I have heart!

I saw my heart doctor last week as I have been having more trouble breathing and am retaining water. Retaining water means my heart isn’t pumping as well as we’d like. He had me get a chest xray and to go back on lasix ( a diuretic), for the water retention. Dr. Devendra said I was a class three/class four in symptom severity of pulmonary arterial hypertension. The classifications are:

Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope.
Class II: Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.
Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less-than-ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.
Class IV: Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity.

I agree. Just talking on the phone puts me out of breath, let alone walking to the phone. There aren’t many statistics regarding PAH and scleroderma. Unfortunately, what little that is known, shows that patients who have PAH with scleroderma have a much higher rate of death than those with PAH without connective tissue disease which has a mortality rate of about 2.5 years after diagnosis without treatment. I have a CAT scan scheduled for next month. If nothing new shows up on the xray, then my dose of Letaris will be changed from 5mg to 10mg next month. I have to see him again the end of March.
When on Letaris, the defining test to see if the drug is working is a six minute walk test. Because avascular necrosis has limited my walking ability, I can not do the walk test. Now, I am actually happy not to do the test since it seems like a 24 hour test rather than only 6 minutes. I will continue to have echocardiagrams to see if the hypertension goes up. It is not as definitive as a heart cath but is less evasive.
Heart failure often causes edema. A weak heart can't pump hard enough to keep up with this cycle. It receives more blood from your lungs than it can pump out into your body. The blood it can't handle backs up and seeps out of your blood vessels into other tissue, including your lungs. Cells get waterlogged. This causes swelling called edema. Edema usually - but not always - shows up first in your legs and ankles.
Why does it usually show up in your legs and ankles? Because good old gravity is pulling all that "loose" fluid straight down. Presto: your feet and ankles look like the Goodyear blimp.

PAH is a complication of systemic sclerosis and adversely affects survival.
The main disturbance resides in constriction of pulmonary arteries and thickening of the pulmonary arterial wall. As a consequence, the right ventricle that pumps blood into the lungs works against an abnormally high resistance. Initially, the right ventricle is able to compensate for the increased workload, but in time, it decompensates, and patients develop right heart failure.
Typically, patients with PAH have no complaints at rest. The main symptom is shortness of breath with activity. Shortness of breath develops initially with exercise, such as climbing up a few flights of stairs.
Later, patients feel limited during activities of daily living, such as bathing or dressing, and ultimately, they feel short of breath even getting out of bed. In advanced cases, chest pain, lightheadedness, or loss of consciousness may occur with exertion and leg swelling may become prominent especially at the end of the day. Lasix makes me pee A LOT and then drink a lot because of dry mouth (sjorgrens) and pee A LOT more.
Happy Note: With part of our rent paid, our landlord is not kicking us out! Even though it is raining, things aren’t as bleak as they may seem.