Category: Fall 2019

Death with Dignity

This article was not written to associate death with PD.  Rather, it was written to say that death is associated with life, and a healthy perspective is to consider how each of us would approach death.  The advance of palliative care in our nation has meant a more enlightened approach to living and dying. (1)

In May 2019 both chambers of the Maine Legislature voted to approve a measure that would allow doctors to prescribe life-ending medication to qualifying terminally ill patients who request it.  The bill, sponsored by Representative Patty Hymanson (D), (a retired physician) was aimed at passing a form of “Death with Dignity,” referred to nationally as “DWD.”  The proposal passed the Maine Senate 19-16, and the House 73-72.  Notably, all five doctors in the Legislature voted in favor of the bill, and one of the co-sponsors, Michele Meyer (D), is a registered nurse.  The text of the bill was very similar to language from Death with Dignity National Center, an Oregon-based group that assisted in writing the first law of this type in the U.S., passed in Oregon in 1997. 

Governor Janet Mills signed the Maine Death with Dignity Act (LD 1313) into law June 12, 2019, taking effect September 19, 2019.  This made Maine the 9th jurisdiction in the U.S. to allow terminally ill, capable adults the option to qualify for a lethal prescription they could self-administer in order to hasten their own death in a peaceful and dignified manner, at the time and place of their choosing. 

THE LAW

As with any law, the details require some attention.  However, the Maine Department of Health and Human Services and Maine Center for Disease Control and Prevention have tried to simplify the law with an FAQ.  According to the Death with Dignity Frequently Asked Questions (2) those who qualify for DWD are adult Maine residents suffering from an incurable and irreversible disease that would “within reasonable medical judgment, result in death within six months,” are capable of making a voluntary informed health care decision, and can self-administer the prescribed dose.  This statement implies that, as LD 1313 states, the person has the “capacity” to make informed medical decisions, is “competent,” and of “sound mind.”  This mixture of medical and legal terms poses problems for patients with cognitive or significant mood issues, and might result in additional psychiatric or neuropsychological assessment.  If either qualifying physician suspects impaired decision-making, the law requires the patient to be referred to a mental health specialist for evaluation.  Should the patient have adequate capacity, the law details a step-by-step process for a patient and doctor to follow, in which a qualifying patient may request verbally that their physician prescribe a drug that would hasten death.  The process would require “among other things, both oral and written requests, witnesses, and the second opinion of another physician.  Every step must be voluntary by both the patient and the physician.”  The “Patient Request for End-of-Life Care Medication” form may be requested from doctors or at the Department of Health and Human Services website. (2)  Alternatively, the patient may make a written request that is “significantly in the form” of the specified written request (outlined in the text of LD 1313). 

“Patients have a right to receive relevant information regarding all treatment options reasonably available for the care of the patient.” The law requires an informed decision.  The patient must be fully informed by the physician of the medical diagnosis, the prognosis, risks associated with taking the medication to be prescribed, and feasible alternatives to taking that medication, such as palliative care, comfort care, hospice care, pain control, and disease-directed treatment options.

A DOCTOR MAY CHOOSE NOT TO PARTICIPATE IN THE ACT

If unable or unwilling to carry out a qualified patient’s request, the doctor is to transfer any relevant medical records for the patient to a new physician if the patient requests.  Any physician (M.D. or D.O.) licensed to practice medicine in Maine (including prescribing controlled substances) may write the prescription for DWD medication.  The law does not extend to other prescribers such as advanced-practice registered nurses and physician assistants.  Pharmacists will participate on a voluntary basis also. As of the date of this writing, plans appear close but not finalized with pharmacies who will be involved with DWD.

LIFE INSURANCE POLICIES

The Department of Health and Human Services will collect forms provided by participating physicians, and the “Act prohibits a life insurance company from denying benefits to individuals who act in accordance with Act.” 

