Category: Fall, 2017 Issue

Palliative Care Essential to Management of Chronic Neurological Disease

by Jessica Vickerson, FNP, MSW

Palliative care (pronounced pal-lee-uh-tiv) is specialized medical care for people with serious illness. This type of care is focused on providing relief from the symptoms and stress of a serious illness. The goal is to improve quality of life for both the patient and the family.

Palliative care is provided by a specially-trained team of doctors, nurses and other specialists who work together with a patient’s other doctors to provide an extra layer of support. It is appropriate at any age and at any stage in a serious illness, and it can be provided along with curative treatment.

Often, Palliative Care is thought to be intended exclusively for people living with life-threatening cancer diagnoses or for those who are at the very end of life.  However, Palliative Care’s key components provide essential support for those living with serious neurological conditions as well. These include the assessment and treatment of physical and psychological symptoms, identification of support for spiritual distress, expert communication to establish goals of care, and assistance with complex medical decision-making.   For example, in the absence of any curative treatment, the management of  Parkinson’s disease remains largely palliative despite the huge advances that have been made in medical knowledge.  The principles of palliative care should be applied throughout the course of the disease and not limited to the terminal end-of-life period. Palliative care experts can support patients and families with navigating the disease, identifying ways to maximize quality of life despite advancing symptoms, and supporting them with advance care planning.

Interested in learning more? Talk with your provider about how to access Palliative Care support. More information regarding Palliative Care can be found at: www.getpalliativecare.org

 

References

Brookdale Department of Geriatrics and Palliative Medicine, Icahn School of Medicine at Mount Sinai (A.S.K., R.S.M.), and the National Palliative Care Research Center (R.S.M.), New York, and the James J. Peters Veterans Affairs Medical Center, Bronx (A.S.K., R.S.M.) — both in New York.

Kelly, A.S., M.D, M.S.H.S  and Morrison, R. Sean, M.D. Palliative Care for the Seriously Ill. N Engl J Med 2015; 373:747-755August 20, 2015DOI: 10.1056/NEJMra1404684

National Collaborating Centre for Chronic Conditions (UK). Parkinson’s Disease: National Clinical Guideline for Diagnosis and Management in Primary and Secondary Care. NICE Clinical Guidelines, No. 35. London: Royal College of Physicians (UK); 2006.

National Coalition for Hospice and Palliative Care. The National Consensus Project Clinical Practice Guidelines for Quality Palliative Care, 3rd edition (2013). http://www.nationalcoalitionhpc.org/ncp-guidelines-2013/

 

 

 

 

How close are we to focused ultrasound for PD in Maine?

Focused ultrasound (FUS) is a specialized technique that was FDA approved to treat essential tremor (ET) in 2016.  Patients undergoing treatment receive a noninvasive MRI-guided procedure in which there is no incision, no breach of the skull as in deep brain stimulation (DBS).  Instead, beams of acoustic energy are directed from 1,024 tiny transducers toward the same target in the brain, the VIM nucleus of the thalamus.  Ultrasound energy combines at the VIM to lesion the nucleus by generating heat powerful enough to coagulate (and thus destroy) tissue.  The result is similar to any intervention that burns, freezes, or otherwise jams the signal in a brain region (much of the programming of DBS for example, is meant to jam a signal).  As of yet, with ET only one side is treated by FUS.

HOW EFFECTIVE IS FUS FOR ET?

In a major U.S. study involving 76 patients with medication-refractory ET, a 32-point clinical tremor rating scale was used to measure outcomes (1).  Hand tremor scores improved after FUS, from 18.1 at baseline to 9.6 three months after the procedure.  In other words, on average, tremor intensity lessened by about 50%.  Patients receiving a sham procedure averaged a drop from 16.0 to 15.8 points.  However, about 14% of the patients had a less than 20% improvement.  At three months, adverse events in the treatment group included gait disturbance (36%) and tingling/numbness (38%).  Slurred speech occurred in 1% of treated patients, but this apparently resolved.  At 12 months, gait disturbance was still present in 9% and tingling in 14%.  Overall, in the treatment group, disability was reduced by about 60% and about half of patients reported improvements in quality of life, which were maintained for the entire year of the study.

WHAT ABOUT FUS FOR PD?

In another trial (2), 30 patients with severe medication-resistant tremor underwent FUS:  18 with ET, 9 with PD, and 3 with both ET and PD.  The average age of the study population was 68.9 ± 8.3 years, with an average disease duration of 12.1 ± 8.9 years.  Patients with PD showed an average reduction in motor score (part III of the Unified Parkinson’s Disease Rating Scale) from 24.9 ± 8.0 to 16.4 ± 11 at one month, and 13.4 ± 9.2 at six months after treatment.  Over the two- year follow up, tremor reappeared in two of the PD patients and in two who had combined ET-PD.  In this study, reportedly no adverse event lasted beyond three months.

