Expert Guest Chat Log
Topic: The Latest Ph Therapies
Date: February 5, 2001
Guest: Robert Schilz D.O., Ph.D.
<Moderator> ***ATTENTION***
<Moderator> ***Welcome to the PHCentral Guest Expert Chat
<Moderator> ***Our Guest Expert today is Dr. Robert Schilz, a pulmonologist
from Cleveland Clinic in Cleveland, Ohio
<Moderator> ***Our topic is - Latest PH Therapies
<Moderator> ***The moderator today is Michael Szczepkowski - that's me!
<Moderator> ***Here are the guidelines for today's chat:
<Moderator> ***If you have a question, please send a "?"
<Moderator> ***I will keep track of who is next in order and then....
<Moderator> ***call on you to ask your question.
<Moderator> ***Dr. Schilz will answer your question and then I will ask if you
to ask a followup question if you have one.
<Moderator> ***When you see some text followed by "..." that means the
person... ***When you see some text followed by "..." that means the
person...
<Moderator> ***still has not finished typing.
<Moderator> ***It would also help if you had your question typed and ready to
submit when I call on you.
<Moderator> ***There will be a log for today's chat posted on PHCentral.
<Moderator> ***OK, we are ready to begin!!***OK, we are ready to begin!!
<Moderator> *** If you have a question...
<Moderator> ***please type and send a "?"
<alex> ?
<El> "?"
<Dr. Schilz> Good evening, everyone. Thank you for your chat invitation this
evening.
<Nancy A> ?
<Moderator> Go ahead, Alex
<alex> hi dr schilz and Michael will flolan'ers be given bosentan/beraprost and
will it have to go thru a clinical trial
<monica> ?
<Vi> ?
<alex> hello?
<Moderator> He is typing
<alex> sorry
<Dr. Schilz> Alex, at this time I am not aware of protocols which will include
patients already on prostacyclin in endothelin antagonist trials.
<Moderator> Alex, do you have a follow-up?
<alex> is that something that will come in the future?
<Dr. Schilz> As you may know, endothelin antagonists are a novel class of
agents which may impact different elements of the pulmonary vasculature
compared to the prostacyclins. For this reason....
<Lori> "?"Lori
<Dr. Schilz> the use of this agent in addition to prostacyclin is an exciting
concept which I suspect will be investigated.
<Moderator> EI, you are next, go ahead.
<El> Hi Docotor S. I'm a fellow Ohioan. I am also interested in Bosetan and
Beraprost-would a doc switch someone doing WELL on Procardia to opne of
these?
<El> I mean ONE of these
<Dr. Schilz> El, I am afraid that I will have to give an answer which you may
see again this evening and that is -it depends....
<Dr. Schilz> many therapies in PH are added to medicines already given.
Certainly if calcium channel antagonists have normalized your functional
capacity and pulmonary pressures...
<Dr. Schilz> then consideration of additional therapy probably would not add
much. If however this is not the case then additional therapies as you suggest
may be a consideration,
<Moderator> EI, do you have a follow-up?
<alex> el left
<Moderator> Nancy, you are next.
<Nancy A> are there medications to control CREST resulting in slow/stop SPH
progression?
<Dr. Schilz> Nancy, I want to make sure that I understand your question first
are your asking about therapy targeted against CREST or against the
associated PH?
<Nancy A> had hoped to attack the CREST and subseqent PH -- just
DX....clinical trials specifically avail at cleveland clinic for CREST/SPH (early
DX)-expecting referral to CC
<Dr. Schilz> Nancy, I hope this answers your question-
<Moderator> Dr. Schilz is typing...
<Dr. Schilz> The management of CREST syndrome (Calcinosis cutis,
Raynauds, esophageal dysmotility, sclerodactyly and telagiectasias) depends
on the extent of skin, kidney, esopahgus and lung involvement..
<Amy> "?"
<Dr. Schilz> As in all patients with a question of PH, the cause and presence
must first be identified before talking about therapy......
<Dr. Schilz> CREST, in some cases can be associated with a scarring lung
disease which can cause low oxygen levels and PH........
<Dr. Schilz> additionally, small vessel changes can also lead to PH. Current
therapies for PH include: prostacyclin agents.......
<Denis> ?
<Dr. Schilz> calcium channel antagonists. Some recent trials using new agents
were just completed probably with more .....
<Amy> Amy "?"
<Dr. Schilz> on the way which I suspect will include patients with scleroderma
or CREST. I hope that answers your question- These therapies are available
at our (and other )PH centers
<Moderator> Nancy, do you have a follow-up?
<Nancy A> my doctor here in Dayton has recommended referral to you
personally, working to get approval (USAF -TRICARE). CCB has improved
dyspnea.
