PHighter Friday: Jenny Janzer

My childhood was a dream, a dream that I woke up from on April 6^th 2001 as I held my parents hands, shaking in a doctors office with ringing in my ears- you know, the stuff of movies, just… not the kind you want to experience. I was only 11 years old when I was told I had Primary Pulmonary Hypertension (That’s what they called Idiopathic back then). I was stage 4 and pretty sick… the doctors told my parents there was a chance I could be dead within 6 months. This week I just surpassed my 15^th year of dominating PH. I never thought I’d make it this far, but I’ve got a fighting pulse within me that refuses to quit.  My name is Jennifer Janzer. The name Jennifer means, “white wave,” or “white shadow,” or sometimes, “the fair one.” There’s a reason I tell you this because for those first 11 magical dream years, I grew up on a lake and from the minute I could, I’d spend every day in the water. I embodied my name- white wave. My mom called my sister and I her little fish. We would snorkel, just lay in the water, rock hunt, swim out over the weeds to sandy spots or catch frogs and turtles while swimming amongst them. I played in the lake, I bathed in the mud, I was an earth-kid, claiming Mother Nature as one of my nurturing parents. April 6^th 2001 I was told I would not be able to swim ever again. It was probably the biggest blow they could have given me at that time. I also had to start on an IV pump right away, which would pump a drug called Flolan into my chest. I had no idea what this meant and went to sleep for the surgery thinking when I woke up, I’d be fixed, not attached to a machine that my life depended upon.

This change skyrocketed me into maturity. I no longer understood what my peers did, boys didn’t matter, the right shoes or clothes didn’t matter, making sure I had medicine with me 24/7 mattered, making sure I changed the ice packs on my Flolan every 4 hours mattered and keeping me isolated is what mattered. My doctors upon diagnosis, whether they meant to or not, instilled a fear within my parents and I that crippled us while I gritted my teeth through middle school. I’ve blocked out a lot. By high school I realized how ridiculous some of these rules were and fought against them like a little rebel. I wanted to be normal. I was in severe denial. I sobbed while watching my friends swim every summer, sometimes sitting on the pier, watching, listlessly chatting. I lost friends at school; people would point and whisper, “there goes the diseased girl.” People feared me- people didn’t understand me.
I was pulled from gym, but actually quite ecstatic about that as I had never been good at it. I was always last for the mile run, passing out and vomiting while we ran laps. Flolan was my lifesaver, but it left me miserable with side effects. I began to call myself the “blood leopard” because of the bright hot painful red rash the Flolan left all over my body, including my face. It’s this hideous splotchy white and red rash. I still have it today. I spent so many mornings as a teen crying and frustratingly trying to cover it up with layers of caked on make up and tears. I’d skip some days of school just because I hated how much I looked and felt sicker than anything. The pain drove deep into my bones and I became severely underweight. The side effects of this medicine make me miserable. I went through a depression and my anxiety grew to the point I became agoraphobic and couldn’t leave the house without having debilitating panic attacks.

At 18, I finally sought out counseling. I overcame that anxiety and so much more. I finally then, reached out to other PH patients, for the first time, breaking my shell of denial. My life began to brighten. I saw so many other PH patients going to school or working jobs. I had finished high school and decided to try college. I’d see doctors and when they’d find out I had PH they would say, “wow well good for you for going to college,” as if they knew I could never finish, but still felt like telling me, “hey, nice try.” It may have taken me seven years but I graduated with a Bachelor in English/ Creative Writing with a concentration in poetry. Writing has been my cure in many ways. 

If I wasn’t sick I’d love to be a geologist however, out in the field, digging up rocks, feeling the earth change beneath me, measuring its growth in sandstone dunes and river-carved valleys. PH took a lot but it also gave me a lot. I learned responsibility, I learned to laugh at myself, and I learned to love every miniscule moment. I know it sounds silly but I never find myself bored anymore… the world is bursting with possibility and even on the days I am restricted to my bed I’ve learned to value it and find ways to occupy myself if even just in my mind. I’ve always had a wild imagination. Any time I’m feeling good or even “okay”, it’s a good day. I no longer carry the denial or angst or hatred I did as a young teen. I grew to accept my rash- and found the best make up to conceal it on my face- (Kat Von D’s Tattoo cover-up make up has been my life saver, I so wish I had it through high school.) As of right now I’m on or have tried pretty much every medication for this disease. My story is so long that if I keep going much longer I’ll type you a novel. I dabbled in SubQ Remodulin for 2 years, but got sicker. However, in those two years I got so much freedom from the mixing and 24 hour watch of my pump that it was worth it. Flolan saved my life again as I went back on it at age 20. I also am on Opsumit, Warfarin, Digoxin, Lasix, Oxygen and my other least favorite- Adcirca; again for the bummer side effects it gives.

