Haley Is My Hero

This site is dedicated to our little hero, Haley.  She is a brave little girl who suffers from a rare seizure disorder called Dravet syndrome, or Severe Myoclonic Epilepsy of Infancy (SMEI).  Haley has been battling this disorder since whe was five months old.  There is no cure for Dravet syndrome and Haley will never out-grow her seizures.  Despite all of the hardships in her life, Haley remains a happy, loving, smiling little girl who has taught us much about what is really important in life.  She has inspired many others through her tenacity and positive attitude towards life.

Dravet syndrome was first described by French physician, Dr. Charlotte Dravet in 1978.  It is a rare disorder caused by a genetic mutation in a protein that regulates electrical activity in the brain.  People with SMEI experience almost every type of seizure known.  The first seizure usually occurs in an otherwise normal, healthy infant before one year of age and is usually associated with fever.  Initially, generalized tonic-clonic seizures (Grand Mal Seizures) occur every few months and tend to turn into "status epilepticus" (a prolonged seizure that is very difficult to stop, even with medication).  Other seizure types begin to appear during the toddler months and seizures become more frequent, sometimes occurring hundreds of times a day.  Most children with Dravet will experience a plateau or regression of developmental skills during the second year of life.  Individuals with Dravet syndrome have only an 85% chance of surviving to adulthood.

Haley and family with Dr. Charlotte Dravet

 The seizures associated with Dravet syndrome are very difficult to treat.  Many of the anti-epileptic drugs used in the U.S. are not effective for this disorder, and several of them can even make the seizures worse.  The European Union has recently approved two medications, Clobazam and Stiripentol, for the treatment of SMEI.  People with rare, life-threatening diseases can import medications that are not approved by the FDA into the U.S. on a compassionate use basis.  However, most insurance companies will not cover the cost of these drugs, which can be very high. (Haley's neurologist can not even prescribe them, even for compassionate use since they are not FDA approved) 

Due largely in part to the efforts of the Dravet.org, more patients are being diagnosed with Dravet syndrome every day and at an earlier age.  (Haley was seven when she finally received an official diagnosis)  Until recently only about 500 cases had been reported world-wide.  However, it has been estimated that one peron in 20,000 has SMEI.  This means that 334,000 people in the world currently have Dravet syndrome, and one baby is born in the U.S. every other day with this rare condition. 

Dravet.org is an international partnership of parents and professionals united in the purpose of creating greater awareness and  understanding of Dravet syndrome.  The group was originally founded by parents in October 2005 as the IDEA League (International Dravet syndrome and Epilepsy Action League).  Its mission is to promote awaresness about and research for Dravet syndrome and related genetic, febrile sodium channel epilepsies and to provide resources and support to improve quality of life for affected individuals and their families.  It is the only organization dedicated specifically to this invaluable purpose.

Thanks to the hard work of members of Dravet.org, the FDA has recently designated Stiripentol as an "orphan drug" for the treatment of Dravet syndrome.  Due to the enormous cost of research, pharmaceutical companies are reluctant to study medications that will be used to treat only a small number of patients with a rare disease.  The "orphan drug" program offers incentives to companies who want to study such drugs and apply for full approval inthe U.S.  Biocodex, the French manufacturer of Stiripentol, has already indicated its willingness to work toward full U.S. FDA approval of Stiripentol.

"We know that all things work together for good to those who love God"   Romans 8:28

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~Haley Is My Hero~