<Debra L. Branscomb> ?
<Dr. Schilz> I am glad that you are feeling somewhat better - will be happy to
help.
<Moderator> Monica, go ahead.
<Nancy A> thanks-looking forward to meeting you
<monica> I have mild PH and will be starting on CCB's soon. One thing that
scares me is edema. Does everyone experience this to some degree? Are
there those that do not experience it?
<Dr. Schilz> Monica - you are correct in that edema or swelling of the feet can
occur in patients that use CCBs......
<Dr. Schilz> this can happen in patients without PH that take CCBs for other
reasons. Not all patients have this side effect....
<Dr. Schilz> probably <25%
<Moderator> Monica, follow-up?
<monica> yes
<annie> ?
<monica> Do CCB's slow the progression of PH,help it regress or just help to
manage the symptoms?
<Dr. Schilz> In patients that "respond" to the administration of CCBs,
improvement of symptoms and regression of .....
<Dr. Schilz> right heart strain and pressures have been documented.
<Moderator> Vi, you are next.
<Vi> What is used to treat exercised induced ph with no known cause? I have
been through neuro-muscular & rheum. cardio & pulm. consults all under the
direction of one doctor with every known test short of one which is the lung
biopsy in which they don't know where to biopsy so have decided against that.
<Dr. Schilz> Vi, the issue you describe is a very interesting one - as always the
causes and exclusion of other factors which can lead.....
<Dr. Schilz> to significant pressures during exercise must be carefully excluded.
If this has been done then the patient with elevated....
<Moderator> Dr. Schilz just lost connection.....
<Moderator> He is signing back on...
<Moderator> please hold on...
<Moderator> Still signing on...
<alex> welcome to phcentral Jim!
<JimNY> Jim NY
<Moderator> I hear his foot steps...
<alex> lol
<El> I know how it is- lost connection earlier and didn't get to read ans to my ?
<Moderator> False alarm...
<Moderator> I am on the phone with him...
<Vi> ok
<El> sooooo. . .have to read the log!
<Moderator> Sorry for the delay...
<alex> el, there will be a log of this chat where you can see the response
<El> i know- can't wait to read it
<alex> :-)
<Moderator> Here he is....
<Moderator> He will continue to answer the last questions....
<Vi> ty
<Dr Schilz> pressures only with exercise is typically treated with "usual"
therapies which may consist of ccbs, or prostacyclin agents depending on the
.....
<Dr Schilz> severity of pressures and symptoms.
<Moderator> Vi, do you have a follow-up
<Moderator> Vi??
<Vi> no thank you Dr. Schilz
<Moderator> Lori, go ahead.
<Lori> <Lori>When should one start therapy, at a certain pa pressure? I have
sph due to restrictive lung disease as a result from radiation, also I desaturate
with brisk walking or elevation. .
<Moderator> btw, there will be a transcript of the chat posted on PHCentral.
<El> ?
<Moderator> So you will not miss anything.
<Dr Schilz> Lori, you actually have a complicated series of points within your
question.....
<Dr Schilz> In general, there is no magic number or pressure signifies the need
for treatment of PH in all cases from all causes....
<Dr Schilz> Your report of symptoms, desaturation and restrictive lung disease
from radiation brings up an entirely different discussion.......
<Dr Schilz> Without wishing to be repetitive, the contribution of low oxygen
levels, lung destruction and the effects of pulmonary hypertension per
se..........
<Dr Schilz> on your symptoms must be carefully assessed prior to initiating
therapy. One principle however regardless of the cause of ......
<JimNY> Any comment on the use of ARB for initial treatment of newly
diagnosed mild PH. This course of action was rec by my cardio to my interist
<Moderator> LoriJim, please wait. I will add you to the list
<Dr Schilz> your low oxygen levels is that oxygen supplementation in general
should be given to keep saturations within reasonable ranges ....
<Dr Schilz> during rest and exercise,
<Moderator> Lori, do youhave a follow-up?
<Lori> >Lori> Would an echogram be the best way to be monitored?
<Dr Schilz> Echocardiograms provide one piece of information - exercise
capacity, oxygen levels, in some cases pulmonary function tests.....
<Dr Schilz> may be as or even more important in following your response to
therapies.
<Moderator> Amy you are next.
<Pauline> jim.what's arb?
<Amy> Dr. Schilz, I have copd/sph and would like to know if there are any
clinical trails available. I understand flolan has not been approved for
emphysema. I have already progressed to stage three and comfortable at rest
with 02. I was evaluated for a lung transplant and breast cancer was found
almost two years ago so as you can see I'm desperate. I'm on norvasc and
cardizem but I have not improved with sob. I would appreciate any advice. (5
years) due to breast cancer last year.