There are other considerations, and as of yet there is no FDA approval for PD.  At the annual meeting of the American Academy of Neurology (AAN) held this past spring in Boston, the topic of FUS in PD was discussed (3).  Dr. Paul Fishman noted that while some results were very positive in studies, going through the procedure itself might be uncomfortable.  Some patients felt like the metal frame required for the procedure was uncomfortable, and many complained of a feeling of heat or sense of “whirling.”  Since the original study, another 186 patients have been followed in open label studies of FUS.  According to Dr. Fishman, 83% of adverse events were rated as mild and 2% as severe.  Some of the adverse events had been persistent, and he noted, “This is not a risk-free technique,” though the overall incidence of serious adverse events was less than that of DBS.  Dr. Michael Okun also spoke at the AAN meeting on this topic.  By comparison, during DBS implantation a specialist is able to verify that the lead is in the exact right target of the brain; whereas “with ultrasound you cannot test to make sure you have hit the right target.  If you have adverse events that include paresthesias in the face, tongue and leg, you missed.”   There is also data showing that tremor may, more or less, “creep” back in the months after a FUS.  More concerning, he noted,

“You can’t troubleshoot an ultrasound lesion. When you have a problem, you’ve got a problem.  The lesion is irreversible.  It can’t be programmed or modulated.“

The opposite is true of DBS, wherein programming changes can often alleviate a particular symptom.

In September 2015, Kimberly Spletter of Maryland was one of the first PD patients to receive FUS, and was featured on Michael J. Fox Mobile News (5).  There is a 5-minute video on the site with some impressive before/after footage and interesting graphics.  In Spletter’s case, the indication was not tremor, but her advanced dyskinesias (6).  For this, a different target was chosen for FUS, the globus pallidus.  She was one of forty patients were enrolled in a study of FUS in PD.  It is my understanding that the study is completed, but the data is not yet published.  To see an follow up video of her in January, 2017 click this link: http://www.localdvm.com/news/maryland/more-than-a-year-after-breakthrough-parkinsons-disease-treatment-woman-does-better-than-expected/642454168

WHAT ABOUT MAINE?

As for FUS in Maine, we are yet to have a case done here.  I spoke with functional neurosurgeon Dr. Anand Rughani on the topic, who gave the following statement:

“It is an interesting option to consider for lesioning.  The concept of lesioning is not new in treating movement disorders such as essential tremor and Parkinson’s disease.  A lesion in the thalamus, for example, is called a thalamotomy, and has been widely used to treat tremor.  Other methods of creating a lesion include radiosurgery using Gamma Knife, which is not an actual surgery, and radiofrequency lesioning, which is an actual surgery.  In general, a few comments can be made when comparing lesioning to stimulation, as in deep brain stimulation.  Lesions can only be done on one side of the brain.  Lesions are not usually as durable as stimulation, meaning that the benefit may not last as long.  The side effects of lesions are not reversible in the same way that they can be with stimulation.  While there is now FDA approval for the treatment of essential tremor using focused ultrasound in the US, patients with Parkinson’s disease will need to consider this option through participation in an experimental trial.”

FUS is done in a few academic centers, such as Brigham and Women’s in Boston (4).  On the BW Neurosurgery website, the following key considerations are listed:  currently, insurance plans do not cover this procedure, and not everyone is eligible for focused ultrasound; eligibility requires evaluation by a neurologist and a neurosurgeon.  One consideration is skull thickness; too much is not good for FUS.  Senator Anthony Pollina of Vermont, for example, was disqualified from FUS for his PD for this reason, and opted instead for DBS (7).

Thus, there is a lot to be hopeful about with this new procedure, but it will be some time before it is available in Maine.  More data needs to be collected, and FDA approval given.  Still, over time it is likely that the procedure will continue to be refined and advanced.

REFERENCES

  1. Elias, et al.,  A Randomized Trial of Focused Ultrasound Thalamotomy for Essential Tremor. N Engl J Med. 2016;375(8):730-9.
  2. ZAaroor, et al.  Magnetic resonance-guided focused ultrasound thalamotomy for tremor: a report of 30 Parkinson’s disease and essential tremor cases. J Neurosurg. 2017 Feb 24:1-9.
  3. Susman, E.  Pro and Con: Is Focused ultrasound More Effective than DBS for Parkinson’s Disease? Neurology Today. 2017;17(1):34-5.
  4. http://www.brighamandwomens.org/Departments_and_Services/neurosurgery/NeurosurgicalTechnology/default.aspx?sub=1
  5. https://www.michaeljfox.org/mobile/news-detail.php?how-focused-ultrasound-helped-my-dyskinesia-from-parkinson
  6. https://www.michaeljfox.org/foundation/news-detail.php?first-patient-treated-in-dyskinesia-study-using-ultrasound-technology
  7. https://vtdigger.org/2017/01/16/digger-dialogue-surgery-gives-sen-anthony-pollina-new-lease-life/#.Wclu3VtSyM9

