210: Suicide Attempts in the Terminally Ill

FAST FACTS AND CONCEPTS #210
SUICIDE ATTEMPTS IN THE TERMINALLY ILL
Sean Marks MD and Drew Rosielle MD

Background Chronic or advanced medical illness is a significant risk factor for suicidality (1). This Fast Fact discusses evaluating and responding to suicidality in patients with life-limiting diseases. Note: this Fast Fact does not address cases of ‘physician assisted suicide’ which have occurred after a deliberative process including psychiatric screening such as in Oregon in the US (2).

Ethics
• Core Principles: It is a core obligation of physicians to prevent a patient from initiating suicide and to intervene medically to prevent a patient from dying after a suicide attempt (3). This obligation can include detaining and restraining patients against their will and the use of invasive medical interventions such as mechanical ventilation if needed, although such restrictions to a patient’s liberty should be kept to the minimum necessary. Most suicidal patients are considered impaired by depression or other mental illnesses and their actions are not considered autonomous, thus justifying detaining patients and providing medical interventions against their will (4). Such interventions can create further opportunities to treat the patient’s psychiatric condition, and only 20% of people who are prevented from committing suicide subsequently complete another attempt.

• Exceptions at life’s end: The above logic is less compelling in terminally ill patients who have attempted suicide, particularly with short prognoses (e.g. less than 1 month). In these patients most medical interventions are unlikely to restore health or significantly alter the dying process in a way that would materially benefit the patient. Clinicians may opt to forgo certain interventions (e.g. mechanical ventilation), particularly if family/proxy decision makers consider such interventions inappropriate in their dying loved ones. In these situations clinicians are advised to 1) verify the certainty of the very short prognosis, utilizing consultants liberally, 2) discuss with proxy decision makers all possible treatment plans including, when feasible, less-invasive supportive care options with treatment limitations (e.g. ICU monitoring, gastric lavage and charcoal administration for an overdose, but establishing a do-not-resuscitate/do-not-intubate order even if the patient deteriorates), and 3) seek ethics consultation.

• Advance Directives: Advance directives such as living wills stating a patient’s wish to not be mechanically ventilated are not binding in the setting of a suicide attempt. However, they should be honored in patients with short prognoses per the discussion above. Advanced directives specifying treatment limitations should be re-evaluated if it is suspected those statements were made during a period of undetected depression (5,6).

Epidemiology and Risk Factors The actual rate of suicide attempts in terminally ill patients is unknown, and there has been limited research into specifically ‘terminally ill’ populations. What is clear is that advancing age and psychiatric comorbidity are risk factors for suicide (along with male sex, AIDS diagnosis, a family history of suicide, and uncontrolled pain) (7,8). Cancer patients have nearly twice the incidence of suicide than the general population (rate of 31.4/100,000 vs. 16.7/100,000 patient-years) (6). Lung, stomach, and head and neck cancers have the highest suicide rates among all cancer types (9). Up to 8.5% of terminally ill cancer patients express a sustained and pervasive wish for an early death, and in one survey of terminally ill patients, 10% of patients reported “seriously pursuing” physician assisted suicide (10,11). Rates of actual suicide attempts are presumably lower.

Assessment All patients with life-limiting illnesses should be routinely assessed for depression and mood disorders (see Fast Facts #7, 43); depressed patients should be screened for suicidal thoughts. Patients who admit to suicidal thoughts or a desire for hastened death should be asked about specific plans for self-harm, past history of suicide attempts, access to firearms or other lethal means to carry out a suicidal act, and level of support/supervision available in the home (e.g. family caregivers). Although some clinicians may be concerned that exploring suicidal thoughts may make suicide more likely, there is no evidence that this occurs. Many ill patients who express a desire for death are simply communicating unresolved emotional and existential concerns about dying: see Fast Facts #156 & 159.


Responding to Suicidal Intent All patients who are seriously threatening self-harm, or who have pervasive thoughts of ending their life, should be evaluated urgently by a psychiatrist (12). Immediate resources depend on local availability and can include prompt evaluation by an established psychiatrist, medical or psychiatric urgent care clinics or emergency departments, or even voluntary hospital admission. Options include voluntary psychiatric treatment, arranging 24 hour safety monitoring from the patient’s family and friends, introducing home hospice or home nursing support, removing means to carry out a suicidal act, and imposing emergency detention. For disabled patients close to death, removing the means of self-harm (e.g. limit access to pain medications as long as a reliable family member can administer them) and providing close supervision through, for instance, hospice services are often sufficient and minimally restrictive.

