Last month in Caregivers 101: Amyotrophic Lateral Sclerosis (ALS) Part 1, we discussed the nature of ALS and how it impacts its patients and their families. This month, we continue to learn about ALS, including its risk factors, symptoms, and the treatments and therapies that can improve quality of life for patients and caregivers.

ALS Risk Factors

ALS is commonly diagnosed at age 55 and strikes men 20% more often at that age than women. That ratio eventually evens out as patients age, so that 60% of all patients in the ALS CARE Database are men. Military veterans are twice as likely to develop ALS as is the general public, and while the reason for this is still under study, factors like exposure to certain metals or chemicals, traumatic injuries, viral infections, and intense exertion appear to play a role. According to the Mayo Clinic, confirmed risk factors for all ALS patients include:

  • “Heredity: Five to 10 percent of the people with ALS inherited it (familial ALS). In most people with familial ALS, their children have a 50-50 chance of developing the disease.

  • Age: ALS risk increases with age and is most common between the ages of 40 and 60.

  • Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.

  • Some studies examining the entire human genome (genome-wide association studies) found many similarities in the genetic variations of people with familial ALS and some people with noninherited ALS. These genetic variations might make people more susceptible to ALS.

  • Smoking: Smoking is the only likely environmental risk factor for ALS. The risk seems to be greatest for women, particularly after menopause.

  • Environmental toxin exposure. Some evidence suggests that exposure to lead or other substances in the workplace or at home may be linked to ALS. Much study has been done, but no single agent or chemical has been consistently associated with ALS.”

Scientists are also currently studying other possible causes: gene mutation, imbalance of glutamate in the brain and spine, malfunctioning immune response, and protein hoarding by the nerve cells.

Symptoms, Treatments and Therapies

Signs of ALS usually begin in the hands, feet and limbs, before spreading to other parts of the body. The Mayo Clinic website lists the following difficulties as early signs and symptoms of ALS:

  • “Difficulty walking or doing normal daily activities

  • Tripping and falling

  • Weakness in legs, feet or ankles

  • Hand weakness or clumsiness

  • Slurred speech or trouble swallowing

  • Muscle cramps and twitching in arms, shoulders and tongue

  • Difficulty holding the head up or keeping good posture”

There is no cure as yet for ALS, but there are various therapies, regimens and medication that can have a positive impact on ALS patients. Physical therapy can strengthen the muscles that are still working well, improve cardio stamina and release endorphins to boost mood and morale. Occupational therapists can recommend braces, walkers, wheelchairs and mobility devices like ramps that make movement easier. Speech therapy can improve clarity and volume when speaking and help prepare patients for the time when speech is no longer possible. For example, speech synthesizers like the one Stephen Hawking used can use the patient’s own voice, if it’s pre-recorded. Nutritionists can help ALS patients eat properly to keep weight on, and plan meals that are easy to swallow. Two medications (Rilutek and Radicava) address ALS directly, and there are other drugs that can address the conditions like pain and constipation that develop as the disease progresses. Eventually, the patient will require breathing support, such as breathing masks, mechanical coughers, tracheostomies, and possibly respirators, if the patient wants ventilation support.

 ALS patients require support equipment, medical care and caregiving that can help mitigate the debilitating progression of the disease. There are strategies that can slow or ease the course of the disease or make the progress less disruptive and easier to live with. Caregivers, home care aides and loved ones should all be aware of the signs that could indicate the early stages of ALS and be prepared to fight its progress together.