Category: History

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.

Levodopa at 50

Levodopa, active ingredient of Sinemet, is the most effective drug we have for PD.  The discovery of levodopa as a drug for use against parkinsonism in the late 1960s transformed life for people suffering with the disease.  Prior to that time, the eventuality for many was to experience progressive stiffness and slowness until they became wheelchair- or bed-bound.  I have heard of Parkinson wards in hospitals, where sufferers lingered until they died a premature death related to the complications of immobility.  Levodopa liberated people with PD and brought lifespans to that of the general population.   As happy as the patients were with this potent new drug, the exciting discoveries with levodopa also attracted bright minds to neurology and neuroscience, which led to a better understanding of the brain’s basal ganglia, which is affected in PD, and to related problems, such as dystonia, chorea, and tics.

The historical perspective

No history of levodopa can be complete.  There were many contributors in science and medicine likely not given credit.  I do not review the very interesting story in Oliver Sacks’ book Awakenings, though I recommend that related story regarding his work with parkinsonsim and levodopa.  And, here I do not delve into Eastern medicine or the use of medicinal plants such as Mucuna pruriens, which was discussed in the article “PD and diet” (MPDN winter 2016/2017). Instead I will give a brief history of scientific discovery.  What follows is a timeline as I understand it.  It is also a window into how some of the most complex discoveries in science are made.  It is a story of collaboration of sorts, the sharing of information and sometimes the reconsideration of some fact through the lens of another mind.  It is amazing to me that so many labs from around the world were involved over such a long time.  There is probably an earlier point of origin, but for the sake of starting, I will begin here with a timeline.

In 1895, visible lesions of the upper brainstem (a stalk-like structure connecting the brain and spinal cord) were described at autopsy of PD sufferers by Brissaud, who was a professor of pathology in Paris.  Brissaud proposed these lesions were significant, but could not define precisely how.  At the time, function of this structure was not known.  Over the years, dopamine would be detected in the brain but it took time to determine the origin or function.  Thus, it was an unconnected discovery when dopamine was synthesized in London by George Barger and James Ewens in 1910.  At the same lab, Henry Dale discovered dopamine was chemically similar to epinephrine.  There were limitations in biochemistry, and not until three decades later did Peter Holtz of Germany discover the enzyme which converts levodopa to dopamine (aromatic-L-amino-acid decarboxylase, a.k.a. dopa decarboxylase).  This discovery would become very important because it gave researchers a mechanism to form dopamine in the brain, though at the time the meaning of this discovery, that it would help people with PD, was not yet understood.  You and I can stand in the future and easily see why this discovery was important.

Dopamine, if given by mouth, cannot get from the bloodstream to the brain because it cannot cross the protective blood brain barrier, which shields the brain from many, but not all compounds in the blood. 

On the contrary, levodopa is not blocked, and can cross the blood-brain barrier, after which it will be converted by this enzyme to dopamine.  That is the basis of treatment with levodopa.  However, that would take years to grasp.  In fact, around that time Herman Blaschko in Cambridge hypothesized that levodopa and dopamine could be converted to epinephrine and norepinephrine (adrenaline), which was later proven, but again, missed the significance of dopamine in PD.  In the next several years dopamine was found in other bodily organs such as the adrenal glands, the heart, and the kidneys, all interesting discoveries apart from the immediate significance in PD.

Redirection back to PD came in 1953 when Drs. Greenfield and Bosanquet of Queen’s Square, University of Oxford, reported the loss of pigment cells of the brainstem substantia nigra (which produce dopamine).  In 1956, Oleh Hornykiewicz began working in Blaschko’s lab to clarify whether blood pressure was directly affected by dopamine, versus some breakdown product.  He proved dopamine lowered blood pressure and that levodopa had a similar effect.  In the 1950s and 60s American cardiac researcher Bernard Brodie showed that the drug reserpine lowered serotonin levels, which he proposed was responsible for controlling blood pressure and the heart rate.  His postdoctoral fellow, Arvid Carlsson, was given the task of investigating how the drug reserpine does this.  Carlsson argued that it was not serotonin, but a different related molecule responsible for lowering blood pressure.  After returning to Sweden and starting his own research own laboratory he showed that an important side effect of reserpine could be reversed by levodopa and not serotonin.  Reserpine depleted dopamine in the lab rabbits’ brains, resulting in a drug-induced parkinsonian syndrome, and in high enough doses, total unresponsiveness.  Even in that state, when given levodopa, the rabbits’ ears would pop up and they would become alert.  Kathleen Montagu, in London at around the same time, was the first to prove that dopamine was present in the brain.  Carlsson developed a new technique to measure dopamine in tissue, and showed soon after that dopamine was present in the brain, but depleted with reserpine and restored with L-dopa.  Two medical students in Carlsson’s lab, Ike Bertler and Evald Rosengren, mapped the distribution of dopamine in the dog’s brain, and showed concentrations in the basal ganglia.  This work was repeated in humans by one of Carlsson’s collaborators, Isamu Sano in Japan.

