Kerry Sheltra (left), assistant technology professional with Numotion, and Gretchen Relva, PT, help Susan, a patient with ALS, with a wheelchair fitting in the Neuromuscular Center at the Hospital for Special Care.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. For people with ALS, also referred to as Lou Gehrig's disease, motor neurons will eventually die and eradicate the brain's ability to initiate and control muscle movement.
Despite having no known cure or effective treatment to halt disease progression, a multidisciplinary care approach and the use of adaptive equipment and technological advancements can help persons with ALS (PALS) manage symptoms, maintain as much independence as possible and prolong survival.
"We recognize that this disease impacts all aspects of a person's life: physical functioning, spiritual life, work and family," said Susan Walsh, RN, MSN, ACNS-BC. "These patients require the best that healthcare offers: a multidisciplinary approach that addresses all aspects of an individual and their family and connects them with community resources such as home care and hospice. We provide resources during every phase of the illness as needs change."
Walsh is the regional nurse coordinator for the Greater Philadelphia Chapter of the ALS Association and program coordinator at Penn State Hershey Medical Center's ALS Clinic, an ALS Association Certified Clinic. The ALS Association Greater Philadelphia Chapter serves Central and Eastern Pennsylvania, Central and Southern New Jersey, and Delaware.
The Hershey Medical Center ALS Clinic exists because of the Greater Philadelphia Chapter, Walsh told ADVANCE. Many of the clinic's services, such as social work, are non-billable but are integral parts of the team approach. "Because of the support of the ALS Association, the certified clinics have a unique opportunity to provide these patients with the best possible care," Walsh explained.
The diagnosis is the first big hurdle patients and their families need to overcome. At Hershey Medical Center, Walsh educates patients on the individual nature of the disease and its progression-no two patients will progress at the same time or with the same symptoms. "We address patient's concerns about the diagnosis on day one because people hear 'ALS' and respond by imagining the worst-case scenario," Walsh said.
ALS clinics require their patients to visit every three months for a multidisciplinary appointment to monitor disease progression and manage changes. "We recognize that this disease includes everyone so we invite families to come with the patients-and we have designed our program around that notion," Walsh said.
The multidisciplinary team generally includes physicians, nurses, occupational therapists, physical therapists, speech-language pathologists, respiratory therapists, social workers, dieticians and pastoral caregivers. At the 3-month visit, patients take a breathing test to monitor breathing function, and are evaluated by each team member.
Jennifer Klapper, RN, MSN, CNS-BC, helps patients anticipate their healthcare needs and discuss what will work for them. "It's a delicate topic so I approach it with sensitivity and care," said Klapper, a mental health nurse with the ALS Association, Greater Philadelphia Chapter and the ALS Center at the Penn Medicine Neuroscience Center at Pennsylvania Hospital in Philadelphia.
Klapper asks patients to define their idea of optimal health moving forward and helps them articulate what is important to them as individuals. "I ask if they are looking at longevity or quality because everyone is different," she shared. "It's important to explore individual approaches, but also the background of multi-generational family and culturally-based expectations for how to behave when a loved one is ill."
According to Wendy Barnes, LSW, a social worker with the ALS Association, Greater Philadelphia Chapter, a multidisciplinary team approach provides an opportunity for patients to obtain help from more than one type of professional. "The patients have medical and physical needs, but they also have social and emotional needs," she said. "Our team gets to know the whole person and the family, allowing us to address multiple issues and concerns immediately as time is a huge factor with ALS."
The Neuromuscular Center at the Hospital for Special Care in New Britain, Conn., was established in 2007 and treats more than 1200 patients with chronic neuromuscular diseases annually. PALS are evaluated by eight healthcare providers at HSC, including physicians, respiratory therapists, nurses, OTs, PTs, SLPS, dieticians and social workers.
"We use a team approach to care with one-stop shopping for appointments," said Kevin J. Felice, DO, chief of the Neuromuscular Center and director of the ALS Clinic. "This approach has been proven through research to improve quality of care for patients until we find a cure for this devastating disease."
There are currently about 250 PALS living in Connecticut and Hospital for Special Care treats about 220 of those cases. The clinic is just one of seven in the country to be dually certified by the ALS Association and the Muscular Dystrophy Association.
"These patients need a knowledgeable group of people to help them with their healthcare and home care needs and to navigate our challenging healthcare insurance system," Felice said. "It's a difficult ordeal for patients."
Zahira Paul, MS-OTR/L, staff level II therapist, ALS Association, Greater Philadelphia Chapter, has been a member of the multidisciplinary team at the Penn State Hershey Medical Center's ALS Center for 8 years. "When our patients come for their checkup, the visit is exhausting because they fatigue easily," she said. "We do what we can to streamline the appointment."
