What is Spinal Muscular Atrophy?

What is Spinal Muscular Atrophy

Awareness is one of the most effective tools you have as a family caregiver. This tool gives you the ability to react to new challenges and demands within your senior care journey in a way that is effective, personalized, and fits with your and your elderly loved ones’ care goals.

For some seniors who deal with inactivity due to physical, balance, cognitive, or other challenges, one risk that they may face is spinal muscular atrophy. As their family caregiver, being aware of spinal muscular atrophy, commonly abbreviated as SMA, and its issues can help you to respond effectively.

Unless you know someone with spinal muscular atrophy, it’s quite possible you’ve never heard of the disease. Yet, 1 in 50 people carries the disease and 1 out of every 6,000 to 10,000 people develop it.

If you have a family member with SMA, learning more about the disease can help you be prepared for the future and ensure they receive the best possible care.

What is Spinal Muscular Atrophy?

SMA is a disease that affects the body’s ability to control muscle movements by attacking and destroying the lower motor neurons.

The condition is characterized by muscles that are shrinking due to extended inactivity, however, this condition does not occur in all people who are inactive. Instead, it is a genetic, progressive disease that directly impacts the motor neurons of the brain stem and spinal cord.

Taking a closer look at the name of the disease can be helpful in understanding it. The majority of the nerve cells that are responsible for controlling muscles are found in the spinal cord, thus the word “spinal.” The word “muscular” refers to the fact that the disease affects the muscles and the word “atrophy” describes how the muscles are affected. The muscles become smaller, or atrophy, because of inactivity.

Causes of Spinal Muscular Atrophy

SMA is a genetic disease caused by a defective SMN1 gene. The gene is responsible for creating a protein that is needed for healthy motor neurons. When there isn’t enough of the SMN protein, the lower motor neurons lose vitality and muscles grow weak and waste away. The trunk and upper arms and legs are most affected by the disease.

Types of Spinal Muscular Atrophy

There are four types of SMA. Spinal muscular atrophy is categorized using the age of onset, the severity of the symptoms, and the progression of the disease.

The four types of spinal muscular atrophy are:

Type I: Type I SMA is also called Werdnig-Hoffman disease. This form appears at birth or shortly after. Most children affected with Type I SMA are unable to sit upright or hold their heads up. They also have developmental delays.

Type II: Type II SMA becomes apparent between 6 and 12 months of age. People with this type are able to sit upright but might need help to get into position. However, they cannot stand or walk without help.

Type III: Type III SMA is also called Kugelberg-Welander disease and develops between the ages of 2 and 17 years. Individuals with this type walk with an abnormal gait and have trouble running or climbing steps. They may suffer from frequent respiratory infections but generally live a normal lifespan.

Type IV: Type IV SMA occurs most frequently in adults over the age of 40.

Symptoms of SMA Type IV include:

  • Progressive limb weakness that usually begins in the muscle groups of the thighs and then the upper arms
  • Slow, but progressive muscle wasting
  • Decreased reflexes in the deep tendons
  • Very slow decrease in the ability to walk or move the lower limbs
  • Cramping or marked stiffness in the muscles
  • Twitching or spasms in the muscles
  • Marked weakness

Kennedy’s Disease: Aside from the main types, there are other forms of SMA. One of them is called Kennedy’s disease and occurs somewhere between the ages of 15 and 60 years. The disease is progressive and usually starts with weakened muscles in the face, tongue, and jaw. Over time, the muscles in the arms and legs also become weak and the person loses feeling in their hands and feet.

Diagnosis of Spinal Muscular Atrophy

Spinal muscular atrophy is a rare disease, meaning that many medical professionals are not necessarily familiar with the condition. If your seniors are at risk of this disease, it is vital that you seek out assistance from the right professionals.

Diagnosis is through blood testing to detect a specific gene, a muscle biopsy, or electromyography-electrical testing of the muscles.

Treatment of Spinal Muscular Atrophy

Generally, your treatment team will include a variety of professionals, including:

– Occupational therapists
– Neurologists
– Physiotherapists
– Dieticians
– Exercise therapists

These professionals can work with your parent after diagnosis to create an approach that involves muscle stretching exercises, pain relief approaches, muscle stimulation, and activities that encourage ongoing muscular health.

The most important thing to keep in mind when it comes to caring for a senior coping with SMA is preventing further muscle loss and maintaining the health of the muscle that is still left.

While there is no cure for this disease, using these approaches can help to slow the progression of the disease and keep the body healthier and stronger for longer.

Home Health Care Services in Florida

If your family member has been diagnosed with SMA, home health care services can be extremely beneficial in allowing the person to remain at home.

A home health care provider can help with dressing, bathing, and personal care. They can also perform light housecleaning, cook meals, and do laundry, which may become difficult for your family member as muscles atrophy.

When you hire a home health care provider through an agency, you can specify how often you would like the home care provider to visit the house. They can come daily or a few times per week. They can also come for just a few hours or for a full day. Home care providers can also be hired to stay overnight.

If you or an aging loved-one are considering hiring home health care services in Florida, contact the caring staff at Sonas Home Health Care today. (888) 592-5855

Sources:
https://www.mda.org/disease/spinal-muscular-atrophy
https://ghr.nlm.nih.gov/condition/spinal-muscular-atrophy
https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Spinal-Muscular-Atrophy-Fact-Sheet
http://www.counsyl.com/diseases/spinal-muscular-atrophy/

Conversations