Spinal muscular atrophy (SMA) causes muscle weakness and muscle wasting, which can also occur in the respiratory (breathing) muscles. Muscle weakness surrounding the lungs, as well as other factors, can put people with SMA at risk of developing pneumonia.
Pneumonia is an infection of the lungs caused by viruses, bacteria, or fungi. People with SMA may also develop aspiration pneumonia — a form of the illness caused by the aspiration (inhalation) of food or drinks into the airways.
Although pneumonia can be scary, there are steps you can take to help prevent it while living with SMA.
There are several reasons why people diagnosed with SMA are at greater risk of other conditions like pneumonia, including:
A person with SMA may have severe weakness in the muscles surrounding the lungs, which can reduce the power and effectiveness of their coughs. A weak cough can make clearing the airways more difficult, which means that more bacteria and other substances can infiltrate and infect the lungs.
People diagnosed with SMA often have underdeveloped lungs. Underdeveloped lungs pose a danger because they are small. When pneumonia invades an underdeveloped lung, it can more easily take over the whole lung because there is less surface area to infect.
Alone, underdeveloped lungs or a weak cough can make a person more susceptible to pneumonia, and together, they raise the risk even more.
People diagnosed with SMA are also more likely to struggle with aspiration pneumonia. Aspiration pneumonia can occur when food or drink is inhaled into the lungs rather than swallowed into the stomach. People with SMA are at risk of aspirating (breathing into the airways) food when the muscles in their throat are not strong enough to properly swallow.
SMARD1 is a very rare type of SMA. It specifically pertains to the lungs and causes significant breathing problems and respiratory distress. Children with this diagnosis have weaker lungs than those diagnosed with SMA and may be in greater danger of pneumonia.
There are several actions you can take to avoid aspiration pneumonia and bacterial or viral pneumonia.
In order to avoid aspirating food or beverages, take the following precautions whenever you eat or drink:
Foods and beverages that are too thick and sticky — such as peanut butter — or foods that are too thin and runny — such as soup or juice — can cause issues with swallowing. Also be careful with foods that seem difficult to swallow, such as tough pieces of meat.
It is important to take your time when eating and drinking. Chew your food thoroughly before you swallow it. Rushing through a meal or beverage may cause you to splutter, choke, or inadvertently aspirate food or drinks.
Make sure your food is cut into small pieces. Never put anything larger than a bite-sized piece of food into your mouth. Taking smaller bites can also help you slow down while eating, which decreases the risk of aspiration.
Take care to focus only on your meal while eating. Watching TV, talking on the phone, or otherwise splitting your attention between your meal and another activity can be distracting and increase your risk of aspiration.
Keep your upper body as straight as possible while eating and for at least an hour after you eat. It may be helpful to prop yourself up on a pillow or in a bed that lifts your shoulders. You should aim to keep your upper body at least at a 45-degree angle.
If you are taking steps to prevent aspirating food or drinks but are still having difficulties eating or drinking safely, you may want to talk to your doctor or a neurologist about a feeding tube.
Feeding tubes can provide nourishment directly to your stomach, so you don’t have to worry about chewing, swallowing, or aspiration. There are different types of feeding tubes. For example, a gastrostomy tube or a nasogastric tube can work for people diagnosed with SMA. Talk to your health care provider or medical team to find out what will work for you.
People with SMA are more likely to develop pneumonia after coming down with viral or bacterial illnesses compared to the general population. Because of this greater risk, you should take extra precautions to avoid illness, especially during cold and flu season. Prevention strategies include avoiding some regular activities, especially if there will be many people present or if they are not taking precautions to prevent the spread of any upper respiratory infections they may be carrying.
Some steps you can take to help prevent viral or bacterial pneumonia with SMA include:
In general, research suggests that flu and pneumococcal (pneumonia) vaccines can reduce the chance of severe illness for people with SMA. You should always talk to your doctor about which vaccines you should receive, given the specifics of your particular situation.
Your situation may change from year to year. If your immune system is compromised, for example, your doctor may recommend avoiding vaccines for a year or postponing them until your immune system is stronger.
Make sure that you and everyone in your home washes their hands for at least 20 seconds several times a day, including after coughing, sneezing, and using the bathroom.
Alert those you live with about your increased risk of pneumonia if you get sick. Ask these people to take extra precautions, including covering all coughs or sneezes appropriately and staying away from other people who are sick.
Avoid sharing cups, bowls, plates, straws, or other utensils with others — even people in your home. Don’t eat food off anyone else’s plate. Ask the people closest to you to follow these same rules for your protection.
Depending on the form of SMA that you have, there may be medical options that can improve your respiratory function. For instance, children with SMA type 1 who take Spinraza (nusinersen) have improved respiratory function over those who don’t. Talk to your medical team about what SMA treatment options may be helpful.
If you feel sick or like you are not breathing well, get medical help as quickly as you can. Fast intervention can make a huge difference in whether you develop pneumonia — or, if you do, how severe the infection becomes and how quickly you recover.
If you even think that you may have come down with something, let your doctor know as soon as possible. They will be able to determine what type of respiratory care you need, such as noninvasive ventilation (breathing support) or invasive ventilation. Working with your doctor on the right solution can help you avoid respiratory failure or additional health problems.
On mySMAteam, the social network for people with SMA, members come together to ask questions, give advice, and share their stories with others who understand life with spinal muscular atrophy.
Have you or a loved one had pneumonia while living with SMA? What do you do to reduce your risk? Share your experience in the comments below or by posting on mySMAteam.