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Our PHight with Pulmonary Hypertension
If you have read the About Me page on my blog then it is no secret that I personally am battling for my life against Pulmonary Hypertension (PH). This post is long however I hope you take a few minutes out of your day to hear my story. I often ignore that I am sick, most of my friends can attest to that. I rarely talk about the ugliness of PH because quite honestly, I can’t truly handle it on my own. So it’s not just my battle; it is my family’s battle. When I say it is ugly, I mean fugly! It steals everything from you, even things you didn’t know you needed. I have posted photos about my disease along with links about it throughout this post. Please take the time to see and hear about PH from not just me but from other sufferers and from the experts trying to find a cure for this disease.
Before I get into the personal details of it all, let me first explain why I am willing to put all this information out there to see. First off, I am a nurse. One of the greatest responsibilities a nurse has is to educate patients and their loved ones about disease processes, symptoms, treatment options including side effects and how to generally manage the disease. I am a very proud nurse; I worked hard to earn my degree having decided to pursue it in my later years. College is hard but really hard when you do it in your early 30s. Becoming a nurse was one of my greatest accomplishments in life (my marriage and children come way before it). So as a nurse, I feel it is my responsibility to educate as many folks as I can about PH. Secondly, November is Pulmonary Hypertension Awareness Month. So wear those periwinkle colored ribbons . . . yes, I know, crappy ribbon color that is also associated with eating disorders awareness. PH is so darn rare it doesn’t even get it’s own ribbon color! I personally think the ribbon should be in a zebra stripe pattern, after all, it is known as the “Zebra” disease. Lastly, I have a selfish motive; I need PH awareness to increase. With awareness comes financial support, with financial support comes research and with research comes treatment options. Maybe one day there can be a cure. So let’s start from the beginning, my beginning.
In my last clinical rotation in college, I was blessed with the opportunity to be on the telemetry unit at Fairfield Medical Center (FMC) in Lancaster, OH. I really liked the unit. It had a focus of course on heart disease, which was one of my favorite systems I studied. I thought it would be a great opportunity if I could work in the unit. I inquired and literally was hired before I even graduated and before I took the NCLEX. Thankfully, the NCLEX was a breeze (trust me, I studied hard for it) and started working at FMC in the middle of July 2005. Part of the hiring process was a TB test provided by Employee Health. In nursing school, TB tests were performed yearly in order to participate in the clinicals. My results were always negative. This time around though, the site itched and although I tried not to scratch at it, I did. So the site was inflamed when the employee health nurse read it. She ordered a chest x-ray, which is standard practice after a positive TB skin test. A chest x-ray can confirm or rule out TB. So off I went for the chest x-ray and scheduled an appointment with the Employee Health’s doctor so he could read the results.
I returned to Employee Health and the doctor informed me I did not have TB. Whew! I was glad to hear that. Then he said, there’s something wrong though with your pulmonary artery. He showed me the x-ray and informed me that it was grossly enlarged and I should follow up immediately with a cardiologist. Of course I am a new nurse and didn’t know if I was looking at my pulmonary artery or a big boot because that’s what it looks like on an x-ray. I just nodded my head and said, “ok”. I really didn’t give it much thought other than I would go ahead and follow up with a cardiologist. Since my insurance was through FMC, I had to pick a cardiologist affiliated with the hospital. Since I was a nurse on the telemetry unit, I basically knew every cardiologist because I at one point or another took orders from them. Although I had been on the telemetry unit for 3 months in clinicals and as a new RN, I still went ahead and asked some of my new nurse friends who they would suggest as a cardiologist. Hands down, Dr. Michael Meleca was always chosen. I scheduled an appointment with Dr. Meleca who recognized me as a nurse on the unit. He ordered a battery of tests and I distinctly remember him saying that he hopes there is a tiny hole in my heart because that could possibly be repaired fixing the PH however he was suspecting it could be PH itself. I remember quietly praying, “Please let me have a hole in my heart.” Who wishes that on a random day in July? LOL I had began reading up on PH and didn’t like what it said. I was really praying for a hole in my heart. I kept telling myself that they wouldn’t find anything and I just needed to lose weight and exercise more. I went through several tests within a one-month period. My last test was a right heart cath. Turned out I ended up with a huge hematoma at the insertion sight. I was off work while I was recovering from it all.
Steve and I met with Dr. Meleca a few days later. He informed us that it was in fact primary PH (meaning no cause, I just have the blasted crappy disease) and that it was a very serious disease. What stage? Stage 3. Just like that, our world turned upside down. I still am in awe in how I was diagnosed. It was purely by accident my PH was found on an x-ray. Remember, I’ve never had a positive TB test anytime in the past 5 years and I was tested yearly. I literally owe my extended life to the employee health doc who took the time to actually read my x-ray appropriately. PH is often misdiagnosed and diagnosed in the very late stages after someone is extremely symptomatic. As he was talking, I kind of tuned him out. How serious can it be? I didn’t even learn about it in nursing school? Hypertension? Blah, give me an anti-hypertensive. I’ll decrease my sodium intake and all should be ok. Then I came out of my daze when I heard Dr. Meleca say, “I can’t treat you. You need to see an expert and I am referring you to Dr. Curt Daniels with OSU.” Dr. Meleca was very sincere and sweet to us. But I also picked up the concerning tone of his voice. Maybe this is bad? Nah. Maybe?
