(Note: This article is based primarily on information provided by the National Institutes of Health [NIH].)
Anyone providing care for loved ones living with frontotemporal disorders (FTD), sometimes called frontotemporal dementia, faces a host of challenges. (Frontotemporal dementia has gained celebrity attention in 2023 after it was revealed that actor Bruce Willis was diagnosed with it.) A person with FTD can exhibit a range of symptoms, including unusual behaviors, emotional problems, trouble communicating, and difficulty walking. Adding to the challenge, and the stress, is the fact there is no cure for FTD and no way to slow it down or prevent it.
FTD can be hard to diagnose because the symptoms are similar to those of other conditions. So it can at times be overlooked or mistaken for something else, such as depression or other mood disorder. To make matters more confusing, a person can have both FTD and another type of dementia, such as Alzheimer’s disease. Finally, because frontotemporal disorders are rare, physicians may be unfamiliar with the signs and symptoms.
In the early stages, it can be hard to know which type of FTD a person has because symptoms and the order in which they appear can vary from one person to another. Also, the same symptoms can appear across different disorders and vary from one stage of the disease to the next as different parts of the brain are affected. With some frontotemporal disorders, the primary symptoms are problems with speech or movement, rather than dementia symptoms. Physicians and psychologists diagnose the different forms of FTD based on a person’s symptoms as well as the results of brain scans and genetic tests.
FTD is uncommon and tends to occur at a younger age than other forms of dementia. Roughly 60% of people with FTD are 45 to 64 years old. The disorder is progressive, meaning symptoms get worse over time. In the early stages, people may have just one symptom. As the disease progresses, other symptoms appear as more parts of the brain are affected. It is difficult to predict how long someone with FTD will live. Some people live more than 10 years after diagnosis, while others live less than two years.
In most cases, the cause of a FTD is unknown. Scientists are only beginning to understand the biological and genetic basis (such as gene mutations) for the changes observed in brain cells that lead to FTD. It is known that individuals with a family history of FTD are more likely to develop such a disorder.
Three types of Frontotemporal Disorders
The NIH identifies three types of frontotemporal disorders:
- behavioral variant frontotemporal dementia (bvFTD)
- primary progressive aphasia (PPA)
- movement disorders
The most common, bvFTD, involves changes in personality, behavior, and judgment. People with this disorder may have problems with cognition, but their memory may stay relatively intact. Symptoms can include difficulty with logical sequencing of steps needed to solve problems (what to do first, second, etc.) or prioritizing tasks; repeating same activities or words; acting impulsively or saying or doing inappropriate things; becoming disinterested in family or activities they used to care about. Over time, language and/or movement problems may occur, with the person needing more care and supervision.
Primary progressive aphasia involves changes in the ability to communicate — to use language to speak, read, write, and understand what others are saying. People with PPA may have one or mor of these limitations; they may actually become mute or unable to speak. Many people with PPA develop symptoms of dementia. Problems with memory, reasoning, and judgment are not apparent at first but can develop over time. In addition, some people with PPA may experience significant behavioral changes, similar to those seen in bvFTD, as the disease progresses.
Two rare neurological movement disorders are associated with FTD: corticobasal syndrome (CS) and progressive supranuclear palsy (PSP). These may affect thinking and language abilities as well. There is a progressive loss of the ability to control movement, typically beginning around age 60. This can even limit, for instance, the simple ability to close buttons on a shirt or operate small appliances. There may be muscle rigidity and difficulty swallowing. Symptoms may appear first on one side of the body, but eventually both sides are affected. Occasionally, a person with CS first has language problems or trouble orienting objects in space and later develops movement symptoms. Not everyone who has CS has problems with memory, cognition, language, or behavior.
PSP causes problems with balance and walking. Affected people typically move slowly, experience unexplained falls, lose facial expression, and have body stiffness, especially in the neck and upper body — symptoms similar to those of Parkinson’s disease. A hallmark sign of this disorder is trouble with eye movements, which may give the face a fixed stare. Problems with behavior, memory, problem solving, and judgment can also develop.
Other movement-related types of FTD include frontotemporal dementia with parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS). The first can manifest as movement problems similar to those of Parkinson’s disease. FTD-ALS is a combination of bvFTD and ALS (commonly known as Lou Gehrig’s disease.) In addition to the behavioral and/or language changes seen in bvFTD, people with FTD-ALS experience the progressive muscle weakness seen in ALS, fine jerks, and wiggling in muscles.
Providing care to someone with Frontotemporal Disorders
Caring for someone with a frontotemporal disorder (FTD) can be hard, both physically and emotionally. Caregivers may face challenges with managing not only the medical and day-to-day care but also changing family and social relationships, loss of work, stress, decisions about long-term care, and end-of-life concerns—all of which can negatively affect the caregiver’s own health. Spouses or partners may find themselves also having to take on more routine household responsibilities that their partner can no longer perform. Children may suffer the gradual loss of a parent at a critical time in their lives. Family members and friends may feel alienated or embarrassed by the person’s behavior.
Managing behavioral symptoms can involve several approaches. Here are some strategies to consider:
- Try to accept rather than challenge someone with behavioral symptoms. Arguing or reasoning will not help, because they cannot control their behaviors or see that they are unusual or upsetting to others. Instead, be as sensitive as possible and understand that it’s the illness “talking.”
- Take a “timeout” when frustrated — take deep breaths, count to 10, or leave the room for a few minutes.