 

Vitamin D

Levels of vitamin D are lower in PD patients worldwide (1).  Several neurologic disorders are associated with vitamin D deficiency, including MS, stroke, and other neurodegenerative disorders.   We are not sure yet what this means, or what all of the functions of vitamin D are (though what is known is extensive).   It seems clear, however, that keeping a normal level is a good idea.

Doctors measure vitamin D with a blood test.  The test checks levels of 25-hydroxy D total, a combination of D2 and D3.  We get vitamin D2 from diet, and some conversion is made when we are exposed to sunlight and UVB enters the skin, helping us produce the active vitamin D3 form.  According to Mayo Medical Laboratories New England, 10-24 ng/mL = mild to moderate deficiency, and optimum levels in the normal population are 25-80 ng/mL.

Interestingly, vitamin D receptors are found all over the brain.  One location that is relevant here is the receptors on large neurons of the substantia nigra, the darkly pigmented structure of our upper brainstem, which produces dopamine.   These cells are unfortunately lost throughout disease in PD.  The progressive depletion of these neurons over several years ultimately results in enough of a dopamine deficiency for the first motor signs of PD to show (tremor, stiffness, slowness).   As disease progresses, one has fewer of the cells, and therefore, less dopamine.

I am going to get a little technical here.  One thing that vitamin D does in these cells is to increase an enzyme called tyrosine hydroxylase.  This enzyme converts the amino acid tyrosine into dopa, a precursor of dopamine.  Therefore, we may need vitamin D to make dopamine efficiently.

Researchers have shown in mice that knocking out the vitamin D receptor on these neurons will result in a PD-like motor impairment (2).  This led the same investigators to question whether low vitamin D might predispose people to neurodegenerative disease.  Further, they wanted to know if keeping levels in the high normal range would make a difference.   In a 12-month study with 114 PD patients who averaged a vitamin D level of 22.5 ng/dL, 56 were given 1200 IU of vitamin D daily and 58 were given placebo.  At the end of the study, the treatment group had a higher vitamin D level, with an average of 42 ng/dL.  The very simple Hoehn and Yahr stage was stable, but not the more detailed UPDRSIII motor score.  This is not an impressive result, but it was a limited study.

The jury is still out on vitamin D levels in PD.  A normal level is probably a good idea for many reasons, but it is not clear if it will have an effect on disease progression.  There is no data on high levels, except to say that taking too much can cause toxicity.  Acute vitamin D toxicity may cause confusion, excess urination and thirst, loss of appetite, vomiting, and muscle weakness.  Chronically high levels of vitamin D may result in kidney stones, loss of bone mineralization, and pain.  So, as with any vitamin, work with your doctor to make sure your level is normal.

REFERENCES

  1. Lv, et al.  Vitamin D Status and Parkinson’s disease” as systematic review and meta-analysis. Neurol Sci 2014;35:1723-30.
  2. Suzuki et al. Does vitamin D arrest the progress of PD?   American Journal of Clinical Nutrition. 2013;97:1004-13.

 

Life. Death. Everything in between.

by Grace Plummer, LCSW

Hi there.

My name is Grace Plummer. I have been a clinical social worker for about a decade now and practice currently at Mid Coast Medical Group Neurology.  I work with Dr. Bill Stamey as a Behavioral Health Clinician; hereafter abbreviated as BHC.

Dr. Stamey invited me to write a piece for his Maine PD News introducing myself and with a greater goal of discussing how the role of a BHC may be beneficial for someone living with Parkinson disease. His offer appealed to the amateur writer in me who initially thought that it’d be both interesting and useful to write about the most troublesome non-motor symptoms noted in patients with early and late stage PD, as well as how we know that there are skills that can mitigate these disturbances. I first thought I would get very clinical and talk about the prevalence of depression in PD, how it is undertreated and how seeing a BHC preventatively or at the onset of symptoms can reduce the burden of this psychiatric phenomenon.

But after tossing my preliminary ideas around for a week or two I reminded the writer in me that I am not a very good scientist or thorough researcher (we’ll leave that to Dr. Stamey, right?!) and so ultimately decided that I’d be wiser to speak organically and conversationally about myself, and then anecdotally about the observations I have made since joining the team here at the neurology practice.

I hope that is okay. Here goes…

In my pre-bedtime thoughts last night as I was reflecting on my day after tucking my twin daughters into bed, I became aware again of this recent theme that has been playing over and over in my mind since becoming a parent just a few short months ago.