References
1. Hendin H. Suicide, assisted suicide and medical illness. J of Clin Psychiatry. 1999; 60 Suppl 2:46-50.
2. Death With Dignity Act Report 2007. State of Oregon Department of Human Services. Available at: http://www.oregon.gov/DHS/ph/pas/index.shtml. Accessed November 10, 2008.
3. Lo B. Resolving ethical dilemmas: a guide for clinicians – 3rd Edition. Lippincott Williams & Wilkins, 2005: 256-9.
4. Miller RD. Need-for-treatment criteria for involuntary civil commitment: impact in practice. Am J Psychiatry. 1992; 149:1380-4.
5. Ganzini L, Lee MA, Heinz RT, et al. The effect of depression treatment on elderly patients’ preferences for life sustaining medical therapy. Am J of Psychiatry. 1994; 151:1631-6.
6. Sontheimer D. Suicide by advance directive. J Med Ethics. 2008; 34:e4; doi:10.1136/jme.2008.025619.
7. Farberow NL, Banzler S, Cutter F, Reynolds D. An eight-year survey of hospital suicides. Suicide and Life-Threatening Behavior. 1971; 1:184-201.
8. Rabins PV. Prevention of mental disorder in the elderly: current perspectives and future prospects. J Am Geriatric Soc. 1992; 40:727-33.
9. Misono S, Weiss NS, Fann JR, Redman M, Yueh B. Incidence of suicide in persons with cancer. J Clin Oncol. 2008; 26:4731-4738.
10. Chochinov HM, Wilson KG, Enns M, Mowchun N, Lander S, Levitt M, et al. Desire for death in the terminally ill. Am J Psychiatry. 1995; 152:1185-91.
11. Emanuel EJ, Fairclough DL, Emanuel LL. Attitudes and desires related to euthanasia and physician-assisted suicide among terminally ill patients and their caregivers. JAMA. 2000; 284:2460-2468.
12. Block SD. Chapter 6: Assessing and managing depression in the terminally ill patient. Physician’s Guide to End of Life Care. Snyder, Lois ACP Press, 2001.

Author Affiliations: Medical College of Wisconsin, Milwaukee, Wisconsin.


The original version of this Fast Fact can be found at EPERC.

#209: Malignant Pericardial Effusions

MALIGNANT PERICARDIAL EFFUSIONS

Vincent Thai MD

Introduction   Malignant pericardial effusions (MPEs) are a rare complication of advanced cancer, but are associated with high morbidity and mortality.  This Fast Fact discusses the diagnosis and management of MPEs.

Epidemiology and Prognosis   Approximately 10% of patients with cancer develop cardiac metastases, with ~75% of these affecting the epicardium (1, 2).  Only a third of these, however, will develop clinically significant MPEs (1).  Lung and breast cancers are the most common causes.  MPEs are associated with a poor prognosis.  Studies suggest a median survival of 2-3 months after a MPE is diagnosed, with a mean survival of 5 months for solid tumors and 20 months for hematologic malignancies (3, 4).

  

Physiology and Symptoms  The pericardial space is normally filled with less than 50 ml of serous fluid. As this volume increases due to epicardial or pericardial metastases or lymphatic obstruction, both right and left ventricular failure can occur due to inadequate filling.  Signs and symptoms include peripheral and pulmonary edema, chest discomfort, cough, shortness of breath, and orthopnea.  Severity of symptoms depends on the volume of the MPE as well as the rapidity of its accumulation; severe cases can present with cardiac tamponade and shock.  An echocardiogram is indicated whenever a MPE is suspected.  Not only does it confirm the presence of an effusion, but its findings can dictate whether or not urgent treatment is indicated (e.g. if signs of tamponade are evident).  A diagnostic pericardiocentesis or pericardial biopsy is sometimes needed to confirm the cause of the effusion.

 

Treatment Options    

·   Systemic chemotherapy or radiotherapy are effective for chemo- or radio-sensitive tumors such as previously untreated breast cancer and many lymphomas. Reaccumulation rates for both modalities are about 1/3 overall, depending on the patient’s overall course and response to therapy (5).

·   Pericardiocentesis results in immediate symptom relief in most patients, however the effusion may rapidly re-accumulate in many patients, needing repeat pericardiocentesis (within 1-2 weeks in some series) (6). 

