Re: Pulmonary Hypertension, explained

[Guest guest]

Wow Tina this is great information and kind of makes very clear what Joyce is up against. Thanks for sharing all this, just makes me know I have to keep prayin!

Beth in NC age 48 Fibrotic NSIP 06/06

"For as long as I shall live, I will testify to Love."

Pulmonary Hypertension, explained

Hi Kerry: Thanks for sharing this information about Joyce. My number is also "64", which is actually a percentage, so 64%. This is considered Class III/IV Pulmonary Arterial Hypertension or, PAH, or just PH. The World Health Organization definition of Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. These patients are comfortable at rest, but less than ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope. And the definition of Class IV: Patients with pulmonary hypertension resulting in inability to perform any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may be present at rest, and discomfort is increased by any physical activity. The World Health Organization also says, normal pulmonary artery systolic pressure at rest is 18 to 25 mm Hg,

with a mean pulmonary pressure ranging from 12 to 16 mm Hg. This low pressure is due to the large cross-sectional area of the pulmonary circulation, which results in low resistance. An increase in pulmonary vascular resistance or pulmonary blood flow results in pulmonary hypertension. The drug they are prescribing for Joyce would be known as Flolan. The following is the information relative to Flolan,(Generic = Epoprostenol) . The following information is supplied by the Pulmonary Hypertension Association on their website, www.phassociation. org.What is epoprostenol? Epoprostenol is an intravenous medication used to treat pulmonary arterial hypertension (PAH). It is a synthetic (not obtained from other humans or animals) form of a substance made by the body called prostacyclin. It was approved by the FDA in 1996 for the treatment of pulmonary arterial hypertension in patients with New

York Heart Association Class III or IV symptoms. How does epoprostenol work? Epoprostenol dilates blood vessels, prevents the smooth muscle cells in their walls from contracting, and reduces the stickiness of circulating platelets which might otherwise sludge up the vessel. It also helps the right side of the heart to better pump blood through the lungs. There is evidence that prostacyclin production is reduced in patients with pulmonary arterial hypertension (PAH). Improvement in exercise capacity and sense of well-being has been demonstrated in short-term studies of epoprostenol. Studies also showed improvement in survival in patients with idiopathic PAH. Several longer-term series have suggested significant survival benefit when compared to predicted survival or to historical untreated control patients. How is epoprostenol given? Epoprostenol is given by continuous intravenous

infusion through a permanent catheter placed in one of the large veins going to the heart. It must be given this way because epoprostenol lasts for only a very short time (3-5 minutes) in the bloodstream. A pump (the CADD Legacy pump) is used to deliver the drug. The drug comes as a powder and must be dissolved in a special alkaline solution (diluent) which increases its stability in the bloodstream. The drug solution is then put in a cassette which attaches to the pump. If the solution is to be kept longer than 8 hours, it must also be kept cool, usually by means of freezer cold packs placed around the cassette.Epoprostenol must be initiated in a setting with adequate personnel and equipment monitoring and emergency care. At most centers, patients are started on epoprostenol in the hospital. A dedicated nurse clinician is mandatory to help manage problems related to side effects of

epoprostenol and to ensure appropriate dosing. In addition, the nurse clinician must teach patients how to mix epoprostenol, keep the central venous catheter clean, and manage the delivery pumps. Dosing of epoprostenol Epoprostenol is dosed according to a patient's body weight, in kilograms (kg). The starting dose is generally 1-2 nanograms (ng) per kg per minute. The dose is increased according to the response and side effects of each patient. Usual long-term doses range from 20-40 ng per kg per minute. What are the major problems with epoprostenol?A major risk is interruption of the infusion, which in some patients can cause severe worsening of their PAH and even death, in just a few minutes. Therefore, patients should always have a prepared backup medication cassette and an infusion pump nearby. In an urgent situation, epoprostenol can be given through a peripheral vein.A

second major risk is that of infection related to the chronically indwelling intravenous catheter. The infections may involve the catheter site or the catheter itself, and this may lead to an infection in the blood stream. Any sign of infection warrants immediate medical attention. The infusion must be prepared daily, which can occupy up to an hour of time. What are the frequent side effects of epoprostenol? Epoprostenol infusion causes a variety of systemic side effects, partly related to its action as a vasodilator. These include headache, flushing, jaw pain, bone pain, diarrhea, palpitations, and rashes. The number and severity of side effects varies among patients. How is epoprostenol supplied?Epoprostenol is supplied as a powder in concentrations of 0.5 mg and 1.5 mg. The alkaline solution or diluent is provided in 50 ml vials. Epoprostenol is then dissolved in the diluent. Each