These findings led Carlsson to speculate, at the 1959 International Pharmacology meeting, that Parkinson disease was related to dopamine.

Following this, in Vienna, Oleh Hornykiewicz began to measure dopamine both in people with Parkinson’s and with post-encephalitic parkinsonism, and in 1960 published a paper showing a marked depletion of dopamine in the basal ganglia (specifically the caudate and putamen) of patients with both of these conditions, but not in people with other brain disorders such as Huntington chorea.

How dopamine was formed in the brain was not known until the early 1960s, when Toshiharu Nagatsu, a postdoctoral fellow at the National Institutes of Health, discovered the enzyme tyrosine hydroxylase, which converts the amino acid tyrosine to levodopa.  Thus, it was known that proteins break down to release amino acids such as tyrosine, which is converted to levodopa by tyrosine hydroxylase.  Levodopa is then converted to dopamine.

In 1966, Oleh Hornykiewicz proposed that dopamine deficiency in the striatum of the basal ganglia is correlated with most of the motor symptoms of PD. 

Over the next several years, using the new technique of histofluorescence, Swedish researchers Annica Dahlström, Kjell Fuxe, and Nils-Eric Andén mapped dopamine pathways in the brain and discovered the nigrostriatal pathway. Meanwhile, Ted Sourkes and Louis Poirier in Montreal demonstrated that, in animals, striatal dopamine levels fall when the nigra is injured.  This connected the work of the two groups.

Dopamine as a treatment

In 1960, Oleh Hornykiewicz had begun to consider levodopa as a possible treatment for PD.  He and Walther Birkmayer, a Viennese neurologist, found that intravenous injections of levodopa produced dramatic, though short-lived benefits in PD patients.  Investigators around the globe demonstrated sometimes positive and sometimes negative results with higher doses of levodopa.  For example, Pat McGeer in Vancouver failed to benefit patients with 5 gram doses of the drug D, L-dopa.  A major limiting factor was nausea (and perhaps medications used to prevent nausea, which we now know can block dopamine in the brain and cause parkinsonism).

In 1967, Greek researcher George Cotzias showed in a U.S. trial that if one starts with a low dose of levodopa and gradually increases, up to 16 grams daily, benefit can be found without nausea and vomiting. This was verified in several double-blind studies and was the birth of modern treatment.

Nausea was a factor because of the dopamine decarboxylase enzyme, which converts levodopa to dopamine in the body.  As above, dopamine cannot cross the blood-brain barrier, and causes nausea.  However, the addition of carbidopa to levodopa would increase the strength of levodopa about four-fold and allow most of the drug to reach the brain.  This is because carbidopa blocks dopa decarboxylase.  Thus, the drug Sinemet, a Latin derivation meaning “without vomit,” was made.  Over time, researchers would show that lower doses were effective and perhaps less associated with complications, another complex history, for another time.

Different forms of levodopa

There have been multiple variations on carbidopa/levodopa, such as immediate release, controlled release, and the orally dissolving Parcopa formulation.  We have also seen the addition of entacapone (a COMT enzyme inhibitor) in the drug Stalevo.  In 2015, the FDA approved Rytary (a combination of long and short-acting carbidopa/levodopa) in a capsule of pellets, which are absorbed at different rates in the GI tract and allow a wider interval between doses in advanced disease than one would expect with the immediate or controlled release forms alone.  The Duodopa dopamine pump was approved in Europe over a decade ago, and the Duopa dopamine pump was FDA approved in 2016.  Duopa is a gel with a concentration of 20 mg levodopa per 5 mL infused continuously to the GI tract over the course of 16 hours per day.   It requires a PEG-J tube (a tube from the small bowel sticking out of the body just below the rib cage) which is attached to the pump.  It is typically used in patients who would not qualify for deep brain stimulation but suffer from uncontrolled motor fluctuations.