We left and I went home. I did what everyone does when they want more information. I went to the internet. I read up on PH from personal testimonies, support websites and everywhere else I could think of on the web because everything you read online is 100% true (I am being sarcastic for those that don’t know me that well). The information was overwhelming. I was bug-eyed from all the internet searching and literally in tears. Turns out PH was serious. Without treatment, folks who are symptomatic and do not receive treatment usually die within 3 years. Even with treatment, many folks die around 5 years after diagnosis. Um, I was 33 years old and had an almost 9-year-old little boy who needed his momma, how can I have this? Why do I have to have it?! How the hell did I end up with a disease that only 1-2 in a million get and can’t win the damn lottery?! So I did what most folks do, I ignored the problem. I did nothing about it. I rarely talked about it and tried my darndest to wish it all away. I returned to work at the hospital but it was physically draining in the worst way. The transferring, stooping, standing and constant movement on the unit was too much for me to handle. I was angry and frustrated. I was however fortunate and fell into another RN position as a psych nurse in a crisis unit. Yes, at times it was physically demanding but a lot of time was spent documenting and honing in on my therapeutic communication skills. I didn’t necessarily have to lift, stoop or stand on my feet for an entire shift. Plus, community service was such an instrumental part of my life that working in a crisis unit serving mostly the needy was right up my alley. Was I symptomatic? Well, yes. However I had unconsciously compensated in different ways. In general, I moved slower. Not slow enough for anyone to notice but slow enough that once in a while, I could catch my breath.
For 6 months, I went on this way. I worked 9-hour shifts and would come home to sleep 12 hours from exhaustion. I barely saw my husband and son. I was either working or sleeping. I couldn’t balance home and work like most folks do because I was just plain tired, tired all the time. Dr. Meleca’s office was relentless. They called at least once a week to schedule a follow-up. I ignored the phone calls. But I’ll be honest; I was haunted by my diagnosis. When all was quiet, I laid awake shivering in fear. All I could think about was I have PH and I am going to die. It was my own personal hell. I didn’t even really talk about it with Steve and we really kept as much information away from our son as possible. I finally answered one of Dr. Meleca’s office calls and scheduled an appointment with Dr. Meleca in January of 2006. We met with Dr. Meleca and we had a very long conversation on the seriousness of this disease and that Dr. Curt Daniels can help me. Dr. Meleca stated he had worked with Dr. Daniels and he is the foremost expert in the area. His office scheduled an appointment with Dr. Daniels in February 2006.
In hindsight, some things make sense. For example, when I was in labor with Stevie, I blacked out. I was stuck in labor for too long and they were thinking about doing a c-sect. The lights were dim and Steve went to the restroom of the birthing suite to put on scrubs. Next thing I know, the lights are all on and my bed was surrounded by nurses and doctors. I was dazed and confused. They gave me valium and then I was happier than a fairy on a soft, purple mushroom. I was rushed into an OR and Stevie was delivered by c-sect. An EEG the following day showed nothing. Even as a child, I had 5 seizures. Every time, the EEG showed nothing. I was put on phenobarbitol and that was that. Maybe I was blacking out from a lack of oxygen? Who knows? I remember walking from one building to another on campus and having to stop at every bench on the way. My nursing classmates just laughed and took the many breaks I needed with me to get from one class to another. I was always tired. Super duper tired. I just figured it was the crazy schedule I was running, being an at-home mom with Stevie and trying to go to school full-time. Yep, the signs were all there. I just didn’t see them at the time.
Steve and I met Dr. Daniels and his nurse, Julie, at our first appointment at Ross Heart Hospital at The Ohio State University. He knew his stuff and Julie was amazing. Julie provided sound information while also being genuinely compassionate toward us. I can tell you from being a nurse that if your doctor is a jerk and egotistical, that is probably a good thing. You want your doctor to be overconfident; you want a doctor who believes they can “save” you. You want someone who thinks of himself or herself as a God to medicine. You really want a doctor who “knows it all” when you are fighting a horrible disease. You don’t want a huggy bear, what you really want is a superhero. However, if you can find a doctor that is all that and who also is genuinely compassionate, you have struck serious gold. That is what Dr. Meleca is and that is what Dr. Daniels is; they are both pure gold. Wow, I should clarify what I am saying, lol. Dr. Meleca & Dr. Daniels are top-notch; clearly experts in their respective fields. They are confident but not in a “jerk” kind of way because they take the time to be human toward you about a disease that just isn’t humane. They are absolutely amazing individuals. As an RN, I respect both of them greatly, but more importantly, as a patient I highly recommend and respect them as well. I think we hit it off easily because Dr. Daniels mentioned his mother was a psych nurse and I think Julie respected the fact that I too was an RN.