·   Pericardial sclerosis involves instilling a sclerosing agent with the intention of scarring the pericardium to the epicardium, preventing reaccumulation of the MPE (similar to pleural effusions – see Fast Fact #157).  Multiple agents have been studied including doxyclycline, minocycline, and bleomycin.  Success rates (no reaccumulation at 30 days) are about 70-90% (7, 8).  Longer term success rates have not been defined due to the poor survival of study patients. The major side effect is chest pain (50-70%), cardiac arrhythmias, and fever (8, 9, 10).  In head to head comparisons with doxycycline, bleomycin has been shown to have fewer side effects and to lead to shorter hospitalizations (10, 11, 12).

·   Surgical decompression therapies range from less invasive (balloon pericardiotomy, subxiphoid or thorascopic pericardiostomy) to more extensive (open thoracotomy with pericardial stripping).  A pericardial ‘window’ (which allows ongoing drainage of fluid externally or internally such as into the pleural cavity) is often created.  Case series have suggested reaccumulation rates with surgical therapies are low (less than 15% up to 10 months out) (13, 14, 15).

 

Decision-Making   The treatment of MPEs depends on how urgently treatment is needed, the likelihood of the tumor responding to anti-neoplastic treatments, and the anticipated survival of the patient.  A multidisciplinary approach to decision-making, involving input from medical and radiation oncology, cardiology, and thoracic surgery is recommended. Simple pericardiocentesis may be appropriate for patients with short prognoses (less than 1 month), particularly if their MPE is not expected to re-accumulate in their remaining life-span.  A symptomatic patient with no signs of tamponade and a chemotherapy-sensitive tumor such as untreated breast cancer may receive a durable response from a pericardiocentesis for symptom relief, followed by chemotherapy.  Patients with longer prognoses (>1 month) who are expected to re-accumulate their MPEs will likely benefit most from sclerosis or surgical decompression; there is no clear evidence currently suggesting one strategy is superior to the other.  Symptom directed care without specific intervention for the MPE is an appropriate option for patients with very short prognoses and for those who decline more invasive treatments.

 

References

1. Klatt EC, Heitz DR. Cardiac metastases. Cancer. 1990; 65(6):1456-59.
2. Abraham KP, Reddy V, Gattuso P. Neoplasms metastatic to the heart: review of 3314 consecutive autopsies. Am.J.Cardiovasc.Pathol. 1990; 3:195-198..
3. Moores, D.W, Allen K.B, Faber L.P, Dziuban S.W, Gillman D.J, Warren W.H., Ilves R, Lininger L, Subxiphoid pericardial drainage for pericardial tamponade, The Journal of Thoracic and Cardiovascular Surgery 1995, 109 (3), 546-552
4. Dosios T, Theaskos,N, Angouras D, et al. Risk factors affecting the survival of patients with pericardial effusion submitted to subxiphoid pericardiostomy. Chest. 2003; 124:242
5. Lamont E, Hoffman PC. Oncologic Emergencies (chapter). In: Principles of Critical Care. 3^rd Edition. McGraw Hill; New York: 2005. 
6. Laham RJ, Cohen DJ, Kuntz RE et al. Pericardial effusion in patients with cancer: outcome with contemporary management strategies. HEART. 1996; 75(1):67-71.
7. Lashevsky I, Ben Yosef R, Rinkevich D, Reisner S, Markiewicz W. Intrapericardial minocycline sclerosis for malignant pericardial effusion. Chest. 1996;109(6):1452-54.
8. Maher EA, Shepherd FA, Todd TJR. Pericardial sclerosis as the primary management of malignant pericardial effusion and cardiac tamponade. J Thoracic Cardiovascular Surg. 1996; 112(3):637-643.
9. Ben Yosef,R.; Phefer,R.; Ge,A.; Catane,R. Management of malignant pericardial effusion Harefuah, 1988, 115, 5-6, 138-141, ISRAEL
10. Liu G, Crump M, Goss PE, Dancey J, Shepherd FA. Prospective comparison of the sclerosing agents doxycycline and bleomycin for the primary management of malignant pericardial effusion and cardiac tamponade. J Clin.Oncol. 1996; 14(12):3141-47.
11. Yano T, Yokoyama H, Inoue T, et al. A simple technique to manage malignant pericardial effusion with a local instillation of bleomycin in non-small cell carcinoma of the lung. Oncology. 1994;51:507-509.
12. van Belle SJ, Volckaert A, Taeymans Y, Spapen H, Block P. Treatment of malignant pericardial tamponade with sclerosis induced by instillation of bleomycin. Int.J.Cardiol. 1987; 16(2):155-160.
13. Galli M, Politi A, Pedretti F, Castiglioni B, Zerboni S. Percutaneous balloon pericardiotomy for malignant pericardial tamponade. Chest. 1995; 108(6):1499-1501.
14. Palacios IF, Tuzcu EM, Ziskind AA, Younger J, Block PC. Percutaneous balloon pericardial window for patients with malignant pericardial effusion and tamponade. Cathet.Cardiovasc.Diagn. 199; 22(4):244-49.
15. Ziskind AA, Pearce AC, Lemmon CC, et al. Percutaneous balloon pericardiotomy for the treatment of cardiac tamponade and large pericardial effusions: description of technique and report of the first 50 cases. J.Am.Coll.Cardiol. 1993; 21(1):1-5.

