When you look at me, what do you see?

               

What I see, is a normal healthy looking person that seems to be happy.  However; Looks can be deceiving. 

As PH (Pulmonary Hypertension) affects our lives, many people look at us and say: "You Don't Look Sick" But inside we are.  What is simple for so many is difficult for us. Let me ask you this question!

When you exercise, how do you feel afterward?   Are you sweaty, have a rapid heartbeat, a tight, heavy feeling in the chest, short winded and red in the face? Now, take all of those normal effects from exercise and imagine how you would feel having those effects when all you do is stand up from sitting down a few moments ago. How about walking up 3 stairs out of 15 to get to your bedroom or bathroom! Sometimes, for some, it is a constant and it never leaves them, making them feel like that just sitting or lying down in a resting state.  This is (what PH Patients call) a PH Day.

I am one of the lucky ones, and hide it very well, but many are not able to. They are on Oxygen 24/7 and they have portable pumps with IV’s that they tag along as their side kick.  A majority of the time you will see me like the pictures above. There are days that I look like the picture on the left, with what many mistake as “Sunburn”, but that is my medication face, pretty isn’t it!  This disease does not just affect our breathing; it affects our family life and our entire way of living. Too often we hear; Exercise more, eat healthier, what about herbal supplements? There are so many alternative medicines out there that can help you with your lungs, you just aren't willing to try them.  SERIOUSLY, that right there is the most obscene thing you could have just said to me, why? Clearly you are ignorant to the fact that Pulmonary Hypertension is NOT something that can be cured with a supplemental based, non-FDA approved medication and some of the supplemental herbs that you are referring to can actually cause my body more harm than good.  So let me explain to you what Pulmonary Hypertension is and how it affects my body.

Pulmonary Hypertension ~ The basics 101

It is a rare lung disease that causes the arteries in your lungs to become very narrow, filling with plaque, causing the pressures in the arteries of the lungs to double, triple and sometimes quadruple in measurements because there is not enough blood flow to the blood vessels. A normal range of pressure for any one person is 8-20 mmHG of pressure AT REST. Anything that is over 25 mmHg at rest is considered High pressure.  When that happens the right side of the heart starts to become enlarged and has to pump harder. Eventually, it would lead to death if not properly treated.

The FDA started to approve drugs in 1995 to help control and stabilize patients that were being diagnosed with this debilitating disease. As of today, there are a total of 14 drugs that help "slow" the progression of this disease, NOT CURE IT! 

Now that I explained a little about the disease, I would like to share how I deal with and manage this disease which lives inside me.

“I am the voice of my disease, Pulmonary Hypertension”

There are many that have no idea how they got the disease, which is called Idiopathic, no known cause.  There are some that have it due to underlying illnesses, such as Scleroderma, Sarcoidosis, Lupus, and Lyme’s Disease etc.  I got it because I inherited it.  Did I have it all my life then? Yes, I have the BMPR2 gene mutation which sat dormant until the age of 40, in other words, I am a genetic malfunction. *laughing* It resides within me, but I do not allow it to hinder my life.

People with PH talk a lot about “HOPE” and there are so many reasons to be hopeful. Earlier I stated there are 14 FDA approved drugs to treat PH. Without Research, we would still be without medicine. 

Imagine working 80 hours a week and being told at the day of diagnosis that you are no longer able to work. Devastating news to say the least, because I enjoyed working. I was a construction manager that entailed working within all climates of weather and lifting heavy materials when needed. To now having to wrap myself up in cold weather so my lungs don't feel like they are freezing when I breathe, to having to make sure I have some type of high SPF lotion on in the warm weather so that I do not burn. Pain in the neck, but again, not as bad as most.  

I am on 2 of those approved medications and they are helping me tremendously. 

When I was first diagnosed in 2012, my PA pressures were 108. That is really high, as I said 8-20 mmHg is normal range being at rest. So imagine being told 108 AT REST! I do semi-annual doctors visits which entails doing a 6 MWT.  The 6 MWT consists of walking for a full 6 minutes with a pulse ox monitor to determine how low your Oxygen level goes while exerting yourself, as well as your heart rate.  You are to try and walk as far as you can within those 6 minutes.  When first diagnosed, I couldn't even finish the full assessment leaving me at 12 laps equivalating my walk distance to be 256.5 meter/841 feet.

With these two medications and a lot of listening to my doctors and my body, this is what I have accomplished in the past five years dealing with this disease.

I am still pretty active. I watch my sodium and fluid intake, as many of us have an issue with edema caused by either Congestive heart failure which is caused by the PH, or the medications can cause us to get edema. Therefore; we have to watch how much fluid and sodium we are using throughout the day so we are not over loading our bodies with excess fluid.   I am on a 2000 mg sodium restriction with 68 fluid oz restrictions. 

During the past 5 years, I have lost a total of 74 pounds by watching what I eat and doing light exercise.  When I say light, I am referring to walking, doing light housework, and I dance while I cook and clean. The one thing my doctor told me at the onset of my therapy, "The more you work with your medication, the more your medication will work with you." Keep moving! If you sit dormant like a couch potato how do you expect the medicine to work if you aren't working with it?  So, I walk!  When I feel like I am over doing it or my body tells me I am tired, I stop and rest. When you learn to listen to your body, the more limits you know you can make for yourself for your stamina.  I usually do 20 minutes of work at a time, rest for 40 then go about my time all over again. If I need a nap, I take one. Sometimes it’s a 40 minute nap, sometimes it turns out to be 4 hours, depending on how my body is feeling.  

Keep your mind busy with other things. The less stress you have in life, the better your health will be.  I took up crocheting a while ago, and it really helps to calm my nerves.  I also, took up sewing and I so enjoy making things on my sewing machine.  The more you can focus outside of the disease, the better your mind and heart will be. Do relaxing things. Read a good book. I don't know how many books I have read over the last five years, oh who am I kidding, I am close to 900 since 2014, but it keeps me focused so I am not always thinking of my illness.   I do not allow my illness to define me; I define it, in more ways than one.

Get involved with advocating for the disease. Help spread the awareness.  If you aren't sure where to start, contact me, I'll point you in a few directions. 

Don't be a hermit!  Winter months, bundle up and just step outside for a few minutes just to get some air.   In the cooler temps of Spring, sit on the porch with a cup of tea and listen to the birds.  Summer, well we know how that works, heat/humidity swelling, not fun. But it gets cooler in the evening, go to a park close to you and sit on a bench and people watch or take a small stroll. Keep yourself motivated. 

Every year since 2014 we have held a 5K Run/Walk for awareness of Pulmonary Hypertension. Each year I give myself a goal to beat the previous years time.  I had my 6 month follow up the other day and during my 6 MWT I walked 1560 feet in 6 minutes, which is just a little over a 1/4 of a mile.  The entire 5K is 3.10 miles. My doctor told me he wants me in a Pulmonary Rehabilitation program to help me build endurance and strengthen my lungs. Am I going to do this, OH YES, why?  Because it will help me in the long run with breathing correctly and living a healthier life with an illness I didn't ask for.  It will also help me beat my record of 1:05:25!  I know I can do it, you know why?  I'll tell you why, DETERMINATION and PERSEVERANCE! 

                              

I may have Pulmonary Hypertension, but it certainly does not have me.  I am beating this disease, one step at a time, in my time, not its time.  We have no set limit when life will end, so why sit around and waste what precious time we have.  Take it for all it is worth and Live it to the fullest, we only have one life and its ours for the taking.