Showing posts from November, 2012

Annnnnnd, It's Over!

Today is the last day for Pulmonary Hypertension Awareness Month. I'd like to think I helped spread some awareness through my blog, my Facebook statuses (which were the same as  my blog, mostly lol), and this year, through my PH display at 2 hospitals! I'm very happy I was given permission to do the display, and I am hoping I can do it all over again next November! Until then, I will continue to try making people aware of this crappy disease, because:

-Anyone can have PH
-Age and gender make no difference
-The symptoms mimic other illnesses, so a person can be misdiagnosed for years
-If not treated as soon as possible, it can lead to right heart failure

Just A Quick Fact

I get asked often when I'm seen by someone wearing the oxygen if I used to smoke. No. Never. Dated a smoker a long time ago, and it almost felt like I was smoking, but no. I've never touched a cigarette.

PH is NOT caused by smoking.

There is my simple PH fact of the day!

Is And Is Not

I wrote this a few weeks ago to include on my PH display board since I had some space and needed something to fill it!! Since I'm winding down on PH Awareness month, I thought I'd share this info again!
Pulmonary hypertension


High blood pressure


Pulmonary hypertension




Pulmonary hypertension




Pulmonary hypertension




Pulmonary hypertension


Sleep apnea


Pulmonary hypertension


High blood pressure in the lungs


Pulmonary hypertension


Life-threatening if not treated


Pulmonary hypertension


Diagnosed by right heart cath


Pulmonary hypertension




Pulmonary hypertension


Life changing


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 (pulmonary hyp…

Spoons Anyone?

Quite a few years ago, I heard about the Spoon Theory, a story written by a woman, Christine Miserandino, who is living with Lupus. She was trying to explain to a friend how she deals with her energy every day, and the friend didn't get it. So she explained it by using spoons. 
The Spoon Theory is a story that can fit almost anyone living with a chronic disease, not just Lupus. It completely fits the life of someone dealing with pulmonary hypertension. There are some days when I might have a ton of spoons, and I can do a bunch of things and feel pretty decent. There are even days when I miraculously end up with some extra spoons by the end of the day! And then there are those days when I might drop all  my spoons on the floor the minute I get out of bed in the morning. I have often used this story to explain to my family and friends how it feels to live with PH. They seem to understand when I tell them I don't have enough spoons to deal with something. It sucks, but at least …

Over A Century Ago

The first ever reported case of Pulmonary Hypertension was recorded 121 years ago.

37 years ago, still not much was known about the disease.

Today, we have 9 FDA-approved medications to help improve symptoms experienced by the disease.

There is still no cure.


A PH Tip

Nothing makes my heart start racing with immense anxiety when I am faced with something most people take for granted: stairs. Or, a hill. I immediately think, "Oh crap, I have to get up that! I'm gonna die!" It may sound silly, but I truly hate the feeling of a rapid heartbeat and being so short of breath I think I might pass out. And the sad thing is, I've been slowly increasing the grade on the treadmill at rehab so I can face stairs or a hill a lot better. I do think it's helped me a little, but I still have that fear of climbing!!
So, here's a PH tip for going up stairs: Go up backwards. Seriously, I have heard some PHers talk about this, but all I could think was how utterly ridiculous that must look! Well, I tried it several times this summer. And guess what? You look utterly ridiculous, BUT, it's so much better than going up the regular way! I would get to the top and realize I wasn't going to die! The shortness of breath was minimal, and my he…

Giving Thanks

Today is Thanksgiving here in America, and it happens to be a favorite holiday of mine. Mainly because of the food! lol No really, Thanksgiving is great to spend time with family and friends, and to eat. A lot. :)

I happen to be thankful for so many things. My family is my life, my friends are wonderful, and my health is stable at this point in time. I have a roof over my head, clothes to wear, and a very loving "kid" I just adore.

And it may seem odd to say this, but in a way I'm thankful for PH. Obviously I don't love having this illness, nor do I wish it upon anyone. But what I'm thankful for is that it's given me a sense of purpose. It's allowed me to help others. It's given me time. Time to spend with my family and friends since I don't work. It's given me the ability to empathize, to feel a connection and understanding with those dealing with PH or any sort of illness. It's allowed me to appreciate the little things in life. Had I be…


Have you ever wondered how it actually felt to have pulmonary hypertension? I mean, I can say to you that I get short of breath just making my bed sometimes.  Getting dressed some days, especially after a shower, can be a challenge. And in the winter, if I happen to go out to a store by myself, I drive right back home if I can't find a handicap parking spot because I know parking any farther will make me gasp by the time I get into the store.

But are you wondering what that actually FEELS like? Well, try this experiment. Walk up a flight of stairs. But walk up those stairs breathing through a straw. Not just any straw. Use a straw meant for stirring coffee. While you're at it, plug your nose while going up those stairs, breathing only through that tiny straw. Are you gasping for breath by the time you get up there? Is your heart racing, feeling like it's going to bound out of your chest down the street?

Yeah? Well, now you know what it feels like I try to vacuum one rug. O…

It's Personal - Part III

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 …

It's Personal - Part II

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 …

It's Personal - Part I

My personal PH journey began when I was diagnosed at 9 months old. After two trips to a free clinic to get my shots, and the same doctor at the clinic insisting something serious was wrong with my heart, my parents took me to a cardiologist at Buffalo Children's Hospital. I was immediately taken to the back despite the room full of parents waiting their turn to see the doctor. Apparently I looked a bit too blue. After many tests, including a right heart catherization, it was determined that I had a severe heart defect (atrioventricular canal), and pulmonary hypertension. This meant that I was born with both problems, and no one knew until 9 months later. The AV canal - which is an atrial septal defect (ASD) and a ventricular septal defect (VSD) - could not be repaired by the time it was found. My parents were pretty much told to take me home and love me the best they could, because it was unknown how much time I'd have here. I can't imagine what they must have felt being t…

Where To Turn?