The original version of this Fast Fact is on the EPERC website.  

#208: Clinical care following withdrawal of dialysis

FAST FACTS AND CONCEPTS #208

CLINICAL CARE FOLLOWING WITHDRAWAL OF DIALYSIS

Sara N Davison MD and Drew A Rosielle MD

 

Background   Fast Fact #207 discussed decision-making around dialysis discontinuation; this Fast Fact addresses care of the patient around the time of discontinuation.

 

Communication and care-planning at the time of dialysis cessation

·         Counsel about what to expect: mean survival following dialysis withdrawal is 8-10 days (although rarely can be many weeks).  Address the likelihood of progressive encephalopathy.

·         Counsel about symptoms (see below).  Reassure patients/families that these can be adequately treated, although drugs with sedating side effects may be necessary to ensure comfort.

·         Recommend continuing fluid restriction (< style="mso-spacerun:yes">  Discuss diet: a liberal, pleasure-based diet is appropriate for many patients although they should be cautioned it could worsen symptoms from edema.

·         Address potential care sites for the final days of life.

·         Review other medical treatments the patient is receiving and discontinue those that will not improve their quality of life while dying; clarify treatment limitations including resuscitation (code) status.

·         Provide emotional/psychological, spiritual, social work, and bereavement support services.

 

Symptom Management   In one cohort of hospitalized patients who stopped dialysis confusion/agitation was reported to affect 70% of patients, followed by pain (55%), dyspnea (48%), nausea (36%), twitching/seizures (27%), anxiety/psychological distress (27%), pruritis (24%), and peripheral edema (21%).  Because of a paucity of clinical research, the following recommendations are largely based on clinical experience and pharmacologic common sense. Many drugs which were previously cleared by dialysis may need to be dose-adjusted or discontinued.  Treatment plans should be frequently re-evaluated, with particular attention to the use of scheduled medications. 

·         Pain management:  Acetaminophen is the agent of choice for mild pain.  Fast Fact #161 addresses opioid use in renal failure.  Fentanyl and methadone are considered safest after dialysis discontinuation, although methadone should only be initiated by clinicians familiar with its use.  Toxic hydromorphone metabolites, previously cleared by dialysis, can accumulate rapidly once dialysis is stopped and it should be used with caution and close monitoring of side effects.  Gabapentin and pregabalin quickly accumulate once dialysis is stopped and should be discontinued or severely dose-reduced (see Fast Fact #49).

·         Shortness of breath:  Oxygen, positioning, and opioids are the mainstays of therapy (see Fast Fact #27). Ultrafiltration is not recommended as it can be distressing for patients/family to see the patient back on a therapy which appears similar to hemodialysis. For the occasional patient who has a residual urine output of >100ml/day, high dose diuretics can be used.   

·         Anxiety/agitation/restlessness:  Assure pain and psychosocial issues are addressed.  Haloperidol or benzodiazepines are effective.  Haloperidol may lower the seizure threshold and the metabolites are excreted in the urine and feces so it is recommended to dose at half the typical starting dose following dialysis withdrawal. While benzodiazepines do not accumulate in chronic kidney disease, clinical experience supports starting with very low doses in this population. 

·         Restless legs:  Clonazepam is particularly useful for the restless legs associated with uremia (0.5 – 2.0 mg bid). Clonidine (0.1-0.2 mg bid) can also be used.

·         Muscle cramps: Dialysis patients are often treated with quinine sulphate which accumulates rapidly once dialysis is stopped and should be discontinued.  Clonazepam and other benzodiazepines are better in this setting.

·         Nausea:  Reduced doses of metoclopromide (starting at 5mg bid) are effective for gastroparesis.  Uremia-induced nausea often responds well to dopamine antagonists such as haloperidol and prochloperazine which are often sedating in the context of uremia.  Ondansetron has some advantages as it is less sedating and does not accumulate in kidney failure. 

