Stone Soup

 

 

My instincts about where Lil is right now have been proven to be correct.  Our trip to the NIH did that for me... for us. 

It is just so amazing to go there and be where people know this disease well.  The "Angel Doc" as I dubbed her last year is just amazing, her knowledge is phenomenal.  But all of the people we come in contact with are truly wonderful and they know the disease.  Can you tell how meaningful that is for me?  Of course, everything we heard from them we will talk to Dr. R and Dr. B about and get their take on it as well before any final decisions are made but what we heard sounds pretty promising.

The NIH is suggesting that we increase Lil's Humira.  If you all remember correctly, Humira is not even FDA approved for Lil's age group.  Being immunosuppressed for a long period (and the more suppressed you are) comes with risk.  But they believe increasing it will give Lil a better quality of life.  I would like that... It is also something Dr. R and I have discussed.  The docs do not want to stop suppressing her immune system until she is pustule and papule free for one year.  At this point, she still gets pustules and papules daily and has pain 3-4 days a week. 

So what is the good news?  For starters she is in the 70th percentile on her growth chart.  Seeing the growth she has made since last year felt so good especially since she lost so much weight this winter with the development of her mouth lesions. 

It is the mouth lesions and genital lesions that may bring us some conclusions however.  There is a very rare disease called Behcet's disease or Behcet's syndrome that is consistent with this pattern.  Chris and I did some superficial research on it and we still have more to do.  The flip side is that she may not even have Behcet's and the bottom line is that there is no treatment for Behcet's.  We would be following the same treatment plan anyway. 

We also had to do another skin biopsy.  When they took the last sample, it grew fibroblasts but not keratinocytes.  They are not sure if this is a process of her disease or a problem with the sample.  So they took another sample to see.  The lead is that if it is a process of her disease that could tell them something about her disease process.  What that is I have no idea.  This is all high level stuff that only the Angel Doc understands.  Even the other doctors did not understand its significance!  Dermatology agreed with this assessment but they thought it was less important and most likely a problem with the sample. 

What else?  Lil can start living more of a normal life.  We can try giving her some vaccines!  She cannot get any live vaccines because she is on Humira but she can get more than she has now.  So what does that mean?  She can try to go to school.  They recommend we wait until the Spring and sign her up for just a month to see how it goes.  They mentioned that maybe she can go to school when cold and flu season is not in.  This is what many of the kids in our support group do.  Although it is not complete, it is a big deal. 

Her genetic screening is still not done.  This is pretty frustrating as we have been waiting for this for about two and a half years but they said it should be done in the next two weeks.  Her interferons are high as well.  I have no idea what that means but I am going to do some research on that as well, just no time yet.  I do know it is all part of the disease process they are tracking to see if they can figure out how to stop it. 

We need to keep her mouth especially clean this winter.  No mouth kisses, no sharing food and drinks.  They told us this may help reduce the mouth and lip lesions.  I will be sterilizing her stuff and am thankful for dishwashers! 

They probably do not want to see us for two years!  That is unless something crazy comes back from the tests they did or something happens with Lil. 

This is all huge for her and for us as a family.  It feels like we can begin to move on which as I said is how I have been feeling anyway.  Like I said, we have to review all of this with Dr. R and Dr. B and get their opinion.  They have the ultimate say in what we prescribe and how we treat her (and for good reason, they know her best).  But I believe they will agree with most if not all of it. 

Between all of this and finding our wonderful nanny, I feel like I can go back to work.  The problem with my career is that it is too late for me to do that this year.  We have one more year to live like this as long as nothing big happens with Lil.  It scares me to death to be honest because I feel like every time I relax and think we are in a good place, something big does happen.   So I will be on edge but this is all a process.  This winter will tell us so much. 

One Foot Two Foot Red Foot Blue Foot

People always tell me I am a strong person.  You all tell me all the time that I am an inspiration to you.  I never felt like any of that was true.  I always felt like I do what I have to do.  Your mom dies and you are 9 months pregnant, what choice do you have but to keep putting one foot in front of the other?  Your baby gets one of the most rare diseases in the world but again what choice do you have?  You put one foot in front of the other.  In between all of that you have life's normal blows. 