MAINE DEATH WITH DIGNITY

The effort to bring a DWD law to Maine has been a long time coming.  Maine Death with Dignity (formerly known in Maine as It’s My Death) is a 501(c)(3) public charity organization providing services, education, and end-of-life advocacy. (4)  Per the MaineDeathwithDignity.org website, the law is fully compatible with hospice and palliative care, and 90% of those in legal jurisdictions who choose DWD are also enrolled in and receiving hospice care at the time of their death.  If not already enrolled, people who qualify for DWD are encouraged to enroll in hospice when they bring up DWD.  Hospices provide additional medical, physical, emotional, and spiritual support to the person and their family.  Hospice also facilitates disposition of the body after death, indicating to local coroners and medical examiners that the death was expected. 

It should be noted that if a person has a functioning implantable cardioverter defibrillator (ICD), their cardiologist must contact the manufacturer prior to the chosen day of death, and request that they send a technician to turn off the defibrillator. 

The person thinking of DWD should also be aware that if they have a terminal illness, they may have a limited window of opportunity to use the DWD laws.  Because of the decline of dying persons, they may gradually or suddenly lose competence and become ineligible to use the law.  Their physician must remain able to participate as well.

I asked Valerie Lovelace, founder and executive director of Maine Death with Dignity, how she came to be such a devoted advocate for DWD.  “I originally became interested in these issues because of the death of my sister in 2009 just after she turned 49.  People were afraid to talk with her about death because she was so young.  I cannot tell you how many people said ‘you’re not supposed to die at 49.’  I don’t know where they got that rule.  But, if a death could be described as beautiful, she had a beautiful death.  Later, when I was a hospice volunteer, I began to witness some of the terrible ways people can die.  As a part of a master’s degree capstone project, I researched the law.  In 2013, at the time Vermont passed its law, Maine’s attempt, LD 1065 failed.  When I read the Maine bill, I realized it was poorly written, didn’t have safeguards, and it was actually an active euthanasia bill, not what we want.  I became the default person in Maine who understood the law.”

“I feel that when a person is dying, that person should be heard and seen, and have whatever they need at their disposal to make it a bearable process for them.  I don’t feel like that is a radical idea.”

Valerie Lovelace,
Executive Director of Maine Death with Dignity

Valerie Lovelace first founded a 501(c)(3) organization called It’s My Death in 2013 for educational purposes and to deal with issues around death and dying.  “The organization was not active politically.  We provided state-wide education when requested so people could understand the legislation.”  It’s My Death underwent a name change to Maine Death with Dignity in September 2019.  A major focus of the organization continues to be “providing fact-based information regarding death with dignity laws, or medical aid in dying, in the United States,” per the website, which goes on to state, “Our goal as Maine Death with Dignity is to continue the mission of engaging the public to increase awareness of any topic related to death and dying (including using the words ‘death’ and ‘dying’ and avoiding euphemisms like ‘passed’ or ‘passing.’).”  The role now is education, making sure providers and patients understand what the law does, and what it doesn’t do.  “It’s about allowing people to have that choice if that is what they need.”

MAINE AND DWD OVER THE YEARS

Death with Dignity has a history in Maine going back to at least 1994, with LD 1420, which like other bills prior to LD 1313, did not pass in the State Legislature.  LD 1420 sought to allow a medically assisted death as a choice for competent terminally ill adults, or for individuals in a persistent vegetative state (a condition similar to coma, and implying extensive brain damage).  In 1995 LD 748, and again in 1998 LD 916, would have allowed qualified terminally ill patients to use a legal process to request  life-ending medication to use at a time and place of their choosing (modeled on the Oregon Death with Dignity Act).  In 2000 LD 2348 was a citizen’s initiative, also modeled on Oregon’s Death with Dignity Act.  In 2013 Representative Joseph Brooks (I) sponsored LD 1065, which would have allowed patients to declare a request for end of life that included an active euthanasia option.  LD 1065 failed the House (43-95) (just a week after Vermont passed Act 39).  In 2015 LD 1270, which was modeled after the Vermont law and sponsored by Senator Roger Katz (R), was the first successful attempt at a Death with Dignity law in the legislature, passing in the House (76-70) though failing in the Senate (17-18).  In 2017 LD 347, sponsored by Senator Katz, passed the Senate by a single vote, but failed in the House (61–85).  This vote came a short time after Governor Paul LePage reported openly that he would veto the bill.  Finally, in 2019 LD 1313 was sponsored by Representative Patty Hymanson (D), a retired neurologist.  LD 1313 included a number of safeguards and enhanced Senator Katz’s previous efforts.  In 2018-2019 there was also an ongoing referendum effort, and Maine Death with Dignity Political Committee gathered 72,079 valid Maine voter signatures (over 9,000 more than required).  However, the referendum became unnecessary when LD 1313 became law.

