Dying Muscles: Understanding ALS (Part 2)

Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease that causes motor neurons and nerve cells to degenerate to the point where all voluntary functions like movement, speech, breathing and swallowing are lost. Since it is incurable and eventually fatal, treatment generally focuses on controlling symptoms, extending lifespan, and adaptive measures. You can also book an appointment with a top neurologist in Pakistan if your condition worsens.

Complications:

• Breathing Problems result from gradual paralysis of the chest and neck muscles and are the most common cause of death in ALS-afflicted individuals.
• Speaking Problems often begin as occasional slurring and gradually deteriorate into unintelligible speech.
• Eating Problems result from the eventual weakening and paralysis of throat and facial muscles, making it easy for patients to develop malnutrition and contract pneumonia by accidentally getting foods, liquids, and even saliva, into their lungs.
• Dementia, particularly Frontotemporal Dementia that affects decision-making, behavioral control, emotion and language may also develop in some individuals.
• Neuropathy, i.e. pain caused by nerve damage may also be experienced by some patients alongside muscle cramps.

Alternative options are recommended to help optimize remaining functions in case of breathing, eating, and speech problems.

Treatment:

Along with a primary neurologist, a complete ALS treatment program constitutes specialists from multiple other medical fields for all-inclusive care:

1- Medication:

As of now, only two drugs have been approved for exclusively treating ALS:

• Riluzole in pill form reduces levels of glutamate, a chemical that transfers messages between the motor neurons and nerve cells and is present in high quantities in ALS-affected individuals. However, it only increase life expectancy by 2-3 months and may induce side-effects like liver function changes, dizziness, and gastrointestinal conditions.
• Edaravone noticeably slows motor decline, particularly if treatment is initiated from the early stage. Administered monthly for 10-14 days via intravenous infusion, it may cause serious allergic reactions in patients sensitive to sulfur due to a high sodium bisulfate content. Other side-effects include hives, bruising, swelling, shortness of breath and gait issues.

2- Therapies:

• Breathing Options: Patients are usually recommended assisted breathing devices to aid them in night breathing and later, for permanent use. Options include Mechanical Ventilation, Non-Invasive Ventilation (NIV) in which oxygen is delivered via a mask over the nose and/or mouth (particularly when muscles are unable to regulate oxygen and carbon dioxide levels), and surgical Tracheotomy; i.e. a hole at the front of the neck connected to a respirator via a tube to help the lungs inflate and deflate.
• Physical Therapy: Low-impact exercises like walking, swimming and stationary cycling can help preserve and optimize muscle strength and improve cardiovascular fitness, while range of motion exercises can help prevent muscle spasms and shortening in early-mid stage patients. Moreover, in addition to acclimatizing patients with braces, walkers, and wheelchairs, physical therapists may also suggest installing assistive devices like ramps to aid mobility.
• Speech Therapy: With speech therapists, early-stage patients can learn adaptive techniques to make their speech more clear-sounding. Mid-to-late stage patients can learn and adopt non-verbal forms of communication like alphabet boards and replying to yes-or-no questions via simple gestures. Computer-based speech synthesizers (in which the patient’s own voice can also be stored while they are still able in a process called ‘Voice Banking’ for later use) and text-to-speech tablet applications are also viable options.
• Nutritional Support: Since weight loss weakens ALS patients, nutritionists and neurologists can work to help the patient, and later their caretakers, prepare a meal plan comprising easy-to-swallow yet nutritionally-sufficient meals. Suction devices to prevent choking by removing saliva and excess fluids may also be used. Patients will later require feeding tubes inserted into the stomach when eating becomes too difficult.
• Psychological Support: The gradual loss of bodily control and independence coupled with realization of the eventual outcome can easily cause patients to become anxious and depressed, which can escalate disease progression. Therefore, regular psychological support alongside medicinal treatment is necessary for both the patient and their family.
• Occupational Therapy: Not only do occupational therapists help patients find ways to continue working alongside steady muscular decline, but can also help patients get accustomed to adaptive equipment to aid them in independently performing daily activities, as well as suggesting home modifications to improve accessibility.

ALS is a fatal condition, but with the right treatment and a positive mindset patients can make the most of their bodily functions and improve their quality of life. Consult with your doctor immediately if you or a loved one has been diagnosed with ALS for prompt and adequate treatment. You can also book an appointment with a top Neurologist in in Lahore, Karachi and Islamabad through oladoc.com, or call our helpline at 042-3890-0939 for assistance to find the RIGHT Doctor for your neural concerns.