Newbie info/ to treat or not to treat RP

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Martha and all Newbies,

If you have not read Dr. Trentham's article about RP on the internet you

should do that first. Search on keyword TRENTHAM It should be the

first option your search engine finds. Print several copies to give your

docs and to carry with you if you are away from home.

RP is one of the diseases that is a real catch 22. If you don't take

treatment you might be OK if your case is mild enough. But it is a

PROGRESSIVE disease and even though it may remit for a while, it comes back

and does more damage. It is ultimately fatal if left untreated. Drugs can

control its progress and the sooner you start taking it the better your

chances of long survival and less disability. But the side effects of the

medicines are also risky. You simply have to decide if the risk is worth the

benefit. Not everyone will suffer the worst side effects. You should ask

your doctor about the percentages of people who do.

Risk without treatment: Lots of pain, saddle nose, floppy ears, blindness,

deafness, collapsed bronchial tubes, damaged heart valves and aorta

(surgery), collapsed rib cage -- etc., and death.

Your doctor should be a Rheumatologist who has experience with Vasculitis if

not with RP. If he does not order proper testing of your hearing, eyes,

heart and bone density (maybe lung capacity, too) in order to monitor

progress of your RP it may indicate he doesn't understand the disease. Find

one who will order it.

In some cases if you have bad side effects you can change to a med. that

won't affect you as much. In other cases you may be stuck with only one or

two choices because of having Vasculitis along with the RP.

Do not put yourself on any vitamin or supplement without checking drug

interactions first. Sometimes a pharmacist will know more than your doctor

and you can tell the doctor what the pharmacist said when you ask about

taking something new.

Almost all RP patients must take prednisone. They start at a high dose and

gradually work down to a maintenance dose or nothing at all. If there is a

flare they increase it again. It is an anti-inflammatory. RP is an

inflammatory disease.

Dose of prednisone during a flare is often related to body weight.

Prednisone side effects:

1) Bone loss. I take FOSAMAX and Monistat Soy (natural

progestin for older women) and at least 1500 calcium daily. Will not know

how bones are affected until next scan.

2) Large Muscles weaken. Legs get really skinny on high

dose (I started at 80 mg and legs weakened quickly). Right kind of exercise

like no-pressure rotations on a stationary bike (I started at 3-5 minutes,

now up to 15 minutes) will keep legs, arms (swing 2# weights, nothing

heavier) in conditon so they come back to normal quickly as dose is reduced

to 10 mg maintenance. Dose is increased again with flares.

3) Feeling " wired " can't sleep. I use Restoril, sometimes 2

if I wake up around 2:00 am and can't get back to sleep for 30 minutes --

take a 2nd then. If the sleep aid your doc prescribes doesn't work, ask for

a different one until you find one that does.

4) Feeling quick tempered or weepy: I get nasty from time to

time but my husband knows it is the Pd, not the real me. He just reminds me

he knows that so I can feel a little guilty (:-)))). When I find myself

overreacting to something (quick to tears) I take St. 's Wort 500s 3x a

day for a week or two and it really helps me keep a level mood.

5) Bone marrow problems and potential Leukemia with

prolonged use.

The object of taking an immuno-suppressant with prednisone is to try to get

you off the prednisone completely or down to a much less risky dose. 10 mg.

is about what the body produces naturally and the risk of Leukemia is much

reduced if you stay on that dose for a long time.

6) Puffy face and weight gain. Prednisone takes the fat from

under your skin and redeposits it around your face, shoulders and tummy

first. You get " thin skinned " and a " flushed " look. You get a moon face

(round and puffy) and " beer belly " (I look 7 months pregnant!) but your legs

and arms might be really skinny. Prednisone increases your appetite and the

higher the dose the worse it is -- if you have good eating habits to start

and know that your hunger is Pd, not real hunger you may be able to control

your own appetite by training yourself to ignore hunger pangs at the wrong

time of day. Knowledge is power!!! As the dose is decreased that is easier

to control. Prednisone makes you hold fluid and you may " blow up " like a

balloon. That effect will also diminish as your dose decreases. Drinking

more water sometimes helps you to get rid of of the fluid, strange as that

sounds.

7) Facial hair grows. I have a light fuzz on the sides of my

cheeks and grew a mustache of fine but darker hair which I bleached with

facial hair bleach from a beauty supply shop. When it was still noticeable

after bleaching I removed it with NUDIT for facial hair removal --

recommended by a dermatologist.

