Therapy Interventions in Advanced ALS

As ALS advances, respiratory and nutrition therapists are often involved in sustaining life. Breathing equipment and surgical interventions are required. Respiratory and food therapists/dieticians can significantly educate patients and caregivers about the options available.

Respiratory therapy

The role of respiratory therapy is to educate the patient on the breathing process and medical equipment that can make breathing easier or possible. After the ALS diagnosis, several tests may be completed to establish a baseline of breathing capacity.

A forced vital capacity (FVC) and other pulmonary function tests (PFTs) may be performed if the patient has weakness in the mouth. The other option that avoids a mouthpiece is the maximal sniff nasal capacity force (SNIF, the sniff nasal pressure.)

Additional oxygen is not generally prescribed for all ALS patients, only those with medical conditions that require it. It can harm, so it should only be used under the advice of the patient’s physician.

The first breathing machine option that a respiratory therapist might educate the patient on is non-invasive ventilation that assists the air entering and exiting the lungs. This device usually uses a mouth or nosepiece, and the air is delivered under pressure by a little, portable machine. A pulmonologist often prescribes it.

The respiratory therapist may show the patient their options to see which is more comfortable.

There are two pressures, one for inhalation and one for exhalation. It is also known as a BiPAP machine or a bilevel airway pressure machine after the company that created it. This machine is used as needed.

If air is needed regularly, the respiratory therapist may be involved in education surrounding the use of a permanent form of ventilation called a tracheostomy.

Here, a hose in the air pipe is attached to a ventilator that works on a timed cycle. Maintaining the tracheostomy tube is taught, including changing supplies and suctioning mucus. Patients on ventilators can live everyday lives, as the ventilators are portable, and a speaking valve can be added to allow for speech.

Nutrition therapy

A registered dietician can provide nutrition therapy when a feeding tube is indicated.

They can help the patient decide when nutrition and hydration are insufficient according to their patient’s report, clinical signs, or laboratory values.

A feeding tube becomes necessary when eating causes fatigue, weight loss, decreased energy, problems swallowing and aspiration (food goes down the windpipe), putting them at greater risk of pneumonia. Getting a feeding tube earlier than needed is essential. To adequately recover from the placement of a feeding tube, the patient needs to have adequate nutrition intake and respiratory function of a forced vital capacity of not less than 50% as a predictor of ALS survival.

A feeding tube is recommended when food intake drops below the recommended levels for meat/protein, dairy, fruits and vegetables, grains, and starches, and less than estimated calorie and protein intake. If urine is straw-colored, it is a sign of dehydration, another reason for feeding tube placement. A nutrition therapist can guide the patient further regarding the need for a feeding tube and its benefits and use.

Although a physician prescribes a feeding tube, a nutrition therapist assists the patient in learning to live with it.

A feeding tube is about one-quarter of an inch in diameter and is flexible and provides a different route for nourishment for what once was solid food, plus liquids and medications.

A nutrition therapist can discuss the options regarding different types of feeding tubes. There are two types of tubes – a percutaneous tube that goes through the skin and is called a PEG tube (Percutaneous Endoscopic Gastrostomy) and the RIG tube (Radioscopically Inserted Percutaneous Gastrostomy).

The PEG tube is inserted via a telescope down the throat, while the RIG tube is inserted using the guidance of an x-ray after barium has been placed in the stomach. Gastrostomy tubes are always placed in the stomach, unlike other tubes.

A nutritional therapist will educate the patient and caregiver on the preparation for a feeding tube insertion, the benefits of having it inserted, and help alleviate the concerns about having a tube inserted. A gastroenterologist may insert a tube by endoscopic technique, or an interventional radiologist may perform a RIG insertion.

A feeding tube is a safe way to obtain nutrients, liquids, and medications. Your nutritionist will discuss what foods can be safely eaten depending on your ability to swallow.

Troubleshooting the feeding tube

A pharmacist should be consulted regarding the delivery of particular medication by feeding tube as some can be crushed and delivered by feeding tube, while others, such as enteric-coated or time-released pills, cannot.

If a patient can eat food safely by mouth, they can supplement by tube feeding if needed. The tube can be the only way to deliver nutrition as problems develop with swallowing. Foods can be blended into a thin mixture and used with a feeding tube.

The patient should consult their nutritionist. At some point, a nutritionist may recommend formula be used with the tube; however, this does not need to be considered in the middle stage of ALS.

A nutritionist or dietician will help determine formulas that should be used with the feeding tube once it becomes the sole source of nutrition. Formulas contain different calorie amounts, so the feedings can be achieved with less volume and provide adequate nutrition. A patient’s calorie count with ALS varies if they also have other diseases.

A nutritionist can guide the patient and caregiver on the type of feeding to be given. There are three types – bolus, gravity feed, and continuous feed.

Bolus feeding is done one can at a time. Gravity feeding sees food placed in a feeding bag and put on a pole two to three feet above the patient to allow the formula to flow gradually through the tube for at least 30 minutes. Continuous feeding is a constant amount of formula throughout the day or night.

The patient must remain upright or at a 45-degree angle while the formula is administered and for one hour after delivery.

A nutritionist will guide the patient and caregiver on the feeding tube's proper use and care (flushing). They will also guide the patient on needed calorie counts and types of fluid in addition that can be used with a feeding tube besides formulas, such as juices and almost any other clear liquid.

Breathing and nutrition are two significant factors that are diminished in ALS and require close monitoring with the progressive nature of the disease. There are non-surgical and surgical approaches to both. The respiratory therapist and the nutritionist/dietician play critical roles in the care of the ALS patient approaching the advanced stages.