Oscar Buzz: How Eddie Redmayne Brought ALS To Hollywood
ALS: What's in a Name?
Did you dump ice on your head in the ALS Ice Bucket Challenge last summer? Have you seen the highly acclaimed film The Theory of Everything? Then you know something about Amyotrophic Lateral Sclerosis (ALS). But should you learn even more? Unlike breast cancer or heart disease that garner massive amounts of fund-raising through their colorful campaigns, ALS is not as well-known. In fact, according to The ALS Association, only about 50 percent of the nation knew about ALS (Lou Gehrig's disease) prior to the Ice Bucket Challenge. Many did not know that Lou Gehrig, a beloved American baseball legend, died of ALS complications in 1941.
ALS Doesn't Play Favorites
ALS knowledge is now building. ALS is a fatal disease without a cure (yet), and ALS plays no favorites - it can strike anyone. Approximately 5,600 people in the U.S. are diagnosed with ALS each year. The neurodegenerative disease usually strikes patients between 50 and 70 years of age, and affects men slightly more often than women.
ALS leads to a slow degeneration of nerve cells that control muscle movements and an eventual loss of muscle control. The capacity to think and remember is not usually affected. However, weakness, muscle wasting, paralysis, and loss of lung function eventually occur.
ALS: It's Progressive
As painstakingly depicted by actor Eddie Redmayne who played theoretical physicist Stephen Hawking in the Oscar-nominated film "The Theory of Everything", ALS is a fatal, progressive neurodegenerative condition leading to muscle wasting, paralysis, and loss of vital functions such as speech, swallowing, and eventually breathing. The life expectancy in ALS ranges from 2 to 5 years after diagnosis, although about 20 percent of patients may live longer. Hawking himself has drastically beat the odds, now 50 years after his diagnosis and 72 years old. The cause of ALS remains unknown, but in about 10 percent of cases, it appears to be inherited through genes.
What Causes ALS?
The cause of ALS is under research, but may include:
- Autoimmune disease: In ALS, the body attacks its own nerves.
- Chemical imbalance: Too much glutamate, a chemical that initiates nerve signals, may cause the motor neurons to die.
- Frequent chemical exposure: Exposure to chemicals such as fertilizers and pesticides used in lawn care.
- Genetics: Your risk is greater if a family member has thyroid disease or an autoimmune disease.
Are There Any Treatments for ALS?
At this time, only one medication, riluzole (Rilutek), is approved by the FDA for treatment of ALS. Riluzole, which blocks the nerve cell messenger glutamate, is not a cure for ALS but may delay disease progression and extend survival for several months. More recent reviews have shown that survival may even be longer than originally seen in clinical trials with riluzole, although randomized controlled studies are needed to confirm this extended survival benefit. One common class of blood pressure drugs known as Angiotensin Converting Enzyme (ACE) Inhibitors may help to reduce the risk for ALS, but more research is needed here, too.
What's On the ALS Horizon?
Although riluzole an FDA-approved treatment for ALS, it can have limited effectiveness. New treatments are needed for ALS. MN-166 (ibudilast) is an investigational drug under research by MediciNova for use in ALS. Ibudilast is an oral phosphodiesterase (PDE) inhibitor and macrophage migration inhibitory factor (MIF) blocker that suppresses inflammatory chemicals. Ibudilast's anti-inflammatory and neuroprotective actions have shown an effect in ALS and multiple sclerosis, substance abuse and addiction, and chronic neuropathic pain. The combination of riluzole and ibudilast is also being studied.
Clinical Trials: An Option for You?
Many ALS clinical trials are ongoing. In fact, patients can review clinical trials that are currently enrolling patients. Using a search tool provided by the ALS Association, current trials can be viewed and patients can contact their doctor for further discussion to determine if joining a trial might be a good option.
FDA-approved drugs that have shown success such as fingolimod (Gilenya) in multiple sclerosis, or rasagiline (Azilect) in Parkinson's disease, are actively being research for ALS. Examples of other medications under study that are currently enrolling participants include: ibudilast, L-serine, and cannabis sativa extract.
One Disease: Many Needs
The viral Ice Bucket Challenge media blitz resulted in over $100 million in donations for ALS research and support - a very good thing! Plus, the film "The Theory of Everything" brings to light the physical and emotional struggles of this condition. But ongoing awareness and education are key to treatment discoveries and a cure. That's where we all come in, whether it be ALS, pancreatic cancer, Parkinson's disease or any other disease without a cure. Pick one close to your heart, and continue your campaign individually with community fund-raising, disease awareness campaigns, and partnering with national associations such as the ALS Association.