What is Pulmonary Hypertension?
Pulmonary Hypertension is a rare and incurable disease that affects
approximately 2-3 patients per million worldwide each year. It can affect
small children, as well as the elderly. Men are not exempt but it most often
strikes young women in the prime of their lives. It causes high blood pressure
in the lungs, which produces progressive breathlessness and ultimately threatens
life itself. The average time to diagnosis in patients with PH is two years.
The reason is due to that fact that an extensive workup is necessary to clarify
the cause of a patient's elevated pulmonary artery pressure, and the early
symptoms are non-specific. Common symptoms of PH are shortness of breath,
weakness, and fainting episodes.
Pulmonary hypertension is caused by certain forms of congenital
heart disease, lung disease and blood clots in the lung arteries. It is also
associated with collagen vascular disease, portal hypertension (usually caused
by liver disease), diet drugs, HIV infection and some other rare diseases.
In some cases no cause can be identified and these cases are called primary
pulmonary hypertension (PPH).
Life expectancy depends on many variables. Without knowing the
specifics of each case it is impossible to even guess. Patients with severe
forms of pulmonary hypertension have a shortened life expectancy, but recently
developed treatments can often help these patients.
Treatment
Today there is treatment to help almost every PH patient. Doctors
might begin by trying to lower lung pressures with calcium-channel blockers.
The next step is usually to try epoprostenol. Then a transplant is considered.
Always discuss your treatment with your health care provider. Be sure to ask
questions if you don't understand, and ask for information on options, risks,
possible side effects, drug interactions and dosages. Learn as much as you
can about PH and treatment options so you can make educated decisions.
When PH is caused by a blood clot (or clots) in a pulmonary artery,
a surgical procedure called a pulmonary thromboendartectomy may restore almost
normal blood flow to the lungs. Most clots form in a leg, break off, and travel
to a lung. If they do not dissolve (most do), and if they lodge in the lung,
they age and become almost like scar tissue. When the clot grows into the
wall of the vessel, it may take a decade or longer before the overloaded vasculature
that remains becomes damaged from the extra load it is carrying, and PH symptoms
may appear. Not all chronic clots cause PH.
Just as a lack of oxygen can cause PH, PH sometimes causes reduced
oxygen in the bloodstream. Low levels of bloodstream oxygen make PH worse,
so often PH patients need supplemental oxygen. Oxygen is available in several
forms. It can be delivered to your home in waist-high tanks, from which you
can refill smaller, portable tanks; you can rent an oxygen concentrator, which
is rather noisy and must be cleaned frequently; or you can use the more convenient
Oxylite portable system (where available) that has a smaller, lighter container
than a regular tank, and that saves oxygen by delivering it in pulses. Oxygen
can be inhaled through a facemask, or the more preferred cannula (nose prongs).
If you are using street drugs, especially cocaine and methamphetamine,
you've got to get appropriate treatment and quit. Consider joining a support
group.
Is Pulmonary Hypertension Hereditary?
Approximately 6-10% of cases of primary pulmonary hypertension
are hereditary. Therefore whenever a patient is diagnosed with PH all first
order relatives (siblings, children and parents) should be screened for the
disease. The best screening test is an echocardiogram. Many women with PH
can't get pregnant, but if they do the maternal mortality is in excess of
50%.
Daily Living & Wellness
Plan to do the tasks that require the most energy when your energy
level is highest, usually in the mornings. Do things you don't want to do
in the morning while you have more energy to get it over with and out of the
way. Take time to rest, plan sit-down jobs for those in-between energy and
no-energy times.
Avoid doing jobs that you know will be stressful or too tiring
for you. You have to know your own limitations, and then make the necessary
decision as to what is right and good for you. What we want to do, and what
we can do can be two very different things.
