I Miss My Memory

The old me had an excellent memory. Excellent I tell you. Where is it? I want it back. Sadly, many people with PH have memory problems. It's because the condition stops oxygen from being in your blood (the heart pumps it to the lungs where they convert the air to oxygen and distribute it throughout your body.) You get so frustrated because you know what is happening but are powerless to stop it.

I have been on the Adcirca for 15 days now. It's too soon to know if it's working. I had a few really bad days recently and going to complete my cardiac rehab was really tough. I had to force myself. I finish tomorrow and I will be so glad to be through with it. It's a good thing to do to recondition your heart after surgery but when you are struggling with the PH it's rough. For some reason I have nights I cannot fall asleep. Other nights I sleep ten hours and wake feeling tired. I sleep with a cpap machine and oxygen. Right now I have dark circles under my eyes. I need to go to bed. More later.

Denial

I belong to a new group and I don't want to be a member. Not that there is anything wrong with the lovely people I have met in person, on the phone and in an online chat room. I just don't want to have this fatal condition. Sometimes it's overwhelming. I don't want to feel like that. I have to work on my knee jerk reaction to things. Yesterday my mail order company (which my insurance forces me to use) messed up. They mailed me blood thinner in the generic form where I can only take the name brand Coumadin. My doctor had called them about this before. They needed a new script and his office faxed one over without checking the written as dispensed box (they didn't see the need as the doctor had called and asked my record to be notated.) I yelled then called them and the woman kept telling me that I hadn't followed their guidelines. I kept saying they had the doctor's comments on file and she agreed but said I needed the rx rewritten and the box checked. I had already paid for this script and to her credit she got a supervisor to refund it since the pills had to go in the garbage.
I weighed myself and gained five pounds in fluid over the past two days. This is not good. Today I am not touching anything with a drop of salt in it. I am really concerned because this is the way you get into a fluid crisis. I broke down and had two slices of pizza on Friday. Big mistake. I can't have that much salt. I have made a pot of low sodium chicken soup for dinner. Just hoping that the diruretics get this off of me and fast.

Side Effects

I have learned that Adcirca has side effects. Didn't want to read them before I started it but I swear I have experienced almost all of the reported ones. Nausea hit me first but I fell asleep with my head elevated.Woke up with a headache (another one) and felt somewhat ill during the night. Second night I experienced bad leg pains and some back pain (symptoms 3 and 4.) I was afraid to take anything for pain as you are supposed to run anything you want to take by the pharmacist first. I had to endure leg pain until 8 a.m. when I got through to one. He said to try the Tylenol and if it didn't work I could take my prescription pain reliever. Whew. Hoping for some relief and I got it. By the way, have I mentioned that this isn't the kind of drug you pick up at CVS? There are a few companies that distribute it and you have to order it a week in advance. It comes via UPS. They say it will take a few months before I know if it's working so why do I have the side effects immediately? I am hoping that means it's helping right away to open up the blood vessels to the lung. I had to cancel rehab this morning as my legs could barely move. Hopefully next week will find me with more energy and less pain/symptoms.

Starting Adcirca

My insurance company sent the approval, the drug company is subsidizing my copay and I will only be paying $30.00 per month. The adcirca arrived today. Oh happy day. I was so happy to get it and took it after reading the side effects. You can get headaches, nausea, lightheadedness and fainting. I have to get up slowly at least at first until I see how this will effect me.

You can't drink with the medications for this disease which I am sure is a big adjustment for some people.

Today at rehab I ran into a problem. I was having a bad day and just felt a bit of tightness in my chest as I was on the treadmill. My heartrate was too fast and they made me slow way down and then rest and stay awhile until the rate dropped down. It did and I came home and took it easy. I am currently reading the patient handbook for PH. It explains, in layman's terms, everything you need to know about the disease. Since it's not a common thing it's good to know things you might not know, even if you are communicating with a few other patients. Almost the day I was diagnosed I found a great website for the Pulmonary Hypertension Association with links for lots of things. I joined and ordered the guide from there. A great resource for any new patient, or even one who just wants to know more. I have learned about receptor cells and many other things.

