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 ...

Exercise. It was something I was pretty much told to stay away from when I was growing up. Doctors  initially couldn't give my parents a direct answer about me taking gym class, so they finally decided that I would do better without it. So, my entire school career, I never took a gym class, and I never participated in sports. Even though I ran around with my sistores and neighborhood friends playing Hide N Seek and Capture The Flag, it would exhaust me, and I'd have to rest for an awfully long time. So, no exercise regimen or sports for me! Then, when my PH symptoms got worse, and I eventually was referred to Cleveland Clinic, I was told I'd have to do a 6 minute walk. I had no idea what that really entailed, but I tried to start walking on my sistore's treadmill to "practice," and holy moly, did I feel terrible!! But, I kept trying as much as I could to walk so that maybe I could "pass" this 6 minute walk thing. Unfortunately, when it got t...

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 ...

This afternoon I had to get some blood work done for two different doctors. One script was for my regular testing to make sure my liver still likes the PH medication I've been on for almost 14 years, and to make sure I'm not pregnant (don't really need to do it, I know I'm not lol), and the other script was to check hormones, thyroid and a few other things for a problem I've been having. I went after volunteering, and the place was nice and quiet, so I was out of there shortly after. Before I left the building, I decided to use the ladies' room. While I was washing my hands, I looked in the mirror and noticed all the gray hairs that were coming out of the top of my head. My normal reaction to seeing them is "Ugh, I really need to get my hair done!" But today, my instant reaction to seeing them was: "I should be thankful I have gray hairs, because it means that God's allowed me to age." It took me aback that THAT thought popped into my h...

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...

The combination of my PH and congenital heart disease is known as Eisenmenger's syndrome. A phriend's mother posted all about Eisenmenger's today on Facebook, because her daughter also has Eisenmenger's syndrome. Since Jane let me, I'm just copying and pasting her post here! Eisenmenger's is also considered a rare disease by NORD. I couldn't find out approximately how many people have the disorder though but it has to be under 200,000.  Eisenmenger syndrome progresses over time as a result of a complication of a heart defect and the effects of high bl ood pressure in the lungs. This hole in your heart causes blood to flow or circulate abnormally in your heart. Oxygen-carrying blood (red blood) from the left ventricle mixes with oxygen-poor blood (blue blood) from the right ventricle. This mixed blood then returns to your lungs instead of going to the rest of your body, causing high blood pressure in your lungs. High blood pressure in your lungs (pulmonar...

I was never allowed to take gym when I was going to school. I really can't remember what I used to do in grade school when my class walked down the street to the gym. Most likely it was just staying in the classroom doing something. In middle school, I would walk down to the 1st grade to help out the teacher. It was then that I realized I wanted to become a teacher myself! Many times a person might change their mind about "what they wanted to do when they grew up." I never did. I went to high school, and by the time I graduated, I still knew that teaching was what I was supposed to be doing. I got my undergrad degree in elementary education, and after that I started subbing. At that time, I didn't have to go to grad school for my Master's right away, unlike now. I'm actually happy about that, because I didn't know what I wanted to study. Subbing lasted for a month before I got a job as a teacher at a day care. That job lasted two months before I became a p...

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...

There are a few types of PAH (pulmonary arterial hypertension): Idiopathic PAH: The cause of PAH cannot be found. Heritable PAH: Formerly known as familial or genetic PAH, this type can be inherited, and is relatively uncommon. Associated PAH: PAH is associated with another disease or condition, such as connective tissue disease, congenital heart disease, chronic liver disease, HIV, drugs and toxins, and more. CTEPH (chronic thromboembolic PH): PAH is a result of blood clots in the lungs. CTEPH is the only type of PAH that can POSSIBLY be cured by a surgery called pulmonary thromboendarterectomy (PTE), which removes the blood clots from the lungs. My PAH is a result of congenital heart disease, so I am in the Associated PAH category. I was diagnosed with atrioventricular canal, which is a fancy way of saying I have 2 holes in my heart. Yippee!! The holes have never been repaired, so I've been living with both for 37 years (so far!).

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 ...

