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The early age of onset of many frontotemporal degeneration (FTD) spectrum disorders and the changes in behavior and language that can accompany them can make living with FTD a challenge for both caregivers and patients. Patients with FTD can be otherwise young and physically healthy, making behavioral management even harder. Although most people are sensitive to the memory struggles that accompany a diagnosis of Alzheimer’s disease, the communication difficulties and unusual behaviors found in many forms of FTD can leave caregivers and patients feeling socially isolated and misunderstood. In this section, we offer suggestions for symptom management as well as resources that will improve coping.
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Common Medications & Treatments Although research is constantly advancing, there are currently no disease-modifying or curative treatments available for FTD. However, medications are often prescribed to help manage symptoms such as changes in behavior, mood, or motor abilities. All medications can have potential side effects or adverse effects depending on the dose and an individual’s tolerance, so we recommend “starting low and going slow,” meaning we very gradually increase most medications’ dosage to find the lowest dose that provides an effective benefit. Some medications may be beneficial for certain individuals, while others may worsen on the same treatment, so it is critical to work closely with your care team to understand specific risks of each drug and monitor an individual’s response over time. The prescribing provider will also work with you to assess for any interactions between drugs, potential allergic responses or increased risks of adverse effects based on your medical history. The categories below represent a sampling of available medications for symptomatic treatment applicable in FTD, but the inclusion of a medication on the list does not imply an endorsement of its use for all individuals. ANTIDEPRESSANTS: Antidepressant medications are used to treat depression, anxiety, irritability and ritualistic or compulsive behavior, commonly seen in behavioral variant FTD. A subset of antidepressant medications called Selective Serotonin Reuptake Inhibitors (SSRIs) work by increasing the level of a neurotransmitter, serotonin, in the brain. These medications, such as sertraline (Zoloft), are generally safe and well tolerated in older adults, and can be beneficial for both mood and disinhibited behavior. A different medication called Trazodone, works similarly by modifying serotonin levels, but often can cause more sedation or drowsiness, than SSRIs. Due to this, trazodone is often prescribed for sleep disruptions or significant agitation. Atypical antidepressants, buproprion (Wellbutrin) and venlafaxine (Effexor) are known to have a more “activating” effect and therefore can be helpful in cases of significant apathy or loss of motivation. However, these medications may also increase an individual’s risk of agitation and lower the seizure threshold, so they should be used with caution in appropriate patients. NEUROLEPTICS: FTD and related disorders have the potential to alter how an individual interprets the world around them, which can result in seeing things that are not present or believing things that are not based in reality. If a person is unable to understand their environment or respond appropriately, there can be increased risk of physical agitation or aggressive behavior. Neuroleptic medications, also known as atypical antipsychotics, are prescribed primarily to treat psychiatric symptoms, such as hallucinations, delusions or agitation that poses a safety risk. Second-generation atypical antipsychotics include quetiapine (Seroquel), risperidone (Risperdal) and olanzapine (Zyprexa). These medications work by blocking receptors of neurotransmitters dopamine and serotonin to reduce severity of agitation and psychosis. Although second-generation atypical antipsychotics are generally well-tolerated, there is increased risk of potentially fatal stroke or death in elderly patients with dementia, and these medications can cause sedation or motor side effects, so they are generally reserved for only after all other strategies have proven to be insufficient. ANTICONVULSANTS: Anticonvulsant medications are traditionally prescribed to reduce epileptic activity or seizures, but they can also act to stabilize mood and behavior. Some of these medications may be used alone or in conjunction with an antipsychotic medication to help manage agitation in unsafe situations. Frequent laboratory tests are required to monitor levels of the drug in the blood to assure the concentration is within the therapeutic range. DOPAMINERGIC AGENTS: Dopaminergic agents such as carbidopa-levodopa (Sinemet) are first-line treatments for motor symptoms experienced with Parkinson’s disease. Although the efficacy is often not as significant in other disorders such as FTD, these medications in small doses may help reduce symptoms such as tremor, stiffness and bradykinesia, or slowed movement. Common side effects include nausea and dizziness, but these medications may also worsen impulsivity or hallucinations, and therefore must be used with caution in FTD and Lewy body disease. Acetylcholinesterase inhibitors: Acetylcholinesterase inhibitors such as donepezil (Aricept), rivastigmine (Exelon) and galantamine (Razadyne) have been approved by the FDA to treat mild to moderate Alzheimer’s Disease. Cholinesterase Inhibitors are designed to increase the level of acetylcholine in the brain. Acetylcholine is a neurotransmitter involved in brain cell communication, important for memory, judgment, and attention. This class of medications may potentially help with cognitive symptoms in logopenic primary progressive aphasia or Corticobasal syndrome, as these diagnoses can have underlying Alzheimer’s disease pathology, but they may worsen symptoms seen in other subtypes of FTD. Research also suggests that cholinesterase inhibitors are effective in treating neuropsychiatric and cognitive symptoms in Lewy Body disease. OTHER: Namenda (Memantine) is approved for use with vascular forms of dementia and moderate to severe Alzheimer's disease. While clinical reports suggest that this medication may be helpful in some FTD patients, a recent double-blind placebo-controlled trial failed to demonstrate any efficacy for this medication.
