What is Lynch Syndrome?

Lynch Syndrome is a hereditary condition that places people at an increased risk of developing certain types of cancers. People with Lynch Syndrome have a problem with DNA replication which creates a gene mismatch. This mismatch predisposes individuals to developing cancers of the colon, endometrium, stomach, ovary, liver, pancreas, small bowel, urinary tract, brain and the sweat glands of the skin. These cancers often occur earlier in life than cancer in the general population and generally before the age of 50. Once Lynch Syndrome is identified in an individual, family members should be screened to determine if they also have Lynch Syndrome. If a parent has Lynch Syndrome, their children have a 50% chance of also inheriting the condition.

Identifying Lynch Syndrome early in life allows individuals to follow surveillance plans for cancer prevention. Careful follow up for individuals and families lowers the risk of dying from Lynch Syndrome related cancers.

The chart below shows the lifetime risk of certain cancers for people with Lynch Syndrome as compared with people without Lynch Syndrome in the general population.

 

Cancer Risk of Individuals with Lynch Syndrome without Screening & Surveillance vs. Non Lynch Risk

CANCER	LYNCH RISK	NON LYNCH RISK
Colon	50-82%	5-6%
Endometrium	25-60%	2-3%
Stomach	6-13%	Less than 1%
Ovary	4-12%	1-2%
Hepatobiliary	1.5-4%	Less than 1%
Small Bowel	3-6%	Less than1%
Urinary	1-4%	Less than1%
Brain	1-3%	Less than1%
Skin (sebaceous)	1-9%	Less than1%
Pancreas	1-6%	Less than1%

Data taken from Dinh et al. (2011)

To watch a video about Lynch Syndrome by Heather Hampel, a genetic counselor at The Ohio State University Comprehensive Cancer Cancer. Click the photo below if you have Adobe Flash Player installed or if not click the link below the photo.

OR

Click Link to Heather Hampel utube video

References

Dinh, A. D., Rosner, B.I., Atwood, J. C., Boland, C. R., Syngal, S., Vasen, H. F., … Burt, R. W. (2011). Health benefits and cost-effectiveness of primary genetic screening for Lynch Syndrome in the general population. Cancer Prevention and Research, 4, 9–22.  Retrieved from http://cancerpreventionresearch.aacrjournals.org/content/4/1/9.full. doi:10.1158/1940-6207.CAPR-10-0262

Lynch, H. T., Jascur, T., Lanspa, S. C. & Boland. R. (2010). Making sense of missense in Lynch Syndrome: The clinical perspective. Cancer Prevention and Research, 3, 1371-1374. doi:10.1158/1940-6207.CAPR-10-0204

Joanne Ngeow and Charis Eng. Population-based universal screening for Lynch Syndrome: Ready, set… how? (2013). Journal of Clinical Oncology, 21, (20), 2527-2529. Retrieved from http://jco.ascopubs.org/content/31/20/2527.full

OSUhumangenetics. (November 7, 2012). Heather Hampel discusses Lynch Syndrome. http://www.youtube.com/watch?v=k_07Be7p2_8

Surveillance and Compliance and Management, oh my!

                                                                                                                                               Google Images 2014

You have been diagnosed with Lynch Syndrome.  It may seem absolutely overwhelming.., there is so much to learn and so much to think about.  

Because persons with Lynch Syndrome have a higher risk of different types of cancer, it is important for them to be vigilant in managing and monitoring their health. Part of this process is called surveillance and it refers to watching the condition of different organs in the body that are predisposed (may have a higher risk) of cancer.

Some of the management steps (Lynch Syndrome International, 2013) that your physician might review with you to monitor your health might include:

• Colonoscopy: A procedure where a small tube with a camera in it (called a scope) is inserted into the colon through the anus. This allows a physician to look for changes and abnormalities.  
• Esophagogastroduodenoscopy: A procedure where a small tube with a camera in it (called a scope) is inserted through the mouth, down the esophagus, into the stomach and then into the first part of the small intestine.  This allows a physician to look for changes and abnormalities.  
• Endometrial Sampling for women: The endometrial sampling involves a preparation that is similar to getting a pap smear except that an instrument is inserted into the uterus and takes a tiny piece (biopsy) of the uterine lining.  The biopsies are sent to the lab and microscopically assessed for cancerous changes
• CA-125 for women: This is a blood test that is called Cancer Antigen 125.  The test measures a type of protein that can be elevated in cases of different cancers such as endometrial or ovarian, as well as, some benign conditions.
• Urine Cytology:  This involves a collection of a urine sample that is sent to the lab. Cells from the urinary tract are assessed microscopically for cancerous changes
• Dermatological screening: This involves a full body screening by a dermatologist

While there is an entire team of health professionals that includes physicians, physician’s assistants, nurses, geneticists, lab technicians and other personnel to help monitor and address changes,  responsibility for your health ultimately falls to one person.., YOU.

That is where compliance comes in. Compliance is doing what needs to be done to manage your own health.  

There are many tests available to monitor your health status and many professionals behind those tests who are ready and waiting to help you actively monitor Lynch Syndrome.  A person has no control over their inheritance of the genetic disorder that causes Lynch Syndrome but they have complete control over the surveillance and management through their own compliance with recommendations for those with Lynch Syndrome.

