Spinal muscular atrophy

What is spinal muscular atrophy?
Spinal muscular atrophy, or SMA is a neuromuscular condition that is caused by a genetic mutation. The problem occurs at the neuromuscular junction, the crossover between the nervous system and the muscles. This means the muscles do not get the correct messages and therefore do not perform their required function. Because the problem occurs at the very end of the nervous system, right at the muscle crossover, the central nervous system is not affected and people with DMD tend to have normal intelligence. Because the heart and lungs are made of muscle, these become affected in spinal muscular atrophy, depending on the type of SMA a child has, and troubles with breathing are common. At BBP, we are following all of the latest research, and are very hopeful that new treatment drugs will be discovered soon.

Our superheroes with SMA enjoy doing lots of the same things their friends get up to! Although their life is different, they never let this get in the way of living an enjoyable and adventurous life. Our clients frequently face challenges as they get older and learn more about their condition and how it affects them. They have access to an excellent team of support, who work closely Team BBP to ensure the most sensitive and empathetic approach is made to all treatment.

How can physiotherapy help?
Physiotherapy takes many different forms for our superheroes with SMA. We tailor our approach to the individual child, their personality and what is important to them. This can be anything! We are working on improving arm and hand strength for one client to use their tablet, and another superhero is improving her leg strength to maintain her transfers, so she can transition into a rollercoaster when she goes to Disneyland!

Strengthening is a huge part of physiotherapy intervention for SMA superheroes, and usually the proximal, or furthest muscles are affected first. We aim to strengthen and maintain movements in all affected joints, to aid participation and prevent breathing problems for as long as possible.

We work closely with wheelchair services, NHS professionals and occupational therapists to ensure the best balance of postural management and independence is achieved for our SMA superheroes. We base all of our goals around our clients’ wishes. Some of our superheroes want to work on their independent or aided walking for as long as possible. Others want to explore other options for speed and independence, and some want a mix of both! We will get to know your superhero, what makes them happy and their favourite activities so we can tailor all of our intervention around them.

Assistive devices
As technology is improving, so are adaptive and assisted devices for people with disabilities. There is a huge world of tailor made and off the shelf gadgets and we love exploring and trialling these to suit our SMA clients.

Maintaining range of movement
We measure range of movement regularly and prescribe active and passive stretches and sustained positions when required. We liaise with all other professionals involved in your superhero’s care, and when conservative management is not enough, we will recommend other avenues of intervention.

Function and participation
All of our goals are bespoke to our clients’ needs. This could be having fun and playing with toys, socialising with friends, maintaining independence or going partying! Whatever we work on in physio sessions, is sure to help towards a functional goal in our superheroes’ lives.

To find out how we can help your SMA superhero, contact us.