container of dissolved epoprostenol can be used one time only. Once dissolved, the medication can be stored cold for up to 48 hours. How do patients obtain epoprostenol?Epoprostenol must be prescribed by a physician and insurance approval must be obtained prior to starting therapy. Once approved by insurance, epoprostenol is then sent directly to patients by either of the two specialty pharmacies: Accredo Health Inc. or Caremark. Will insurance pay for epoprostenol?The cost of epoprostenol is approximately $100,000 per year but may be higher depending upon patient dose. Most insurance, as well as Medicaid and Medicare, will pay for epoprostenol. Could a patient be allergic to epoprostenol?There are no reports of this and it is unlikely that this would occur since prostacyclin is made by the body. Patients may, however, develop severe side effects (listed above). No medications are

prohibited with the use of epoprostenol. Maybe this is too intense, but I researched all of this as I was diagnosed at the same percentage as Joyce and was very concerned. I am not a Flolan patient at this time, as my doctor has opted for me to try Tracleer first. So far, I can't complain too much. I've dealt with worse and I am head strong. I don't feel well most days, but that has been the "norm" for so long, I am just beginning to understand I have changed and I will learn to adapt to my new self. Take care and God Bless,TinsIPF 04/07 PH 11/07 Ohio>> I called again looking for an update and announcing myself as > Joyce's friend Kerry looking for an

update. To my surprise it was > Joyce herself answering. I would've said hold my calls. Though she > claims to still be a little doped up after the heart caths she > sounded completely lucid to me and she's doing well. She said that > the cath showed a level of 64 (I don't know the measurements this is > in folks, just what she told me) and that the level it should be is > 24. So, yes she has PH, big time. They also found 2 blockages (and > I think she said one was 70%), but they did not put in any new > stents at this time. Rather than do the Revatio or Tracleer, she > said they are going to give her a different med that is administered > thru a port (I had never heard the name of it, nor had she so I have > no clue how to spell it... she said it's called "Flo - something", > how's that for a specific detailed report? No criticism allowed >

folks). She is going to be in the hospital for 5-7 days so they can > see how she tolerates this new medicine. They are going to try to > get this PH under control before addressing other things like the > stents for blockages and any new meds like the Rituxan. She said > once again how much she really likes and is impressed by this new > doctor. I am so glad for that. I told her since she had such a big > day today and all her family around the last 2 days that I would > leave her be. I will go visit over the next couple days and be so > glad to do it because w/ her busy and crowded household lately > visiting has just not been as easy. I told her I had posted earlier > and would post again now w/ the latest.> > Good day from the Queen and her humble messenger girl,> Kerry> IPF '01> S.

IN>

[Guest guest]

Tina

Incredible Information. Thank you so much.

> >

> > I called again looking for an update and announcing myself as

> > Joyce's friend Kerry looking for an update. To my surprise it

was

> > Joyce herself answering. I would've said hold my calls. Though

> she

> > claims to still be a little doped up after the heart caths she

> > sounded completely lucid to me and she's doing well. She said

that

> > the cath showed a level of 64 (I don't know the measurements this

> is

> > in folks, just what she told me) and that the level it should be

is

> > 24. So, yes she has PH, big time. They also found 2 blockages

> (and

> > I think she said one was 70%), but they did not put in any new

> > stents at this time. Rather than do the Revatio or Tracleer, she

> > said they are going to give her a different med that is

> administered

> > thru a port (I had never heard the name of it, nor had she so I

> have

> > no clue how to spell it... she said it's called " Flo -

something " ,

> > how's that for a specific detailed report? No criticism allowed

> > folks). She is going to be in the hospital for 5-7 days so they

> can

> > see how she tolerates this new medicine. They are going to try

to

> > get this PH under control before addressing other things like the

> > stents for blockages and any new meds like the Rituxan. She said

> > once again how much she really likes and is impressed by this new

> > doctor. I am so glad for that. I told her since she had such a

> big

> > day today and all her family around the last 2 days that I would

> > leave her be. I will go visit over the next couple days and be

so

> > glad to do it because w/ her busy and crowded household lately

> > visiting has just not been as easy. I told her I had posted

> earlier

> > and would post again now w/ the latest.

> >

> > Good day from the Queen and her humble messenger girl,

> > Kerry

> > IPF '01

> > S. IN

> >

>

[Guest guest]

Thanks Tina this was very informative.

