Daily Ponderings

The only cure for PH is a lung transplant. Not everyone is eligible for one, not everyone wants to go through one. It is not an easy fix. Transplant brings along many complications, and an entirely different set of medications for life. This is why we need more PH awareness and a cure!!

Ventavis (iloprost) was the first FDA-approved inhaler treatment for symptoms of PH. It was approved in 2004. Ventavis is taken 6-9 times a day, depending on the response of the patient. Ventavis can cost around $60-70,000 a year. The biggest complaint I've heard from patients on Ventavis is the amount of times a person needs to take the treatment a day. In order to fit up to 9 treatments, it needs to be done about every 2 hours. Another complaint is the setup and the cleanup, which can be time consuming. However, Ventavis has helped many PHers, so I guess you just have to look at the benefits outweighing the annoyances.

Adcirca (tadalafil or Cialis), was FDA-approved to treat PH symptoms in 2009. It works the same way Revatio works. The difference is that Adcirca is taken once a day, whereas Revatio is taken three times a day.

Revatio (sildenafil or viagra) was FDA-approved for treating PH symptoms in 2005. It works by relaxing the blood vessels in the lungs, which allows the heart to pump more blood into the lungs easier than when the vessels are constricted. Revatio is taken 3 times a day, 8 hours between each dose. Revatio costs at least $10,000 a year. Nitrates or nitric oxide should NOT be taken with Revatio, as it can cause an unsafe drop in blood pressure. I began Revatio on August 16, 2007. I felt the need to add something along with the Tracleer, because I didn't feel it was doing enough for me almost a year after taking it. I still waited pretty long before asking about Revatio, but when I finally did, my PH doctor agreed to let me try it. I really feel that the combination of Tracleer and Revatio have been keeping me stable in the last few years! I hope it continues that way, but if I need to, I can increase the amount of Revatio I take before considering another drug to add to my daily regime...

Letairis (ambrisentan) is another oral medication that can be used to treat symptoms of PH. It was FDA-approved in 2007. It works much in the same way as Tracleer does, only it is taken once a day. Also, just recently monthly bloodwork to check liver enzymes are no longer required, although some doctors may want their patients to continue to get theirs checked. Letairis can cost around $70,000 a year.

Tracleer (bosentan) became the first oral treatment for PH, being FDA-approved in 2001. It is taken twice a day, 12 hours apart. Tracleer can cause liver damage, which is why monthly bloodwork is required by all PH patients taking this med. Tracleer can also cause severe birth defects, which is also why women taking Tracleer must also have a monthly pregnancy test. Tracleer costs almost $70,000 a year. Tracleer was the first PH med that I started. I took the first pill on Oct. 12, 2003. While I didn't notice any huge difference for quite awhile, the first tiny inkling I had of the med working for me was almost a month after I had started it. I had to go vote, and on the way back to my car from the building, it suddenly dawned on me that I wasn't as short of breath as I usually get from walking a distance. I got in my car and cried. Months later, I was dancing at my sister's wedding. I couldn't believe what a change Tracleer made in my daily living, and it made me incred...

Remodulin (treprostinil) is another treatment for PH that can be delivered in three ways: by IV through the veins, or subcutaneously (under the skin), or by inhalation (called Tyvaso). There are also trials that are being conducted right now for oral Remodulin, and hopefully that will be approved at some point as another way to use this med. Depending on the dosage a patient is given, Remodulin can cost at least $100,000 a year. If I were to need another drug added to the regimen of pills I'm taking every day to treat my PH symptoms, I would probably ask my doctors about adding Tyvaso. Once again, the IV treatment would be risky. The huge drawback with the subq version of Remodulin is the pain. I have known many PHers who just can't bear the pain that goes along with that way of using Remodulin. So I'm not sure I could do it, either! But the inhaled version seems to be much easier, so I would go along with that IF I were to need something more.

Flolan (epoprostenol) was the first FDA-approved treatment for PH symptoms. It only lasts a few minutes in the blood stream, which is why it needs to be delivered continuously by IV through a permanent catheter placed in a large vein going into the heart. Complications can include jaw pain, foot pain, headaches, rashes, as well as site infections. Not all patients experience all these problems. Flolan can cost approximated $100,000 a year, but may be higher depending on a patient's dosage. I have not been put on Flolan to treat my PH symptoms, and I may never need it. It's a powerful medication that has helped many PHers improve, but the risks may outweigh the benefits for some. In my case, because of the 2 holes in my heart, my cardiologist feels that Flolan would be a last resort option for me, most likely if I were waiting for a lung transplant. The risk of line infections is too great at this point, when I'm doing considerably well. But I know if I ever needed Flolan, I...