They were aggressive right from the get go. I was immediately put on medications to help handle the symptoms. This included diuretics, potassium, coumadin, digoxin and oxygen. Ironically, these are all drugs I knew inside and out because they are commonly prescribed for cardiac patients. I also was started on PH specific medications that included Ventavis (an inhaled medication), Revatio and Tracleer. Now here I was, a gal who was never compliant with birth control pills often resulting in multiple periods in one month who with a snap of a finger became someone who has to take 15-20 pills a day. Then the Ventavis. What a beast of a medication! I had to prepare the medication in the nebulizer; it took approximately 10 minutes to inhale the medication and then 15 minutes to clean up the nebulizer. I was expected to do this every couple of hours while awake. Do you know how fast 2 hours comes by? My work was very cooperative and allowed me the opportunity to take my inhaled medication in the med room. That was nice of them but realistically on a crisis unit, the opportunities to stop and take 15-minute breaks didn’t happen at regular intervals of time. I just couldn’t, no matter how hard I tried at the time, be compliant with Ventavis. In hindsight, I wish I had tried harder. I believe I didn’t try harder because I was still in denial about being sick. My next scheduled appointment with Dr. Daniels was in June 2006. From February to June, I declined dramatically. I could barely walk across the unit or my home. I was exhausted, I mean to the bone exhausted. I had chest pain and shortness of breath all the time, even at rest. I was often light headed and dizzy. My ankles became cankles swelling to elephant size. My fingers were always tingling. You know, when you sit on your leg and you sat for too long thus occluding blood flow and your foot fell “asleep”? That pins and needle feeling is what I am talking about. I was so short of breath that I would take a deep breath in and it felt like the wind I sucked in would slam into a brick wall with no where to go. I just couldn’t ever really catch my breath anymore. I was still exhausted sleeping considerably longer than most folks. I was miserable. In the back of my mind that “3-year” limit on life was slowly tearing at my soul.
We went to our follow-up appointment and it was evident in my 6-minute walk how poorly I was doing. Dr. Daniels said it was time to get even more aggressive. It was time for Remodulin. Remodulin is a potent vasodilator; basically it would open up my arteries. Oh I should explain the arteries part. Well, let me give you a short cardiovascular lesson. You breathe in oxygen. The pulmonary veins bring the oxygenated blood to the left side of your heart. Your heart pumps it out to feed that oxygenated blood first to the heart and then to the rest of your body. The deoxygenated blood returns to the right side of your heart. The right side of your heart pumps the blood into the huge pulmonary artery that breaks up into a gazillion tiny pulmonary arteries that cover both your lungs. Is it a gazillion? I don’t know, just think about a huge tree. You have the thick trunk (that comes off from the heart) that branches out to thick branches. Then those thick branches have smaller branches coming off of it. Then even more branches coming off the smaller branches. It just keeps branching out until the branches are super duper tiny. Those super duper tiny tree branch looking vessels are what covers both your lungs (they are still called pulmonary arteries). So the pulmonary artery is not just one artery, it is in essence the whole tree from the trunk to the branches to the tiny sticks of branches. So these tree branches (including the trunk) if you will, are progressively thickening. The diameter of the tube that blood flows through is shrinking and not shrinking uniformly. So this decreased blood flow increases the pressure in the pulmonary arteries. So the right side of the heart, pumps harder trying to force that blood up into those branches to get to the lungs. The valve between the right side of the heart and the pulmonary artery starts leaking. Since the right side of the heart is working so much harder, it begins to thicken and enlarge. It is after all a muscle; if you work a muscle then it gets bigger. The heart just wears out. The human body is an amazing thing. We have checks and balances. We have the ability to compensate when one part isn’t working. My heart is compensating but as with all things, you can only compensate for so much for so long. Cardiology is essentially plumbing. If a pipe gets clogged up, the pressure builds up and things back up. So the pressure has to be relieved. Some PH meds work at opening up the blood vessels while others try to slow down the thickening of the artery walls that is causing the vessels to “clog up”. You have to attack from every angle to decrease the progression of this disease. I had done my research about Remodulin. It is a potent vasolidator. So potent that it takes like a drop to help open up your arteries but the drop’s effect is short lived. It only can open up the arteries for a short period of time so you have to have a continuous slow flow of medication for it to keep working at opening up those arteries. I knew what he was saying. I would need a central line into my chest and the tip of that line would end just above the right side of my heart. The medication would then be slowly pushed right through my heart into the pulmonary arteries. A central line?! I have done my fair share of central line dressing changes and pushing medications through them, now I needed one permanently?! I bursted into tears sobbing hysterically. Dr. Daniels grabbed my hand and sat there quietly until the sobbing ended. He told me this was the best chance I had of prolonging my life. We scheduled the surgery for the central line insertion a couple weeks later. Steve and I left Dr. Daniels’ office and I cried for days on end.
I was born in the Philippines. I believe I swam before I could crawl. I love to swim. I love long showers and hot bubble baths. I love relaxing in a hot tub. I love water in the worst way. I knew that with a central line, all that would end and would end forever. How do I deal with that? Steve took me to Kalahari Waterpark resort the weekend before the surgery. I swam, and swam, and swam. I swam so much that Steve had to finally ask me to come out of the water. I remember distinctly floating in the pool, dunking myself under and holding my breath while I slowly sank down to the bottom of the pool. I literally tried to soak in all the water.
