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.