I know I have mentioned that I have Eisenmenger syndrome. Tonight I found a great link describing exactly what it is! I hope you're able to spend a few minutes reading it! https://www.memorialhealth.com/healthinfo/content.aspx?pageid=P08482 If you don't want to click on the link, the info is copied below: Eisenmenger Syndrome What is Eisenmenger syndrome? Eisenmenger syndrome is a condition that includes a collection of symptoms: cyanosis (pale blue or grayish skin due to decreased oxygen in the blood), pulmonary hypertension (high blood pressure in the blood vessels of the lungs), and erythrocytosis (increased number of red blood cells in the blood due to decreased oxygen in the blood). Eisenmenger syndrome primarily affects adolescents and adults with certain congenital heart defects that were repaired late (after the first year of life) or that were never repaired. Eisenmenger syndrome, however, can occur in infants or children born with pulmonary hypertension; therefore, sy...

Oxygen is something that some pulmonary hypertension patients may need. Some patients need it more than others, some may only need it at night, and some don't even need it at all. I am one of those who was told to use it at all hours of the day, despite the fact that my oxygen saturation levels (sats) never get to 90%. Normal oxygen sats are from 95-100%. For me, because I have Eisenmenger's, it's pretty much my norm to be hovering in the high 70 to mid-80% range when at rest. When I exercise or vacuum or something more physical, I dip into the low 60s, sometimes even into the high 50% range. Yes, that would be deadly for any normal person, but I am not normal!! lol I found out this past May that there isn't any proof that oxygen really helps Eisenmenger patients. I can actually understand why that is, since no matter how much oxygen I'm on, I will never get my sats into the normal range, and that especially goes for when I'm physically active. I thought hearing...

Pulmonary hypertension may be suspected after having an echo, however, this is not a definite way to diagnosis PH. A true diagnosis of PH is done by having a right heart catherization (RHC). During this procedure, an accurate measure of the pressures in the lungs are taken to determine whether or not someone has PH.

On Tuesday, K. told me that he saw a buck crossing near the area where he was getting his truck looked at. I was a little sad because I was hoping to see a buck when we walked along the trail a couple Saturdays ago. So we made plans to either try going Tuesday night, or maybe Wednesday, and also maybe this Saturday if the weather wasn't too bad. So Tuesday night, I had a dream: K. and I were walking along a trail, and K. went ahead of me to see if he could find a shortcut. I stood waiting for him and looking around, when suddenly I heard a noise behind me. I turned slowly, and there standing so close was a huge buck! I tried not to gasp since I didn't want to scare it away, but I definitely dropped my jaw in shock. And instead of backing away slowly, I just reached out my hand. The buck moved a little closer and allowed me to pet it. I PET A BUCK!! Now, I know in real life you can't do that, but this was a very cool dream!! So, I'm standing there petting this enormous b...

PH = pulmonary hypertension, a general term used to describe PH. PAH = pulmonary arterial hypertension, which means the right side of the heart is affected. PAH can be treated by 9 currently available FDA-approved PH meds. PVH = pulmonary venous hypertension, which means the left side of the heart is affected. Unfortunately, for PVH, many of the treatments available for PAH cannot be used to treat symptoms. These meds can actually worsen PVH patients. Hope this isn't too confusing!!

Pulmonary hypertension can happen without any known cause, which is rather frustrating for those who are diagnosed this way. This is known as idiopathic pulmonary hypertension. Pulmonary hypertension can be diagnosed secondary to a cause, like connective tissue diseases, congenital heart defects, chronic liver disease, HIV, drugs, and toxins. This is known as associated pulmonary hypertension. Pulmonary hypertension can also be genetic, which means it can be inherited. This is known as heritable pulmonary hypertension.

....it's PH Awareness Month!! Once again I'm going to try blogging every day for PH Awareness. I hope I can do it, since I've been bad at blogging every day!! Pulmonary hypertension is high blood pressure in the lungs. It is rare and it is incurable, and can be life-threatening if not treated. Anyone can have pulmonary hypertension. It does not discriminate by age or race. However, it does occur more frequently in women of child-bearing age. Pulmonary hypertension symptoms include shortness of breath, dizziness, and fatigue. Simple daily tasks can become very difficult with PH. Pulmonary hypertension is not a contagious disease. Very often, people with PH do not even look sick. For this reason, it's often considered an "invisible" health problem.

