Klug, Jessica R. RN
FRONTOTEMPORAL DEMENTIA (FTD) is a progressive and incurable form of dementia that typically affects men and women about equally in their late 40s and 50s.1 The behavioral variant of FTD was previously known as Pick disease, named for Arnold Pick. He was a physician who, in 1892, discovered abnormally excessive clusters of the protein tau in two lobes of the cerebral cortex in patients who were considered too young for dementia.2,3 Two other types of FTD, which are characterized by progressive language or motor decline, are beyond the scope of this article.
Little is understood about this disorder, which is characterized by focal frontal and/or temporal lobe degeneration. Brain cells that make up these lobes are damaged, leading to atrophy, disruption of cell function, and eventually cell death.2
Signs and symptoms
Obtaining a comprehensive health history is important because up to 40% of those with FTD have a significant family history of this disease, suggesting genetic transmission.4 Because of the lobes of the brain affected, the patient's signs and symptoms often help clinicians recognize FTD. The brain's frontal lobe is responsible for personality, behavioral responses, planning, judgment, and decision making;the temporal lobe is responsible for emotional responses and language skills, such as speaking and interpreting.2,5 Signs and symptoms of FTD reflect disruption of these functions. (See Focusing on two lobes.)
The first noticeable sign of FTD is usually a progressive change in personality, including abrupt and frequent mood changes.2,5 Patients lose insight into their behaviors and lose control of emotions and social and sexual inhibitions. These patients may display a lack of empathy for other people as they lose their judgment and the ability to think logically.4 Families of affected patients describe unusual behaviors, including yelling profanities in public, overeating, and neglecting personal hygiene.5,6
Unlike those with Alzheimer disease, patients with FTD don't have lapses in memory. This is a clinical finding that helps the healthcare provider make a diagnosis.2 Additional clinical manifestations may include being unable to perform a task without becoming distracted and having trouble with activities of daily living requiring complex skills, such as managing medications. As the disease progresses, the signs and symptoms become more noticeable.5,6
Diagnosis and prognosis
Once the signs and symptoms are apparent to family members, the disease may have already progressed significantly. During this time, magnetic resonance imaging of the brain may demonstrate atrophy and small lesions in the frontal and temporal lobes, key signs of this disease.2 A healthcare provider may also perform diagnostic studies to rule out other disorders that present in a similar manner as FTD. A brain autopsy is required for a definitive diagnosis.
Meanwhile, the disease progresses steadily and sometimes rapidly. The patient may live with the disease for less than 2 years to over 10 years.6
What can nurses do to support the patients, families, and caregivers? By taking a step back and understanding the impact that FTD has on patients and loved ones, nurses can help ease the necessary but painful lifestyle transition.
When diagnosed with FTD, most patients aren't aware of the ramifications and the devastation felt by their family. They may continue as if nothing is happening due to the difficulties of processing language, among other physiological reasons.2 But for the patient's family, the diagnosis may be a shock. Once family members realize that time left with their loved one as they knew him or her may be limited, they may experience a whirlwind of emotions.
“It is the longest, most painful journey imaginable to watch your vibrant loved one gradually decline, becoming someone you can hardly recognize, and then to become dependent and die within just a few years,” explains one patient's spouse.7 Because most patients affected are in their late 40s and 50s, many are still working, raising children, and sometimes even caring for their own aging parents.8 Now spouses who are also parents must take on the role of a single parent. Patients are physically present, but over a short period, they change emotionally and cognitively and can no longer meet family needs and expectations.
For most caregivers, losing their loved one to FTD while trying to deal with behavioral changes can be the most difficult part of the process.9 Nurses can help by providing support, active listening, and education about the disease with clear verbal and written instructions; helping them find financial support; and referring them to respite care resources, such as adult daycare and community support groups.5,7
Educate families about the signs and symptoms of burnout such as depression, lack of sleep, and emotional outbursts so they can maintain their own health.10 Families and caregivers should make sure they get enough sleep instead of relying on unhealthy behaviors such as smoking or excessive use of alcohol or caffeine.10,11 Setting realistic goals and following a flexible daily routine with the patient eases the process for everyone involved.11
Monitor the patient for any signs of abuse by stressed caregivers and contact adult protective services as needed, following facility policy.8 Because patients with FTD are often unaware of their actions, they may become verbally or physically abusive to their caregivers; caregivers who are experiencing burnout may reciprocate the abuse. To minimize the risk of an altercation, caregivers should avoid arguing with the patient and consider changing the subject to ensure both caregivers' and patients' safety.12
Providing care to patients
No FDA-approved medications can slow the progression of FTD. Fortunately, research is being conducted, with a potential for clinical trials in the future.4–6
Antidepressants and antipsychotics are frequently used to help treat behavioral signs and symptoms.2 Helpful nonpharmacologic methods to control behavior include avoiding confrontation, redirecting the patient's attention, employing behavioral modification techniques, creating a calm environment, and helping the patient maintain a regular sleep pattern.1,9
Nursing interventions focus on comfort care and interventions to keep patients and families safe. Patients with FTD lose the ability to make sound judgments and may be easily distracted, creating an unsafe environment.9 Modifying the home environment and increasing supervision can help improve safety. In the home, falls become a major risk for patients with FTD. Advise caregivers to make sure all excess clutter is put away, pathways are kept clear, carpeting and flooring are secured, and lighting is adequate.13 Other interventions can include physical, occupational, and speech therapy and an exercise program.1 Although interventions won't prolong the patient's life, they can provide a better quality of life during this time. (See For more information about FTD.)
Although scientists haven't yet found a cure or treatment to slow the progression of this devastating disease, nurses can provide optimal care for their patients and families. Early recognition of signs and symptoms gives nurses the chance to educate those affected and provide support that may help patients function as well as possible. When nurses support patients and their families, they can enhance the quality of life for all involved.
For more information about FTD
- National Institute of Neurological Disorders and Stroke. Frontotemporal Dementia Information Page. 2014. http://www.ninds.nih.gov/disorders/picks/picks.htm.
- Association for Frontotemporal Degeneration. http://www.theaftd.org.
- National Institute on Aging. Frontotemporal Disorders: Information for Patients, Families, and Caregivers. 2014. http://www.nia.nih.gov/alzheimers/publication/frontotemporal-disorders/types-frontotemporal-disorders.
4. Rabinovici GD, Miller BL. Frontotemporal lobar degeneration: epidemiology, pathophysiology, diagnosis and management. CNS Drugs. 2010; 24:(5):375–398.
7. McFarland DM. The journey through Pick's Disease with a loved one: a personal account. Int Nurs Rev. 2010; 57. (1):142–144.
8. Chow TW, Pio FJ, Rockwood K. An international needs assessment of caregivers for frontotemporal dementia. Can J Neurol Sci. 2011; 38:(5):753–757.
12. Alexander M, Larson EB. Patient information: Dementia (including Alzheimer's disease)(Beyond the basics). 2014. http://www.uptodate.com