<Dr Schilz> Amy, you are correct that Flolan has not been approved for
emphysema and PH. The transplant issue and previous diagnosis of cancer is
also.....
<Rosalind Feldstein> All new to me. Drs advising cardiac catherization
<Dr Schilz> a difficult issue. Management of the symptoms and consequences
of emphysema is difficult. Adequate oxygenation is .....
<Amy> I go back to UAB tomorrow...with the news norvasc has not worked
what can I do? I will not give up
<Dr Schilz> important at rest, sleep and exercise. Appropriate use of inhaled
agents is also important. The limitation in patients with.....
<Dr Schilz> emphysema is often these and not the SPH per se although some
patients do have high pressures.....
<Dr Schilz> the remaining treatment options for emphysema may include lung
volume reduction therapy under certain........
<Dr Schilz> circumstances. There are no current trials specifically for PH and
emphysema. CCBs were certainly a reasonable agent to try.
<Moderator> Amy, do you have a follow-up?
<Amy> Everyone pray for my family. Thank you Dr.Schilz.
<Moderator> Denis, you are next.
<Dr Schilz> Thank you Amy
<Denis> Can CREST be associated with or diagnosed as, chronic
thromboembolic diesease ?
<Dr Schilz> Denis, It is possible to have CREST and CTEPH which are related
or totally unrelated. I will expand......
<Dr Schilz> It is possible to have both CREST and unrelated pulmonary emboli
which do not resolve leading to the syndrome of CTEPH....,
<Dr Schilz> CREST belongs to a family of connective tissue diseases which can
include Lupus Erythematosis, Scleroderma or polymyositis....
<Dr Schilz> there are occasionally overlaps between these. This is important
because tendencies to form blood clots can be......
<Dr Schilz> associated with some of these overlap syndromes and lead to
CTEPH.
<Moderator> Denis, do you have a follow-up?
<Denis> I have been diagnosed with lung damage from chronic thromboembolic
disease, and am set to have a thrombo-endarterectomy Could it be something
else?
<Moderator> Reminder, if you have not aswered the Treatment Poll on the
PHCentral hoem page, please do!
<Dr Schilz> If appropriate testing to identify these chronic, unresolved
occlusions has been done and they are of a location and magnitude.....
<Dr Schilz> suggesting that they are the cause then endarterctomy may well be
appropriate.
<Moderator> Debra, you are next.
<Debra L. Branscomb> Debra?i have pph I have had a lung bx i also have
severe problems withright chf and renal insufficiencyI can not tolerate ccbs
what would be the best therapy for me i avserage 2-4plus pitting edema
<Dr Schilz> Debra, It sounds like you are having quite significant symptoms of
right heart strain. I will however at the risk of repeating (again)....
<Dr Schilz> say that thorough testing to diagnose the cause of PH and
symptoms must be completed prior to suggesting therpy.......
<Dr Schilz> Assuming you have pulmonary hypertension which is idiopathic
(aka "primary") the severity of symptoms would .......
<Dr Schilz> suggest the instituition of prostacyclin.
<Moderator> Debra, do you have a follow-up?
<Debra L. Branscomb> no
<Moderator> Annie, you are next.
<annie> Can Flolan cause abdominal fullness and/or bloating?
<Dr Schilz> Annie, Flolan can lead to ascites which leads to a fullness or
pressure. Flolan can also lead to nausea or loose bowel.......
<Dr Schilz> movements which may be interpreted as bloating or fullness. To
complicate this matter, PH with significant right......
<Dr Schilz> heart failure can also lead to the abdominal symptoms you describe.
<Moderator> Annie, do you have a follow-up? EI will be next and then JimNY
will be last.
<annie> thank you very much. Was concerned about abdominal fullness.
<Moderator> EI, go ahead
<El> It seems me most doctors beone's PPH cannot actually *regress*w/ CCbs,
however *regression* may be possible lieve PPH cannot actually *regress*
w/ Procardia therapy (like some do believe it regresses w/ Flolan), should they
cons-since most doctors feel
<El> Sorry- that is not typed correctly!
<El> you can just ans the 1st part
<El> no regression (lung remodeling) w/ CCbs . . . right?
<Dr Schilz> EI, Thanks for the clarification- I thought my eyes were going. As I
think you are implying, the definition of "regression"......
<Dr Schilz> needs to be understood to answer this question. Many researchers
and physicians know that protacyclin has many properties.....
<Dr Schilz> some of which may lead to fundamental changes in narrowed and
thickened blood vessels to actually help remodel damage....
<Dr Schilz> seen in PH. Since the benefit of prostacyclin is often seen in
patients which do not respond to acute vasodilator testing....
