Sam Shepard ALS Disease
Sam Shepard ALS Disease – The Oscar-nominated nominee and Pulitzer Price winner Sam Shepard died on Thursday, July 27, at his home in Kentucky, USA. The sad news about Sam’s death was reported by the New York Times. According to family representatives, Sam died at the age of 73 years after battling ALS disease.
Sam debuted in front of the camera in 1970 and was later popular with his acting in several film titles such as The Right Stuff, Steel Magnolias, Days of Heaven, All the Pretty Horses and more recently, Mud and August: Osage County.
Shepard died three children, Jesse, Hannah, and Samuel Shepard. Hannah and Samuel are the result of her marriage to actress Jessica Lange, whom she dated for nearly 20 years before parting in 2009.
Definitions and Overview ALS Disease
ALS is Amyotrophic lateral sclerosis, a degenerative disease in motor nerve cells that develops rapidly and is caused by damage to nerve cells. Sometimes this disease is also called Lou Gehrig’s disease (Lou is a New York basketball player who quit his profession after being declared to have the disease), the disease has not yet known the cause and treatment.
In the United States, more than 5,000 men and women are diagnosed ALS every year. On average, patients can live 2 to 3 years. About 20% can last up to 5 years, and very few survive one to two decades.
The brain communicates with other body parts by sending signals through “thin fibers” called nerve fibers. Some parts are responsible for vision, feeling and hearing function, while others serve to help conscious muscle movement, such as walking, talking, and eating. Nerve cells originating from the brain and into the spinal cord (the spinal cord) then to the muscles are called the motor nervous system. The characteristic of ALS is the deterioration of this motor nerve. In the end the motor nerve will die so that the signal from the brain can not pass through it. As a result, the brain loses control of the muscles, and the body becomes smaller.
The exact cause of decreased motor neuron function is still an intensive research subject. So far, more than 90% of cases are considered unrelated (sporadic). However, about 10%, is a familial case of ALS or FALS. In FALS, more than one family member, usually including a parent (autosomal dominant), is diagnosed with this condition. Patients with FALS will experience symptoms earlier if compared with sporadic ALS. FALS patients are also at risk of frontotemporal dementia (decreased brain function due to damage to the front of the brain) due to C9orf72 gene mutations.
One of the first studies on FALS states that gene mutations as responsible for the production of SOD1 enzymes are causing enzyme poisoning.
- Muscle Weakness
- Muscle cramp
- Difficulty breathing and swallowing
- Loss of control on the muscles
- Inability to stand, walk or lift
- Speech is not clear
- Strange feeling about fatigue
- Saliva is excessive and uncontrollable
- Atrophy (shrinkage) muscles
- Reflux of acid
- Excessive saliva
- Impaired absorption of nutrients
Because the disease affects the motor neuron, the patient will not lose cognitive function and other mental disorders. He can also maintain other body functions such as seeing, hearing and feeling.
Who to See and Available Treatments
The worsening of disease conditions and symptoms varies among patients. Sometimes, losing control of the muscles is so small that new patients can be diagnosed when they reach advanced stages. Normally, patients feel there is something wrong when they start to become stiff.
This disease does not have a standard check for diagnosis. Patients often visit a general practitioner for an initial examination. The doctor will then perform a diagnostic exam such as rontgent and if necessary MRI scans. If allegedly suffering from ALS, the patient is then referred to a Neurologist.
The neurologist will recommend a full body examination and include the neurological (but not limited to:
- Lumbar puncture to obtain spinal fluid samples
- Nerve conduction study (NVS), which measures the rate at which neural signals are transmitted to the nerve to detect nerve or deterioration.
- Cervical spine myelogram is an imaging test by inserting a contrast material (or dye) in the spinal canal (spinal canal) using a spinal needle
- Neural tissue sampling (biopsy)
- Thyroid Test
- Urinalysis (urine examination)
If a patient is positively exposed to ALS, a neurologist will immediately undertake a management and care plan. So far, Rilutek (riluzole) is the only drug approved by the FDA for use in ALS patients. This drug is a glutamate inhibitor that will help patients prolong survival for several months and improve their ability to chew and swallow them.
Other treatments are used primarily to relieve symptoms. For example, drugs such as tinzanadine may be given to reduce spasticity (muscle stiffness) while gabapentin helps with pain management. If the patient has difficulty swallowing saliva, the doctor may also prescribe amitriptyline.
The ALS Association conducts various studies and experiments to determine the cause and treatment of the disease effectively. The $ 100 million they have received from the Ice Challenge Event, for example, will be used to initiate ALS ACT (ALS Accelerated Therapeutics) in the search for a treatment that can reduce inflammatory processes in nerve cells and reduce the production of certain proteins.
As the disease progresses, the neurologist can suggest the following:
- Respiratory devices and therapies such as CPAP (continuous positive airway pressure) and BiPAP (bi-level positive airway pressure)
- Physical therapy can consist of low intensity exercise or light stretching to prevent rapid muscle shrinkage, which is exacerbated by sitting or lying down for long.
- PEG (percutaneous endoscopic gastronomy), is a channel inserted into the stomach to facilitate feeding and prevent choking.
- Speech therapy
- Wheelchairs or walkers to move
- A high-calorie diet that is easy to eat.
Many of the ALS patients are depressed. Although antidepressants may be helpful, counseling and familial support is necessary for the patient.
Doctors from patients should also work closely with other healthcare professionals such as ENT doctors, nutritionists, psychologists and therapists when making and changing treatment and treatment.
The patient’s family also needs to adjust their lifestyle to improve the quality of life further. They can create a gentle staircase or one level home with large house planning for quick and easy movement. Patients can also benefit from health care providers such as nurses who can quickly serve their particular medical needs at home.