Familial Adenomatous Polyposis
What is the treatment for FAP?
There is no known medical cure for FAP. Surgery is required because
of the large number of adenomas and the 100% risk of colon cancer.
In FAP, removal of the large bowel, or colon, is standard treatment
and is called colectomy.
Since the colon is not primarily involved in how food is digested,
you will continue to absorb food in the small intestine.
It is more difficult to accept the need for colectomy when you
may feel well. You may ask why surgery cannot be delayed. By the
time symptoms do appear, the type of operation and your future may
be compromised by the presence of a cancer. Consider the ongoing
need for colonoscopy and the possibility of overlooking faster-growing
adenomas which have reached the 5-10 year stage. For anyone with
FAP, accepting the idea of preventive surgery is the first
step. Talking with other people with FAP who have had the same operation
is useful. Ask for a referral to a colorectal surgeon who
specializes in diseases of the large intestine. Bring a list of
written questions, or have someone accompany you to the surgeon,
so that you will have a better understanding of your treatment.
Timing is an important concern. For school-age patients, the summer
months or other holiday time periods during the year are generally
preferred. Home tutoring is an option offered by many school boards
when surgery occurs during the school year. Talking with the guidance
counsellor and home room teacher will enable parents to plan accordingly.
It is important to include adolescents in these plans and to prepare
them for their hospital stay. Remember that most patients with FAP
are asymptomatic and otherwise well, improving their healing ability
The following operations all include removal of the colon
and focus on the rectum. The stage of disease and the number of
adenomas in the rectum may influence the type of operation which
is recommended for each person with FAP.
Ileorectal Anastomosis, or IRA, means that the last
part of the small intestine (ileum)
is joined (anastomosed) to 10-15 cm of the rectum. This procedure
may be suggested for someone who has minimal rectal adenomas.
The average hospital stay is 7 days. The average recovery
time at home is 6 weeks.
Since you will experience several bowel movements a day,
it may be helpful to talk with a dietitian about your own
diet. Some people report no food intolerances while others
discover that a food diary may be helpful postoperatively.
In general, remember that potential problem foods may only
be a concern as the body adapts and this may take up to a
year. You will be your own best food detective and should
not restrict yourself unnecessarily. Anyone who has an IRA
retains part of the rectum which must be checked every 6 months
to l year for life. As long as the lining of the rectum remains
intact, adenomas may form. A hollow metal instrument with
a light source, called a Sigmoidoscope,
is generally used and no sedation is required. Bowel preparation
is needed to clear the rectum of stool and gas.
also known as the pelvic
pouch, is generally a two-stage procedure. In the
first operation, after colectomy, the upper part of the rectum
and lining of the lower rectum are removed. The muscles controlling
the urge for a bowel movement remain. A new rectum is created
from the patient's own
in the form of a pouch which is brought down into the pelvis
and joined to the anus.
Time is needed for the area to properly heal and to ensure
good function of the pouch. For this reason, a temporary opening
is made from the ileum to the outside of the abdomen. An external
appliance is worn to collect waste products and is referred
to as an ileostomy.
During the average 10-day hospital stay, a specialist nurse
called an enterostomal therapist, or ET, will teach you how
to manage your ileostomy. Prior to your admission, you will
have a chance to meet the ET and to ask questions so that
you are better prepared for your operation. The dietitian
will discuss the need to maintain a diet during this healing
period and to ensure an adequate intake of fluids. You can
ask for an illustrated pelvic
pouch booklet to guide you with detailed explanations.
A support group for people with a pelvic pouch is available
at Mount Sinai Hospital. An x-ray of the pouch is taken after
6 weeks to ensure that the pouch is functioning. In general,
the second operation is performed after 3 months and is a
brief procedure to close the ileostomy, followed by an average
6-day hospital stay. The number of bowel movements varies
in individuals but adjustments in diet or medication may be
needed, either during the recovery period or long term.
Anyone with a pelvic
pouch will require regular examination of the
pouch to rule out any new adenomas. This operation may be
recommended for anyone with a large number of adenomas in
the rectum. Due to the technically demanding nature of the
procedure, pelvic pouch surgery should be performed by experienced
Proctocolectomy means that the
entire colon and rectum are removed. A permanent ileostomy
is formed and a stoma
is created, usually to the right and below the navel so that
waste products can pass into an external appliance. This operation
is not a common option for FAP but may be suggested if rectal
cancer is found; or a desmoid tumour has formed within the
abdomen; or other technical problems such as scarring are
identified in the ileum. The average hospital stay is 10 days.
In the pre- and postoperative period, the ET nurse will not
only teach you how to manage your ostomy but will offer practical
suggestions. The presence of an ileostomy does not prevent
sexual relations. There is a period of adjustment for both
partners who may benefit by sharing their concerns with each
other, the patient's doctor, and the ET nurse. During the
recovery period, this support may strengthen how a patient
feels about himself or herself as a person. Sexual expression
is a total body response involving emotional and physical
needs which allow us to reach out to others. The United
Ostomy Association of Canada, provides assistance through
local chapters, trained visitors, and the " Ostomy Canada"
Women with an ileostomy may conceive. To allow for adequate
healing, patients are encouraged to wait at least 1 year after
bowel surgery before becoming pregnant. X-rays of the colon
should be postponed until after the child is born, although
other diagnostic procedures may be carried out during pregnancy.
Continent ileostomy, or
pouch, involves the removal of the colon, rectum,
and anus. An internal abdominal pouch is created with a nipple-shaped
valve to prevent stool from leaking. The end of the ileum
is brought to the surface of the abdomen and a stoma
is fashioned. To empty waste products, you insert a tube into
the pouch. A gauze patch is worn over the stoma. No external
appliance is needed. This operation is performed infrequently
today and 10-20% of people with a Kock pouch have required
surgical revision of the nipple valve due to its intricate