Caring for a Child with Spinal Muscular Atrophy

January 20, 2021
Janelle Thomas MSN, RN
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If your child has been diagnosed with spinal muscular atrophy, you may still be trying to process the information and what it all means, while at the same time looking for ways of making your baby’s life as comfortable as possible. Are there any things they will be able to do for themselves? What type of caregiving will they require? You may also be wondering if the condition will mean that they will never be able to lead an independent life. Turns out, it depends on the type of spinal muscular atrophy, as well as on the severity of the condition.

Overview of Spinal Muscular Atrophy

Spinal muscular atrophy (SMA) occurs when the SMN1 gene doesn’t make enough of a protein that’s needed for motor neurons (nerve cells) in the brain and spinal cord to work properly. As a result, the brain is unable to send signals that control the movement of muscles. When a child has SMA, many of their muscles shrink from underuse. In the beginning, this may make bigger movements — such as sitting up and walking — difficult. But, as the disease progresses and the child gets older, the child may find it hard and/or painful to even breathe and swallow.

While spinal muscular atrophy makes it difficult for a child to move, it has no effect on their brain’s cognitive and intellectual abilities. Therefore, your child will still be able to understand conversations and build relationships with others.

Causes of Spinal Muscular Atrophy

Spinal muscular atrophy is a genetic disorder. A child gets one copy of the SMN1 gene from each parent. If only one parent has the disorder, the child will likely not experience SMA. However, they can pass it on to their own children. If, on the other hand, they get the condition from both parents, symptoms of SMA will develop.

Types of Spinal Muscular Atrophy

There are several types of SMA. The symptoms your child experiences will depend on the variant of the condition they develop.

Type 0 Spinal Muscular Atrophy

Type 0 SMA is the most severe type of spinal muscular atrophy. It develops while the baby is still in the womb — causing them to move less during pregnancy. Babies who are born with Type 0 SMA have joint problems and very weak muscles. As a result, many do not survive, due to the condition making it too difficult for them to breathe.

Type 1 Spinal Muscular Atrophy

Type 1 SMA is also severe and makes it difficult for a child to support their head or sit up without help and without leaning on something. Just as with Type 0 SMA, Type 1 eventually makes it difficult for a baby to breathe. For this reason, most children with the condition don’t live past infancy.

Type 2 Spinal Muscular Atrophy

Type 2 SMA often develops in children up to 18 months of age. It can range from mild to severe. This means that you may not notice anything at the beginning, since your child may be able to sit up and learn how to stand and walk on their own. However, you may notice feeding and breathing difficulties.

Type 3 Spinal Muscular Atrophy

Type 3 SMA (Kugelberg-Welander disease) is one of the mildest modalities of spinal muscular atrophy. While the condition is genetic, a child may not experience any difficulties with their muscles until they’re between two and 17 years of age. Many are able to walk normally, but find it difficult to get up from a sitting position, go up flights of stairs, and run. Eventually, they may need a wheelchair.

Type 4 Spinal Muscular Atrophy

Type 4 SMA manifests in adult patients. The first muscles to feel weak tend to be the limbs — although physical therapy may help ease symptoms and help a person lead a relatively independent life. People with Type 4 SMA may also experience twitching and breathing difficulties.

Caring for a Child with Spinal Muscular Atrophy

Which type of treatment a child will need depends on the type and severity of SMA. The most common forms include:

1. Medications

Spinraza is an FDA-approved medication that increases the amount of protein that should be created by the SMN1 gene. Regular doses spread out over several months may help your child breathe easier and utilize their motor functions better. This type of medication is administered through a spinal tap.

2. Managing Breathing

Being unable to breathe is the main cause of death for children with severe SMA. Therefore, before taking your child home from the hospital, learn how to clean their airways — and learn it well enough so that you can teach it to family members who may assist you with caregiving. Also, learn how to clear mucus to prevent complications from infections.

3. Creating a Nutritional Plan

Since some types of spinal muscular atrophy make it difficult to swallow, children with SMA have a difficult time gaining and maintaining a healthy weight. If this is the case with your child, it’s crucial to design a nutritional plan that works best for them — and that all caregivers are familiar with.

4. Physical and Occupational Therapy

Although wheelchairs and orthotics are common devices used to assist your child with mobility, physical and occupational therapy will help promote muscle strength. Stronger muscles can help promote an independent lifestyle even if they depend on a wheelchair or orthotics for mobility.

5. Medical Intervention

Children with spinal muscular atrophy may likely need a tracheostomy and a G-tube. They may also need to wear a splint or brace if they develop scoliosis. If SMA causes scoliosis to become severe, your child may also need to undergo surgery.

Contact Care Options for Kids for Home Health Care

It can be hard to balance your time between work, home, and caring for a child. That’s why our team of professionals at Care Options for Kids is here to help.

Our home health care services offer support in the comfort of your home. We refer loving and competent nurses to provide customized care for families — from a few hours a day to around-the-clock supervision. Contact us directly to speak with a home health care professional or request a free in-home assessment. Together we can determine the best plan of action to keep your loved ones happy and healthy.

If you or a loved one are considering Pediatric Home Health Care Services, contact the caring staff at Care Options for Kids. Call today at (888) 592-5855.