Showing posts from November, 2010
A lung transplant is usually a last resort for a pulmonary hypertension patient if all other meds are not working or have stopped working well. It is up to the PHer if they want to go through a transplant or not. Having a transplant may mean a cure from PH, but it is not without problems. However, there are many PHers who have survived for many more years with their new lungs!

I have had many phriends who have either had a lung transplant many years ago, or who've had one recently, or who are still waiting. There are some who are doing well, past 10 years with their new lungs! There are a few who didn't make it past a few years with their new lungs. Many phriends who have faced the transplant option have decided to go for it, but I do know a few who do not want to have surgery. It's a personal choice, because it's really trading one disease for a whole other set of problems. But for those who get through transplant and recovery well, their lives change so much, and they…
All day events with no rest usually make a PHer pretty tired. I suppose anyone could be rather tired from something like that, but for someone with PH, it usually means the next day is already planned as a rest day. Today was one such day for me, although it was a great day! The holiday event went well, and most of the people who bought things from me were people I already knew. But that's ok! It was fun, though, talking to people she's never met and selling something to them. Makes me happy! At the end of the event, since we had one last minute person added to the group, I was given $15 to donate to the PH Association instead of everyone getting a dollar back for the purchase of a table. I thought that was so nice! Now I'm home for the evening, and taking it easy!! This week I'll be able to work on orders that I've gotten in the past week or so. I'm so thankful to be keeping busy!


When someone with PH eats waaay too much, they get increased shortness of breath. This is because the stomach is so full that it's pushing everything up and the lungs get more constricted, making it a bit harder to breathe!

This is what happened to me on Thanksgiving! lol Our family went non-traditional this year. None of us really felt like cooking the huge meal, so we ordered Chinese food instead. We had to buy it the night before, so it did end up being a bit of work trying to fit everything into glassware that could be shoved in the oven to reheat, or into something microwaveable. And of course, we had sooo much food! But it was a great meal, and everyone enjoyed it and the company! Mandy came over with her little family to have dessert (we still had pies!), and it was a very nice evening spent with my family. I don't think it matters what we eat on Thanksgiving, as long as we are together and having a good time is what counts!

I got home from my parents' and took a bath…
Living with PH means trying to avoid stress. Stress takes a major toll on our health and can make us sicker and keep the meds from working properly.
There are times when a PHer has a very good day, in which they sometimes try to do everything on their "To Do" list that they can't do when it's a bad PH day. This, unfortunately, often leads to a day or two of bad PH days afterward. Often this can be very discouraging.

I am one of those who usually overdoes it when I'm having a good day. I KNOW I should be taking it easier, and that I don't have to accomplish everything I need to do in one day, but I'm hard-headed and I just don't learn! So then I need to take at least a day to just do nothing because I went overboard the day before that. It's a vicious cycle that I just don't seem to break out of! lol
Some people with pulmonary hypertension may be able to continue working after being diagnosed. However, there are many who cannot keep up with the demands of the job they are in because it is too physically (and perhaps emotionally) draining.

There would have been no way I could continue working as a preschool teacher, yet I would have if my doctor had not told me to quit my job. When I look back at how I felt when I was working, I wonder how the heck I ever got through my days!! The sheer exhaustion I felt when coming home from work every day was terrible, and while I'm sad that I can't continue teaching in a classroom, I'm so thankful that I'm feeling so much better than I did back then because I'm no longer working.
"But you don't look sick," is a very common phrase so many PHers hear, and sometimes it is very irritating. This is when The Spoon Theory comes in handy. The story can be read here.
Exercise is an important part of maintaining health, but it can be very difficult for PHers to do. Many PHers are short of breath with minimal exertion. Each patient is different. There are some who attend pulmonary rehab, or have asked their doctor what type of exercise may be beneficial to them. Cardiovascular activities and ones focusing on muscle tone are important! PHers must not over-exert while exercising, and must give themselves enough recovery time to see if they can continue with more activity.

I have said so many times how much pulmonary rehab has really made a difference for me since I started it over 2 years ago. There are some days when I'm dragging trying to get through exercising, but there are some day when I really do feel great. It's an incredible feeling to be able to exercise when it's something I never did growing up. Not to mention, all the muscles I have, especially in my arms! Whoo hoo! LOL
Many people living with PH must watch their salt and sodium intake because of possible fluid retention. Fluid retention can make it hard for a PHer to breathe. The need for diuretics is very common for PHers to help them lose excess fluid. Watching salt intake, buying low salt or no salt foods, cooking fresh food and using lots of spices and herbs are some ways for a PHer to control salt consumption in their diet.