A TIMELINE OF DWD LAWS IN THE UNITED STATES

  • Oregon (Death with Dignity Act; 1994/1997)
  • Washington (Death with Dignity Act; 2008)
  • Vermont (Patient Choice and Control at the End of Life Act; 2013)
  • California (End of Life Option Act; approved in 2015, in effect 2016)
  • Colorado (End of Life Options Act; 2016)
  • District of Columbia (D.C. Death with Dignity Act; 2016/2017)
  • Hawaii (Our Care, Our Choice Act; 2018/2019)
  • Maine (Death with Dignity Act; 2019)
  • New Jersey (Aid in Dying for the Terminally Ill Act; 2019)

Montana:  In 2009, Montana’s Supreme Court ruled nothing in the state law prohibited a physician from honoring a terminally ill, mentally competent patient’s request by prescribing medication to hasten the patient’s death.  Since the ruling, several bills for and against DWD have been introduced, none of which have passed. (3)

WASHINGTON STATE AND DWD

I thought it would be useful to discuss these issues with someone outside Maine who is involved in DWD.  A key figure is Carol Parrot, M.D., retired from anesthesiology in 2012 after 30 years of practice in Seattle.  Dr. Parrot and I spoke by phone.  She generously gave up about an hour of her time and related that her retirement is actually very busy due to her ongoing voluntary commitment to DWD in Washington.  “I work for free and see patients to qualify them for DWD.  It’s all I do.  Every patient I see is either dying, or thinks they are dying.  I spend a lot of time talking with their doctors, and obtain relevant medical records.”  She joined the Washington State Volunteer Retired Physician program, “which is wonderful.  I see patients for no charge because they qualify as underserved.  In fact, in Washington State, DWD patients are underserved because Catholic healthcare will not allow their doctors or nurses to engage with DWD.”  As of 2016, over 40% of the healthcare in Washington has been provided under one of three different Catholic systems. (5) 

I asked Dr. Parrot how she became involved with DWD.  “Before I retired, my two best friends and my stepfather died of cancer.  My stepfather was one of the most brilliant people I have ever known.  He worked for IBM, and in the end couldn’t make his computer work.  He begged me to help him, and I couldn’t.”  Her friends both died very difficult deaths from breast cancer, and she did not have the knowledge to qualify them for DWD before each lapsed into a coma.  These were critical events in her life.  “When I finally retired, I wanted to do something that nourished my soul, and this does.  And, these are the most appreciative patients.  By the time they find me (the vast majority of my patients are referred to me by End of Life WA, a non-profit agency in Washington that helps patients seeking help with end of life issues), most have been looking for a while.  Or, they are dying fast, and they are frantic.  I usually handle about four or five patients in a month, but I have had eight in a week.  There are some days I spend 12 hours on this.  My husband tells me I don’t have the concept of retirement down.”

Dr. Parrot tells me she has kept an eye on DWD laws in this nation, and has advice for doctors in Maine.  Implementing DWD “starts out slow in every state, and there is no reason everyone needs to reinvent the wheel.  We have a wheel that is getting streamlined.”

“Medical Aid in Dying is actually a new branch of medicine that is evolving.  In medical school we were never taught about how to really talk to patients about death, and certainly not taught about offering patients a choice to hasten death.”

Carol Parrot, M.D.

