Living With PH: Jan Janus
What do you see when you look at me? What I see is a normal, healthy looking person who seems to be happy. However; Looks can be deceiving.
As PH 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 PH patients. Let me ask you this question:
When you exercise, how do you feel afterward? Are you sweaty, do you have a rapid heartbeat, a tight, heavy feeling in your chest, are you 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 three stairs out of 15 to get to your bedroom or bathroom? For some, it is constant and never leaves, making them feel that way 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 unable to. Some are on oxygen 24/7 and have portable pumps with IVs that tag along as their side kick.
This disease not only affects our breathing; it affects our family life, and entire way of living. Too often we hear, “You do not look sick,” or, “How serious can it be if you aren’t using oxygen,” or, “Exercise more, eat healthier, what about herbal supplements, or plant based diet,” or, “There are so many alternative medicines out there that can help you with your lungs; you just aren’t willing to try them.”
Those questions and statements can be offensive and frustrating to those who have PH. PH is not something that can be cured with a supplemental based, non-FDA approved medication, and some supplemental herbs can actually cause my body more harm than good. Although the plant based diet is a great way to help get healthy, it is not a sure cure for PH either, as there is no cure.
As for me not looking sick, you are correct, and that is why it is called an invisible disease. Often times the person doesn’t look sick, but they are. Do I tell you, you don’t look like you have diabetes so you really don’t have it? Let me explain to you what PH is and how it affects my body.
Pulmonary Hypertension: The Basics
PH is a rare lung disease that causes the arteries in your lungs to become very narrow, 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 can lead to death if not properly treated. The FDA started to approve drugs in 1995 to help control and stabilize patients who were being diagnosed with this debilitating disease. As of today, there are 14 drugs that help slow the progression of this disease, but not cure it.
There are many who don’t know how they got the disease, which is defined as idiopathic, no known cause. There are some that have it due to underlying illnesses, such as Scleroderma, Sarcoidosis, Lupus, and Lyme’s Disease. 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. It resides within me, but I do not allow it to hinder my life.
Now that I explained a little about the disease, I would like to share how I deal with and manage it.
Pulmonary Hypertension: Management
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. I am on two 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 the normal range being at rest. So imagine being told 108 while at rest, with no activity. That is extremely high.
I go to semi-annual doctor appointments, which entail completing a six minute walk test. The six MWT consists of walking for a full six minutes with a pulse oxygen monitor to determine how low your oxygen level goes while exerting yourself, as well as monitor your heart rate. You are to try and walk as far as you can within those six minutes. When first diagnosed, I couldn’t even finish the full assessment, leaving me at 12 laps which is a distance of 256.5 meters or 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 overloading our bodies with excess fluid. I myself am on a 2000 mg sodium restriction with 68 fluid oz restrictions.
During the past five 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! I walk, I ride a stationary bike, and I play Wii Fit. 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 can make for yourself. 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 four 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. The heat and humidity cause swelling, which is not fun, but, it gets cooler in the evening. Go to a park close to you and sit on a bench; people watch or take a small stroll.
Keep yourself motivated. Every year the community holds a 5K Run/Walk for Team PHenomenal Hope to raise awareness and funds to fight PH. Each year I give myself a goal to beat the previous year’s time. I had my six month follow up recently and during my six MWT I walked 25 laps, which is a distance of 475.0 meters, or 1560 feet (just a little over a 1/4 of a mile). My last echo, which was in August, showed my estimated pressures at around 13. Before you all tell me that is an estimation, yes, but my echo’s always match my RHC, so I am happy with the estimation.
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, yes! Why? Because it will help me in the long run with breathing correctly and living a healthier life. It will also help me beat my record of completing the PHenomenal Hope 5K in 1:05:25! I know I can do it through determination and perseverance.
I may have PH, 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 idea when life will end, so why sit around and waste what precious time. Take it for all for its worth and live it to the fullest; we only have one life and it’s ours for the taking.
