Only Part of the Answer: Medications and FTD
Partners in FTD Care, Fall 2019
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Frontotemporal degeneration is clinically and biologically complex. There are currently no medications to slow or stop its progression, nor are there any FDA-approved treatments. Medications approved for other conditions, however, can be used as part of an individualized care plan to address some FTD symptoms. Effective pharmacological treatment requires correctly diagnosing the FTD subtype, a careful and patient approach to prescribing, and adjusting medications based on ongoing observation of problematic behaviors. Educating and engaging care partners as full members of the care team is essential. The case of Jane L. shows how patient and ongoing engagement with knowledgeable professionals can help a family navigate the complex emotions and heart-breaking decisions faced in FTD.
The Case of Jane L.
Early symptoms and diagnosis
Jane was a successful real estate agent living in a Boston suburb, where she was known as a loving wife and caring mother of her two children, Maddie and Eli. But beginning in her mid-50s, Jane began displaying changes in her behavior, personality and judgment, raising concerns among her family. She began posting inappropriate comments on Facebook, and spent hundreds of dollars on lottery tickets. She lost interest in old hobbies such as gardening, walking and doing the daily crossword. Most troublingly, she was bilked by scammers, who convinced her to give them nearly $150,000 in her family’s savings.
Once a warm, friendly and social person, she became agitated, paranoid and secretive. At work she was quick to anger, snapping at colleagues. Due to Jane’s increasingly antisocial behavior and declining performance, her employer put her on medical leave until she underwent a psychiatric evaluation.
Jane’s psychiatrist recognized that her symptoms were both unusual and seemingly increasing in severity. She immediately referred her for neurological and neuropsychological testing at a local interdisciplinary clinic. An MRI revealed bi-temporal atrophy, greater on the left than the right. While the family had not expressed concerns about her language or comprehension, the neurologist identified impairments in her ability to name objects.
Based upon the findings, Jane was diagnosed with behavioral variant frontotemporal degeneration (bvFTD) with features of semantic variant primary progressive aphasia (PPA), meaning she often struggled to understand the meaning of words. Clinic staff gave her family information about bvFTD and encouraged them to meet with both the clinic’s geriatric psychiatrist and its social worker for further disease education, supports and resources.
Jane’s husband, Charles, was overwhelmed—he was in denial about his wife’s dementia, and how drastically it would change their lives. He began to disengage, spending more time at his office rather than confront the problems at home. Despite his children’s insistence, he refused to talk to them about making a care plan, and declined to meet with clinical staff. For her part, Jane did not appear concerned about her diagnosis, but rather angry that she had to undergo testing. She believed nothing was wrong, and that she would soon return to work—but her employer would not re-hire her due to her diagnosis.
Family grapples with planning care
At home all day with no structure and little social interaction, Jane became more susceptible to financial scams. She spent significant portions of each day in contact with scammers; and despite her children’s attempts to convince her otherwise, she could not understand that they were deceiving her. Eventually they tried to take control of her banking accounts, disable her cell phone and cut off her internet access, but these attempts were successful only in infuriating their mother and creating even greater tension.
Eli met with the neurology clinic’s social worker, who informed him that the family would have to take steps to protect the family’s finances, and that his mother would need additional supervision during the day. Convinced that Jane’s anger, mistrust and aggression precluded such measures, Eli asked if any medications could be prescribed to “stop” or “control” her more dangerous behaviors, and possibly to address her language and memory changes. The social worker encouraged him to see the clinic’s geriatric psychiatrist, who had expertise in FTD. She would explain that medications can be helpful in conjunction with behavioral and safety interventions, and that the family would still need to find additional supervision for Jane at home. The next available appointment with the psychiatrist, however, was three months away.
Seeing that the family was struggling, the social worker tried convening a family meeting to discuss a plan for care, engagement and safety. But Charles declined to participate, saying he was too busy with work and that his children could fill him in on the details. The social worker said his presence was important and encouraged him to reconsider joining the meeting.
Incorporating medication and non-medication interventions
During the family’s three-month wait for the psychiatrist’s appointment, the social worker regularly met with Jane’s children to identify triggers of her agitation. Based on those triggers, the social worker suggested ways to anticipate and respond to her behaviors, as well as techniques that could alleviate her distress. For example, they discussed how to simplify her environment, minimizing overstimulation—and, therefore, her frustration. They learned the importance of adapting the way they spoke with her, creating structure and predictability in her days, and taking her outside the house for outings with friends, or by attending a day program for people with young-onset dementia.
The social worker suggested a speech therapy evaluation for communication strategies and an occupational therapy evaluation to identify home modifications to keep Jane safe. Her children were also receptive to attending an FTD support group and learning self-care methods. After implementing some of the modifications, they began seeing improvements at home. They began to understand that managing her more challenging behaviors took trial and error, and they felt more comfortable asking the social worker for advice when certain techniques proved ineffective. Knowing they could always ask the social worker for help empowered them to try out different approaches with their mother. One approach that worked surprisingly well was bringing friends over to spend time with Jane. Agitated at first, she eventually adjusted to these visits and began to look forward to them.
