RP or retinitis pigmentosa
is a group of diverse retinal dystrophies that affect the rod photoreceptors and later, cause cone degeration as well. There are basically 4 types of RP. Sporadic where there is no family history of disease, Autosomal Dominant which has strong familial history but also best prognosis, Autosomal recessive and X linked in which only males are affected and females are carriers of the disease.
Symptoms start with a defective night vision or nyctalopia
and poor dark adaptation with a progressive constriction of visual fields. There are many syndromes with systemic findings along with RP. An ophthalmic evaluation starts with a retinal exam and includes an ERG, EOG ,dark adaptation tests and visual field testing. Unfortunately, the prognosis of RP is poor. Studies show that 25% of patients do retain vision possible to read with throughout their working lives. Younger patients upto 25 years of age have vision better than 6/60. Patients older than 50 years have vision worse than 6/60. There is no proven treatment for reversal or to stop progression of RP till date. However, promising studies are being carried out in the field of gene therapy
that claim to improve vision in affected patients. Trials are being conducted where artificial retinal implants and even developing photoreceptor cells are used. There is evidence that states that Vitamin A
daily along with Omega 3 fatty acids found in fish can retard progression of the disease. At present, you may advise members of your family to try to make the best use of their vision with the help of Low Vision Aids
like magnifiers, television monitors and book readers. Also, since you have a high risk of disease being passed down in the family, genetic counselling
is required to know the risk with regard to future generations.