Understanding Disease Progression and Advanced Parkinson’s Disease

When it comes to determining the best treatment options for their patients with Parkinson’s, healthcare professionals rely on knowledge of disease progression. Parkinson’s is degenerative, so many people living with the disease will require a different approach to care at each stage. These stages are determined by consensus among Parkinson’s experts, and are based on research observations of patients’ commonly-shared symptoms.

For people with Parkinson’s, it is important to discuss disease progression with your healthcare team in order to better understand your treatment, therapy, self-management, and medication options. By being familiar with the different stages of Parkinson’s disease (PD), you can also become better equipped to recognize the symptom changes that may indicate disease progression into a later stage.

Parkinson’s Disease Progression

Parkinson’s starts in what is classified as the “premotor stage,” when symptoms, mostly non-motor, begin to occur. The onset of early symptoms often begins 5 or more years before motor symptoms develop. These symptoms start off mild, and may go undetected for many years. In this stage, symptoms can include depression, sleep disturbances, constipation and bowel changes, autonomic nervous system dysfunction (which impacts the control of unconscious body functions), and a reduced sense of smell.

The disease then progresses into early-stage Parkinson’s, in which rigidity, dyskinesia (tremors), bradykinesia (slowed movement), and akinesia (freezing) may develop. With further progression, some people with PD may also experience vision impairment and mild cognitive impairment. In the advanced stages, frequent falls are common, as is the onset of dementia or psychosis.

One of the most commonly-cited systems measuring the progression of Parkinson’s, called the Hoehn and Yahr scale, classifies the disease into the following five stages, based on motor symptom progression and personal independence (Hoehn and Yahr, 1967):

• Stage One: mild symptoms like tremors on one side of your body, and changes in posture, walking, and facial expressions
• Stage Two: symptoms worsen to tremors and rigidity on both sides of the body; walking issues and poor posture may be noticeable to others
• Stage Three: loss of balance and slowness; falling becomes more common, and it may be difficult to dress and feed oneself
• Stage Four: movement may require a walker or other assistive device, and it becomes increasingly difficult to perform daily tasks unassisted
• Stage Five: it may become impossible to stand or walk, and a wheelchair is required; hallucinations and delusions may also occur

Evaluating Disease Progression and Healthcare Options

Parkinson’s is difficult to predict, and its progression can be a vastly different journey for each person with the disease. In order to get the best healthcare possible, it is important for people with PD to self-report their symptoms and experiences, and work with their healthcare teams to build personalized care plans.

When healthcare professionals evaluate people with Parkinson’s, they may rely on a few different classifications of disease progression. The most commonly referenced evaluation system in Parkinson’s research, the unified Parkinson’s disease rating scale (UPDRS), groups together different patient evaluation techniques and disease progression scales, in order to build a holistic clinical rating scale. This scale is formulated in four parts, which include questionnaires for guiding healthcare professionals’ assessment of patients with Parkinson’s, as well as for patients to self-report their symptoms and experiences (Goetz et al., 2008).

Evaluations like the UPDRS help healthcare professionals map the progression of their patients’ Parkinson’s, in order to determine the efficacy of treatments, therapies, and interventions, as well as inform new treatment options. Clinical studies often evaluate the condition of their research participants using the same scale, which makes it easier for healthcare professionals to compare research findings with potential healthcare outcomes for their own patients. For example, if a certain medication is clinically proven to help improve motor function in people with Parkinson’s evaluated to be in a certain stage of disease progression based on the UPDRS, healthcare professionals may prescribe such a medication to their patients with a similar UPDRS evaluation to that of the research participants.

Each stage of Parkinson’s requires a different type of care. During early stages, people with PD can receive treatment from a general neurologist, and self-manage with oral medications and exercise. As the disease progresses, many healthcare professionals will also refer their patients with PD to physiotherapists, occupational therapists, speech-language pathologists, counsellors, and social workers, depending on their needs. In advanced stages, many people with PD will lose their independence in some aspects of life, and require additional support from their carepartners, or home and community care workers.

Symptom management in the later stages of Parkinson’s can be challenging without specialized care and multi-drug regimens. Sometimes, device-aided therapies like Duodopa® (levodopa-carbidopa intestinal gel) or Deep Brain Stimulation (DBS) are also necessary for people with PD to maintain their quality of life. These types of advanced Parkinson’s care are best provided by movement disorder specialists, who are experts in treating people with PD and other neurodegenerative conditions like Huntington’s disease, dystonia, and essential tremor. A clear and concise definition of advanced Parkinson’s disease helps primary care physicians know when it is time for their patients to see a movement disorder specialist.

