Pulmonary Hypertension, explained

[Guest guest]

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,

Tins

IPF 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

>