The central line was inserted without any problems. A special nurse came to the hospital to teach both Steve and I how to mix the medication, hook up the extension tubing and how to prime the line using a portable IV pump. We had to change the medication, the extension tubing and pump every 48 hours. I would need CVC dressing changes a few times a week at first and then once a week or as needed once the site was no longer draining. I needed supplies upon supplies: clean gloves, sterile gloves, medication, saline, 10 mL syringes, 20 mL syringes, extension tubings, caps, alcohol pads, dressing kits and tons more. I couldn’t handle all the details. I was still angry I had a central line. Steve listened; practiced and mastered dressing changes and changing my IV med. Dr. Daniels asked if I would consider not working anymore. Stop working? It was my escape! I needed to work; I needed to just be a nurse! I returned to work the very next week (crazy, I know). I just wanted to be a nurse and be “normal”. I pinned the tubing and the pump to my bra. My immediate supervisor and the medical director were aware of the central line, other than that, no one else knew. It was my dirty little secret that I was sick and had a central line. The side effects? Woah the side effects! Jaw pain, ankle pain, GI distress and migraines! Really horrible migraines! I’ve literally have had a migraine every minute of everyday since I was started on Remodulin. It never ends, only dulls to a tolerable level at times. I was hospitalized 3-4 times a year, always in ICU because of exacerbations of PH or complications related to PH. I was hospitalized for normally 1-2 weeks at a time. It was a constant battle to control my disease just enough for me to work. Often, my family suffered. I gave all that I had to work and then would come home and crash for hours on end, days even, from the exhaustion of pushing too hard. This will always be one of the biggest regrets in my life. I missed out on so much selfishly trying to pretend I wasn’t sick.
When I was in nursing school and learning about diseases, I would rate and compare the diseases. I would say things like I’d rather have this versus that. Well, one, I didn’t even learn about PH in college and two, I had no choice. I have PH. Do you wonder what it is like to have a central line and a pump attached to you 24/7? Let me tell you, it is my ball and chain. My leash if you will. I have to tuck my pump under my pillow when I go to sleep. When I roll over in the middle of the night, I inevitably wake up just enough to ensure my line isn’t tangled. This is on top of my pretty Darth Vader C-pap mask with another line that oxygen is fed right into. Yep 3 tubes at night, yippie me! When I get up in the morning, I grab my pump first and then my glasses. I have to take into consideration what I wear for the day. Does it have pockets where I can put my pump? Or do I have to wear the chain around my neck (yay, another leash I say sarcastically)? Vacations you ask? Well, I pack like I’m going on a very long extended holiday. I have to pack enough supplies for the time we are gone and then pack extra for any possible problems we will have. We have to leave room for oxygen tanks. Then Steve has to organize an oxygen condensing unit to be delivered to our hotel room. An open bottle of Remodulin has to be refrigerated. Does the hotel have a refridgerator in the room? How far is the nearest major hospital? I mentally have to prepare myself for metal detectors because I have to show them my pump and explain what it is. It is quite embarrassing. Then it’s the little things, like grocery shopping. I can’t just grab my purse and go shopping. I have to pick stores that have electric scooters or make sure my own electric chair or wheelchair is in the car. If I have to use my electric chair or wheelchair then I have to have someone with me to remove them from the car and reload them afterward. Let’s be honest though, can I really go out alone? I am always a bit scared to venture out. I park in a handicapped spot. I can’t lift anything I buy into the car (unless it is bags of toilet paper, lol). Then I worry about being a target. If you are going to rob someone, wouldn’t it make sense to knock off the chick with oxygen on and some type of tubing hanging out from under her shirt? Then other issues crept up which I am sure are somehow related to PH or the medications I am taking. I no longer absorb adequate amounts of iron. So now I have to go to the hospital once a month for a couple hours to get Iron IV. I have to have blood draws monthly to check and make sure my blood is thin enough. They also check my liver levels monthly because some of my PH meds are super toxic to my liver. I also take a crazy amount of potassium because that too is consistently low in my monthly blood tests and I’m not naturally absorbing it in my diet anymore. So it’s a bunch of things. PH is affecting all of me in ways I didn’t think it could.
