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.