How cute is this? My little sweetie in her school uniform. :-)
She was only there for a few weeks before we started making all the trips to see the neph, but they did an amazing job of caring for her... and accommodating me! ;-) Thank you nurse Lisa!!
AAAAAHHHHHHHHHHHHH!!!!!!!!!
OK.... I just had to get that out of the way. I am at my wits end with all of this medical mystery crap. I'm angry and scared and totally confused by all the confusion and lack of concrete answers.
I thought that finally getting someone to SEE what I have known was happening to my baby girl would make me feel better. I actually did feel better for a minute, it was one of those "See, I'm NOT a hypersensitive, overreacting, crazy mother who always thinks her kid is sick!" moments that we all have after seeking an elusive dx's for our child. Of course that quickly faded into, "Oh my God.... I was right." :-( Then the anger started to build. The "what the hell is wrong with these stupid, arrogant doctors who refused to listen to me" type of anger~ combined with some seriously harsh feelings toward some family members who have constantly trivialized my concerns. I really wasn't sure if I wanted to climb a mountain and scream HOW DARE YOU!! to them all, or just take my little girl, crawl in a hole and cry. I guess what I'm getting at is that I don't feel better knowing I was right.
We finally have the biopsy results, here's the abbreviated version;
"The kidney biopsy shows an immune complex-mediated glomerular disease with focal-segmental sclerosis." "Immunoflorescence shows predominantly mesangial, granular staining for IgA(3-4+), IgG (3+), IgM (1+), C3 (2+)."
"There are at least 5 segmental sclerotic lesions identified."
"Numerous large electron-dense deposits are seen in mainly paramesangial location."
yada,yada,yada
What does all that mean? It means that Emma's immune system is attacking the filters in her kidneys, and filling them with not one immune complex.... but with many varieties of immune "crap". The sclerotic lesions are a sign of chronic irreversible damage and if this process continues her kidneys will eventually be destroyed.
The pathology said it looked like IgA nephropathy with FSGS (focal segmental glumerulosclerosis) OR Lupus Nephritis ~But~ the nephrologist says it's Not IgA... there are too many antibodies and anomalies... it has to be Lupus or some similar complex MCTD (mixed connective tissue disease). Because these complex immune diseases are treated by rheumatologists we have to get the final diagnosis from one of them and they will decide how to best treat her....... What?? The kidney Dr. can't treat my kid's kidney disease?? Fabulous!
We did get a Rx for ace inhibitors that will need to be compounded into a liquid form by our local pharmacist, (you should've see the look on his face when he was reading the Rx, poor new guy...). That will keep her blood pressure from rising and is supposed to help slow the protein loss in her urine. I really, really hope it helps because it has minimal side effects and should not impact her blood sugar. The next step is starting steroids and that is NOT going to be fun, but we will deal with it the best we can.
These "answers" open an entire world of new questions. The obvious questions are about her kidneys and the treatment she will need and when/ if this will eventually progress to renal failure. The nephrologist says there is no answer for that; some kids never get much worse once treatment is started, some start dialysis in a matter of weeks and need a transplant asap.
The other questions are about what else may be happening in her little body. Pediatric SLE and other mixed connective tissue diseases don't just go for the kidneys....
I'm pretty numb right now, and too busy moving to spend too much time thinking about what the future will hold. We close on our house this Friday and move into our new place 400 miles away on Monday. What a week! I'm reading endless medical journals at night searching for any studies that will help shed some light on her prognosis, so I'm totally exhausted, but I can't make myself stop. I'm also back to taking Effexor, which makes feel like an emotionless robot. I actually think being a numb, emotionless robot is about as good as it gets for the time being. ;-) I'll go back to dealing with that anger and sadness eventually, but for now they will just have to wait... right along with those elusive answers I want.
We will leave for Dallas... again.... tomorrow. We will be meeting with Dr. Baum, Emma's nephrologist, to discuss the results of her biopsy. I already know that they found auto-immune kidney damage, but I don't have a definite answer on a diagnosis, a "stage", a prognosis or treatment options. I hope we have most of those answers after this visit.
Emma is actually doing well overall, and seems happier now than she has in months. She went through a very difficult phase when we stopped her celebrex a few months ago, she was miserable. She refused to leave my side, not even wanting to see her grandparents that she loves so dearly. It was hard on all of us, and almost killed her papa, but she's much better now and back to her happy little self.
I also saw a huge change in her personality after stopping her methotrexate about 8 weeks ago. She started singing the words to every song she heard on the radio, she's trying to read, she's fascinated with building toys and always wants to draw and color. This is not the same kid I had a few months ago, and I could not be happier with the change. :-)
Her MRI results appear to be normal, which is great news. It means that her foot swelling was almost certainly not from rheumatoid arthritis... if it had been there would be damage in the joints by now. (we will get a second opinion from the Scottish Rite docs just to be safe) Of course it also means that her kidney problems have likely been going on much longer than we realized. :-(
These are her puffy little feet last February.... OMG! I sent these pics to her rheumy and pedi since her feet were always like this at the end of the day. It had already been going on for months when I took these... that was 10 months ago....... so scary.