·         Pruritus: Emollients such as hydrourea cream, ondansetron, and antihistamines may be beneficial.  Gabapentin, while effective, is too toxic in this population to initiate its use.

 

References

1.       Germain MJ, Cohen LM, Davison SN. Withholding and Withdrawing from Dialysis: What We Know About How Our Patients Die.  Seminars in Dialysis. 2007; 20:200-204.

2.       Murtagh FEM, Addington-Hall JM, Donohoe P, Higginson IJ.  Symptom Management in Patients With Established Renal Failure Managed Without Dialysis.  EDTNA ERCA J. 2006; 32:93-98.

3.       Chambers EJ, Germain M, Brown E (Eds.).  Supportive Care for the Renal Patient (1st Edition). Oxford: Oxford University Press; 2004. 

4.       Cohen LM, Germain MJ, Poppel DM.  Practical considerations in dialysis withdrawal.  “To have that option is a blessing.”  JAMA. 2003; 289:2113-2119.

5.       Murtagh FE, Chai MO, Donohoe P, Edmonds PM, Higginson IJ.  The use of opioid analgesia in end-stage renal disease patients managed without dialysis: recommendations for practice.  J Pain Palliat Care Pharmacother. 2007; 21:5-16.

6.       Lugon JR.  Uremic pruritus: a review.  Hemodialysis Intl. 2005; 9:180-88.

 

Author Affiliations:  University of Alberta, Edmonton, Alberta (SND), and the Medical College of Wisconsin, Milwaukee, Wisconsin (DAR).

This Fast Fact is also available at EPERC.  

#207: Withdrawal of dialysis: decision-making

FAST FACTS AND CONCEPTS #207

WITHDRAWAL OF DIALYSIS: DECISION-MAKING

Sara N Davison MD and Drew A Rosielle MD

Background   Historically, stopping dialysis was considered by many to be a form of suicide.  However, it is now a widely accepted practice in most countries, with broad ethical and legal consensus that dialysis can be stopped when it is no longer achieving a meaningful goal for the patient. In fact, ~25% of deaths of dialysis patients in North America occur after its cessation.  This Fast Fact reviews key issues pertaining to the decision to stop chronic dialysis; Fast Fact #208 will discuss the care of patients after it is stopped.

 

Why dialysis is stopped   The goal of dialysis is not only to prolong life by providing renal replacement therapy, but to maintain a patient’s quality of life at an acceptable level (see Fast Fact #163).  Discussions to stop dialysis usually occur when:

·   Dialysis is no longer serving to substantially prolong life or is only prolonging a patient’s death (e.g., a patient dying from advanced cancer or sepsis with multiorgan system failure).

·   The burdens of dialysis and its complications outweigh its life-prolonging benefits to a patient (e.g., a patient with progressive frailty who is becoming bedbound, a patient with severe cognitive failure).  In these scenarios dialysis is likely to prolong life but is not helping to restore a patient to an acceptable level of quality of life as assessed by the patient or her/his surrogate decision maker.

 

Demographics   The demographics of dialysis withdrawal have been studied at length.  Patient characteristics associated with withdrawal are older age, female, white race, longer duration of dialysis, higher educational level, living alone, severe pain, and comorbidity (with chronic or progressive diseases).  Ethnic differences have been observed, with African Americans and Hispanics being less likely to stop dialysis than European Americans.  Reported prevalence levels of patient decision-making capacity at the time of withdrawal vary considerably with estimates ranging from 37% to 80%, suggesting cognitive failure drives many of these decisions. Nephrologists rate cognitive and physical functional status as the most important factors for their decision-making around stopping dialysis, and 93% of North American nephrologists report a willingness to honor a patient’s request to stop, even if they have a personal preference to continue.  Internationally, practices vary tremendously, with much lower rates of dialysis cessation in Japan compared to North America, for instance.

 

Responding to a request to stop dialysis   For patients who are otherwise dying, counsel about terminal care issues surrounding dialysis withdrawal (see Fast Fact #208).  For patients not otherwise close to death, explore reasons for withdrawal, especially for treatable factors that might contribute to the desire to withdraw dialysis.  For patients whose desire to stop dialysis is being driven by factors that are potentially ameliorable, clinicians should make sure that the decision to stop dialysis is fully informed, including the possibility that some concerns could be addressed.  These include:

·   Inadequately treated depression, anxiety, pain, and other physical or psychological symptoms (including spiritual and existential suffering)

·   Dissatisfaction or difficulties with dialysis itself (e.g., modality, time commitment, or setting of treatment)

·   Inadequate social support, or concerns with being a burden to loved ones. 

