PSC and others

[Guest guest]

Hello to PSC's,

I found your support group three weeks ago. I have been reading your

questions and encouraging messages each day. I was not aware of AIH and

PBC and the other complications dealing with liver disease. I am now less

focused on my problems and will be sensitive to the many other kinds of

liver problems that are causing so many people pain. Perhaps, if I share

my situation and the things I have learned, I too can help someone figure

out what to do.

My name is Duke. I am a 61 year old male with PSC (primary sclerosing

cholangitis). I live in Nacogdoches, Texas, USA and I am listed as Status

3 on the liver transplant list at St.Lukes hospital in the Houston, Texas

Medical Center.

My wife, Carolyn, would like to talk with the spouses of other PSC

patients. Sometimes I forget what she is doing or trying to do as I go

through my ups and downs. For example, I never use curse words, but she

says I do when the pains are severe and the emergency room personnel are

not fast enough in doing what I want. And I hesitate to continually tell

her about my many aches and pains because there is little she can do to

help me and it just makes her feel worse.

My understanding of the transplant list is that a Status 3 is ill but can

usually continue to live at home and continue to work. Status 2B is very

ill and may or may not stay in the hospital. Status 2A is usually staying

in the hospital. Status 1 has only a few days to live. (Please correct me

if this is wrong)

PSC is the hardening of the bile ducts inside the liver. I am told PSC

effects one in 100,00 persons, mostly men. An x-ray of a normal liver

looks like a healthy tree in winter with many small limbs without leaves.

An x-ray of my liver looks like the limbs are being pruned each year.

I was very ill in the Fall, 1995 but a local internist could not be

definite about what my numerous blood tests were showing. He sent me to a

teaching hospital in Houston where they took one quick look at the test

numbers and said you have 8 to 10 years to live without a liver transplant.

Of course they did the additional tests (biopsy, colon, etc.) which

confirmed the PSC,

The first two years I had an ERCP every 6 months and things went well on an

out-patient basis. For the ERCP some doctors spray a local anesthetic in

the throat. I asked my local doctor to not use the spray and he agreed.

An intravenous sedative is necessary while my gastroentologist moves the

endoscope (a thin flexible tube) through the mouth, the esophagus, the

stomach, and into the duodenum. This provides a visual exam of the area.

Then a catheter (narrow plastic tube) is used to inject a dye into the

biliary ducts for an x-ray. A plastic stent (tube) is inserted into the

liver to help it drain. During my first ERCP they removed over 60 stones

of varying sizes from the common bile duct. Now we find only a few stones.

My gall bladder tests show no problem but the stones continue to appear.

My doctors always provide Polaroid photos of the stones, the ducts, etc. I

like this but some of my family members do not want to see the pictures.

Once I had the helicobacter pylori bacteria in my stomach which had to be

treated first, then we did the ERCP a few weeks later.

One time I went to the emergency room for jaundice and have returned to the

emergency room several times for pain after going home from the ERCP.

Currently, I have an ERCP every three months. A week or two before I am

due for a new stent I get high fevers (102-103) and low temperatures (97

-96), chills, a variety of aches and pains, more itching, and little sleep.

After each ERCP I now have lots of pain in the area directly below the

sternum. I have been staying in the hospital 3 or 4 days using Demerol or

morphine for pain relief.

In May, 1999 I went to Houston for the 3 days of tests. According to one

of the Liver Center's coordinators the pretransplant evaluation tests costs

$30,000 and consisted of the following:

a. thallium treadmill stress test (uses a nuclear tracer for the heart)

b. bone density

c. venous Doppler study of arteries and veins in the legs

d. carotid Doppler study of arteries and veins in the neck

e. EKG

f. 2D echo Doppler cardiology

g. abdominal ultrasound with Doppler to include portal vein size

h. PA/lat chest x-ray

I. pulmonary function with ABGs (oxygen in the blood)

j. lab work (17 vials of blood for various tests).

The purpose of these tests is to determine if there are other medical

conditions that might cause the transplant to fail such as:

a. tumors in the liver, colon, kidney, or pancreas

b. active infections

c. alcoholic behaviors

d. heart disorders

e. pulmonary (lung) disorders

f. acute liver necrosis

g. insulin-dependent diabetes

h. cancer in other organs

At this time they gave me 2 to 3 years to live and sent my name to my heath

insurance company as a future transplant recipient.

I pray for God to cure this illness. Or at least stop it from getting

worse. It may be that God's answer is my transplant team in Houston. If

that is the case I must decide whether or not to actually go through with

the procedure. Yes, I do have this choice. It is very expensive and the

expense continues until I die. Sometimes I think my retirement money might

be better spent by providing for my wife of 39 years and by helping my 3

grandchildren pay for college.

Medications I am currently using which may or may not work include:

*Actigall--Works only for stones that are made of cholesterol. Suppose to

decrease secretion of cholesterol made by

the

liver into bile.

*Lactulose--For bowel movements and mental confusion

*Vicodin--For pain between ERCPs

*Levaquin --For infections following an ERCP. This is the one

antibiotic I can take.

