Condition Basics

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS) damages the nerves in the brain and spinal cord. These nerves control the ability to move muscles. ALS slowly gets worse. Over time, it often becomes harder to walk, speak, eat, swallow, and breathe. But some people live for many years, even decades, after they learn that they have ALS.

Finding out that you have ALS may be overwhelming. You may feel many emotions and may need some help coping. Seek out family, friends, and counsellors for support. Treatment for ALS helps you stay active as long as possible. Your doctor may prescribe medicine to prevent muscle cramps or stiffness, improve appetite, and relieve depression and pain.

ALS is also known as Lou Gehrig's disease or motor neuron disease.

What causes it?

Doctors don't know what causes ALS. In about 1 case out of 10, it runs in families.^{footnote 1} This means that 9 times out of 10, a person with ALS doesn't have a family member with the disease.

What are the symptoms?

The first sign of ALS is often weakness in one leg, one hand, the face, or the tongue. The weakness slowly spreads to both arms and both legs. This happens because as the motor neurons slowly die, they stop sending signals to the muscles. So the muscles don't have anything telling them to move. Over time, with no signals from the motor neurons telling the muscles to move, the muscles get weaker and smaller.

Over time, ALS also causes:

• Muscle twitching.
• Trouble using your hands and fingers to do tasks.
• Problems with speaking, swallowing, eating, walking, and breathing.
• Problems with memory, thinking, and changes in personality. But these are not common.

ALS doesn't cause numbness, tingling, or loss of feeling.

How is it diagnosed?

To find out if you have ALS, your doctor will do a physical examination and will ask you about your symptoms and past health. You will also have tests that show how your muscles and nerves are working.

It can be hard for your doctor to tell if you have ALS. It may not be clear that you have the disease until symptoms get worse or until your doctor has done more testing.

Just because you have muscle weakness, fatigue, stiffness, and twitching doesn't mean that you have ALS. Those symptoms can also be caused by other conditions. So talk to your doctor if you have those symptoms.

Tests to confirm ALS or look for other causes of your symptoms include:

• Electromyogram (EMG), which helps measure how well nerves and muscles work.
• Magnetic resonance imaging (MRI), which can show problems or injury in the brain.
• Nerve conduction studies to test nerve function.

If your doctor thinks that you have ALS, he or she will refer you to a neurologist to make sure.

How is ALS treated?

There is no cure for ALS, but treatment can help you stay strong and independent for as long as possible. Treatments may include:

Physiotherapy and occupational therapy.

These therapies can help you stay strong and make the most of the abilities you still have.

Speech therapy.

This can help you with coughing, swallowing, and talking after weakness in the face, throat, and chest begins.

Supportive devices and equipment.

These can help you stay mobile, communicate, and do daily tasks like bathing, eating, and dressing. Some examples are canes, walkers, wheelchairs, ramps, handrails, raised toilet seats, and shower seats. You can also get braces to support your feet, ankles, or neck.

Medicines.

These may help slow the progression of ALS, relieve your symptoms, and keep you comfortable. There are medicines that can help with many of the symptoms you might have. These symptoms may include muscle problems (stiffness, cramps, twitching), drooling and extra saliva, depression and mood swings, and pain.

A feeding tube.

This can help you get enough nutrition to stay strong as long as you can.

Breathing devices.

They can help you breathe more easily as your chest muscles weaken.

Hospice palliative care

Hospice palliative care is a type of care for people who have a serious illness. It's different from care to cure your illness, called curative treatment. Hospice palliative care provides an extra layer of support that can improve your quality of life—not just in your body, but also in your mind and spirit. Sometimes hospice palliative care is combined with curative treatment.

The kind of care you get depends on what you need. Your goals guide your care. You can get both hospice palliative care and care to treat your illness. You don't have to choose one or the other.

Hospice palliative care can help you manage symptoms, pain, or side effects from treatment. It may help you and those close to you better understand your illness, talk more openly about your feelings, or decide what treatment you want or don't want. It can also help you communicate better with your doctors, nurses, family, and friends.

How can you care for yourself?

• Exercise and stretch your muscles as long as you can. Talk to a physiotherapist about exercises you can do.
• Eat small, frequent meals. Choose soft foods that are easy to swallow. Try to sit up when you eat. Eat slowly.
• Learn about devices that can help you avoid injury and stay independent:
  • A neck (cervical) collar can support your head if the neck muscles get weak.
  • Foot and ankle braces, a cane, a walker, or a wheelchair can help you move around.
  • A ramp over stairs can allow you to get into and out of your house in a wheelchair.
  • Handrails or a shower seat can keep you from falling in the shower. A higher toilet seat can help you use the toilet by yourself.
  • If you have trouble talking, a voice amplifier or an erasable writing pad can help you communicate.
• Consider joining a support group. Sharing your experiences with other people who have the same problem may help you learn more and cope better.
• Be safe with medicines. Take your medicines exactly as prescribed. Call your doctor or nurse advice line if you think you are having a problem with your medicine.
• If you have not already done so, prepare an advance care plan. This is instructions to your doctor and family members about what kind of care you want if you become unable to speak or express yourself.

What decisions will you face as ALS progresses?

As ALS symptoms get worse (progress), you may have to choose which treatments you want for issues like breathing and eating problems. For example, would you consider using a machine to help you breathe if your breathing problems become severe? Do you want a feeding tube placed in your stomach if you are losing weight, unable to get enough calories by eating, or if you lose your ability to swallow?

As you make these choices, keep in mind that what's right for one person with ALS may not feel right for another. It's also okay to revisit your choices throughout the course of the disease. You may change your mind over time. Be sure to talk about your treatment options and share your concerns with your doctor.

If you or a family member has ALS, learn as much as you can about the disease and how to take care of it. How much treatment you want for the problems caused by ALS is a personal choice that only you and your loved ones can make. Your values, wants, and needs are important things to think about as you make choices about your care.

Planning ahead

You may want to put your health care choices in writing. This is called an advance care plan. It gives you control over your own medical care when you can't make decisions or speak for yourself. You may also want to choose a friend or family member to speak for you. This is called a substitute decision-maker. Making these plans ahead of time will help make sure that your health care choices are followed.