The levodopa drug development pipeline

The accordion pill, a novel gastro-retentive delivery system, is being developed by Intec Pharma. The pill expands like an accordion in the gut to keep it there while it slowly releases medication.  The accordion pill has recently completed a phase II trial of 60 PD patients with doses of 250-500 mg levodopa over a 7-21 day period, given once or twice daily, and per the company’s website (unpublished data) there was a reduction in OFF time and dyskinesias.  The baseline characteristics of the patients, including disease severity, were not apparent on the website.  The drug will need to complete a phase III trial before FDA approval is sought.

Subcutaneous levodopa (a patch/pump system, clinical trials identifier ND0612), by Neuroderm, is a subcutaneous delivery system of up to 360 mg levodopa over a 24 hour continuous infusion of a patch/pump (a patch with a small needle placed under the skin).  It is designed for moderate to severe PD as an alternative to the Duopa pump or deep brain stimulation.  Two phase II studies have shown the drug is safe and tolerable.  I understand the phase III studies will begin soon.  At this point, Boston will probably be the closest option.

Inhaled levodopa, CVT-301, is a self-administered, inhaled form of levodopa by Acorda Pharmaceuticals which has recently completed a phase III trial with a new drug application submission.  The idea with inhaled levodopa is to have rapidly effective levels of the drug in the blood, and subsequently, the brain.   Thus, the drug would be ideal for rescue from unpredictable off times, such as at a restaurant or a movie.

More delivery systems are being investigated.  Levodopa it seems, is going strong at 50.

 

200th Anniversary of An Essay on the Shaking Palsy

In 1817 James Parkinson, a member of the Royal College of Surgeons in London, published his famous paper, An Essay on the Shaking Palsy (1).  In this document he described “shaking palsy (paralysis agitans).”*  This term was used in reference to a partial description given by the Greek physician Galen in the second century A.D.**  Parkinson’s general description for recognizable cases was that of “involuntary tremulous motion, with lessened muscular power, in parts not in action and even when supported: with a propensity to bend the trunk forwards, and to pass from a walking to running pace…”  This is, of course, a state of advanced disease with tremors, rigidity, subjective weakness, flexion of posture, and festination of gait.   However, he was a student of history, a physician, scientist, and paleontologist.  He knew the value of taking a detailed account of the natural history of disease, and meant not just to describe obvious or striking examples, but also to give physicians and science at large a formal understanding of the genesis of paralysis agitans.  He took care to make others understand the indolent and secretive inception of this condition, which he explained is difficult to establish.

“So slight and nearly imperceptible are the first inroads of this malady, and so cextremely slow its progress, that it rarely happens, that the patient can form any recollection of the precise period of its commencement.”

He described history taken from his own patients, and in two cases, histories taken from sufferers he met on the street.  The patients discussed as case examples included a 50 year-old gardener, a 62 year-old prior attendant at a magistrate’s office who, after eight years had become disabled and was the “inmate of a poor house,” a 65 year-old ex-convict of a Spanish prison, a 55 year-old man whose onset had been during an acute inflammation five years earlier, and a 72 year-old man who’d lived a life of temperance.  He finally described a man he had simply observed walking in a public street.  With these histories and observations, Parkinson carefully described the relentless forward march of disease in a detailed fashion reflective of his classical education.  However, there was also embedded in his lengthy paper the soul of the patient, and the agony of his era, when no western medicines were known to be of benefit.  Parkinson gave voice to his patient “harassed by this tormenting round,” when trying to simply eat with utensils.  He described a sense of loss and the intensity of effort devoted to living with this disease.

“The submission of the limbs to the directions of the will can hardly ever be obtained in the performance of the most ordinary offices of life…Walking becomes a task which cannot be performed without considerable attention.  The legs are not raised to that height, or with that promptitude which the will directs, so that the utmost care is necessary to prevent frequent falls.”

Parkinson discussed multiple motor and later non-motor issues characteristic of the disease which are commonly reported today.  He reviewed key signs of disease at length and proposed a mechanism for paralysis agitans which correctly identified the brainstem as a focus of spread.

“A diseased state of the medulla spinalis, in that part which is contained in the canal, formed by the superior cervical vertebrae, and extending, as the disease proceeds, to the medulla oblongata.”