*Cholestyramine--For itching (not working yet, only been using it for one

week). Suppose to remove excess bile acids.

*Protein tablets--For fatigue

*multi-vitamins

*chewy chocolate For calcium

*milk thistle--Because the Dean of Purdue University's pharmacy school

recommends it. I got my Ph.D. at Purdue.

Other concerns I have but have found no relief include:

1. Declining muscle strength. I seldom can open ordinary bottle lids,

move the furniture, and do yard work that was so easy a

few years ago.

2. Irregular sleep patterns. I tend to sleep for an hour and a half, wake

up for two hours, then sleep for maybe two hours, and wake up again. Maybe

this is age related and not PSC related.

3. Leg, feet, toe cramps. Sometimes a hot bath helps.

4. Declining sexual urges. Maybe this is also age related. But it might

be the medications or the liver.

5. Depression. I do not like the side effects of the various medications

offered. So I try to keep a positive outlook with my family and at work.

When someone asks how I feel, I usually say " fine. " I am not sure they

really want to hear the details.

6. Money. Each time I have another hospital procedure or lab test it

takes months for the insurance company and the hospital to decide exactly

what I should pay. It takes many telephone calls to many different

offices. Each time we have to explain the situation. Thus, we simply wait

for several months before we actually send money even though the bill says

we owe " X " amount now. This adds to our frustration.

7. Probably have others but unable to recall at this time.

I search for causes but no one agrees on any one thing. Some studies

suggest this condition can be present for many years before we know of its

presence.

For example, in the mid-50's I was in the Marine Corps and in the summer of

1957 I was 3 miles from ground zero in the largest nuclear bomb test ever

conducted in Arizona. I still wonder if that bomb test is partly

responsible for both my daughters having thyroid problems. And I wonder if

it contributed to my autoimmune disease. The Veteran's Administration says

they have no evidence supporting these problems, but they do have evidence

related to cancer in the participating troops and citizens living in

neighboring states.

I have always been sensitive to cold weather. My hands and feet have

always been cold. When my daughters were young I would coach their many

teams or attend their athletic events and wear a jacket. Of course, other

parents asked why I was wearing jackets in July. Even now I wear a jacket

in my office, when eating in restaurants, at the movies, etc. Now I know

that many PSC's are sensitive to cold.

Since 1960 I have known that my bowel movements were not normal. No tests

showed anything definite, nor did the over the counter medicines provide

relief. Now I use Lactulose twice a day to help and to relieve the

occasional mental confusion due to the back up of bile (ammonia).

In 1987 a local internist suggested I had arthritis. But I decided my

complaints were related to a very stressful educational program I was

directing at the time. The aches and pains continued but we could not

definitely say it was arthritis.

In 1990 I went to a hospital emergency room in Ft. Worth, Texas where one

of the physicians on duty looked at my blood tests and suggested I stop

drinking alcohol. In my 61 years I have never had any kind of alcohol. I

don't even like mouth wash with alcohol. And I have never used any illegal

drugs. But the physician did not recommend that I see anyone else for

follow-up studies.

Perhaps GRIEF is not a cause but a contributing factor. In 1994 my

youngest daughter died. She was 27 at the time. My wife moved to Houston

to help with our 4 year old grand daughter. I remained in Nacogdoches

where I was chairman of a very large and successful university education

department. I resigned this position 9 months later. I wasn't doing a

good job and I was getting sicker. I tell my physicians this story but

they are not sure the effect of grief on the autoimmune system. But I

believe it is a factor worthy of research.

One of my problems is diet. Again, I have not found agreement regarding

what I should eat. Fatigue is always a problem so I have been eating extra

protein, both animal and vegetable. I have tried to eliminate salt as my

ankles sometimes swell. Too many sweets seem to make me weak. Sometimes I

eat small snacks in the morning and afternoon, but I cannot tell if this

really helps.

Carolyn and I are interested in the living donor program at Virginia

Commonwealth University. Their transplant team has performed over 40

transplants, using both related and non-related donors. They take 40% or

even 50% of the donor's liver and it will regenerate in a few weeks. It

takes longer for the recipient to be up and about.

My team leader in Houston has performed similar transplants, but only adult

to children. He is not ready to put my wife at risk. She and I have the

same blood type (A negative) but she has not been tested for tissue type or

liver size. Plus, my health care provider does not cover living donor

procedures. But I am still attempting to change their minds. It seems to

me this program is better because I can have a new liver now, while I have

a better chance for recovery. If wait until I am sicker (a status 1 or 2)

my chances will be less.

I hope my story is helpful. Someone may have questions they would rather

not ask on this list serve so just use my regular email and I will share

what I have done and learned.

Also, someone may want to contact Carolyn using my regular email.

Thank you for this list serve. I hope many physicians and researchers are

reading it daily. Maybe they can gain insights, develop new research

projects, and try new procedures for their patients from our many stories.

It is truly amazing how this technology allows us to communicate with

people in so many distant place. I am using a power mac G3 for this email.

I hope the numerous computer programs in use now have not scrambled my

sentences and paragraphs into something that adds to you confusion.

Thank you for taking the time to share.

Duke