Non-Medical Interventions for FTD Caring for a person living with FTD can be very challenging- individuals with FTD can experience language and processing difficulties as well as distressing personality and behavior changes. Non-medication interventions are crucial in helping an individual with FTD maintain independence for as long as possible and manage difficult social situations. Non-medical interventions refer to an approach of managing behaviors that encourages adjustment and control of the environment, rather than the administration of medications. For example, if an environmental trigger is causing behavioral distress, we work to remove the trigger to improve agitation. Non-medical interventions can be used alone or in conjunction with medical therapies for symptom management. Non- pharmacological approaches are the first line of therapy in mild stages of dementia. Creating a consistent, engaging routine of social, physical and cognitive activity can be just as important, if not more so, in managing symptoms of FTD.
Self-Care SUPPORT Caring for a person who has been diagnosed with FTD can be physically exhausting as well as emotionally stressful because of the extreme personality changes and language difficulties that can accompany these disorders. Building a support system is necessary for caregivers to enhance their coping strategies. Talking to other caregivers can be helpful in terms of sharing experiences, identifying resources, and problem solving. Having the opportunity to speak with others living with a similar experience can also be therapeutic. Caregiver support groups are a good source of education about FTD spectrum disorders and vital social connection. There are several options for support groups including face-to-face, online and telephone support groups. For a list of support groups visit The Association for Frontotemporal Degeneration's (AFTD) support page. Caring for a loved one with FTD may be different than caring for one with Alzheimer’s disease (AD) or other dementias. While memory loss is the primary feature of AD, caregivers of those with FTD often have to cope with unpredictable behavior, lack of empathy, lack of available resources and may have to educate the friends and family who are unfamiliar with FTD. Depression, anxiety, social isolation, fatigue, irritability and grief are signs of caregiver stress. It is important to pay attention to these feelings and seek necessary treatment. Calling on the help of others can be difficult for caregivers. Often, caregivers are hesitant to ask for help. Getting support from others will be invaluable. Create a circle of support or a list of friends, family and colleagues who you can call on for help. Start by asking small requests. Reaching out will be difficult at first, but the more you ask, the easier it will become. RESPITE Caring for someone who has frontotemporal degeneration can be physically and emotionally demanding. Respite care is the short-term provision of care that is designed to give relief to caregivers. This can be done in the home, daycare facility or residential facility setting. Respite care may be done by volunteers or paid health aides and other trained staff. Many caregivers use informal respite such as help from family or friends. Others seek out professional respite services offered by nursing organizations, such as short-term stays in nursing homes or assisted living facilities.
Driving and FTD Driving is a complex, highly demanding activity. The act of driving requires a person to simultaneously perform multiple tasks of high cognitive demand. A person must be able to interpret the driving environment, anticipate the actions of other drivers on the road, make an immediate, rapid decision and physically act accordingly in a safe manner. This requires memory, judgment, attention, visuospatial navigation, executive function and a precise motor response, all of which can be significantly impacted by FTD. FTD is a significant risk factor for dangerous driving. Individuals with frontotemporal degeneration may present with progressive disinhibition, slowed processing and therefore slowed reaction times, difficulty with navigation and reduced judgment. These symptoms unfortunately drastically increase a person’s risk of endangering themselves or others while they are on the road. Clinicians in Pennsylvania are considered mandated reporters, meaning they are legally required to report potentially unsafe drivers due to cognitive or medical conditions, to the Department of Motor Vehicles. Importantly, if a person with a documented diagnosis that affects cognitive ability is found to be guilty in a motor vehicle accident, insurance can potentially deny coverage of damages, which may result in significant liability of the driver with FTD or a related disorder. If it is unclear if someone is able to drive safely, your neurologist may recommend a formal driving evaluation. This is often performed by a driving specialist or occupational therapist, and generally includes both a neuropsychological assessment and a behind-the-wheel test. This provides a comprehensive, in-depth analysis of a person’s ability to perform the functions required for safe driving at this stage of their disease. Techniques for reducing and eliminating driving. Driving is often a symbol of independence, so the decision to stop driving or remove your loved one’s access to driving can be very difficult. Although an FTD diagnosis does not necessarily mean the individual should immediately cease driving, we recommend discussing this sensitive topic as early as possible in the disease course to provide sufficient time to adjust to this change and implement an alternative transportation plan. Efforts should be made to include your loved one in this conversation and to allow them to voice their concerns and priorities, which can increase the likelihood of adherence to these changes. Public transportation is often ideal for individuals with mild cognitive symptoms, but may be too complex to safely navigate as symptoms progress, especially for individuals with difficulty with executive function, navigation or memory. Alternative modes of transportation include coordination of rides from friends or family, community-based senior or disability transit programs, ride-sharing services that can be tracked and scheduled ahead of time such as Uber or Lyft, or medical paratransit options for eligible patients. It is important to work with your care team to navigate this significant change; our goal is always to prioritize the safety of the individual with FTD and the community, while maintaining as much independence, quality of life and autonomy as possible.
