Friedreich’s Ataxia is a rare genetic disease that causes damage to the nervous system. This can result in movement problems, including dysphagia. Symptoms of Friedreich’s Ataxia usually start to show up in childhood. As the patient ages, the disease progresses. While the disease does not effect cognitive abilities, it does cause unsteady movement and sensory issues. Some patients develop heart problems or diabetes as well.
The impairment of the muscles needed to swallow causes dysphagia in Ataxia patients. Like the rest of the symptoms of this disease, dysphagia worsens over time. Usually, dysphagia starts in the mouth of Ataxia patients. Then, issues may start occurring in the pharynx and esophagus as well. Because Ataxia causes issues with the coordination of the mouth, pharynx, and esophagus, patients may have trouble swallowing food and drink.
Symptoms Of Dysphagia
The most common symptom of dysphagia is trouble swallowing food and drink. This can also include getting food stuck in the throat or cheek, coughing, or choking.
Patients who choke on food a lot due to dysphagia usually end up with recurring infections. All dysphagia patients are also at risk of malnutrition, dehydration, poor oral health, significant weight loss, and recurring chest infections. Patients also commonly miss doses of important medication because they can’t swallowing them. this can cause them to be over or under medicated.
Managing Dysphagia In Friedreich’s Ataxia Patients
A team of medical professionals is needed to properly manage dysphagia in Friedrich’s Ataxia patients.
A dietician will help ensure that a patient doesn’t suffer from malnutrition and dehydration. Physiotherapists and occupational therapists will help patients correct their posture to compensate for swallowing difficulties. They may also demonstrate muscle exercises to strengthen muscles used for swallowing.
With patients who are missing medication because they can’t swallow properly, they may be switched from a pill or tablet to a liquid formula if available.