I also suffer from Ulcerative Colitis, Psoriasis, Asthma, and had Graves’s disease until I got that raging hormonal thyroid scraped out.  Life hasn’t been easy but it’s taught me to appreciate everything. It also taught me the value of people, true people that care. It’s rare to find a gem amongst all these grains of sand and the select support system I have I couldn’t be more grateful for. They make life worth living. I’ve found other things I love- music, for example. I don’t even care about what I look like anymore or who thinks what of me, I’ll drag my o2 tank right into a punk mosh pit, I’ll haul it around while I dance until I can’t breathe at folk punk shows and rock n roll festivals. I may look ridiculous, sometimes I get weird looks or rude comments but I feel strong. The more I smile, the better I feel. I also found a love in unsuspected places- abandoned buildings. Maybe I feel a connection with them but there is something exhilarating yet calming and just “right” about visiting them and photographing them. I can take the grimiest looking building and turn it into art with the right lighting and angles, or take a decayed peeling rusted post and showcase it’s abstract beauty, forcing people to look at things they would probably never think twice about. Forever, though, writing will be my special outlet to the constant pain PH produces and the constant fear, guilt, and anger it brings into my life.


Speaking of fear, since I was 11 and learned exactly what a lung transplant was, and had to listen to exactly what the surgery entailed, it has been my biggest life fear… and now it is my reality. My family was not ready for this; they have been my biggest support system. If it weren’t for them I wouldn’t be where I am right now. My mom gardens and when I was younger we had to come up for a new name for transplanting flowers because I couldn’t even hear the word “transplant” without having a panic attack. I’ve come a long way since that, but I’m still terrified, and I now need new lungs or I will most likely be dead in 1 to 2 years- or so my doctor said. Yeah, I’ve heard that before, DUDE, but I’m a WARRIOR and I don’t plan on letting this disease defeat me anytime soon. Life has given me a lot of lemons. And I don’t sugar coat it, no, I’m not about to make some lemonade. I eat that lemon, rind and all and spit the seeds out to the sky, grinning.

So, as much as this next step scares me, as much as I shake at night, and quietly sob through every morning, I will get through this, because like everything else I have to and I do. Looking back at what my name means, I now more so embrace the idea of the white shadow and the fair one… fair however, as in I keep a balance in my life. And white shadow as in, I will bring light to even the darkest corners I find myself in. SO! Bring it on, body! You may be trying to kill me, but I am not letting that happen anytime soon!

You can find me/my art and writing on these social media sites:

gofundme for my double lung transplant: www.gofundme.com/2bp6gthb
Instagram: @mostpulp
Tumblr: mostpulp.tumblr.com
Etsy: mostpulp.etsy.com

PHighter Friday: Kellie

Life beholds many unforgettable experiences.  One of those cherished moments occurred for me on February 1, 2007 when Paul completely surprised me with his marriage proposal.  I was ecstatic to say the least, and we quickly set to work on planning our August wedding.  In mid-February the wedding planning came to a brief halt as I dealt with a short bout of the flu.  Once I was feeling better I dove back into wedding planning mode! However, something was not right as I became more active.  I was unusually short of breath during my workouts.  After a few weeks of trying to "get back into shape" I decided it was time to schedule a doctor visit.  The results came back fine, so once again I decided to start the workouts and push even harder.  By May my breathing was continuing to get worse.  Paul convinced me to set up another doctor appointment.  By the time I set up this appointment I could barely walk from my car into work without having to stop and rest.  At this second visit my doctor decided it was time to look at my heart.  A few tests were done and an echocardiogram was scheduled for a couple days later. 

On Friday, May 25, 2007 I arrived for my echo a bit apprehensive, but hoping I would receive a simple explanation for my shortness of breath.  It wasn't long before I sensed something "wasn't right" during the echo, but the technician wasn't about to reveal what it was to me.  I will never forget the look on my doctor's face when he came in to break the news to me that my pulmonary pressures were extremely high, there was major concern about my heart, they were going to do a few more tests, and then I would probably have to be admitted to the hospital that day (which I was). 

