Frontotemporal dementia (FTD), a progressive neurological disease, is misunderstood and poorly diagnosed. Although it’s often considered a rare disease, research shows it’s the most common form of dementia for those under the age of 60. Part 1 of this series explained what it is, signs and symptoms, and the importance of not waiting to explore these signs with a physician.
In part 2, we explore more about the disease, looking at the hereditary factor of FTD, ways to provide support, and resources for families.
When families receive a new diagnosis of FTD, Paul Schulz, MD, neurologist with UTHealth Houston Neurosciences and professor and director of the Neurocognitive Disorders Center with McGovern Medical School at UTHealth Houston, believes it’s most important to affirm they will have lots of questions. He has so much information to share, he said he obviously can’t do it during the first one-hour visit.
As a next step, Schulz describes possible symptoms that could appear as the disease progresses.
“There are 10 or 20 symptoms that may or may not ever arise, and we’ll talk about them along the way, so they can think about them and be prepared for them,” said Schulz, the Rick McCord Professor in Neurology and Umphrey Family Professor in Neurodegenerative Diseases at McGovern Medical School. “I want them to understand they’re related to this disease and not something different.”
One example is a swallowing change. If someone sounds like they’re choking, it might be time to get a swallow test. Later on, patients might stop eating, as though they forget to do it. Schulz describes that as a crisis, and they have to decide what to do.
“I tell them ahead of time they need to make that decision now, so there is a plan if it happens,” Schulz said. “People can go about three days without drinking water, if they decide not to tube feed. We’ll make them comfortable, they won’t feel any pain or thirst. They’ll just slowly slip away when the time comes.”
Medications are available to improve quality of life, such as the one to help “replace the filter” for those who have outbursts of anger. Sometimes it helps completely and sometimes 50%, Schulz relates.
“A lot of the patients develop depression and anxiety,” Schulz said. “We can treat that, of course. Bad behavior, per se, is not as easy.”
When talking about heredity with a disease, Schulz said he hates to reveal too much to families with the condition. For most diseases he sees, the hereditary component is low. With Alzheimer’s, for example, people will have an autosomal dominant form about 1% of the time, caused by rare inherited gene mutations.
“But with FTD, it’s more like a 40% family history,” Schulz said. “If there’s not five people in your family with it, then it’s probably the no-hereditary form.” Those in the 40% hereditary group can often name parents and older relatives who had the same condition.
Schulz said many of the people participating in his Alzheimer’s trials before 2011 probably didn’t have Alzheimer’s, in retrospect, because physicians couldn’t verify the diagnosis while the patients were alive.
“And we couldn’t tell FTD from Alzheimer’s, because we weren’t even aware of FTD until the ’90s,” Schulz said.
Currently, Schulz leads a genetic FTD study that explores the safety and tolerability of a gene therapy for those with FTD who have a mutation in the progranulin gene. Schulz is hopeful the Food and Drug Administration allows the small trial to progress to more people once it is proven safe. He wants more FTD patients involved, because he’s looking for a specific gene of the 18 genes they believe they have a chance of affecting.
Similar to Alzheimer’s, Schulz said it’s challenging for loved ones supporting a patient with FTD. He can work on easing the symptoms for the patient, but the one who cares for the patient has the most stress.
“It’s unbelievably stressful, and you see it in the families as they come in for appointments,” Schulz said. “The spouse starts looking ragged, their loved one is no longer on the right sleep schedule, and the spouse wakes up, because it’s disruptive.”
People with FTD require total care, at some point. One thing Schulz suggests for spouses of patients with either form of dementia (Alzheimer’s and FTD) is to give themselves permission to let go a little bit. He said many people take their vows “till death do us part” very seriously — even if it harms their own health. He gently suggests having their loved one moved to a facility where they can see them every day, but get the sleep they personally need at night, while a nurse cares for their spouse.
“It’s really tough to talk people into that, but it’s probably one of the most helpful things I do for quality of life,” Schulz said. “For spouses dealing with this very stressful disorder, I explain that eventually everybody does it. It’s normal. It doesn’t mean you failed. You’ve been a wonderful spouse for them, and you’ll still see them every day.”
Patients with dementia never die of the brain change, said Schulz, but the complications from the disease, like pneumonia and blood clots from being bedbound. The length of the illness is variable from person to person. Schulz has seen people live with it one year, three years, and up to 15 years.
“FTD is a tougher disorder right now,” Schulz said. “We don’t have ways of affecting the underlying process, but I would argue we do make quality of life a lot better. We have lots of interventions along the way, but we’re not at the point of a cure.”
Explore the following FTD resources:
- Association for Frontotemporal Degeneration (AFTD): A Pennsylvania-based nonprofit that offers support, research, disease overview, ways to get involved, and support for health care professionals. The website also lists support groups available throughout the United States for those who care for or love someone with FTD. The list is arranged by state and city.
- FTD Disorders Registry: This online community is for those interested in participating in research and learning the latest on clinical and science updates.
- Remember Me FTD: Two women on opposite coasts, Rachael and Maria, created the Remember Me Podcast to generate awareness of dementia and foster community — after they realized they had a shared experience of caring for parents with FTD. They have created an online community and “strive to normalize grief, generate connection, and provide a safe space for people diagnosed with FTD and their loved ones walking the journey alongside them.”
- FTD support group in Houston: One of the listings with AFTD includes the Houston group that meets virtually the fourth Tuesday of each month, 7-8:30 p.m. The group includes people dealing with a similar diagnosis in different stages. Schulz said some people come back 10 years after their family member died of FTD to help others navigate the journey.