- Regarding language issues and communicating, it helps to speak slowly and clearly, using simple sentences. Limit choices and offer specific choices. Open-ended questions, such as “What do you want to do today?” are more difficult to answer than specific ones, such as “Do you want to go to the park or for a walk?” Be ready to wait for responses, and ask for clarification if you don’t understand something. Gesturing, drawing, and using labeled photos of people and objects may allow you to communicate without talking.
- Maintain a regular schedule, reduce distractions, and modify the environment to reduce confusion and improve the person’s sleep.
- If compulsive eating is an issue, consider supervising eating, limiting food choices, locking cabinets and the refrigerator, and distracting the person with other activities.
Problems with physical activity for people with Frontotemporal Disorders
Several types of FTD cause problems with movement, including difficulty balancing, walking, and swallowing. Medications and physical and occupational therapy may provide modest relief for such movement symptoms. A team of specialists — doctors, nurses, and speech, physical, and occupational therapists — familiar with these disorders can help guide treatment. Medications are available to treat certain behavioral symptoms. Always consult with a physician regarding medications.
For people with progressive supranuclear palsy, sometimes Parkinson’s disease drugs provide temporary relief for slowness, stiffness, and balance problems. Exercises can keep the joints limber, and weighted walking aids — such as a walker with sandbags over the lower front rung — can help maintain balance. Speech, vision, and swallowing difficulties usually do not respond to any drug treatment. Antidepressants have shown modest success. For people with abnormal eye movements, bifocals or special glasses called prisms are sometimes prescribed.
People with FTD-ALS typically decline quickly over two to three years. During this time, physical therapy can help treat muscle symptoms, and a walker or wheelchair may be useful. Speech therapy may help a person speak more clearly at first. Later on, other ways of communicating, such as a speech synthesizer, can be used. The ALS symptoms of the disorder ultimately make it impossible to stand, walk, eat, and breathe on one’s own.
Impact of Frontotemporal Disorderson work life
People living with FTD may have difficulty with basic work skills, such as organizing, planning, and following through on tasks. Activities that were easy before might take much longer or become impossible. People may lose their jobs because they can no longer perform. As a result, the caregiver might need to take a second job to make ends meet, or reduce their hours, or even quit working to provide care and run the household. An employment attorney can offer information and advice about employee benefits, family leave, and disability if needed. Workers diagnosed with FTD can qualify for Social Security disability benefits through the “Compassionate Allowances” program (800-772-1213), a
program that helps individuals with certain serious conditions access benefits quickly.
Long-term care for Frontotemporal Disorders
For many caregivers, there comes a point when they can no longer take care of the person with FTD without help. The caregiving demands are simply too great, and the person may need around-the-clock care. As the disease progresses, caregivers may need home health care services or to look for a residential care facility, such as a group home, assisted living facility, or nursing home.
End-of-life care for someone with Frontotemporal Disorders
People with FTD typically live six to eight years with their condition, sometimes longer, sometimes less. Most people die of problems that can arise from the FTD (e.g., trouble swallowing, balance problems causing injuries, etc.) Though difficult, it’s important to plan for the end of life. Legal documents, such as a will, living will, and durable powers of attorney for health care and finances should be created or updated as soon as possible after a diagnosis of FTD or a related disorder. An attorney who specializes in elder law, disabilities, or estate planning can provide legal advice, prepare documents, and make financial arrangements for the person’s spouse or partner and dependent children. If you do not currently have such an attorney advisor, check the National Academy of Elder Law Attorneys. In Colorado see https://www.justia.com/lawyers/elder-law/colorado. The American Bar Association can also help you find qualified attorneys. Another option is to research local bar associations which can help identify no-cost legal aid options. Colorado Law Help (https://lawhelp.colorado.gov/legal-aid) or Colorado Legal Services (https://www.coloradolegalservices.org/) may be of help.
Frontotemporal Disorders caregiver support in Colorado
Do not forget to take care of yourself too. FTD presents unique and daunting caregiver challenges. To help YOU stay healthy, the NIH suggests:
- Stay current with your regular health care.
- Ask family and friends for help with child care, errands, and other tasks.
- Spend time doing enjoyable activities, away from the demands of caregiving. Arrange for respite care — short-term caregiving services that give the regular caregiver a break. Or take the person to an adult day care center, a safe, supervised environment for adults with dementia or other disabilities. Check for locations of such resources at https://cdphe.colorado.gov/prevention-and-wellness/healthy-eating-and-active-living/health/facilities-and-providers-by-type.
- Join a support group for caregivers of people with FTDs. Such groups can be a valuable resource to share experiences and tips with others who may be in the same situation. Nonprofit organizations and community organizations may provide online or in-person support groups.
Contact the Association for Frontotemporal Degeneration at www.theaftd.org (866-507-7222) for valuable resources. The Alzheimer’s Disease Research Centers may also offer education and support groups.
To learn more . . . and maybe do more . . . about frontotemporal disorders
An online search of the phrase “information on frontotemporal disorder” will bring you an array of sources, including the Mayo Clinic, Cleveland Clinic, Johns Hopkins, the National Institutes of Health, and more.
Do you know someone living with FTD? Researchers are looking for people to participate in a study to track disease progression and advance treatments. Visit the ALLFTD study page to learn more.
Volunteers are also needed for clinical trials that are testing treatments for FTD. By joining one of these studies, you may learn more about how to manage FTD symptoms and contribute useful information to help others in the future. Families affected by inherited and familial forms of FTD can help scientists advance research by participating in such clinical studies and trials. If interested, check one or more of the following:
https://ftdregistry.org/find-a-study
AFTD – The Association for Frontotemporal Degeneration (theaftd.org)
