Twenty years ago, Jackie and her husband Phil moved to a retirement community near Silicon Valley. In 2007, at age 65, Jackie began experiencing unexplained falls. A major fall late that year alerted Jackie and Phil that they were dealing with a significant issue. Within two years, she began to have trouble speaking. Her words became slurred and her speech was slow and soft, making it difficult to understand. She developed double vision.

Jackie experienced changes in thinking, including loss of executive function. For example, she was no longer able to plan and coordinate family gatherings, and she seemed unusually upset with lively dinners that featured quick-paced conversations. By 2010, she could no longer pay bills, use a computer or follow in-depth conversations. Jackie also developed a wide gait; someone said she “walked like a drunken sailor.”

Despite many appointments with different neurologists and rheumatologists over four years, no diagnosis offered was able to explain these symptoms. Eventually, in 2011, Jackie saw a neuro-ophthalmologist and a movement disorder neurologist. Both specialists diagnosed her with PSP, which explained the variety of neurological symptoms affecting her gait, balance, speech, vision and thinking. While the diagnosis presented bad news, the family was also grateful to know what they were dealing with for the first time.

The movement disorder neurologist explained that most people with PSP develop dysphagia and severe swallowing problems, and that the most common cause of death in PSP relates to aspiration pneumonia or the inability to swallow. He encouraged Jackie to consider whether she wanted a feeding tube – well before the crisis of pneumonia or an inability to swallow developed. Jackie and Phil talked this over many times: Jackie was clear that she did not want a feeding tube. The couple communicated this wish to their children.

Preventing Falls

Shortly after learning the diagnosis, Phil attended a PSP support group meeting in his local area to learn about falls prevention. Falls were Jackie’s first symptom and posed an immediate and ongoing risk in daily activities. Group members accepted that their loved ones’ falls could not be eliminated entirely, and shared strategies to minimize falls and make them as gentle as possible when they did occur. Members recommended early consultations with physical and occupational therapists. While group members did not know of a physical therapist (PT) experienced with PSP, they recommended one with experience in Parkinson’s disease, where falls are also a problem.

The PT became a valued member of Jackie’s care team. She required help from in-home care aides who assisted her with getting up each morning, getting dressed, having breakfast, exercising, bathing and getting dressed again. The PT trained Phil, Jackie and the aides how to use a gait belt to steady Jackie throughout these activities. (A gait belt is an assistive device that is secured around a person’s waist to allow a caregiver to grasp the belt to help in transfers, sitting and standing and walking.) If Jackie did fall, Phil or the aide could use the gait belt to help guide her to the ground.

The PT also showed how to help Jackie get up from the floor after a fall, which was important for everyone’s safety. The aides allowed Jackie to rest on the floor for as long as necessary, with a pillow and possibly a blanket for comfort. Then, when she felt able, they helped her crawl to a sofa or sturdy chair. They instructed Jackie to get up on one knee, and then, with Phil or the aide using the gait belt, provided her with gentle assistance to stand up. In this way, she was helped onto the sofa or chair to rest.

The occupational therapist (OT) evaluated Phil and Jackie’s home with an eye to minimizing falls. Throw rugs were removed; a bed handle was installed to help her get in and out of bed, and grab bars were placed in the bathroom. The OT suggested the right kind of shower bench and toilet seat riser.

Like many people living with PSP, Jackie was impulsive. She would get up from a chair and start walking without her walker. Due to cognitive changes, it was not possible to educate Jackie about her own safety and have her remember to use her walker at all times. The OT suggested using a chair alarm and bed alarm so that at least someone nearby would know that Jackie was on the move.

The Importance of Exercise

The movement disorder neurologist who diagnosed Jackie told her that exercise is the only treatment that seems to slow the progression of PSP. Jackie started with a treadmill that was already in the home. Modifications were made to her exercise regimen due to the extreme risk of falls, and an “emergency off” cord on the treadmill saved Jackie from rubber burns if she fell. Later, she transitioned to a recumbent bike and did other seated exercises. The home care aides assisted Jackie with exercise as well as activities of daily living throughout her time at home.

In 2012, one year after diagnosis, Jackie had another PT evaluation and learned that it would be safer if she used a walker. The PT recommended a walker designed for people with Parkinson’s disease. These walkers have larger, more durable wheels than the standard aluminum walker, and offer braking systems and seats so the user can rest. Phil and the home care aides continued to use the gait belt when Jackie walked with a walker. The PT also offered training on how to closely escort someone who walks with a walker.

Jackie’s vision problems, including downgaze palsy and double vision, meant that walking was even more challenging because she could not see the floor. The neuro-ophthalmologist had tried prism glasses, lenses to help keep the eyes working together and aligned, to see if that would help Jackie, but they were of no benefit.

Dysphagia

Swallowing problems often begin four to six years into PSP. In 2012, Jackie began having mild dysphagia, which started with periodic coughing during and after meals. This was more of a problem at dinner, when Jackie was more fatigued. Phil and the care aides turned off the TV and eliminated other distractions during meals. Phil made sure the care aides knew how to use the Heimlich maneuver in case of choking. Jackie also began drooling, which indicated infrequent swallowing, rather than excess saliva. Also, left on her own, Jackie sometimes shoveled food, so only a certain amount could be put in front of her at any given time.