Offer to evaluate and treat these concerns; consider a time-limited trial to see if a patient’s quality of life can be improved.  However, once a clinician feels a patient or surrogate is making a fully informed choice that is consistent with a patient’s values and goals, that decision should be honored.  Proactively address any concerns patients may voice about the ethics of withdrawal.

 

Broaching dialysis withdrawal   Clinicians who are concerned continuing dialysis is no longer benefitting a patient due the reasons described above should broach discontinuation with the patient and family.  This discussion should occur as part of a larger goals-of-care conversation which addresses prognosis (see Fast Fact #191), patient/family assessment of quality of life, and establishes realistic care goals.  Dialysis should be discussed as part of an overall medical plan and framed as how it can or cannot address the care goals.  “Dialysis will likely make your mother live longer.  However – given everything that has been happening – it is not going to improve her strength, memory, or ability to take care of herself.  Based on what you’ve told me about your mother and what is important for her, I would recommend stopping the dialysis as it is only serving to maintain her in a state she would find unacceptable.” 

 

References

1.       Galla JH and the Renal Physicians Association/American Society of Nephrology Working Group. Clinical Practice Guideline on Shared Decision-Making in the Appropriate Initiation of and Withdrawal from Dialysis.  J Am Soc Nephrol. 2000; 11:1340-1342.

2.       Murtagh F, Cohen LM, Germain MJ. Dialysis Discontinuation: Quo Vadis?  Advances  Chronic Kid Dis. 2007; 14(4):379-401.

3.       Holley JL, Davison SN, Moss AH. Nephrologists’ Changing Practices in Reported End-of-Life Decision-Making.  Clin J Am Soc Nephrol. 2007; 2:107-111.

4.       White Y, Fitzpatrick G.  Dialysis: prolonging life or prolonging dying? Ethical, legal and professional considerations for end of life decision making.  EDTNA ERCA J. 2006; 32:99-103.

5.       Cohen LM, Germain MJ, Poppel DM.  Practical considerations in dialysis withdrawal.  “To have that option is a blessing.”  JAMA. 2003; 289:2113-2119.

 

Author Affiliations:  The University of Alberta, Edmonton, Alberta (SND), and the Medical College of Wisconsin, Milwaukee, Wisconsin (DAR). 

This Fast Fact is also available at EPERC.  

#206: Genetic screening and DNA banking at the end of life

fast facts and concepts #206

Genetic Screening and DNA Banking at the End of Life

John M. Quillin PhD, Joann N. Bodurtha MD, and Thomas J. Smith MD

Background    Many dying patients voice concern for the health of surviving family members.^{1, 2}  The most common causes of death can cluster in families, and this clustering can reflect shared family genes. About 5% to 10% of cancers are strongly hereditary,³ and a family history of heart disease is well established as a risk factor for the disease.^4-6  Family members may benefit from knowing their genetic risk, and offering testing can be a generative act for a dying patient.  Today’s genetic tests can identify known markers of disease for only some families, so testing is most helpful if it includes the affected patient (otherwise ‘negative’ test results are less informative). In addition, more informative genetic tests will likely be available in the future. Once patients die, however, their DNA is no longer readily available for this future testing. On the other hand, decisions about genetic testing are complex and can have profound emotional, familial, and financial impacts on those affected and should not be pursued hastily. 

 

Possible Genetic Conditions    If a patient or family member asks if survivors could be affected by the patient’s disease, consider recommending genetic testing or banking for known genetic disorders (e.g. hemochromatosis, cystic fibrosis) and in the following settings:

·     Earlier-than-expected age at diagnosis (e.g. breast cancer before age 50)

·     Multiple primary cancers (e.g. a history of both colon and ovarian cancer)

·     A major birth defect (e.g. spina bifida, congenital heart defect) or multiple minor physical anomalies

·     Profound hearing or vision loss without an environmental explanation

·     Developmental disability or autism

·     Disorders of sexual development

·     Unusually tall or short stature compared to relatives

·     Unusual skin pigmentation, such as ≥6 café-au-lait spots, or lumps (e.g., multiple lipomas)

·     Congenital myopathy or muscular dystrophy

·     Cardiomyopathy or arrhythmia without clear cut cause, or at an age earlier than expected

·     Suspected connective tissue disease (e.g. hyperflexibility)

·     Excessive bleeding or clotting tendencies not associated with medication or comorbidity

·     Seizures without an identifiable etiology

 

Talking to Patients and Families   There is no consensus on who should be approached for discussion of familial risk, and to date no studies on effective communication strategies for genetic screening at the end of life are available, despite acknowledgment of a practice gap.^7-9   In practice, a straightforward invitation for discussion of the topic may be effective: “I’d like to talk with you about your family health history. Some health conditions tend to run in families and knowing your health history and seeing if it is connected to your illness could help others in your family to stay healthy. Most diseases are not strongly genetic, but sometimes it is helpful to have a genetic test or store a blood sample for testing later.” 

