Restraint is a positive intervention in the management of Rett syndrome
Recently, RSAA became aware of a number of Victorian parents who were being advised to stop restraining their Rett syndrome children and adults, as too were carers. As to why this sort of thing is still happening is hard to understand, as splinting, which is the most common method used in Rett syndrome to manage hand behaviour, is an effective intervention for improving the quality of life for persons with the condition.
In general, having Rett syndrome means an affected individual will experience problems with the hands/arms such as
great difficulty in being able to move or use them in a purposeful manner;
constant but unwanted repetitive movement, including placing hand (s) in mouth;
deformity of finger (s);
skin breakdown and/or infection of hand (s), mouth sores; and
self-injury, be it by hitting the face, hair pulling or biting the hand (s).
A positive approach in dealing with these problems, regardless of the Rett syndrome person’s age, is to actually intervene. As mentioned above, splinting is the most common form of intervention used by parents to both modify and assist hand movement, promote hand skin care, reduce the possibilities of finger deformities, mouth sores and/or self injury. Other effective methods may include use of a ribbon or an elastic wrist band to attach the arm to an armrest; brushing the hands/arms; holding the non-dominant hand; clothing the hands with mittens/socks; verbal commands; wrist weights; and distraction, either by engaging the hands in a stimulating activity or where possible, have an individual grasp an object. Splinting, banding, weighting and holding, all restrain, but importantly, each interrupts a behaviour that a person with Rett syndrome usually cannot.
Current Victorian State Government legislation relating to disability is contained in ‘The Disability Act 2006’ . It includes the words ‘mechanical restraint” which mean “the use, for the primary purpose of the behavioural control of a person with a disability, of devices to prevent, restrict or subdue a person’s movement but does not include the use of devices —
(a) for therapeutic purposes; or
(b) to enable the safe transportation of the person.“
The highlighted text is applicable to persons with Rett syndrome.
Restraint, be it in the form of a device or technique, is an important therapeutic intervention in both the management and treatment of the hand/arm movement disorder that is usually present in Rett syndrome and the problems that this causes. The device or technique used to restrain is, in fact, an aid. In Rett syndrome, judging an intervention by its effectiveness as a therapy or treatment is the important thing, NOT the terminology associated with a device or a particular technique. Provided the restraint is used properly ie., there is an understanding as to why it is being used, its effectiveness is regularly assessed, it is used when needed, and it is tolerated by the person to whom it is intended to benefit, it is a therapeutic measure and not contrary to the disability legislation in place in Victoria. The legislation pertaining to disability in other States and Territories needs to be examined to see how it impacts on the quality of life of a person with Rett syndrome.
In her publication ‘The Rett Syndrome Handbook’ (2nd edition, 2007, page 123), Kathy Hunter stated that “It is important to recognise that she (the Rett syndrome individual) is not causing the repetitive hand movements to happen. They are happening to her. They are not due to self-stimulation. Even in situations where she wants to, she is usually unable to break up these movements on her own. They may be subdued, but the hand movements are continuous during waking. They increase in intensity when she is under stress, whether positive (happiness, eagerness) or negative (discomfort, anger)……………………..Tolerance for inhibiting her hand movements differs from one girl to another, but many girls seemed to be relieved when the splints are put on.“
On page 251 of the same publication, Kathy further advised that “Children who put their hands in their mouth often get sores on the hands or around the mouth. Keeping the skin dry is the ultimate goal in treatment (of the sores) but this is often difficult to do. Antibiotic cream should be used if there is infection, along with keeping the hands and mouth as clean as possible. Cold weather can make this a difficult challenge. Arm or elbow splints are effective in keeping the hands away from the mouth and are usually well tolerated.”
Physical therapists, Susan Hanks (Oregon Health and Science University) and Meir Lotan ( Israeli Rett Centre) referred to splinting in their article ‘Physical Therapy Intervention for Individuals with Rett Syndrome’ which was published in ‘The Scientific World Journal’ in 2006 (Volume 6, pages 1314-1338). They stated that “The use of splinting and intensity of splint usage is controversial among different practitioners. Some will generally refrain from splinting the child’s hands as a regular intervention, while others believe that splinting often improves the child’s function and enhances her awareness in the environment. Nevertheless, the authors agree that splinting or arm restraints have been found to be helpful in the following cases:
– situations where skin problems develop as a result of constant hand-to-mouth behaviour;
– when restraining the less-active hand, it was found that the individual with Rett syndrome increases control over her other hand;
– when the stereotypical movements prevent the child from being properly fed (i.e., if the child is constantly putting her hand in her mouth throughout meals);
– when it disrupts the child’s social interaction (For example, a child with Rett syndrome was integrated into a regular school, but was not approached by normal peers due to constant drooling and spitting over her hands. The use of splints, preventing her from putting her hands in her mouth, turned things around and the child was immediately accepted by her peers).
The July 2002 edition of the Rett Syndrome Association of Australia newsletter, featured an article on the issue of hand management in Rett syndrome, the main contributors to which were a parent and her daughter’s occupational therapist. A review of the literature up to that point in time is described as too are the long term adverse effects of the daughter’s hand wringing/mouthing, the interventions (including restraint) used and the outcomes of such. Clicking on the following file will enable that article to be accessed. Hand management in Rett syndrome
During January 2017, M who was 45 years old and the Rett syndrome woman referred to the preceding newsletter article, had her personally made-to-fit right hand splint removed. The decision to do so was made by her mother Lara and the supervisor of the residential unit in which M lived.
Splinting her right hand made it easier for M to use her left to feed herself. However, as M had been capable of using her left hand for such over a long period of time, removal of the right hand splint wasn’t viewed as a problem.
A number of issues arose over the three week period that this change was in place. The house supervisor reported that –
M was having trouble balancing when standing and walking
M’s concentration was severely compromised
There were significant changes in her posture when standing, sitting or in her wheelchair
M was unable to stand up after sitting at the table or when in a armchair
M was unable to assist with sitting and positioning herself in her wheelchair
M was unable to place her feet on the wheelchair footrests.
As the only thing that was different in M’s life over the period that these problems were apparent, was an unsplinted right hand, her mother and the supervisor reintroduced her splint after which the issues listed above, ceased. The supervisor felt that M was more confident, secure and capable, as a result of having the right hand splinted.
Lara stated that she “was amazed at the outcomes of this unplanned experiment (with my daughter) and hopes that awareness of such, will be helpful to parents, carers, Rett individuals, therapists and health professionals.”