This work called attention to a disease which had not been described or categorized in western medicine and opened the door to 200 years of progress in the understanding, treatment, and hopefully prevention and cure.  In the days of James Parkinson there were no medications in Europe known to improve symptoms.  His patients were doomed to a progressive disability and no social safety net.  Those without family able to care for them presumably died in the poor house, the prison, or on the street.   Even in the United States, as late as the pre-levodopa era of the 1960s, some hospitals contained Parkinson’s wards in which patients who had lost the ability to walk passed their days in bed, and once completely deconditioned, often succumbed to aspiration pneumonia or deadly blood clots caused by immobility.  The dawn of levodopa treatments ended those wards.  Life expectancy of most PD patients became that of the general population, a huge victory which cannot be understated.  Though the disease still has the potential to be devastating, we now live in a time in which we understand much of the genetic and molecular mechanisms of this incredibly complicated syndrome affecting so many systems of the human mind and body.  Medications have advanced and deep brain stimulation is in common use.  Ongoing trials of vaccines, monoclonal antibodies, stem cells, and other interventions give a great deal of hope.  I thank James Parkinson and appreciate his discipline and deeply scientific approach, as well as his profound empathy for those who suffer under the disease of his namesake.

 

FOOTNOTES

* It was not Parkinson, but the French physician Charcot who would in 1850 apply Parkinson’s name as eponym.

**Ancient Ayurvedic texts refer to the syndrome of PD as Kampavata, though it appears this was unknown in western literature at the time of An Essay on the Shaking Palsy.

 

REFERENCE

  1. Sherwood, Neely, and Jones (London, 1817).

http://neuro.psychiatryonline.org/doi/abs/10.1176/jnp.14.2.223?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub%3Dpubmed

 

 

 

 

Camp Lejeune

In January the Public Health website of the U.S. Veterans Affairs (1) reported that “From the 1950s through the 1980s, people living or working at the U.S. Marine Corps Base Camp Lejeune, North Carolina, were potentially exposed to drinking water contaminated with industrial solvents, benzene, and other chemicals.”  The VA has established a presumptive service connection for disability claims of Veterans, Reservists, and National Guard members exposed to contaminants in the water supply at Camp Lejeune from August 1, 1953 through December 31, 1987, if they later developed PD or one of seven malignancies.  The new rule will be in effect March 14, 2017, and is funded by an allocation from the final days of the Obama administration.

This is part of an ongoing story.  Contamination was tragically not detected for decades.  Since the base opened in 1942, up to 30 supply wells were used on the 236 square mile base.  Water from the supply wells was mixed at treatment plants before being used by residents of the base.  Reportedly, tests of sampled Camp Lejeune water from 1980-1985 detected toxic solvents.  Contamination points were identified as a landfill on the base used for chemical dumping, leaks from underground storage tanks, and “waste disposal practices” (2).  The main contaminant in the system was trichloroethylene (TCE) with a maximum detected level of 1,400 µg/L, followed by tetrachloroethylene (PCE) at 100 µg/L, benzene, and breakdown products of TCE: trans-1,2-dichloroethylene (DCE) and vinyl chloride.  U.S. maximum contaminant levels (MCL) for TCE, PCE, and benzene are 5 µg/L. TCE, vinyl chloride and benzene are all classified as a human carcinogens (3,4,5).  PCE is a “likely” or “probable” human carcinogen.  TCE exposure has been identified by multiple authors as a risk factor for PD (6).

The Environmental Protection Agency (EPA) placed the site on the Superfund program’s National Priorities List (NPL) in 1989 because of contaminated groundwater, sediment, soil and surface water.  Next, the EPA, the North Carolina Department of Environment and Natural Resources (NCDENR), and the US Navy investigated the site and “took steps” to clean the base.  For some time since, the wells have been closed.  The problem is not over, and is not small.  Multiple sources cite a Department of Veterans Affairs estimate that up to 900,000 service members might have been exposed to the contaminated water.  It is not clear what number of family members or civilian workers were exposed.  And, diseases such as those reported may be many years in the making before one is even aware they are sick.  However, following discovery of clusters of cancers and other illnesses among those who lived, served, and worked on the base, increasing calls came for investigation.  Grassroots efforts to acknowledge the problem began, multiple legal actions were taken, and political attention was given.  In spite of these efforts, the issue was not as widely known until 2008 when Congress ordered the VA to notify exposed veterans of the problem.  There were, of course, calls to treat those with conditions related to these exposures.

In 2012 the Camp Lejeune Health Care Bill, signed into law by President Obama, covered 15 conditions related to contaminated water exposure, most of them cancers.  The law states “VA provides cost-free health care for certain conditions to Veterans who served at least 30 days of active duty at Camp Lejeune between January 1, 1957 and December 31, 1987.”  Eligible veterans were allowed to enroll in VA health care and receive medical services for covered health conditions at no cost.  Families of vets who lived on the base were included.

