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Knowledge of this complex disease can help enhance home care
Most people only think about amyotrophic lateral sclerosis (ALS) when someone they know is diagnosed with it. The fatal neurological disorder is known as Lou Gehrig’s disease, named for the legendary ballplayer whose battle drew the nation’s attention. Only a few months ago, ALS again was in the spotlight when it claimed yet another baseball great, Jim "Catfish" Hunter.
Nearly 5,000 people in the United States are diagnosed with the disease each year, and about the same number die, according to the ALS Association in Calabasis Hills, CA. During the course of the disease, as ALS robs the muscles of vital motor cells, many patients end up in home health care. But because the disease moves erratically, causing different symptoms in different patients, health workers may be confused and disturbed by its progression.
An inservice that explains what ALS is, what it does, and how to help patients and families cope with its physical and emotional burdens can make the difference in keeping patients independent as long as possible, says Andrea Versenyi, CSW, of the Greater New York Chapter of the ALS Association. "I really try to move away from the idea that this is an incurable illness and there’s nothing we can do,’ to show that there are a great number of things we can do to address specific symptoms," she says.
Versenyi conducts inservices for home health agencies to educate them about the specifics of the disease and the particulars of care needed by ALS patients. She first outlines the physiology of ALS, which is a progressive neuromuscular disease that destroys the nerve cells that form pathways between the brain, the spine, and the muscles of the body. As those nerve cells, called motor neurons, die, the brain is unable to start or control voluntary muscle movement. "I can think, Move my hand,’ but unless those motor neurons, the nerves that control motor movement, pass that message along to the muscles, nothing will happen," Versenyi explains.
A person with ALS loses the ability to speak and swallow, eventually becomes totally paralyzed, and ultimately dies of respiratory failure. Because only motor neurons are affected, ALS doesn’t harm a person’s senses of sight, hearing, smell, taste, or touch. More importantly, a patient’s intellectual abilities are not impaired — a point that Versenyi stresses in her inservices.
"One of the biggest complaints that people who have ALS have about their care is that they may be paralyzed and unable to talk, but they’re completely alert," she says. "A lot of people say that as they begin to talk with a slurred voice, people tend to ignore them or treat them like they’re stupid. I stress that in most cases of ALS, there’s a very vital person trapped inside that body — a very frustrated person."
ALS occurs all over the world, among people of all races and socioeconomic backgrounds. Patients have been first-diagnosed as young as their 20s and as old as their 80s, but normally the onset of ALS comes between ages 40 and 60. Although its causes are not known, about 5% to 10% of ALS cases occur multiple times in families, and a defective gene is believed to be a possible link.
The average life expectancy from time of diagnosis is only two to five years. But thanks to recent advances in medical care, patients are living longer. Up to 10% survive more than 10 years after diagnosis, according to the ALS Association. Currently, there is only one treatment that has been found to affect the progression of ALS, although there are many other medications to manage its symptoms.
"We do talk to our people about being hopeful; if not hopeful for a cure, at least hopeful that we can manage their symptoms and keep them comfortable," says Bettie Neal, RN, BSN, of the Greater Philadelphia Chapter of the ALS Associ ation. "And then, [we look] at their quality of life."
Among the most important issues for patients with ALS, Versenyi and Neal say, are mobility, eating, communication, bowel management, and breathing.
o Mobility: As ALS progresses in a patient, muscles can be affected in two very different ways, Versenyi says. If the upper motor neurons are affected (those running from the brain to the spinal column), a patient may experience spasticity or stiffness. Lower motor neuron damage, along the pathways from the spine to the muscles of the body, causes the muscles to become weak and atrophy.
"A lot of times, these things are combined," Versenyi says. "So there will be different effects in everybody who has ALS. You can work with 10 different people who have ALS, and they can show completely different symptoms. That’s a little bewildering to [health care] workers. They had one person who had ALS, and that person couldn’t walk, and suddenly they work with someone else who has ALS, and [that person] can’t talk."
Someone suffering from spasticity will walk with what Versenyi calls a "Frankenstein step," stiffly and awkwardly, because he or she cannot bend at the knee. Medications can help relieve the stiffness, helping patients to use whatever strength they have more effectively. Improv ing range of motion and learning to conserve energy can help patients cope with muscle weakness.
