Remodulin

Well, my doctor is back and I go into the ICU at Roseville Kaiser on Monday to start the Remodulin. I may be pretty sick the first few days until we get my dosage adjusted. The nurse with Acreedo, the company who supplies Remodulin came over last night. She went over how to prepare the drug for infusion and how to change and clean my line. She was excellent in her explanations. It takes some time and strict adherence to cleanliness and sterility. Kim and Wayne sat with me through the lesson so that they understand what is involved in case I need help. They are so supportive of Brian and I, going to support groups and learning what they can to help us. We could not be going through this without their help. It is scary knowing I am going to go into the hospital only to get sick, when most times you go in to get better. The end result will hopefully enable me to breathe better. Sometimes you have to take the bad with the good and this is one of those times. I am happy to be going on Remodulin instead of Flolan which is twice as much work and has to be kept on ice. You know me, I love the heat and carrying an ice pack 24/7 isn’t my idea of cool. So next time you see me, I will be the one with the oxygen tank on one side and the Remodulin pump on the other. I will have to accessorize accordingly!

Catheter probllems

When I left the hospital on Wed. I was given a number to call on Thursday. My dressing had seeped on Wed. night so at least I knew that had to be changed, but I was not given instructions how to do this, nor was I given supplies to do so. I called the number yesterday, (Thurs.) and the infusion department did not have a record of me or orders for me. I called my Dr.’s office, knowing he was out of town, but left a message for his assistant. I did this several times. After several more calls, I got the assistance of someone in the infusion department. She said she had left a message for my doctor earlier but she would see what she could do for me. I never received another call. Today, I decided I had better just go to the hospital to go to my Dr.’s office and see if I could get helped in person. If they couldn’t help, then I planned on going to the ER and staying there until something was done. I was not given blood thinner meds to stop any clotting in my tube and no instructions on its care. A catheter of this kind has risks of infection, blood clots and sepis.....When I got to my Dr.’s office, his assistant said he had left a message with the on call doctor. Apparently he had gotten my messages but didn’t let me know. It took about two hours, but he managed to get me some help in the infusion clinic. Course, he didn’t tell me that until after waiting for a while, I asked him what was going on. (He needs some lessons in communication!) My Doctor will not be back in the office until Wed. The infusion department flushed out my catheter with saline and heparin, an anticoagulant. Then they changed my dressing and scheduled me to come every day around 3pm to do the same again. They said they would cover me until Wed. when my doctor will be back. Their supervisor said they needed to do so to legally cover the hospital’s butt.... I was surprised they said that in front of me but I think they were shocked that I had been left high and dry after such a procedure. Apparently, my doctor had left orders for me to get the catheter put in. The department who does that had an opening and put me in at that time, hence the short notice. After it was placed, there were no further orders so no one knew what to do. The infusion department mostly works with cancer patients and does not deal with my kind of catheter. I think one of the problems is the nurses do not see this type of catheter too often. In fact, I was told that the 3 nurses who helped me today would be gone this weekend and I needed to explain my type of catheter to whomever is working this weekend. They said they were just covering me until my doctor gets back and I won’t be seeing them when he does. I think the other problem was the assistant did not follow up with me or with the on call doctor. I think he just assumed I was taken care of. And you know what happens when we assume....we make an ass of ourselves. Anyway, I think I will be in the ICU the end of next week getting my medication started. In the meantime, you can catch me at the hospital every day around 3 pm.

Catheter

I received a call yesterday afternoon to get my catheter put into my chest today. It was an experience that I don’t want to repeat as it is painfully uncomfortable. But the team were so nice and tried to make me as comfortable as possible. I have a tunnel catheter that comes out above my left breast. The tube goes inside up my neck and then down into my juggler vein. Tonite it is pretty sore. I have to call my Dr. tomorrow and I imagine I will go into the hospital soon to start the med, remodulin. The food and drug administration approved a inhalation form of the drug this past week but the drug won’t be available for a few months. My dr. didn’t think I could wait for it since when I saw him it was still going through the approval process. Tomorrow we are checking out a program through Sutter that may work for Brian, for in-home care, meds, drs. and everything he would need. I have great hopes that we can get involved in this program. They would be on top of any new meds that may work for him. He is doing ok. He can still laugh and joke with me. I am worried about him when I go into the hospital since he depends on me so much and is scared when I am not around. Four days could be a long time for him. But he can come and spend the day with me in my room and will have family around to care for him. I just like to keep him happy and not worried.

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?