This 2017 decision taking effect 3/14/17 is an amendment to the 2012 law and has come after intense scrutiny by several government agencies.

Data collected on those who lived or worked at Camp Lejeune by the Agency for Toxic Substances and Disease Registry (ATSDR) (7) noted that civilian workers would likely have had the highest exposure to toxins in the the water.  A study compared 4,647 Camp Lejeune workers with a similar number from the same time period at Camp Pendleton in California.  These full time civilian employees started working at either base between April 1973 and December 1985.  Researchers carefully screened workers and asked about water consumption and bathing (some showered on the base).  In order to give enough time to detect the slow development of some diseases, they evaluated the health these large cohorts from 1979-2008, noting among multiple other serious health issues, higher rates of PD among the Camp Lejeune civilian workers with higher cumulative exposures to the contaminants.

Federal Register (8) published an online article in January stating this final rule establishes a presumption that Parkinson’s disease, which had originally not been included in the Camp Lejeune Act, is a condition for which there is “strong evidence of a causal relationship and evidence that the condition may be caused by exposure to the contaminants.”  Parkinson’s disease was included as a presumptive cause of disability due to a recommendation made by the Institute of Medicine (IOM), part of the National Academies of Science, in a 2015 report (9).

In Maine there are over 127,000 veterans (10).  At the time of writing this article, I have been unable to determine by online sources or by inquiry to the Department of Veterans Affairs the number of Mainers who served, lived, or worked at Camp Lejeune.

 

REFERENCES

1. http://www.publichealth.va.gov/exposures/camp-lejeune/
2. Bove, et al., Mortality study of civilian employees exposed to contaminated drinking water at USMC Base Camp Lejeune: a retrospective cohort study. 2014;13:68.
3. Environmental Protection Agency (EPA): Final health assessment for TCE. 2011.
4. Guha, et al., Carcinogenicity of trichloroethylene, tetrachloroethylene, some other chlorinated solvents, and their metabolites. Lancet Oncol 2012, 13:1192–1193.
5. Chiu, et al., Human health effects of trichloroethylene: key findings and scientific issues. Environ Health Perspect 2013, 121:303–311.
6. Locke, et al., Solvents and Parkinson disease: a systematic review of toxicological and epidemiological evidence.Toxicol Appl Pharmacol. 2013 Feb 1;266(3):345-55.
7. https://www.atsdr.cdc.gov/sites/lejeune/qa_healthstudyactivities.html
8. https://www.federalregister.gov/documents/2017/01/13/2017-00499/diseases-associated-with-exposure-to-contaminants-in-the-water-supply-at-camp-lejeune
9. https://www.nap.edu/catalog/18991/review-of-va-clinical-guidance-for-the-health-conditions-identified-by-the-camp-lejeune-legislation
10. http://www.maine.gov/veterans/about/media-kit/demographics.html

What is deep brain stimulation?

Your brain is the most complicated machine known, a mass of billions of interconnected  neurons, each of which may form an average of about 7,000 synapses (connections with other neurons), forming your personal and highly unique connectome – the map of all of those connections.  At each of these synapses, electrochemical signaling fires in patterns that may be individual or in networks, which generate a signal strong enough to detect with electrodes on the scalp.  This is what allows us to study brain waves on an electroencephalogram (EEG).  Brain electrical activity can also be changed in a way that helps patients with neurologic diseases, and is the basis of deep brain stimulation (DBS).

However, the story of how we got here goes back farther than most would guess. For example, in the first century A.D. the Roman physician Scribonius Largo described using the electric ray fish, torpedo, to shock the heads of headache sufferers (1).    Since the common ray can deliver a charge of up to 200 volts to immobilize prey, one can imagine the effect on a person might be less than pleasant.  Electric fishes were actually used in European medicine at least into the eighteenth century.  There were many other experimental uses of electricity, on living and expired subjects, in centuries past.  Thus it was not so unusual that in the early twentieth century electroconvulsive therapy was established in psychiatric medicine.

Work with electricity also led to a greater understanding of how our brains operate. In the 1930s, Canadian neurosurgeon Wilder Penfield famously mapped functional regions of the brains of awake patients with electrical stimulation during surgery (2).   He was not alone in using electricity to study or modulate the brain.  These early pioneers led the way to the formation of DBS.

DBS as we know it today begins in the early 1950s, when Spanish neuroscientist Jose Delgado reported implantation of electrodes in humans (3).   A decade later he described  radio-equipped electrodes which he had placed in various animals, and in 25 people.  In 1963, he demonstrated he could stop a bull from charging (or at least veer off-course) with the device. Over the following decades, investigators around the world experimented with brain electrodes.  Two groups in 1991 ushered in modern technology when they successfully demonstrated that DBS could treat tremor (4,5).