How I Got Here

In July 2009 I suddenly developed pneumonia. I have never been the same. I was on oral antibiotics and returned to my full job a few weeks later. In September I suddenly began making horrible noises when I breathed and found myself twice in the ER of the local hospital. Both times they felt I needed IV steroids and I was hospitalized, once for two weeks. During the second admission I kept insisting something else was wrong. The lung doctor I was using then tested me for auto immune diseases and found "nothing wrong". I asked to have my cardiologist, Dr. D. called in as well. He agreed when another doctor said they thought I might have a valve infection. I had a test called a TEE and they determined that my mitral valve was infected. (My aortic valve was mechanical and they felt it odd that it was the mitral and not aortic that became infected.) I had a PICC line put in and was sent home under the care of an infectious disease doctor who had me on IV antibiotics for six weeks. At the end of that time I returned to work again. I really thought things were okay until my legs and feet were swelling. I had to purchase EE width shoes and even queen sized knee highs cut off my circulation. It was awful. At times my feet were numb. I went back to Dr. D and he said I had some fluid and gave me a low dose of lasix to take. It didn't really help but I went to work each day. Before I knew it I had pneumonia again (thanks to a sick coworker) and was hospitalized in critical condition. A few days earlier my local hospital ER told me to go home as my pneumonia was "UNIMPRESSIVE". Less than 48 hours later I was told by three doctors at a major university hospital it was one of the worst pneumonias they had ever seen. I was coughing up blood for a few days. After that pneumonia I was so weak. Within a few weeks of being discharged my shortness of breath was horrible. I went to my local internist who told me she felt I had heart failure and needed the cardiologist to take care of this. I went to him again and again he did an echo but told me things were okay. (NOTE: if you don't feel the doctor is correct, get another opinion!) I went back to my lung doctor and she could tell I was overloaded with fluid (pleural effusions, which is fluid in the sack that hold the lungs.) I went to the hospital and had a litre of fluid removed from the lung. I went home and felt so much better. By this time I could not lay down flat and could not breathe bending over or other positions. The lung doctor ordered me oxygen. I ended up back in the hospital with fluid overload. By that time I needed oxygen 24/7. EVEN with the oxygen, at times I gasped for breath. I couldn't even shower without the steam throwing me into gasping fits. It was hell. Late spring I was hospitalized again (this was about my fifth time) and they removed over three litres of fluid from the pleura again. I had to have a tube put in my chest which can collapse your lungs. Not a pleasant experience but one that probably saved my life. During that time I knew I had to find another cardiologist. Dr. A had an echo done and they saw the pulmonary artery pressure was extremely high. For those of you who understand, it was about 90. That is severe PH. He also saw that my mitral valve needed replacing immediately. The combination of the two was why I couldn't breathe. They had to send me to Philadelphia as I needed a heart catherization and the mechanical aortic valve was in the way. I needed a small hole made in my septum by a doctor who did this frequently. I went to Hospital of the University of Pennsylvania where I met the wonderful and caring Dr. Hirshfeld. He gave it to me straight and told me I needed the surgery and he was willing to arrange it there if I would agree to it. I had the surgery July 27th where I received a mechanical mitral valve and hoped to have the PH lowered significantly, if not returned to normal. Sadly, this did not happen. It was lowered to about 70 but since it should be 30, it is still considered in the severe category. Dr. Hirshfeld got me in with a top PH specialist at that hospital (after confirming with a follow up right heart cath that my pressure remains elevated to this level.) The new doctor wants me to receive a medicine, adcirca, to see how much this will lower the number. I am still on a CPAP machine at night, which has oxygen bled into it. It's been over a week and I am waiting for my insurance company to approve the medicine. I have no idea what my copay will be but the costs of these drugs are astronomical. More about that in another entry.