One approach is for OT and PT to schedule their time together, Paul shared. "Our evaluations and treatment plans are related so it just makes sense," she said. "Before the appointment, we get background from the nurses because they are more involved in the day-to-day function such as feeding, bathing and dressing, mobility issues and whether or not the patient is having falls."
Zahira Paul, MS-OTR/L, measures patient Samuel Daub's hand and grip strength in the ALS Center at Penn State Hershey Medical Center.
Physical and occupational therapists focus primarily on safety and energy conservation techniques with mobility and activities of daily living, while speech-language pathologists address speaking, swallowing, chewing and proper positioning for breathing.
"I educate my patients about ALS to prepare them for what they can expect but I don't want to overwhelm them," said Kamille Sprenkle PT, DPT, ACCI, ALS Association, Greater Philadelphia Chapter, and ALS Center at Lehigh Valley Health Network in Allentown, Penn. "Education is powerful and helps patients identify things before they become too big to handle."
PTs assess PALS for posture, mobility, range of motion and breathing techniques, Sprenkle explained. "I may recommend a lift chair, shower chair or raised toilet seat to prevent expending too much energy," she said.
The patient's goal, according to Sprenkle, is usually to maintain independence for as long as possible. "As they get weaker, we need to educate on prevention of falls," she said. "We need to educate family members on safe transfer techniques. We don't want them to fall because their arms cannot support them in a fall."
Paul focuses on upper extremity function and looks at the patient's daily schedule to see what activities can be accomplished more easily. Her primary goals are energy conservation, safety, and helping patients do what they enjoy most.
According to Sprenkle, patients can still do the things they once loved, just differently. "If a patient used to love to run, I may suggest walking instead, with frequent breaks to sit and rest," she said. "We frame things in a new way so they can still enjoy their pastimes without compromising safety."
Paul prefers to counsel her patients on accepting help when they are fatiguing too much rather than providing a lot of adaptive equipment. She acknowledges that it's difficult to ask for and accept assistance but helps PALS save energy for more important activities. "If you want to go to your grandson's baseball game, then you don't want to exhaust yourself dressing and bathing," Paul tells patients. "Maybe your wife can put your shoes and socks on for you so you can conserve energy."
In treating PALS, OTs need to think about activities of daily living in a different way. "As OTs, we try to strengthen and improve our patients' function but we can't with these patients," Paul said. "They get weaker and more tired as time goes on. Even when a patient is able to stand to shower, we suggest they rest on a bath seat to conserve energy for an activity later in the day."
"My primary role in the clinic is to identify any immediate services my patients may need and to prevent crises down the line," said Gretchen Relva, PT, neuromuscular PT, Neuromuscular Center at Hospital for Special Care. "Safety is always my top priority."
For patients needing adaptive equipment, the clinicians at HSC have access to the ALS and MD Associations' loan closets to get equipment, including canes, long-handed sponges and other handheld adaptive devices, quickly while negotiating insurance coverage. PALS can trial equipment and then purchase elsewhere or take home to use for a short time.
Relva offers fittings for custom wheelchairs. "I try to match my patients with equipment that will progress with them, such as chairs that accommodate head control and micro light switches, so the patient can be as independent as possible throughout the disease course," Relva said.
If within travel distance, the patients may visit the clinic for assistance in procuring braces or to receive training for new assistive devices. Relva serves as a liaison to the community for patients who are unable to return to the clinic. She connects PALS with home care resources, and is available if another therapist needs to call with questions or to troubleshoot issues.
As the disease progresses, the patient's muscles of the mouth, throat, lips, and tongue become weaker, causing increasing difficulty swallowing and speaking, explained Merisa Palovcak Allen, MS, CCC-SLP/L, ALS Association, Greater Philadelphia Chapter, and Penn Medicine's Pennsylvania Hospital ALS Center.
"I tell my patients, the muscles you eat with are the same muscles you talk with," Allen said. "The more they work those muscles throughout the day, the more fatigued they will become."
Allen tests strength, sensation, and range of motion of muscles needed for speech and swallowing. "I incorporate food modifications if they are having fatigue from chewing," she said. "We can modify the foods they eat to chew more easily. I may thicken their liquids, change the cup or straw they drink with, or change head positioning when eating and/or drinking."
Eating is a social activity, Allen explained. "When eating becomes a chore or when a patient is eating just to maintain weight, it may be time to augment with a feeding tube," she said. "People are scared of feeding tubes so I talk to patients and families about what is involved and how it can help."
Allen helps patients understand the positives of augmenting with a feeding tube. "It's meant to help and ease frustration for these patients," she explained. "Patients can still eat with a feeding tube. They may enjoy a big breakfast, and then use the feeding tube for dinner when they are too fatigued for food. Or they can use the feeding tube to take medications if they are having trouble swallowing pills."