I think I am one of the lucky PHers who really don't have issues with salt and require the need for diuretics. This doesn't mean that I don't watch my sodium intake. I don't cook with salt 95% of the time, and I read labels and avoid products with alot of salt in them. And I like to cook fresh as much as I can. Let's face it, PHer or not, salt really isn't good for anyone out there!! And it really boggles my mind watching people, like my dad, pour salt all over their food. I actually find that much salt really disgusting!!
Being diagnosed with pulmonary hypertension can be an emotional rollercoaster for most PHers. They go through denial and anger quite often before they can finally wrap their head around what they are dealing with now. Learning to cope with limitations can be extremely frustrating, but eventually most PHers learn to live with a new "normal."
All the meds for pulmonary hypertension have side effects. Some sides don't stick around long. Some do. Side effects include one or more of the following, depending on which med/meds are taken: headaches, nausea, swelling in the lower extremities, liver damage, vision changes, nasal congestion, jaw pain, dizziness, nose bleeds, difficulty sleeping, heartburn, flushing, diarrhea, bone pain, rashes, infections, throat irritation.

When I started Tracleer, I got headaches, but I was nauseous for almost 2 weeks before that went away. When the dose was increased the next month, I went through the same side effects for a couple weeks, and since then, it hasn't bothered me.

When I started Revatio, the headaches were really bad for a couple weeks. Excedrin seemed to help, but they were almost constant before they started dissapating. And then I increased the dose, and they came back. The one side effect I get most often from the Revatio, usually after my last dose of the night, is stuffi…

Today is PH Blogging Day!

A few years ago, a phriend wanted to do something for PH awareness month, but being that she could not physically go out and about and spread the word, she came to me with an idea to blog about PH. I thought it was a wonderful idea! So in my post today, I decided to share how I've felt growing up with this disease, before being treated, and even after.

I have said before that I was born with pulmonary hypertension and congenital heart disease. Neither were discovered and diagnosed until I was 9 months old. The doctors couldn't repair the 2 holes in my heart because it was too late to do so, so they told my parents I might not live until 1, or I might live 50. In 15 more years, I WILL hit that 50 year mark, and I hope to do so while still in pretty good shape.

Obviously, I remember nothing as a baby with PH. I only know my mom told me that I was a pretty good baby. I didn't cry much, probably because crying made me way too tired. I didn't gain pounds, I gained ounces. Bre…


I had a dream last night, or early this morning, about black lab puppies that were being offered up for adoption. When I got up and thought about it this morning, I realized this was actually a Part II dream, because I remember dreaming about the puppies before. The first dream, they were too little for me to take home if I wanted them. By the time I had this dream, the puppies were much bigger. And for some reason, they were no longer black. Actually, they kept going back and forth from being black labs to golden labs, depending on the scene. But in the dream, I kept trying to figure out whether I wanted one or both of them. My family kept telling me I couldn't have either, because how could I possibly take care of them? And that thought also kept going through my mind. I'd never be able to bring it outside all the time, especially in winter, and I could never take it for a walk because I just don't have that kind of stamina. I remember someone ended up with one of the pu…
Tyvaso, or treprostinol, is another inhaled treatment meant to relieve PH symptoms. It works by opening up the blood vessels in the lungs, which helps to increase activity in the patient. The difference between Tyvaso and Ventavis is that Tyvaso inhalations are not as long, and does not need to be done as many times a day. This does, however, depend on a patient's needs.
Ventavis, or iloprost, was the first inhaled treatment for PH symptoms. It works by direct vasodilation of the blood vessels in the lungs. Using Ventavis has shown improvement in shortness of breath and an increase in activity. Ventavis treatments are done every 1.5-2 hours a day, from 6-9 times a day. The cost of Ventavis is around $60-70000 a year.
Adcirca, or tadalafil, or Cialis, treats symptoms of pulmonary hypertension much in the same way as Revatio. It works by dilating the blood vessels in the lungs, allowing more blood to flow and increase physical activity in the patient. The major difference between Revatio and Adcirca is dosage. Revatio is 3 times a day, Adcirca is just once. Important to note, with both of these meds, nitrates must never be taken! It can cause an unsafe drop in blood pressure.

I forgot to mention yesterday with my post on Revatio that I've been taking it since the summer of 2007. I have found that the combination of Tracleer and Revatio have improved my ability to do physical activities, such as pulmonary rehab. I'm so thankful that right now, these 2 meds have allowed me to be a bit more active than I was many years ago!
Revatio, or sildenafil, or Viagra, is another pill to treat PH symptoms. It works by relaxing and widening the blood vessels in the lungs to allow the heart to pump more blood through the lungs. It helps to reduce blood pressure in the lungs and allow more physical activity. It is taken 3 times a day. It costs around $10000 a year.
Letairis, or ambrisentan, is another oral med to treat symptoms of PH. It works much in the same way as Tracleer does, blocking endothelin to allow for reduced blood pressures in the blood vessels, and increased activity in the patient. Monthly blood tests are required to check for liver damage and pregnancy. It costs around $48,000 a year.
Tracleer, or bosentan, was the first oral med treatment approved for PH. It works by blocking the effects of endothelin, which causes blood vessels to narrow, and scarring and overgrowth of the muscle walls of the blood vessels of the lungs. Tracleer can reduce the blood pressure in the lungs and improve activity and wellbeing. It is taken twice a day. Liver tests must be done monthly since it can cause liver damage. Women must do pregnancy tests monthly since Tracleer can cause major birth defects. It costs around $50k a year.