My sister dying has basically taken me to my knees.  I feel like I must be doing something wrong in this life.  I just try so hard to be a good person, to do the right thing, but it always seems like God is punching me in the gut and saying "How about this? Can you take this too?"  The answer right now is no.  No I cannot take any more. 

I want to focus on the positives.  We just found the best nanny for LillyAnna.  She is exactly what we need for Lil.  She takes her out everyday, gets her moving and interested in life, she wants to start a homeschool with her, and on top of all of that my house is cleaner and laundry is done when I come home. 

LillyAnna has been doing well.  She has her bad days, days when she is logy or has pain.  She is still getting pustules but our life is finally starting to fall into a rhythm of taking care of her.  After three years we are figuring out life with Lil. 

Nettie is loving school and has matured so much.  She works hard and is becoming such a little woman. Nettie loves to dress up and is starting to be able to really play with Lil.  They have been playing dolls a lot.  Chris is just the most amazing little boy.  He is so smart and kind.  He is intuitive and amazes us every day.  We just finished reading The Twits (just the two of us) and it was so much fun. 

My husband and I have a strong relationship and I do not know what I would do without him.  He is such a strong support to me and he loves me so much.  His love and care is literally what gets me through some days.

My sadness just takes over some days.... and stress.  I feel like it is time for me to work more and we need the money so badly.  The financial strain of living like this for three years is really taking its toll.  It scares me to work more and be less in touch with Lil but now that I have a steady nanny that I can count on and who is so in tune with Lil and her needs, I feel like it is time to try to work more.  So if anyone knows of any part time, flexible work- I am available!  I am really thinking about going back to work full time next year. It scares me but I do believe it is something that has to be done.  This winter will tell all. 

Just thinking about all of this makes me so anxious and sad.  It makes me feel like I am giving up on Lil having a better life.  It is another level of accepting that this is her life and will be forever.  I feel like I have failed her.  I cannot make her better, no matter how much I am home with her.  This is it.  You would think I would know all of this after three years but accepting that your life is dramatically changed is a slow process.  I also do not know what it would be like to work full time, if I could even do it while still taking good care of her.  However, I know it is time to try.  We are struggling right now to pay our bills let alone do anything more than that.  I cannot let the twins do things they want to do, I cannot even buy them shoes right now.  As I have said before, we cannot live on people's generosity anymore so please do not take this as anything but me putting my feelings out and sharing what our life is like. 

I am scared, I am sad, I am disappointed in myself.  I failed my sister and now I am failing my baby.  Many of you will think that is ridiculous.  You will tell me how ridiculous it is but I am telling you how I feel.  I am also trying to get out of this rut.  I am trying very hard.  I am trying to focus on gratitude.  I am taking each day as it comes, enjoying every minute with my kids and trying to love every minute of my life but I am down right now.  I am down in that place where most times even putting one foot in front of the other seems insurmountable.  I put a smile on my face because it makes me feel better and it is what people want to see. 

Grief comes in waves.  This is a wave.  It is just a big one and one that has knocked me down hard.  But I am getting up, slowly and surely.  I know I will feel better.  I am so lucky in so many ways. Thank you for listening. 

The Birthday Queen

 

 

My Dearest LillyAnna,
            This week we celebrated your Fourth birthday.  This year you have grown in so many ways.  I am amazed every day by you.  Each day you show us a strength and love of life that is beyond anything I could imagine.  You still wake up most days not feeling very well but it never stops you.  You live with pain almost every day but you have continued to meet milestones that three years ago I would never have thought you could meet. 
            This year you had your first sleep over.  Of course it was a family sleepover and not a friend sleepover but you loved the independence just the same.  You take your suitcase out and pack it up all by yourself, you fill it with favorite books and some candy.  I have to remind you that clothes need room too! 
             You also always have to have your green or blue blankie.  You call them your "shovey" blankets because they are trimmed in satin material and when you drink a bottle (yes, you still drink a bottle) you rub your little hands on the soft edges.  I love it because an old friend of mine is the one who made them (Thank you Michele) for the twins when they were born but you have taken them as yours.  They go everywhere you go, or at least one of them.  At night when we lay with you as you go to sleep (yes, we still lay with you to go to sleep) you pull that blanket up to your chest and flatten it out so you can "shove" on it.  You pick whether you want to lay with me or your dad.  For most of this year you had to have daddy at night but just recently you have wanted me.  And after your shots, which you still get weekly, you always want me too. 