Allen is determined to find a way to help patients enjoy the foods they love most. For a patient who loves peanut butter but cannot swallow it, for example, she may suggest putting it in a smoothie or present it in another manageable form. "We are here to make things as easy as possible for our patients who have this life-changing disease," she said. "I don't want to take anything more away from these patients than the disease already has."
Allen also teaches her patients alternative and augmentative ways to communicate, such as nodding the head, blinking eyes or using letter boards, when speaking becomes more difficult.
Another important member of the team, according to Allen, is the assistive technologist, who explores augmentative communication tools with physicians and speech-language pathologists. "Many patients now use tablets and applications to communicate with others because they are more readily available and less expensive than going through insurance," Allen explained. "Eye-gaze communication systems run upwards of $16,000 but a tablet is about $600 plus a few dollars for apps."
According to Allen, PALS use tablets to listen to audio books, video chat with friends and family, and as remote controls for televisions. "Tablets are more technologically advanced for our patients' needs," she shared.
"Computers have given these patients a new lease on life," Paul said. "Within last 10 years, patients have progressed from manual to power chairs that are controlled by eye blinks and joysticks."
Preparing for the Future
Being prepared for the changes that will eventually come with the ALS diagnosis is an important part of the care plan. "It's our job as therapists to predict the events PALS may encounter in the future and get them thinking about it," Paul shared. "Sometimes we have to give them the extreme scenario to get them to make changes."
Perhaps one of the most difficult parts of the disease for patients to manage is the progression of symptoms. Allen encourages her patients to keep a log of noticeable changes to discuss at each clinic appointment.
"I tell my patients I don't have a crystal ball," Allen explained. "You may stabilize for a few months with your current swallowing issues or you may progress quickly. It's not going to change drastically overnight, but the disease will progress."
For patients who are progressing quickly, or experiencing rapid weight loss, Allen may suggest that the physician see the patient more often than once every 3 months. "It's a great concern if patients are losing weight despite diet recommendations," Allen said. "I ask my patients to weigh themselves weekly to be aware of any big changes."
According to Paul, family dynamics and living situations vary among her patients and can affect the level of care they receive. "Some patients don't want to make home modifications and we need to respect their wishes while keeping them safe," she said.
For patients who live alone, Paul and her colleagues may need to broach the topic of future planning and discuss the options of a nursing home or assisted living facility if there is no family support. If a patient lives in a two-story home with a second floor bedroom/bathroom, then they need to start thinking about moving to the first floor or even moving to a one-floor townhouse.
Adaptive equipment can help PALS maintain some sense of independence in the home. Paul likes to discuss what options patients have in advance of need so they are prepared when the time comes. "I let them know equipment we have available and what they will need to purchase," she said. "If a patient is experiencing lower extremity weakness, then we know a wheelchair is on the horizon. The process of obtaining a wheelchair can take six months for evaluation, fitting and reimbursement."
Paul stresses the importance of discussing her patients' emergency plan. Especially for wheelchair- and bed-bound patients, there needs to be a contact or way to get them out of the home in the case of emergency. "I urge my patients to call their local EMS or fire department to let them know their address houses an individual with a disability who is not mobile enough to leave the house safely in the case of an emergency," she stated.
The patient and family centered palliative medicine initiative at Lehigh Hospital provides resources that help practitioners address end of life issues. "We try to make each day the best it can be and help our patients achieve the most pleasure they can," Sprenkle said. "We know the end will ultimately come but we don't know when so we help the patients be in charge of their own lives."
"Everything changes rapidly with this diagnosis so it's important that we help our patients look at the positive aspects and save energy for their favorite activities," Allen said.
Is a Cure in Sight?
Research is underway in many facilities worldwide to find the cause or causes of ALS, understand the mechanisms involved in the progression of the disease, and develop effective treatments.
HSC is actively conducting neuromuscular research and clinical trials, in concert with research partners in New York City and New England. The research team conducts epidemiology studies looking at ALS throughout the course of disease. One study uses a diaphragmatic pacing device to increase pulmonary function and survival in patients with ALS; other studies are testing various medications to help improve the lives of patients living with ALS.
HSC recently completed two large studies and the team is prepared to embark on new studies looking at compounds and medications that promote motor neuron survival in mouse models and cultures before conducting safety and efficacy in human clinical trials.
Patients and caregivers at Penn State Hershey Medical Center are given an opportunity to meet with clinical researchers. "They want to ask questions, learn what's new and find out what areas are of interest to researchers," Walsh said.
"We are challenged by a terrible disease that is currently incurable," Felice said. "We have to care for our patients who are living with this disease, that's our immediate focus and initiative."