My grandmother was a fiercely independent person. After my grandfather died suddenly in his 70s, she lived alone for several decades. She made fancy butter cookies and packed them in colorful tins for her grandchildren. She did the crossword puzzle every day, in pen. Despite her diagnosis of small lymphocytic lymphoma (SLL) – a subtype of chronic lymphocytic leukemia (CLL) that primarily affects the lymph nodes – she never complained. Over the course of the disease, she eventually began to require frequent red blood cell transfusions and came to live with her son’s family. Unfortunately, shortly thereafter she fell and broke her hip. Hospitalized and sedated to ease her pain, she was unable to communicate her healthcare preferences. Who should decide the next steps in her care?
- The orthopedic surgeon – willing to operate
- The son, who is next of kin – who wants everything done
- The grandson, a specialist in geriatric medicine and the named proxy on her health care directive – who doubts any benefit from surgery
- All of the above
- None of the above.
Answer at end of article.
Over the past 20 years, great advances have been made in the management of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). Several recent studies have shown remarkable improvements in the length of survival from diagnosis, but all of us, including those with CLL, will eventually come to the end of our life. In this article we will try to help you plan for that inevitability, to empower you to get the care you want, to avoid care you don’t want, and most importantly to reduce stress and conflict among those who may need to make decisions for you.
The key strategy to planning end-of-life care is early engagement in the process of advanced care planning. This is the effort to think about and communicate your values and preferences with both loved ones and health care professionals. For those with CLL/SLL, this should begin shortly after diagnosis; it does not need to wait for severe illness, disability or advanced age.
The first key issue to consider is who should make healthcare decisions for you if you are too sick to communicate these decisions yourself. This is called a healthcare proxy, or a proxy decision maker. Typically, people choose a spouse or a child who lives nearby. This decision should not be taken lightly. The person chosen should be willing to serve in this role and be willing to honor your preferences, even if he or she doesn’t like them. For example, you might feel that you would not want to live in a nursing home due to advanced Alzheimer’s disease. Would the person you name as your proxy be able to withhold medications used for CLL/SLL, accepting the consequences of an earlier death related to the blood disorder versus a later death related to the Alzheimer’s?
In order to formalize your appointed healthcare decision maker in the event you can no longer communicate for yourself, you must complete a form called the Durable Power Attorney for Health Care (DPAHC). Though many primary care and hospital settings carry these forms, they can also be found on the internet. For legal validity, such a form requires signatures of either two witnesses OR a Notary Public but does not require an attorney. Even when there is no major medical decision required, a DPAHC assists in communication between health care providers and family in the event of a serious medical condition. For example, in the absence of a DPAHC, privacy laws and regulations could prevent loved ones from being informed of important information about someone involved in an accident.
Once you have identified your healthcare proxy, the next step is to consider and outline your healthcare preferences in the event you can no longer communicate them yourself. General questions to consider include, “Would I want to use technology such as a ventilator or feeding tube to sustain life if I were permanently unresponsive?” Another relevant question is, “Would I want treatment for life-threatening infection (a common cause of death among those with blood disorders like CLL/SLL) if there were no treatments remaining to control the underlying disease?” It is not necessary to make concrete decisions about such issues in advance; even providing general direction about values or preferences for your proxy decision maker(s) can reduce stress and conflict should the need for their help arise.
It is important to communicate your preferences regarding healthcare decisions, organ donation, and funeral plans clearly to your family members. One way to do that is to write them down in a legally binding format such as a Health Care Directive or Living Will. This protects loved ones and health care providers from lawsuits and other types of conflict if your documented preferences are honored. Some states have specific forms, but all states honor signed and witnessed forms completed in other states. Many states combine Health Care Directives and DPAHC into one form.
For help with the process of advance care planning, talk to your health care team. Medicare and many other insurers cover the cost of time spent in the clinic covering such issues. Some clinics train and assign nurses or social workers to take the lead on such discussions. It is best if your proxy decision maker(s) participate in these discussions.
Finally, the forms you complete need to be accessible if and when they are needed. Health care systems will copy forms into their records systems, but there may be difficulty accessing them by other health systems. The best approach is to also have copies in the files of all of your doctors and with your health care proxy.
Thinking and talking about end-of-life issues should be a routine part of care among those with CLL/SLL, their families, and their health care teams. This can start shortly after the shock of the diagnosis wears off. Ongoing consideration of the choice of proxy and preferences for care are needed as the blood disorder progresses, and as other serious illnesses may develop. Communication and documentation will reduce stress and conflict for loved ones when tough decisions need to be made.
Answer to the question at the beginning of the article:
Legally, the proxy decision maker can decide whether or not to proceed with hip surgery. In reality, differences of opinion among everyone in my family led to an effort to control pain without sedation so that my grandmother could communicate for herself. Through use of medications placed directly into the spinal cord (e.g. an epidural, as used in many deliveries and leg surgeries), my grandmother opted to follow the lead of her son, who had hopes for recovery. The comfort of this shared decision-making process eased everyone’s grief when she passed peacefully several weeks after surgery.
Edward Ratner, MD is a specialist in geriatric medicine and palliative care, who is an Associate Professor of Medicine at the University of Minnesota.
Amber Koehler, PA-C is an Assistant Professor of Medicine in the Division of Hematology at Mayo Clinic, Rochester, Minnesota
Originally published in The CLL Tribune Q3 2018.