             The coaches at Graylyn Crest let you join the swim team this year even though you could not really swim.  This was huge for you because you are not allowed to go to school.  Being a part of the team made you feel like such a big girl.  I will never be able to thank Coach Amber enough for how she included you and made you feel like part of that team.  Now it is the end of the summer and you are swimming.  Of course, your winter swim coach (Danielle) had a lot to do with that too!  You hid your swimming from me for weeks.  I could not believe it.  You would not let any of us see that you could swim but Melissa caught you swimming on your own one day and then it was all over.  You have been all over the pool and I have been right by your side.  You have the biggest look of accomplishment on your face each time you swim further.  The best part of swimming this summer is that it has helped with your pain.  One day I asked if you needed pain medicine (you were obviously hurting) and you told me you did not need it because you were going to go swimming and that makes you feel better.  How are you this aware at 4?  It blows my mind. 

             This year you were a Philadelphia Mummer for the first time.  We all ended up in the show believe it or not.  You were so sick and ended up in the hospital the next day but true to you, you refused to leave the Convention Center and stayed for both shows.  Poppop was so proud and Golden Crown even dedicated that year's show to you.  We were all in awe of your determination to be in the "Big Show".  What we discovered the next day was that your mouth was full of mouth sores that turned out to be Pyoderma lesions.  This is particularly scary, you are only the 6th case as far as we know of PG in the mouth.  These mouth sores have affected you all year but again, you keep on. 
            The other big milestone this year is that you have started riding your big girl bike.  We are not taking the training wheels off yet but you are determined to keep up with your brother and sister.  You speed down the street behind them trying to catch up.  Again, your look of accomplishment is priceless. 

             Your favorite things to eat have not changed much.  You love my meatballs and gravy and can already roll a meatball all by yourself.  You can stuff peppers and crack eggs, you can even separate them!  It is your favorite thing to stand at the kitchen counter and cook with me or stand at the sink and 'wash dishes' while I am cooking.  Chicken and chocolate and pasta are your favorite foods. 
            You are a four year old in every sense of the word.  I watch you wanting to play and engage with Nettie and her friends even though part of you still wants to hang back and be with me.  You are trying to figure out how this whole social structure works and where you fit into it all.  My heart hurts for you that you do not have your own group of friends.  You not being able to go to school is not easy for any of us.  You want to go to school so badly but the doctors are still telling us it is not a possibility.  I am hoping that will change for you but who knows what is going to happen.  For now, you make friends when you can.  You have made two friends at the pool and it is fun to watch you interact with them but it also hurts me to watch sometimes because I want you to have everything. 
          I think we have finally found a nanny who will meet your needs wonderfully.  It is a hard expense on us but you need someone who is going to stretch your thinking and get you moving when you are able.  Each day I come home after you have been with her, you are just so happy.  I have not been emotionally available to you and Chris and Nettie and Daddy lately.  I lost my sister this year.  I know I cannot allow my sadness to affect you but it is so hard.  I am so sad and so anxious.  You do not deserve it and you do not need it.  You have your own battles to fight.  But someday you will understand the love between sisters, its ferocity. 

         Your party was especially fun for you this year.  You were so ridiculously happy to have everyone around.  No one could believe you chose a Star Wars theme.  The funny thing is, you wanted that last year too!  You are definitely your own person girl!  You really loved opening all of your presents.  I am always excited for Christmas but after watching your excitement when opening gifts this year I am even more excited.

           I love you LillyAnna Rose.  I hope I am doing everything I can for you.  I want you to have everything in life you deserve.  Knowing how much pain you are in some days, knowing how badly you fight to live the biggest life you can, I can only hope I am guiding you the way I should be.  There is no road map for you.  We are on our own path.  I just want it to be amazing for you in its own way.  We all love you so much.  Happy Birthday.
                                                                             Love, Mom