PHighter Friday: Kimberlee

I was diagnosed in 2009 with idiopathic pulmonary hypertension at the age of 30. I believe I started experiencing symptoms six months to a year prior: extreme shortness of breath, syncope, chest pain, dizziness, fatigue, and cough. So, I scheduled an appointment with my primary care physician who then referred me to a pulmonologist. The specialist did electrocardiograms and pulmonary function tests, and concluded that I had exercise-induced asthma.

I knew it had to be something else because my mother is a severe asthmatic and commented, “I do not experience what you do on my worst days.” I continued to seek medical attention from hospitals and physicians, but was always told it was asthma, so use inhalers and lose weight - later it was noted the weight gain was from fluid retention. I was also told that I was fine, and what I was experiencing was all in my mind. And, finally, I was told that I must be using illegal drugs because of the symptoms (I never used illegal drugs or smoked.). I thought I was going crazy because I knew I was sick, but the medical community was of no help to me — I was dumbfounded. I continued to pray and hope whatever was wrong would be revealed soon.

Each day I went to work, I had a rough time with my patients because I could not perform my duties due to my limited mobility and declining health.  On one particular day I was very symptomatic and briefly fainted, so my best friend who was also working and another nurse took my vital signs, which were normal with the exception of my oxygen saturation and heart rate. I determined I had to go home because I was very weak, so we immediately notified the Charge Nurse, and my friend took me to my car; she offered to take me home, but I insisted I could do it.

I drove to my parents’ house because I knew I could not make it up the stairs to my apartment and I wanted my mom. As soon as I arrived I immediately told my parents what happened, so they called 911. I was taken to the emergency room by ambulance and tests were periodically done, but doctors did not believe I had a serious condition, and the hospital wanted to release me. My mother immediately spoke on my behalf for them to transfer me to the hospital of her choosing and they complied with my mother’s demand. Upon arrival I became unresponsive and tests were urgently performed, including various x-rays, echocardiogram and right heart catherization, which lead to the diagnosis of pulmonary hypertension by two physicians who are also pulmonary hypertension specialists in separate facilities.

My family was told that if the first hospital had released me, my chances of survival were low because I was at stage 4 pulmonary hypertension. Phenomenally, I survived, and once I became responsive, I was told the news that ultimately changed my life forever. “You have pulmonary hypertension.”  I was clueless on what this meant for my future.

I am now living a “new normal.” I currently am on combination therapy: treprostinil (continuously through a central venous catheter and a portable infusion pump), ambrisentan, riociguat, and calcium channel blocker. I also use several other medications to help ease the daily side effects of the PH medications. I used supplemental oxygen 24/7 at the beginning; then after doing pulmonary rehabilitation for three months, I was able to switch to during exertion or as needed. After two more separate admissions to the pulmonary rehabilitation program, I am fortunate that my activity tolerance is increasing.

Most importantly, I am determined not to allow PH to stop me from pursing my educational goal.  If I give PH the power, then I lose respect for myself. I must be my own advocate and cheerleader.

Also, I lead four support groups because I believe no one should face this journey alone. In addition, I participate in PHA events: I have spoken at PHA’s Congressional Luncheon and two of PHA’s International PH Conferences. I also advocate for those with medical disabilities, speak at community events and at my church.

Additionally, I am a member of the Social Media Advisory Board for PHA, and part of PHA’s Generation Hope, and a mentor for United Therapeutics’ PH peer network program. I am also the co-founder of a very active patient-led PH group on Facebook, PH Family, a growing global forum since 2010 for patients and caregivers to voice questions and concerns about this life threatening disease and to support each other.    

Pulmonary hypertension has taught me to live life; I am more appreciative of my quality of life than the quantity. I am blessed to have loving parents because without them and my faith, I would not be able to overcome the daily challenges I face. This is my journey and I must be a testimony, so that others will see that there is life after being diagnosed with a chronic illness.

As a PH community we must stand together and be “empowered by hope.” Every day I want there to be a medical cure for this rare disease, but until there is, I must cherish my moments with my family, friends, and church family because they are a wonderful support system. Pulmonary hypertension has changed my life drastically, but I am determined to ensure it does not negatively impact my life entirely.  I know what is important to me; therefore, I will not allow PH to have power over my destiny.

My next goal is to return to the working world and have the most important titles: “Mrs.” and “Mommy.” My journey is not yet complete — to be continued!

My contact: livingwithph@gmail.com

PHighter Friday: Janeris

 Pulmonary Hypertension is a disease I had never heard of until it almost killed me. I've suffered with Lupus since 1997. I was 16, a senior in high school, and began having severe joint pains and fevers. I've dealt with the countless effects of that deadly disease for so long, I didn't look too much into it when I started having shortness of breath in 2006. I figured the fatigue, shortness of breath, and rapid heartbeat were just part of it. I went to a couple of doctors who just confirmed what I thought. It was the Lupus. They'd tell me to take more Prednisone for the inflammation and I did. The shortness of breath kept getting worse. I couldn't bend over without getting dizzy. The doctors I saw would say my heart was working pretty hard but for me this was all normal. I went on living; suffering.

