The progressive paralyzing muscle disease that killed esteemed physicist Stephen Hawking has claimed the life of Sandra Bullock’s longtime partner.
Photographer Bryan Randall, 57, privately battled amyotrophic lateral sclerosis (ALS) for three years before succumbing to the disease this week, according to a statement.
The progressive disease, which has a life expectancy of only two to five years, causes the muscles to wear down. This leads to a fatal paralysis of the whole body.
The Centers for Disease Control and Prevention (CDC) estimates that 31,000 Americans are living with ALS; on average, 5,000 new patients are diagnosed annually. In the UK, the condition is also called motor neurone disease and affects around 5,000 people.
What is ELLA?
ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord.
The condition is also known as Lou Gehrig’s disease, after the baseball player who was diagnosed with it.
As motor neurons die, muscles begin to waste away. This causes a loss of muscle control that worsens over time. Eventually, a patient will not be able to control the muscles needed to move, speak, eat, or breathe.
The cause of ALS is still unknown. About 5 to 10 percent of cases are inherited, according to the ALS Association, while the other 90 to 95 percent have no genetic link.
Bryan Randall, 57, a longtime partner of Sandra Bullock, died this week after a three-year private battle with ALS.
Esteemed physicist Stephen Hawking was diagnosed with ALS at age 21. He beat the odds by surviving 53 years after diagnosis.
What are the symptoms?
ALS usually starts with muscle weakness that gets worse over time.
According to the Mayo Clinic, other symptoms include difficulty walking or doing daily tasks, tripping or falling, weakness in the legs or feet, weakness in the hands, difficulty speaking or swallowing, crying or laughing randomly, and changes in mood. thought or behavior.
The condition tends to start in the hands, feet, arms, or legs before spreading to other parts of the body.
In later stages, most of the voluntary muscles are paralyzed and breathing is severely compromised. This bad breath can cause fatigue, confused thoughts, headaches, and susceptibility to pneumonia.
People with ALS usually do not have pain.
Who is at risk?
Military veterans are more likely to be diagnosed with ALS, according to the ALS Association, but it’s not clear why.
In families with familial ALS, there is a 50 percent chance that each offspring will inherit the gene mutation, which could cause them to develop the disease.
Although symptoms can start at any age, they most often develop between the ages of 55 and 75, according to the National Institutes of Health (NIH). The risk increases up to age 75.
Men are also more likely to develop the disease than women. However, after the age of 70, the sex difference disappears.
ALS occurs most often in white and non-Hispanic adults, although it affects all races.
Some research suggests that smoking and exposure to lead or other environmental toxins might increase the chance of ALS, although the findings are not conclusive.
How is it diagnosed?
ALS can be difficult to diagnose in the early stages, since the symptoms are similar to those of other diseases. However, a patient may have biopsies of their muscles or nerves, as well as blood and urine tests to rule out other causes.
Also, an MRI of the brain and spinal cord can reveal changes in ALS.
In some cases, an electromyogram (EMG) may be performed. This is when a needle is inserted through the skin into the muscles to see if there is damage.
Similarly, doctors can perform a nerve conduction test, which measures the ability of nerves to send signals to muscles and can determine if a patient has nerve damage.
How is it treated?
In April, the Food and Drug Administration (FDA) approved the first drug, sold under the brand name Qalsody, for ALS cases with a genetic mutation known as SOD1. This is present in approximately two percent of cases.
There is no cure for ALS and the disease is fatal. It is also not preventable.
Life expectancy is around two to five years, although some patients may live longer. Hawking, for example, lived with the condition for more than 50 years after his diagnosis.
In April, the Food and Drug Administration (FDA) approved the first drug, sold under the brand name Qalsody, for ALS cases with a genetic mutation known as SOD1. This is present in approximately two percent of cases.
The drug is injected into the spinal canal every few weeks.
Qalsody is thought to reduce levels of a protein that has been linked to nerve cell damage. However, it does not stop the progression of the disease.
Three other drugs have been approved in the US: Riluzole, Edaravone, and Sodium phenylbutyrate-taurursodiol, although none significantly slow the disease.
