Monday, November 21, 2011


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!!

Sunday, November 20, 2011

Inhaled Medication

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.

Saturday, November 19, 2011

Oral Medication IV

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.

Friday, November 18, 2011

Oral Medication III

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 regimen.

Thursday, November 17, 2011

Oral Medication II

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.

Wednesday, November 16, 2011

Oral Medication

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 incredibly thankful for the advances in science since the time I was diagnosed with PH as a baby!!

Tuesday, November 15, 2011

Another Medication

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.

Monday, November 14, 2011

The Gold Standard

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 would do it. Anything to keep me going, even for a little bit, is worth it to me!

Monday, November 07, 2011

Eisenmenger Syndrome

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!

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, symptoms related to cyanosis may occur much earlier in life.

What causes Eisenmenger syndrome?

Eisenmenger syndrome progresses over time as a result of the effects of high blood pressure in the lungs. This high blood pressure, or pulmonary hypertension, occurs because of congenital heart defects that cause blood flow from the left side of the heart to the right side of the heart (left-to-right shunt). Congenital heart defects of this type include:

*patent ductus arteriosus (PDA) - a connection between the aorta and the pulmonary artery which allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs.
*atrial septal defect (ASD) - an opening in the atrial septum, or dividing wall between the two upper chambers of the heart known as the right and left atria.
ventricular septal defect (VSD) - an opening in the ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles.
*atrioventricular canal defect (AV canal) - a complex heart problem that involves several abnormalities of structures inside the heart, including an ASD, VSD, and improperly formed mitral and/or tricuspid valves.

Because the pressures within the left side of the heart are normally greater than those within the right side of the heart, an opening between the left and right side of the heart will cause blood to flow from the left side of the heart into the right side. This left-to-right shunting of blood within the heart causes increased blood flow in the blood vessels of the lungs. The increased blood flow in the lungs' blood vessels causes increased pressure in these vessels (pulmonary hypertension).

If the pulmonary hypertension continues without treatment, the pressure in the right side of the heart may increase to the point that the right side pressure is greater than the left. When this occurs, blood will flow from the right side of the heart to the left (right-to-left shunt), which means that oxygen-poor blood is mixed with the oxygen-rich blood pumped out to the body from the left ventricle.

A reversal of the shunt resulting in a right-to-left shunt causes insufficient oxygen in the blood. In addition, high pressure in the lungs causes progressive changes in the pulmonary blood vessels which result in irreversible damage to the lining of these blood vessels. Fibrosis (the growth of scar tissue due to infection, inflammation, injury, or even healing), and thrombus (clotting) may also occur. The changes inside the lung blood vessels may be referred to as pulmonary vascular obstructive disease or secondary pulmonary arterial hypertension (PAH).

What are common symptoms or complications of Eisenmenger syndrome?


cyanosis (pale blue or grayish skin due to decreased oxygen in the blood)
dyspnea on exertion (shortness of breath with activity)
shortness of breath at rest
chest pain or chest tightness
heart palpitations ("skipping beats or racing")
dizziness or syncope (fainting)
paresthesias (numbness and/or tingling of fingers and toes)
blurred vision


blood clots (e.g., deep vein thrombosis in extremities)
hemorrhage (bleeding)
brain abscesses

Worsening of symptoms or new onset complications can be early warning signs of changes in overall condition and should be evaluated by your physician as soon as possible.

The symptoms of Eisenmenger syndrome may resemble other medical conditions or heart problems. Always consult your physician for a thorough evaluation.

How is Eisenmenger syndrome diagnosed?

A child or adolescent with a history of an atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), or atrioventricular canal defect (AV canal) that has been unrepaired or repaired after one year of age is at risk for developing Eisenmenger syndrome.

The physician may note a change or increase in intensity of a cardiac murmur (a blowing or rasping sound heard while listening to the heart that may or may not indicate problems within the heart or circulatory system) during a routine physical examination. Complaints of change in exercise tolerance or any associated shortness of breath, chest pain, or palpitations, especially with activity, should be reported to the physician for further investigation.

The physician will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. Other tests that may be performed to help with the diagnosis include the following:

blood tests - tests to evaluate for an elevated red blood cell count (erythrocytosis) and anemia.

chest x-ray - a diagnostic test which uses invisible X-ray energy beams to produce images of internal tissues, bones, and organs onto film. There may be changes that take place in the lungs due to extra blood flow that can be seen on an x-ray.

electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress.

echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the PDA, and determine how large the opening is, as well as how much blood is passing through it.

cardiac catheterization - a cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Right Heart Cath: pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Left Heart Cath: contrast dye may also be injected to more clearly visualize the blood flow and structures inside the heart.
magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. MRI may be used to aid in estimating pulmonary artery pressures in the lungs and to clarify any blood shunting.

Treatment for Eisenmenger syndrome:

Specific treatment for Eisenmenger syndrome will be determined by your physician based on:

age, overall health, and medical history
rate of progression and extent of the disease
tolerance for specific medications, procedures, or therapies
expectations for the course of the disease
personal opinion or preference

The goals of treatment for Eisenmenger syndrome are aimed at decreasing the pulmonary artery pressure, improving oxygenation, and decreasing degree of cyanosis and erythrocytosis. Treatment methods may include, but are not limited to, the following:

medical treatment
Medical treatment for Eisenmenger syndrome is based on symptoms. Types of medical treatments include:

Medications that help to decrease pulmonary hypertension may be prescribed. These medications include calcium channel blockers, prostacyclin, and endothelin receptor antagonists, and work to dilate blood vessels which helps to lower the blood pressure in the lungs. If right heart failure develops, diuretics (water pills) may be prescribed to help decrease blood volume.

