Friday I saw my pulmonary Doctor in the morning and my Rheumy in the afternoon. I am going to go on the IV medication, Remodulin. It is one of the medications delivered by a continuous infusion, meaning the drug is delivered steadily to the patient through a small pump outside of the body into a permanent catheter intravenously in my chest. I am still going to be taking the Letaris and the Rovatio (a pill containing the active ingredient in Viagra), in addition to this new drug. Romodulin costs about $100,000 per year, in addition to my other drugs including the Letaris which costs $60,000. At some point you have to say how much is a person worth and these orphan drugs are way too expensive! I would like to think I am worth $200,000 a year, but the government doesn’t think so since they don’t pay me that much in social security....The bionic man was worth 6 million and that was in 1974. I guess I am small potatoes compared to him...but then I am not saving the world....
In order to improve the flow of Remodulin, I will also begin taking Coumadin, a blood thinner. REMODULIN is a type of medication called a prostacyclin analog. It acts similarly to the prostacyclin that is found naturally in the human body, which helps keep the blood vessels open and working properly. Prostacyclin medications work by opening the blood vessels and may reduce the buildup of cells around the blood vessel walls. Blood returning to the lungs is pumped through the lungs by the right ventricle of the heart. Blood pressure in the lungs is ordinarily rather low, for example 20/10, in contrast to body blood pressure which is usually around 110/70.Resistance to blood flow through the lungs puts a strain on the right ventricle. At early stages, the right ventricle is able to compensate but as the resistance increases and the pressures go higher, the right ventricle cannot keep up. At early stages, shortness of breath occurs with moderate physical activity but as it worsens, it takes less and less physical activity to cause shortness of breath.
The pressure in the lungs can be elevated for several different reasons in scleroderma. The left ventricle can become stiff or weakened, leading to back pressure into the lungs. This is called heart failure. The distinctive problem in scleroderma is narrowing of the small lung arteries from progressive scarring of the inner lining of the small artery which is what is happening to me.
I won’t start on the drug until probably September. I have to have the catheter put in and there is a lot of paperwork for that and to get the Remodulin...when I am finally put on the drug, I have to be in the hospital for 4 or 5 days to make sure I can take it ok and I am trained in the care and use of it. This is the one drug that is stable at room temp so I won't have to carry an ice pack with me in addition to my oxygen and the drug pack.
So, I know what my plans are for the rest of the summer...What are yours?