Thanksgiving is...

I created this blog for two reasons.  The first was to be able to tell about Lil's disease in detail and what it is like to be her mom without the pressures of facebook.  The other was to spread the word about her symptoms in the hopes of helping people who may be experiencing something similar or possibly even help Lil.  Unfortunately for all of you, the side effect of that is you hear an awful lot of negative things about our lives.  You do not often hear about how grateful I am for all that we have. 

I know how very lucky we are that LillyAnna can run and play, that she is smart and can think on her own.  The physical therapists we worked with at AI were absolutely astonished she began walking during her time there with the excruciating pain she experienced in her legs but she did and she can still walk.  She can eat, drink, walk, and breath.  My favorite is that she can play.  She loves to play.  She pays for it the next day or like today she paid for it tonight either by vomiting or having a lot of pain.  But this girl can play.  And we would never live this life without our support system.  We would not be here without our doctors and nurses and hospital staff.  We have a house, we have heat, we have money to buy food.  We have all of you. 

Then there are the little things.  The smell of sautéing garlic, my kids hair when they wake up in the morning and roses.  The feeling of jumping into a pool on a hot day, sand in your toes, the memory of my grandmother.  Reading.  Cucumbers.  Cuddles.  Laughing with your best friend. 

I know how lucky we are, how lucky Lil is.  Sometimes I need reminding of how good we have it and sometimes I need reminding of how hard we work.  Depends on the day.  Today I enjoyed the day, enjoyed the cuddles and my family... oh and turkey too:) 

Absolutely Almost

It took the nurses and social workers at AI a few months of kind cajoling to get Chris and I to attend our first support group meeting.  I kept thinking , and telling them, that we did not belong there.  I knew that so many people who went to that group had kids who are in wheelchairs or were as medically complex as our beautiful friend Lucy.  But the more groups we attended the more I realized that we did belong there. I realized that Lil's pain, her medical issues and how it affects our family put us right in the mix.  Last night at our meeting, a wonderful new family to our group leaned over to me at the end and whispered, "I feel like maybe we don't belong here.  She is not as complex as kids like yours."  I started with a smile and proceeded to tell her why she too belongs here. 

I truly believe we, our little family, is here in this place for a reason. 

So how is Lil?  We are a little perplexed to be honest.  She is not coming out of this flare as quickly as we thought she would, as quickly as she has come out of flares before.  This is especially strange because we just increased her Methotrexate.  If you remember, the docs at the NIH noted that the Methotrexate had more of an effect than the Humira.  So has the methotrexate hit its plateau? 

The NIH also asked us to get more blood work in their concern for her kidney and liver numbers.  Those numbers were still elevated.  They are not very high but elevated and staying elevated.  My concern is that she is saying she has pain in her belly and side and those numbers are elevated. 

She is limping when she wakes up telling me she has pain in her legs and hips.  This usually works itself out in an hour or so but it should be controlled with the increase in the methotrexate.  In reading the Facebook page on Pyoderma Gangrenosum I am learning so much about the pain that comes along with this disease.  The adults that have the disease say that even loud noises and vibrations can cause the "PG" pain to act up.  Lil has been having these crazy temper tantrums, it takes almost nothing to set her off.  I am wondering if she is just walking the line of keeping herself under control between having some level of pain and because she is three that when she loses it, she really loses it.  Little Chris had some serious temper tantrums at three but Lil is putting him to shame.  She does not have them often but they come out of nowhere and they are major and then she falls right to sleep. 

The other piece of the puzzle is that she is out and out refusing, and I mean refusing to take her medications.  It started about two months ago and I remember we were in New York visiting my friend Alana.  I was fighting with Lil to take her meds and Alana asked why I thought she was fighting so hard. My response was that I think she is just tired of it all.  I mean think about how you feel after being sick for a week or two.  For over two years, every night she has taken 4-8 vials of oral medication some of which tastes terrible no matter how they flavor it.  There is nothing she eats consistently enough that I can put it in food.  There are still days she just simply does not eat.  I reached out to the docs, our wonderful nurse friends... We are spending 2 hours a night trying to convince her to take the meds only to have to sit on her and squeeze her already ulcerated lips and cheeks so I can get the syringe to the back of her cheek which leaves me and her crying.  The best part?  She throws it all up in the end.  So I have stopped.  Thursday night we had a breakthrough!  I filled a couple of syringes with Gatorade and gave them to the twins and they all took 'medicine' together.  She took 3 of 5 with no fussing.  I was all smiles.  Before bed, she threw up anyway.  I just CAN NOT WIN!  My only consolation is that maybe she would have been throwing up every night anyway. 

