Prophylactic surgery is intended to prevent the development of disease. It is different from therapeutic surgery in that patients are usually asymptomatic and the procedure is not immediately necessary for their well-being. This form of surgery is most common in individuals predisposed to developing cancer from inherited genetic mutations, most of which are diagnosed through genetic testing. Many hereditary-based cancers are very aggressive in nature and do not respond well to conventional treatment. In families where genetically-related cancers are widespread, prophylactic surgery may be one of the few options for at-risk individuals to preserve their health. Its use varies according to population characteristics, risk perception and prevailing values. It is a very difficult decision to make, given that prophylactic surgery is irreversible, with potentially serious consequences for those considering it. Yet, it has the potential to favourably affect survival in the long term for those at the highest risk of mortality.
Why it’s important
Prophylactic surgery is often recommended based on the assumption that an equivalent reduction in cancer mortality cannot be achieved through screening for certain types of cancers. Gastric cancer has one of the quietest presentations due to poor screening sensitivity, and is typically only diagnosed at an advanced stage.The same argument applies ovarian cancer which is often not detected until the cancer has metastasized.Therefore, the screening methods for these types of cancers are not effective, and can result in the discovery of the cancer at a very late, and more lethal, stage.
Individuals with specific genetic mutations are at a significantly higher risk of developing certain cancers. Nearly 100% of individuals with familial adenomatous polyposis will develop colon cancer, most by age 40 if they do not undergo a prophylactic colectomy. Patients with a MEN2 mutation also have a 100% chance of developing thyroid cancer in their youth, and thus a total thyroidectomy is necessary to save their lives.A truly preventable procedure for this mutation can be achieved most reliably in infancy or early childhood, underlying the importance of screening additional young relatives of known carriers as soon as possible. There are additional mutations with a moderate to high probability of developing cancer. A compromised BRCA 1 or 2 gene in women imposes a lifetime risk of 60-80% for breast cancer, as well as a 16-40% probability of ovarian cancer
Throughout all genetic mutation types, there is a common theme regarding their danger in causing cancer to develop at such an early age. For example, 7% of young adults with familial adenomatous polyposis will have already developed colorectal cancer before the age of 21. Many genetically linked thyroid cancers strike before the age of one. Therefore, one cannot overemphasize the importance of screening for genetic and familial cancers. Physicians may attempt to maximize the use of an organ prior to its removal, while any cancer present is still “in situ.”
When it should be considered
Prophylactic surgery should only be recommended after a positive genetic test result. Health professionals should consider these results in conjunction with the patient’s previous medical history. A health professional will strongly advocate for risk-reducing surgery if their patients have been diagnosed with a localized or associated cancer prior to genetic counselling after the results come back positive. Often, the timing of the surgery is determined by the pattern of the disease as well as the age of onset for that person’s relatives. Health professionals must consider whether the health benefits of prophylactic surgery (cancer prevention) outweigh the costs of the procedure (morbidity) prior to recommending it. This explains why people at only average risk of developing cancer should not pursue this course of action.
Benefits of prophylactic surgery
Prophylactic surgery has proven to be extremely effective in reducing the risk of cancer-related mortality. Patients with hereditary breast-ovarian cancer syndrome can expect a 90-95% reduction in expected incidence of ovarian cancer after surgery. If this surgery is performed prior to menopause for BRCA 1&2 carriers, the risk of breast cancer also drops by 50%. Overall survival rates will always improve if the surgery is performed before cancer is diagnosed. However, no one should assume that surgery would stop cancer from developing elsewhere in the body either through an associated genetic linkage or from environmental factors.
An important benefit from the surgery is a decrease in anxiety, worry and uncertainty in comparison with those who chose surveillance options.
Trade-off: surgery versus lifetime management
When considering surgery, it is important to balance how easily treatable the cancer is with its actual lifetime risk and aggressiveness. Regular surveillance contributes to early detection of breast cancer, but it will not prevent the cancer itself. For example, patients at higher risk for colorectal cancer would have to weigh the morbidity and stress associated with constant biopsies and aggressive polypectomies to continuously remove pre-malignant lesions. The age of the patient is also important when assessing surgical options as opposed to surveillance. The older the patient, the higher the risks associated with surgery. Older individuals considering prophylactic colectomy would have to be evaluated for sphincter and bowel function as well as co-morbidity in other organs prior to being recommended for surgery. Collaboration among health professionals can go a long way to conducting a proper evaluation of the patient prior to offering all potential risk-reduction options.
