This is an interesting study published in May 2017 about palliative care discussions (or the lack there of) among 22 Canadian patients with multiple system atrophy (MSA) seen at a movement disorder center. “Palliative care discussions” include diagnosis and symptom management, prognosis, goals of care, and comfort measures. Interestingly only 16 of the 22 patients discussed these issues with their MDs, according to medical records. (So maybe not all “discussions” are recorded in patient charts.)
You can find the full article here: (available at no charge)
(Thanks to long-time friend Vera James for sharing this link with me.)
The authors argue that “Early and effective advance care and end-of-life planning discussions are…essential for effective palliative care in MSA. … [These] discussions … will allow people with MSA to make treatment decisions that better align with their goals of care prior to an emergent situation.”
And the authors argue that advance care planning must taken into account respiratory dysfunction. “Respiratory dysfunction, including but not limited to stridor, is the leading cause of death for people living with MSA, and it may present acutely with or without prior evidence of stridor.”
Perhaps these discussions are not occurring because stridor and sleep apnea are NOT the most common symptoms seen in MSA? Both symptoms occur among a minority of patients. Among the 22 Canadian patients, the most common symptoms were parkinsonism, orthostatic hypotension, GI/GU dysfunction, gait impairment, and ataxia.
But even patients with “modified diets did not have a documented discussion about possible future requirements for artificial feeding.”
However, if you have stridor, your medical team is more likely to discuss advance care planning with you (and document it in the medical record). “Five of the six patients with stridor had a documented discussion about tracheostomy before one was required for life-sustaining purposes. Of the three patients who required emergent tracheostomy, one had a documented discussion declining tracheostomy in the management of stridor. Nevertheless, this patient was later intubated in intensive care secondary to respiratory failure.”
Of the 22 patients, 6 were deceased. Though one of those six had a DNR (do-not-resuscitate) order in place, resuscitation was attempted by the care facility where the patient was living. And only 2 of those six had a healthcare agent listed in the medical record. Four of the six died in ICU. Of those four, “discussions surrounding resuscitation and goals of care took place” days or hours before death.
The authors say:
“Our chart review shows clear gaps in the current approach to advance care planning, goals of care and palliative care discussions in patients living with MSA, at least at our centre. Important advance care planning and end-of-life discussions took place very late in the disease trajectory and often at a time when the affected person was no longer able to participate. While our study is limited to a retrospective review of charts at a single centre, it nonetheless shows significant gaps in the provision of care.”
To remedy this situation, the authors propose a framework for palliative care discussions in MSA:
“In the early stages of MSA, it is important to determine who the person with MSA would like to have present for important healthcare discussions in general, and then establish each patient’s readiness to discuss end-of-life decisions. Since diagnosis of MSA is difficult, there may be a period of ambiguity regarding the diagnosis, and the patient might not yet be receptive to hearing about the disease trajectory and his/her prognosis. Nevertheless, even without a determinate diagnosis, identifying personal supports and discussing personal values will help to inform and simplify future discussions regarding end-of-life care, when appropriate. Information regarding diagnosis, expected disease trajectory, symptom management and prognosis should be disclosed to the person with MSA and his/her family or an appointed decision maker, where possible. Physicians should initiate and document the results of important discussions, after assessing the patient’s readiness for such discussions. These include the identification of a substitute decision maker or power of attorney for healthcare, the presence of advance care plans, preferences regarding artificial nutrition and hydration, including their expected benefits or the lack thereof, end-of-life care wishes and preferred place of death. While tracheostomy may extend the length of life, it does not, in our view, extend or improve the quality of life. Therefore, it is important to discuss tracheostomy and to document the patient’s wishes surrounding tracheostomy before an unexpected emergent event.”
The authors expect the neurologist to be active participants in the palliative care process. Neurologists can also make referrals to more specialized palliative care services.
“For some people, a referral [to palliative care] at the time of diagnosis might be appropriate, whereas for other people it may be more appropriate to initiate a referral once further severe symptoms develop, including dysphagia, stridor, sleep-disordered breathing, severe orthostatic blood pressure changes, increasing falls or other symptoms of distress.”