Nasal regurgitation — instances when food or fluids you’ve swallowed come up through your nose — can be scary for people living with spinal muscular atrophy (SMA) and their caregivers.

SMA can range in severity across its different types, but it tends to cause weakness in the muscles that are involved in the basic life functions of eating and breathing. Living with a neuromuscular disease such as SMA can cause dysfunction in your ability to chew, swallow, and suck — all of which are necessary for proper food and drink consumption and nutrition.

Read on to learn more about what nasal regurgitation is, what causes it in SMA, and ways to best manage it with your providers.

What Is Nasal Regurgitation?

Nasal regurgitation is a type of swallowing problem. The medical term for problems with swallowing is “dysphagia.” Nasal regurgitation happens when food or fluids that you’ve consumed travel backward into the nose. Usually, this occurs when the nasopharynx — the part of the throat connecting to the nasal cavity — fails to close completely due to nerve and muscle abnormalities.

Specifically, nasal regurgitation happens due to problems with coordinating contractions of the pharyngeal muscles (a group of muscles in the throat). In a motor neuron disease like SMA, muscle function is impaired due to damage to the central nervous system, leading to impairments in swallowing.

Experiencing nasal regurgitation can be quite traumatic and even painful. Sometimes, there are symptoms over a longer period of time that can indicate that there may be a swallowing problem, including:

• Sore nose or sore throat
• Coughing during swallowing
• Nasal irritation or hoarseness
• Trouble breathing/respiratory distress
• Chest pain
• Difficulty making food and liquids into a bolus (soft ball) while chewing
• Difficulty pushing food to the back of the mouth
• Needing extra time to chew
• Reflux or burning sensations
• Vomiting

If you or your loved one with SMA is experiencing any of these symptoms, it’s time to seek guidance from a doctor who specializes in neuromuscular disorders.

What Causes Nasal Regurgitation in SMA?

SMA is a complex disease with multiple types and varying degrees of severity. In severe forms of SMA type 1, progressive muscle weakness can affect a person’s throat muscles. This can cause infants to aspirate, meaning they accidentally breathe in fluids or foods backward into the lungs.

Aspiration is dangerous because it can lead to life-threatening choking and infections, such as aspiration pneumonia. People living with SMA type 1 usually lose all swallowing functions before even starting on puree and solid food intake. Therefore, they are at high risk of aspiration.

That being said, people living with SMA types 2 and 3 may also experience difficulties in swallowing. In fact, in a study of 108 people with SMA types 2 and 3, 31 percent of participants reported experiencing problems with choking on food and/or liquids. Seventeen percent said they experienced dysphagia with foods and/or liquids.

On the other hand, people living with the mildest form of SMA — spinal muscular atrophy type 4 — might not have nasal regurgitation symptoms until much later in life. At that point, symptoms are typically mild and are related to mouth opening.

Treatment for Nasal Regurgitation

Until 2016, no specific treatments were available to treat the muscle degeneration leading to nasal regurgitation in SMA. In 2015, the U.S. Food and Drug Administration (FDA) approved Spinraza (a formulation of nusinersen) as a disease-modifying therapy for SMA, changing how doctors treat the condition. This has led to improved survival among people with SMA by slowing disease progression.

As survival rates for people with SMA increased, doctors started taking a rehabilitation approach to treating swallowing problems. Other treatment options then became available, including gene therapy onasemnogene abeparvovec-xioi (Zolgensma) and the medication risdiplam (Evrysdi). From there, doctors shifted their focus to prescribing newer medications that could improve overall quality of life for people with SMA, including their ability to swallow.

People with SMA type 1 typically still need nutrition support and assistance managing their airway secretions for breathing. Treatment is usually in combination with various types of equipment to help with respiratory muscle impairments.

Treating nasal regurgitation and swallowing disorders involves more than just medicine. Generally speaking, treatment for swallowing difficulties involves a combination of the following:

• Changes in diet
• Use of feeding tubes
• Swallowing therapy to work on muscle strength and chewing and swallowing techniques

Although certain SMA treatments show promise in helping with swallowing difficulties, the current research on their effectiveness is limited. Nevertheless, research has shown that people with SMA who are not taking a disease-modifying therapy have significant challenges in their swallowing function.

There are other important interventions available to treat nasal regurgitation, depending on the severity of the symptom. In severe cases, certain types of feeding tubes may be needed, including temporary nasal feeding tubes (called nasogastric tubes) and permanent gastrostomy tubes — also called percutaneous endoscopic gastrostomy, or PEG, tubes.

Speech-language pathology also plays an important role. Speech-language pathologists are health care professionals who are part of a rehab team to help with speech, language, and swallowing disorders. They play an integral role in helping people with SMA types 2-4 by instructing on diet modification, eating postures, and even stretching some of the joints involved in chewing.

Left untreated, nasal regurgitation not only increases a person’s risk of choking and infection but can also lead to dehydration or malnutrition.

If you are concerned about nasal regurgitation for yourself or a loved one, it’s important to talk to clinicians who have experience with muscular disorders as soon as possible. They will likely perform an endoscopy — a procedure that can take a video of your gastrointestinal tract — to help diagnose exactly what is causing your nasal regurgitation to help guide your treatment plan.

Talk With Others Who Understand

MySMAteam is the social network for people with SMA and their loved ones. On MySMAteam, more than 2,000 members come together to ask questions, give advice, and share their stories with others who understand life with SMA.

Are you living with SMA and concerned about nasal regurgitation? Share your experience in the comments below, or start a conversation by posting on your Activities page.