Definition

Retinitis pigmento (RP) is a collection of genetic disorders involving damage and death of cells receiving light in the retina. These cells are sensitive to light and are in the back of the eye. The RP is a rare disease that attacks one in 4,000 people in the United States and around the world. Research data in South Asia shows that there are more than half of the genes in South Asian population, especially the Pakistani and Indian regions that are connected to the RP. 

Cause

Pigmentosa (RP) retinitis is a genetically inherited disease and causes cells that receive light or so-called photoreceptors to be damaged or unable to function properly. Most of the RP cases are caused by mutations in the rhodopsin gene. The age at the start of the symptoms of the RP, the speed of the symptoms development, and the loss of the vision function depend on the gene-depletion pattern in their respective families. In the RP there is damage to photoreceptor cells hitting the stem cells and conical cells in the retina. The stem cells are in the outer layers of the retina, these cells serve to see in a dim and dark atmosphere. The cone cells serve to see in clearer detail on the bright state. A total of 90 genes have been identified to cause the RP.

Risk Factors

The main risk factor that you can experience RP is the history of RP disease in the family. Genetic patterns derived in each family can be different depending on mutation types and mutated genes.

Symptoms

At the initial stage of the RP, the stem cells will be damaged first so that the symptoms felt are narrow to the point of view that is narrow to the point of view such as seeing using a tunnel (tunnel vision). Other symptoms that you can experience such as myopia or faraway exposure. In some patients, sharp vision can be normal. The speed of development of symptoms is different in each individual. The initial age of the initial symptoms of the RP is different in each person. Most individuals experience blindness at the age of 40 from the journey of RP disease. Anatomical changes or shapes of the retina and the development of anatomical damage experienced must be assessed using an ophthalmoscopic by an eye specialist. 

Diagnosed

Diagnosis RP is enforced by doctors using oftalmoscopy. Ofalmoscopy is a tool to see the portion of the function or the back of the eye. The eye parts that can be seen using the otohalmscope include the retina, coroid, eye nerve center, and blood vessels. During the analysis of the oscillations, a picture of the dark spots that are deposits of pigments in the retina will be seen. Another examination that can be carried out by doctors to enforce the RP diagnosis is:

1. Electroretinogram (ERG): The function of this tool is to measure the electrical activity in cells in the retina. This examination is sensitive in examining the RP.
2. Examination of the field of view: This examination is used to assess the severity or magnitude of damage to photoreceptor cells in the retina to your field of view. Examination can be carried out in two ways, namely by confrontational tests and examination using perimetry tools. The confrontation test uses a doctor's field of view which is compared with the patient's field of view. Perimetry examination uses red light that will be fired and the patient will press the button if he sees the light.
3. Genetic examination: performed on several people to examine genetic mutations. Information regarding genetic mutations is important to know the descent pattern of RP in the family.
4. Examination to detect other diseases that resemble RPs such as syphilics. Syphilic examination uses a patient's blood test. 

Laksana Procedure

Implementation for the RP includes early detection and regular monitoring of symptoms development. Until now, there is still no definite therapy to treat and stop the journey of the RP disease.

1. Annual examinations are recommended to detect complications that can disrupt vision and become aware of the development of the disease.
2. If you experience complications from RPs such as cataracts, cataract surgery can help with eye vision function.
3. Avoid smoking, smoking can accelerate the development of the RP disease compared to those who do not smoke.
4. The use of glasses that can block sunlight up to 550 nanometers accompanied by protective glass on the side is recommended.
5. The use of vitamin A supplementation or the use of other drugs is consumed according to the doctor's advice.
6. Some drugs such as oral acetazolamide and topical anhydrase carbonic inhibitors can help reduce RP symptoms.
7. Other drugs such as calcium antagonists show some benefits but the efficacy and safety of these drugs are not fully understood.
8. Reactoxy medication must be stopped and used carefully in patients with RP. Some drugs including drugs to diagnose erectile, isoretinoin and other retinoidal dysfunctions. The use of these drugs and neurotoxy drugs must be used in accordance with the recommendations and supervision of doctors.

Complications

Some of the complications that can be quelled in patients with RP are cataracts, glaukoma, keratonus, and the release of the vitreous part that sticks to the back of the retina (posterior vitreous detonation). 

1. Cataract: Cataracts are a state where eye lenses become more opaque progressively which results in blurred vision. Cataracts in patients with RP are caused by an inflammatory process caused by RP. The most common type of cataract that occurs in patients with RP is a posterior subcapsular dwarf. 
2. Glaukuma: Glaukuma is a disturbance that occurs in several parts of the eye and is caused by an increase in eyeball pressure. Glakoma in the RP case is associated with migration or displacement from pigment to the back of the eye booth and blocking the movement of fluid in the eye.
3. Posterior Vitreous Detachment (PVD): The occurrence of liquid accumulation is caused by loose retinal layers and fluid buildup in the back of the retina. The accumulation of fluid will tear the retina from the eye and cause loss of eye vision function.

Prevention

Until now, no way has been found to prevent the development or emergence of the RP. This is because the RP is caused more by genetic disorders that occur in the retina. Therefore, periodic examination is quite important in knowing the early symptoms of the retinopathy of the pigmentosa.

When Do You Have To Go To A Doctor?

Immediately go to the doctor if you have the following symptoms:

1. Improved vision at night or in an environment that does not have good lighting.
2. Perturbative vision when looking at objects in a bright environment.
3. The loss of sight on the side or seeing like from the keyhole.
4. Difficulties in assessing the surface depth of objects such as when walking on the side of the road or climbing stairs.

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