In addition to medical interventions, Versenyi also discusses devices that can help people with ALS remain mobile. For example, a person with foot drop, or a bending of the foot due to muscle weakness, can wear a foot orthosis, a plastic guide that holds the foot in place to help facilitate walking.
"If somebody has hand weakness, they may not be able to write or brush their teeth or go to the bathroom independently," she says. "I may discuss the range of devices that can assist in doing those tasks to maintain independence.
"Perhaps someone speaks and swallows very well, but if the arm is weak, they can’t lift the fork to their mouth and there will be nutritional impairment, not because they’re unable to swallow but because they’re unable to actively lift the food to their mouth. Unless somebody is feeding them or they learn techniques to conserve energy, they’ll have problems."
As patients become more immobile, it’s important to check on them regularly because they may be unable to call out or even push a panic button to ask for help. Caregivers also should shift the patient regularly to maintain skin integrity.
o Eating: Versenyi says there are thousands of muscles that allow person to speak and swallow food and liquid. ALS can interfere with those functions in myriad ways. "Maybe a person can swallow well but can’t move food to the back of their mouth. Or maybe they can move the food but they can’t chew the food."
Diet modifications that can help include switching to a pureed diet. Thick liquids tend to go down more easily than thin ones. Neal says water can be one of the most difficult things to swallow. Also, foods such as a chunky soup that contain both liquid and solid parts present challenges. "Soups with chunks in them are about the worst because [patients are] trying to negotiate two very different things," she says.
If a person is having difficulty eating because of swallowing difficulties or other problems, it’s time to suggest a change, Versenyi says. "They have to decide that if a meal is taking two hours, is it just too tiring, and is it time to try something different?" Ultimately, the patient may require insertion of a feeding tube when even swallowing liquids is too difficult.
o Communication: Loss of muscle control can cause patients with ALS to slur words or lose voice volume, and it an affect tongue and lip movement so they cannot actually form sounds.
"They frequently need in the beginning to see a speech pathologist," Neal says, "to work on communication skills — helping with breath support, helping them accept the need to do alternative communication to supplement their speech either writing or alternative augmentative communication, such as computer-type devices."
Adapt to technology
Versenyi says patients can use a phrase or picture board as well, but these can take time to use and interpret. A common computerized device allows the user to type words, which then are recited by a synthesized voice.
She says it is important when communicating with an ALS patient via computer to ask how the patient wants that communication to take place. Some people prefer that the listener wait for the computer to say the words, while others prefer that the listener read over the patient’s shoulder so the conversation can go more quickly.
The technology can cause problems for users. "They have problems with people answering the phone and getting the computerized voice," Versenyi says. "They hang up, thinking it’s one of those calls you get from marketers. It’s just one of the little things, one of the difficulties and frustrations of living with this illness." She says health care workers need to continue to communicate with an ALS patient, even when that communication becomes difficult or time-consuming.
o Bowel management: Patients with ALS can suffer from severe constipation for a variety of reasons, Neal says. "There’s usually several components of it. One is their immobility; they have loss of abdominal tone and can’t push."
Patients also can suffer from excessive saliva, and the most common treatments for that can cause constipation as a side effect. In addition, patients may restrict water intake, either because of difficulties swallowing or because they cannot get to a toilet easily and want to prevent accidents.
"You put all those together, and they can have major problems with their bowels," Neal says. "They need constant assessment of [bowel function], and bowel regimes [need to be] changed. Very rarely does one bowel regime fit the bill for any length of time."
ALS patients are prone to ileus, or bowel obstruction, which requires frequent assessment and early intervention, which usually consists of adjusting liquid, fiber, and laxatives to keep the bowels moving. "That’s something that home care nurses in particular need to be aware of," she says. "Therapists need to help with the transfer and toileting issues. If [the patient] can’t get to a toilet or a commode, that needs to be addressed."
o Breathing: Because respiratory failure is the usual cause of death for a person with ALS, continual assessment of breathing capabilities is essential in home care. Neal acknowledges that the assessment can be difficult because the loss of respiratory support, like other ALS symptoms, affects people differently.
One important condition to watch for is carbon dioxide retention, which can cause a person to become confused or lethargic. Home health workers should check routinely for those signs and should ask patients if they experience morning headaches or excessive yawning, other symptoms of CO2 retention.