Jackie’s neurologist ordered an evaluation with a speechlanguage pathologist (SLP). She gave Jackie exercises to do at home that would maintain strength in the swallowing muscles. Perhaps due to apathy (a symptom occasionally seen in PSP), Jackie wasn’t interested in doing these exercises daily. Jackie and Phil agreed to focus on physical exercise.

Transition to Assisted Living

In 2013, Phil was diagnosed with a heart condition that caused fatigue and weakness. He became unable to assist in Jackie’s care at home. At the encouragement of their children, they decided to move into an assisted living facility together. The couple lived in the same one-bedroom apartment. Phil needed no care, while Jackie needed a moderate-level of care initially, to assist her with dressing and bathing. Eventually, Jackie became bladder incontinent and needed assistance with toileting. Phil partnered with management at the facility to help them learn about PSP, develop a plan of care and arrange training in how to assist Jackie.

Assisted living facility staff were greatly concerned about Jackie’s falls. Phil educated them that falls were a part of PSP and a risk that he and Jackie accepted to help her remain as active as possible. The caregivers were unfamiliar with using a gait belt or how to help someone get up from the floor after a fall. With Phil’s support, the facility arranged for Jackie’s PT to demonstrate these techniques during two staff meetings. Use of the new skills by the caregivers was reinforced by the administration and Phil. Staff recorded the number of falls and the severity, to report to Jackie’s neurologist.

Jackie needed more rigorous leg exercise than was provided through the seated exercise class at the assisted living facility. While the facility had equipment with a recumbent seat that worked both arms and legs simultaneously, the caregivers could not consistently help Jackie with her exercise. Phil hired a fitness trainer and private-duty aides to assist with daily exercise.

When Jackie and Phil moved into the assisted living facility, Jackie was coughing with every meal. Fortunately the care staff found another couple willing to share a table with Jackie and Phil. The wife had Alzheimer’s disease and was largely unaware of the noise accompanying mealtime. The facility administration ensured that the kitchen and dining staff were familiar with the Heimlich maneuver.

Jackie’s neurologist ordered a modified barium swallow study, which was done by the same speech/language pathologist Jackie had seen earlier. Phil was allowed to be in the exam room and could see evidence of “silent aspiration” when liquids went down the air pipe and Jackie didn’t cough. The SLP demonstrated that liquids thickened to the consistency of honey could be handled more safely. She showed that encouraging two swallows per bolus of food (a double swallow) was of great benefit. While additional strategies were tried, neither tucking the chin to the chest during the swallow (chin tuck) nor using a straw helped Jackie.

Phil and Jackie tried various liquids, pre-thickened or with thickening agents. Jackie preferred liquids thickened with SimplyThick®, especially if they were chilled. By 2014, meals were taking two hours each. Phil hired additional private-duty aides to provide assistance, as the care facility staff could not devote such time to one resident. Also, Jackie lost the ability to hold eating utensils. The assisted living staff was not permitted to provide hand-feeding, but privately-hired aides could. The administration and Phil worked together to ensure careful coordination among the entire care team.

In early 2015, seemingly overnight, Jackie lost the ability to swallow. By this time, she was largely mute. While it was impossible to have a traditional conversation with her, she knew who she was, who Phil was, and what was going on. Phil asked if she wanted to hold firm on her decision to not use a feeding tube. He asked her a series of yes or no questions, for which he asked her to hold up one finger for yes and two fingers for no. Phil learned that Jackie had not changed her mind. Hospice was contacted and provided wonderful assistance. Within about two weeks, Jackie died peacefully, surrounded by Phil and her children.

Another decision Jackie had made early on was to donate her brain upon death. Phil carried through with these wishes. Within a few months of Jackie’s death, Phil received a report confirming the PSP diagnosis.

Questions for Discussion
(Use for staff in-service training or in resident-specific situations.)

The neurologist who diagnosed Jackie encouraged her to consider if she would want a feeding tube. Why?

Nearly everyone living with PSP will experience swallowing problems. The most common causes of death are related to loss of swallowing ability – either aspiration pneumonia due to severe dysphagia or total loss of the ability to swallow. Making and communicating a decision about a feeding tube and other advanced health care practices is difficult in a crisis situation. Early discussion and documentation of advanced health care preferences and power-of-attorney are especially important for any neurodegenerative disorder in which cognitive capacity is expected to diminish over time.

What strategies did the physical and occupational therapists offer to reduce Jackie’s risk of falling?

The physical therapist evaluated Jackie for mobility assistance devices and recommended the gait belt and walker. The PT provided the family and caregivers specific instruction on how to escort Jackie when walking and how to help her get up off the floor safely and comfortably after a fall. Having knowledge of balance problems was important for the PT to be able to offer an exercise regimen that suited Jackie’s interest and level of ability. The occupational therapist provided a safety evaluation of the home environment, with special attention to the bathroom, a room with multiple risks for people with PSP. Throw rugs were removed, a bed handle was installed and grab bars were placed in the bathroom. The OT suggested the right kind of shower bench and toilet seat riser.

What strategies did the speech-language pathologist (SLP) offer to reduce the risk of aspiration pneumonia and facilitate swallowing?

The SLP evaluated Jackie’s voice, speech and ability to swallow while she was living at home. She offered Jackie exercises to maintain strength in the swallowing muscles. When Jackie transitioned to assisted living, the SLP conducted a modified barium swallow study to evaluate her ability to swallow various thicknesses of liquid, with and without a straw, with and without a chin tuck and with a double-swallow after each bite or drink. Using thickened liquids and encouraging a double swallow helped Jackie.