Overall she is not as bad as she was before the NIH but she is not 100%.  It is just so frustrating to see this continue to go on and on and on.  The twins know she is better than before but they also hear us fight with her every night, they lay in their beds in the dark and hear her throw up.  They watch her run around and cough and us grab a bin.  I am so thankful for their school though.  Chris flat out told me he feels very safe with his teachers.  Both of their teachers told me how the twins are able to talk about Lil in school.  This is so huge for Chris.  Nettie is much better at talking about her feelings. 

We also had a talk about how the Congressional Visit went while I was at the NIH.  Here is what I have learned.  Families with kids with rare chronic illnesses have to do crazy things to take care of their kids.  They sell their homes and buy smaller ones.  They get divorced and sometimes actually have to live apart for a time to prove it.  When we applied for SSI the social worker was so touched by our story she was in tears.  Very kindly she told us if Chris could figure out how to lose his job she could help us because he makes too much money.  Too much money is over $25,000 a year.  We are supposed to support a family of 5, one who is medically complex, on $25,000 a year?!?!  I am glad Congress wants to help but it is going to take some serious legislation to help and I am worried that there are just not enough of us to make a change.  Someone mentioned they heard that there was going to be a change in that we would be allowed to have a savings account for our medically complex kids that would not affect their Medicaid.  Wahoo!  Thanks a lot (insert sarcasm).  One of the new things I have been reading about on the PG Facebook page is adults having to get amputations.  Once again, I have no idea what the future holds for Lil. 

The reason Chris and I have been able to keep living the life we live is you.  I am not blowing smoke here.  I am being totally serious.  We have actually talked about having to sell the house and downsize.  Or move to an area where houses cost less.  I just cannot work as much when she is very sick.  Is this fair?  I don't know.  Should we have a donation page?  I don't know.  It is something Chris and I talk about all the time.  How we spend money, how careful we are is a constant conversation in our house. I do not know what is going to happen but I can tell you we are doing the best we can to be responsible and we appreciate you so much.  You have kept life stable and normal for our kids, you have given our kids the little things that make their lives better, healthier.  I see how the government helps other people.  Should they be helping us? The conversation we had last night with others in our position was wonderful.  All we can do is keep pushing. 

I love how she is just chillin' talking to her aunt in this picture!  Aunt CoCo is trying to convince her to take her meds and Lil is calmly telling her all the reasons she does not want to!

All About Me

I love music, all kinds.  I love opera (although I have not been able to go in years), I love classical, I love blues, and I love old school rap. Finding this new radio station that plays all old school rap that I listened to in high school has been a goldmine.  The other day I was driving alone in the car and a song came on that brought out my inner Snoop and before I knew it was bee bopping along singing my heart out.  And then it was like someone threw a cold bucket of ice water on my head and I stopped. 


Chris has been really worried about me.  He says I am miserable.  I just stare off into space and I cannot relax.  He is right.  We have been blaming it on my headaches but actually my headaches have been slightly better.  So what is it?  What has me crying in parking lots and unable to sleep?  Why can't I enjoy a song in the car? Why am I at a kids' birthday party staring out the window?  I do nothing for myself.  I do not go out with my friends...ever.  I don't go to yoga or even spend time with my husband.  If Lil is starting to feel better, then why?