I spent the majority of my time on Friday and Saturday cleaning much of my apartment. Oh, I did the dishes and cleaned the bathroom, and vacuumed, but I also did a major dusting in 2 of my rooms, the bedroom and the living room. I have to admit that I did so much dusting as a kid for weekly chores every Saturday that it doesn't cross my mind to dust that often here. In fact, I don't remember the last time I dusted. Which is BAD. And I'm sure it's not good for my lungs. So, I spent a lot of time cleaning things, and throwing things out. I have a hard time parting with stuff sometimes. I see something I've been holding onto for years (sometimes decades), and every time I do clean, I think, "Hmmm, I could still use that," or, "Oh, I remember this!" And then I'd put it back where it was sitting forever. So, I chucked out a ton of items yesterday including jewelry (that was beyond tarnished), some stuffed animals, lots of old candles, candle stand...

Yesterday, I went to a park that had a lot of different trails you could walk, and it was huge. Now normally, I'm not always a fan of walking. Treadmill, sure. Walking down the street,fine. Going for a very long walk, especially when the temperature isn't all that warm, ummm, no. And that is my automatic thought, nope, I can't do it. Well, my biggest motivation to at least try it out this time was K. lol After getting some hot drinks, we got to the park, and began walking along one of the trails he thought I'd be able to do. It was nice, I'd honestly never been on trails before. We eventually started seeing deer, which was cool! I mean, I've seen deer before, but mostly dead on the side of the road. That is not cool! We kept going and going, stopping every once in awhile when there was a bench along the way. I was enjoying myself, not minding the weather too much, and not feeling too badly or terribly short of breath. Shocking for me!! The trail seemed to go on ...

A couple months ago, I emailed my PH doctor about possibly getting off of Coumadin, which is a blood thinner. I've been on it for about 10 miserable years. I was put on it the first trip I made to the Cleveland Clinic, and I just really have not enjoyed being on it. So I thought I would just ask about the possibility of being on a daily baby aspirin instead. I asked my PH doctor to consult my cardiologist. And then I waited and waited for an answer. Several weeks later, I emailed my PH doc again about another question. I just wanted to know, for the heck of it, if I'd be able to get a tattoo. I sent the email off, and again I waited. This time, the wait wasn't as long. Within a couple days, my doc emailed back. He said he hadn't even answered my first email (duh!). He said first of all, that he had consulted with my cardiologist, and they both were not sure I needed to be on Coumadin. My mouth dropped, and quite honestly, I started shaking with happiness!! WHAT?? I was ...

Yes, I'm slacking again. I would like to talk about a certain topic I've had a hard time with for a long time while living with PH, and that would be dating. Now I'm not a newbie at it, I have dated, and I have had a boyfriend, but it was years ago. Too many years ago. It was also before I had to wear oxygen all the time, and before I had to start taking major meds to treat PH. After all that happened, it was really hard to find someone who wanted to deal with everything. One person I found and truly loved was Euan, but he also had major health issues going on, and so it made it so much easier for us to relate and eventually fall for each other. And sadly now he is gone. I've been on several dating websites, but most recently I've been on one that is totally free. I purposely put pictures up with me wearing oxygen, because well, that's the truth. I figure if someone doesn't like it, they don't have to say anything. Why lie and have someone find out later...

I have been so neglectful in posting. Most of the time when I want to write, it's so late that I decide to maybe wait until the next day to do it. And then of course, I have so much to write about now that it would take me all night. lol My sister got married on Sept. 24th, and it was a pretty perfect day all around for her and her new husband. Nothing really went wrong or haywire, and the weather was just so beautiful! I didn't have to worry about carrying around a sweater, since it ended up being sunny and warm. The ceremony was wonderful, there were lots of people in the church. The family pictures were taken in a very beautiful building in downtown Niagara Falls called The Giacomo. Bridal party pictures were taken at The Giacomo as well as the Niagara Falls state park. By the time we got to the reception, my feet were killing me from walking around so much that I took my shoes off and didn't put my replacement shoes on until after dinner! There were around 240 guests ce...