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Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a nervous system disease that attacks nerve cells called neurons in your brain and spinal cord. These neurons transmit messages from your brain and spinal cord to your voluntary muscles - the ones you can control, like in your arms and legs. At first, this causes mild muscle problems. Some people notice:

  • Trouble walking or running
  • Trouble writing
  • Speech problems

Eventually, you lose your strength and cannot move. When muscles in your chest fail, you cannot breathe. A breathing machine can help, but most people with ALS die from respiratory failure.

The disease usually strikes between age 40 and 60. More men than women get it. No one knows what causes ALS. It can run in families, but usually it strikes at random. There is no cure. Medicines can relieve symptoms and, sometimes, prolong survival.

NIH: National Institute of Neurological Disorders and Stroke

Respiratory Failure

What is respiratory failure?

Respiratory failure is a condition in which your blood doesn't have enough oxygen or has too much carbon dioxide. Sometimes you can have both problems.

When you breathe, your lungs take in oxygen. The oxygen passes into your blood, which carries it to your organs. Your organs, such as your heart and brain, need this oxygen-rich blood to work well.

Another part of breathing is removing the carbon dioxide from the blood and breathing it out. Having too much carbon dioxide in your blood can harm your organs.

What causes respiratory failure?

Conditions that affect your breathing can cause respiratory failure. These conditions may affect the muscles, nerves, bones, or tissues that support breathing. Or they may affect the lungs directly. These conditions include:

  • Diseases that affect the lungs, such as COPD (chronic obstructive pulmonary disease), cystic fibrosis, pneumonia, pulmonary embolism, and COVID-19
  • Conditions that affect the nerves and muscles that control breathing, such as amyotrophic lateral sclerosis (ALS), muscular dystrophy, spinal cord injuries, and stroke
  • Problems with the spine, such as scoliosis (a curve in the spine). They can affect the bones and muscles used for breathing.
  • Damage to the tissues and ribs around the lungs. An injury to the chest can cause this damage.
  • Drug or alcohol overdose
  • Inhalation injuries, such as from inhaling smoke (from fires) or harmful fumes
What are the symptoms of respiratory failure?

The symptoms of respiratory failure depend on the cause and the levels of oxygen and carbon dioxide in your blood.

A low oxygen level in the blood can cause shortness of breath and air hunger (the feeling that you can't breathe in enough air). Your skin, lips, and fingernails may also have a bluish color. A high carbon dioxide level can cause rapid breathing and confusion.

Some people who have respiratory failure may become very sleepy or lose consciousness. They also may have arrhythmia (irregular heartbeat). You may have these symptoms if your brain and heart are not getting enough oxygen.

How is respiratory failure diagnosed?

Your health care provider will diagnose respiratory failure based on:

  • Your medical history
  • A physical exam, which often includes
    • Listening to your lungs to check for abnormal sounds
    • Listening to your heart to check for arrhythmia
    • Looking for a bluish color on your skin, lips, and fingernails
  • Diagnostic tests, such as
    • Pulse oximetry, a small sensor that uses a light to measure how much oxygen is in your blood. The sensor goes on the end of your finger or on your ear.
    • Arterial blood gas test, a test that measures the oxygen and carbon dioxide levels in your blood. The blood sample is taken from an artery, usually in your wrist.

Once you are diagnosed with respiratory failure, your provider will look for what is causing it. Tests for this often include a chest x-ray. If your provider thinks you may have arrhythmia because of the respiratory failure, you may have an EKG (electrocardiogram). This is simple, painless test that detects and records your heart's electrical activity.

What are the treatments for respiratory failure?

Treatment for respiratory failure depends on:

  • Whether it is acute (short-term) or chronic (ongoing)
  • How severe it is
  • What is causing it

Acute respiratory failure can be a medical emergency. You may need treatment in intensive care unit at a hospital. Chronic respiratory failure can often be treated at home. But if your chronic respiratory failure is severe, you might need treatment in a long-term care center.

One of the main goals of treatment is to get oxygen to your lungs and other organs and remove carbon dioxide from your body. Another goal is to treat the cause of the condition. Treatments may include:

  • Oxygen therapy, through a nasal cannula (two small plastic tubes that go in your nostrils) or through a mask that fits over your nose and mouth
  • Tracheostomy, a surgically-made hole that goes through the front of your neck and into your windpipe. A breathing tube, also called a tracheostomy, or trach tube, is placed in the hole to help you breathe.
  • Ventilator, a breathing machine that blows air into your lungs. It also carries carbon dioxide out of your lungs.
  • Other breathing treatments, such as noninvasive positive pressure ventilation (NPPV), which uses mild air pressure to keep your airways open while you sleep. Another treatment is a special bed that rocks back and forth, to help you breathe in and out.
  • Fluids, often through an intravenous (IV), to improve blood flow throughout your body. They also provide nutrition.
  • Medicines for discomfort
  • Treatments for the cause of the respiratory failure. These treatments may include medicines and procedures.

If you have respiratory failure, see your health care provider for ongoing medical care. Your provider may suggest pulmonary rehabilitation.

If your respiratory failure is chronic, make sure that you know when and where to get help for your symptoms. You need emergency care if you have severe symptoms, such as trouble catching your breath or talking. You should call your provider if you notice that your symptoms are worsening or if you have new signs and symptoms.

Living with respiratory failure may cause fear, anxiety, depression, and stress. Talk therapy, medicines, and support groups can help you feel better.

NIH: National Heart, Lung, and Blood Institute

Degenerative Nerve Diseases

Degenerative nerve diseases affect many of your body's activities, such as balance, movement, talking, breathing, and heart function. Many of these diseases are genetic. Sometimes the cause is a medical condition such as alcoholism, a tumor, or a stroke. Other causes may include toxins, chemicals, and viruses. Sometimes the cause is unknown.

Degenerative nerve diseases include:

  • Alzheimer's disease
  • Amyotrophic lateral sclerosis
  • Friedreich ataxia
  • Huntington's disease
  • Lewy body disease
  • Parkinson's disease
  • Spinal muscular atrophy

Degenerative nerve diseases can be serious or life-threatening. It depends on the type. Most of them have no cure. Treatments may help improve symptoms, relieve pain, and increase mobility.

Medicare

Medicare is the U.S. government's health insurance program for people age 65 or older. Some people under age 65 can qualify for Medicare, too. They include those with disabilities, permanent kidney failure, or amyotrophic lateral sclerosis.

Medicare helps with the cost of health care. It does not cover all medical expenses or the cost of most long-term care. The program has four parts:

  • Part A is hospital insurance
  • Part B helps pay for medical services that Part A doesn't cover
  • Part C is called Medicare Advantage. If you have Parts A and B, you can choose this option to receive all of your health care through a provider organization, like an HMO.
  • Part D is prescription drug coverage. It helps pay for some medicines.

Neuromuscular Disorders

Neuromuscular disorders affect your neuromuscular system. They can cause problems with:

  • The nerves that control your muscles
  • Your muscles
  • Communication between your nerves and muscles

These disorders can cause your muscles to become weak and waste away. You may also have symptoms such as spasms, twitching, and pain.

Examples of neuromuscular disorders include:

  • Amyotrophic lateral sclerosis
  • Muscular dystrophy
  • Myasthenia gravis
  • Spinal muscular atrophy

There can be different causes for these diseases. Many of them are genetic.This means they are inherited (run in families) or are caused by a new mutation in your genes. Some neuromuscular disorders are autoimmune diseases. Sometimes the cause is unknown.

Many neuromuscular diseases have no cure. But treatments may improve symptoms, increase mobility, and lengthen life.

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