Lately most people have been saying that they are assuming Lil is doing well because I have not been posting much on Facebook. There are a few reasons I have not. One is that I have spent the last year redefining myself and in turn her. As the years have progressed, our lives have been less and less defined by Lil's disease and diagnosis. That does not mean her daily life is not affected by it and in turn the daily lives of her immediate family. One way I dealt with this was to start the Love, Life and LillyAnna Facebook page so that her updates are separate from my own. However, it is taking awhile for people to catch up:). Another is that I am now working full time and Lil is going to school.... Kindergarten, so we have our lives to focus on and not just illness; which is wonderful. Yet another is that we just simply do not focus on it as much, we focus on living life to the fullest. Sometimes that is easy and sometimes it is really, really hard.
Lil has what is called an Invisible Disease. She looks completely normal most days. She acts completely normal most days. But she is far from normal. This makes living this life that much more difficult. It is the rare person who can truly understand what our life is truly like. The people that live that life with us get it... they see how she is up multiple times a night in pain, they see how she hobbles down the stairs in the morning like a 90 year old instead of a 6 year old. They see the days she cannot get off the couch. They see everything in between. Her school is wonderfully understanding. Her nanny is an integral part of our lives. But yes, this little girl struggles every day.
In fact, it was so interesting going to the NIH. We were able to evaluate how Lil is doing compared to two years ago in a more formal way. It is funny to me how everyone thinks she is doing so much better but I think she is doing worse and so does her hospitalist. When we compared our notes from two years ago to now, the NIH said she is doing worse and gave reasons why. I think the change is that we have learned how to live this life. That is something I never thought would happen. People used to say things like 'this too shall pass.' It would make me so sad. I thought, "not this, it is never going away." And to an extent, I am right, it is not going away. But it has passed in the sense that we are a different family now and we know better how to navigate what it means to live with Lil's disease. I mean, there was a time I never thought I would be able to work full time again and look at me- building a career! When we evaluated how she was doing, it was clear that 2 years ago she was doing better than she is not, clinically. She is having more fevers and they are higher. She is having more pain and it is affecting her more. When you see her, she is pushing through. She is pushing as hard as she can to look like and feel like a normal kid.
That leaves us with the question of why Lil is getting worse instead of better. In every other case of Infantile Idiopathic Pyoderma Gangrenosum, the baby has gone on a steroid or a steroid and then Remicade and gotten better. Lil is the only one that has not.
I do not totally understand what the NIH was telling us but they found a gene variant that is unique to her out of their pool of 200,000 applicants. There is a possibility that Lil may have a brand new disease- something no one has ever seen. After reflecting on this information, I remembered that years ago someone told us about that possibility. The NIH wants us to come back and bring the twins. They will draw blood from them. If they have the same gene variant, we have to start from square one. If they do not, we may have found that brand new disease.
So the twins have to consent to have their blood drawn and be a part of the study; which they have to us but they will have to sit in that little room, sign their own names on pages of paper work (it is like buying a house!) and be treated like little adults. I think they are both excited for their own reasons to be a part of this process. It is a helpless feeling for us all to watch Lil live this life day in and day out.
How does this help Lil? Her lesions are not being controlled. They are starting to attack the tissue inside of her mouth and who knows where else. In fact, we came home and two nasty lesions showed up behind the two teeth that are growing in right now. She says they do not hurt but they look nasty. She is still complaining of pain often. They think (from where she was describing the pain) that it could be something in addition to the arthritis we already know she has and is being controlled (at least this is how I understand it). They are doing to do an MRI (in addition to the DEXA scan and chest CT they did this time) to see what could be causing the pain.
Watching that doctor go over my baby's skin with a fine tooth comb felt so good. That is how I look at her. This doc, the one I call The Angel Doc, knows this disease inside and out. It is an indescribable connection. The funny thing about the NIH is that I never know when they are going to pay attention to us and when they are not. Right now they are paying attention. Whenever the docs pay attention to Lil, I get nervous. I should say extra nervous.
Until we figure this all out, we are just doing the best we can with what we have. Medicaid is trying to get us a robot that will go to school for Lil on the days that she cannot go. It is a pretty cool program. Our only concern is that she goes to Montessori school which is very hands on. But her school is amazing (Montessori Learning Center) and is totally willing to work with us. What I wanted them to do is give us financial assistance for some of Lil's care. It costs us an astronomical amount a month. We fit into a weird category. We do not require nursing care but we do require more care than a typical child. Medicaid is finally catching up with this group of kids and just created a patient care coordinator for families like us; in fact, I think we were her first case.
The idea of having a brand new disease kind of puts me in a tailspin. It brings me back to years ago. In fact, the whole visit to the NIH did. It made me reflect on where we are and on how far we have come. I mentioned earlier in this post that I never thought we would be here. There was a time I thought that because Lil would always have this disease it meant that our lives would always be the same. I was right in that Lil's disease is a constant. It affects our lives every day and it affects all of us albeit in different ways. However, we are growing and changing with it. I went to dinner with friends. I WENT TO DINNER WITH FRIENDS. Two years ago I would have said that was impossible. I would have said I could not. We have been spending time at my father in law's shore house. While there, I actually lay on the couch and snuggle with my kids. Two weeks ago I would have said I could not. I have committed to better nutrition for myself. It was not an easy commitment. Four months ago I said, "I cannot." I do not often talk about my constant and chronic migraine pain. But I have committed to taking care of myself in that way and I actually exercised (for 5 minutes!) the other day for the first time since Lil got sick. Do these things sound silly to you? They should. I have convinced myself that they were impossible for the past 5 years. They may not seem big to you but they are HUGE to me. We are redefining ourselves for sure. It is beautiful and horrible all at the same time. I could not do it without my tribe (you know who you are) and my friends who take the time to love us and understand us. My tribe is teaching me that I can live life to the fullest and it does not have to compromise Lil or her care; that loving life actually enhances it. My tribe is teaching me that I can move on with my life. My tribe is teaching me it is okay to be a leader. My tribe is teaching me it is okay to be weak. We are growing alright. I can't say I would not have life any other way. My baby lives her life in pain. It is debilitating pain. But it is cool to see the family into which we are blossoming....