IPF2/07 IL

--- Tina wrote:

> Hi Kerry: Thanks for sharing this information about

> Joyce. My

> number is also " 64 " , which is actually a percentage,

> so 64%. This is

> considered Class III/IV Pulmonary Arterial

> Hypertension or, PAH, or

> just PH. The World Health Organization definition

> of Class III:

> Patients with pulmonary hypertension resulting in

> marked limitation

> of physical activity. These patients are comfortable

> at rest, but

> less than ordinary physical activity causes undue

> dyspnea or fatigue,

> chest pain or near syncope.

> And the definition of Class IV: Patients with

> pulmonary hypertension

> resulting in inability to perform any physical

> activity without

> symptoms. These patients manifest signs of right

> heart failure.

> Dyspnea and/or fatigue may be present at rest, and

> discomfort is

> increased by any physical activity.

>

> The World Health Organization also says, normal

> pulmonary artery

> systolic pressure at rest is 18 to 25 mm Hg, with a

> mean pulmonary

> pressure ranging from 12 to 16 mm Hg. This low

> pressure is due to the

> large cross-sectional area of the pulmonary

> circulation, which

> results in low resistance. An increase in pulmonary

> vascular

> resistance or pulmonary blood flow results in

> pulmonary hypertension.

>

> The drug they are prescribing for Joyce would be

> known as Flolan.

> The following is the information relative to

> Flolan,(Generic =

> Epoprostenol). The following information is

> supplied by the

> Pulmonary Hypertension Association on their website,

>

> www.phassociation.org.

>

> What is epoprostenol?

> Epoprostenol is an intravenous medication used to

> treat pulmonary

> arterial hypertension (PAH). It is a synthetic (not

> obtained from

> other humans or animals) form of a substance made by

> the body called

> prostacyclin. It was approved by the FDA in 1996 for

> the treatment of

> pulmonary arterial hypertension in patients with New

> York Heart

> Association Class III or IV symptoms.

>

> How does epoprostenol work?

> Epoprostenol dilates blood vessels, prevents the

> smooth muscle cells

> in their walls from contracting, and reduces the

> stickiness of

> circulating platelets which might otherwise sludge

> up the vessel. It

> also helps the right side of the heart to better

> pump blood through

> the lungs. There is evidence that prostacyclin

> production is reduced

> in patients with pulmonary arterial hypertension

> (PAH). Improvement

> in exercise capacity and sense of well-being has

> been demonstrated in

> short-term studies of epoprostenol. Studies also

> showed improvement

> in survival in patients with idiopathic PAH. Several

> longer-term

> series have suggested significant survival benefit

> when compared to

> predicted survival or to historical untreated

> control patients.

>

> How is epoprostenol given?

> Epoprostenol is given by continuous intravenous

> infusion through a

> permanent catheter placed in one of the large veins

> going to the

> heart. It must be given this way because

> epoprostenol lasts for only

> a very short time (3-5 minutes) in the bloodstream.

> A pump (the CADD

> Legacy pump) is used to deliver the drug. The drug

> comes as a powder

> and must be dissolved in a special alkaline solution

> (diluent) which

> increases its stability in the bloodstream. The drug

> solution is then

> put in a cassette which attaches to the pump. If the

> solution is to

> be kept longer than 8 hours, it must also be kept

> cool, usually by

> means of freezer cold packs placed around the

> cassette.

>

> Epoprostenol must be initiated in a setting with

> adequate personnel

> and equipment monitoring and emergency care. At most

> centers,

> patients are started on epoprostenol in the

> hospital. A dedicated

> nurse clinician is mandatory to help manage problems

> related to side

> effects of epoprostenol and to ensure appropriate

> dosing. In

> addition, the nurse clinician must teach patients

> how to mix

> epoprostenol, keep the central venous catheter

> clean, and manage the

> delivery pumps.

>

> Dosing of epoprostenol

> Epoprostenol is dosed according to a patient's body

> weight, in

> kilograms (kg). The starting dose is generally 1-2

> nanograms (ng) per

> kg per minute. The dose is increased according to

> the response and

> side effects of each patient. Usual long-term doses

> range from 20-40

> ng per kg per minute.

>

> What are the major problems with epoprostenol?

> A major risk is interruption of the infusion, which

> in some patients

> can cause severe worsening of their PAH and even

> death, in just a few

> minutes. Therefore, patients should always have a

> prepared backup

> medication cassette and an infusion pump nearby. In

> an urgent

> situation, epoprostenol can be given through a

> peripheral vein.

>

> A second major risk is that of infection related to

> the chronically

> indwelling intravenous catheter. The infections may

> involve the

> catheter site or the catheter itself, and this may

> lead to an

> infection in the blood stream. Any sign of infection

> warrants

> immediate medical attention. The infusion must be

> prepared daily,

> which can occupy up to an hour of time.

