Amyotrophic Lateral Sclerosis (ALS)

Also known as Lou Gehrig's Disease Named after the New York Yankees baseball star who played first base and was diagnosed with the disease in 1939 and died in 1941 but, contrary to popular belief, this disease had been discovered almost 100 years earlier.

History ALS discovered in 1850 by English scientist Augustus Waller who didn’t know he had discovered ALS but saw nerve fibers that had extreme atrophy 19 years later a French neurologist Jean-Martin Charcot took credit for it in publishing the disease in a scientific journal

Objectives What is this disease? Signs and Symptoms Probable causes of ALS Forms of ALS Diagnosis of ALS Medications you will see for ALS patients Prognosis (outcome) Stats for ALS

What is this disease? a progressive neurodegenerative disease “ALS is a condition in which cells of the brain and spinal cord are lost.” This affects mostly motor neurons the cells that control needed voluntary muscle activity such as speaking, skeletal muscle movement, breathing, and swallowing.”

What is ALS cont… When the motor neurons eventually die, the ability of the brain to control muscle movement is lost. Causing paralysis of essential body systems. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller).

Signs and Symptoms Early symptoms Increasing muscle weakness “especially in the arms and legs” Difficulty speaking Trouble swallowing Problems with breathing Twitching Cramping of muscles (mostly hands and feet)

Signs and symptoms cont… Late Symptoms Extreme muscle atrophy Reflexes that are slow to non-existent Excessive drooling Babinski's sign: “the big toe dorsiflexes and the other toes fan out “ Increased spasticity (muscle rigidity) Weight loss Choking Cardiac arrest due to respiratory arrest usually resulting in death

Probable Causes of ALS Free radicals. The inherited form of ALS often involves a mutation in a gene responsible for producing a strong antioxidant enzyme that protects your cells from damage caused by free radicals — the byproducts of oxygen metabolism. Glutamate. People who have ALS typically have higher than normal levels of glutamate, a chemical messenger in the brain, in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.

Probable Causes of ALS cont… Autoimmune responses. Sometimes, a person's immune system begins attacking some of his or her body's own normal cells, and scientists have speculated that such antibodies may trigger the process that results in ALS. ALS is not contagious.

Forms of ALS Sporadic - the most common form in the United States - 90 to 95% of all cases. Familial - genetic “accounts for a small amount of cases in the United States” - 5 to 10% of all cases. Guamanian - an extremely high incidence of ALS was observed in Guam in the 1950's. Due to toxins in the water and food .

How to diagnose x-rays MRI Blood and urine studies muscle and nerve biopsy electomyography (EMG) nerve conduction velocity (NCV) spinal tap Even with all this technology ALS is extremely difficult to diagnose. This is because many diseases mimic signs of ALS

Prognosis Usually end up needing to go to a nursing home or have hospice Only half of all people affected with ALS live three or more years after diagnosis.

STATS Based on U.S. population studies, a little over 5,600 people in the U.S. are diagnosed with ALS each year (approximately 15 new cases a day.) Most people who develop ALS are between the ages of 40 and 70 ALS is 20% more common in men than in women. 93% of patients are Caucasian.

Stages of ALS The natural course of ALS consists of 6 stages. Stages are based on the progressive loss of function in trunk and extremity muscles. Identifying the stage can assist physical therapists in designing intervention throughout the disease process.

Stage 1 Patient is independent in mobility and ADLs. Muscles may just feel mildly weak. Physical therapy consists of: Patient and caregiver education Energy conservation Psychological support AROM and stretching of affected joints and resistive strengthening exercises of unaffected muscles using moderate weights and aerobic activities (eg, swimming, walking, bicycling)

Stage 2 The patient has moderate weakness (eg, foot drop, fine motor skills). Assess for appropriate assistive devices to support weak muscles. Physical therapy: Patients are encouraged to continue stretching and AROM exercises, strengthening of unaffected muscles, and aerobic activities as able. In addition, the patient and caregiver are instructed in performing AAROM and PROM of affected joints to prevent contractures.

Stage 3 Patient still ambulatory but severe weakness of certain muscle groups. Patient may be unable to stand without help Mild to moderate limitation of function. Physical Therapy: Assistive devices (AFO, neck collar, splints, electric WC) Continue exercises to weak muscles Decrease energy expenditure Improve patient’s safety and mobility

Stage 4 Severe weakness of the legs and mild involvement of the arms. Patient uses a WC but may still be able to perform most ADLs. Physical Therapy: PROM and AAROM to prevent contractures Strengthening of any uninvolved muscles. As mobility decreases the need to inspect skin for pressure areas increases. Need for sleeping and sitting systems that allow position changes and pressure relief.

Stage 5 This stage is characterized by progressive weakness and deterioration of mobility and endurance. WC bound when out of bed. Arms now exhibit moderate to severe weakness. Transfers may require lift Patient unable to move themselves in bed and chairs Pain is a major problem ( immobilized joints, etc.) May have breathing problems Physical Therapy: Stretching, ROM and massage Modalities for pain Assistive devices Caregiver instruction

Stage 6 Bed ridden and requires max assistance with ADL Positioning Pain continues to be a major problem Breathing a problem Physical Therapy: Goals at this stage are similar to those of hospice care: Address patient’s and caregivers needs Maximize the quality of each day Caregiver instruction to continue PROM, stretching, transfers, massage, airway clearance techniques.

Common MEDS Most commonly seen is riluzole Rilutek© is aimed at symptomatic relief basically maintaining optimal quality of life. Side note : there is no drug interactions to any of the drugs we use as paramedics. Medical marijuana Baclofen or diazepam may be used to control spasticity Trihexyphenidyl or amitriptyline may be prescribed for people with problems swallowing their own saliva.

Meds Cont…. O2 and gastric tubes maybe prescribed as well due to trouble breathing and choking Coming Soon due to Stem cell research maybe a cure ?