Amyotrophic Lateral Sclerosis (ALS) is a progressive neuromuscular disease that attacks motor nerve cells in the brain (upper motor neurons) and spinal cord (lower motor neurons). In its initial stages, it causes loss of motor control. However, as the disease advances, it leads to motor impairment.
ALS is also known as Lou Gehrig’s disease, named after the American baseball player who was diagnosed with it.
Early symptoms and signs of ALS
ALS is the common type of motor neurone disease. It inhibits the motor neurons from sending impulses to the muscles, thus resulting in severe muscle loss and atrophy. The earliest symptoms and signs of ALS can be:
- Weakness and fatigue
- Tripping and falling
- Loss of motor control (in hands and arms)
- Dropping things (due to lack of motor control)
- Deterioration of muscle in arms and legs resulting in impairment
- Uncontrollable periods of crying or laughter
- Slurred speech
- Difficulty in speaking (voice-projection troubles)
- Cognitive and behavioural changes
- Inability to perform simple everyday tasks
As ALS advances, patients may also exhibit other symptoms like:
- Respiratory difficulties (including shortness of breath)
The stages of ALS
*Note to patients and caregivers: Although we have tried to make this section as informative as possible, it may be a little overwhelming for you. If you or your loved one are not at a particular stage just yet, we recommend speaking to your doctor and understanding the subsequent stages.
Early Stage: In this phase, the earliest symptoms of ALS begin to set in. Muscle weakness, stiffness, cramping, etc. may be felt by the individual. If the symptoms start in the limbs, it is termed as limb onset ALS. If the symptoms affect speech, it is referred to as bulbar onset ALS. At this stage, the individual may not experience any pain or much physical discomfort other than difficulty in performing mundane tasks.
Diagnosis Stage: This stage brings in the doctor’s diagnosis. If you’ve experienced any of the symptoms mentioned in the Early Stage or other symptoms we’ve mentioned earlier in this article, then please visit your doctor ASAP. In this stage, the loss of motor control and muscle weakness becomes even more pronounced. Your doctor will be able to make an ALS diagnosis based on your symptoms and further tests. Your medical history can also determine your condition.
The additional tests that doctors use are:
- Complete blood count (CBC) and blood test
- Urine test
- Muscle and nerves biopsies
- MRI scans
- Electrodiagnostic tests including electromyography (EMG) and nerve conduction velocity (NCV)
- Thyroid and parathyroid hormone level test
- Myelogram of cervical spine
- Spinal tap
- Neurological checks
While ALS can result in cognitive and behavioural changes, patients are generally able to retain their mental and reasoning ability. They will also be able to ascertain the comparative deterioration of muscle and motor function as they move from one stage to the other.
Involvement of Second Region (Middle Stage): By this stage, the voluntary muscles become paralysed. Respiratory problems begin to emerge, and some unused muscles may also shorten. This shortening of muscles is irreversible and is known as contracture. It affects the strength and function of joints, for eg. the patient may find it difficult and even painful to straighten their joints
Due to muscle weakness and loss, eating and swallowing become very challenging. The risk of choking also increases around this time.
A very peculiar phenomenon in this stage is the pseudobulbar effect . Some patients may burst into laughter or tears without any provocation or explanation.
Involvement of Third Region (Middle Stage): Mobility becomes increasingly difficult by this stage. Chronic headaches, mild to severe pneumonia, fatigue, and other ailments begin to affect the patient’s quality of life to a great extent. Dysphagia and speech irregularities set in, ultimately leading to the patient being fed through a tube (in the next stage). Patients in this phase are extremely vulnerable to respiratory issues (and probable failure).
Mandatory Gastronomy Stage: This is the fifth stage of ALS. The patient experiences severe dysphagia and will have trouble with maintaining their optimum weight and nutritional intake. By now, most patients have switched to a liquid diet (tube feeding).
While tube feeding may feel like the end of the road, we’d like to take a moment to assure you that things can get better. With tube feeding, the patient and caregivers can rest assured that the body is being given adequate nutrition and hydration. Research shows improvement in survival rate, quality of life, and even mental wellbeing of the patient with the introduction of tube feeding.
Late Stage: This stage can be devastating for both the patient and their loved ones. By now, nearly all the voluntary muscles have lost complete function, with the muscles that support respiration also exhibiting severe degeneration. The patient’s blood oxygen levels may drop drastically owing to difficulty in breathing. Doctors may put the patient on a ventilator.
Final Stage: For a patient with ALS, respiratory failure is the most common cause of death, followed by pneumonia and cardiovascular ailments.
Over the years, stem cell research and stem cell therapy have made remarkable progress in its application for the treatment of ALS. Patients have shown significant improvement after receiving stem cell therapy using bone marrow.
Founded by Dr. Na’eem Sadiq, India’s renowned stem cell specialist and neurologist, Plexus is the country’s leading hospital for stem cell therapy with over a decade of experience and expertise in stem cell research.
Without worrying about a cure for ALS, what we as caregivers can do is offer a better quality of life for the patient. And that is why Plexus offers the best treatment for ALS in the form of regenerative rehabilitation, which includes stem cell therapy, physiotherapy, speech therapy, and occupational therapy.
Book an appointment with us today.
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What do the final stages of ALS look like?
The final stages of ALS involve severe loss of motor function (including paralysis in limbs), dysphagia, respiratory issues, and even cardiovascular ailments. The patient will need maximum support from doctors, therapists, and loved ones to help them through the last stages. Loss of independence can take a severe mental toll on the patient, and we as their support team need to be mindful and empathetic.
How long does each stage of ALS last?
ALS progression isn’t always a straight line, and each patient’s case can be different. Each stage can last anywhere from weeks to months at a time.
What does ALS progression look like?
Following a timely diagnosis, ALS can progress from the second stage to final stage over a period of 2 to 5 years. However, there have been cases where patients have lived for 20 years or more (post-diagnosis).
How do you slow down ALS?
Timely diagnosis, regular therapy and check-ups, medication, and exercise can slow the gradual deterioration of muscles caused by ALS.