Palliative care can help you cope with the progressive symptoms associated with bulbar onset amyotrophic lateral sclerosis (ALS).

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a neurodegenerative condition affecting motor neurons — neurons responsible for voluntary muscle control — in the brain and spinal cord.

It’s a progressive, terminal disease that ultimately leads to muscle wasting and paralysis.

Most people experience limb onset ALS, in which symptoms begin your arms or legs. But 25% of cases are bulbar onset ALS, in which symptoms start from affected neurons in the corticobulbar area of the brain stem.

In bulbar onset ALS, control of muscles of the face, head, and neck is affected before the weakness of the limbs or trunk develops. Significant speech impairment, tongue immobility, and difficulty swallowing are common early stage symptoms.

Bulbar onset ALS is considered one of the most severe variants of ALS. It’s associated with cognitive decline, decreased quality of life, and shortened survival expectancy.

Bulbar onset ALS vs. ALS with bulbar involvement

Bulbar onset ALS describes when ALS starts in the motor neurons that affect your face, neck, and head.

ALS with bulbar involvement refers to ALS that starts in other areas of the body and progresses to involve the bulbar region.

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The exact cause of bulbar onset ALS is unknown. In general, 90% or more of ALS cases are sporadic, meaning they happen without the influence of hereditary or genetic factors.

Approximately 10% of ALS cases can be traced to specific genetics.

Although its cause remains unknown, bulbar onset ALS appears to differ from other forms of ALS. It may have different neuropathology, and a small 2017 study found the condition was associated with more extensive white and gray matter loss in the brain than limb onset ALS.

Some research suggests that altered glucose metabolism may also play a role in the development of bulbar onset versus limb onset ALS.

Bulbar onset risk factors

Overall, ALS risk is associated with being white, being over the age of 40 years, and having a family history of ALS. Cigarette smoking may also increase your chances of developing this condition.

A review from 2019 notes bulbar onset ALS, specifically, may be more common among females, people living with cognitive impairment, and seniors.

ALS is a progressive condition, but how rapidly motor neurons are affected can vary between people.

In bulbar onset ALS, early stage symptoms include facial muscle weakness and difficulty swallowing. In advanced stages of the condition, posture challenges develop and you may have difficulty holding up your head.

Approximately one-third of people experiencing bulbar onset ALS will also develop uncontrollable episodes of laughing or crying, known as a pseudobulbar affect.

Research suggests symptoms in other areas of the body tend to appear an average of 7 months after bulbar onset symptoms. Secondary areas affected earlier than 7 months are associated with shortened life expectancy.

The full progression of bulbar onset ALS occurs over an average of 2 years.

There is no cure for ALS and no medication that can stop its progression.

A doctor may prescribe medications for certain bulbar onset symptoms, such as:

Ultimately, treatment involves a focus on quality-of-life measures, like supporting breathing, improving communication, and managing pain.

A speech pathologist can work with you to develop new ways to communicate and make speech understandable. As ALS progresses, gesture-based language and the use of technology help you continue to communicate.

Early stage bulbar onset ALS, in which swallowing and chewing are impaired, is initially treated with dietary changes. These changes aren’t always extreme. For example, cutting your food into smaller pieces can help, as can choosing foods that are broken down quickly by chewing.

In time, the paralysis of the head, face, and neck muscles may require you to switch to meal-replacement beverages and nutrient-dense liquids.

As bulbar onset ALS progresses, the risk of aspiration and weight loss increases, and the placement of a feeding tube becomes necessary.

On average, ALS treatment can cost between $16,000 and $200,000 annually, depending on the stage of the condition.

In 2022, the ALS drug AMX0035, newly approved by the Food and Drug Administration (FDA), weighed in at the cost of $12,500 for a 28-day prescription. The cost was slightly lower than the cost of Radicava (edaravone), an older ALS medication costing approximately $170,000 annually.

Private insurance

How much coverage you’ll receive from your private insurance company for ALS treatment varies by insurance company. In a 2014 case study, insurance covered 85% of the $1,433,992 10-year cost.

Veteran benefits

ALS is considered by the Department of Veteran Affairs to be a military service-connected condition. This means you may be able to receive full coverage under your veteran benefits if your condition occurred during or was aggravated by military service.

Social Security Disability Insurance (SSDI) and Supplemental Security Income (SSI)

ALS qualifies for SSDI, which can provide you with a monthly disability income based on your previous earnings.

You may also be eligible for SSI, a federal program that offers funds specifically for basic needs like food, clothing, and shelter.

Medicare and Medicaid

Medicare can cover up to 80% of the fees associated with ALS, under both Part A and Part B plans.

Medicaid is a joint state and federal program to provide healthcare coverage to those living with ALS and other conditions. Exact coverage percentages vary by state.

ALS is a fatal condition. The average survival expectancy for bulbar onset ALS is 2 years compared with 3 to 5 years for limb onset ALS.

Palliative care with a multidisciplinary team of experts can help ensure your physical, spiritual, cultural, and psychological needs are taken care of throughout ALS progression.

Having the services of an end-of-life doula, a nonmedical professional who can advocate for your well-being, can also be comforting. In addition to many other roles, they can act as an intermediary with family and friends and bring a sense of understanding regarding the process of death.

Living with ALS

Receiving an ALS diagnosis can be distressing, but learning more about the condition, its management, and where to find support can help.

If at any time, day or night, you feel overwhelmed by what ALS means for you, you can speak with a trained mental health representative by calling the SAMHSA National Helpline at 1-800-662-4357.

ALS support groups and resources are available at:

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Bulbar onset ALS is a variant of ALS that starts in the corticobulbar area of the brainstem, affecting control of the muscles of the head, face, and neck.

It’s associated with quick disease progression and a decreased life expectancy.

While all forms of ALS are fatal, palliative care and working with a diverse group of healthcare professionals can help preserve your quality of life for as long as possible.