The family had high expectations for the psychiatrist appointment, hoping the doctor could prescribe medications to ease Jane’s more difficult behaviors and improve her thinking. As the date approached, Eli told the social worker he was worried that Jane would refuse to go, or would go but angrily storm out mid-session. The social worker relayed these concerns to the psychiatrist—who, because of her FTD expertise, had already anticipated the possibility of such behaviors.
The entire family, including her husband, Charles, attended the appointment. As they told the psychiatrist about the financial scams and the stress it caused, Jane showed no emotion. She demonstrated profound impairment of judgment, insight and abstract thinking. The psychiatrist placed her on a low dose of citalopram, an SSRI antidepressant, to address impulsivity and disinhibition; the dosage could be slowly increased as needed. Its possible side effects include nausea, lightheadedness, diarrhea and/or constipation.
The antidepressant may make Jane’s behaviors easier to address, but likely will not make the symptoms go away entirely, the psychiatrist said, adding that it would likely take some time before the family notices a difference. In any case, Jane will still need supervision, the doctor said. She asked Eli and Maddie to keep a log of Jane’s reactions to the medication and whether any new symptoms emerge.
Finally, Eli asked about a medication to improve her thinking. The psychiatrist explained that medications available to improve cognition for people with Alzheimer’s disease were unfortunately counter-indicated for those with FTD.
At the follow-up appointment three months later, Jane’s children reported that their mother was less hostile overall on the new medication, although she had erupted at the recent court appearance at which her husband was granted guardianship. Eli also shared some of her more troubling new behaviors: absentmindedly burning pots on the stove, drinking alcohol during the day and driving erratically. The psychiatrist assessed that the SSRI had minimal impact on Jane’s behavior, so she added divalproex, a mood stabilizer, to her regimen.
Charles appeared frustrated with his wife for her disruptive and unsafe behaviors, and with the clinic for being unable to stop them. The psychiatrist re-emphasized that medication alone would not address all of their concerns, nor would medication alone keep her safe. Jane should stop driving and should not be left alone, the doctor reiterated, and suggested meeting with the facility social worker to discuss support options. The doctor also gave the family her phone number and encouraged them to call at any time with questions.
Aside from occasional visits from neighbors and friends, Jane was mostly alone during the day. Despite encouragement from the psychiatrist, the social worker and his children, Charles remained convinced that his wife would refuse a hired caregiver, and that she would not fit well into an adult day program. (He also thought such services were too expensive.) After a scary driving incident, in which Jane tried to speed past an on-duty ambulance, Maddie decided to leave her job to care for her.
Adjusting with disease progression
Friends pulled away. Jane was increasingly difficult to be with— insulting others, acting restless. She developed hyper-orality; she craved sweets and was unable to regulate how much she ate. (After talking to the social worker and psychiatrist, Maddie started locking the pantry, limiting portions and keeping snacks in her purse to distract her mother when needed.) She impulsively pulled a fire alarm at church, causing the fire department to show up. Family began to carry items for Jane to fidget with as a distraction, and used AFTD’s Awareness Cards to request patience with her behavior in public.
Maddie started attending an FTD support group; her father reluctantly joined her the following month. Both felt relieved to learn their family was not alone on the FTD journey. Other spouses of people with FTD welcomed Charles with open arms— they understood and validated his experiences, allowing him to share his feelings. He learned that his assumption that Jane would react negatively to hired caregivers was preventing her from getting the care she needed.
Caring for her mother every day, Maddie observed that the divalproex had decreased Jane’s aggression but did not affect her impulsivity and compulsive behaviors. Jane developed a new fixation with picking up sticks and trash from their street, even with traffic present. She would obsessively check the mailbox, and open and close the fridge. Maddie tried to engage her mother— and keep her safe—by leading her to her past interests, including gardening, doing crossword puzzles and walking.
At the next psychiatry visit, Maddie asked about medication to dampen the intensity of these compulsive behaviors, which might let them hire a home care worker or attend a day program with less resistance from Jane. The doctor suggested adding quetiapine, an atypical antipsychotic, to her regimen, but noted it carried the risk of increasing falls, particularly at higher doses. Desperate to control Jane’s impulsivity, her family agreed to the change.
Jane responded well to the new medication, so—with the encouragement of his support group—her husband agreed to try a day program. To his surprise, it went well. Jane was active and mentally engaged. She no longer resisted showering, and she slept better at home.