DBS was FDA-approved in 1997 for tremor associated with essential tremor and Parkinson disease (6). In 2002, the indications were expanded to include other symptoms of Parkinson disease.   Previously, patients may have undergone surgical procedures such as thalamotomy or pallidotomy, which affected or diminished motor symptoms by surgically destroying a specific brain region. After DBS was approved, it became the preferred, or much more commonly administered, treatment.  There are several reasons for this, such as the facts that DBS is not intended to destroy brain tissue, is adjustable and programmable, and is at least as equally effective as the older surgical procedures. DBS is also reversible.  It may be turned off and sometimes will be removed, though this is uncommon.  It has been implanted a great deal since 1997.  According to the International Neuromodulation website, between 1997 and 2012 there were over 80,000 DBS implants around the world for a variety of indications (7).

Who should have DBS?

In Parkinson disease, the procedure is usually intended to treat disabling motor complications, tremor, and dystonia refractory to medical treatment.  For example, patients may want DBS when dyskinesias become intolerable, or when medications fail to work in a predictable way.  Some patients complain of sudden, unpredictable off time when medications should be working.  DBS may help, as it is a continuous delivery system instead of the pulsatile approach of medication dosing.  There are also PD patients whose tremors respond inadequately, or not at all, to medications.   Typically, if other symptoms of PD such as stiffness and slowness respond to medication, then DBS may be a good option for tremor control in these patients.  DBS does not cure PD, but helps to diminish some of the motor symptoms.  Because of this, patients are usually able to cut back on medications, and thus, medication side effects.

And, who should not have DBS?

Medtronic, the manufacturer of the first, and still most widely used DBS platform, offers the following exclusion criteria:   no longer responsive to medications; severely disabled, even in the best “on” state; medical conditions that prevent surgery; and onset of dementia. It may be hard to define exactly when a person has reached each of these states, and this is where having a team-based approach to assessing the patient can be helpful.  In Maine, a team composed of a neurosurgeon, movement disorder neurologists, neuropsychologists, and specialized nurses meet to discuss potential cases and outcomes.

What are the risks? 

Risks of surgery can be serious and fortunately occur in only a small minority of patients. These can include stroke, paralysis, coma, death, bleeding in the brain, cerebrospinal fluid leak, seizure, infection, allergic reaction to implanted material, confusion, pain at surgery sites, and headache.   These risks should not be taken lightly and should be discussed at length with your doctor prior to making a decision to proceed.  Since no two patients are the same, there may be other risks unique to that person, and sometimes the risks are too great.

Programming of DBS is meant to stop motor symptoms of PD, but may also cause unwanted side effects, such as tingling, temporary worsening of symptoms, speech problems (for example, slurred speech or trouble producing words), double vision, dizziness, weakness of the face or limbs, temporary worsening of dyskinesia, coordination problems, feelings of shocks or jolts, numbness, and very rarely, behavioral disturbance.  Usually, programming changes can eliminate these issues.  In the rare circumstance where relief from side effects cannot be found, DBS can be turned off.

Who has had the procedure in Maine?

Lots of people.  Before 2013, patients were implanted in other states.  Since 2013, dozens of implants have been done in Maine with Dr. Rughani.  I asked a couple of my patients about their experience as I was writing this article.

Barbara Keezer of Augusta, Maine, told me that she had suffered with PD for over 14 years when, in 2005, the development of uncontrolled dyskinesias led to deep brain stimulation with Dr. Van Horne in Boston.  She states she “could tell a difference right away, dyskinesias stopped.  I couldn’t walk before the surgery because I never stopped moving. I was in a wheelchair, but after DBS I could walk by myskeezerself.” Her husband Gordon notes that she had several good years.  Still, with a 25 year course, the disease has progressed. In the last 2 -3 years she has needed a wheelchair again, and a lot of help from him.  “She has gone downhill gradually with the Parkinson’s, you can’t change that.  But, she still wants to do things, to be independent.”   Barbara states her husband is always reminding her “don’t do that by yourself.”  He says, “I have to watch her, and make sure she doesn’t fall.”  Yet, Barbara reports that DBS is still helpful and keeps her doing what she can.  “If you turn the DBS off, I can’t move at all.  So, it’s doing something good.” For her it was, and still is, a definite benefit.

