Many people have heard of amyotrophic lateral sclerosis, also known as ALS. This disease was originally made famous by Lou Gehrig, and more recently by the Ice Bucket Challenge. It is a neurodegenerative disease that affects strength and movement. Our movement is controlled by neurons. Neurons connect our brain to the rest of our body. In ALS, the neurons break down. This can be caused by genetics, but it is much more often spontaneous. The exact cause of the disease is unknown, which makes it difficult to treat.
What are the early symptoms?
Everyone experiences ALS differently, especially when the disease first presents. It most often presents itself in people that are 50-70 years old, more commonly in men than women. People most often experience weakness in their hands in feet. Because ALS affects neurons in both the brain and the limbs, it can cause a mix of weakness. Some people make experience flaccid muscle weakness, and others experience spasticity, similar to tight, twitching muscles. Some people may experience difficulties walking or balancing. Others may have non-specific changes in their voice, like hoarseness or a “shaky” voice. They may also have difficulty swallowing. This can cause some people to lose weight.
What are the late symptoms?
Late in the disease, many people are unable to walk. They also have difficulty eating and drinking due to the degeneration of the motor neurons that affect swallowing. When people have difficulty swallowing, they are at higher risk for a certain type of respiratory infection called aspiration pneumonia. Normally, people are able to protect their trachea, their “wind-pipe”, when they swallow. People with ALS are unable to do so, so food or liquid can get into their lungs and cause an infection. They can also suffer from malnutrition due to their inability to eat. Additionally, patients with late stage ALS may have cognitive impairment. In fact, 15% of late stage patients have symptoms of frontotemporal dementia, a particular type of dementia that affects memory and personality.
How is ALS diagnosed?
ALS is largely diagnosed clinically, meaning that a doctor puts all of the symptoms together and can make a diagnosis. They may use certain tests to aid their diagnosis if they are unsure, but it is not necessary. Some of these tests may include electromyography and nerve conduction studies. These run electric currents through muscles and nerves and see if they fire as they are supposed to. Those who have ALS have muscles and nerves that fire with a specific pattern. Doctors may also decide to use an MRI, but this is optional.
What is the prognosis?
Unfortunately, ALS is often devastating. Motor neurons affect more than just strength and movement. They also affect the ability to eat, drink, and breathe. When motor neurons break down, the diaphragm, the muscle beneath our lungs that is responsible for breathing, no longer works. On average, patients with ALS will go on to have respiratory failure and subsequent death within 3-5 years.
What are the treatment options?
Patients with ALS are normally treated by a neurologist, a specialist that studies the brain and neurons, and how they interact with the body. They normally recommend a multifactorial approach. This generally includes physical therapy, speech therapy, social work, nursing, and other resources. Physical therapy is helpful because it not only strengthens the existing muscles, but also helps patients learn new techniques to navigate their new symptoms. Speech therapy sounds misleading, but is also important. Speech therapy actually refers to swallowing. These specialists will help patients strengthen their swallowing and learn new techniques to decrease aspiration. There is one medication that is formally approved to treat ALS, called Riluzole. Riluzole has been shown to slow the progression of ALS. It is neuroprotective and reduces the spasmodic qualities of the muscles affected by ALS.
How do stem cells treat ALS?
Stem cells are a special type of cell in the body that can become many different types of tissue. They can become brain cells, skin cells, muscle, or bone cells. They can be found in many different parts of the body. Human embryonic-derived stem cells are particularly promising. These come from umbilical cord tissue.
Stem cell transplant is a newer technology. Stem cells are particularly helpful in ALS for many reasons. One reason is that neurons normally do not like to repair themselves. They are very slow growing cells. Stem cells, in contrast, are made to grow and replace many types of tissues. They can be programmed in such a way that they support already existing motor neurons or they can replace old, degenerated neurons. There are many studies that are still ongoing, but the early phases are promising.
Stem cell use for ALS is an emerging field. At the Stem Cells Transplant Institute, we have developed state-of-the-art treatment protocols for ALS patients in culture expanded cells. If you are interested in learning more about how you can use this technology for your own health goals, please contact our institute today.