With UpToDate Debbie finds in-depth information on
primary biliary cirrhosis and porphyria
Debbie, a 48-year-old woman from Tacoma, Washington, had an
aunt and uncle that both passed away at a young age from primary
biliary cirrhosis (PBC). PBC is an autoimmune disease where the
small bile ducts within the liver are slowly destroyed, eventually
leading to cirrhosis and liver failure.
“I read everything I could
find in UpToDate on primary biliary cirrhosis, porphyria
and liver disease”
Debbie experienced some of the same symptoms that her aunt
and uncle did, including extreme itching, fatigue and jaundice, and
decided to see her doctor. Her doctor ordered a liver enzyme test
and the results were about three times higher than the normal range,
indicating the need for a
liver biopsy
to learn more.
In addition to the symptoms above, Debbie also suffers from
an inherited rare blood disease called
Acute Intermittent Porphyria (AIP).
The porphyria causes her to have acute attacks of abdominal pain
every two to three weeks and, if she is exposed to sunlight for more
than 10 minutes she will get a rash. Debbie explains, "I have these
attacks and end up going to the emergency room, but the doctors
don't know enough about the condition and they treat the symptoms,
and unfortunately a lot of times when they treat the symptoms they
make things worse."
Debbie uses UpToDate to understand what is
happening with her body
Debbie found a link to UpToDate's web site in some
materials provided by her physician and visited the site to better
understand what was going on with her body. "I read everything I
could find in UpToDate on primary biliary cirrhosis,
porphyria and liver disease," said Debbie.
“UpToDate gave me
insights that I wasn't finding on other internet sites”
She brought the information from UpToDate to her
doctors. Debbie says, "The doctors were very happy that I had the
information with me. They told me that it helped them a lot." Also,
because porphyria is inherited, she is using UpToDate to help
her write a paper on the condition for her kids so they can
understand it and have it in their medical records.
Debbie will have a liver biopsy, to provide information about the severity of her liver
injury to determine if she has PBC, and to see if she will require a
liver transplant. She will see a porphyria
specialist in the next month to determine next steps for her
treatment.
"UpToDate gave me insights that I wasn't finding on
other internet sites. It was more in-depth, which was good," she
explains.
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