Daily Ponderings

The moment I was born, I was a sick baby, even though doctors couldn't figure that out until I was 9 months old. I don't know what it's like to be totally healthy. I spent the first 28 years of my life knowing I had 2 holes in my heart, and pulmonary hypertension (PH), although no one really explained that second half to my parents or myself. Then when my daily symptoms of shortness of breath, and extreme fatigue turned into an elephant sitting on my chest constantly, I was eventually introduced to the PH world. I started seeing doctors at the Cleveland Clinic in Ohio who specialized in the disease, plus a cardiologist for adults living with a congenital heart defect. I was eventually started on a PH medication, and that first shipment of medication almost 16 years ago led me to the online world of a PH community. From message boards to chat rooms, I suddenly realized that I WAS NOT ALONE!! The PH world became MY world for so long. I would read messages for hours, replying ...

This past weekend flew by so quickly! It's hard to believe it's already Monday again. So, I'll sum up the weekend (which means, I will elaborate on everything lol). Friday was spent with my Dad at the Cleveland Clinic for my annual check up. We left a little after 6am since my first appointment was at 10 and it takes about 3 hours to get there. But, I'd been up since 4:45 for whatever reason, so I was rather tired! When we got to the Clinic, I had my first appointment, which was an echo. The technician who did mine was so thrilled because he never gets congenital heart defects, and he said I was the most exciting case he was going to have all day (granted, it was 10am, I sure hope I wasn't the only interesting case lol). After that was over, I got some blood work done, and then my Dad and I had time to eat some late breakfast/early brunch before my next appointment. The Clinic has different cafes in many of their buildings for people to get something, and the one ...

Last year, before the PHA International Conference, I found out that the PHA was looking for people to help them with videos they wanted to make about people living with PH secondary to another illness or condition. This included people living with Scleroderma, Lupus, HIV/AIDS, and Congenital heart disease. Oh! Congenital heart disease! Well, that was me! So as much as I was very afraid to be in front of a camera, because I'd never been filmed before, I told them I could volunteer if they needed me. And guess what? They needed me! So on the Saturday of the conference, I went to an assigned room to film. I was interviewed, answered lots of questions, and felt like I was there for an hour. lol I was wondering when the videos would be made, and found out a couple weeks ago that they were all done! Here I am, along with another congenital heart patient, and MY cardiologist from the Cleveland Clinic! I really respect him and all the research he likes to do about people living with m...

I talked about Remodulin yesterday as being an IV medication to treat PAH symptoms. Remodulin can also be administered subcutaneously, meaning, under the skin. Subq Remodulin works in the same way as IV and inhaled Remodulin, by relaxing the blood vessels to allow more blood to flow and carry oxygen around, which then allows the heart to pump easier. Subq Remodulin requires site changes every so often, and are usually put into the abdominal region. Subq Remodulin can be quite painful for most users.

Many PHers are on one or more of the following conventional drugs to treat symptoms: Calcium Channel Blockers (CCBs) - These are oral meds which relax the muscles around blood vessels which allows better blood flow. CCBs can also help to regulate heart rates. CCBs are only effective in less than 10% of PH patients. Digoxin - Digoxin can help the heart pump. It can also help regulate the electrical activity in the heart. It is an oral med taken once a day. I've been on this medication for over 35 years!! Warfarin (Coumadin) - Warfarin is a blood thinner taken once a day. It must be carefully regulated, so bloodwork is required often. Warfarin can be affected by foods and other medications, so a person taking it must make sure they monitor what they eat and the other things they ingest closely. I was on Coumadin for at least 10 years, and finally stopped it last year to try a baby aspirin instead. I can tell you that I don't miss Coumadin at all!!! lol Diuretics - These m...

The right heart catherization mentioned in my previous post is the Gold Standard to diagnosis of pulmonary hypertension. But while it is very important, there are other tests given to help determine what may be causing the PH, if anything. These tests include: Blood tests - An arterial blood gas (abg) is totally not a fun test at all. It's a blood draw, but the blood is taken from the artery, not the vein. It hurts!! But what it tests is the amount of oxygen in the blood. If there isn't enough, the patient may need to be put on supplemental oxygen. Other blood tests check for liver and kidney function, thyroid problems, collagen vascular disease, and any signs of infections or HIV. Chest Xrays - The chest xray can determine if the right ventricle or pulmonary arteries are enlarged. It also can show if someone has scarring or emphysema. Electrocardiogram - This test checks the electrical impulses of the heart. Pulmonary Function Tests - PFTs measure how much air your lun...

A pulmonologist or cardiologist are doctors who may see only a handful of PH patients in their practice in their career. Some have no idea how to treat PH. Some might think it's not that big of a deal and not offer treatment at all, just monitoring. When a newly diagnosed PH patient comes onto the message boards or into the chat room, the #1 recommendation given to them is FIND A PH SPECIALIST!! What's the difference? A PH specialist IS a pulmonologist or a cardiologist who either sees PH patients exclusively, or the majority of their patients are PHers. It is very important to see a PH specialist since they understand better what to do to help a PHer manage their symptoms. My own personal experience involves my pediatric cardiologist. Since my PH is due to congenital heart disease, I followed a pediatric cardio for most of my life, up to my early 20s. Dr. G. was wonderful, and I really loved him! But when my symptoms started worsening in my early 20s, he gave me inhalers to ...