Forty-three year-old Melinda Jewell has young-onset PD, and in 2013 had right brain DBS in Boston. In 2015 the left brain was implanted by Dr. Rughani in Portland.  She is happy with the results and tells me the local Jewellexperience was ”really good, with a great team.”  She was a little nervous about going through it a second time in a different place. At the procedure,”Dr. Rughani talked to me and I think he was distracting me a little,” she smiles.  “And, the anesthesiologist was funny.  He made jokes and I listened to country music.  I forgot to be nervous and I am glad I did it.”  As to whether or not it has been helpful, she says, “Yes! I still have some issues, but I probably wouldn’t be working now if I hadn’t had DBS.”

PPatricia Joyceatricia Joyce has had PD since 2005, and underwent DBS surgery in 2014 with Dr. Rughani. She notes, “I didn’t think the surgery was bad at all.  It was pretty cool actually. They warned me that the worst part was staying still for so long. I did get a little itchy, but other than that, it was fine. I’m happy I did it, and I’d do it again.”

Still, as Dr. Rughani notes in our interview, the risk and benefit should be taken very seriously, and carefully considered before proceeding with surgery.

1. Debru, A. The power of torpedo fish as a pathological model to the understanding of nervous transmission in antiquity. C. R. Seances Soc. Biol. Fil. 2006;329: 298–302.

2. Penfield, W., Boldrey, E.  Somatic motor and sensory representation in the cerebral cortex of man as studied by electrical stimulation. Brain  1937; 60, 389–443.

3. Delgado et al.  Technique of intracranial electrode implacement for recording and stimulation and its possible therapeutic value in psychotic patients.  Confin. Neurol.  1952;12:315-19.

4. Benabid et al.  Long-term suppression of tremor by chronic stimulation of the ventral intermediate thalamic nucleus. Lancet 1991; 337:403–406.

5. Blond, S., and Siegfrid, J. Thalamic stimulation for the treatment of tremor and other movement disorders. Acta Neurochir. 1991; S52: 109–111.

6.http://www.fda.gov/newsevents/newsroom/pressannouncements/ucm451152.htm

7. http://www.neuromodulation.com/deep-brain-stimulation

A brief history of the PD non profit groups in Maine

In the summer issue we ran a story about the New England Parkinson’s Ride, an annual event which donates proceeds to the Michael J. Fox Foundation.  This issue I will focus on two other groups which have raised funding for either national or local PD efforts.

The Maine Parkinson Society (MEPS) is currently headquartered in Bangor and is a 501(c)(3) charitable organization with a mission “to improve the quality of life for an estimated 7,000 Maine people affected by Parkinson’s disease (PD). This is achieved through educational programs, by providing respite care services to health care workers and caregivers who work with people with PD, and by funding other programs that work to find the cure.”

MEPS has worked to spread information since its beginning.  The Secretary of State accepted the group’s articles of incorporation in July 1998.  Per the Lewiston Sun Journal, at the time, PD was known as the “disease of the invisible,” and interviewees noted that funding for PD research ranked 32nd, “just ahead of sleep disorders.”  They stated that amount of money was equivalent to only six percent of the total directed to AIDS research.  The article reported, “The Maine Parkinson Society is intended to provide greater visibility and to improve service to victims of the disease.”

At that time, MEPS was based in Portland and the original president of the group was Gregory Leeman, a human resource director of Blue Rock Industries, who was quoted as saying there were then no movement disorder neurologists in Maine.   He despaired as a PD sufferer, “the variety of drugs used for treatment adds to the confusion   It is difficult, even when you keep daily charts as most of us do, to find a smooth drug regimen.  It all adds up to a roller coaster ride that leaves patients in an emotionally drained state.”

Vice president of that first board was Karen Bardo of Alna, who worked as an administrative secretary in special education.  She had been diagnosed just two years prior and was reported to be the glue that had kept the effort to form MEPS going.  Ms. Bardo kept up that enthusiasm for several years, and would later serve as MEPS president.  Other initial members included treasurer Leonard Kaminow, M.D. (then and now in the practice of neurology in Scarborough), and secretary Harold Jones of Augusta.  Of note, Mr. Jones, the father of Karen Bardo, would also go on to become president of MEPS for a time, and passed away due to complications of heart disease in December 2008.   A touching portrait of his life was published in the Kennebec Journal, and in the winter 2009 edition of the Maine Parkinson’s Pages.

MEPS hosted many activities over the years to raise both awareness of PD and funds for the mission of the group.  Often, MEPS worked in collaboration with the already-established Maine Chapter of the American Parkinson Disease Association (APDA), which had been a long-time fundraiser and helped to organize support groups throughout the state.