 

Genetic counselors, medical geneticists, and genetic nurses can facilitate comprehensive genetics evaluation, assist in test selection, provide informed consent, and educate patients and family members about indications for and costs of testing and banking. Genetic consultation is usually covered by major insurers and Medicare, especially for patients with active disease such as cancer.

 

DNA Banking   Banking involves drawing blood for long-term storage at a DNA banking facility.  DNA banking is typically not covered by insurance; costs vary from one to a few hundred dollars. Facilities offering DNA banking vary in their informed consent requirements and documentation for ownership of samples (e.g. who is authorized to submit a sample to a laboratory for genetic testing).  Banking can be particularly helpful considering turn-around-time for genetic test results can be long and more informative tests may become available in the future.  In addition, given the complex and emotional decision making that can be involved in genetic testing, banking gives family members time to seek counseling and to carefully consider such decisions, without feeling pressure to pursue testing before a loved one dies. 

 

Resources    Clinical laboratories that currently offer DNA banking can be found at http://www.genetests.org. Providers can search for nearby genetic counselors and geneticists at http://www.nsgc.org and http://www.acmg.net. General resources for clinicians about genetic counseling and testing can be found in references 10-13. 

References

1. Duggleby W, Wright K. Elderly palliative care cancer patients' descriptions of hope-fostering strategies. Int J Palliat Nurs. 2004;10:352-359.
2. Skirton H, Frazier LQ, Calvin AO, Cohen MZ. A legacy for the children--attitudes of older adults in the united kingdom to genetic testing. J Clin Nurs. 2006;15:565-573.
3. Offit K. Clinical Cancer Genetics. New York: Wiley-Liss, Inc.; 1998.
4. Hunt SC, Gwinn M, Adams TD. Family history assessment: Strategies for prevention of cardiovascular disease. Am J Prev Med. 2003;24:136-142.
5. Murabito JM, Pencina MJ, Nam BH, et al. Sibling cardiovascular disease as a risk factor for cardiovascular disease in middle-aged adults. JAMA. 2005;294:3117-3123.
6. Lloyd-Jones DM, Nam BH, D'Agostino RB S, et al. Parental cardiovascular disease as a risk factor for cardiovascular disease in middle-aged adults: A prospective study of parents and offspring. JAMA. 2004;291:2204-2211.
7. Kirk J. The family history of cancer - a common concern in palliative care. Progress in Palliative Care. 2004;12:59-65.
8. Lillie AK. Exploring cancer genetics and care of the family: An evolving challenge for palliative care. Int J Palliat Nurs. 2006;12:70-74.
9. Quillin JM, Bodurtha JN, Smith TJ. Genetics assessment at the end of life: Suggestions for implementation in clinic and future research. J Palliat Med. 2008;11:451-458.
10. Pletcher BA, Toriello HV, Noblin SJ, et al. Indications for genetic referral: A guide for healthcare providers. Genet Med. 2007;9:385-389.
11. American Society of Clinical Oncology. American society of clinical oncology policy statement update: Genetic testing for cancer susceptibility. J Clin Oncol. 2003;21:2397-2406.
12. Genetics and Your Practice. March of Dimes.  Available at: http://marchofdimes.com/gyponline/index.bm2.  Accessed July 10, 2008.
13. National Office of Public Health Genomics.  Centers for Disease Control and Prevention.  Available at: http://www.cdc.gov/genomics/.  Accessed July 10, 2008.

 

Author Affiliations:  Massey Cancer Center, Virginia Commonwealth University.

This Fast Fact is available at EPERC.

#205: Destination Ventricular Assist Devices for Heart Failure

FAST FACTS AND CONCEPTS #205

Destination Ventricular Assist Devices for heart failure

Heather Ferris MD, PhD and Susan Hunt MD

Introduction   About 250,000 Americans have end-stage heart failure (‘Class IV’ by the New York Heart Association criteria, meaning patients cannot carry out any physical activities without discomfort and experience dyspnea or angina at rest).  Fewer than 1% of patients, however, will receive a heart transplant. The left ventricular assist device (LVAD) was initially designed as an implanted mechanical circulatory support to extend the life of patients awaiting heart transplants (“bridge therapy”).  In 2002, the FDA approved the LVAD not only as “bridge therapy”, but also as an alternative to transplantation, or “destination therapy.”