All I want to do is be with her.  Right now she is what makes me happy.  Yes, the twins make me happy.  Chris, when you read this, I am sorry.  I love you.  Although I do not want to compare Lil to a small sweet child who is dying I remember once when Lucy was dying I was sitting in the room talking to Nicole, her mom.  We were talking about how everyone kept telling her to eat.  She just looked in my eyes and said "Eat?" and  we both shared a moment of understanding.  That understanding was not necessarily about a dying child.  For me it was about a child in pain.  When your baby, your baby who you are supposed to protect and care for and advocate for and take care of, that helpless child with her still soft and chubby arms and feet, and kissable cheeks, who likes to cuddles and have blankets is in pain you do not want to do anything for yourself.  You just do not care.  That whole thing about 'if you don't take care of yourself you can't take care of her' is bullshit.  When she can't eat for days at a time because her belly hurts so much you do not want to eat.  When all she wants to do is sit and watch movies because her hips hurt, you want to carry her around.  My little girl can't wear socks some days because it hurts too much.  Socks hurt too much.  As a mother how am I supposed to allow myself to enjoy anything when my baby's feet hurt so much that she cannot wear socks?  Or her mouth, from ulcers, hurts so much she cannot eat? 

How can I feel ok about who I am as a person when I then have to sit on that beautiful baby and squeeze her cheeks while she screams that I am hurting her to get her to take 4-7 vials of medicine every night only to watch to her vomit it all up.  I have tried everything else, I am as gentle as I can be, I have reached out to the doctor and our nurses.  I can't stop thinking about it and I hate myself for not being able to figure it out.

My mother in law has recently helped us find a Pyoderma Gangrenosum Facebook group.  Chris is very concerned about me reading it.  The problem is, and he agrees with this, we are learning so much.  When these adults describe their pain and daily struggles, we know how Lil feels.  For example, she will sometimes say her scars hurt.  I always felt torn by that when there are no open wounds on them.  Why would they hurt when they look so good?  Until the other day when I found a post on this group from a person who described what his scars, which are 4 years old feel like.  Here are some phrases from him and comments from him and his fellow PG sufferers:  intense, insane, life changing pain, exploding from the inside out, pliers ripping my toes off, searing hot knife constantly slashing my flesh, vice grip constantly gripping my foot, red hot ants trying to chew their way out of my flesh, pain never goes away, issues depending on movement and how clothes affect my skin, hurts now as much as it did when it was open.  Many comments just simply agreed with the descriptions and talked about pain killer options.  These terms have been running through my brain like a continual recording. 

And so, is there emotional energy leftover for Chris and Nettie?  How about my husband?  Where do I fall in the hierarchy?  Exactly...you do the math. When people compare their lives to mine mentioning that mine is more difficult I always say everything is relative and I mean it.  However, in this case I have to say only a few moms can understand what I mean.  Moms who have seen their kids in pain know what I mean but only to a certain extent.  Moms who have watched their kids live with pain over long periods of time, who have had people disbelieve their pain, who have had to fight for them day in and day out.  These moms can truly sympathize with the depths of my pain and sorrow right now. 

So why am I sharing this self pity with you?  I am usually pretty good at dealing with things while they are going on; I pull on my big girl boots, lace them up tight and do what has to be done.  But when things start to calm down, that is when my strength wears thin.  It is always after she starts to feel better that I fall apart.  Don't worry.  My pendulum will fall back to middle, it is just a matter of time. 

Have You Seen My Dragon?

It has taken me a few days to process everything we saw and heard.  One thing everyone has to understand is that we are not going to have answers from this trip and we may never have answers.  Another thing is that I know it is hard for those of you who are close to us to get 'the story' from the blog but at least if you read the blog you have the base story and we can discuss the rest with you.  As Chris likes to put it, "Even I read the blog!"  

I am going to give you the details I feel are necessary for you to get the big picture.  If you have questions I am happy to answer them!  There is a comment section on the blog that I am not very good at utilizing but I would like to get better at using.  We can also use Facebook.  Those of you who are close family members, we will talk about it, of course!

The NIH is doing a clinical research study on a group of auto-inflammatory diseases.  Pyoderma Gangrenosum is the process of an auto-inflammatory disease.  It is not actually a stand alone disease.  That is why it is so strange in infants.  In adults it is the process of, or it comes along with, Chrons or Ulcerative Colitis or something like that but in infants it comes without an underlying disease.  There is no prognosis for Lil's disease. 

