Post from Hobb

[Guest guest]

I wrote to Hobb regarding kidney involvement. She answered me today

and I would like to share her post with you. She has so much information it

is amazing. Such an interesting lady. Wish she had more time to be in the

group again.

Hi ,

You are right about me staying busy. I am in the middle of revising a

workbook I wrote to go with a conference presentation on doing genealogical

research in the countries that were once the Austro-Hungarian Empire. I

will be giving 4 hours of talks at a conference in Canada in July and have to

have the workbook and reference CD I am making to go with it ready by then.

It would be a piece of cake except that all the information is in German and

I still have to do some translations to be sure I have not omitted something

important. I am beginning to feel some time pressure.

Leo is 71 and he is robust as ever - he plays golf as often as possible. He

has finally gotten hearing aids and wears them most of the time. He spent

too much time around cannons as an army Lieutenant.

We are hoping to see our daughter and granddaughter (now one year old) during

the summer. It will be the first time flies from Singapore with the

baby all by herself -- a 25-hour trip! Her husband will join them later.

<< How is your RP behaving?

>>

I seem to be in remission. Except for the purple spots that pop out here and

there and the neuropathy tingling in lower legs there are no really

significant symptoms.

I am still a bit misshapen in the middle and my face, although smaller, is

still not " my " face. Hair has grown reasonably but I am afraid to stop the

Rogaine because I will then find out how much hair I would have if I did not

use it -- all hair resulting from use of Rogain will usually fall out if you

stop it. I don't have to wear a wig and enjoy the wavy hair that replaced my

old crop but the extra strength Rogain makes me grow a delicate mustache that

I have to remove about every six weeks or so.

By the way, I was using Nudit to take that off and told the list it worked

well. But it was a bit irritating. Today I tried the new Nair for the face

and it is much more benign -- at least on my skin. I would recommend it over

Nudit to remove facial hair from prednisone or anything else.

<< I do have a question for you. I know you had a problem with bleeding

kidneys. We have a group member who has had blood in her urine since 2000

and the drs are just now concerned. But they aren't rushing out to do

anything. If you have any information on this I would deeply appreciate it.

I know she is scared, but won't let on and I think she needs more aggressive

treatment. They told her normal is 3-4 and she has 20 cells. >>

I can't help but wonder what kind of doctor either patient has. If either

one is not satisfied with the doctor's attitude about the condition of her

kidneys she should make an appointment with a Nephrologist. There are some

tests that should be done before seeing one and they should have been ordered

by any doctor with even the slightest amount of protein or blood in the urine

of an RP patient or creatinene. A 24 hour urine catch with tests for blood,

protein, creatinine is pretty important as an indicator if the amounts of any

of those are alarming.

I still have to have 24 hour tests every three months although those during

the last year indicate that the kidney damage I had is either healed or the

damaged parts of the kidneys have simply stopped functioning at all. What is

left is functioning OK - there nas been no blood for a very long time and the

protein count that topped out at over 1000 (units) for 24 hours is now down

below 100.

The numbers your patients got sound as if they are made for a little sample.

They really should demand the 24-hour test.

As far as I am concerned, my kidneys would still be bleeding and passing

protein if it weren't for Cytoxan. Prednisone alone simply is not enough.

What's more, delaying use of an immunosuppressant to " see if pred can do the

job " means that you have delayed the time when the second drug will really

" kick in. " Immuno suppressants don't get rid of antibodies already in your

blood - they merely suppress production of new antibodies. It takes almost

six months before the little beasties are diluted enough for symptoms like

increasing kidney damage to be halted and it takes even longer before healing

really affects how any damaged organ functions.

When much are dealing with kidneys and aortas the patient should be given the

opportunity to choose whether to start a potentially toxic immuno suppressant

sooner or later, it should not be just the doctors decision.

There is another point about symptoms of kidney damage that occurs with RP

when the RP appears to be a recent development. Some of the research I have

read seems to say that RP will " eventually " damage kidneys. When kidney

damage occurs in the early stages it can signal the presence of another

vasculitis like Wegner's or polyarteritis or SLE, etc., with the RP. Tests

for all of those problems should be done as well as for abnormal levels of

myrlooperoxidase (sp?) and proteinase 3. Sometimes the latter will be

present for quite some time but the ANCA antibodies will not show up unless

there is a vasculitis specific flare in progress -- as opposed to an RP

flare. I was tested for ANCA three times over two months period before I

finally showed a positive P-ANCA. But all that time the myeloperoxidase and

proteinase 3 were abnormal - which was one of the reasons the docs kept

testing for the ANCA until they found it.

Any biopsied tissue positive for vasculitis (ANCA antibodies) will fluoresce

in a certain light. Biopsied tissue must be preserved in a special manner in

order to be properly tested for fluorescence -- it cannot be treated with

formaldehyde or other similar preservatives.

Surgeons cannot assume that labs will automatically preserve the tissue

properly and patients should ask their surgeons if they have instructed the

lab. I had a lung biopsy (not a minor event when they collapse a lung and

remove a 6 " strip of tissue) and the hospital lab screwed up and failed to

preserve some of the tissue for fluorencense testing -- the most important

test and main reason for the biopsy. Result was that start of my treatment

was delayed an additional six weeks while the docs set the samples to Mayo

and elsewhere to see if anyone could get a vasculitis diagnosis from them.