Power of Attorney, Guardianship, and Legally Authorized Representative Impaired Decision-Making Ability To make a rational, sound decision, a person must be able to comprehend information, retain this information, organize their thoughts to come to a reasonable conclusion that is in their best interest, and then communicate this decision to others. FTD disorders can affect a person’s reasoning, insight, judgment, language and ability to comprehend information. If any of these cognitive functions are negatively impacted, decision making can be impaired. As FTD progresses, we anticipate increased difficulty with complex decision making, especially as it pertains to medical, financial, and legal matters. Individuals with FTD are often more vulnerable to financial exploitation or may place themselves or others in danger due to reduced insight or poor decision making. Therefore, as early as possible in a person’s FTD journey, we recommend discussing who will make decisions on behalf of the individual if or when they are no longer able to do so. Power of Attorney What is Power of Attorney? Power of Attorney (POA) is a legal document that authorizes a person trusted by the individual with FTD to make decisions for them and act on their behalf. A POA can be a single person assigned to make all decisions on behalf of an individual, or can be divided between a Durable Financial POA, who would oversee financial affairs such as bank accounts, paying bills or directing investments, and a Durable Healthcare POA, who would consent for medical decisions, including end-of-life healthcare, for the person with FTD. A POA can be changed or revoked at any time as long as it is determined the individual has the capacity to do so. “Durable” implies this legal authorization remains in place even if a person is mentally or physically incapacitated due to illness. How do I choose a Power of Attorney (POA)? A power of attorney can be a spouse, family member or friend who is trusted to understand the wishes of the person with FTD and act accordingly on their behalf. This decision can be complex, so it is recommended to create and sign these documents with an experienced elder care attorney, who can assure all requirements are met from a legal perspective. The timing of these conversations is critical; in order to establish POA, a person must have the capacity to do so, meaning they must understand the nature and weight of assigning a POA, and must be able to effectively communicate their wishes. Your neurologist can help determine if your loved one is capable of assigning a POA. We strongly recommend having these conversations as early as possible to best protect your loved one and allow them to meaningfully contribute to the discussion. Our goal is always to maintain autonomy and respect the person with FTD’s preferences as much as possible while maintaining physical safety and financial security. Guardianship What is Guardianship? If an individual has not designated a power of attorney and no longer has the capacity to do so, or if a person with FTD refuses to assign POA but due to their disease is making impulsive or unsafe decisions with poor judgment, it may become necessary to ask the court to appoint a guardian to manage their affairs. Guardians are given the legal authority to care for the personal and property interests of another person after they are deemed mentally incompetent or lacking the ability to care for themselves and make sound decisions. The following criteria generally must be met for a court to appoint a guardian: -The individual is not able to make decisions regarding his or her own affairs. -The individual has not executed or does not have the mental capacity to execute, a power of attorney. -Serious harm may come to the person if a guardian is not appointed. Types of Guardianship Courts can grant guardianship of the person, guardianship of the person’s estate or property or combined guardianship for both. A guardian who has been given responsibility by the court for both the personal well-being and financial interests of the ward is known as a general or plenary guardian. The court can choose to appoint one individual as guardian of the person and a different individual as the limited guardian of the person’s estate. As a guardian, you accept the legal responsibility to protect the individual and their assets. How do I obtain Guardianship? If you believe that your loved one needs a guardian to prevent serious harm to their person or their estate, you will need to hire an experienced attorney and petition the court for guardianship. Guardianship takes significant control away from an individual and therefore should only be pursued after all other avenues have been exhausted. Establishing guardianship requires medical evaluations, extensive legal documentation of risks or behaviors, and a court hearing, all of which can unfortunately be very time consuming and expensive for families, and the process can take multiple months. However, if guardianship is necessary, it is again recommended to initiate the process as early as possible to proactively protect your loved one, both physically and financially, rather than respond after a crisis when the situation can be both more stressful and expensive. Legally Authorized Representative What is a Legally Authorized Representative (LAR)? A legally authorized representative is defined as “any individual person, judicial body or other body of individuals who are legally authorized under state and federal law to consent to research participation on behalf of a designated person.” A legally authorized representative is necessary when an adult is unable to make or communicate an informed decision due to mental or physical impairment. This authorization applies to both medical decisions within a person’s clinical care and decisions to participate in elective research studies. If already in place, a POA or guardian can act as a legally authorized representative. If neither are yet established, an LAR is often next of kin, such as a spouse or adult child. Getting Started It is always best if you and your loved one consult with a lawyer who has experience in drafting powers of attorney. Resources for finding a lawyer in your area: National Academy of Elder Law Attorneys, Inc. https://www.naela.org// Pennsylvania Bar Association https://www.pabar.org/site/