Then there’s the other problem. I don’t have any cognitive impairments (not yet at least, lol). So my brain says, “yes, I am doing this today” but it doesn’t communicate this to my physically limited body. I am always trying to do something because I think I can and then I am emotionally crushed when the realization sinks in that I physically can’t do it. For example, every year I buy flowers thinking I am going to plant them in my garden. I get down there and dig one hole. I am so exhausted from digging that one hole that I can’t plant the flower. Then I am pissed. Then there’s my hubby who always quietly lets me do what I want but is there to catch me when I falter. So Steve has to finish what I started and yippie, now to go with my anger and frustration, I am consumed with guilt. I won’t even begin to talk about the economic hardships. PH is freaking expensive. Even after qualifying for financial assistance for my medications, the financial burden of PH is overwhelming. I would have to say other than anger, PH also brings out a lot of guilt. I am always, always feeling guilty for something. Sometimes it is just as simple as not being able to carry in trays for my son’s football team’s Friday night meals or not being able to do dishes after dinner. My son is a sophomore and I worry everyday about how I am going to walk across the football field for Senior’s Night even though it is 2 years away. I don’t want to use my electric chair. I want to walk holding my son’s hand proudly as he takes his final walk across the field. Am I going to be able to walk it? Update: I proudly walked our oldest son across the football field senior night! Definitely a life highlight. I always think I am going to make dinner. I start it, and after a few minutes I just can’t stand anymore. I have to sit because just standing in place is physically exhausting for me. But here comes Steve to finish again what I failed to. He never complains. He just does it but that doesn’t stop me from feeling overwhelmingly guilty all the time. Yep, PH sucks. It sucks big time in every way possible. I just couldn’t PHight this disease without my family and my friends, I just can’t.
I continued to work for five more years in various settings and positions. All the time I was sick and an IV pump hung off my bra. And I loved every moment of it! When I was at work, I wasn’t sick. I was actually taking care of those who were sick. In order to keep up the pace and charades though, I had to increase my Remodulin dose weekly. Dr. Daniels asked every time we saw him to please stop working. I kept telling him not yet. Although he was concerned, I believe he respected my decision. My Remodulin dose got all the way up to 256 ng/kg/min. Trust me, that is an insanely crazy high dose but that’s what I needed to keep working. Steve moved mountains taking care of me. He took care of ordering all the supplies and medications, preparing my IV pump, doing the dressing changes, filling up my pill case and making all the doctor appointments. Steve also did most of the cooking and between Steve and our oldest son; they took care of all the chores. Steve even showers me daily. Yes, with a central line you can shower. You have to use an Aquaguard (it is a dressing that goes over your site with adhesives around the edges and not in the middle so it doesn’t stick to your CVC dressing). The only problem is that I am a chick so my central line is inserted into basically a breast so when I have an Aquaguard on, I can’t move. Any subtle movement and the seal around the edges are broken and my CVC gets all wet necessitating a dressing change. My husband literally has and continues to move mountains to take care of me.
I slowly got over the embarrassment of being “handicapped.” Although I had a handicap placard from the beginning (Dr. Daniels insisted on this), it was hard for me to park in a handicapped spot. Because I hid signs of my disease (mainly my IV line and pump under my shirt), I was embarrassed. I felt like everyone was staring at me and whispering “there’s nothing wrong with that lazy woman”. So I would get out of the car when I parked in a handicapped spot and walk with a limp pretending my disability was somehow related to a deformity in my legs. I know, stupid, but people are that shallow and judgmental. Sometimes the sickest person you will ever know is sitting right next to you looking absolutely “normal.” Don’t judge, seriously, just don’t. I would struggle with a shopping cart and stop every 20 feet to rest. I always shopped at mega stores like Walmart and Meijers because you could always find a shelf or furniture to sit and rest on. Of course, I would pretend I was just “testing” out the furniture because again, my disease was my dirty little secret. It took a good two years before I finally used an electric scooter in the store.
I can walk about 20 feet before having to take a break. I can’t dance anymore, which sucks because I seriously could have been a Fly Girl or Solid Gold dancer back in the day (am I showing my age yet? LOL). I can’t sing a song and that is probably a good thing, I sucked at carrying a tune. I can’t lift more than 15 pounds. This is probably the worst limitation to deal with. I can’t tell you how painful it is to not be able to lift our baby Spencer up in my arms and give him a big hug. I can’t go up a set of stairs. When you walk up stairs, the pressures increase in your chest, it literally is painful when I try to climb stairs. I have to put my feet up for at least 1/2 the time I am awake otherwise I can easily challenge an elephant in ankle circumferences. I can’t lay flat on my back. I literally struggle to breathe when I am laying flat. I can’t pick up things from the floor. Stooping down, even when doing it slowly, makes me really dizzy. I can’t get stupid drunk, seriously, everyone should get stupid drunk every once in a while but I know that alcohol doesn’t mix well with most of my medications. I can’t do the obvious things like tubing down a snowy hill. I can’t ski anymore and trust me, I loved snow skiing. I already told you about the water bit. I can tell you that I actually dream about water. I dream about floating in the ocean, jumping against waves and sinking on purpose in a pool. What I wouldn’t do to experience that weightlessness again. I can’t light candles thanks to all the oxygen tanks in my house and trust me, I am pretty sure I could have owned stock in Party Lite or Yankee Candles in my younger years. I can’t stand for very long. I always have to have something near to sit down on. I weigh less now than I have for almost 15 years but I still wear the same size clothes. One of the million symptoms of PH is abdominal swelling. So I always look pregnant, always. I have to use the same path when I wear my oxygen. I have a condensing unit with a 50 foot cord. Yep, I can walk the length of our house (taking breaks of course) because our home is roughly 50 feet in length. BUT you have to backtrack in the same path because 50 feet of tubing can wrap around a gazillion things and there’s nothing fun about getting horse-collared in your nostrils by a twisted and shortened O2 tube. A family favorite past-time was bowling. Can’t do that either no matter how light the ball is. It just takes so much out of me to swing that ball down a lane. PH is known as the blue lips disease because folks in the advanced stages experience enough oxygen loss that they have bluish lips and fingertips. Currently, my heart is working double-time compensating for the increased pressure. It’s all just a matter of time.