An MRI of the brain can also show small lesions that occur with vasculitis

and can help in diagnosis.

An MRI or CT scan of the kidneys may show some specific glomul....(sp?)...oma

or scaring that indicates RP or vasculitis damage.

A nephrologist may want to do a kidney biopsy but any one of them who is

honest with his patients will discuss how reliable the biopsy is -- generally

it is VERY difficult to get the right tissue sample and the biopsy is

inconclusive. It should be a last resort. The reason a nephrologist

should be consulted is for best input on just how alarming the amounts of

blood and protein in the urine might be when the PCM seems to be unconcerned.

A nephrologist may insist on aggressive treatment when the PCM is reluctant.

I was led to believe that ANY amount of protein and blood in the urine needs

diagnostic testing ASAP.

With some types of vasculitis, (Polyarteritis and Wegners) if the patient is

not treated aggressively kidneys can fail in two years (I was a third of the

way there and am now healed to the point of approaching normal.)

Have you seen the article by Karin Harp at

http://www.emedicine.com/med/topic2000.htm ???

Harp may be a colleague of Buckner - she is from Seattle if I recall.

The paper prints up to 33 pages with references. On page 6 the third

paragraph says complications of RP include vertigo, tinnitus, hoarseness,,

etc... and glomerulonephritis-associated renal failure. Any doctor treating

an RP patient should be given a copy of that paper with that paragraph

highlighted.

Page 12 has more on the subject -- says that 29 or 129 (22%) of RP patients

seen at Mayo clinic had evidence of glomerulonephritis based on renal biopsy

or presence of microhematuria (blood) and proteinuria (with one of these at

least a 2+ (!!) on urinalysis). Biopsies showed segmental necrotizing

glomerulonephritis or renal vasculitis.

Patients with renal damage .. older...more likely to have extrarenal

vasculitis and arthritis. Renal involvement can greatly affect life

expectancy except when the patient is already " elderly " (like me -- I am now

almost 66).

The top paragraph on that page gives the survival rates based on available

data but just because there have not been problems from toxic medications

within 5 years does not mean there won't be any. I have found research that

says that people taking Cytoxan for 15 years without any bladder problems

(hemocystic cistitis) will suddenly show pre-cancerous cells in the bladder.

So those of us who have to take these drugs the rest of our lives have to

maintain vigilance no matter how long we have been taking them.

Speaking of the bladder, when there is blood in the urine it may be important

to a nephrologist to be sure that it is not coming from the bladder. It is

also a bad idea to start taking something like Cytoxan which can cause

bleeding from the bladder without being sure that the bladder is intact when

you start the drug. So a cystoscopy is usually needed.

All of the immuno suppressants have their unpleasant side effects and can

effect the liver and bone marrow. However, the side effects of the drug

should not influence the dr. and patient too much when deciding which one to

use. When Wegner's or Polyarteritis is present with RP, then Cytoxan is the

BEST drug at present (Methotrexate is not used for Wegner's or Pa).

Switching to Imuran may be possible after symptoms are controlled but the

best prognosis regarding the disease itself will result with Cytoxan. Of

course this treatment can be almost as bad as the disease if you happen to be

susceptible to the side effects before it has done its job. The patient

should be given all the information about any drug that will be used for

treatment and allowed to give some input on preferences. Some people may be

willing to take the risk of organ damage rather than face drug side effects

and vice versa.

Dr Harp's paper says that the 5-year survival rate for RP with systemic

vasculitis like I have is 5 years with secondary infections being the primary

cause. That means we have to be very careful about exposing ourselves to

flu bugs, colds, relatives and friends who have any respiratory problems,

etc., forever. My family is aware that when anyone has a cold, they simply

stay away from me. My husband will even sleep in a different bed if he is

stuffed up because he says it is not worth the risk of exposure to be in bed

with me.

There are Lab workups suggested on page 15 of Dr. Harp's paper. There is an

email link for Dr. Harp at the bottom of the " Author Information " on the

first page of the article. I just noticed -- she is from Portland, not

Seattle.

By the way, when I was still on the list I was taking Fosamax to counteract

the calcium depleting side effects of prednisone and my own post-menopausal

symptoms. After I left the list I had to stop taking it because I began to

develop GERD from it. I understand that the newer forms of Fosamax are not

as hard on your stomach but tell the list that if they wake up at night

feeling like there is a burp that wants to come up and drinking water doesn't

help, they probably have GERD. It can be from Fosamax, from high dose

Cytoxan and from some immuno suppressants.

My doc switched me to Miacalcin (nose spray for calcium bonding). I have not

had a bone density test since the switch, will probably get one soon. I had

insisted on staying on the Fosamax when she first suggested my burpy-ness was

because of that and I ended up staying on long enough to cause some scarring

in my esophagus the created a slight narrowing. I began to experience pain

when swallowing so a Gastroenterologist did an endoscopy (with biopsy) to

stretch the esophagus and look at what was going on down there.

My point is that you should not tolerate too much burpiness or painful

swallowing or spasms when you swallow. Find out if one of your meds is

causing it and what can be done right away. If the esophagus gets irritated

too much it can lead to cancer of the esophagus (my uncle had that...) and

you should not ignore it as something that can't be helped. If you cannot

change meds there may be other steps you can take or you may have to schedule

exams that check the esophagus every so often just to be sure it is OK.