much or sleeping well and why when I do hold her, she is putting her head on my shoulder or squeezing my neck so hard it makes me want to cry (or take a picture). I know why she has been falling a lot too. Blood work today shows that Lil has iron deficiency anemia. 

The doc says not to freak out. At least I know I was right and something has really been bothering her.  I do not know how serious this is yet.  It could be just because she drinks so much milk.  That is what the good doc thinks it might be.  It could be that she is bleeding internally so we are going to get a stool sample Tuesday to make sure.  Of course any issue with red blood cells makes me worry about lymphoma which is one of the possible side effects of Humira. 

I have to talk to Dr R about if this could also be the reason her lesions are acting up and her temperature has been going up and down like a roller coaster.  Seriously, her temp will go from 98.9 to 100.6 back to 99.9 in a matter of two hours.  Her lesions are acting up with the same speed.  I actually sat and watched three pustules pop up, get red and sore and pus filled again within hours and then go back down again in the next 24 hours.

The Little House

I do not like to complain and I do not like to feel weak.  It seems to me that the more you complain, the worse things seem.  Then they actually get worse.  It is not just about complaining out loud either it is also the complaining you do in your head.  I try to combat this cycle by thinking positively and comparing my situation with others that have it much worse.  This usually works for me.  Not wanting to feel weak is a separate issue but works in the same way.  If you start down the slippery slope of helplessness, you are at the bottom in the blink of an eye.

My problem right now is that there is so little room for error. 

The smallest things (or what has generally counted for small in my mind) now nudge me with their gnarly little toes down that slope as I am grasping and clawing for dear life just to stay where I am.  A good friend reminded me that I started this blog to help myself.  It is hard to be honest.  I want to be that strong, positive person even here where there is a strange sense of freedom and safety but I also feel that nudge and I will do whatever it takes not to slip further.  If that means talking about how we have no money or talking about how weak I feel or talking about how I am feeling sorry for myself right now than that is what it means.  I will rise above it. 

Life sucks right now.

I have given up my career (yes temporarily).  It is hard for me to not be a teacher.  I have been teaching for thirteen years.  Although it was not always wine and roses, it fulfilled me and made me happy.  I could go to bed at night knowing I was doing good.  The notes, emails and comments I have received from former parents since I announced my resignation are heartfelt and they have one theme in common… I did good and I made a difference. I truly believe that all jobs have their importance and meaning.  My husband is a plumber for example… I mean really, where would we be without toilets and running water?  I find meaning in my current job of bartending and serving.  I look at each table or customer I have.  I think about what brought them out to dinner, what trials and tribulations they have suffered, what they might be celebrating and I think about how I can make that experience better for them.  Or even how I can make their day better by simply being kind or making sure they have full drinks or giving them good food that will nourish their bodies and souls and make them feel good.  I can hear the groans now!  I know it is cheesy and over the top but it is really what I think so just suppress your disbelief and keep your eyes in your head.  But making an impact on a child’s life is special and it gave me meaning.  For now, I have to give that up.  I also have to give up the money that goes with it which is very scary and very hard and makes me a little resentful.  I do not want to have to tell my son that he cannot get the Marvel Comic ice pops because they are $3.99.  But the truth is, I can still get him the Jolly Rancher ones that are on sale for a dollar and that is something.

I have a headache twenty four hours a day, seven days a week.  I take pain killers for them.  The amount I have to take just to get through the day without vomiting just reminds me of my mother's addiction which eventually killed her.  The pain makes me irritable and it never goes away.  That is not exactly true.  It went away for about 15 minutes the other day and I was so excited.  If you have ever taken hard core pain killers you know the joys that go along with them.   Constipation, stomach upset, etc etc.  Good times my friends, good times.  The stress and exhaustion from worrying about Lil and money and the twins and my husband and the late nights three times a week are causing all sorts of other stuff to go wrong with my body too. 