What can I do right now? I can listen, truly listen when a friend is in need. I can cuddle up on the couch with Spencer and look at picture books with him. I am able to go to our oldest son’s extra-curricular activities and cheer him on (granted I am usually in my wheelchair or my electric chair). I can craft. I can still use my hands to make amazing and fun things. I can watch movies and love watching The Voice. Adam Levine could have been the father of my children (just kidding, my whole heart belongs to Steve) but the man is a cutie patootie, am I right?! I can volunteer at my son’s school. Granted, I am limited in what I can physically do and my volunteering can’t really be on a set schedule. I do have bad days frequently but I try really hard to stay active in our oldest son’s life. I can take pictures. That is something I have always loved to do. Now I can’t always get into that crazy position to capture the best picture but my pictures still turn out pretty good if you ask me. I can read and boy do I love to read. I can maintain a blog about family, crafts and cooking. No, I can’t post daily but I try to post fairly regularly. Lastly, I can love. I live life to the fullest. When you are diagnosed with a progressively debilitating disease with no cure, you just live life differently. Don’t get me wrong, I have always been a very passionate person who wore her heart on her sleeve. I have always told folks how I felt. I love so deeply that just thinking about those I love easily gets me teary-eyed. I have this dreaded, awful, death sentence of a disease hanging over my head and you know what? If God said I will take PH away but you will also lose your husband and your children . . . well then you best believe if suffering from PH means I get to be with my husband and children, bring it! Because they are my whole life. Give me all the pain you got. I will take it all just to have my family. All my suffering is worth it in the end because I am able to kiss my sons good night and good morning everyday. Also every night, I fall asleep in my husband’s arms and wake to his kisses every morning. Yep, my family makes it all worth it so I keep PHighting!
So what’s currently left for me? I have been referred for a double lung transplant at the Cleveland Clinic. I went through the initial telephone interview. The dude on the line said that Cleveland Clinic’s survival rate was slightly better than the national average because they “do more lung transplants.” I’m sure he thought that it would be comforting that they “did more” but in my mind I was thinking, “Um no thanks, not interested in the busiest drive thru for lung transplants.” I know, irrational thought. Doesn’t matter anyways, at this time I don’t qualify because if you didn’t know it, there is a weight requirement for transplants and my fluffy self is just over the required max weight for my height. That’s ok though. The survival rate nationally is around 45% 5 years after a lung transplant. The body ALWAYS eventually rejects the lungs. So for those who think a transplant is a cure, it is a temporary fix. A really big band-aid costing around $650,000 for such a low survival rate. I am not ready for a transplant. Yes, I know it is the last treatment option. Yes, I have reached the last treatment option but I am ok. Right now, I don’t have a statistic telling me my probability. I could die tomorrow, next year, 5 years from now or a couple dozen years from now. I like not knowing. The survival rate is way too low and I would spend everyday counting down to the 5 year mark. I would be consumed with time and I know I can’t live like that. I am just not ready for the double lung transplant. I am ok going on with the way things are right now. I’d rather deal with the side effects, the limitations and everything else for one more day with my husband and children. So for now, I am waiting on the transplant and praying a new treatment option for PH is found in the near future.
So my daily struggles became routine. We as a family were managing this dreaded disease called PH. Then the unthinkable happened, I became pregnant. I remember seeing the positive result on the pee stick and literally hitting the floor. I was crying and howling because I knew that this could kill me. I cried for hours on end nonstop to the point I was struggling to breathe. But my mind was made up; we were having this child come hell or high water. I know, you are thinking what the heck were we doing not taking precautions? Well, let’s talk about that. After Stevie was born, we waited two years to try to have another child. Nothing happened. After a year of failure we went to a fertility expert, 3 to be exact. We tried everything. I took all the pills I could to increase the likelihood of getting pregnant. We made dietary changes and tracked everything. We did this for 3 more years. In the end, we were told I could not get pregnant because my body wasn’t releasing eggs anymore or some mumbo jumbo like that. They pushed for an in vitro fertilization (which at the time would be approximately $10,000 with no guarantees). I’m not sure if it was the money or the “do your thing in a cup” part but Steve took my hand and said he wasn’t comfortable going that far and that maybe this is what God intended for us. Maybe we were to only be blessed with just one miracle? I agreed, if it wasn’t meant to be then it wasn’t meant to be. We were already blessed with one and we were so thankful for that. Then a couple years later came the PH diagnosis so the thought of having another child just faded away. Well, turns out all those fertility experts were a little wrong. Here I was pregnant 12.5 years after our only child was born! We called our family doctor and scheduled an appointment to confirm the pregnancy. Yep, I was pregnant. Dr. Barbara Wynkoop (again, another doctor who is made of pure gold) with a tear rolling down her cheek grabbed my hand and said, “I am going to pray for you.” Yes, it was every bit that serious and Steve and I knew it. Dr. Wynkoop has been our family physician for over 17 years. She treats Steve & I along with our kids. She is truly a patient advocate and if you are looking for the best in family medicine, Dr. Wynkoop is the one you are searching for. She is truly