What is the ALS Functional Rating Scale (ALSFRS)?

When an individual is diagnosed with amyotrophic lateral sclerosis (ALS), it is essential to continuously monitor their physical health for any changes so that they can receive the appropriate care.

One of the key elements in providing support relies on the monitoring of specific functional changes such as speech, movement, walking and the ability to swallow.

This is undertaken using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS). It is widely employed for patients around the world, including in the USA.

What is the ALSFRS-R?

The ALS Functional Rating Scale is designed to provide a quick and uniform assessment of patients and determine how the disease is affecting them at any given time.

It covers four functional areas: speech, swallowing and upper and lower movement of the extremities.

Within these areas there are various measures including handwriting, walking, using eating utensils, and climbing the stairs.

The original ALSFRS was updated in recent years to include extra measures and is now referred to as ALSFRS-R, with the ‘R’ standing for revised. These new measures are dyspnea, orthopnea and respiratory insufficiency.

What does the ALSFRS-R involve?

The purpose of the ALSFRS is to provide an ongoing measure of how the disease is affecting the individual who has been diagnosed with ALS. Twelve measures are scored from 0 to 4.

Zero means there is no function at all and 4 means there is full function.

ALS can affect different parts of the body depending not only on the individual but the type and progression of the disease itself. The ALSFRS-R severity scale is an important tool for measuring the progression of ALS in a diagnosed individual, but is not always useful when comparing symptoms between different patients.

It does, however, deliver a relatively high degree of agreement when the scale is used by different examiners.

The patient is tested at regular intervals and this gives practitioners the ALSFRS-R slope, a graph which indicates the speed and progression that potentially lies ahead. Other measures, such as vital capacity concerning the lungs and the Sickness Impact Profile (SIP), can be used with the ALSFRS-R to provide a more detailed analysis of the patient’s condition.

ALSFRS-R Measures

The individual measures of ALSFRS-R will vary depending on the progression of the disease. There are 12 parameters and a top score of 48 which you would expect with a fully functional and healthy individual.

1. Speech: The first measure on the ALSFRS scale, a score of 4 would be normal speech and a score of 0 would mean complete loss of useful speech.
2. Salivation: A score of 4 would be normal salivation, 2 would mean that someone was producing moderate amounts of saliva with some drooling and a score of 0 would exhibit a lot of drooling that requires clearing with a tissue.
3. Swallowing: Being able to swallow food is a key indicator and a score of 1 or 0 usually means that the individual requires a feeding tube and assistance.
4. Handwriting: As ALS progresses, it severely affects fine motor movements such as the ability to write. The patient’s writing may become increasingly untidy and proceed to the point where they are unable to hold a pen at all.
5. Cutting food and handling utensils: This is subdivided into those who have a feeding tube and those that don’t. A score of 0 means that the individual is unable to feed themself and needs assistance.
6. Dressing and hygiene: In the early stages of the disease, individuals can dress and maintain levels of hygiene. As ALS progresses, they will need more help from a caregiver.
7. Turning in bed and adjusting bedclothes: A score of 0 means that the individual is helpless and has to be turned physically and have their bedclothes adjusted.
8. Walking: This is one of the most obvious signs of progression. In the initial stages, walking may be difficult but manageable. Later on, loss of complete function means they are unable to walk even with a stick or frame.
9. Climbing stairs: As with walking, this ability begins to degenerate over time and loss of limb function means that individuals are unable to climb the stairs at all.
10. Dyspnea: The individual begins to suffer from shortness of breath when performing everyday tasks such as walking or dressing.
11. Orthopnea: The individual has shortness of breath when lying in a prone position, for example, when they are in bed.
12. Respiratory insufficiency: This is a more severe symptom seen in the later stages of ALS and refers to the patient’s inability to breath properly without assistance.

Summary

The ALSFRS-R severity scale is a useful tool for practitioners and patients to gauge the progress of the disease and regular testing can help prepare for future challenges.