The majority will attend mainstream school and achieve academically. To reach their full academic potential it is important that the education authority and school are aware of resources that may be required: this may include a one-to-one classroom assistant, appropriate classroom furniture and access to an individual computer, hearing or visual aids, and extra time to complete tasks that may not seem difficult but which may require more effort to complete due to the physical problems associated with the disorder.
At the present time there has been no research carried out that explores the psychosocial development of individuals affected by MPS VI.
However, it is important to consider how the additional challenges in life may be experienced. People with MPS VI can adapt socially and emotionally in different ways to new challenges or problems. The adolescent and teenage years may be more difficult because of all the physical and psychological changes that occur, whilst also having to cope with the problems caused by the disorder itself. Mental health is an important issue and it is therefore vital that steps are taken for an appropriate psychology referral as part of a comprehensive, on-going package of support.
Siblings also need to be considered. No formal studies have been carried out to assess the psychosocial effects of MPS VI in siblings. It is not uncommon for unaffected siblings to feel somewhat neglected or less important in the family unit as the greater share of attention is placed on the needs of the affected person. Parents may need to monitor the broader impact of the disorder on siblings and seek medical or psychological help if necessary.
Independence should be encouraged as much as possible. It may help to meet or be put in touch with other teenagers and adults with the same or similar conditions.
Developing the necessary skills to lead an independent adult life can be very difficult and some may experience difficulty in establishing independence from their family - an important step in achieving social maturity. Adults with MPS VI have the potential to live independently and claim appropriate financial support to purchase the services needed, including, for example, that of a personal assistant.
Adapting the House
Appropriately adapted living space will greatly enhance the development of independent living skills. Where mobility is restricted, a wheelchair may be required. In addition, pain and joint stiffness, when associated with short stature, may impact on the person’s ability to undertake personal care and daily living tasks. Should this be the case, a carer may be required. If a wheelchair is needed, plans to adapt the home should allow adequate space to accommodate it, and it may also be important to think about for the future even if a wheelchair is not needed at the time. It is, therefore, prudent to plan ahead whenever possible.
The disabilities caused by the disorder should not prevent people from accessing meaningful employment. There is considerable responsibility on the part of employers under the Disability Discrimination Act to meet the needs of employees with a disability.
Taking a break
Caring for a person with progressive disability is hard work that is physically and emotionally tiring. Parents will need regular breaks so they can continue providing care without becoming exhausted. Brothers and sisters also need to have their share of attention and to be taken on outings that may not be feasible with an affected child.
Palliative care is any form of medical care or treatment that concentrates on reducing the severity of disease symptoms. The goal is to prevent and relieve suffering and to improve quality of life for people facing serious, complex illness and that of their family. This may include respite care, symptom management and bereavement support and may extend over a period of time. It is important to talk with your medical team to ensure you are aware of and have access to the various services and support networks that are available..