In honour of Rare Diseases Day, we invite you to learn about how Lauren supported a young girl with Rett Syndrome.
Discover her inspiring journey and the strategies she used to provide optimal support.
Let’s raise awareness and show our support for those affected by rare diseases.
Rett Syndrome:
A rare neurodevelopmental genetic disorder, it affects 1 in 10,000 girls born each year and is rarely seen in boys. Rett syndrome can affect some children more than others and symptoms can vary from child to child. Some common symptoms include: low muscle tone, spasticity (particularly in the legs), loss of purposeful movement of the hands (repetitive movements), unsteady walking often progressing to loss of mobility, scoliosis, difficulty swallowing and seizures.
Is there a cure?
There is no cure for Rett Syndrome at this time, therefore treatment is focused on managing symptoms. Children with Retts will often have the support of multiple healthcare professional such as: OccupationalTherapists, Physiotherapist and Speech and Language Therapist.
Supporting a Little Girl with Rett Syndrome
In her previous role as an Occupational Therapist in the wheelchair service, Lauren had the opportunity to support a young girl with Rett Syndrome. Here, she shares her experience and the strategies she used to help this little girl achieve optimal support.
I saw a young girl for a wheelchair assessment as a standard commercial buggy was not providing thecorrect postural support required.
The patient presented with seizures, hypotonia, poor control of trunk and head and external rotation and abduction at the hips. Parents were struggling to safely feed this young girl due to her positioning. This patient was unable to walk and required a wheelchair that would ensure she could remain safe, support post seizure recovery and enable her to be supported to assist her utilisation of her minimal hand function.
What I used to achieve optimal support for this patient and why:
Tilt in space wheelbase: this helped with head control and maintaining upright posture.
Pivot Wing headrest : used to support head into midline, preventing lateral drop of head.
FormAlign Support backrest with Lo-rise brackets and curved lateral pads: The curved lateral support contoured around the patient’s trunk, enabling maximum surface area contact, therefore improving comfort. Using the Lo- rise brackets enabled me to have extra height adjustment when it came to positioning the lateral supports. This also helped to future proof the provision as these brackets could then be adjusted as the patient grows- or transferred over to a bigger backrest in time.
Chest harness with a Harness retainer kit: The chest harness provided anterior force to prevent pt falling forwards in her seating. The harness retainer kit was really helpful as it allowed me to adjust the harness height exactly where I wanted it to achieve the optimal pull required. Again, this helped to future proof the provision as the positioning of the harness could be easily moved as the patient grew.
FormAlign STX: The STX cushion was used to correct external rotation and abduction at the hips to ensure patients lower limbs kept in line with her pelvis. The deep ischial well helped to secure the pelvis into a neutral position, helping to prevent sliding in her wheelchair. This cushion was coupled with a two-point pelvic belt to further secure the pelvic position. I ordered a slightly wider STX than required and moved the thigh guides inwards to meet patients current hip width requirements. As children often grow quickly, I wanted to ensure I had a cushion I could adjust the width of when needed to meet patient growth in future.
For more information on Rett Syndrome, please visit the NHS website