My daughter is sick.  LillyAnna still vomits every night.  She is no longer vomiting twenty times a day and that is good.  But I know, and this is not me being negative, it will come back.  And if the pattern stays true to form it will be even worse next time.  We have avoided doing something more drastic because she gained 0.1 ounce at her last appointment.  When I asked Dr. R what else we were going to do he gave me a look that said "this is as good as it is going to get Gina".   The medications we are giving her are awful.  She is in a good cycle right now but a good cycle still means elevated temperatures, complaints of pain in her arms and legs, vomiting, bloodwork, hair loss, injections, medication schedules, trouble sleeping because of some kind of discomfort and the great unknown of this whole disease.  This all starts to seem like normal but when I tell someone the story I realize how truly remarkable it is.  She is the seventeenth reported case in the whole world!  We know nothing.  There is no help out there for us.  No website, no information on the internet, no "this is what you can expect", no foundation, and the only support group has become a bit of a life line and that is Chronically Cool Families group at AI or what I like to call The Island of Misfit Toys. 

I was almost in tears there the other night.  Sometimes I just feel like maybe I am crazy.  People look at me like I am nuts when I tell the story, I say she is not feeling good or describe how we know NOTHING.  Especially when she is running around looking and acting like the most perfect baby alive.  I shared this during the group and I will be damned if not every parent sat up a little straighter and started nodding until finally my good friend said, "Gina, we all feel that way."  At least I knew I was not crazy but it is also scary that my situation fits in with those situations because those kids are sick.  And then I remember that my kid is sick.

I feel guilty for complaining.  My baby goes home from that hospital.  My baby is not dying.  She can walk, she can breath, she eats, she is smart, and on and on.   Then I chide myself for feeling guilty because I know that she is still sick and that this is still traumatic to my family.  My son is a mess with worry.  How does a four year old who is smart and intuitive handle all of this, what is it doing to him? I don't know and I hope he will find a way to channel it in a positive way but I still worry.   You can see it in his eyes, he knows too much.  The therapists tell me not to talk about it in front of him.  It seems we forget too often. 

I am not who I used to be.  Listen.  I will be honest.  I am the friend who always forgets your birthday but buys you gifts that suit you for no reason.  I am the friend who will not call you back because I just simply forget but I know the ins and outs of your family and I pay attention.  I am the person who is over generous because I just like to make people happy.  Lately, I forget even more. Pay less attention.  Say the wrong thing.  REALLY never call back.  And has no money.  Who am I?

And even though we do not have the money for it we are having a big party for her birthday.  Last year we celebrated her birthday at AI and they did an amazing job of making it special.  But we were in the hospital.  This year we are not in the hospital and we are not in the same place we were so we are going to celebrate.  Thank you to all of you who are coming out to help us do that and thank you to all of you who are going to bring stuff (you will be getting a request soon grandparents! LOL!).  We are celebrating a very special little girl and her brother and sister too.  Just pray it does not rain and we do not fill the pool to bursting (we have VERY large families:)  Actually, let's fill it and make it the most fun party they have ever seen!!

I am sorry.  I am sorry I have to let you all buy me things, I am sorry I can't concentrate fully on the important things in your life.  I am so sorry I do not have the emotional energy to share your hurts.  I want to, I really want to.  This is just really getting to me and it is getting to my whole family.  It just keeps going on and I am getting so so tired.  I don't mean to give myself props but I play it off well.  I do not complain (save for this whiny post), I stay positive and I do whatever I have to in order to continue to give to other people and be the person I was.  Because of this and as time goes on, people are beginning to treat me as if my life is normal.  Like this is all a choice.  I cannot blame anyone.  I did ask you all to do just that and I stand by it.  I cannot continue to live life in crisis.  This is our life.  No matter how hard it is, it is life and we have to live it.  I am hoping that by getting this all out I will be able to get my head out of my ass, stop crying and feeling sorry for myself.  Most of all I would like to stop feeling angry.  I am angry and hurt.  It does not feel good.  I like it better when I can let things go.  So this is my rant.  I am letting go... right here and right now.  I am probably going to cry for a bit before bed but when I wake up tomorrow I am going to choose to have a better day and that is that.