The waiting game.

Ok, so I know I'm lame. I haven't posted in, cough** cough** three months cough** cough** I kept waiting for some new info and well we don't have a whole lot. Hmmm, Let's see the Nation Institutes of Health's Rare and Undiagnosed diseases program (mouthful I know)is still looking at her, which is a good thing, although fairly neutral since we still don't know if she's been accepted. UCSF is thinking on her and is trying to contact the NIH and have no idea how that is going. So on this front we are still waiting.

I can say the adoption paperwork is coming along. We've finished all our classes and I've started getting background stuff turned in. I'm still working on the homestudy, which is just a ton of questions. But I can understand why, they want to be sure these kids get a good home. So its work work work on the paperwork for now.

As for a good home, my oh my do we need a bigger one. The housing market out here stinks right now, so we feel stuck in our 2 bedroom and we really really need at least 1more bedroom. We are contemplating renting this one out but that just seems like a ton of work, ick. So on this front also we are waiting.

Well thats about it. We did have a great Halloween party last night. Bunch of folks from our home group and some friends from church. I can say by 10pm we were all tired and ready for bed. Getting old stinks. I remember when the evening used to start at 10pm. I guess that was before kids and days that started at 5:30/6:00 in the morning. Sleep holds a lot more meaning than it did when I was young and stupid.

Ah well, we did have a great time visited, ate a lot of food (not enough though I still have leftovers) and played a couple silly games. Oh yes and watched the Corpse Bride and Young Frankenstein.

so I say to you "hrmmmmmm" and hope to have good news later.

Steph

Moving right along

So things are a good right now. Looks like it was the medication from last month, I'm not sure it was compounded right. This months batch seemed to work a lot better.

Kiddo made it through summer school and has a couple weeks off before regular term. We have an appointment at UCSF in a few weeks with a new GI doc. We are hoping fresh eyes might see something.

And even better news, I am getting a mini vacation. A friend of ours is taking Naomi overnight so I can go on a getaway with the girls. Mommy's version of a doctor's appointment. It will involve shopping and eating so I am way good.

On sort of a side note, I'm thinking of taking my blog in a more family general direction. There is a lot going on that doesn't involve Naomi and her health and I really don't want to maintain two different blogs. I'm just not that motivated.

So for our first family announcement, we have started the adoption process! Soon we start the paperwork process!! So papercuts here I come.

Hanging in there

Its been awhile since I've posted as for a short while everything was quiet and not much was going on. Once being diagnosed with generic "liver disease" they put Naomi on Actigall. And after a few weeks it really did help. She could focus on things besides scratching, she was staying awake in school and participating more and in general was a much more pleasant kiddo. You could change her diaper without hands frantically scratching her legs and back. And she wasn't constantly rubbing her legs and feet together. It was nice.

Sadly for whatever reason the last two plus weeks have shown a marked increase in itching and general crankiness. We thought at first it was because of an ear infection she had, but that has since cleared up and she is as itchy as ever. I sent an e-mail to her GI doc and he starts talking about liver transplants and scares the crud out of me. He orders some tests which we had done today, and Praise God! they have come back normal. So what is causing the issue I don't know. I do know that for the first time in months I've had to physically restrain her from scratching because she couldn't stop and was making herself bloody, and that she wakes up just crying and scratching in the early morning. One decision we have made is to go ahead and get an consult to UCSF, her primary doc put that in today so we shall see how soon they can get us in.

Its not always bad, she has her good moments and we enjoy those when we can. For now we pray and keep her as comfortable as possible. The Actigall was working and we can pray it will work again.

I'll post as things go on. God Bless you all!

Not much else to do

So today we finally get this from Dr. D.

Naomi's final blood test result, the Prometheus IBD Serology 7, has just been received at my office and the results are negative. That is, she does not have subclinical Crohn's disease or ulcerative colitis that could cause recurring pancreatitis. This completes an exhaustive work-up for all known causes of pancreatitis and hepatitis. My best guess is that Naomi has a previously undescribed genetic syndrome leading to both chronic pancreatitis and hepatitis. At this point there is no additional treatment for Naomi.

Guess now we just do nothing? What do you do with information like this? I am at a loss.

Bummed

So my excitement at having some sort of diagnosis for Naomi and her liver has been short lived. All the tests done to see what type of autoimmune hepatitis have come back with no markers for that particular disease. So we are left with no clue as to why her liver is the way it is. And it doesn't look like they are going to leave the autoimmune hepatitis label on it either. I talked to one of her doctors who read part of the report where the pathologist at UCSF says "never seen anything like this." Great is about all I can say. We are waiting for a couple more test results to see if she has inflammatory bowel disease but I'm not holding my breath. The question now is if it's time to head down to UCSF and have many fresh pairs of eyes look at her. So much for a diagnosis and potential treatment.