Surgical complications / lifetime changes
A prophylactic salpingo-oophorectomy will cause surgically-induced menopause, which affects a woman’s quality of life. The risks are higher for women who undergo surgery before the age of 45, which creates complications given the early onset nature of genetic cancers. Early menopause causes an increased risk of osteoporosis, evidenced 3-6 years after surgery. It can also lead to increased risk of cardiac disease and cognitive impairment, emphasizing the importance of proper lifestyle habits regardless of the presence of a genetic mutation. It has been proposed that women who have lived in families at higher risk are more aware of healthy lifestyle habits and keep more physically fit.
Hormone Replacement Therapy (HRT) is used by some women to counteract a decline in sexual function caused by the surgery. However, there is a growing connection between HRT and breast cancer. Talk you your physician first about the risks and benefits associated with HRT.
The decision to undergo a prophylactic gastrectomy should not be taken lightly, because the procedure carries a nearly 100% lifetime morbidity rate, which includes symptoms such as weight loss, bacterial overgrowth, fat mal-absorption and micronutrient deficiencies. A dietician will also be needed post-surgery to counsel the patient on permanent changes to their diet.
For compromised MEN 1 & 2 carriers, a complete thyroidectomy is required resulting in the patient taking thyroid hormone replacement therapy for life.
The prospect of removing a completely normal organ or tissue in a patient who is perfectly well can be very hard to sell. Meanwhile, patients are trying to obtain a “guaranteed” cancer risk prior to deciding on prophylactic surgery. The individual’s perception of the effects of the disease has come to be recognized as an important indicator of how the patient will psychologically cope with their health. For some, the desire to alleviate anxiety through surgery may be present, albeit independent of objective facts.
There are two major fears present in all patients seeking genetic testing: the fear of being a carrier and the fear accompanying the prospect of prophylactic surgery in the absence of clear, clinical findings. For individuals exposed to high incidences and death of the disease among relatives, cancer is personified as an “omnipresent family member.”
“The cancer of my mother is always there… it lives with us, sleeps with us, eats with us… everywhere.”
Personal risk is synonymous with a “timer” associated with their parent or other relative’s age of diagnosis. That age number is a strong, subjective threat to overcome; however the age of children themselves when their parents got sick also strongly affected their experiences and attitudes to the “terminality” of cancer. There is a trend for very young girls with few memories of a parent’s illness to postpone surgery for risk-reducing mastectomies and oophorectomies. If they are older, there is more “path dependency” in terms of allowing it to affect their life in a very similar pattern to how their parents dealt with the disease. It takes objective counselling and support to break through deep anxiety in order to effectively explain the actual risk of developing cancer in patients with long-held perceptions of their health. Most importantly, it takes sensitivity and support.
The importance of self
The overall decision-making process for pursuing prophylactic surgery revolves around self-preservation. However, there are four specific dimensions of the self, and all must be considered to varying degrees due to the effects that surgery will have on each of them. On occasion, certain dimensions will dominate due to either cultural or religious reasons.
- Physical health
- Self-identify (as a woman with respect to breasts and ovaries)
- Relationships with others
- Emotional well-being
The process begins with patients imagining how the effects of cancer screening, prophylactic surgery, or cancer itself would change each of those dimensions. When individuals were not ready for imagined changes, or saw them conflicting with their perceptions of self, they made decisions that reinforced their current identity of self. When individuals were able to accept imagined changes, they made decisions that enabled them to accommodate or prepare for expected alterations to those dimensions.
Context of decision-making
1. Characteristics of healthcare services
Patients’ efforts to make decisions were influenced by their perceptions that the health care system was financially stressed or stretched. Under this context, individuals became more self-reliant, proactive and pushed for additional consultation to protect their final decision.
2. The level of risk for certain mutations
The fact that not all genetic risks are 100% creates a complex environment because of the uncertainty of developing cancer and the irreversibility of the prophylactic surgery. This has led to indecision about when is the “right time” to make decisions. In some cases, patients reported receiving little guidance beyond encouragement to reflect on their own needs and values. This is quite different compared to conventional cancer management practices, where health professionals are far more direct and have clear preferences in their recommendations.
3. Gendered roles
Family planning decisions and the importance of being present for various life stages may play an important role in deciding whether to pursue prophylactic surgery.