our family’s guardian angel. When one of us comes down with something, she is quick to check the rest of us and treats our whole family. She works hard to achieve optimum health for all of her patients…even patients as sick as me. We called Dr. Daniels to break the news to him. Dr. Daniels was quick to schedule a meeting. We met with Dr. Daniels and his nurse, Julie, and he informed us of all our options that included abortion. Dr. Daniels said my survival rate was 50% within the first 48-72 hours after delivery as my body tries to adjust to the dramatic fluid change. My heart can only take so much. I then asked him, “What is the baby’s survival rate?” He responded, “100%.” That is all we needed to hear to solidify our previous decision to have this baby. Dr. Daniels referred us to Dr. David Colombo also with Ohio State University. He specializes in high-risk obstetrics and worked closely with Dr. Daniels treating preggos with cardiac disorders. Another amazing physician! For the next few months, I soaked in every wonderful experience of pregnancy. Feeling the flutters and then the kicks. It is true, people glow when they are pregnant! Secretly though I was also preparing to die. I wrote my good-bye letters. I created a DVD to be played at my funeral. I wanted to do as much as possible to lessen Steve’s burdens once I was gone. I went into the hospital a month and one week early of our baby’s expected due date. They “optimized” my heart and prepared me for the planned c-section under general anesthesia. As much as I prepared to die, I really wasn’t ready. I just wanted to hold our baby. I kissed Steve last as they rolled me into the operating room at Ross Heart Hospital at The Ohio State University one month before our baby’s expected due date. Was this the last time I would kiss the love of my life? I remember crying quietly as they put the mask over my mouth and I quickly drifted off to sleep. I woke later that day to find out we had a healthy baby boy who was in NICU at the main hospital. Yes, our baby was born at Ross Heart Hospital completely healthy 4 weeks early! They took an OR normally used for bipasses and such and turned it into an L&D room. The Ohio State University Ross Heart Hospital is an amazing place with amazing staff members who make the impossible possible!
I am not going to lie, the first 48 hours was a complete blur. I was unconscious for most of it but the staff at Ross Heart Hospital stayed on top of everything! They worked diligently to get me back to my optimum. On the 3rd day I couldn’t take it anymore, I needed to see my baby. I begged Dr. Laxmi Mehta to please let me see my baby. Let me tell you, Dr. Mehta is another doctor I would highly recommend! She is head of the Women’s Cardiovascular Health program at The Ohio State University. She has it all: beauty, brains and the biggest heart. She looked at me and said that if my heart rate accelerated beyond an acceptable point that the baby would have to leave the room. I had been battling tachycardia ever since the baby was born. My heart rate was in the 120s even at rest. Trust me when I say I was deep breathing something fierce trying to consciously control my heart rate! The NICU brought our baby, Spencer, to my ICU room. My heart rate didn’t increase; actually, I think I stopped breathing. Here he was, the baby I was ready to die for laying in my arms grasping my finger. He was perfect, as perfect as our oldest son, Stevie Jr. I was released from the hospital a few days later. All in all, I was hospitalized 2 weeks. That is nothing short of a miracle for someone with my condition who shouldn’t be having a baby. This is all due to the excellent team care I received from Dr. Daniels, Dr. Mehta and Dr. Colombo. To say my delivery was a coordinated event is such an understatement. All these doctors who were going to do everything in their power to help me survive and hold my baby carefully planned every detail of my delivery out months in advance. I am forever grateful for Dr. Daniels, Dr. Mehta and Dr. Colombo for daring to go against all odds and support our decision to have this baby. It was nothing short of a miracle. The American Heart Association in Columbus, Ohio, has a Red Dress Campaign Luncheon every year. In 2010, they asked me to be one of the speakers at their luncheon to help increase awareness about women’s heart diseases. Below is the video they taped that was played before my live interview at the luncheon. It was in essence, my “coming out” moment. It was the first time I talked openly about having pulmonary hypertension.
The healing after Spencer was slow and hard. I have absolutely NO regrets about having him however there is also no denying that it physically took a toll on my body. I returned to work 3 months later and quickly dropped my hours. I also for the first time showed evidence of my illness to my coworkers. I wore my oxygen in the office. I physically didn’t have the strength to keep working the crazy hours I was working. My work was very supportive and agreed to cut my hours back drastically. After a year, I finally came to the point in my professional career where I was able to accept that I gave all that I could give to the nursing profession. It was time to slow down and preserve whatever is left of my body. This was not an easy decision. My last job was my favorite. I was the ADON for Lifesaver Health Care and worked with the most amazing nurses. My boss, the DON and owner of the company, Barb Green, was not just my boss but truly was and still is my friend. It was hard to walk away from that, like I said it was the best job in the world. Barb was sad to see me go however fully supported and understood why I was leaving. I am forever grateful that my last nursing job was at Lifesaver Health Care. I have to say too that if it wasn’t for the miracle of Spencer, I believe I would have worked myself right into my grave. The birth of Spencer pushed my body over the edge and I was able to objectively see how far I had progressed in my disease. Remember, up to this point, I was still in denial. I wasn’t sick at all, even if I was taking 20 pills a day, wore oxygen most hours of the day and had a central line going into my chest pumping medication 24/7. Nope, I wasn’t sick at all, lol.
