Summer Summer Moon
Boy. Medicine. Trying to come to grips with Jon’s possible pulmonary hypertension. This is not a diagnosis you want. Even with advances, and they have been considerable, the fate of those with the disease have, to use Jon’s phrase, shortened horizons.
Just finished reading a 2006 article replete with medical shorthand, acronyms and formulas. I finally got it. This is a disease of the circulatory system of the lungs. Due to a variety of initial causes (and they are important in prognosis, but not diagnosis) the blood vessels in the lung become compromised, requiring increased pressure to push blood through them for its necessary oxygenation. The right ventricle of the heart pushes blood into the lung after it has been deoxygenated in its journey through the body. To produce the pressure required to pump the blood through the compromised lung circulatory system the right ventricle has to work harder (pump harder).
Due to the lung’s normally highly efficient circulatory system, the right ventricle has evolved a thinner wall than the left ventricle which pushes oxygenated blood through the body which requires greater pressure. As a result, when a diseased lung forces the thinner walled right ventricle to push harder, it eventually widens under the pressure, which makes its pumping less efficient, which makes it work even harder, which increases the dilation until the left ventricle becomes involved as the widened right impinges on it. This process defines the phrase vicious cycle. Then, at some point, the heart itself cannot produce enough pressure to effectively circulate the blood and heart failure ensues.
Treatment regimens are complex, only a few aimed at the actual problem, the circulatory system of the lungs, and all of those drastic. The advanced therapies (I don’t understand this use of the term.) are all symptomatic, that is, they reduce the load on the right ventricle by dilating blood vessels and improving circulation within the lungs, for example, but they don’t go to the problem itself and therefore ultimately prove inadequate.
So much about survival depends on etiology and we don’t know that in Jon’s case. Yet. Nor do we know to a medical certainty that he has the disease. An echocardiogram on July 1st will provide more information though catheterization of the right ventricle to determine it’s health is the final diagnostic step.
We’ll proceed as a family, figuring out what we can do for each other.