August Honors the Spinal Muscle Atrophy (SMA) Community
Creating awareness since 1996. The tight-knit community works year-round to raise awareness and funds. We’re looking to help you participate in collaboration with Cure SMA by highlighting a few wellness facts, tips, and fun.
What is Spinal Muscle Atrophy?
According to Cure SMA, “Spinal muscular atrophy (SMA) is a disease that robs people of physical strength by affecting the spinal cord’s motor nerve cells, taking away the ability to walk, eat, or breathe.” SMA is the #1 genetic cause for infantile death.
What are the types of SMA?
According to the Cleveland Clinic, there are four different types of this disease.
- Type 1 (severe) SMA: Progresses rapidly
- Evident between ages 0-6 months; death normally by age of 2
- Floppy limbs and weak trunk movement
- Very limited ability to move
- Hard time swallowing, feeding, breathing, and holding head up
- Type 2 (intermediate) SMA: Progression rate varies
- Occurs between 7-18 months
- Affects the legs more than the arms
- Children will never stand
- Respiratory infections are very common
- Life expectancy from early childhood to adulthood; pending severity of the condition
- Type 3 (mild) SMA:
- 18 months to early adulthood
- May stand or walk, but have trouble getting up
- Mild muscle weakness
- Greater risk for respiratory infections
- Close to normal life expectancy
- Type 4 (adult) SMA:
- Can walk, but progressive muscle weakness and other typical symptoms may appear in the second or third decade of life
What happens in the body?
Motor neurons are slowly destroyed, which wreaks havoc on skeletal muscle activity. Eventually, muscles start to weaken and begin to waste away, causing a twitch (known as fasciculations). Limbs, chest, and throat are highly affected.
What causes this disease?
This hereditary disease is caused by a mutation in the survival motor neuron gene 1 (SMN1). A healthy person will typically produce a protein essential for the function of the nerves which control our muscles. Without the protein, those nerves cannot function correctly and eventually die, leading to muscle weakness (which can be fatal).
What nutritional challenges occur with SMA?
Due to SMA’s nature, the consumption of food from start to finish is an everyday challenge. People with SMA struggle to chew, swallow and tend to aspirate or have gastrointestinal problems like diarrhea,
bloating, or constipation). Some even may have gastroesophageal reflux disease (GERD) or acid reflux, yeast overgrowth, or an imbalance in blood sugar.
People with SMA should work with their health professional – physician, SMA Specialist, and nutritionist – to customize an individualized diet based on their motor function skills. The goal of this ‘dynamic trio’ is to balance calories, fat, carbohydrates, and protein, all while gaining vitamin and mineral supplements into the body.
Nutrition and exercise are essential for children with SMA. Poor nutrition can lead to further weakening of muscles. There is not a specific diet that supports SMA patients. However, a balanced diet is believed to help promote healthy breathing and the immune system.
One parent’s experience helps shine a light on challenges with nutrition
One father, Michael Casten speaks to the challenges of nutrition with his daughter, Ella, who struggles with SMA. He claims that Ella has an increased amount of difficulty digesting long-chain fats (meats and cheeses) and chewing for long periods. She began the ‘Amino Acid Diet’ early in life, which has helped her gain weight and digest essential minerals and vitamins to keep her healthy. Ingredients are mixed up and ingested via a G-tube as explained:
He says, “The mixture recipe varies among people who use it, but in general, there is Tolerex elemental powder, Vivonex Plus elemental powder, Himalayan salt, ubiquinol CoQ10, safflower oil, levocarnitine, vitamin D, NanoVM, MiraLAX, water, Pedialyte, and white grape juice. Sometimes high-calorie baby food is added.”
He later describes making modifications to fit Ella’s needs. Thank you for putting this article out into the internet, Michael. We appreciate the information and hope, passing it along to our readers helps!
More informational links for nutritional needs here:
What exercises help SMA?
There is no physical evidence that states this treatment helps SMA. Patients need to work with a trained occupational therapist to practice safe exercise and to ensure mobility and maintain a quality of life. Many patients utilize tools during exercise support, like orthotic braces or adaptive aids. It’s essential for SMA patients not to over-exercise and to tailor a program to their needs.
For those that are able, physical activity and exercise might help improve muscle and cardiorespiratory function:
- Stretching might be the most effective exercise to try. It helps with improving mobility and range of motion.
- Aerobic exercise builds lung capacity and strength.
- Aerobic activities might help counteract the muscle deterioration that occurs secondarily. Walking and swimming are types of aerobic exercises that may help.
- Resistance Training helps to build muscle up strength. Programs utilize weighted tools such as medicine balls, strength bands, and free weights.
Where can I find caregiver support groups in Williamson County?
We encourage you or your loved one to reach out to one of the many Williamson County Caregiver Support Groups to find peace of mind, offer or receive advice, and be amongst like-minded individuals. You can find a list of organizations online below that offer support as well:
- Living with SMA – Celebrate Empowerment
- Cure SMA
- Spinal Muscular Atrophy Support Groups by Healthline
- Reeve Foundation: SMA
- Children with SMA Support Group
- Never Give Up with Gwendolyn Strong Foundation
- Pediatric Brain Foundation
- Thisbe and Noah Scott Foundation, Inc.
Oh! Tag “#SMAAwarenessMonth” in your social media outreach to help support.
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