On top of this, Lil's case is extremely unusual as I understand it.  I told you before, as the doctors were looking at her wounds they used words like extraordinary, amazing, awesome, unusual.  When you are in a place like the NIH where they have seen and treated multiple cases of adult PG and you hear this... it is not good.  Through the fundraiser my mother in law set up (thank you thank you thank you!) we have finally found another case of Infantile PG.  I have not contacted the family yet but I plan to this week.  They just returned from the NIH.  However, Lil's disease does not look like that child's.  In fact, her disease does not look like any other case they have seen.  The angel doc said they have seen one case that looks a little bit like hers but she is their outlier. 

They want to know why the Humira did not have as much of an impact on her disease as the methotrexate.  Methotrexate is definitely effecting her disease more.  We just increased her methotrexate again so they want to see what is going to happen.   If her flares reduce they will see us in one year.  If they do not, they will see us in six months.  There is one caveat to that scenario. 

LillyAnna's original blood work came back with double negative T cells, a very high number of them.  What does this mean?  Well, this is what has taken me so long to research.  The first response would be to assume she has Alps disease which is a horrible disease.  She DOES NOT have Alps disease.  That leaves the question of why the high number of double negative T cells? 

Extremely simplified:  T cells are made in your bone marrow and are an integral part of the inflammatory response.  Starts to make sense.  Big Picture?  I still do not know.  I have made an appointment with Dr. R to discuss the trip and these results.  At one point we had talked about a bone marrow transplant with Lil.  But as Dr. R very bluntly put it "bone marrow transplants can kill kids" and we were not at a point that Lil was that bad.  By the way, I love that Dr. R is that blunt.  I need that.  No bullshit.  The angel doc is not even close to recommending that either.  Before we left she ran more blood work using Chris as the control.  Dr. R also has some data on this from Lil's previous  hospital stays.  Basically we are still looking into it.  But it is the big white elephant in my brain.

Lil's liver levels are still elevated.  This is common immediately after she gets her medication.  However, we took this blood work three days after her methotrexate and Humira so the levels should have gone down.  This is the second time this has happened this month.  There is some evidence here that her liver is showing that it is unable to handle the amount of medication we are giving it.  What happens next?  I do not know.  I could not ask.  It is one of those 'we will cross that bridge when we come to it' issues.  We will get blood work again in a couple of weeks.  Unfortunately we just increased the methotrexate and that is the medication that is having the biggest impact.  It is also the medication that is most likely causing the liver dysfunction.

She does not have Lupus!  Good news!

Iron is consistently low enough that the angel doc wants us to give her iron supplements daily.  Have you ever smelled liquid iron supplements?  They taste and smell like pennies.  I can't even get her to eat some days....

One thing that has been gnawing at me, Dr. R and Dr. Brescia is that her inflammatory markers do not go up when she has a flare.  The angel doc told us this is normal for PG.  It made me feel so much less crazy!!!!!

I met Lucy's mom in the laundry room at AI.  We started talking and she began to lead me along this yellow brick road of having a chronically ill child and raising a family at the same time.  While in the play room at the Children's Inn at the NIH I met a lovely woman who was at the same place I was when I met Lucy's mom.  I was able to begin her walk down that yellow brick road and she thanked me.  She seemed so much more calm when I said good bye.  Her boys where so beautiful.  I hope I did Lucy proud.  Lucy's life had so much meaning for so many people but this is Lucy's legacy for me, to be able to help families who are just beginning this journey the way that Lucy's mom helped me.
We are doing it Lucy:) 

Just Me and My Little Sister

I remember when Lil was born and we used to read the book Just Me and My Little Sister.  My favorite part was when they go to the park and the little girl (the new baby) falls off the slide, she gets muddy and miserable and cries.  Her older brother picks her up, quickly soothes her and takes her home.  Chris always loved this part of the book when Lil was a infant.  He often asked when he would be able to take Lil to the park by himself.  These days I have been watching their relationship grow and grow and become more mature.  They are very similar you see. 