After being admitted to the hospital the panic really set in.  As I was being wheeled away for a right-heart catheterization, I remember looking into Paul's eyes and both of our eyes immediately pooled with tears...tears of fear and the unknown. After the catheterization I was sick, chilled and scared.  Whatever I was given at that point knocked me out for the night.  The next eight days I slowly learned more about my disease and the aggressive treatment regime I would be starting to try halt this progressive illness.  It was decided that triple-therapy was in my best interest, so I began sub-q Remodulin, Tracleer and Revatio.

I spent the next three months focusing on healing and learning how to manage my medications so I would be ready to walk down the aisle.  It was a summer filled with much pain and suffering, but I was determined to move on and live life to the fullest.  By the grace of God, I had the most fabulous wedding day.  On August 11, 2007 I was able to marry my best friend and companion for life.  The challenges of the previous three months had only brought us closer together and made me all the more sure that God was taking care of me in a special way through the gift of my husband. 

One of the most difficult realities of my illness was the loss of being able to bear children. Although my heart ached, I was blessed with an adorable nephew and a beautiful niece, as well as a classroom full of first grade students to enrich my life. I was thankful to be alive and thankful to be back working full-time with children. I was gifted a notepad with the saying, “keep a dream in your pocket and faith in your heart.” This immediately became my motto, as it deeply resonated with the way I wanted to live my life. So I kept on dreaming and had faith in God’s plans for my life. I trusted that my life would continue to be filled with blessings, many greater than I could imagine.

In 2011, my husband and I felt fortunate with my improved and stable health condition and felt it was time to pursue adoption. Just as we finished the paperwork to be listed as a “waiting family,” PH was brought to the forefront in our lives when my mother was unexpectedly diagnosed with PH. That’s a whole other story in itself, but it was a magnificent reminder that anything is possible with God. We witnessed a true miracle in my mother’s recovery and are blessed to have her here with us today.

In 2012 the greatest blessing of our lives came into the world. My heart sings that we were chosen to be the parents of a beautiful baby girl. I will never forget the day she was born and the love that swelled inside of me as I took my first look at her. God had a plan, and it was even better than I could have imagined. My heart that once ached with loss is now bursting with love and I feel at peace. As I watch her play, learn and grow, I am reminded everyday how beautiful and precious life truly is. I am blessed to be a mom.

Since my diagnosis, I was determined to thrive, not just survive. I have taken an active role in doing my best to lead a healthy lifestyle that has allowed me to thrive. I immediately began a low-sodium diet. It was a difficult change in the beginning, but I could sure tell a difference. Prior to my diagnosis I was active in sports that my body no longer tolerates. I have since found new activities that I enjoy and are a benefit to my health. I enjoy yoga and mediation. I love going for walks and recently got a Fitbit so I can track my daily steps and activity. I have also delved into the world of Young Living Essential Oils and am so excited to have something that helps me as I manage the side effects of all my medications and has given me a boost in my overall feeling of wellness. 

I am thrilled to be involved in the study with the implantable pump for Remodulin. It is such a delight to swim and shower without worrying about an external line and pump. It’s amazing to think of all the advancements in therapy that have come about since my diagnosis 7 years ago and I trust that even better things are coming.

I am thankful for the continued support of wonderful family, friends and medical professionals. I am grateful for the Pulmonary Hypertension Association and all that has been accomplished thus far. I will continue to PHight and give my support until a cure is found!

Although the path can be bumpy at times, I am confident that God is with me every step of the way.  I am keeping a dream in my pocket and faith in my heart because anything is possible with God!

http://phight4wellness.wordpress.com/

PHighter Friday: Donna

 I had my son in April 1988. He was 4 weeks premature because I had pre eclampsia. He was perfect though. I was very lucky. When he was 3 months old I traveled with my parents to Hawaii to visit my brother and his baby girl. She was born 2 weeks exactly after my son. We were there for 2 weeks.  One day while we were there, my brother, his wife, and I decided to climb Diamond Head.  We were climbing and my sister in law was just bouncing up the volcano.  I was having a rough time and said, well, I can't blame it on just having a baby because she just had one too. Then we finally got to the top and there were about 30 stairs straight up and I looked up and said. What's wrong. I can't do this. But my brother was very patient and I finally got to the top and the view was GREAT.

