How to use the Guide

The guide is divided into Sections; each Section focuses on a specific aspect of SMA management, how and when this should be assessed, and the options for intervention. You will see within each Section there are often different care and management guidelines and options based on physical milestones or type of SMA, as described below. As SMA affects each person differently, not all options may be appropriate for every individual.

The most common form of SMA is broadly known as ‘5q SMA’ and is often further described or classified as a type of SMA according to the age of onset of symptoms and the physical milestones reached:

Table 1. Clinical classification for SMA (Table adapted from RS Finkel et al, 2017, p.597)

It is important to remember, however, that there are no distinct lines that distinguish the childhood onset types of SMA from each other and that the impact of 5q SMA varies greatly between individuals, just as it does in the adult onset form. Additionally, now that some individuals  are receiving drug treatments, there may be even greater variation in the impact. It has therefore become important when discussing care and management, to look not only at the clinical  classification of the person’s SMA, but also at what physical milestones they have reached. These may or may not be the same as in the above table.

It’s important to always remember that the guidance is general, and every child, young person and adult is different. What is an appropriate intervention for one may not be appropriate for another.

In this guide, for simplicity you will find the following summary words used to show which care and management guideline applies to which groups of children and adults:

non-sittersThose who are unable to sit
sittersThose who are able to sit but not walk
walkersThose who are able to walk

Guidance may also refer to the type of SMA as in table 1 or refer to ‘early onset’ SMA. This is when symptoms start before six months of age; typically those with ‘early onset’ are non-sitters. ‘Later onset’ SMA refers to symptom onset after six months of age; typically those with later onset include both sitters and walkers.

When you are discussing the management of SMA with healthcare professionals, it may be helpful to ask them what Section to refer to in the family guide.