Daily Ponderings

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...

It's been, once again, forever since I last wrote in this blog. I have always done that with any diary/journal/blog I've had since I was little and had my Cabbage Patch Diary (which is still in my drawer!). Sometimes, I am just not inspired to write. Sometimes, I feel like my writing is boring. I think I need to change that. I was thinking of finding some sort of article or challenge of writing almost every day on different topics. I'll have to do some research. But, tonight, I at least wanted to brush off the proverbial cobwebs of my site, to get some words out there in the internet. And, I wanted to also put it in "writing." I've been happy lately, and that speaks volumes to me.

Another summer-like day in October happened today, and that was alright with me! I am loving this weather, although I am very aware that it will end soon enough. I haven't yet switched over my wardrobe, and plastic hasn't been sealed up over the windows. I'm still hoping I can hold off on that for another couple weeks. We'll see! Today, I had a PH support group meeting. I've sort of lost count of how long I've run this support group, but I know it's well over 10 years. I enjoy having meetings, because I get to see my phriends! But, I REALLY enjoy the meetings where I have a speaker, which I've had for almost all the meetings this year. It's very hard to come up with new topics after having a support group for so long, especially when not too many new people show up, or come once and then never again. I have repeated a few topics, but there are still a few that I haven't had at all. So, I need to prepare for next year's meetings soon so I ca...

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 ...

Today wasn't the greatest day for me in dealing with anxiety. It started with a phone call just after 8am that I didn't answer because I wasn't even really awake at that time. The phone number showed up as "Restricted," so I ignored it, and then listened to the sound my phone makes when someone leaves a voicemail. About half an hour later, since I couldn't go back to sleep, I listened to the message from the nurse at my gynecologist's office, telling me to please call back as soon as I could. Wonderful. I was trying not to worry about it as I called back 10 minutes later, but I had to leave my name since the nurse wasn't available at that moment. I continued to get ready for pulmonary rehab, but my mood just wasn't the greatest, and I could tell the anxiety was creeping up. Exercising helped a little, but trying to keep myself occupied when I got back home was hard. I eventually showered, and called the office back after drying my hair. The "p...

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...

So what do you do with a child who has a disability? And what do you do with a child who may or may not live for very long? Well, you do what is most important: you love her and let her live life as best she can. I spent my childhood playing with my sisters (whom I affectionately call sistores), running around with them, but always taking breaks. My mom signed me up for dance lessons, but I only made it through one class before she realized I wasn't doing that great. So I took organ lessons. I joined the Girl Scouts, which was fairly easy for me to do. I did Art Wheels. I did what I could do to have some fun. I lived my life! Yes, there were many hard times. Yes, I very often got short of breath running around. Yes, I took a LOT of naps. And I still do! Yes, I got sick often.  But I learned my limits, pushed past them many times, but I did ok. And I think the biggest reason why I'm still here today is because of my parents, especially my mom, and my sistores. I love my family d...

Back in 1975 when I was diagnosed with PH, there wasn't anything to cure it, nor anything to treat it. My parents weren't given too much hope, and I can't imagine what that must have been like for them to know that I could die from this crappy disease at any point in time, whether it would be within my first year, or 50 years later. Somehow I managed to live for 28 years before starting one of several meds that had been FDA-approved by that time (in 2003). Today there are 9 drugs available to treat the symptoms of PAH, but the fact remains that there still isn't a cure. YET! The approved therapies for treating PAH symptoms are: *Conventional Therapies *Oral Treatments *Inhaled Treatment Options *IV Treatments *Subcutaneous Treatment *Lung Transplant

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 ...

My trip to Cleveland to go for my appts went well! I saw my cardiologist on Wednesday afternoon, after my echo was done. My cardio is always late for appts. Unless you're the first one of the day, you're fine! But my appt was at 4pm, and we didn't see him until after 5. Sometimes the waiting can be irritating, BUT, I absolutelly love him and he will stay with you to discuss things in detail for as long as you want. This is why his appts run so behind every day! Anyway, my echo results were very good! My heart is not enlarged at all, which is awesome! While the pressures in my heart are still estimated to be very high, my cardio said that for me, he's not really sure that matters at all. Being an Eisenmenger patient, my body has adjusted to living this way for so long, and the pressures aren't going to really get better no matter what meds I'm on. But I'm functioning pretty well, my exercise capacity has been good this year, and that's what counts the mos...