The most important assessment for an infant is how well they can swallow. Difficulties with this are known as dysphagia which brings a risk of inhaling food or drink (aspiration), which can cause a chest infection. Shortly after diagnosis, children should have a swallow test (video fluoroscopic swallow study). If the test shows there are problems swallowing safely, there are two temporary ways of providing food through the nose: a nasoGastric (NG) tube which then goes into the stomach, or a nasojejunal (NJ) tube which goes through the small intestine. A longer-term option that is recommended is a gastrostomy tube or PEG endoscopy which is when a tube is surgically put through the skin and into the stomach. Some experts recommend an additional surgical procedure to reduce reflux. Another issue can be if jaw muscles start to stiffen which can make it difficult to chew and swallow. If a child or adult is experiencing difficulties with any of these things mentioned above please talk to the medical team in for advice.
In addition to weak swallowing muscles and dysphagia contributing to poor calorie intake, non-sitters use a lot of energy to breathe and fight chest infections. This means they are at risk of undernutrition and may have difficulty gaining weight. Many types of diet are possible and each person needs one that is individualised, ensuring adequate fluids. Live bacteria and yeasts (probiotics) these are especially beneficial for the digestive system. Non-sitters may also need medication to help with constipation and their reduced ability to empty their stomach as their food may move more slowly through the digestive system.
In the event of illness, it is important to consult the medical team about care and management as it is essential extra fluids are given early and that salts in the blood are monitored. Having SMA means that metabolising fat normally can be difficult and can sometimes lead to an excessive build-up of ketones and other byproducts. To avoid this, a steady diet with sugars and protein to limit the breakdown of fats to make energy is recommended. This is especially important during illness, and it is recommended that nutrition with sugars and protein is given within six hours of becoming ill and continued. Fasting should be avoided.
It is important to see a dietitian to advise on an appropriate individualised diet’