8) Eye pressure increases. Danger of glaucoma or other damage

from high eye pressure. Be sure to have regular eye exams to know your

status in this respect. Also for signs of RP related problems in the eyes

(Scleritis). Per list members eye pressure usually goes down when prednisone

is decreased. But what is too high varies from patient to patient. Any eye

pain must be reported at once.

9) Really bad heartburn. Ask your doctor to give you

something for that when he prescribes the prednisone. I took Prelosec twice

a day until I got down to 20 mg. prednisone. Now I take only one and some

Tums if I need something more from time to time. Drink lots of water.

Prednisone alone generally does not control the disease. Dr. Trentham's

article and the links to the home page there will give you a good description

of all of the drugs that can be used to control RP. Which one you use

depends on your own reactions and just what your symptoms are. For example,

one list member said that Dapsone is especially good for ear problems (inner

ear edema can lead to constant dizzyness to the point of room spinning and

nausea, ear-ringing and eventual deafness). Although I have definite inner

ear problems I don't take Dapsone at present because I also have polyangiitis

(vasculitis) which caused bleeding lungs and kidneys. Those symptoms have to

be controlled because they are more life threatening than the ear problems so

I have to take Cytoxan. If the polyangiitis seems to be in full remission I

may be able to change to another more benign medicine later that is more

specific for RP symptoms.

Side effects of cytoxan include damage to the bladder and liver, bone marrow

suppression and anemia, tummy problems, bleeding (gums especially),

bruising, little purple marks on skin that come and go and shingles --

especially if you have already had shingles. You have to be sure to drink

LOTS of water and void with the slightest urge in particular within first

four hours after taking the med. (I told my husband I should replace my

computer chair with a potty chair!!)

Hair loss is also significant. I lost a lot of mine -- wear a wig in public

but have been using Rogain 5% for men for about 45 days and have a lot of new

growth. You may actually lose some hair from being really sick before being

diagnosed like I was. It is hard to tell if I lost the hair from that or

from the cytoxan or both. All I know is that I am still taking 100 mg

cytoxan a day and the hair is regrowing since I started using Rogaine. It

might have come back without the Rogaine -- we will never know that for sure.

Cyclosporin is a hair grower. Organ transplant patients who take Cyclosporin

with Cytoxan don't have much hair loss per a cousin who is a cardiologist at

Cleveland Clinic. Some RP patients take Cyclosporin. It may increase the

growth of facial hair as one side effect. I don't know what the others are

because when I asked my doctor about it the response was that adding

Cyclosporin with its side effects was unnecessary because I was doing so well

on the Cytoxan alone.

Immuno-suppressants will make you more susceptible to infections if you are

often in crowded, unventilated places or on flights over two hours in an

aircraft. Avoid those situations as much as possible. Wash your hands

religiously in rest rooms and if you have to touch a doorknob when leaving

have antiseptic gel in your purse to put on your hands afterwards or keep a

paper towel in hand to cover anything you touch as you leave. Avoid touching

your eyes, mouth or nose as much as possible.

Keep a list of all your medications, Dr. Trentham's article, and your

doctor's name, address, phone numbers, pager, email, etc. for emergency

contact in your car's glove compartment and in your purse. If you have an

insurance case manager or medical insurance contact, add that info along with

your insurance numbers. Make sure it says how much your medicine should be

increased if there is an acute flare. Always have it with you when you are

away from home. Make sure family members have a copy and know what to do for

you and who to call if you should be with one of them when a flare occurs --

like vertigo so bad you cannot walk. Have an alternate doctor's name in

case your regular one cannot be reached if you need hospitalization -- work

out who that will be with your doctor. It is usually someone in the same

office who your doc will keep informed about you so you can get help when the

regular doc is away.

My first flare was in November of 1998. By June of 99 I had double pneumonia

and bleeding kidneys. Was finally diagnosed in August/September 99 and began

prednisone late August, Cytoxan late September. After less than 5 months on

treatment all the internal bleeding has stopped, kidneys seem normal. My

prednisone is already down to only 15 mg and will be 10 mg by next month. My

puffy face is going away (the tummy is still there) and legs are almost

normal strength.

When the flu was the worst in our city we did not go to church for two weeks

because of the risk of exposure. I had both flu and pneumonia shots this

year and so far have had no problems. My doc has recently given me

permission to take longer flights without putting on a surgical mask after

two hours because I am doing so well. I will be at two conferences over the

spring-summer, maybe three. She said OK as long as the rooms involved are

large and well-ventilated.

I cannot imagine where I would be without treatment -- probably on the road

to rapid kidney failure or death from pneumonia. In my opinion the risk

from side effects of the drugs is one I am willing to take if certain painful

death is the other option.

Hobbs

Colorado Springs