In terms of the experience of a DWD, Dr. Parrot can speak from what she has learned in her practice, and from data collected in her state.  She notes there are a few different combinations of drugs used to hasten death (which she helped to develop).  A person who has ingested one of the drug combinations tends to fall asleep within 5-10 minutes, and 75% of deaths occur anywhere from 15 minutes to 3 hours.  The longest death recorded with a commonly used combination of meds was 39 hours (a significant outlier, see reasons below).  Two more recently offered drug combinations are noted to have had longest time intervals of 12 and 16 hours.  Dr. Parrot notes factors that might prolong that time (and thus should be taken into account by DWD doctors), include slow gastric emptying, poor absorption of drugs from the GI tract, or ability to take larger doses of drugs (high tolerance).  In fact, doctors give special or “red flag” consideration to people with cancer exposed to high doses of narcotics (by mouth, patch, or IV pumps), those with uncontrolled pain, IV drug abusers, people who have a high tolerance to alcohol, people with biliary (the bile system) or pancreatic cancer, and people who weigh over 300lbs.  People who have had excessive weight loss at the end of their life might absorb the drugs more slowly. 

Dr. Parrot also relates that people who are thinking of the DWD option need to first consider the following: they must make sure they have a terminal illness which is documented by a physician, “in the chart that this person will probably die within six months.”  It is not always easy for doctors to make that determination.  And, many doctors are reluctant for stated employment, moral, philosophical, or religious reasons to assist with DWD.  As above, physicians and pharmacists are not required to participate in DWD, and many are not as familiar with the laws as the people who might want to take this option.  Dr. Parrot advises that these people or their families ask doctors, their hospice personnel, or search the internet to find information about where to get help.  “Patients get discouraged and become anxious when their doctors refuse or delay granting them this option and they have to find new doctors.” (7)

The 2018 Death with Dignity Act Report in Washington (7) indicates a total of 267 individuals ranging in age from 28 to 98 years were dispensed the medication in 2018 (the highest number since the law took effect in 2009).  The report notes 158 different physicians prescribed, and 61 different pharmacists dispensed the medication.  Of those receiving a DWD prescription, 96% were white, 44% were married, 70% had at least some college education, 75% had cancer, 10% had a neuro-degenerative disease (including amyotrophic lateral sclerosis, or ALS), 15% had other illnesses (including heart and respiratory disease, and unknown illnesses), and 94% had private, Medicare, Medicaid, other insurance, or a combination of health insurance.  Participants shared the following concerns with their health care providers: loss of autonomy (85%), loss of the ability to participate in activities that make life enjoyable (84%), loss of dignity (69%).

IN CLOSING

DWD is personal, and may be a difficult decision.  If one qualifies and elects to proceed, it is ideal to have the support of others who are close.  Most patients who elect to use the DWD option choose to die at home and surrounded by loved ones.  But, the end of suffering does not come about as rapidly as simply making a big decision.  There are safeguards built into the law that prevent rash, or sudden use of DWD.  After making an oral request, a person must wait at least 15 days to sign the written request.  Then, at least 48 more hours must pass between the time of the written request and the writing of the prescription by the doctor.  This is obviously something that must be well thought-out and discussed in advance with all concerned.  That may be hard.

We all hope not to face the chaos of a painful or disabling terminal illness.  Whatever we may want, we do not often get to choose our illnesses, and the unfamiliar country of a life-taking disease can be difficult to travel.  Conversations around these issues, especially choosing to end life, can be some of the hardest that people have with healthcare workers and loved ones.  I say this based on my experience as a human being, a member of a family, and as a doctor.  To focus on the last perspective, in the nearly two decades since I left medical school I have had thousands of patient encounters among the office, hospital rooms, the intensive care unit, and the emergency room.  People have sometimes asked for help that I could not give, and related suffering in ways not limited to the physical: anguish, fear, feelings of futility, frustration, and sometimes hopelessness.  Now the law and the conversation have changed, and recently I heard from a patient with a chronic and progressive disease that just knowing there is an alternative gives a little peace.  

