Parkinson’s Disease & End of Life

End-Stage Parkinson’s Disease & Risk of Death

It’s hard to think about the tough stuff. The what ifs, the worst-case scenarios, the unknowns.

Parkinson’s disease (PD) is a progressive condition, which means that it gets worse with time. However, for many people, the worsening is gradual, maybe even imperceptible at times. This affords you many years of good living – exercising, spending time with family, maybe even working. But we would be remiss if we did not educate you about the full spectrum of possibilities, since many of you want to know what obstacles you may face down the road and how to prepare for them.

Over time, for some people with PD, symptoms can become advanced and much more difficult to navigate. To help you, we created a special set of blog posts about advanced PD to address many of the What If’s of Parkinson’s Disease (authored together with Dr. Pravin Khemani, a Movement Disorders specialist in Seattle, WA).

A question we often get asked is – can advanced Parkinson’s disease lead to death and if so, how? When a person is first diagnosed with PD, they are often told “You will die with Parkinson’s disease, not of Parkinson’s disease”. This means that PD is not a fatal disease per se and end of life often occurs at an old age from another medical illness entirely. But is that true in all cases?

This week we will try to answer this important question.

Relationship between Parkinson’s disease and mortality

Multiple studies have investigated the connection between PD and mortality. Results are not consistent across studies, and some conclude that PD does not increase mortality over the general population. Other studies conclude that people with PD have a mortality ratio of about 1.5 as compared with the general population. This means that the rate of observed deaths among all people with PD is about 1.5 times higher than would be expected in the general population.

However, even studies that conclude that PD overall does not confer an increase in mortality over the general population, find that people with PD who have certain characteristics are at an increased risk of dying. These characteristics are associated with advanced PD and include:

• Longer duration of disease
• A higher Hoehn and Yahr motor stage with increased level of motor disability
• Cognitive dysfunction
• Hallucinations
• Presence of comorbid medical conditions

How can certain symptoms of advanced PD increase risk of dying?

Advanced PD symptoms can contribute to an increased risk of dying in several ways. At this point we are not able to stop the progression of PD, however there are some steps you can take that may minimize certain risks, so it is important to understand the symptoms of advanced PD and how they can be managed.

1. Falls – Due to increased balance problems as well as the presence of neurogenic orthostatic hypotension (NOH), people with advanced PD are at an increased risk of falls. Falls are one of the major causes of emergency room visits and hospitalizations for people with PD. Falls in turn can cause life-threatening injuries such as traumatic brain injury. More commonly, falls can cause fractures which are also linked to an increased risk of mortality. Although not every fall can be prevented, physical therapy to improve balance and assess for the appropriate walking aid can be very helpful. Assessing bone density on a regular basis is also important to prevent fractures in the case of a fall. In addition, NOH should be routinely assessed for and treated, to minimize the chance of falls.
2. Pressure ulcers – Due to increased immobility, people with advanced PD are at more of a risk of developing pressure ulcers, a breakdown of skin and underlying tissue resulting from prolonged pressure on a particular part of the body. If not promptly recognized and treated, pressure ulcers can lead to infections of the underlying tissues and even result in sepsis (a life-threatening illness caused by your body’s response to an infection). The chance of pressure ulcers can be minimized by frequent repositioning, protection of bony prominences that are in contact with resting surfaces, and maintenance of good nutrition.
3. Pneumonia – People with advanced PD may have progressive difficulty with swallowing, which can lead to aspiration. Aspiration occurs when saliva or food enters the lungs instead of the gastrointestinal tract and can cause pneumonia. In addition, people with advanced PD often have a weak cough which means they can’t cough up what they aspirated, and also can’t clear their airways of secretions once pneumonia has started. Pneumonia has been found to be a more common cause of death among people with PD than the general population, highlighting the contribution of swallowing difficulties in mortality in PD. In addition, those with PD can die from choking accidents. In order to prevent these problems, talk with the neurologist about a swallow assessment that can determine which foods are safe for the person with advanced PD to eat. For some, swallowing therapy can also be instituted to improve swallow function.
4. Other infections – Increased frailty is linked to advanced PD. In general, the frail elderly have less reserve to fight infection and withstand new medical problems. Therefore, in the advanced PD population, a minor medical problem such as a urinary tract infection can spiral into a bigger problem that can lead to more widespread infection and even death. A hospitalization for a seemingly treatable cause can disrupt the delicately balanced equilibrium at which the frail elderly lives.

A minor hospitalization, that in a less frail person would be easily weathered, can unfortunately spiral into permanently increased disability and death in the following ways:

1. Increased confusion – with the abrupt change in his/her surroundings, the person with advanced PD may get more confused. Increased hallucinations and agitation are common. The person may pull at their IV and increase their risk of infection. Medications to control hallucinations and agitation may be introduced to help but can also cause problematic side effects.
2. Increased risk of falls – with increased confusion, the person with advanced PD may try to get out of bed and fall. Often, the nursing staff will identify the person with advanced PD as a fall risk and not allow the person to get out of bed without help. This is important to prevent falls, however, can lead to much more immobility than is typical for the person with advanced PD, causing further disability.
3. With increased agitation and the nursing staff’s desire to prevent falls, restraints may be ordered to ensure the person stays safe in bed. These can further increase confusion, immobility, and disability.
4. With an inability to get out of bed, the person with advanced PD may have a urinary catheter placed. This increases the risk of a urinary tract infection, which in turn can lead to a larger infection
5. In a hospital setting, the person with PD may not get their medications on time, which will invariably lead to more immobility and disability. It can also impact swallow and increase the chance of aspiration in the hospital.

These are just some of the ways in which a medical event can lead to death much more easily in someone who is already frail from PD than in someone who is not.

How will the person with advanced PD experience death?

The journey for every person with PD will be different. Their symptoms, disease progression, access to medical care, and their concurrent medical issues (among other things) may vary widely and play a role in their life and their death with PD. As described above, the person with advanced PD typically dies from an event that occurs on top of the increasing levels of frailty and disability that build up over time. That event may be a fall, an infection, a choking event, or another medical issue that leads to a hospitalization and causes a more precipitous decline.

Regardless of the cause, when a neurologist assesses that the possibility of death within a particular time frame is likely, hospice care can be initiated. This is different from palliative care, which is a clinical approach that focuses on improving quality of life for people with complex diseases. Palliative care can begin at any point during the disease progression and work in conjunction with the treatment plan. Hospice care, on the other hand, is provided to improve the quality of life when end of life is near.

In summary, while it is true that PD in and of itself is not fatal in the same way that a rupture of a brain aneurysm or a massive heart attack may be (although both of these disorders can be survived under the right conditions), the disability caused by PD can contribute to death in a variety of ways as outlined above.  Educating yourself on the various possibilities and areas of concern may help you better prepare for the future, minimize some risks, and possibly enhance or prolong quality of life.

Tips and Takeaways

• Some studies show that the rate of deaths among all people with PD is about 1.5 times higher than would be expected in the general population
• Although PD is not fatal in and of itself, certain characteristics of advanced PD are associated with an increased risk of death including cognitive decline, hallucinations, and an increased level of motor disability
• Advanced PD can cause falls, pressure ulcers, swallowing difficulties and general frailty, which are all linked to death
• Proactive steps can help minimize certain risks, and possibly prolong quality of life
• View our other blog articles about advanced PD to learn more and help you navigate these challenging issues