>

> What are the frequent side effects of epoprostenol?

> Epoprostenol infusion causes a variety of systemic

> side effects,

> partly related to its action as a vasodilator. These

> include

> headache, flushing, jaw pain, bone pain, diarrhea,

> palpitations, and

> rashes. The number and severity of side effects

> varies among

> patients.

>

> How is epoprostenol supplied?

> Epoprostenol is supplied as a powder in

> concentrations of 0.5 mg and

> 1.5 mg. The alkaline solution or diluent is provided

> in 50 ml vials.

> Epoprostenol is then dissolved in the diluent. Each

> container of

> dissolved epoprostenol can be used one time only.

> Once dissolved, the

> medication can be stored cold for up to 48 hours.

>

> How do patients obtain epoprostenol?

> Epoprostenol must be prescribed by a physician and

> insurance approval

> must be obtained prior to starting therapy. Once

> approved by

> insurance, epoprostenol is then sent directly to

> patients by either

> of the two specialty pharmacies: Accredo Health Inc.

> or Caremark.

>

> Will insurance pay for epoprostenol?

> The cost of epoprostenol is approximately $100,000

> per year but may

> be higher depending upon patient dose. Most

> insurance, as well as

> Medicaid and Medicare, will pay for epoprostenol.

>

> Could a patient be allergic to epoprostenol?

> There are no reports of this and it is unlikely that

> this would occur

> since prostacyclin is made by the body. Patients

> may,

=== message truncated ===

________________________________________________________________________________\

____

Never miss a thing. Make Yahoo your home page.

http://www.yahoo.com/r/hs

[Guest guest]

I sure hope Joyce got as good and clear information as we just did.

I'm sure she did, sure she insisted upon it. But, again, this is such

an information full group. From something as simple as me wanting to

get home from ER to read what was previously said about Levaquin

(choose info here over hospital) and getting input on my six days of

prednisone to something as advanced and complex as Flolan. It's also

comforting to know that when it comes down to it, we have access to

such advanced and expensive medications. Surely if they can provide

her with $100,000 medication they'll get her oxygen deliveries and

mattress straight. They fight for $95 mattresses with expenses like

this.

Its so nice now to have some feel for what Joyce is experiencing this

week and understand the 5-7 days.

> >

> > I called again looking for an update and announcing myself as

> > Joyce's friend Kerry looking for an update. To my surprise it was

> > Joyce herself answering. I would've said hold my calls. Though

> she

> > claims to still be a little doped up after the heart caths she

> > sounded completely lucid to me and she's doing well. She said

that

> > the cath showed a level of 64 (I don't know the measurements this

> is

> > in folks, just what she told me) and that the level it should be

is

> > 24. So, yes she has PH, big time. They also found 2 blockages

> (and

> > I think she said one was 70%), but they did not put in any new

> > stents at this time. Rather than do the Revatio or Tracleer, she

> > said they are going to give her a different med that is

> administered

> > thru a port (I had never heard the name of it, nor had she so I

> have

> > no clue how to spell it... she said it's called " Flo -

something " ,

> > how's that for a specific detailed report? No criticism allowed

> > folks). She is going to be in the hospital for 5-7 days so they

> can

> > see how she tolerates this new medicine. They are going to try to

> > get this PH under control before addressing other things like the

> > stents for blockages and any new meds like the Rituxan. She said

> > once again how much she really likes and is impressed by this new

> > doctor. I am so glad for that. I told her since she had such a

> big

> > day today and all her family around the last 2 days that I would

> > leave her be. I will go visit over the next couple days and be so

> > glad to do it because w/ her busy and crowded household lately

> > visiting has just not been as easy. I told her I had posted

> earlier

> > and would post again now w/ the latest.

> >

> > Good day from the Queen and her humble messenger girl,

> > Kerry

> > IPF '01

> > S. IN

> >

>

[Guest guest]

Tina,

I'm well speechless. At first in reading your post, I'm thinking oh

great I knew I wouldn't have to look this up somebody would

understand and translate what I reported back. Then I'm saying yep,

sounds exactly what Joyce has been dealing w/ the new and increased

shortness of breath, and w/ less activity and the pain! Of course

she suspected this and was soo right. Then, I'm reading the details

on this medicine and wow, this is some scary stuff. I can't imagine

all the trouble to administer this and the seriousness of it. This

scares me. Do Tracleer and Revatio have anywhere near these

concerns? I do sooo appreciate you explaning this and researching

it and posting it. I'm glad I know how much more drastically

serious this is, way more than I could have ever imagined. I just

don't know what else to say, I do so truly appreciate it, but

honestly there goes my sleep tonite.

