While most patients with ALS initially develop muscle weakness in their hands or limbs, a small number of patients will develop bulbar symptoms at the very beginning. Bulbar symptoms refer to weakness in the bulbar muscles, which are muscles that control speaking and swallowing. Eventually, all ALS patients will develop bulbar symptoms, though the severity of the symptoms will vary from individual to individual.
The reason why swallowing is affected in patients with ALS is that the act of swallowing requires the involvement of several types of muscles, including those located in the tongue, lips, jaw, and throat. Without the coordinated function of all these muscles, swallowing can be severely impacted, which can cause food or liquid to go down the breathing pipe instead, leading to major issues such as choking or breathing difficulties.
There are some strategies that people with ALS can employ to better manage swallowing difficulties:
- Eating smaller bites of food and drinking smaller sips of liquids can make it easier to swallow.
- Swallowing two or three times with every bite can help ensure that all food is cleared from the throat.
- Changing the head position can help the patient swallow more safely.
- Tilting the chin down can help stop food from going down and getting suck in the windpipe. Tilting the head backwards can also help.
- Before eating dry foods, it helps to moisten the food with a liquid (sauce or gravy) to stop the food pieces from getting stuck in the throat. Also, taking a sip of liquid between bites can help lubricate the throat.
- Avoid foods that are a mixture of solids and liquids (i.e. soup with chunks of meat) as many patients with ALS find it hard to properly swallow such foods. Foods with a single type of consistency, such as cream soup, is often easier to swallow.
- If swallowing solids food gets too hard, then the patient may need to switch to pureed food, or other types of soft food such as mashed potatoes or pudding.
- For liquids, it is often easier to swallow thicker liquids than thin liquids. Therefore, patients can switch from a thinner liquid such as milk to a thicker liquid like smoothies or milkshakes. Furthermore, there are commercially available thickeners that can be added to any beverage.
- If swallowing saliva is an issue, which is often the case in patients with ALS, then there are medicines that are available that can help reduce the amount of saliva produced by the body.
- Suction machines can also help remove excess saliva so that the patient does not have to swallow it.
Ultimately, it will become extremely difficult to swallow enough food and drink, which can lead to malnutrition. At this point, most ALS patients will obtain a feeding tube, which can help obtain all the nutrition that is needed.
Nuedexta as a potential treatment for bulbar symptoms that can improve speech and swallowing
First approved to treat pseudobulbar symptoms in patients with ALS, Neudexta (dextromethorphan HBr and quinidine sulfate) is now being investigated as a potential treatment for bulbar symptoms. Pseudobulbar symptoms are a common complication in ALS patients in which they laugh or cry involuntarily.
Nuedexta is an oral medicine that is composed of a combination of dextromethorphan and quinidine, a combination called DMQ. Since its approval, physicians have been noting that patients with ALS report improvements in speech and swallowing and the ability to control oral secretions (saliva).
Hence, researchers have followed up on this by conducting a phase 2 clinical trial to see if Nuedexta actually does impact bulbar function in ALS patients. Researchers recruited 60 ALS patients, who then received either Nuedexta or placebo for 28 to 30 days, followed by a 10-day to 15-day washout period (in which they received no medication).
Results indicated that each domain of the CNS-BFS, a self-reporting tool that investigates speech, swallowing, and salivation, improved following treatment with Nuedexta. Furthermore, the ALS functional rating scale (ALSFRS-R) Bulbar component improved in patients treated with Nuedexta compared to placebo. Nuedexta did not affect the motor or respiratory components of the ALSFRS-R scale.
Anticholinergic drugs and botulinum toxin
Signaling from nerves controls functions such as salivation. Blocking these signals, known as acetylcholine signals, can help decrease levels of salivation. Therefore, physicians often prescribe anticholinergic drugs to ALS patients to control salivation. However, there are several side effects to these drugs including dry mouth, dizziness and constipation.
Botulinum toxin helps reduce salivation in a similar way to anticholinergic drugs (by reducing nerve cell signaling). However, the use of too much botulinum toxin can make swallowing even more difficult, and doctors often give lower doses due to this potential side effect.
Radiation therapy to treat excess saliva involves use of an electron beam that is aimed at the salivary gland on just one side of the mouth. In one study, patients who received this therapy noted less saliva flow within weeks. Furthermore, before radiation treatment, the average participant in the study produced about 2.5 grams of saliva in five minutes. However, after treatment, this dropped to approximately 0.5 gram. The benefit of radiation therapy towards stopping excessive saliva lasted for at least two years, leading the researchers to postulate it was likely a permanent therapy for this issue.