A person gets slammed with a diagnosis of PH, probably after months or even years of trying to figure out what is wrong with their body. What in the world do they do now?? Who do they talk to about it? Family and friends may try to understand, or may not even be supportive. Finding other people who also live with PH can help a "newbie" in so many ways. Many years ago, before the internet changed the world, it was rather hard meeting people with PH. Today, the world wide web can bring people together in chat rooms, message boards, and social networking sites.Websites, such as the PHA and PH Central, can also provide information on how to get in touch with a PHer in person, through support groups. A newly diagnosed PH patient does not have to feel so alone with their disease!

At Risk

Although PAH can be diagnosed in anyone regardless of age, race, or gender, there are certain risk factors that can make some people more likely to get the disease. These risk factors include:

-Family history
-Obesity and obstructive sleep apnea
-Other diseases
-Drugs and toxins

You can read more about these risk factors HERE!

Does That Come With Sides?

All of the PAH medications come with side effects. Some people experience side effects for a little while, and then they don't deal with them any longer. Some people have side effects constantly. Side effects can include one or more of the following, depending on the medication(s) being taken:

-Low blood pressure
-Fluid retention
-Irregular heart rhythms
-Excessive bleeding
-Liver failure
-Blurry vision
-Loss of hearing
-Nasal congestion
-Muscle pain
-Jaw Pain
-Bone pain

Under The Skin

I talked about Remodulin yesterday as being an IV medication to treat PAH symptoms. Remodulin can also be administered subcutaneously, meaning, under the skin. Subq Remodulin works in the same way as IV and inhaled Remodulin, by relaxing the blood vessels to allow more blood to flow and carry oxygen around, which then allows the heart to pump easier. Subq Remodulin requires site changes every so often, and are usually put into the abdominal region. Subq Remodulin can be quite painful for most users.

IV Medications for PAH

The very first type of treatment available for PAH was Flolan (epoprostenol). Flolan has been considered the Gold Standard in PAH treatments for a very long time. It's an IV med that goes straight into the heart via catheter. It dilates the blood vessels and allows the right heart to work more efficiently in pumping the blood. Flolan has a very short half-life, which means the medication only lasts a few minutes in the bloodstream. A pump connected to the line helps to administer the med, and the dosage is adjusted according to the patient's  body weight in kilograms. Flolan must be kept cold, and ice packs are used all the time while using the med.

Another type of IV medication is Remodulin (treprostinil). It also helps to relax the blood vessels to allow for better circulation of the blood in the body. Remodulin is requires a catheter and a pump to administer the med, but it has a longer half-life than Flolan. It also does not require ice packs for usage.

Room temperature ep…

Inhale, Don't Exhale!

Currently, there are two inhaled medications for PAH. These are nothing like the asthma meds you may be familiar with!

*Ventavis (iloprost): When used, Ventavis causes direct vasodilation of the blood vessels in the lungs, meaning the blood vessels relax and open up to allow more blood flow through the vessels. Ventavis treatments are done around 6-9 times a day, depending on how the PH patient responds and feels.

*Tyvaso (treprostinil): Tyvaso works very much like Ventavis, in that it goes directly into the lungs through inhalation, and opens up the blood vessels to allow more blood through the vessels. Tyvaso is done in 4 separate treatments a day, spaced evenly apart.

Oral Medications

There are currently 4 oral medications approved by the FDA to treat PAH symptoms.

*Tracleer (bosentan): Tracleer is an ERA, which stands for endothelin receptor antagonist. Endothelin causes the blood vessels to narrow. Tracleer blocks endothelin, which is produced in overabundance in PAH patients. This helps to reduce blood pressure in the lungs, and to improve activity level. Tracleer is taken twice a day, 12 hours apart. Tracleer requires monthly bloodwork to check for liver failure.

*Letairis (ambrisentan): Letairis is also an ERA, and also works very much in the same way as Tracleer does. It is taken once a day. Letairis does not require bloodwork, although some doctors may still want labs checked occasionally.

*Revatio (sildenafil): Revatio is a PDE5 inhibitor. This means that Revatio relaxes the blood vessels of the lungs to increase blood flow, making it easier for the heart to pump. Revatio is taken three times daily. Revatio is commonly know as Viagra!

*Adcirca (tadalafil): …

Conventional Drugs

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 meds hel…

Mmmmmm, Drugs

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

Types of PH

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!).

Too Many Initials

PH = pulmonary hypertension: I use PH since it's easier to say or type, but it's just a general reference to pulmonary hypertension. 
PAH = pulmonary arterial hypertension: PAH is when the right side of the heart needs to work harder to get blood pumped through the narrowed arteries in the lungs. If the right side needs to work harder, it becomes enlarged, and can eventually fail.
PVH = pulmonary venous hypertension: PVH is PH due to left heart disease, like systolic dysfunction, diastolic dysfunction, and valvular disease.

Testing, 1,2,3

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 lungs can …

Doctor Doctor

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 t…

Right Heart Catherization (Sounds Fun, Right??)

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 pulmon…

The OTHER Hypertension

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, thank you!

Sometimes It's PH!

Shortness of breath.

Extreme fatigue.


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.


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!!

It's November!! You Know What That Means!!

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 long after …