The unusual behavior of someone with frontotemporal degeneration (FTD) is not always what it seems. Because people with FTD are often unable to express their needs verbally or accurately, treatable medical conditions and pain are easily missed causing unnecessary distress to the patient and complicating care management.

First back pain, now FTD

Joan Brown is a disabled 63-year-old college professor who suffers from moderate-to advanced frontotemporal degeneration (FTD) with significant problems with behavior and language.

Dr. Brown married and had twins in her late 30s. Lean and active, Dr. Brown ran track and played tennis during high school and college. She developed back pain while carrying her twins but began to run marathons and continued until she developed FTD.

At age 53, she completed menopause and became emotional. Her behavior became more impulsive and she began to “pull away” from her family. Students complained that her behavior towards them was inappropriate. This was attributed to “the change” and estrogen replacement was tried without success. Routine bone density scans revealed osteoporosis. Over the next three years, x-rays done for pain revealed degenerative joint disease and degenerative disk disease in her spine. She sought chiropractic care and acupuncture routinely for pain management.

Dr. Brown’s initial symptoms of FTD were identified at age 56 when she developed agrammatic /non-fluent primary progressive aphasia. She was able to understand written and spoken language but lost the ability to produce words. Early in the disease, she experienced great difficulty lecturing. The first few years after diagnosis, she was able to continue teaching via computer as she was able to type her lectures. At age 58, she could no longer communicate verbally with colleagues and retired from teaching.

Increasing FTD symptoms

At age 61, Dr. Brown became mute. She no longer understood what people were saying to her. She no longer complained of pain, and when asked if she had pain, she shook her head “no.” Believing the pain had resolved the family stopped the pain treatments.

Her behavior was impulsive and she was unable to perform even simple household chores. Her grooming had suffered, and she had frequent urinary and fecal incontinence when in a mall or social situation but refused to wear incontinence garments. She spent her days roaming her neighborhood, occasionally going too far, necessitating the rescue squad for assistance locating her. Her husband confined her to their home where she roamed 12 to 14 hours per day in a precise pattern. She resisted her husband’s help with foot and toenail care. When she stopped sleeping at night at age 62, her family decided to place her in a memory care facility.

On admission to memory care, Dr. Brown continued her roaming with an increasing sense of urgency as days progressed. She began to yell spontaneously and was resistant to care. The staff contacted her doctor for something to calm her down. Ativan was prescribed, but it worsened the agitation and was stopped. The facility asked for an antipsychotic. Both risperdone and quetiapine were tried with minimal success. Citalopram, an antidepressant was also tried and failed to help.

Three weeks ago, Dr. Brown fell while trying to climb out of a window. She was sent to the ER where staff and family questioned her repeatedly about pain. She always responded by shaking her head “no,” and was returned to the facility. Staff reported she was becoming aggressive and resistant to care. Whenever approached by staff towards her right she would strike out. It became impossible to dress her. Her vocalizations increased to screams, especially at night. When no one was near her, she would retreat to bed and moan occasionally.

A sharp CNA’s observation

Staff noted her grimacing during mealtime. Her primary nursing assistant commented that she felt Dr. Brown might be in pain, although her idea was dismissed as she continued to deny it. The staff considered a referral for an inpatient acute care psychiatric admission to manage “her aggressive problem behaviors,” when her CNA spoke up again: “I think Dr. Brown is in pain because she won’t let me touch her right side.” An X-ray of her right arm and shoulder revealed a fracture of her clavicle (collarbone). An orthopedic surgeon ordered a right arm splint to be worn at all times and prescribed one gram of acetaminophen, three times a day. Staff was much more careful not to move or jostle Dr. Brown’s right arm and shoulder when providing care. They were careful to approach her from the left and not use her right shoulder when helping her to move. Gentle heat was provided for comfort.

Dr. Brown stopped vocalizing and once again allowed her CNA to bathe and dress her. The staff began using the PAINAD Scale to evaluate for the presence and intensity of pain. With Dr. Brown’s history of chronic back and joint pain, the providers and care staff decided to treat the pain by preventing it. The acetaminophen was continued indefinitely and the staff monitored carefully for “breakthrough” episodes of pain. A physical therapy consultation was ordered to assist with maintaining joint mobility and for use of modalities such as massage or electric stimulation to minimize pain.

Questions about the case:

How did Dr. Brown non-verbally express pain?

Dr. Brown’s non-verbal expressions of pain included: roaming, resisting care and yelling spontaneously, all with increasing urgency over time. She was more sensitive to touch on her right side, grimacing, striking out when approached from that side, moaning, retreating to bed, screaming and increased agitation.

Why was Dr. Brown’s pain initially not treated?

Dr. Brown was an unreliable source of information for pain evaluation. When asked if she had pain, she answered “no.” Individuals with FTD and other dementias with loss of verbal skills may be unable to understand and respond accurately to questions. They may automatically respond “no” to questions they do not comprehend (as in Dr. Brown’s case). Due to Dr. Brown’s negative responses, the caregivers’ observations of her pain were dismissed.

While behavior changes are recognized as a hallmark of FTD, many of the behaviors seen in a person with moderate to advanced FTD (i.e., refusing dressing or personal care, compulsive walking or altered sleep patterns) could also have a medical cause. Behaviors that might trigger a nursing assessment in someone with Alzheimer’s disease are often attributed to the FTD itself. Dr. Brown’s changes in behavior were attributed to increased agitation and depression. Several medications, including an antipsychotic, were tried with no success.

What effective pain evaluations and treatments were provided for Dr Brown?

The CNA’s observation of Dr. Brown’s reactions (grimaces, sensitivity to touch on the right side) and assertive communication of a suspicion of pain prompted a medical evaluation. Dr. Brown received an X-ray, orthopedic consult and treatment of the fracture that included a splint, routine acetaminophen and gentle heat. Facility staff used specific care techniques, i.e., approach from the left, avoid stress on the right shoulder and gentle movement. They then took a preventive approach to pain using the PAINAD Scale (see Resources section) for ongoing observation of pain, routine acetaminophen and physical therapy techniques.

What history of physical conditions contributed to Dr. Brown’s pain?

Dr. Brown developed back pain while carrying her twins. She was a marathon runner. She has a history of osteoporosis, degenerative joint disease, and degenerative disk disease. Recently she had a fall. Her compulsive walking and poor foot care may have also caused pain for some time prior and exacerbated care management challenges.

How could that information have informed more proactive care?

Her family and caregivers did not understand the importance of Dr. Brown’s history of painful physical conditions once she was diagnosed with FTD. Such awareness may have prompted them to question her denial and look more closely at behavior changes. Had the caregiving staff noted her history of physical symptoms, (chronic back and joint pain) and prior treatment (acupuncture and chiropractic care) they may have recognized the presence of untreated pain earlier. Use of AFTD’s Partners in FTD Care Daily Snapshot Tool is one way to capture critical history from the family prior to placement. Dr. Brown resisted personal care at home, was not sleeping, roamed and was unable to communicate. This information would have provided a baseline for evaluating and managing Dr. Brown’s pain after transition to the facility