I met my now husband in 2008 and our relationship quickly moved toward marriage. I warned him that I had a lot of issues and might not be able to have a baby. He said that he didn't care. He said God would make everything ok.  He was very supportive through it all.  I still worked out every week and was very active in church. My lack of health couldn't stop me.  We got married less than a year after meeting. It was just in time for my biggest supporter to get the test of his life. We chose to get married in Mexico. It was 4 wonderful days spent with family and friends. We stayed 2 more weeks for our honeymoon. For someone with breathing problems, Mexico is not the place to be. It seemed much harder to breathe in Mexico than in Miami. We still did everything we set out to do. We went zip lining, swimming with dolphins, walking in the city and more. Most of it, however was done with my new husband carrying me. Everyone thought it was so cute because we were honeymooners but I was in severe chest pain and honestly just couldn't walk. The last 2 days of our honeymoon were the worst days of the trip. I began hallucinating and having severe chest pain. I thought of calling 911 but was afraid I'd be stuck in Mexico longer than I wanted. I sucked it up and relaxed until we went home. We requested a wheelchair at the airport but still didn't think about asking for oxygen. I didn't know what was wrong. The air in the plane was much thinner and I thought I was going to die.

We went from the airport in Miami straight to the hospital where I stayed for 3 weeks. I just wanted to go home to be with my husband for the first time. My oxygen levels were at 50%. I was immediately put on oxygen. This is where I started to hear that I had problems with my lungs and might need a lung transplant. Once I left the hospital I sought out a pulmonary specialist. I found a great one in Cleveland Clinic who knew exactly what to do. I was quickly taken in for a right heart cath among other important tests. I was finally diagnosed with Pulmonary Hypertension and put on the right medication. I survived on Revatio for a while. But having to take it 3 times a day proved too much for me and within time I had worsened my condition by not taking the medicine properly. In 2011 I started passing out often. I once passed out at a doctor's appointment and was taken to the emergency room. That was the beginning of my new life. There I was put on Veletri, an intravenous drug. It was a tremendous loss of independence and control. It was a severe shock for me.

I was so sad and had a hard time picking myself up. Every night was hard for me. Tears would soak my pillow.  I didn't understand my new body changes. There was constant urinating, swollen ankles, hot spells, weakness, jaw pain, headaches, you name it, I had it. How would I ever get through this? I felt my life was over.  My church family came through to make sure I did get through it. They knew I couldn't cook, clean, or barely move. So for weeks they came and took me to doctor's appointments, cooked, and were right there for me. What a team God placed in my life. I am loved! Another thing that got me through it was something that my Veletri nurse told me. She said I was allowed 5 minutes daily to feel sorry for myself. After that I was to enjoy the rest of my day. Eventually I didn't need those 5 minutes. That was an important part of the early stages of my recovery.

I began to see that light at the end of the tunnel again. I knew there was a purpose, I just had to find it. Time passed and I was tired of being tired. I had gained 30 lbs. from the prednisone and lack of activity and was still having a hard time breathing. But after 3 months it was time to get back to work as a full-time photojournalist at a local newspaper. Slowly, I regained my strength and started looking for alternatives. I found a functional medicine doctor in my area and she taught me the importance of the right diet for my body. My joy came from documenting my journey on a blog  called My Clean Cutting Board. It dealt with showing people what I was going through and how I was improving my health through whole foods. What a difference eating right made. I took out gluten, dairy, potatoes, eggs, coffee, alcohol, and a few other foods that were causing inflammation and a negative immune system response. I started to get better faster than I thought I ever would. I started the gym again full force. I started working at my photo studio, which I run on the side of my full-time job at the paper. I started singing in church again. And less than  1 year later my husband and I began talking about adoption. He saw my progress and together we decided that we were ready to start a family.

Soon the good food changed my body completely. I was taken off the prednisone and the doses of some of the other drugs I was on were lowered. I was so happy. Just 2 years after I was put on Veletri, we became foster parents. Doctors said I was in good health and gave me clearance for this huge task. We got a 2 year old boy in May of 2013. I realized then that all I had gone through was for this moment. You see, he is autistic and was a handful. He cried when he heard loud noises. Not out of fear but out of pain. I couldn't control him or anything he went through. He was a little Tasmanian Devil.  Through my diet change I had learned about the benefits of a gluten and dairy-free diet on children with autism. I cried tears of joy when I realized all my struggles meant something. I changed his diet and he got better. As I was taking care of him God was taking care of me. I continued to get better and they called me to take in a little baby girl. She was 3 months old. I didn't know how I would do this. Here I am with two babies within a year,  health issues, and 2 full-time jobs? Was I nuts? I knew God was in control. He had a plan for me and He wouldn't let me down.

