Dr Patrick Deegan, Senior Research Associate and Honorary Consultant at University of Cambridge and Addenbrooke's Hospital, spoke about the potential for independence by people with Gauchers disease . Dr Deegan sees patients at the Gauchers disease Clinic and is closely involved in the Bone Research Project.
Dr Deegan sees patients at the Gauchers disease Clinic and is closely involved in the Bone Research Project. What degree of independence can individuals with Gaucher disease achieve or look forward to? This talk will take you from the health needs of patients to what everyone aspires to in general terms.
In the last 10 years, enzyme replace-ment therapy has had the potential to improve patients' mobility and provide energy to carry out daily activities. Home treatment also provides freedom from hospital visits to having infusions when and where desired, even abroad if properly organised in advance. The prospect of treatment in the form of a tablet would allow freedom from infusions whilst achieving the above aims and the prospect of gene therapy would further provide freedom from regular infusions or tablets.
Independence is highly rated in health-related quality of life question-naires. These questionnaires reflect patients' own priorities. One of these popular questionnaires (EQ5D) has five aspects, three of which relate to independence: self-care, mobility and the ability to perform daily activities. Independence is valued in many areas: independence of the individual from family, neighbourhood and state; independence of the family from neighbourhood and state; and of the neighbourhood from the state. In addition national groups, such as the Gauchers Association, value their independence from industry, government and sometimes doctors.
The Individual and the Family
Adolescents value their independence as they grow away from their families. However this may vary between different cultures where some young people are encouraged to fly the nest early and are even sent to boarding school whilst others continue their close involvement with parents, staying in the family home after marriage and having children of their own. A new word KIPPERS has recent surfaced. It means Kids In Parents Pockets Eroding Retirement Savings. Parents accompanying their adult offspring to hospital appointments are sometimes regarded as suspect in Britain but we all need support. Those of us who receive it from our families should not be made to feel ashamed.
Similarly how to arrange the transfer of care of young adult patients from paediatric services to adult services has not yet fully been resolved.
The Individual and the Neighbourhood
In this context, individuals who share a concern, interest or disease may benefit from the neighbourhood although perhaps community is a better word. The Gauchers Association is a model of a community Not every individual with Gauchers disease wants to belong to the patient association. This must be respected. But some patients come to value the association in times of need.
Receiving help may be seen as a form of dependency but then, as commonly happens, a feeling of benefit from the community leads to a responsibility to it and sometimes active engagement. The money which has been raised by many members of the Gauchers Association has strikingly illustrated the contribution that patients can make to their support association.
The Neighbourhood and the State
Rather than saying someone should do something, the Gauchers Association has said: 'We should do something and achieved it.' This is a form of independence through engagement with the state; persuading the state to use its power to foster independence. The Department of Health's National Specialist Commissioning Advisory Group's designation of the national Gaucher service has allowed individual patients and the Gauchers Association more control and influence. It is possible to gain one's independence through interaction and dialogue rather than by walking away.
Interaction and Mutual Respect
It is useful to distinguish between independence of, implying autonomy and isolation, from independence within, implying interaction and mutual respect. In our development as people and organisations, we mature from a narrow view of independence to a broader one, which includes responsibility and engagement.
Source: Gauchers News March 2004.
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Gauchers Association 2004.