I can't tell you the number of people who have come into PH chat, or posted on the PH message boards, who say they have been diagnosed with PH after have an echo done. While an echo is used often to try to figure out what's going on, an echo really never definitively diagnoses PH. The echo is an estimate. The numbers on an echo can be waaaaaay off compared to the actual numbers measured during the only test that truly DOES diagnosis PH: the right heart catherization! The right heart catherization (RHC) is an invasive procedure. There is a tube inserted into the heart to measure pressures in the heart and lungs. This tube can be inserted through the groin, neck, or even the wrist nowadays (although I'm not sure how many hospitals are using this method, it's pretty new!). I hear most people can ask for some "happy juice" during the test, since usually the patient is not put to sleep, and it can be a little unnerving to go through. If the pressures in the p...

As part of having pulmonary hypertension, I wear oxygen. I've been wearing it for the past 10 years at least. When I am out and about, I notice people looking at me, but I'm at the point where I don't care anymore. It's a part of who I am, and it gives me the ability to actually BE out and about!  I have gotten my share of random strangers coming up to me asking why I'm on oxygen. Many of them are older, some are also wearing oxygen, others are children. When I am asked why I'm on oxygen, I tell them I have pulmonary hypertension. I'd say about 99% of the people say, "Oh, I have high blood pressure, too!"  Well, that may be true, BUT, pulmonary hypertension has nothing to do with the high blood pressure everyone is familiar with! It is the OTHER hypertension. PH is high blood pressure in the lungs. If it is left untreated, it will eventually cause right-sided heart failure.  Oh, and by the way, my regular blood pressure is just fine, tha...

Shortness of breath. Extreme fatigue. Dizziness. Chest pain. Swelling of the arms, legs, ankles, or abdomen (edema). These are symptoms of so many conditions. Doctors usually consider asthma, COPD, and being overweight right off the bat when a patient complains of these symptoms. BUT!! Sometimes it's PH!! Doctors really need to start including PH as part of the list of conditions to consider when a patient is having these symptoms! To find out more about this new awareness campaign that the PHA started this year, especially geared toward medical professionals, please go HERE !!

PH Awareness Month has begun! And while I have so much information to share about PH and what it is: the symptoms, the treatments, dealing with it on a daily basis, etc, I'm taking the time today to remember my phriends (friends with PH) who have sadly passed away from this crappy disease. I became a part of the PH community when I started my first-ever treatment back in 2003. I didn't know there was a website available, and only discovered it after opening my first shipment of Tracleer. Every shipment includes a lot of paperwork, and a flyer for the PH Association was included. I logged on and began at least a week-long cry fest! The tears were of shock that there were others out there like me. They were tears of joy that there were others out there like me!! I read the message boards for days, every single message, and I started to post when I felt comfortable enough to share my story. It was an amazing experience, to finally be able to relate to others. Not too ...

Sooo, after the PHA conference last month, my phriend and her daughters and grand-daughter and I ventured on to Disney World! We were picked up at the Renaissance around 11am that Sunday, and off we went. It was a rather warm day, but wet and gloomy. I sat behind the driver, and soon we approached the Disney site. I took a picture of the entrance sign, which scared the poor driver because my flash went off and he thought it was lightning. Oops!! Soon we were at our hotel, All Disney Sports. There was a bit of an issue with checking in, and I also couldn't find out where my oxygen concentrator was located. After what seemed like forever, we were told where our rooms were, but only one of them was ready. So we headed to that one, and I was able to rest for awhile. Eventually the other room was ready, and I went down to it with my luggage. Then I had to go find the concentrator. I walked back to the main building, past the pool area. Which was flooded. I had worn my jeans and sn...

I have been a part of the PH community since October 2003, when I started my first medicine to treat PH symptoms, called Tracleer. Included with the first shipment of that med was a flyer for the PHA, so I sat down one morning and typed in the url in the search bar. I spent that day, and most of the week, in total awe. It was an emotional time, reading so many stories of other people who had PH and felt exactly how I felt my entire life. The shortness of breath, the extreme fatigue, the not being able to do anything strenuous without feeling terrible afterward. I cried much of the time. It was an entire new world to me, a world which I actually felt I belonged. I was no longer alone. While the message boards provided a huge wealth of information and personal stories, the chat rooms provided an instant connection. The first few times I ventured into the rooms, I felt totally at home. For anyone who hasn't had the experience of being in a chat room, I highly recommend it, although ...