They both love to build, they love cars, trucks, and they both love Star Wars.  Lil loves 'girly' stuff too but she is the kind of chick who will run around the house in a princess dress and camouflage hunting boots with a battle ax in one hand, light saber in the other on top of being dressed to the nines in princess jewelry.  Their intelligence runs along the same vein as they will attend to a task until its completion no matter what and they are especially in tune to figuring out puzzles or, more so, the way things work. This is a picture of Chris sorting his candy on his first Halloween- self directed!


Since we have returned from our trip, Chris (little Chris that is) has been an absolute mess.  I have not seen him act out this way since he was two years old.  He has been throwing things, screaming, screeching, crying hysterically at the drop of a hat and all day long.  My little boy is in pain.  Something is wrong.  Chris and I have been pulling our hair out all weekend trying to figure out what to do with him.  Chris is a very emotionally intelligent little boy and because of that he needs to know about Lil's disease but we have to walk a fine line between telling him enough and telling him too much.  Having our five year old friend Lucy die had a profound effect on him.  He talks about her all the time.  We do have therapists that the twins work with at the hospital while Chris and I are working with own support group.  We had a special meeting with them before the trip in which they told me they could have video taped Nettie for the perfect therapy session but Chris was a tough nut to crack!  No surprise there!  After many failed attempts at intervention this weekend father and son went for a long walk in the woods but no go.  Finally last night Chris and I were at the end of our proverbial ropes. 

I was in the bathroom with Bubby (AKA little Chris) and he had just gotten out of the shower, a shower which I had screamed at him through when all I wanted to do was let him wash his little body and wrap him in a warm towel and help him get his little boy pjs on and then snuggle him until his pain went away.  But all he was doing was pushing us away and pushing us away and pushing us away.  On top of all this, I have not gotten to the wrap up post of the NIH visit but there are some things hanging over my head that I am running away from. 

After a lot of yelling I finally got him to give in to cuddling in my lap and trying to talk about what is bothering him and it hit me like a ton of bricks.  I finally blurted out, "Are you afraid she is going to die?"  I felt his body go limp and the racking sobs begin.  We both sat on the steamy warm bathroom floor and cried.  I can't take away his pain and his fear.  I can't tell him she will not die.  Although I can reassure him it is not going to happen anytime soon!  We talked about the beautiful Lucy, we talked about the support group, we talked about Lil and her disease.  We talked about our fears and how much we love each other and how much we love our family.  Then we stood up and walked out of the bathroom. 

When I put him to bed we talked some more and this morning even more.  Have I made progress?  Will we keep talking?  I don't know.  One of our new CCF (support group) friends has a son who is losing his fight with a rare disease at 18 years old.  He was not supposed to live past 8 years.  He has an older sister who went away to college this summer.  His mother shared with me that the sister had an awful summer; she described her as a bear!  Mom could not figure out why and it sounded exactly like my weekend!  Finally his sister screamed, "I don't want to leave ___!!  How will I know how he is doing?  How will I know his levels?  What if I miss something?!?!"  Then she just started crying.

If a college student who has been dealing with this her whole life and has an amazing family does not have the skills to deal with these emotions, how can I expect my five year old to?  Having a chronically ill child is not only about taking care of that child, not even close.  It is about fighting off demons and creating celebrations out of thin air for everyone.  It is about making other people understand those demons and celebrations because your support people become your lifeline.  And sometimes it is about letting go of people who break down the delicate fortress you are building no matter how much you used to love them because if you are not with us, you are against us.  Thank you to all of you who are with us. 

My baby boy needed me, he needed all of me this weekend.  I came downstairs after putting him to bed.  It was one of those times when it took every once of my energy to put one foot in front of the other and I even paused a few times because I could not find that energy.  Then I sat on the couch with my husband who made me smile a little.  I ate three ice cream sandwiches which are a big treat for me and my heart swelled with the idea that I was able to afford them through your generosity.  And I thought of you; I thought of the Dragon Mothers from whom I draw strength and I knew I could sleep that night and wake up the next day with new ideas to help this family grow and thrive... not just get through the next day.