I filed for disability. I had heard horror stories about folks being denied and it taking years to battle Social Security. I focused on this expected battle to come. I plotted and planned my appeal. I meticulously gathered all my medical data from every doctor who had treated me over the past 6-7 years. My paperwork was literally 6 inches thick. I was researching attorneys to hire who could file my appeals. Then the letter came, the letter from Social Security. Ha! I’ve got this, bring it on! Then I read it. I was APPROVED. Approved? Approved. Just like that. Approved. I bawled my eyes out. All I could think about was, “Even the physicians at Social Security think I am dying!” Approved. The months of depression were so painful. It was dark and daunting to just get out of bed. No longer did I have somewhere to be; no longer did someone need my help . . . no longer was I needed. I hear people all the time say stupid crap like “I wish I was on disability” . . . no you don’t! You don’t wish to be so sick that you feel you can’t contribute to society.
From a nurse’s perspective, yep we insert IV lines in the hospital and typically they are removed at discharge. Sometimes though, folks need continued antibiotic treatments and are sent home with PICC lines so medications can still be administered via IV. These lines are more secure but still temporary and easily removed. So folks go through usually one month of antibiotic treatment and then return to have their PICC lines removed. I have a single lumen Groshong; a more permanent line that has to be surgically removed; not just pulled out…well, if you catch your line on something and it pulls hard enough the line can dislodge so it is not exactly permanent but considerably harder to pull out. I’ve had a central line for almost 10 years now, TEN. I even had a central line throughout my pregnancy with Spencer (yep, Spencer got Remodulin too which just adds to the miracle of him being a healthy baby boy). So just how many years can someone walk around with a foreign object hanging out of their body? I don’t know the answer to that. I did meet someone at a support group who had a central line for 10 years receiving Flolan (a precurser IV med to Remodulin with both essentially having the same vasodilating effect). I desperately want to live long enough to play with my grandchildren and since Spencer is only 6 years old; that’s at least another 20 or so years. Can someone have a central line for over 25 years? Can my heart continue to compensate for another 25 years? These are questions that scare me because my scientific mind says “No way Jose”. But then my faith steps in. My mind says one thing and my heart says another. I have faith that I will be around as long as God allows it and I have to trust that he knows what is best. Because if I just listened to science, I shouldn’t have survived this long, I shouldn’t have worked as long as I did as a nurse and I certainly should not have had Spencer. Can you imagine life without Spencer? I can’t. Check out my picture below! Wonder what it looks like to have a central line in your chest? This is what the hubby sees everyday when he is showering me or helping me get dressed, my boob with a tube, lol! Seriously though, they need to keep researching and come up with something else just as potent as Remodulin or Flolan that doesn’t require IV tubes going into your chest. It really sucks and I don’t want this boob tube for the rest of my life. Boob tube sucks!
It took a lot of support from my family and friends to get through those dark days. My husband and sons are the loves of my life. Their support in EVERYTHING is my EVERYTHING! Steve took “in sickness and in health” to a whole new level. He is my rock and my anchor. I definitely couldn’t do this without him, seriously, I couldn’t. My friends tell me all the time my disease is no excuse. They accept me for who I am. I am so thankful for Cindy, Beth, Rhonda, Liliana, Angie, Wilma, Karla, Barb, Misty, Brittney and everyone else I am blessed to have as a friend. I got through the darkness of depression because of my friends. My friends all know how much I love them (because I tell them all the time) but I don’t believe they are aware that they brought me out of this darkness simply by holding the light of hope and friendship for me. I am just so blessed in so many different ways! Now I am doing the best that I can both physically and mentally. Yes, I am sick. I am openly admitting it to you. Finally almost 10 years later, I accept this fact! What I can also tell you is that I deserve to have better treatment options. I deserve to swim again. I deserve to survive. So educate yourself more about Pulmonary Hypertension HERE and HERE. Tell your friends about the disease. Heck, donate to the PHA website (they are funding some cutting edge research projects over there). Don’t do it for me, do it my husband and my kids. Do it for all the other husbands/wives and kids out there moving mountains to keep their loved ones around. They deserve better treatment options too. They deserve hope. Every PH patient deserves more hope. I can tell you this, I will continue to hope and pray. Thank you for taking the time to hear my story. It was told from the heart. I have good days and bad days . . . but the good days always outweigh the bad days. I am honestly thankful every time I wake up in the morning and I am still able to take in a deep breath. One more day . . . this I what I ask of God every night. Yes, every year I am adding more to the “I Can’t Do It Anymore” list as my disease progresses. But on the flip side, every year I am also adding more to the “Things I Am Grateful For” list. I hope you will PHight with me!