<Dr Schilz> (unlike CCB's), many feel that what must be going on is true
regression. This is dificult to prove. It is not known however, if patients that....
<Dr Schilz> respond to CCB's may also be undergoing some regression as well.
<Moderator> EI, do you have a follow-up?
<El> What about promising new therapies (Bose, Bera)? Possibility of
"regression"(remodeling)? (THX 4 your time tonite, Dr!)
<Vi> ?
<Moderator> Vi, sorry, we only have time for Jim's question next.
<Dr Schilz> Prostacyclin agents (Uniprost(UT-15), beraprost) are the same
basic molecule which would chemically be expected.......
<ME> May I ask a question?
<Moderator> Sorry ME, we only have time left for Jim's question.
<Dr Schilz> to have similar properties to FLOLAN. Endothelin antagonists are
also intriguing with regard to the possibility of .....
<Dr Schilz> remodelling. Endothelin can cause growth withing blood vessels
under some circumstances, whether long term blockade .......
<Dr Schilz> of this substance can lead to remodelling is unknown but possible.
<Moderator> Jim, go ahead.
<JimNY> Any comment on the use of Diovan or Avapro on newly
diagnosed"mild "PH
<Moderator> Jim, are they CCBs?
<JimNY> ARB
<Moderator> Thanks
<JimNY> angiotensin recept blockers
<Dr Schilz> Jim, There has been interest in the contribution of the angiotensin
system in the systemic response to heart failure in general.....
<Moderator> Dr. Schilz has agreed to answer two more questions - one from Vi
and one from ME. I will call on you after jim's follow-up.
<Dr Schilz> lessons learned from the left heart. Also the possibility that
angiotensin can participate in growth or blood vessel health and....... and the
<Dr Schilz> response has also been postulated. There are some preliminary
investigations with regard to this but I could not recommend that alone at this
time....
<Dr Schilz> that may change in the future.
<Moderator> Jim, follow-up?
<JimNY> this was rec by mt cardiol to my internist as a first treatment for
unloading
<Moderator> Vi, go ahead.
<Vi> Can prednisone especially at high doses of 100mg a day exaserbate ph?
<Vi> I'm sorry I don't know if I spelled exasberate correctly what I mean by
that is worsen ph
<Moderator> We lost Dr. Schilz again...
<Moderator> Please hold....
<Moderator> He is signing on again.
<Moderator> Sorry.
<Moderator> Foot steps again.......
<Amy> <Amy>knock...knock...
<Moderator> Here he is...
<Moderator> He will respond to the las tquestion by Vi
<Dr R Schilz> Vi, Prednisone can lead to salt and water retention which can
make edema worse. There have been a (very) few rare cases where PH in
the setting of a connective tissue disease has actually....
<Dr R Schilz> gotten better with prednisone. That much prednisone certainly is
associated with the potential for significant....
<Dr R Schilz> undesirable side effects if used long term. Its expected benefits
should always be weighed against these detrimental effects.
<Moderator> Vi, follow-up?
<Vi> Thank you for your time Dr. Shilz. I am doing much better being off
prednisone. no further questions . Do you have any questions for me?
<Dr R Schilz> Vi, thank you for the offer but I am not sure I can come up with
any of the quality that all of you have given me.
<Moderator> ME, you are next and last.
<ME> Will Bosanden/Tracleer be available "off label" on a named patient basis
before it gets free approval, say on grounds of "compassion"?
<Dr R Schilz> The matter may be under consideration. At this time open label
use outside of the previous trials it is not available.
<Moderator> ME, follow-up?
<ME> Do you know when and if a decision will be made? Has the safety
protocol already been written?
<Dr R Schilz> I could not answer that.
<JimNY> JimNY
<Dr R Schilz> Thank all of you for the invitation this evening and to the
moderator for all of their help. Have a good evening. rjs
<ME> Thank you for taking my question. I'm just wondering if any of these new
drugs on "the verge" of approval would be available to some patients who
might need them in a "life or death" situation.
<El> Thanks so much for your time, Doctor Schilz!!!! You are greatly
appreciated!!! *YOU TOO, MICHAEL!!!*
<Amy> Amy> Good night Dr.Schilz. And Michael, as always, great chat.
<Moderator> That's it Folks!! Thanks Dr. Schilz!!! Thanks everyone for
attending!!!
<A & C> Thanks Dr. Schilz
<alex> ANYONE WISHING TO STAY IN THE CHAT ROOM AND
DISCUSS THIS CHAT... THE CHAT ROOM WILL BE OPENED
<A & C> Thanks, All
<StacyinCO> Thank you
<JimNY> THx