What can you do?  

• Become empowered through education, participation and control (Powers & Bendall, 2003).  
• Learn about your condition and the guidelines for monitoring and participating in your care.  
• Make recommended lifestyle changes and keep appointments to actively monitor your health and affect outcomes.

March 22nd is Lynch Syndrome Hereditary Cancer Public Awareness Day. Mark this day by reaching out for information.  Lynch Syndrome International is an all volunteer organization with resources for persons with Lynch Syndrome and the healthcare professionals who are helping with their surveillance and management. Their website is www.lynchcancers.org

References

Lynch Syndrome International (2013) Identifying and Caring for Those with the Lynch Syndrome. Brochure.  Vacaville, CA. www.lynchcancers.org

Powers, T., & Bendall, D. (2003). Improving health outcomes through patient empowerment. Journal of Hospital Marketing & Public Relations. 15(1), 45-59.

Lynch Syndrome and Your Family Health History

Retrieved from http://depositphotos.com/search/family-tree.html 

     

 Lynch Syndrome is the most inheritable form of colorectal cancer and is caused by a change (mutation) in a specific gene. Genes are inherited from parents.  Each person gets a set of genes from their mother and their father. If one of the genes inherited from either the mother or father has the mutation for Lynch Syndrome, the child has a 50% chance of developing Lynch Syndrome.  One of the easiest ways to determine if someone is at risk for Lynch Syndrome is to conduct a thorough family history.  Watch this 6 minute video about the value of the family history in your medical care.

Family History: A Window On Your Health

 

http://lynchsyndromeinformation.blogspot.com/2014/02/lynch-syndrome-and-your-family-health.html

    A multi-generation family history is the first step in screening for the possibility of Lynch Syndrome. Lynch Syndrome International recommends a free, public resource available through the Surgeon General's Family Health Initiative addressing documenting your family history. The Surgeon General's "My Family Health Portrait" is an internet based tool that makes documenting your family history easy. It assembles your information and makes a "pedigree" family tree that you can download and print to share with your family and healthcare provider. It takes about 15-20 minutes to complete. 
    
     If you do not want to use an internet based tool for taking your family history you can use this template to collect and document your family history. Be sure to make a copy for other family members and your health care provider.

 

 Name:________________________                      Date:__________

Date of Birth __________ Age ______              Gender (please circle)       female     male      other

Patient cancer history.

(Please circle yes for those that apply to you and no for those that do not apply to you)

                                                                      age at diagnosis              comments

Colorectal cancer             yes        no                    ________            ________________________

Breast cancer                  yes        no                    ________            ________________________

Ovarian cancer                 yes        no                    ________            ________________________

Uterine  cancer               yes        no                    ________            ________________________

Endometrial cancer          yes        no                    ________            ________________________

Kidney cancer                 yes        no                   ________            ________________________

Urinary tract cancer          yes        no                   ________            ________________________

Stomach cancer              yes        no                    ________            ________________________

Brain cancer                     yes        no                    ________            ________________________

Small Bowel cancer        yes        no                    ________            ________________________

Other cancer (please list) ________________    ________            ________________________

Answer the following questions. Circle Y for yes for those that apply to you and N for no for those that do not apply to you.

Y     N             Are you of Ashkenazi Jewish descent?

Y     N             Have you or a family member been told you have Lynch Syndrome?

Y     N             Have you or a family member been test for a hereditary genetic condition?

Please list any questions or concerns you may have in the space below:

Please answer the questions below. Please include parents, brothers, sisters, sons, daughters, grandchildren,     great grandchildren, grandparents, great grandparents, aunts, uncles, nephews, nieces, first cousins and half brothers and sisters

 (Please circle yes for those that apply to your family and no for those that do not apply to your family)

                                                                                                  age at diagnosis                     Comments

Colorectal cancer                     yes        no                               ________            ________________________

Breast cancer                            yes        no                               ________            _____________________                    Ovarian cancer                         yes        no                               ________            ________________________

Uterine cancer                          yes        no                               ________            ________________________

Endometrial cancer                 yes        no                                ________            ________________________

Kidney cancer                          yes        no                                ________            ________________________

Urinary tract cancer               yes         no                               ________            ______________________                     

Stomach cancer                       yes         no                               ________            ________________________

Brain cancer                            yes         no                               ________            ________________________

Small Bowel cancer                 yes         no                              ________            ________________________

Other cancer (please list) _____________________              ________            ________________________

  

                     

References

Centers for Disease Control and Prevention. (2013). Public Health Genomics Family Health History. Retrieved from http://www.cdc.gov/genomics/famhistory/index.htm

Genome TV. (2009, December 15). Family History: A Window On Your Health. Retrieved from https://www.youtube.com/watch?v=ARTFoQ4oHfU

Lynch Syndrome International. (2012). The family history. Retrieved from http://www.lynchcancers.com/index.php/the-family-history

The University of Texas MD Anderson Cancer Center (2008).  Lynch Syndrome (Hereditary Nonpolyposis Colorectal Cancer Syndrome or HNPCC). [PDF]. Retrieved from https://www4.mdanderson.org/pe/index.cfm?pageName=opendoc&docid=2133