Trisha Ferguson

Special Care Patient

Retinitis Pigmentosa

If all you could see is a small circle of vision what would life be like? For many
people with retinitis pigmentosa this is all they know for vision, and some wish they
still had this small tunnel of vision if/when the disease progresses to full vision loss.
Retinitis pigmentosa is a group of inherited diseases that causes retinal
degeneration, the extent of degeneration is different for all cases. As stated on
blindness.org people with RP experience a gradual decline in their vision because
photoreceptor cells (rods and cones) die. Some of the related diseases with RP
include Usher syndrome, Lebers congenital amaurosis, rod-cone disease, BardetBiedl syndrome, and Refsum disease, among others.
The rods affect the outer portion of the retina, and the cones the central
areas. Normally with RP the rods are the first to degenerate, which means that
peripheral and night vision are the affected. Once the disease has progressed to the
cones the person loses color perception and central vision. RP is normally diagnosed
at a young age, and the rate of progression varies person by person.
Allaboutvision.com explains how its very difficult to predict the extent of vision
loss or how fast it will progress when you have retinitis pigmentosa. Retinitis
pigmentosa occurs in about 1 of every 4,000 people in the United States. When the
trait is dominant, it is more likely to show up when people are in their 40s. When the
trait is recessive, it tends to first appear when people are in their 20s.

Unfortunately, for all the people that are affected there is no treatment or
cure at this time. According to medicinenet.com there is has been recent, gene
testing for defects is being done to clarify the basic cause for RP and assist in
ultimately finding a treatment. But for now there are only some therapies and they
are not guaranteed to help or cure RP. Vitamin A therapy has been one
recommendation for RP patients, the recommendation is to use 15,000 IU a day of
vitamin A and E has shown to slow the course of the condition among adults with
typical forms of RP. This same study showed that 400 IU a day dose appeared to
have an adverse effect on the course of RP. A second therapy that has shown to
help with the slowing of RP is a diet high in omega-3 rich foods. Some research has
shown that the combination of Vitamin A plus a diet high in omega-3 rich foods has
shown 20 years of additional useful vision.
For a patient with RP has many obstacles to overcome in life, blindness is not
easy. For their oral care it may be harder for a patient with RP to perform their home
care. Even though dental care for a blind patient are the same as those for someone
with full sight, there may need to be some modifications and during their hygiene
appointment there will need to be some modification. Without their sight patients
with RP may feel nervous and uncomfortable in the dental office because they are
outside their comfort zone and must trust the dental team to lead and guide them
correctly. The sfsu.edu has some great tips for working with patients that are blind
and also who are deaf:
1. Warm hands or instruments before touching the person who is deafblind.

2. Tell the person what each procedure will entail prior to beginning the
procedure. This can be accomplished by allowing the person to
examine the equipment by touch. You can then touch the patients
body part that will be examined by that piece of equipment. Pause for
a few seconds to allow the person to prepare for the exam before it
begins. You can also have the medical/dental professional demonstrate
the procedure on the family member/care provider to model
appropriate responses.
3. Allow sufficient time for the patient to process information. Many
individuals who are deaf-blind require extra time to process
information, and processing time may be increased if the individual
experiences stress or anxiety.
Some of the ideas that I used for my special care patient with RP came
from the site topdentist-ny.com which discuss implementing an oral care
schedule, which can help ensure all oral hygiene requirements are met.
Thankfully my special care patient had awesome oral care and oral care
routine, but did have some staining from his diet. When I brought him back I
made sure to lead him by letting him hold onto my arm. With how tight the
clinic was I made sure to let the other clinicians know that I was bringing my
patient back and that I was going to need a pathway to prevent him from
bumping into chairs and desk.
This seemed to work well and my patient was very comfortable in a
dental setting. When we discussed oral care, he seemed to have a good
routine going, but had a few areas that had some inflammation. When

showing him OHI I made sure to let him hold all the items in his hand and
explained the different aids I was showing him. We discussed using proxy
brush, which he had used in the past. My patient had great ability to move
the proxy brush around in his mouth without the ability to see. It seemed
after so many years without his sight, he had a systematic routine of how he
did his oral care.
One thing that his wife wanted done was for him to bleach his teeth,
due to staining. This option was given to my patient, but he did not seem too
thrilled about the idea of bleaching his teeth. I explained the process to him
and allowed his wife and him time to discuss and make the decision if bleach
trays was something they wanted to do, in the end my patient made the
decision to not bleach his teeth.
I noticed that as long as I explained everything I was doing my patient
was more comfortable with the setting. He did also like to stand most the
time, even when he was at home he stands. So sitting in the chair for a 3
hour appointment was long for him. When I could tell he was getting restless I
would ask if he wanted to take a break and I would lead him around the clinic
to stretch and walk around. He was very grateful for the breaks. He also had
to use the restroom twice, which he did not know where anything in the
restroom was. Since his wife had left for the appointment, I asked the patient
if he needed me to walk him in the restroom and show him where everything
was; he did. After bringing him into the restroom I made sure to let him know
that I was right outside the door and if he needed me to just let me know.

I felt I learned a lot from this experience because it forced me to be

aware of all my patients needs. I also had to make sure that I explained all
parts of the procedure in details, even if I was filling out paperwork I made
sure to inform my patient so he knew I was still next to him. Many people are
affected by disabilities, but with the right tools we as hygienist can help each
one meet their dental goals.

Source Citations:
Baker, John. "TopDentist-NY.com." TopDentist-NY.com. Web. 13 Apr. 2015.
http://www.topdentist-ny.com/dental-care-for-the-blind
Belote, Maurice. "Strategies for Successful Medical/Dental Appointments for
Individuals Who Are Deaf-Blind." California Deaf-Blind Services, 2006. Web. 13 Apr.
2015.
http://www.sfsu.edu/~cadbs/20MedicalAppts.pdf
Haddrill, Marilyn. "Retinitis Pigmentosa." All About Vision. 1 Aug. 2014. Web. 13 Apr.
2015.

http://www.allaboutvision.com/conditions/retinapigment.htm
"Retinitis Pigmentosa." Foundation Fighting Blindness. 2015. Web. 13 Apr. 2015.
http://www.blindness.org/retinitis-pigmentosa#rp.
Weinstock, MD, Frank. "Retinitis Pigmentosa Symptoms, Causes, Treatment - How
Long Does Retinitis Pigmentosa Last? - MedicineNet." MedicineNet. Web. 13 Apr.
2015.
http://www.medicinenet.com/retinitis_pigmentosa/page4.htm