My husband and I have been taking care of our babies for almost 2 years now. We are close to adopting one of them. I have since lost 40 lbs. (They really get you moving don't they?) My husband has been the best thing that could have happened to me. He came at the perfect time. God has a plan for me and I didn't let PH keep me from fulfilling that plan. I could have been in bed feeling sorry for myself. That would have been easy. But I said NO! I got up and chose to change my life. I chose to seek alternatives because I refused to believe that I would need a lung transplant, that I was sick, that I would not survive. I will not be held down. God needed me for something amazing and I didn't want to let Him down. I am beating PH!



My Clean Cutting Board

PHighter Friday: Carole

Like most people with PH my journey began without my noticing it and it gradually built until my old life was no more, a new one was to begin.

After going for a walk with my husband I noticed that my feet did not want to climb the steps, they kept hitting the edges, it was just too hard to pull them up all the way to the top.  I began to find it difficult to walk up the incline to our house.  I felt dizzy and sick and my legs were heavy.

One day after walking through the woods it just got too hard walk the the incline back to my house.  Time for specialist advice.  I was fortunate enough to meet with a doctor who had heard of, though not seen, PH.  He advised my husband to take me home, back to the UK, we were living in Spain at that time. He said I had maybe six weeks to live!!!!

The despair hit me like a brick wall, and, of course, we Googled PH.  Not a good plan at all.  On the journey back home I cried because I could not dress myself!,  looking back I can't believe the despair and hopelessness that hit me.

I was fortunate enough to be met at the Royal Hallamshire  by my A Team, my angels.  I was told not to get out of bed, they feared a heart attack as the right side of my heart was five times the norm.  I was told I had many tests to get through but would be pushed to them all. I must not walk! I was immediately given a cannula in my hand and attached to a drip pumping  iloprost into my body at two minute intervals.  Then the tears began, too many to mention but I remember the professor sitting at the side of my bed and telling me I would need a Hickman line inserted into my body that would enable me to get out of bed and carry on with life as best I could.

Line inserted I went home after five weeks to begin a new phase of my life.  It was hard, the things I could do before I could not now do.  My husband  took over the roll of housekeeper,  he also took over the roll of nurse.  He changed my Hickman pump daily and made sure it was kept clean.  My fingers didn't seem to want to work any more and my brain had turned to mush! I remember one day thinking I would vac the house before my husband came back from the shops and the despair I felt when I realized I could not even push the vac around.  Then came a year of adjustment and yes, I will admit of self pity.  Why me, what life did I now have!  It was hard, so hard but after a year I was given more tests after one of my six weekly visits to my hospital and how can I ever forget the jubilation that I was to see that day.

My bed was crowded around with all my PH team. Beams were to be seen by all.  My specialist doctor was all but jumping with excitement when I was told that the iloprost had worked!  My heart was how the size of a normal persons and my right heart catheter showed it was now 38!  Now a new journey began, one without the Hickman line and one with oral medicines, ambrisenten and sildenifil.

It took three months for the change over to be completed and for my new life to begin.  Slowly my body adjusted to the new medication and slowly I began to climb out of the pit of despair I had been in.  Small steps, baby steps at first.  I was scared but began a regime of walking further each day, always with someone at my side as the fear of dizziness of  collapsing was with me but I persevered,  gradually I began to feel stronger, was able to do more, was able to help in the house, able to shop on my own, able to be my own person again.

Recently I walked my personal best on my six minute walk test of  720 metres.  Some days I can walk eight miles, though in fairness five is the norm, some days I can't do much, hence the roller coaster ride we are on.  At last I had joined the people who were responding, who were living with ph and not letting it beat them.

I remember at the beginning of this PH journey looking out of the window and seeing people walking, just walking and giving it no thought.  How envious I was of them and thought it would never again be me, now though it is.  I am not saying I don't get bad days, we all do but the good far outweigh the bad.  My mobility scooter has not been used very much in the last two years, it sits in the garage and sometimes, though rarely now it gets used.  Most times my legs do the walking!

My advice would be to push yourself a little more each day.  Don't set out to do a marathon, take small steps and find what makes you comfortable.  Listen to your body, if it says slow down then do it, if it says " I can do a little more" then do a little more.

Remember PH medications are changing all the time, research is going strong.  There will be a cure  for this and I believe it will be in my lifetime.  I trial anything I am asked to trial.  I want to be part of the team that finds this cure and beats it!

Stay strong in heart and spirit.  When the bad days hit remember it's a roller coaster, the good days will follow.  Remember we are a PH family and we have friends here who can relate to everything we are going through,  my journey began in a wheelchair but I have fought to use my body again.  Fight, fight , fight and keep a good spirit within  you, believe in yourself.  Most of all remember you are not alone, there are people here who KNOW exactly how you are feeling. 

You walk with friends. Xxxxxx