Aids and Adaptions

At some point all children and adults with NPD will probably require some form of aid or adaptation to assist daily living. This may be as simple as special cutlery to make eating easier or a handrail on the stairs, but at some point may entail major work to adapt the living areas.

The priority is a full assessment as early as the family feel that help would be useful. It is usually the Occupational Therapist (OT) that will come to the home to discuss needs with the child or young adult and their family. It is important that this assessment not only takes into account current needs but possible future requirements. It is useful to have the Clinical Nurse Specialist (CNS) for Niemann-Pick in attendance to help during an assessment, as it is often difficult for a parent to contemplate their child needing hoists or similar aids in the future. It is also difficult for an adult who feels they can be independent to accept that they may be safer with a shower instead of a bath or indeed be better with a ground floor bedroom.

Because the OT and CNS have worked with many children and adults they are able to suggest aids to make life as simple as possible for the affected person. One child may need a raised desk at school to angle their books, which makes looking from book to the teacher and the board much easier. Another may work better on a computer. An adult may be fine with handrails throughout the house. These are just a couple of examples as every individuals needs are different.

As the disease progresses the family are likely to need more help in adapting the house. As mobility decreases the affected person may be in a wheelchair, resulting in adaptations to the access of the house may need to be considered, such as widening the doors and fitting a ramp.  There may also be issues with access to the bedroom or bathroom if these are upstairs. The OT can assess the family home and suggest appropriate changes. In some cases a stair lift may be appropriate, in others a full lift. Alternatively the bedroom and bathroom may need to be moved to the ground floor. The aim is to make life as easy as possible for the whole family over the many years of increasing needs.

Big changes to the home do take time as plans need to be drawn and grants or other finance sought.  It is therefore advisable to make arrangements ahead of time, aiming to implement the required changes before they become necessary. Thinking ahead can be very traumatic for the family, as they have to accept that in a year or two their child or relative may not be able to get upstairs. It is important for the family to talk about future needs with their own nursing team and the CNS.

Other aids that can help the child and family are much more fun. A young child with Type A or early onset Type C can benefit from aids that offer visual stimulation and are calm and soothing. They may not be able to handle and play with ordinary toys but bubble tubes, fibre optic lights and projected pictures and lights are fascinating for the young child. The CNS can assist families to access aids such as these through grant agencies and other sources.

It is important that a very dependant child is as comfortable as possible, so the OT can assess for appropriate seating to support them safely. An adult may primarily have memory or communication problems and may benefit from using a computer and E-mail rather than talking to people.

There are many aids and adaptations that can make what is a difficult time easier and the multidisciplinary team that cares for your child or yourself if you are older will ensure that you and your family access everything you may need.

For more information about any topic mentioned above, please contact the NPDG (UK) on 0191 415 0693 or email niemann-pick@zetnet.co.uk.