Re: W - autoimmune disease to Suzanne - not cp related

[Guest guest]

Suzanne,

I'm going to e-mail my response to the group because even though it is not cp

related, in a sense all the things going on in our bodies are related to each

other in one form or another. So, those of you who only want to read about the

cp related stuff, please feel free to just disregard this message.

First, I'd like to say I cannot imagine how hard it must be to have a child with

this horrible illness. Every day I thank God that it is me and not my daughter,

stepdaughter, or any of my loved ones dealing with cp! Autoimmune diseases are

tricky things - often hard to diagnose and they can present totally differently

in different patients - especially one like lupus. By the way, has Joey been

tested for lupus? Lupus can attack almost any part of the body. The hair loss

could also be due to the trauma on Joey's body from the surgery and perhaps it

will only be temporary. I do have to say that when I received the diagnosis of

autoimmune liver disease, it was probably the scariest and hardest to accept.

It was a total shock because my internal med doc had been saying I needed a

liver biopsy since Aug 02 and my previous GI kept insisting my liver was

perfectly healthly. Finally on Dec 27, 02, my GI did a liver biopsy. On Jan 3,

2003, he told me that the biopsy showed I have autoimmune liver disease.

Depending on who reads the biopsy report, I either had stage 3 fibrosis in my

liver or the beginnings of stage 4 fibrosis, which is actually cirrhosis. Liver

damage is graded on a scale of 0 to 4 with 0 being no damage and 4 being

cirrhosis. The GI doc told me not to worry as it typically responds well to

treatment, which is prednisone and imuran (an immunosuppressant). My initial

thought was, " I'm not taking prednisone. I don't care if I die. " That lasted

less than 5 seconds and I realized I would do whatever I had to do to stay on

this earth because I have many people who love and need me. The liver disease I

have is autoimmune hepatitis. My husband and I have learned not to use the term

autoimmune hepatitis because people (even in the medical field) hear the word

hepatitis and they think hepatitis C - you're contagious and you caught it by

being promiscuous or using IV drugs. As I'm sure you know, autoimmune means

that my immune system is attacking my body, in the case of liver disease, it is

my liver being attacked. The term 'hepatitis' simply means inflammation of the

liver, but people associate the term hepatitis with viral hepatitis. I didn't

'catch' autoimmune hepatitis unless you consider that I 'caught' a gene from

each of my parents that can lead to autoimmune diseases. Only 1 in 150,000

people have autoimmune hepatitis, so it's certainly not a commong illness. The

signs are fatigue, itching, anemia, low platelet count, elevated liver enzymes,

and either a positive ANA (anti-nuclear antibody) or anti-smooth muscle

antibody. It is more common in women with 70% of the people who have it being

female. There are two other autoimmune liver diseases - primary sclerosing

cholangitis and primary biliary cirrhosis. One of those is more common in men,

but I forgot which. Most liver diseases can only be definitively diagnosed by a

liver biopsy.

I was lucky because my liver did respond well to treatment, I was on 20 mg of

prednisone for several months, but by Sep 03, my prednisone was tapered down to

only 2.5 mg a day, which is low enough to not cause the lovely side effects of

long term steriods. I was also on imuran for almost a year. Currently I am off

all meds for the autoimmune hepatitis and they are checking my liver enzymes

every 2 weeks.

As for the IgG deficiency, according to our hematologist, it goes along with

autoimmune diseases. My sis gets monthly IV supplements and it has helped her

tremendously. She has only had one sinus infection since starting the

treatments last Dec. Prior to the treatments, she had been on antibiotics

almost continuously for about 6 years and they could never really get her sinus

and upper respiratory infections to totally clear up. The treatments do give

her a headache and make her feel kind of crappy and tired for a day but she says

it is very worth it because her sinus and upper respiratory infections were so

horrendous. The hematologist told her the only dangerous side effect is

possible very severe kidney damage if you have this one particular gene. If you

have that gene, they will not do the IV immunoglobulin supplements and the only

other option is just continual antibiotics. Luckily, my sis does not have the

gene that would make the supplements damage her kidneys. It was upsetting for

her to know she had to get the supplements, but she really had no choice. She

and her husband have an 8 year old who needs a mommy and with her IgG deficiency

and the recurrent infections, she was very likely to get serious pneumonia that

didn't respond quickly enough to antibiotics and then just drop dead. For she

and I both, the pneumonia vaccine didn't do a bit of good because our bodies

didn't develop any antibodies to the vaccine at all. In fact, the hematologist

at first thought that maybe we had not had the pneumonia vaccine when we both

had it and the flu shot in September!

The genes for autoimmune diseases are found on the short leg of the 6th

chromosome (means nothing to me). In my case and my sisters, it seems we got a

gene from each parent that can lead to autoimmune diseases. They are HLA-B27

and HLA-A2. We know the HLA-B27 came from my dad because my mom was tested for

it and does not have it. Therefore the HLA-A2 had to come from our mom. Even

though my numbers for the IgG deficiency look much worse than my sisters, I do

not get the really bad infections like she gets, at least not yet. For now, I'm

not getting the supplements but may have to in the future.

I certainly understand your apprehension over Joey's liver. I'm not an expert

on fatty liver or steatohepatitis, but I thought it was normally seen in people

who are overweight and have high cholesterol and triglycerides. Personally, I'd

keep a watch on Joey's liver enzymes. They might be high just from the surgery

and implanting the islet cells. However, if they remain elevated, I'd start

talking to the doctors about the possibility of a liver biopsy. For almost a

year prior to my liver biopsy, my liver enzymes ranged anywhere from normal once

or twice to almost 800 (when they should be no higher than 35).

Take care and don't hesitate to ask me other questions. You can write me

offline at karenw@... if you'd like. Make sure you put something about

the panc group in the subject line because I typically delete e-mails if I don't

recognize the address!

karen w

alabama