Relatively speaking, so few people will need DWD, but it might become an important option for any of us.  Whether or not you think DWD would be right for you one day, I advise all adults with a chronic illness to consider a palliative care consultation. (1)  This is a way to plan out what the rest of your life might look like, to have control, and to have contact with people familiar with hospice and other end of life options -including DWD, should you need them. 

Author’s note: I would like to thank Valerie Lovelace and Dr. Carol Parrot for their help in understanding the issues surrounding LD 1313, in connecting me with others integral to DWD, and for their generous gifts of time and insight.  I thank Grace Plummer, LCSW, and Liz Stamey, RN, LMT for reviewing, discussing, and technically editing the article.  Finally, I thank the reader, and hope you will accept this article in the spirit in which it is offered. 

REFERENCES

1.         Palliative Care Essential to Management of Chronic Neurological Disease, Jessica Vickerson, NP, Maine PD News.  Fall 2017   https://mainepdnews.org/2017/10/04/palliative-care-essential-to-management-of-chronic-neurological-disease/

2.         http://www.maine.gov/dhhs/mecdc/public-health-systems/data-research/vital-records/forms/index.shtml  

3.         https://www.mainedeathwithdignity.org/

4.         https://www.deathwithdignity.org/learn/death-with-dignity-acts/  

5.         https://crosscut.com/2014/01/washingtons-looming-catholic-hospital-takeover  

6.         Physician Assisted Dying in Washington State: A primer for participating physicians. Carol Parrot, M.D., Robert Wood, M.D. (10/20/19)

7. https://www.doh.wa.gov/YouandYourFamily/IllnessandDisease/DeathwithDignityAct/DeathwithDignityData      

Online references verified 11/29/19.

Gastroparesis in PD

Periodically I will hear from a patient about excess bloating.  They are not alone.  The gastrointestinal (GI) system is involved to some degree in over 90% of patients with Parkinson disease (PD), which has been discussed here in MPDN.  (1-3)   GI issues may affect drug absorption, PD motor symptoms, and quality of life.   Before we get started with the problems, let’s take a moment for the sake of terminology, to know that doctors tend to divide the GI system as follows: upper GI: mouth to stomach, and lower GI: small intestine to rectum.  The GI tract is controlled to some degree by nerve centers in the brainstem dorsal motor nucleus (DMN) via the vagus nerve “the wanderer.”  There is also an enteric nervous system (ENS), which some refer to as “the second brain,” consisting of about 100 million neurons.  If that sounds like a lot, consider a healthy adult brain, which is estimated to be made up of 80-100 billion neurons (and many, many more glial cells).

Why are there GI issues in PD? 

That is a big topic, too big to cover in depth here.  The issues are likely multifactorial.  Alpha-synuclein aggregation in neurons of the gut and the DMN, and issues with the gut-brain axis are big factors. For more on those issues, read articles 1 and 4 in the references below.

Let’s talk about the stomach

The stomach is a single  chamber, which is functionally considered in three regions: the proximal stomach, the distal stomach, and the pylorus.  These regions operate in unison to maintain normal gastric emptying.  The stomach must also maintain intragastric pressure in order to allow continued meal ingestion until satiation.  In other words, the stomach contains the food you have eaten so that you can eat until you feel appropriately satisfied.   There are many variations on that theme however, which may in part explain some under-, and overweight individuals. Whatever the case, in normal circumstances after a meal, strong contractile pulses in the distal stomach break up food particles.   This allows the stomach to empty into the first part of the small intestine.  But emptying of the stomach may not occur on time.

Delayed gastric emptying affects 70% of PD patients at some point.  If the problem becomes chronic and symptomatic doctors refer to it as “gastroparesis.”  The symptoms of gastroparesis include  nausea, vomiting, retching, early satiety, bloating, loss of appetite, and abdominal discomfort.   Longer PD duration is not associated with worse gastroparesis as you might expect.  However, advancing motor severity (the stiffness, slowness, and tremors of PD) does correlate with worse gastric emptying delay.  This is largely to do with where L-dopa (levodopa) is absorbed: the small intestine.  Obviously, if the drug cannot get there, it cannot be absorbed.  Gastric emptying speed is therefore the rate-limiting step in the absorption of L-dopa.  Because of these drug absorption problems, gastroparesis contributes to drug response fluctuations.  This means some doses of L-dopa might be delayed in working, work only partially, abruptly stop working at an unpredictable time, or not work at all.  (5,6)

Is there a treatment for gastroparesis in PD?