 

The Technology   The LVAD is a surgically implanted pump with two conduits, one of which is implanted in the left ventricle, the other into the aorta.   Blood is pulled from the left ventricle, and pumped into the aorta, increasing cardiac output and reducing heart failure symptoms.  A third conduit passes from the pump through the abdominal wall, and attaches to the LVAD’s battery and control system.  Right and bi-ventricular assist devices also exist, but are not currently approved for destination therapy.  Patients can go home on most types of assist devices.  [CB1] LVAD patients may use a wearable battery system for several hours, which allows them more maneuverability. To qualify for destination LVAD therapy, a patient must have refractory Class IV heart failure, severe systolic dysfunction (ejection fraction <25%),>

 

Outcomes

·   LVADs can prolong life.  REMATCH, a randomized controlled trial, compared destination LVAD therapy to medical therapy.   LVAD patients had 1-year and 2-year survivals of 52% and 29% respectively, compared to 25% and 13% for medically managed patients.  The mean survival for LVAD patients was 14 months, compared to 9 months for medically managed patients.  Depression and health-related quality of life were improved in LVAD recipients.

·   Shorter survival is predicted by poor nutritional status, low serum albumin, coagulation abnormalities, impaired renal function, and signs of right heart failure. A pre-operative risk model has been developed using these factors (Lietz 2005); using this model, the 90-day and 1-year survival for low-risk patients is 93% and 81% respectively, compared to 18% and 11% for high-risk patients.

·   Complications of LVAD therapy include stroke, multi-organ failure, bleeding, thromboembolic disease, and sepsis.  Peri-operative mortality is very high (33%).  Patients generally spend 20% of their survival time in the hospital.  Some patients can perform all activities of daily living, but anxiety among patients and caregivers may be significant.  LVAD alarms, which occur an average of 6.7 times daily, are particularly distressing.

 

Discontinuing LVADs and Care Planning   LVADs may be implanted as a bridge to transplantation, but later become destination therapy when patients are no longer transplantation candidates.  In the rare instance of left ventricular recovery, LVADs can be explanted.  More often, LVADs are removed at cardiac transplantation or, in the case of destination therapy, when severe complications arise.  When the LVAD is turned off, not only does support to the damaged heart stop, but the non-functioning device causes impediment to the pumping of the native heart, thus in a small way hastening death.  Patients usually die rapidly after device discontinuation.  Discussions leading to a decision to discontinue the LVAD should focus on its inability to continue to fulfill its intended goals, and the importance of providing comfort care for a dying patient. 

 

LVAD therapy is a surgical therapy which can prolong life and improve function in selected patients, but is associated with very high mortality and treatment burden.  Discussions with patients and surrogates to clarify prognosis, goals, and endpoints for LVAD therapy should take place before implantation.  These discussions should address the quality of life below which a patient would no longer want to continue mechanical circulatory support, and would want to initiate comfort-only care. Palliative care physicians may be involved in these discussions, and involved in evaluating patients who have suffered complications or request LVAD discontinuation.     

 

References

1. Lietz K et al.  Outcomes of Left Ventricular Assist Device Implantation as Destination Therapy in the Post-REMATCH Era; Implications for Patient Selection.  Circulation.  2007; 116:497-505.
2. Park SJ et al. Left Ventricular Assist Devices as Destination Therapy: A New Look at Survival.  J Thorac Cardiovasc Surg.  2005;129:9-17.
3. Rose EA et al. Randomized Evaluation of Mechanical Assistance for the Treatment of Congestive Heart Failure (REMATCH) Study Group: Long-term Use of a Left Ventricular Assist Device for End-stage Heart Failure.  N Engl J Med.  2001;345:1435-1443.
4. Bramstedt KA, Wenger NS.  When Withdrawal of Life-Sustaining Care Does More Than Allow Death to Take Its Course: The Dilemma of Left Ventricular Assist Devices.  J Heart Lung Transplant.  2001;20:544-548.
5. Slaughter MS et al.  Home Discharge Experience With the Thoratec TLC-II Portable Driver.  ASAIO Journal.  2007;53:132-135.

Author Affiliations:  University of Pittsburgh Medical Center.

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