Advanced Parkinson’s

While there is still some debate among professionals on what exactly classifies as advanced Parkinson’s disease (APD), leading movement disorder specialists from 10 European Union countries came together in a recent study to reach a consensus about its classification. They came up with a simple definition, called Five-Two-One, which determines someone to have APD if they:

• take 5, or more, oral levodopa doses per day,
• have 2, or more, hours a day of “off” symptoms, and
• have 1, or more, hour(s) of moderate troublesome motor dyskinesia (Antonini et al., 2018).

There are, of course, exceptions to this definition, and every case of APD is unique. However, this definition provides a good rule of thumb for neurologists and patients, and allows them to plan their care accordingly. When a patient fits the Five-Two-One definition, it may be time for their primary care physician or general neurologist to refer them to a movement disorder specialist.

Planning for Advanced Stages

Throughout all stages of life with PD, it is important to prepare for what may come next. While these can be difficult conversations to have, being prepared for the future can make a huge impact on improving your quality of life and the lives of your loved ones.

As a degenerative condition, Parkinson’s requires a palliative approach to care from the point of diagnosis. Palliative care refers to a holistic care strategy, involving your whole healthcare team, that takes into account the progressive and life-altering nature of your condition. It means that your healthcare team is focused on maintaining your quality of life, beyond meeting your basic medical needs. As a person receiving palliative care, you are empowered to make informed decisions on the types of treatment and support services you prefer.

There are many ways you can plan for APD, including:

Symptom Tracking: A crucial part of PD care involves tracking your symptoms and reporting them openly and accurately to your healthcare team. Any changes in your condition should be shared with your primary care physician and neurologist, no matter how big or small. A worsening symptom could be a sign of disease progression, medication side effects, or ineffective treatment. By catching these changes as early as possible, your healthcare team can respond accordingly, and support you in maintaining your quality of life.

Mental Health and Independence: It can be difficult to cope with losing your independence as Parkinson’s progresses. Many people with PD also find themselves losing their sense of identity, and their interest in hobbies and leisure, as the disease progresses. Speaking to a mental health professional can help you gain a positive outlook and learn healthy coping strategies. Accepting help, especially from loved ones, may be challenging, but is necessary in order to stay safe and protected from unnecessary harm and injury. A counsellor or therapist can work with you and your family to ensure you feel comfortable and supported in all aspects of your life.

Work: For those diagnosed with Parkinson’s before the age of retirement, it is important to discuss potential workplace adaptations and accommodations with your employer, such as allowing adaptive technology, flexible scheduling, or a reduced workload. Parkinson’s is considered a disability by law, so your employer is obligated to make a reasonable effort to accommodate your needs, and they cannot treat you differently because of your PD. If you do decide to discuss your PD with your employer, you may also find it helpful to connect them with a member of your healthcare team who can provide information on suitable accommodations, and advocate for your needs.

Finances: Depending on your situation, you and your family may be eligible for certain tax credits, benefits, and deductions. Speak to your healthcare team, as well as your accountant or financial advisor, about your eligibility. If you do not have one already, you should also explore your options for a comprehensive insurance plan to cover medical expenses and support services. It is never too early to begin planning and saving money for the future, and as your PD progresses, you can revisit your financial plans to ensure you are prepared for any next steps.

Legal: Discuss advance care planning with your family as soon as you feel comfortable to do so. As PD progresses, it may cause cognitive changes that can impact an individual’s legal authority to make healthcare decisions. In that case, a loved one can be appointed power of attorney to make these decisions for them. It is important to make your loved ones aware of the type of care to wish you receive in later stages of the disease, so they are prepared to advocate for you should they need to.

Sources

Antonini, A. et al. (2018). Developing consensus among movement disorder specialists on clinical indicators for identification and management of advanced Parkinson’s disease: a multi-country Delphi-panel approach. Current Medical Research and Opinion, 34(12). Retrieved from: https://doi.org/10.1080/03007995.2018.1502165

Goetz, C. et al. (2008). Movement Disorder Society‐sponsored revision of the Unified Parkinson’s Disease Rating Scale (MDS‐UPDRS): Scale presentation and clinimetric testing results. Movement Disorders, 23(15). Retrieved from: https://doi.org/10.1002/mds.22340

Hoehn, M., Yahr, M. (1967). Parkinsonism: onset, progression, and mortality. Neurology, 17(1). Retrieved from https://doi.org/10.1212/WNL.17.5.427 

This content was published in the Summer 2020 edition of our quarterly magazine, Viewpoints. The content was accurate as of this publication date.