Maddie began staying with Jane and Charles overnight to help out in the evenings. However, when Jane started waking up every day at 3 a.m., she decided she could no longer provide care overnight. At the next neurology clinic visit, the social worker recommended evening home care, and suggested ways to help Jane adjust to this change. She also outlined long-term care options for Jane. The psychiatrist, meanwhile, started to taper the dosage of divalproex and added oxcarbazepine, a different mood stabilizer.
Thanks to an AFTD Comstock Respite Grant that covered the cost of care for a weekend, Jane’s husband and children went to the cabin they had traditionally visited each summer. This long overdue break allowed Charles to rest and think more clearly about both what he needed and what was best for his wife. He agreed to a plan that included hiring evening care, and resolved to speak up when he could no longer care for Jane in their home.
That point came six months later, when Jane stopped letting others bathe her, brush her teeth, or touch her at all. The family moved Jane into a local nursing home facility. The clinic social worker and psychiatrist suggested to the family ways to support both Jane and themselves as they prepared to make this transition.
At 59, Jane moved into the local nursing home’s memory care unit. The staff, which had FTD experience, were able to engage her and limit how her behaviors impacted other residents. There were occasions, however, when Jane became confused and acted aggressively, hitting and pushing them or taking their food. She grew more confused and afraid; and, due to her aphasia, she could not communicate her needs. The clinic psychiatrist suggested a gradual increase of the quetiapine. The family understood the increased fall risk, but hoped that it would ensure her safety and the safety of others.
Jane went on to experience several falls, including one severe enough to put her in the hospital, where she greatly declined. Charles asked that her psychotropic medication be stopped due to both the falls and the fact that her behavior had become much less disruptive.
When Jane returned to the nursing home she was incontinent and unable to communicate, had difficulty swallowing and was no longer able to walk. Consequently, she met the criteria for hospice care. The hospice care team worked to ensure her comfort and dignity at the end of her life, providing holistic medical care to minimize her pain and discomfort. The hospice chaplain and social worker also offered Charles, Eli and Maddie spiritual support; they helped the family process their sadness and grief at seeing Jane so changed from the wife and mother she had been.
Despite their struggles over the course of the disease, Jane’s family agreed on what she needed most, and eventually found ways to honor the person she was before the onset of FTD. Jane died three months later on hospice care at the nursing home with her family by her side.
Questions for discussion:
What were family’s expectations about medication, and how did they adjust them over the course of the disease?
The family hoped to find a medication that would reduce or eliminate Jane’s behaviors. With the help of clinic staff, and based on their own caregiving experiences, they came to understand that the medications work in conjunction with non-medical interventions. The family also came to appreciate that it takes time to find the most effective medications—and even then, they are subject to constant re-evaluation and adjustment. They learned the importance of documenting the changes they saw, and of staying in close contact with the psychiatrist.
In what ways did the family and care team work together?
The family was fortunate to have access to an interdisciplinary care team made up of social work, psychiatry, behavioral neurology and neuropsychiatry. While not available in every area, this type of comprehensive care ensured that the different disciplines were consulting, communicating and making recommendations together.
Staff educated the family about FTD and set realistic expectations around medications before any writing any prescriptions. While the family waited to see the psychiatrist, Jane’s children met regularly with the social worker to learn how best to interact with their mother, how to adjust her environment, and how to process their feelings of sadness and loss.
What was most effective about the care team’s approach to Jane and her family?
Staff understood that each family member was processing Jane’s FTD at different paces. The psychiatrist realized that Jane’s children were more receptive to her suggestions than her husband. She understood the magnitude of the emotional struggle for Jane’s husband, and over time earned his trust by being available by phone to talk about symptoms or to make medication adjustments between visits. She referred them to the social worker when she sensed that Jane’s family needed more disease education, new ideas for behavioral strategies and support for themselves. They made a point to stay connected to the family, helping them simultaneously navigate community care options and medication management. Over time, the family became open to introducing greater levels of supervision and care that best supported Jane.
Boxer A.L., Tsai R.M. Therapy and clinical trials in frontotemporal dementia: past, present, and future. Journal of Neurochemistry. 2016 Aug; 138(Suppl 1): 211–221.
Jicha, G.A. Medical management of frontotemporal dementias: The importance of the caregiver in symptom assessment and guidance of treatment strategies. Journal of Molecular Neuroscience. 2011 Nov; 45(3): 713-723.
- Information for healthcare professionals on clinical presentations of FTD, diagnosis and treatment
- The Daily Care Snapshot: a tool to empower families to help healthcare professionals facilitate the transition into a facility or community-based setting, and to promote individualized care
- To find an FTD center near you, click here and identify your region, or contact the AFTD HelpLine: 1-866-507- 7222 or [email protected]
- The Medicare Senior Health Insurance Informational Program: Assists individuals and families with questions related to paying for medication in Medicare, among many other things
- Information on medications used in dementia, including FTD, from the University of California, San Francisco Memory and Aging Center