What exactly is Pulmonary Hypertension?
According to the Cleveland Clinic, Pulmonary hypertension is high blood pressure in the arteries leading from the heart to the lungs. Pulmonary Hypertension can be caused by various kinds of conditions. Mine is caused by congenital heart defects and restrictive lung disease.
Some of the symptoms of Pulmonary Hypertension are:
- Shortness of breath (dyspnea), initially while exercising and eventually while at rest
- Dizziness or fainting spells (syncope)
- Chest pressure or pain
- Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites)
- Bluish color to your lips and skin (cyanosis)
- Racing pulse or heart palpitations
When I go to see my Pulmonary Hypertension specialist, I’m always asked or least checked for swelling in my lower legs near my ankles. I take diuretics twice a day along with prescribed supplemental Potassium. I’ve had Heart Catherizations off and on all my life. Starting in 2006, I started the medication Bosentan (aka: Tracleer). Bosentan is said to help improve your energy level and symptoms. For me and with all my health issues, I have not noticed any difference in energy level or symptoms. In fact, I feel worse than when I started the medication. It’s an issue that I’ve been trying to convince the doctor of for the last few years. However, their solution is to add new medication to my regime. So, I’ve tried Adcirca, Revatio and Cialis with no luck. You can find a lot about Pulmonary Hypertension at the Cleveland Clinic Website.
The good thing about taking Bosentan and basically the only reason I’ve stuck with it is one thing. Since taking the medication and having my first heart catherization six months later, the pressures have dropped. So, what is this Bosentan treating? My heart, my lungs or both? That’s what I am going to get clarified during my next doctor appointment. Where and what exactly is the happy middle ground? Are we remembering the quality of life?
My PH Story
While at the 2016 Pulmonary Hypertension International Conference in Dallas, TX on June 17 – 19, I met Steve Van Wormer, who helped create the PHAware Global Association. At the conference, he did interviews of PH patients to help get their PH Stories out to the public to raise awareness of this devastating and currently incurable disease. He asked me to do an interview. I tried my best to do the interview. He was SO gracious when my brain decided to blank out.. I explained that I write much better than I talk. The long term effect of high CO2 on my brain really has affected my memory and recall. Thank goodness for editing… and cut & paste. Hopefully, he can make something out of what I said.
For this reason, I want to get what I REALLY wanted to say here on my blog. Here we go!
My name is Christine Liles and I have Secondary Pulmonary Hypertension. I was born with PH due to Congenital Heart Defects and Scoliosis that caused Restrictive Lung Disease. The doctors knew almost right away that I had Pulmonary Hypertension. The pressures were really high and since I was born in 1969, there were no PH medications or a course of treatment for this very rare disease. So, I grew up living with this disease taking Lanoxin for a short time to help slow my heart rate down. I was restricted from most gym activity because of the shortness of breath.
My parents & sisters were great at providing me with as close to a normal life as possible. Before the age of 10 years old, if my sisters bowled and played baseball in leagues, I was right there with them. Granted, I was much slower but I did it even though the doctors kind of frowned upon it. At the age of 10, my parents finally talked my Cardiologist into performing a corrective surgery on my Ventrical Septal Defect in my heart. My Thoracic & Cardiac doctors, both, didn’t want to do it because they didn’t think I had a chance to make it off the table. It was my most glorious moment walking out of the hospital 7 days later with a patched VSD. With that successful surgery, my PH pressures reduced slightly.
Life really improved until I turned 17 years old. I started having blackouts while driving, began becoming forgetful, and my CO2 was much higher. So I started using oxygen at night. As time went by in my life, I started having more Shortness of Breath. My oxygen flow increased, I started using a bipap to sleep with and I had to stop working all together. This happened when I was 23, just a year after I got married to the love of my life. It was a very difficult adjustment. He knew it was coming.. this health change. I told him when we were dating and then before we married. This is the best I will be. I won’t get better because there are no medications to fix my PH and only one pill that can regulate my heart arrhythmia that I now have. I asked him… Can you handle this? His answer was total honesty which is what I wanted to hear but was hard to hear because he said.. I don’t know. And then, I went on oxygen 24/7. My husband has been my rock and he’s made from the finest quality of human beings… his parents.
In 2006, my Cardiologist asked me to check out the medicine Revatio & Cialis with my pulmonologist. So off I went to see my Pulmonologist who then sent me to see a PH Specialist. I didn’t even know there were PH Specialists. Over the years, after trying most of the PH medications that are available, which there aren’t nearly enough, there is only one medication that my body can tolerate. This is what I will stay on until something new comes along that my doctor thinks might work. I’m not a candidate for lung transplant because of my deformed ribs. I spend my life finding different ways to do the things I need to get done. Picking things off the floor are by using my toes to lift it to my hands. Vacuuming is my husband’s job now. I bend at the waist to wash my hair. I have an adapter for my van to use my bipap on the road for vacations while in the car if have trouble breathing. I do things in short bursts of energy. What takes my husband 2 hours to clean the whole house, takes me all week and even then I can’t get all of the really hard things done.. such as scrubbing the tub, vacuuming or mopping the floors.
This is my life now and I’ve learned to adapt which is the key to keeping myself happy. I’ve found hobbies that I can do that brings me GREAT joy. Just to be able to garden, my husband has built me a raised garden at the height of 16 inches to help me not have to bend down to the ground to grow vegetables. Instead of growing my dwarf fruit trees in the ground, he planted them in half whiskey barrels. I had trouble with dragging a long rubber watering hose, so I asked for a Pocket Hose because it is SOOOO light!
Will there be a cure in my lifetime? I don’t really know. In all honesty, I’m not so concerned for a cure for ME. I’ve had a GREAT life, filled with love and so many adventures. What I am concerned about are all the children who have Primary Pulmonary Hypertension. They need a cure in their lifetime. They have their whole life in front of them but with PH and without a cure, all they have are the medications that are currently available. My passion is for these kids. I know what it’s like to grow up with health problems. PH is no easy disease to live with. Help us. Be PHAware and get the message out that we need a cure. WE ARE DESPERATE TO BREATHE. Check out PHAware.global to see how you can become involved in finding a cure and spreading the word about Pulmonary Hypertension. Or follow on social media @phaware
UPDATE: I’m adding this update to let you know that I am back to taking Tracleer but at a lower dose (62.5mg). Trying to find the RIGHT medicine for my Pulmonary Hypertension has been difficult and frustrating. I also want to recommend going to the webpage Pulmonary Hypertension Association. You can find so much information about PH. PHA offers support and has a community based on fellow PH’ers. I highly recommend them especially if you’ve just been diagnosed with Pulmonary Hypertension.