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 per cent 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.
Through the Familial Gastrointestinal Cancer Registry you can talk with other people with FAP who have had the same operation and get support through a 'buddy system'. 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 and recovery. We provide children's books, instructional videos and relaxation tapes to help with the emotional and physical preparations for the procedure.
The following operations all include removal of the colon (colectomy) 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. Ask your surgeon whether your surgery can be done laparoscopically (through the abdomen) to reduce scarring. It involves the used of special telescopies, cameras and instruments designed to fit through small operating tubes.
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 post-operatively 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.
Restorative proctocolectomy, 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 ileum 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 (stoma) 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 surgeons.
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" journal.
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 Kock 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 per cent of people with a Kock pouch have required surgical revision of the nipple valve due to its intricate nature.