For better or worse, its at least not "normal"

So we finally are getting some answers. Naomi's biopsy came back showing scarring and inflammation. We expected to see a fatty liver due to her malnutrition issues, not this. Dr. D sent the sample down to UCSF to double check and get a pathologists opinion and the best match is autoimmune hepatitis. It's not an exact match, with the patterns I guess, and but all the other tests and what they saw in the biospy leads to an autoimmune hepatitis. We will draw blood later this week to determine which type. I guess its even possible those will come back negative, but this is where everything seems to be pointing. In most cases this disease doesn't show up til folks are in their teens, so she is, as usual, an odd case. Also it is likely that this autoimmune disorder is why she has chronic pancreatitis. Her body is attacking itself.

The good news is finally someone has said to me "we think the itching is caused by the liver." Hallelujah! I've only been saying this for over a year now, and the even better news, there is something she can take to help. Its a bile salt that can help with itching and may even help with the inflammation. And it has few, if any side effects. That she starts right away. The treatment for the hepatitis I'm not sure of, that will be figured out when they get the lab work later this week.

So today I give thanks for a test that's not "normal." We don't want there to be a problem, but when you know something is wrong and no one can figure it out, its so hard. You feel helpless. Now we know where to start and hopefully what to treat to make her life better.

Liver and ERCP

I'm not really sure where to start. I'm pretty wiped right now and have had to fill in so many folks, I really should remember to write here first and then point people in this direction. So, it started last Thursday, Naomi finally had her liver biopsy and an ERCP. A fluoroscope test to see if the ducts near the pancreas and liver are malformed are not. And good news they aren't, all normal. Still doesn't explain her chronic pancreatic problems, but it's one more possibility cleared from the list. The liver biopsy went well and we should hear from it in the next 2 weeks or so.

So the procedure is finished, they check her in to the hospital, run some tests, all is good, its now 6pm and we head home. Get home around 7:30ish. By 8:30 she has a slight fever, by 9:30 we have hives, by 10 pm we are at the emergency room. Five hours of waiting results in the tests saying she has acute pancreatitis, and we aren't "comfortable" handling her case here. (It's a small town hospital and I think Naomi scares them). So its another ambulance ride and this time to Sacramento, finally settled in the hospital room around 8:30 in the morning.

Friday was quiet she slept a lot and seemed to be doing better. Saturday was horrendous. That day was the worst pain I have ever seen this little child in. They keep trying to tell me it was a drug reaction, since she was agitated and crying, inconsolable, and scratching. I kept trying to tell them, this is her reaction to pain, she scratches all the time. They take her off narcotics, I wanted her to have more. It was 12 hours of torture for us watching her cry and nothing helped. They ran a few more tests that all came back "normal" (I would like to add I really really do HATE that word these days). Finally around 7:30 in the evening she calmed. They wanted to tell me it was the benadryll they gave her but that was well over 40 mins before and it went into her central line so it would have had an immediate reaction. I did mention that benadryll makes her awake, but they wanted to try it anyway. I was not lying. Despite having cried almost nonstop for 12 hours, Naomi slept a total of about 3 hours that night. She was awake til 6am. She wasn't in pain by then but she was wide awake. I need a note from my regular doctor that says "Listen to Mama, she knows her child."

Having talked to her regular GI doc on Monday, we think her pancreas sort of "froze" up trapping any bile and causing intense amounts of pain. Around the time she relaxed on Saturday is also the time that bile started flowing from her vent in her g-tube. She put out a ton of bile Saturday night and into Sunday.

Once she was over the pain on Saturday things progressed like they normally do when she has pancreatitis. Her enzyme levels start dropping like a rock, she perks up, we finally fed her late Sunday and she was fine. She tolerated her feeds, her levels stayed lower and Monday we were sent home.

And so what did we learn from this. We learned that if it can go wrong it will, 15-20% of post ERCP patients get an acute flair, that would be us. We also learned that doctors need to listen to the Mama, there were many frustrating moments where I just felt they weren't listening to what I was saying. And finally, I knew this part, but I thought I'd share. Hospital cafeteria's are horrible and the beds are even worse.

I can write all this happily since I can hear Naomi giggling at the TV and rolling around on her mat. She is such an amazing kiddo, you never would have thought she just got out of the hospital yesterday. We are taking the day off today, not because she needs it, but because I need it. She bounces back, I collapse. It is definitely time for a spa day.