Batten disease is a severe inherited neurodegenerative disorder. There are several types of Batten disease based on the age of onset of symptoms, which can begin in patients as young as 6 months old.

These symptoms range in type and severity and may include progressive vision loss, muscle coordination problems, and decreasing cognitive ability. Due to these and other symptoms, malnutrition and dehydration can be a risk, and patients may become increasingly dependent on others for support.

Nutritional supports such as a feeding tube can help manage malnutrition and dehydration in patients with Batten disease.

What is a feeding tube?

A feeding tube is a device used to deliver essential nutrients and liquids directly into a patient’s stomach or intestines. The contents of the feeding tube can be tailored to meet the nutritional needs of the patient and can contain everything needed for a balanced diet.

Feeding can be done intermittently as a discrete dose of food (bolus) or continuously over a long period of time, depending on what is best for the child.

The need for a feeding tube is most common in patients with infantile Batten disease and some types of late-infantile Batten disease.

Tube feeding is often recommended when the child is experiencing difficulties swallowing (dysphagia), has a high risk of food entering the airways while eating (aspiration), or cannot obtain enough nutrients with normal feeding.

Types of feeding tubes

There are several types of feeding tubes that can be used to deliver nutrients.

Nasal feeding tubes do not require surgery but can cause discomfort when applied. They consist of a small flexible tube being temporarily inserted through the patient’s nose and down their esophagus. There are two types of nasal feeding tubes: nasogastric tubes deliver the food to the stomach, while nasoduodenal and nasojejunal tubes bypass the stomach to deliver nutrients to the small intestine. These are generally for short-term use (less than eight weeks) and are very visible.

Gastric tubes tend to be more common for Batten disease patients. These involve surgically inserting a tube into the stomach through a small incision in the abdomen called a stoma, which can be accessed from outside the body. As with nasal tubes, these can be used to deliver food to the stomach (gastrostomy tube), or can have an additional thin tube travelling into the small intestine (gastro-jejunal tube).

Initially, the child will normally have a long tube attached, called a percutaneous endoscopic gastrostomy (PEG) tube. However, these can be replaced with “button” tubes that are more discrete. An extension can then be attached when the food is being delivered.

Risks of using a feeding tube

Nasal feeding tubes have a small risk of damaging the esophagus.

When the gastric tube is being surgically inserted there is a risk of reaction to the anesthetic, bleeding, or infections. There is a risk that the stoma (the site where a gastric tube is surgically inserted) may become infected if not properly cared for.

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