|
 Continuous
intravenous epoprostenol sodium (Flolan ® ) is a long-term,
complex, and expensive therapy. Its pivotal role in the
management of pulmonary arterial hypertension (PAH) is
based on randomized studies that clearly established clinical
efficacy. Subsequent studies have confirmed its benefits
with regard to symptomatic and functional improvement,
sustained hemodynamic effect, and enhanced survival. Initial
studies demonstrated both acute (Figure 1A) and short-term
(Figure 1B) hemodynamic improvement.1,2 Exercise capacity
in epoprostenol-treated patients, as measured by 6-minute
walk test distance, improved during 12 weeks of follow-up
compared with conventionally treated patients (Figure
2).2 Improved exercise capacity, as assessed by improvement
in peak oxygen consumption, has also been documented (Figure
3).3 Importantly, increased survival has recently been
report-ed in two large case series of patients with PAH
(Figure 4).4,5 Although the US Food and Drug Administration
(FDA) has recently approved alternative subcutaneous and
oral drugs, intravenous epoprostenol remains the most
effective agent in the therapeutic armamentarium for PAH
patients with World Health Organization (WHO) Class III
or IV symptoms.
Despite its proved efficacy and cumulative experience
since the commercial availability of epoprostenol in 1996,
intravenous epoprostenol remains a complicated and potentially
dangerous therapy. With the approval of additional therapies
for advanced PAH, the selection of appropriate candidates
for epoprostenol treatment has become particularly challenging.
Health care providers must assess the potential risks
and benefits of epoprostenol therapy compared with alternative
treatment for each patient. This assessment should consider
the patient’s medical diagnosis, comorbidities, psychosocial
status, support structure, financial resources, and stability.
Providers should also consider the available resources
in their own facility to provide the comprehensive and
intensive management that these patients require. Importantly,
although epoprostenol therapy can be life-saving when
used appropri-ately, it can potentially complicate, and
in some cases worsen, symptoms with catastrophic results
if it is incorrectly initiated, administered, or managed
over the long term. Careful atten-tion to four aspects
of treatment is required when consider-ing long-term use
of epoprostenol: (1) patient eligibility, (2) patient
education, (3) drug initiation, and (4) treatment maintenance
and follow-up.
PATIENT ELIGIBILITY
The decision about whether a patient should be treated
with epoprostenol requires consideration of a number of
issues:
- Does the patient have appropriate clinical indications?
- Are there clinical contraindications?
- Are alternative medications more suitable?
- Have issues of medical coverage been defined?
- Is the patient able and willing to learn and comply
with
the regimen?
- Can adequate follow-up be assured?
Clinical indications
Epoprostenol is currently FDA-approved for patients with
symptomatic (WHO Class III or IV) primary pulmonary hyper-tension
(PPH) or PAH associated with collagen-vascular dis-ease
(the scleroderma-spectrum of diseases). At present, there
are no controlled data demonstrating its efficacy in patients
with HIV infection, congenital heart disease, or portopulmonary
hypertension. Because these indications are similar to
those for oral bosentan (Tracleer ® ) and subcutaneous
tre-prostinil (Remodulin ® ), additional considerations
should be weighed in selecting epoprostenol over these
other agents.
Patients with very advanced or rapidly progressive symp-toms
should be considered for early treatment with epoprostenol
since it has proved to be the most effective medical therapy
and improved mortality has been demonstrated with its
use.2 Epoprostenol can be added to the medical regimen
of patients whose condition has failed to adequately respond
or who have not tolerated other medications. This drug
should not be used in those with pulmonary venous hypertension
as no benefit has been demonstrated and there is potential
for worsening.6,7 Central venous access is essential for
placement of a permanent catheter. The presence of supe-rior
vena cava or bilateral subclavian vein obstruction (usually
in the setting of previous central catheters or pacemaker
leads) may be a relative contraindication.
Medical coverage
Epoprostenol is far more expensive than most drugs, its
use sometimes exceeding $100,000 per year. If prescribed
for appropriate indications (WHO Class III and IV PPH
and PAH associated with the scleroderma-spectrum of diseases),
medical coverage is usually available. Prior insurance
authorization is necessary and can be facilitated by the
distributors of the medication. Awareness of reimbursement
issues by caregivers is mandatory, and coordination between
the patient and the distributor is a vital role of an
active pulmonary hypertension clinic.
Patient capability and compliance
Although purely clinical issues regarding treatment selection
are pivotal, other factors may take precedence in matching
the patient to appropriate epoprostenol treatment. Despite
the desire to provide optimal clinically indicated therapy
to all patients, not all are safe candidates. In addition,
health care provider time is a valuable resource; care
of one marginally compliant or competent patient may adversely
affect the care of others. Thus, careful consideration
of factors related to a patient’s willingness and ability
to undergo therapy as well as the level of family and
social support should be addressed before initiation.
These factors are best explored by an expe-rienced and
sensitive nursing staff with specialization in the management
of PAH patients.
Nursing interviews should be conducted with both the
patient and a significant other who will assist and support
the patient at the outset. A number of issues should be
explored with careful questioning. Responses to these
questions do not necessarily preclude therapy, but are
extremely useful in planning for future patient and staff
needs.
Questions to ask after therapy
has been explained include:
- Are there physical limitations, such as digital loss
because of collagen vascular disease or visual problems
or hearing impairment, that may hinder the ability to
manipulate syringes, operate the pump, or hear warning
alarms?
- Are there problems with the home environment that
may preclude safe drug administration and follow-up,
such as absence of satisfactory plumbing, poor home
sanitation, or lack of access to a telephone?
- Is there a reliable family or social support person
to help prepare the medication and manage the infusion
pump?
- Are the patient and support persons committed to taking
the time each day (approximately 1 to 1.5 hours) to
per-form necessary procedures?
Questions practitioners should
ask themselves
about the patient include:
- Is the patient sufficiently at ease to be a receptive
learner
about a complex treatment strategy, or does stress and
agitation warrant deferring?
- Has the patient demonstrated compliance and initiative
by keeping scheduled clinic visits and following current
treatment recommendations?
- Does the patient actively participate in his or her
own
care or allow a significant other to manage it?
- Does the patient have a history of substance abuse
or
mental illness, including depression, that has required
medication or hospitalization, which would be risky
in the
setting of long-term complex intravenous medication
infusion?
|