Came back home, very difficult marriage.  Very difficult. I got sick and couldn't shake the cough so I was sent to a pulmonologist, he gave me inhalers to treat me for asthma.  That didn't work, 2 weeks later I went back and he added more inhalers and did a chest X-ray. He remarked about this big thing on top of my heart but he didn't know what it was. Took the X-ray to the radiologist at the local hospital. That dr said ah that's normal.

Later that year, I was going through a divorce and the doctors I worked for ( I was a nurse), thought my racing heart was from stress so they gave me something for stress. That didn't help my heart slow down so they thought I had Mitral valve prolapse. They gave me a Beta blocker. This was in November 1991. I started the Beta blockers, now no one checked me for anything. I passed out one day at work so they sent me for an echocardiogram.  That didn't show mitral valve prolapse so they sent me the next morning to a cardiologist. I was put on a treadmill and within 3 minutes my heart was racing to 180. They pulled me off immediately. The sweet doctor said "I can't be positive but I think I know what is wrong with you, but you need a right heart cath." Okay this is getting scary but I was young, healthy, free of my horrible ex husband, there couldn't be anything bad.  A couple days later I had my heart catherization, my mom and pastor was there. Well, the weirdest thing happened- my mom acted as if she had been crying and said she was going home to take care of my 3 year old, my dad had him.  Weird, because my mom had never left me in the hospital alone. That evening my best friend came and brought me a beautiful long, lacey, cotton night gown.  So sweet- but unnecessary, I was fine. Then my brother dropped by with a hairbrush, magazines, and chocolate.  That should have sent off warnings but it didn't. Ignorance is bliss at times

The next morning the nurses came and took me for a pulmonary function test. When I got back to my room, my mom was there and my doctor. Mom had tears in her eyes and Dr. Fonenot said down on my beside and said " you have primary pulmonary hypertension ". I said "okay, how do we fix it?"  He said "we don't." I said "okay." He said "it's not okay, you will die in the next 2 years without a heart lung transplant." I laughed and pulled the covers over my head. I looked out from the covers at my mom and she was crying, my doctor was crying.  Okay- this is real.  All I wanted to do was go home and hold my son tight.

The doctor called the next day and said he had found a center that would do my heart/lung transplant in Birmingham Alabama. Wow. That close? About a week after my diagnosis it hit me, I was dying and soon. I cried a couple hours, got down on my knees and asked God to take care of my baby boy.  We scheduled the appointment with UAB in Birmingham for December and  my mom, dad, baby boy and I went to meet Dr. Bourge, the transplant doctor.  He agreed with my local cardiologist and scheduled me for a transplant evaluation for the first full week of January 1992

January came and all arrangements were made for my hospital stay that would take a week. The transplant team had made mom reservations at a hotel close to the hospital and even made arrangements for the hospital day care to keep my son during the day so mom could be with me.  We arrived on Sunday and found the daycare so mom wouldn't have any trouble finding it the next morning.  We found it easily enough but pulled up to the door and they had a sign on the door that they had chicken pox.  We had no choice, we had to leave him there and get exposed.  I was admitted and went through so many test, was told I needed to loose about 10 pounds because I needed to be the size of a 12 year old boy. The tests were never hard but always invasive. They drew around 20 tubes of blood a day. More radioactive tests than I can remember. My veins were so sore and I was so tired and got sick from all the dyes they used. Then on Thursday, the chaplain came in to see me, I had already seen psychiatrists, but they brought in the chaplain, he was one of my old pastors.  I felt so blessed and that I was doing the right thing. On Friday morning I had another right heart catheterization  and the most wonderful thing happened. My pressures were half of what they had been in November.  The Procardia was working. Yay!  The next day we came home with a plan. Low fat, low sodium diet and walking daily. I stopped dying and started living. I dropped the 10 pounds then 10 more. I looked and felt great.

My parents and my son and I spent a week at the beach just tanning, playing, fishing. Enjoying each other and life.  I was working again as a nurse and doing great. The death sentence hung over us but I wouldn't let it dominate me. The next weekend after coming home from the beach, a really good friend asked if I wanted to go out dancing on Saturday night, sounded good.  Mom and dad were watching my son. Well, I was coming out of her hall and this man walked in the front door and we just stared at each other.  He was a cowboy, I was a tiny woman with a big smile. We both will tell you- it was honestly love at first sight.

