Understanding Disease Trajectory with Dr. Lauren Massimo
Dr. Massimo joins us back at the mic to discuss disease progression, what to expect and the general “curvilinear in nature” that FTD typically exhibits. Dr. Massimo thoroughly explains the three stages (mild, moderate & severe) while highlighting the most common complications in FTD. “One of the biggest hurdles you and your family will experience is finding a clinician who will walk this entire journey with you.” And, boy is she right!
The episode begins with questions – is this normal? Is this common? And what will this all look like? As we ask all the hard questions, our dear Dr. Lauren declares that it's “hard to predict.” And, boy is she right!
Dr. Massimo explains that the three stages are defined by how much the patient or person affected with FTD relies on others to accomplish their ADL’s (activities of daily living – think: brushing teeth, getting dressed, cleaning up, cooking, etc). The more support that is needed, the more likely they are further along on the journey. Reminder: FTD is a progressive disease which means throughout the course of the disease, things are getting worse, not better. Some in the later, more severe stages of FTD are generally extremely reliant on their care partner. Before diving into the stages, Dr. Masimo remarks that the average life expectancy for someone with FTD is about 8 years.
Switching gears into defining the different stages, Dr. Lauren begins by explaining that the first stage (Mild) is typically the longest as the brain continues to have compensatory mechanisms that keep normalcy and generally decent functioning. During the mild stage there is “difficulty managing the behaviors, but the ADL’s are completed with relative independence.” Dr. Massimo continues to describe that within the mild stage, there is a decrease in executive functioning but the main shift that family and friends typically see are the unusual, uncanny and peculiar behaviors that develop.
Next, and noted as the “longest” stage, is the moderate phase of the disease. Dr. Massimo notes that this is more of a “collaborative effort” with “most hands on deck.” This is where we typically see the most drastic shift in speech and language as well. Some clinicians believe that within this stage is where the memory components begin to deteriorate. Behaviors tend to worsen and the progression of atrophy in the brain continues to advance.
Lastly, the severe stage. This is where a person will need “complete hands on assistance for all or most ADL’s. “This is where we normally see incontinence present itself and where mobility issues begin,” says Dr. Massimo. During this stage, a person with FTD will generally become dependent on a wheelchair or even become bed bound. Basic functions become slower and less over time and the person is speaking very little at this point.
As Dr. Lauren discusses this, the feelings rush back. It’s all so heartbreaking.
FTD is complicated in general, but UPenn via Dr. Massimo has highlighted the following for top and most observed complications in FTD.
UTI: When incontinence hits, UTI’s become prevalent. If not treated appropriately and quickly enough, the infection can spread and lead to sepsis.
Falls: With gait and mobility issues, falls increase and become more detrimental.
Swallowing/Feeding Issues: Aspirating on food is extremely common in FTD. But, wait – there's more!
Aspiration Pneumonia: After a patient aspirates, the food is completely cleared and sits in the lungs causing this.
Feeding tubes: The efficacy hasn’t been widely studied in FTD, but within the general dementia umbrella – the effectiveness hasn’t been deemed beneficial.
Infected Feeding Tube: not ideal for various reasons
Persistent Diarrhea: The concentrated formula used for the feeding tube oftentimes causes patients to have incessant diarrhea.
The progression of FTD can vary significantly from person to person and is difficult to predict. Clinicians generally describe three stages: mild, moderate and severe or late stages.
The average life expectancy is about 8 years from the start of symptoms, but progression can vary from 2 to 20 years.
The three stages are generally defined by the amount of support and assistance the person needs from others for their activities of daily living. Activities of daily living include self-care, dressing, toileting, mobility and eating.
During the mild stage, the person will present with symptoms and still maintain independence in their activities of daily living. Although behaviors and changes can create significant challenges, the person will be able to compensate in many areas and be relatively independent.
In the moderate stage, the disease continues to advance in the brain and begins to impact other areas of functioning such as speech and language for a person diagnosed with behavioral variant FTD, or to impact movement or behavior for someone with PPA. The person needs more assistance from others for their personal care and activities of daily living.
In the late stage, the person becomes dependent on others for all activities of daily living and presents with more physical changes such as incontinence and mobility issues. In this stage, the person becomes more susceptible to infections such as UTIs, falls, swallowing and feeding changes.
Resources from AFTD
One of the biggest challenges is finding resources to support you through the entire journey. The AFTD HelpLine can help you find specialists in FTD and connect you with FTD support options.
A person living with FTD throughout the mild and even moderate stage may benefit from peer support and assistance to stay active and engaged. Visit AFTD’s webpage for support for people living with FTD.
Many families want to know what to expect through the progression with FTD yet struggle with these hard questions, anticipatory grief and seeing the changes in their loved one. The AFTD resources below can help you recognize this emotional impact and process your experiences.
AFTD Booklet: Walking with Grief: Loss and the FTD Journey
AFTD Webinar: Living the Grief of FTD: The Winding Paths of Loss, Love and Resilience
AFTD’s past issue of Partners in FTD Care focuses on approaches to care in late stage FTD with additional resources: Comfort Care and Hospice in Advanced FTD