I began taking Tracleer on Oct. 12, 2003. It changed my life. I didn't notice it working at first, but after a few months, subtle changes were appearing. I wasn't gasping for breath after walking long distances. It was truly life changing for me. I am so thankful for those people who came up with it as a treatment. I'm hoping those same people can also find a cure!
Remodulin, or treprostinil, works as a vasodilator to widen the narrowed blood vessels in the lungs, and inhibits platelets from clumping. Remodulin can be use by IV, subcutaneously (both use pumps), or by inhalation. Inhaled Remodulin is called Tyvaso. Remodulin can cost around the same amount as Flolan every year, depending on dosage needs of the patient.
Flolan, or epoprostenol, was the first FDA approved medicine to treat symptoms of PH. It is a continuous IV through a permanent catheter placed in one of the large veins of the heart. A pump is used to deliver the med into the system. Flolan works by dilating the blood vessels and helping the right side of the heart pump more blood through the lungs. The cost of Flolan is at least $100k per year, sometimes higher depending on patient dosing.

Perhaps The Coast Is Clear

Last Wednesday I was feeling more tired than usual, but last Thursday I felt as if I might be coming down with a cold. I went to rehab and ran errands, but then I went home. I spent this weekend in the house laying low, overdosing on Vitamin C and tea and soup, and just getting as much rest as I could. I do NOT want to get sick. I think I've avoided getting a cold, though. The only issue I keep having is a slight headache at the base of my neck. Nothing seems to help it, so I'm trying my best to ignore it. Seems to be working.

I did do some things around the house during the weekend, but what I was most happy with was working on jewelry. I made an illusion necklace set and a single illusion necklace on Friday night. Last night and today, I ended up making 12 bracelets and a few pairs of earrings. Friday night and tonight, I sold a few bracelets that I had in my Etsy store. Overall, a pretty good weekend jewelry-wise!

I plan on breaking out of the house tomorrow to get to the pos…
In 1975, when I was diagnosed with pulmonary hypertension, there weren't any treatments available. Twenty years later in 1995, the first FDA-approved treatment became available in the form of a continuous IV med called Flolan. It is still considered the gold standard of treatment for PH, especially for patients who are very ill. Today, there are now 8 FDA-approved treatments for the symptoms of PH. There is still no cure.
Although pulmonary hypertension is a lung disease which effects the heart, there are many pulmonologists (doctors who specialize in lung conditions) and cardiologists (doctors who specialize in heart conditions) who do not understand PH at all. Therefore, PH patients need to see a PH specialist, a pulmonologist or cardiologist whose main or only focus in their practice is treating PH patients.

A reader asked me what my first indication of having PH was, or did my doctors find it. Well, a doctor did realize something was going on with my heart when my mom brought me for my first set of shots at a free clinic when I was a baby. The doctor told my mom to talk to my pediatrician, but that doctor didn't think anything was wrong. So, when my mom brought me for my second set of shots at the same free clinic, the same doctor told her there was something very wrong, and that I looked cyanotic (blue). My mom once again told my pediatrician, and I was scheduled to see a pediatric cardiologist…
Pulmonary hypertension may be suspected by an echo, but the ONLY test used to actually diagnose PH is a right heart catherization. Other tests performed to help determine whether PH is the only thing wrong, or if the person has underlying causes for PH are blood test, chest xray, pulmonary function tests (PFTs), 6 minute walk, nuclear scan, and electrocardiogram.

Another Year of Exercise

This morning I reluctantly got out of bed to get ready to go to rehab and then do some errands. I think I'm coming down with something, so I really debated for quite awhile whether to even go outside. But, I did, and I left a little bit early so that when I got to the hospital, I could see the Financial Advisor about getting help to pay for rehab for another year. Despite how I'm feeling, I'm glad that I went. She was readily available, she remembered me from last year, and she set it up and printed out a copy for myself. She told me next year when I start thinking about turkeys, to come see her again. lol I'm glad that is taken care of. I don't know what I'd do if I had to stop rehab because I can't afford it anymore on my own. I really do think it's helping me so much!
Major types of PH include: IPAH (Idiopathic Pulmonary Arterial Hypertension) - cause is unknown; FPAH (Familial PAH) - the cause is due to genes; APAH (Associated PAH) - the cause is due to another disease or chronic condition, including connective tissue disease, congenital heart disease, chronic liver disease, HIV, drugs, toxins, and more.
You cannot get pulmonary hypertension from smoking. You cannot get it from being overweight. You cannot get it from being lazy and not exercising. Any doctor who tells you this is full of crap and doesn't know a thing about this disease.
Pulmonary hypertension is not regular high blood pressure. It is high blood pressure in the arteries of the lungs that can lead to heart failure.

To Make Aware

November marks PH Awareness month! If I asked 5 people if they knew what pulmonary hypertension was, I would actually be shocked if ONE person knew. This disease really needs more awareness! If you or someone you know are experiencing shortness of breath from doing the smallest task or just walking down a hall, are having fainting spells or dizziness that cannot be explained, or are extremely fatigued all the time, please get to a doctor! Pulmonary hypertension may not be the cause, but it's better to be safe than sorry in getting it checked out as soon as possible!