At first, however, it was not clear the two groups would work together.  The Maine APDA president for 25 years was Carl Barker, who retired from that role in 2009.   Barker, interviewed by phone, looks back on those days and notes, “at first I didn’t see the need for the two programs, and I thought that they ought to join us.  But, when it became clear that they wanted their money to remain in the state, to help people with respite and other projects here, we worked together.”   Barker notes “people who wanted to give money for research were encouraged to give to the APDA as MEPS had no channel for that.”

That they did work together seems evident.  Each organization hosted a variety of their own fundraisers such as bowl-a-thons, walk-a-thons, auctions, and the like.  For several years, they also collaborated on events such as the seminar for Parkinson Disease Awareness Day each April.   In 2000, for example, the Bangor Daily News reported on the seminar, which was held in Augusta.   Speakers included general neurologist Dr. Bernie Vigna of Bath (who passed away last year), and Dartmouth-Hitchcock neurosurgeon, Dr. David Robert, who covered surgical options including deep brain stimulation.  Quotes by Parkinson disease sufferer Viola Macomber were moving.  She had expressed uneasiness about attending but apparently found the meeting very helpful when she stated, “This is incredible!  He’s describing me right down to my fingers locking, that I’ve blamed on arthritis.”

That Spring, the Lewiston Sun Journal reported another collaboration: the APDA and MEPS were “working together to bring a full time PD specialist to Maine so that patients will no longer have to drive to Boston for treatment.”  To attract a movement disorders doctor, the groups applied for grant money to set up an information and referral center.  In July 2001, the Bangor Daily News announced the opening of the American Parkinson Disease Association Information and Referral Center (IRC) at Maine Health’s Maine Medical Center facility in Scarborough.  Lillian Scenna, LSW, would become program manager that first year, and the IRC would eventually move to Bucknam Road in Falmouth for the duration of its existence.  The office, primarily through the efforts of Lillian Scenna, would provide information about PD to the general public; coordinate educational classes; cultivate support groups; publish the Parkinson’s Pages newsletter; maintain a lending library of books; provide assistance using the internet to research health-related questions; maintain a specialized list of community resources for people with PD; manage the administration of the Respite Care Reimbursement Program; and many other outreach efforts to increase awareness and proper care for Maine people with PD. The IRC also fielded a lot of phone calls.  Lillian Scenna estimated she took over a thousand calls per year from PD patients, and the top three questions were related to provider referrals, support group referrals, and medication side effects.  She was a member of the boards of MEPS and APDA, and was active in the planning and execution of most PD related events in Maine.

MEPS and APDA also found success in 2002, when Ed Drasby, D.O., brought the specialty of movement disorders to Maine   His practice evolved over time to include wellness in PD and participation in multiple clinical trials as a Parkinson Study Group site.  Studies included, for example, a trial of the drug pimavanserin, which would go on to become FDA-approved for psychosis in PD April 2016.  Dr. Drasby has been on the MEPS board of directors since coming to Maine, and was a board member of the Maine chapter of APDA.  He was instrumental in building a PD community in Maine and, as an aside, very welcoming and helpful in getting me here.  From the beginning, he asked me to be a part of these organizations.  His help and introductions were much appreciated.  Dr. Drasby retired from practice in October 2014, and though there are other movement disorders doctors in the State, I frequently hear from patients that he is missed. I suppose patients worry that I will feel diminished after such praises, as they are usually followed by something sheepish along the lines of, “no offense.”  Rest assured, none taken.

Regrettably, due to cessation of funding in December 2013, the Maine APDA chapter ended and the IRC in Falmouth closed, taking with it the role of Lillian Scenna.  These days, she is working with people with disabilities, helping them find jobs in her role as social worker at the Pine Tree Society.   Though she is not directly involved in Parkinson disease, she notes her services might be helpful for people with young onset PD.  “I still remember those calls,” she says.

MEPS is still going, with a full board presided over by long-time member Gary Cole.   This April a fundraiser was an event was called STRIDE – WALK – JOG – RUN for Parkinson Disease, which took place at the indoor track of the Boothbay Harbor Region YMCA.   Participants exercised any distance they chose, and endurance athlete Hunter De Garmo completed a 50K run.  According to the Boothbay Register, Hunter’s mother has PD.

For more information about how to help MEPS, please visit their website at http://www.maineparkinsonsociety.org/.  You can also find them on Facebook.