HSP & other rare diseases

All HSPers know they have a rare disease but may not know it is one of 6,000 known by the US National Institutes of Health (NIH) Office of Rare Diseases (ORD).  ORD defines a rare disease (often called ‘orphan disease’) as one with fewer than 200,000 people being affected in the USA.  Another definition is – a disease that affects fewer than 5 people in 10,000. Other estimates suggest that between 6% and 10% of the community suffer from a rare disease.

ORD issue research grants and facilitate partnering of patient support groups with the National Institutes of Health on research programmes.  They generally play a coordinating role with activities associated with rare diseases. Their website is: www.rarediseases.info.nih.gov/
  
The US National Organization for Rare Disorders (NORD) is supported by ORD but runs independently as a non-government charity.  It’s website is: http://www.rarediseases.org/

It has 2,000 patient organizations on its membership and lists 1,160 disorders.  Our disorder is shown as “paraplegia, hereditary spastic” and is number 398 on the list.  The majority of rare diseases are genetic disorders.

The EU has a similar organization to NORD and its website is www.eurordis.org.  Canada and NZ both have patient-representative groups for rare diseases ( www.cord.ca/ and www.nzord.org.nz/ )

The original purpose of these support organizations was to gain access to drugs that were effective on the rare disease but lacked regulatory approval and were not seen by pharmaceutical companies to be a commercially viable proposition to develop for one or more rare diseases.  Hence the term “orphan drugs”. The US has initiated incentives to encourage pharmaceutical companies to invest in more research and development for rare diseases.

While there are no similar rare disease organizations in Australia, the Government commenced an Orphan Drug Programme in 1998 which was modeled on the US programme.  Support for the appropriate drug availability has been provided by forgoing some application fees and streamlining the process including the recognition of FDA approvals. There is still no coordinated approach from Governments or patient groups involved with rare diseases in Australia.