4. Proximity to cancer
In BRCA compromised women, if they had no known relatives and had not provided care to affected family members, they were less inclined to opt for surgery. There was less of a threat to the physical health dimension of self because of the belief that while the BRCA test result was surprising, not everyone was getting cancer. However, if proximity is very close, they may opt for surgery without recommendations.
Decision making process
In patients where the genetic risk is not significantly high enough to merit immediate surgery, the decision-making process can be agonizing. In some cases, the best way to try to create some clarity is to discuss the structural and dynamic issues of this process, which are often very implicit in nature. By bringing these to the surface, this paper truly hopes to offer the means for taking a step back to think about things objectively as they relate to their dimensions of self.
Snap decisions are made with absolute certainty, and are usually based on one’s proximity to cancer. It can lead to conflict within the family should there be any fundamental disagreements with respect to course of action, such as whether prophylactic surgery might interfere with the desire to have children.
Intuitive decision-making relies heavily on knowledge and belief. Intuition carries more weight than trying to gather information or advice on risk. This form helps preserve the dimensions of self that are perceived to be the most threatened. As such, it looks inward and pays attention to emotion even in the face of contrary information. This allows patients to maintain their identities and experience minimum emotional upheaval. While psychologically advantageous, it does disregard objectivity, and has caused health professionals to struggle with this form of decision making, resulting in unsolicited information and advice.
If / then decisions
This is a conditional style which works through potential future decisions to let individuals increase their comfort with their current decision. It allows patients to identify hypothetical situations threatening self-identity, their relationships, emotional well-being and physical health. This process is productive in that it does force you to conceive of significant threats to the self that might compel one to make different decisions than were previously held.
This form of decision-making can occur over months or years. Some consider this style to be optimal given that prophylactic surgery is irreversible, and thus it is easier to consider all the factors. The process involves making tentative decisions and then contrasting them by incorporating old and new information, advice, values and beliefs. You must attend to all of the threats against the dimensions of the self that will occur due to prophylactic surgery. Because contexts change over time (relationships, childbearing, employment, proximity to cancer), the dimension most threatened also changes.
Deferral / postponement
This involves intentionally putting off any prophylactic surgery decisions until the “right time.” It is often selected when the “physical health” dimension of one’s self is the least threatened of the four. This style is often implemented without hesitation, judging surgical decisions to be premature. Some feel that if there is no immediate threat, alternate options may become available with patience. Chemoprevention certainly ties into this decision as well, although it has yet to be recommended as a “primary” form of treatment.
Ultimately, this style does allow you to concentrate on busier, more immediate aspects of your life while reflecting on future surgery. The danger with this style is that one can only wait too long before treatment becomes more extensive.
Recognizing the factors that are involved with major decisions goes a long way to providing the appropriate planning and commissioning of health services. The question will be whether this reflects the needs of the current self or of the future self; and this depends on the quality of the information the patient receives. Several health teams have intentionally delayed surgical appointments by half a year to allow the patient’s decision to mature with greater confidence. It is interesting to note during this “buffer zone” that while positive test results were worrying for carriers, they still enjoyed the option of acting upon risks to avoid cancer, essentially removing a sense of “pre-determined fate” that might have fed their depression. Having the option to act as opposed to simply being diagnosed with cancer was in some ways cathartic.
Breast reconstruction for women
Disease-free survival is the main goal of preventing breast cancer. However, recognition of the importance of the breasts to female physical and psychological integrity is increasingly taken into account. Some have attached very significant personal meaning to their breasts and ovaries. Breast reconstruction has now become an option for many women undergoing prophylactic mastectomies. In some surveys, nearly half of women said that they regard it as a prerequisite for the actual preventative surgery itself. Prosthetics or tissue from another area of the body can be implanted behind or in front of the chest muscle; however utilizing tissue may cause additional complications. There is absolutely no point in saving any portion of the breast that is susceptible to cancer recurrence later in life. As such, adequate pre-operative information is of utmost importance for the women to have realistic expectations, and thus feel satisfied with the final result.
Not all discoveries of genetic mutations will lead to cancer in one’s lifetime. It is unlikely that patients at increased risk will reject all forms of risk-management options, even if they choose to defer prophylactic surgery. One should not underestimate the experiences of family members who suffered and succumbed to cancer, and how that impacts and shapes the views of those now seeking testing. Psychological health should always be monitored throughout the counselling process as well as during the recovery period after surgery. However, the actual decision must be made by the patient themselves, and not the clinician. Understanding the specific decision-making needs will provide insight into extending the preservation of the self, and ultimately, one’s health.
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