Ugggh,

Kerry

IPF '01

S. IN

> >

> > I called again looking for an update and announcing myself as

> > Joyce's friend Kerry looking for an update. To my surprise it

was

> > Joyce herself answering. I would've said hold my calls. Though

> she

> > claims to still be a little doped up after the heart caths she

> > sounded completely lucid to me and she's doing well. She said

that

> > the cath showed a level of 64 (I don't know the measurements

this

> is

> > in folks, just what she told me) and that the level it should be

is

> > 24. So, yes she has PH, big time. They also found 2 blockages

> (and

> > I think she said one was 70%), but they did not put in any new

> > stents at this time. Rather than do the Revatio or Tracleer,

she

> > said they are going to give her a different med that is

> administered

> > thru a port (I had never heard the name of it, nor had she so I

> have

> > no clue how to spell it... she said it's called " Flo -

something " ,

> > how's that for a specific detailed report? No criticism allowed

> > folks). She is going to be in the hospital for 5-7 days so they

> can

> > see how she tolerates this new medicine. They are going to try

to

> > get this PH under control before addressing other things like

the

> > stents for blockages and any new meds like the Rituxan. She

said

> > once again how much she really likes and is impressed by this

new

> > doctor. I am so glad for that. I told her since she had such a

> big

> > day today and all her family around the last 2 days that I would

> > leave her be. I will go visit over the next couple days and be

so

> > glad to do it because w/ her busy and crowded household lately

> > visiting has just not been as easy. I told her I had posted

> earlier

> > and would post again now w/ the latest.

> >

> > Good day from the Queen and her humble messenger girl,

> > Kerry

> > IPF '01

> > S. IN

> >

>

[Guest guest]

Kerry

No Tracleer and Revatio don't have nearly these concerns and,

unfortunately, this probably tells us the level of severity of her

PH. It was painful and scary to read. But, then it also tells us that

she has doctor's up on the latest and trying to hit it as hard as

they can. If there were problems, I'm sure they would revert to one

of the others, but they're starting with the most powerful weapon

they have. I would expect they'll know quickly her tolerance for it

and then how to proceed. I'm sure they feel if she can tolerate it,

then it could be more effective. If not, I'm sure Tracleer or Revatio

will be prescribed.

Yes, I understand there going your sleep, but it also sounds like

she's getting the best of care with nothing spared. When I think of

leading edge and scary I think back to my mother and heart bypass

when it was experimental. It was scary (20% probability not to even

survive the surgery) but it saved her and gave her many more

enjoyable years. We look for miracles and maybe this is the miracle

drug for Joyce. I pray it is.

> > >

> > > I called again looking for an update and announcing myself as

> > > Joyce's friend Kerry looking for an update. To my surprise it

> was

> > > Joyce herself answering. I would've said hold my calls.

Though

> > she

> > > claims to still be a little doped up after the heart caths she

> > > sounded completely lucid to me and she's doing well. She said

> that

> > > the cath showed a level of 64 (I don't know the measurements

> this

> > is

> > > in folks, just what she told me) and that the level it should

be

> is

> > > 24. So, yes she has PH, big time. They also found 2 blockages

> > (and

> > > I think she said one was 70%), but they did not put in any new

> > > stents at this time. Rather than do the Revatio or Tracleer,

> she

> > > said they are going to give her a different med that is

> > administered

> > > thru a port (I had never heard the name of it, nor had she so I

> > have

> > > no clue how to spell it... she said it's called " Flo -

> something " ,

> > > how's that for a specific detailed report? No criticism

allowed

> > > folks). She is going to be in the hospital for 5-7 days so

they

> > can

> > > see how she tolerates this new medicine. They are going to try

> to

> > > get this PH under control before addressing other things like

> the

> > > stents for blockages and any new meds like the Rituxan. She

> said

> > > once again how much she really likes and is impressed by this

> new

> > > doctor. I am so glad for that. I told her since she had such

a

> > big

> > > day today and all her family around the last 2 days that I

would

> > > leave her be. I will go visit over the next couple days and be

> so

> > > glad to do it because w/ her busy and crowded household lately

> > > visiting has just not been as easy. I told her I had posted

> > earlier

> > > and would post again now w/ the latest.

> > >

> > > Good day from the Queen and her humble messenger girl,

> > > Kerry

> > > IPF '01

> > > S. IN

> > >

> >

>