End of Life Planning

Unfortunately, prion diseases do not currently have any current or cure.  Treatment is usually focused on managing symptoms and enhancing quality of life.  Because of this, families must often consider end-of-life issues shortly after receiving a diagnosis of prion disease.  The following provides information regarding several end-of-life issues that patients and families face following a diagnosis of prion disease.

A family will want to contact hospice once a diagnosis of prion disease has been given to the patient.  Although hospice deals with palliative care towards the end of life, there are many other resources that hospice can provide earlier in the disease course.  Although the survival time is variable in prion diseases, the average duration is 4-6 months.  Hence, a diagnosis of prion disease alone should be sufficient for hospice enrollment.  In order to be enrolled in hospice, you will need an order from a physician for hospice evaluation and management.  When investigating which hospice to use, consider the following resources that are often delivered through hospice, though individual programs may vary.

Home hospice versus nursing home: Hospice can be part of the treatment team in virtually any setting.  They can come to your home and do assessments, offer advice on care, and prescribe medications as needed.  The goal of home hospice is to make sure the patient is comfortable and that the caregivers have the resources and equipment (e.g., hospital bed, shower chair, etc.) necessary to provide care.  For a variety of reasons, patients may be placed into a nursing facility where professional services are more readily available.  Hospice can also be part of a patient’s care in a nursing facility.  Many hospices also offer inpatient hospice, which is usually provided to patients that need more assistance with managing symptoms. 

Nursing: Although your loved one’s care is overseen by a physician, nurses provide the majority of hospice care.  Different hospices vary, but most hospices provide nursing visits at least weekly depending on how the patient is doing.  The frequency of visits may increase as symptoms progress.  Nurses provide important assessments, care advice, and medication education. 

Social Work: Most hospices have social workers, which are a very important part of the treatment team.  They can help guide you on topics such as disability, placement, and end of life decisions.  Social workers are also an important support to caregivers and often can be useful in helping to guide how caregivers may better react to various behaviors that a patient may demonstrate. 

Bereavement services: An important goal of hospice is helping families through the grieving process.  As this often starts prior to a loved one’s death, hospices often provide grief counseling prior to a loved one’s passing as well as following their passing.  They may have special grief counseling programs for young children. 

Art therapy:  Art therapy can be used to engage not only patients, but family members as well.  In addition to providing therapeutic benefits, clinicians often like to see artwork by patients to help gauge symptom progression. 

Music therapy:  Music therapy focuses on listening to and making music as part of the therapeutic process.  Music therapy can be helpful for patients with cognitive impairment and/or agitation.

Link: For information on Caregiving for a prion disease patient, see Caregiving Ideas and contact the CJD Foundation Helpline to ask questions and request a Caregiving Guide.

Prion disease can affect younger people, who may still be working and dependent on income from employment.  When patients have to stop working because of their symptoms, loss of income may be an added stress for families.  In those under the age of 62, Social Security Disability Insurance (SSDI) may be an option to help offset the loss of salary.  A useful guide for applying for SSDI can be obtained from the Alzheimer’s Association.  Eligibility criteria for SSDI is subject to change and we recommend that you check with your local Social Security office to determine eligibility.  Sometimes, an eldercare lawyer can be helpful to assist in maneuvering the system in a timely and efficient manner.  Your local Alzheimer’s Association may have a list of eldercare lawyers in your area. 

Although there is usually a two-year waiting period from the time of SSDI approval to receipt of funds, several diseases are granted an exemption from this waiting period through the Compassionate Allowances Initiative.  All prion diseases are considered conditions eligible for this “fast-track” program.  In addition to receiving disability income, SSDI allows individuals to become eligible for Medicare (immediately if your income level qualifies) or Medicare (after two years).

End of Life Decision Making
While it is ideal to examine end of life issues prior to any medical problems, sometimes a diagnosis of prion disease is the first time these issues are raised.  In the event that a patient can no longer express their wishes, either because they are physically or mentally incapacitated, then clinicians will refer to a patient’s Advance Directives.  Advance Directives are the patient’s medical wishes.  There are two types of Advance Directives: Living Will and Healthcare Power of Attorney (POA).  In general, a POA is recommended over a Living Will as medical events may not transpire exactly as a Living Will may be written, resulting in lack of clarity and possible confusion.  Living Wills and POAs may differ depending on what state you live in and you are encouraged to contact your state government or lawyer to ensure that the proper paperwork is completed.  

Living Will: A living will is written very similar to how one might write a Last Will and Testament.  An individual can specify what they would and would not want done in specific medical circumstances.  For example, one might specify that they would wish to be disconnected from a breathing machine should there be no meaningful chance of recovery.

Healthcare POA: A healthcare POA is someone that you designate to make healthcare decisions for you should you be incapacitated.  Ideally, this will be someone with whom you have discussed end of life decisions.  A POA does not have to be a family member. 

DNI (Do Not Intubate):  This is an advance directive that requests that an individual not be placed on a breathing machine.

DNR (Do Not Resuscitate): This is an advance directive that requests that no life saving measures be performed should one’s heart stop working. 

Financial POA: A financial POA differs from a healthcare POA in that he or she is responsible for making decisions regarding your finances and property should you be incapacitated.  Your financial POA can be a different person from your healthcare POA.

Guardian: POAs only hold power as long as the patient agrees to their actions.  There may be circumstances in which an individual may not be competent to make healthcare or financial decisions. In this scenario, guardianship may be appropriate.  A guardian is an individual appointed by the court to direct someone’s financial and health care decisions usually due to an individual being incompetent to do so on their own.  Guardianship laws vary by county and you should check with your county’s court for guidance on the proper procedure in your jurisdiction.  Usually a family member will submit an application for guardianship and a clinician will submit an Expert Evaluation to the court stating that the patient does not have capacity to understand or make sound judgments.  A judge decides whether or not guardianship should be established and who should act as guardian. 

Link: For information on Caregiving for a prion disease patient, see Caregiving Ideas and contact the CJD Foundation Helpline to ask questions and request a Caregiving Guide.