Als Definition : What Is Als Disease (Amyotrophic Lateral Sclerosis)?

What is ALS (Amyotrophic Lateral Sclerosis)?

ALS stands for amyotrophic lateral sclerosis. It is a rare disease of the muscles and the nervous system. In ALS, nerve cells give less and less requests to the muscles. Thereby eventually become paralyzed or shrink them.

ALS usually begins with fatigue, muscle cramps and small muscle contractions. Then the muscles become weaker and they shrivel. This often begins in a hand or foot. Slowly weaken and shrivel more and more muscle.
People with ALS disease sometimes have no reason to cry, laugh or yawn. Ultimately weaken the throat muscles and get someone difficulty speaking and swallowing. The disease is usually worse rapidly.

ALS usually begins between the fiftieth and sixtieth year. It is more common in men than in women.
In most cases, it is not known why a person will get the disease. In five to ten of the hundred ALS patients is a mistake in the genes. The disease is inherited dominantly.

ALS is sadly incurable. There is a drug that slows the disease. Proper medical supervision is very important. Home care can assist in the care and housekeeping. If food is too difficult, you can grind or the sending of a PEG tube in food. In distress, you can choose (home) for respiratory complaints what to cut.

 ALS stands for amyotrophic lateral sclerosis ALS Definition : What Is ALS Disease (Amyotrophic Lateral Sclerosis)?

What you will notice from ALS?

There are three types of ALS: typical ALS, progressive spinal muscular atrophy, progressive bulbar palsy.

Typical ALS begins with fatigue, muscle cramps and small muscle contractions. The muscle contractions cause a kind of wave-like movements on the skin (fasciculations). Cramps are contractions of a muscle that take 30 seconds or longer. They are sometimes painful, especially in the calves.
After that, more and more muscle weakness and atrophy them. Sometimes muscles (for example in the arms and legs), a rigid time before they weaken.
Also, people with typical ALS just cry, laugh or yawn without them there can do something about it.
After a time also weaken the throat muscles and gets the patient difficulties with speech and swallowing. Many patients choke constantly. They can also get trouble breathing. Thus one can become very drowsy and often have headaches.
The disease progresses rapidly. People who once symptoms typical of ALS, live on average three years.

Progressive spinal muscular atrophy is a form of Sekolah Menengan Atas (spinal muscular atrophies). This disease is similar to typical ALS, but is slightly slower. Also, patients are not stiff and they should not laugh, cry or yawn, as with typical ALS.

With progressive bulbar palsy, the muscles of the throat and face the first paralyzed. Also, patients should just crying, laughing and yawning. The weakness is spreading slowly to other muscles. The rate at which this happens varies per person.

How does the doctor determine ALS?

Usually it takes long before a doctor discovers that someone has ALS. This is because there is no way to determine with certainty ALS. The doctor may ask you questions only about your symptoms and examine you physically. During the physical examination, they pay special attention to foreign muscle contractions. If necessary, they can provoke a reflex hammer.

A neurologist will then conduct research to determine if you have no other disease than ALS. She does an electromyogram (EMG) and sometimes an MRI scan.

Treatment for ALS disease (Amyotrophic Lateral Sclerosis)

There is no medicine or treatment which can cure. There is a drug (riluzole) that slows down the disease with a number of months. Probably works this means better if you start there before.

It is the treatment from fighting the symptoms. You will get by swallowing problems too little to eat or drink within, you can consult with a dietitian diet preparations. Many people with AS have a PEG-probe insertion.

In order to improve breathing, there are a few possibilities:
  1. Treatment with medicine for stuffiness.
  2. Rescue breathing with a device.
  3. No ventilation, but only airstacking, or a combination of rescue breathing and airstacking. Thus you keep your lungs as smoothly as possible.
Will you and your loved ones for difficult choices. How long do you want to continue with the treatment? Permanent ventilation can you still keep alive a long time, but you can probably not or hardly more talk. You have 24 hours of care needed. At home will that for most people do not go. It requires at least a lot of family caregivers. In a nursing home you can make in any case.
Talk on time about treatment possibilities as a ban or maybe euthanasia. Make sure your doctor and your family know what your needs are.

Living with ALS

The weakness in ALS is usually pretty quick worse. You run harder, you can use a walking aid. Weak muscles in the hands and arms do such as writing and washing impossible. There are several shower aids and writing aids.
With ALS, you must drink plenty of fiber-rich diet and exercise as much as possible to prevent clogging. Keep your jaw muscles as long as possible. A physical therapist can help you move.

Most ALS patients have difficulty speaking. A speech therapist can help with finding aids, such as sign language, facial expressions or tools when speaking.

People with ALS swallowed rapidly, which can be very scary. People who swallowed too much rather get pneumonia. A PEG tube is necessary if it is swallowed too much.

On average, people with ALS after the first symptoms three years. This is an average. About two out of ten people with ALS survive after the first symptoms for at least five years. And there are people who live ten or twenty years with ALS.

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