Many health professionals also regularly test the patient’s forced vital capacity or the amount of air a person can forcefully exhale. "I know when I’ve talked with people throughout the country who are involved in home care, that might not be something that the local neurologist will recommend, but it’s one of the few things that can be a concrete indicator of a change in respiratory status," Neal says. "If the forced vital capacity is declining, then we know that people are at risk for respiratory compromise and respiratory failure."
As the disease progresses, a patient must decide when breathing has become too difficult and assistance such as a trach and ventilator is needed.
o Psychosocial issues: The wrenching decisions that come with living with ALS — when to submit to a feeding tube or a ventilator, for instance — as well as the knowledge that the disease is ultimately fatal can be an overwhelming burden to the patient and the family.
Versenyi and Neal point to outside forces that can worsen the situation, such as financial concerns and insurance hassles. "Is the caregiver forced to stop working in order to care for the patient? Is the caregiver forced to go out and find a job in order to care for the patient? There are all sorts of ways this can affect a family," Versenyi says. "What effect does it have on a child? What effect does it have on a parent to no longer be able to parent their children in the way they would like?"
The strain of watching one’s body waste away while remaining mentally alert can be frustrating and can lead to control issues. "The patient is dealing with a tremendous loss of control, and they will exert their control sometimes in very negative ways," Versenyi says. "They will refuse to get up at a certain time — if they know they have a doctor’s appointment at 9:30, they won’t get up until 9 o’clock, which definitely doesn’t enable them to get there on time.
"The caregiver will be furious with them. The patient may be doing that because it’s the only way they can have some sort of control, because there’s not a lot they’re able to actively do."
Home health workers need to offer the patient what control they can by allowing the patient to make choices. "I think it’s important when you go in there, to at least say, I want to do things the way you want them done,’" Versenyi says.
"If somebody tends to say no, it’s never right, oftentimes you can give them options that don’t give them room for no,’ [such as] Would you like to have your medication before your shower or after your shower?’ Would you like apple juice or orange juice?’ [Those questions] still give the patient a choice."
Neal says home health staff should be ready to refer patients and families to appropriate community services, including caregiver support groups or other agencies.
Versenyi agrees, noting that it also takes a team of medical professionals to treat someone with ALS. An aide dealing with a very stiff patient can tell a nurse, who can take word back to the physician so a patient might get medication that can help. A speech pathologist can be called in to assist with communication skills.
"I think whoever is in the house should not feel like they have to do it alone," Versenyi says.
Caring for a patient with ALS can be traumatic for home health providers as well, Versenyi and Neal say. Versenyi, a certified social worker, says that before her affiliation with the ALS Associat ion, she worked in hospice and dreaded working with patients who had the disease.
"I think for care providers, usually the ALS patient is of the same age range; they’re a young er population oftentimes," she says. "They may have school-age children at home. I think there’s something that’s emotionally powerful for the care provider in thinking, Here’s a person who’s sort of my age.’"
Contributing to the burden is the horror of how the disease progresses; it makes patients prisoners in their own bodies and plays on the instinctive human fear of suffocation. "I’ll talk a little bit about how respiratory failure with ALS is not like having an asthma attack," she says. "It’s a more gentle process. It’s not a violent process, it can be a very peaceful death." She encourages home health workers to discuss concerns and feelings with others in the agency so they can refocus on helping patients. Doing so can alleviate feelings of helplessness. "Often what I find happen is the aides or the nurses are furious at the agency because they feel that they’re not getting adequate support. And usually it’s not that the agency is not providing adequate support, it’s that what one can do with someone who has ALS never feels like enough."
Neal says even with the constraints imposed by managed care or other insurance, she rarely has seen problems with necessary support not being covered. "As long as we can justify the changes and what our expectations are for the treatment, they’ve approved it. I think that’s what home care needs to understand. It is a disease that’s very tragic, but nobody’s going to keep the nurse or the therapist in there because it’s a tragic disease. They need to be monitoring and making sure that their treatment regime is appropriate and necessary."
She and Versenyi encourage home health agencies to take advantage of the wide network of support available to ALS patients and caregivers. Information and contacts are available through the national ALS Association and its many local branches, as well as through other groups. (For publications and videotapes, see box, p. 136. For Web sites, see Internet Connect, above right.)
"We can give them specific strategies for dealing with ALS, knowing what the illness is, knowing the range of treatments available," Versenyi says. "I love it when home health agencies call me."