Supplemental oxygen may be used during sleep periods or while resting. Use of oxygen may progress to continuous use for symptom relief.

Phlebotomy (removal of blood) should not be done frequently, but may be recommended when erythrocytosis causes hyperviscosity (extreme thickness) of the blood. The volume of the blood removed is replaced with intravenous (IV) saline solution to thin the blood. Phlebotomy is generally done only when symptoms are severe and/or the hematocrit (the percentage of blood that is made up of red blood cells) becomes extremely elevated.

other treatments
Eisenmenger syndrome that develops as a result of a congenital heart defect that was never repaired is generally not treated by repairing the original defect, as it would increase the workload on the right ventricle and lead to a poor outcome. Lung transplantation or heart-lung transplantation may be performed when other treatments are no longer effective.

Long-term outlook with Eisenmenger syndrome:

Some individuals with Eisenmenger syndrome who have no other health complications may live into middle adulthood, and a few may survive into the fifth or sixth decade of life.

Considerations related to Eisenmenger syndrome include, but are not limited to, the following:

Pregnancy is not recommended for females of child-bearing age with Eisenmenger syndrome. Pregnancy may pose a high risk of death for the mother as well as complications for the fetus due to effects of low oxygen levels in the blood.

Any anesthesia and surgery is considered high risk and should be carefully planned. Collaboration with a cardiac specialist is recommended.

Air travel and high altitude exposure requires adequate hydration (fluid intake) and supplemental oxygen to prevent complications.

Severe headache, dizziness, syncope (fainting), or changes in level of consciousness should be taken seriously and evaluated emergently.

Smoking and alcohol intake are not recommended.

Coughing should be controlled or prevented with a strong suppressant medication to prevent risk of pulmonary hemorrhage (bleeding from the lungs).

Flu shots are recommended annually, and pneumococcal vaccine should be received according to the physician's recommendation.

Consult your physician regarding the specific long-term outlook for your child, teen, or young adult with Eisenmenger Syndrome..

Sunday, November 06, 2011


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 this news might mean that perhaps I could eventually wean myself off of oxygen. I've been wearing it for the past 10 years, though, so I was a bit leery. Now, I have always been able to take the oxygen off for awhile (and by awhile, I mean several hours) before I felt the need to put it back on. I've done it so many times, and I've never had major problems. But to go without it for a day? Hmmm, I'm not so sure about that!

So, backtrack to Friday afternoon around 3pm. I had come home after being out for a bit, and the oxygen guy was still at my house because his van wouldn't start. After I brought in my bags, I went out to say hi to him, and chatted for a few before he took off (someone had come to give him a jump by then). I went back in the house and filled the water attachments on the two large liquid oxygen tanks since they were empty, put the tubing on, and went about the rest of my day. Yesterday morning, I had a little bit of chest pain, which is unusual for me, and I tried to figure out what the heck I did to make my chest hurt. A few times, I've lifted something too heavy or did something too strenuous so that I ended up with pain the next day or two. I tried a heating pad for awhile, which didn't seem to help too much. But then I drove out to K.'s house in the afternoon around 2:30, and got back home after 10:30. I felt fine the whole time I was with him, but on the drive home, I could feel the pain again. So after taking a bath, I went to put the tubing back on. Now whenever I leave the house, or even take the oxygen off at home for a little while, I never turn the two tanks off. I just leave them running. So when I went to put the tubing back on, I realized no oxygen was coming out of the nose parts. I knew I hadn't turn the tanks off before I left for K.'s house. And all of a sudden, it dawned on me that I had never turned the tanks on from Friday after the oxygen guy left!!! I wore a cannula as decoration for 24 hours, with no oxygen!!! WHAT THE HECK IS WRONG WITH ME????? No wonder I had chest pain!! DUH!!!! Soooooooo, I went over to the tanks and turned them on to a little bit of a higher number than I'm used to, and since I've had it on since last night, my chest pain is much better!!

So the answer to whether or not I can eventually wean myself off of oxygen? I would have to say that is a no. It stinks to know that, and I kind of figured that I wouldn't be able to, but now I know for sure! I think what I need to do is start writing myself big notes to turn the oxygen on here and at rehab, since in the last several months there have been way too many instances of me wearing tubing without any oxygen coming out of it!! lol


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.

Thursday, November 03, 2011

Cool Dream!

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 buck when K. comes back to find me hanging out with a buck! He was completely shocked himself! And then, I woke up.

Yesterday, I drove to K.'s to go for a short walk along the trails again. While we did see a doe and her fawn rather close, and 7 other deer, we didn't see a buck. I thought it was funny, too, that K. did go up a hill that was sort of a trail to see where it led, and I was listening for anything around me in case my dream came true!! Sadly, it didn't! Maybe we'll try for Saturday again, and hope for the best!


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!!

Wednesday, November 02, 2011


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.

Tuesday, November 01, 2011

It's November, And That Means......'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.