Some doctors attempt to use the dopamine receptor antagonist metoclopramide  (Reglan).  This drug does help empty the stomach, but has the unfortunate function of blocking dopamine receptors in the brain.  This not only prevents dopamine from doing its job, but can cause severe PD symptoms.  While metoclopramide is the mainstay of treatment for gastroparesis in the general population, there is the potential for so-called extrapyramidal side effects such as “parkinsonism.”  In people with PD or parkinsonism, metoclopramide is not suitable for long-term use.   I tend to use this drug very rarely in my practice.

Domperidone is not FDA-approved for any indication in the United States, but is in trials (7), and is used by U.S. movement disorder and gastroenterology doctors nonetheless (usually via compounding pharmacies).  In the U.S., compounding domperidone is an expensive proposition.  Because the drug is available in Canada (and many other countries), it can be purchased much less expensively over the internet (usually from Canada).  However you get it, Medicare and most insurance companies will not pay for it due to the above lack of an indication per the FDA.  In terms of how it works, and whether it might affect parkinsonism, domperidone does not cross the blood brain barrier, has been shown to enhance gastric emptying, and to enhance L-dopa bioavailability (allowing L-dopa to function). It is the drug of choice for delayed gastric emptying in PD in essentially the rest of the world.

Macrolide antibiotics (such as erythromycin) are sometimes used for short periods of time (antimicrobial properties, i.e., action against bacteria, limit its long-term use).  These drugs enhance gastric emptying and are effective in gastroparesis.

It should be noted that a small number of patients with gastric complaints will be colonized with the bacteria Helicobactor pylori.  This microbe is also known to be a culprit in gastric ulcer formation.  Among PD patients evidence of H. pylori colonization is associated with motor fluctuations.  And, after H. pylori eradication, L-dopa absorption and motor fluctuations can be improved.  (8,9)

If you think you might have delayed gastric emptying or gastroparesis, talk with your doctor.  Severe symptoms might require a gastroenterology consultation.  Very mild symptoms might be due to some other factor, or respond to minor medication changes.  

References

  1. What is the Gut-Brain Axis? Maine PD News, April 2017.  https://mainepdnews.org/2017/04/17/what-is-the-gut-brain-axis/
  2. Constipation in PD.  Maine PD News, June 2016.  https://mainepdnews.org/2016/06/12/constipation-in-pd/
  3. PD and diet. Maine PD News, January 2017.  https://mainepdnews.org/2017/01/11/pd-and-diet/
  4. What’s so bad about alpha-synuclein? Maine PD News. April 2016.  https://mainepdnews.org/2016/04/25/whats-so-bad-about-alpha-synuclein/
  5. Doi H, Sakakibara R, Sato M, et al. Plasma levodopa peak delay and impaired gastric emptying in Parkinson’s disease. J Neurol Sci 2012;319:86-88.
  6. Muller T, Erdmann C, Bremen D, et al. Impact of gastric emptying on levodopa pharmacokinetics in Parkinson disease patients. Clin Neuropharmacol 2006;29:61-67.
  7. Pipeline drugs, part 2: gastric emptying agents.  Maine PD News. June 2018. https://mainepdnews.org/2018/06/21/pipeline-drugs-part-2-gastric-emptying-agents/
  8. Lee WY, Yoon WT, Shin HY, Jeon SH, Rhee P-L. Helicobacter pylori infection and motor fluctuations in patients with Parkinson’s disease. Mov Disord 2008;23:1696-1700.
  9. Pierantozzi M, Pietroiusti A, Brusa L, et al. Helicobacter pylori eradication and l-dopa absorption in patients with PD and motor fluctuations. Neurology 2006;66:1824-1829.