We started dating immediately and he was wonderful with my son. We had been going out a couple of months and my mom told me that I needed to tell him about my PPH (pah), so one night we stayed up all night and I finally got up the nerve to tell him I was dying and he held me and said " I already know, Ruthie told me".  My friend. Haha well we dated a while, I had a right heart catherization every 3 months.  Since there were no medications for pulmonary hypertension we had to keep a close eye on my pressures. 3 days before Christmas, Milton, my cowboy, took my son to see a movie in Mobile.  That night after I got off work, Milton asked me to marry him. I asked if he was sure because at the time we thought I only had 1 year left to live.  He said he wanted to marry me even if he only had 1 week left with me. We were married in June 1993.

I continued to have heart catherizations every 3 months. In 1995 I had a punctured lung during one of my heart Caths and I spent a week in the hospital with a chest tube. Horrible pain. I was still holding okay on my PA pressures, they stayed in the upper 40's.

Life was good for a long time, except for all those heart Caths. Somewhere along the line I was put on Coumadin and then lasix. Decided I didn't want to find a different doctor. Dr. Bourge and I didn't really get along all that well. I am a people person, he wasn't. Great doctor just not for me. So I was sent to Nashville and saw a wonderful PH group at Vanderbilt.  I had a bubble study there and they were convinced I had a hole in my heart and they could fix my PH. Wow!  After all this time we had hope. They sent me downstairs to a pediatric cardiologist who showed us what he would use to repair the hole in my heart. So the next morning I had an 8 hour long heart cath looking for a hole in my heart. After all that we were back to my original diagnosis of idiopathic pulmonary hypertension.  I couldn't keep going to Nashville, it was just too far away. So I found a doctor in New Orleans. The closest one yet.  I liked him and I had started getting worse. Luckily Tracleer had just come out so he added that to my calcium channel blocker.  That started working and I was doing better. Then he got an offer he couldn't refuse from Houston so he moved and I was without a doctor. I asked his nurse about someone and she told me about a Dr. DeBoisblanc. Thank goodness, he goes by Dr. Ben. We met and it was a perfect doctor/patient match. He was all about my quality of life like I was.

I started getting sicker again and he had heard about trials of Viagra, so we talked my insurance company into getting me Viagra. They sent me 3 months worth of that golden medication. Haha joke. I started taking it and the headaches were horrible, they just got worse and I started vomiting daily. Talked to Dr Ben and he said to stop taking it.  He was trying to keep me off IV medicines. Then sub-q Remouldin came out and he said. You'll love this. No IV. So my local cardiologist worked with him to get me the medicine. I went in the hospital to get started and trained on it. Everything was good for 24 hours and I got to go home. At the 25 th hour the site pain started. Oh no this is awful. On the 3rd day it got better, then I had to move it and titrate up my dosage.  Here we we again. The worst pain ever.  Then it would get better, then I'd have to start over again. It got so bad I started vomiting all the time again. I only got out of bed to vomit. It was awful. So 4 months of that and Dr Ben looked at me and told me to stop that medicine. He said this is not quality of life. Well then, Ventavis came out. Big bulky pro-dose machine that had to be plugged it. Every 2 hours I had to inhale the Ventavis   No side effects and between Ventavis, Tracleer and Norvasc I was doing great. Then we took a direct hit from Hurricane Ivan and were without power for a week. We had to use an inverter in the car so I could do my Ventavis every 2 hours.  Finally the little portable inhaler for Ventavis came out and I was one of the first people to get it. That made life so much easier.  I stayed on these medications until 2007. I started getting worse again. So as soon as Letairis came out I switched from Tracleer to Letairis .  The Tyvaso came out and my local cardiologist told me it was only 4 times a day.  So I changed from Ventavis because I felt like it would be easier for me. I responded really well to Tyvaso and loved how easy it was to me. I have beed on Tyvaso, Letairis, and Norvasc since 2007.

This is the year 2014 and in November that will be 23 years since I was given 2 years to live. I still have my original organs and hope I always will. I have still avoided IV medications but now there are 3 different ones available if the need arises

I still live my life to the fullest. I get tired more but I am now 53. So is it my PH or is it my age?  Who cares. I am going to live to be an old lady. My son is 26, I've been married to my cowboy for 21 years. I have cows, horses, goats, chickens, a cat and a wonderful Boston Terrier that is my little boy. Haha






*Editor's note: you might recognize Donna's story from my previous post where I posted this video of Donna who shared her PH journey with a local news station (video below.)