My mortality.

My children’s mortality.

The mortality of everyone I love.

I’ve noticed that I spend a fair amount of the free time I have to wander about in my thoughts, which is terrifically minimal since having children, yet enough to capture my full attention when the topic is larger than that of the daily minutiae, thinking about death.

How will I prepare for it? When will it come? How will I teach my children about it as the last stage of development in our short, wild lives?

Some might say that this is premature, or even pathological. I disagree. In my contemplations, and in my work, I have come to recognize that thinking about death is ubiquitous, and maybe even healthy.

We all think about dying, though when it enters our minds and maybe why it is there are variable. That said, I don’t think we are particularly good at talking about it. Now, as mentioned before, I’m not a researcher so I don’t sit with empirical support for this hypothesis in my lap today, but I’d bet that if we looked closely at those of us here in the United States as compared to other cultures in other countries, we’d find that we’re especially ill prepared to talk about mortality. No one’s fault. It just is. We’re scared. We’re too busy. We think it’s morbid to talk about death and dying. We equate dying with serious illness instead of with life.

Curious thing about this observation is that I haven’t met a person yet who doesn’t want to talk about it. In truth I spend a great amount of time helping people sort through their beliefs about life, and their beliefs about death. If words didn’t dilute our hopes and fears, it seems that most of our communication would be about life and death, right? What more is there, really? And what is at the root of depression? Fear, loneliness, sadness. Perhaps worry about our mortality and the mortality of those we love.

It’s relevant to share that I’ve noticed how those of you in the Parkinson’s Clan, (I very respectfully and fondly use the word “clan” to collectively describe those of you in the Brunswick area who have created this caring, connected sub-community to thoughtfully and meaningfully tackle this disease through participation in support group, exercise programming and informal lunch dates) are greatly courageous in your wrestle, perhaps your dance, with the universal , inevitable thing that is death. I’ve sat with you while you’ve cried, begged, cursed, laughed, planned, and celebrated. I’ve watched with enormous regard as you’ve shared your ideas and wishes with me. I’ve truly been honored to be a small part of your clan.

I value this role. My role in the neurology practice as a BHC.

My real work here is to create a space where you can address what matters by uncluttering and removing what may be in the way.

If you’re looking for a place to study and contemplate, whether it be about life or death, I’m here. I don’t have all of the answers but I’m certainly willing to journey into the unknown with you in the hope that living with PD brings you closer to your true self.

And because no one should ever grapple too long alone.

Take good care always,

Grace

 

 

Working out in Belfast

Randall Curtis is in a community of Parkinson Disease (PD) patients who have been through the LSVT BIG program, and have been meeting for a graduate class in Belfast over the last year and half.  Mr. Curtis estimates about 30 have been through the class, and many continue to meet.  “It is twice a month and doing the class is really good for reinforcing the BIG exercises.  We add new moves we don’t do at home.  These are extensions of the program.  The occupational therapy part is always good, working on core and some pretty interesting things.  They added music to try to get us to work with rhythm.  It’s a really great workout.”  He notes that his daughter, Amanda Curtis, PT, is an instructor, along with Patrice Fox, OT.  “They give up their lunch hour for the sessions,” says the proud father and student.

The instructors and I spoke about the program.  Amanda Curtis reports that the class takes place at the Waldo County Hospital rehab facility on the second and fourth Wednesday of each month, and lasts about an hour.  They tend to split into two groups and break the session into 15-minute segments.  They may run through LSVT, then do circuit training on balance, fine motor skills, core strengthening, and boxing.  “We’re pretty proud of it up here, because patients have been able to maintain what they have learned in BIG.” 

She reports that the high intensity exercise takes a lot of effort, especially given the number repetitions.  “We really push people, but it pays off.”  In fact, she and Patrice Fox have recently traveled to St. Paul, Minnesota to become PWR! (Power) certified and to expand their PD workout repertoire.  “I love this population, obviously, and I have a special place in my heart because of my father.  They tell me it is really hard but they want to keep coming back because it works.  And they do, they come early and stay late.  For me there is something really rewarding about that.”

Patrice Fox noted, “One thing I really love about the group, aside from the physical gains they are making, is that this has become more of a support system.  When people exercise together it gets them talking about what they are doing, about medications, and about other issues that they have in common.  I think it makes them realize they are not alone on this journey, and there are other people here to support them.”  She finds that the camaraderie is important and that “they encourage one another without realizing it.  It’s wonderful to watch it happen.”   It goes the other way too.  She feels supported by the all the positive feedback, and was very touched when a man in the class had a revelation of sorts with her when he realized it was not just him.  “It opened up his world to see that there are others with PD.”