I was diagnosed with PNH fifteen years ago, at the age of 51. This is my story.
I grew up on the West Side of Los Angeles. Raised by a single parent, my mother was a strong woman of faith. My father, the eldest of twelve, died of a heart attack when I was about nine months old. Four of his six brothers, and a couple of my cousins died at an early age. My mother always said, “those Harness men have bad blood.” Little did I know what she meant at the time.
Aside from the scholastic team sports growing up, I was an avid surfer, and loved skiing, mountain biking, and golf. Typical California kid, I guess. But surfing was my passion. I always thought of myself as a survivor and trusted in my ability to control my destiny. My health was a source of foolish pride, and the last thing I worried about.
I met Cindy at the age of 32, and we got married two years later. She has always been my best friend, and soul mate. I truly believe that our marriage was part of God’s plan and purpose in our lives. By late 2006, Cindy had been insisting I get a physical. She apparently had been noticing some bruising and lower energy levels, which I denied. So, in December of 2006, I had a physical performed by my primary care physician, and the labs came back less than stellar. With platelets at 25,000, and WBC and RBC below normal, I was referred to Cancer Care Associates in Redondo Beach, CA.
I was initially referred to the head physician of CCA, but he was out of town. Consequently, I was assigned to another doctor who spent about six weeks trying to diagnose this disease. After a bone marrow biopsy, and a lot of blood work, he thought I might have some form of hemolytic anemia, or worse Leukemia, and started treating me with B12 shots. I’ll never forget sitting in the chemo ward getting the first B12 shot in my right arm. The blood flowed like water through the bandage down my arm, and all over the sleeve of my white shirt. I went to my car and wept for probably the first time in my life. This was the start of the darkest time in my life, and a fiery trial that seemed very strange to me. One I had no control over.
By an unusual twist of events, the doctor I was assigned to at CCA had done his internship under a highly regarded hematologist at UCLA. He recommended I see his former mentor, and with a simple referral I had a foot in the door with one of the leading teaching physicians for hematology and medical oncology in the country. We had hope, and trust that God was answering prayers, just not in the way we expected.
By February 2007, all the major CBC counts were below normal. Platelets were 19,000, RBC was 3.0, and I had major hemolysis occurring. I was at high risk for blood clots, stroke and heart attack. UCLA ordered a second bone marrow biopsy and I was immediately diagnosed with Paroxysmal Nocturnal Hemoglobinuria, an orphan disease that apparently is not hereditary.
At last we could put a name to what was happening to me. Cindy contacted AA&MDS (as it was known then), and they not only sent her some very helpful literature immediately but reached out with the promise of support with information. Cindy has repeatedly said that this information allowed her to at least focus on getting the facts when she was at her lowest point.
Eculizumab was approved by the FDA in March of 2007. Though it was a highly anticipated drug for the treatment of PNH, it had not been widely used beyond the clinical trial phase at that time, and it was dreadfully expensive. For some reason, my doctor didn’t think I was a good candidate for ATG, so I was treated with steroids; i.e. prednisone, and cyclosporine. By March, my platelets hit a low of 16,000, but we stayed the course. I had some serious bruising all over, leg and abdominal pain and infected gums. I lost a lot of weight and fighting an immune deficiency all the while trying to keep up with the daily grind at work.
My doctor advised against all high impact sports, and airline travel due to the risk of blood clots and strokes. Surfing was out, but golf seemed like an acceptable pursuit. Business travel was another dilemma altogether. I had two platelet transfusions within the first six months of 2007 in order to get my platelets up to a level where I could fly. I will never forget the first transfusion on the day before the Super Bowl. It was done at a local hospital close to home. I was given Benadryl orally, and put on a slow drip. It took over one hour to complete the process while the bag of platelets hung there with the afternoon sun coming through the window. I flew to Arizona the next day for our annual key producer meeting, when I started feeling the effects of the first wave of an infection stemming from the blood transfusion. By Monday morning I was as sick as I had ever been in my life. To the very core of my being, it felt like death was at my door. It was at this moment where God was my refuge and strength, and only source of hope.
By June, my platelets were up to 31,000, and the doctor lowered the dose of prednisone, and prescribed Bactrim and leucovorin in addition to cyclosporine. By July, my platelets were over 50,000 for the first time! However, over the course of the next two years my lab results were up and down, and the meds were adjusted accordingly. By December of 2009, my platelets were 107,000 and I never looked back. In retrospect, I had taken prednisone daily for eighteen months, and cyclosporine for over five years. By May 2012, I stopped taking all prescription meds, and switched to over-the-counter folic acid, iron and a daily low dose aspirin. I still take this regimen today.
Going forward, I’m told this is chronic, and will live with this for the rest of my life. But I never let it define who I was. I still have some hemolysis occurring, but WBC, and RBC are in the normal range. Platelets range from 130,000 to 150,000, and all is good. Now retired, I’m still married to the love of my life, and we volunteer, travel and spend time with loved ones. I got back in the water after eight years, and at 66 I’m amazed how crowded the line-ups have gotten. My boards have gotten bigger, and the surfable wave are smaller but it’s still a thrill. Every day is a gift from God.
Hope is not something we can “talk ourselves into,” or get by looking at the bright side of things. This hope is not in the fact that God is going to fix every problem we face in the way that we want. He rarely does. The hope is that we know God is going to go through the difficult times with us and comfort, strengthen, guide and even carry us through perilous times. Our hope also comes from knowing that however God allows our circumstances, we can trust Him with them. If we ask for hope, He will give it to us. Hope is not in our circumstances, not in other people, not in things ultimately going our way, but hope is in our Heavenly Father whose unfailing love sustains us.
I learned to rejoice in the small victories, which gave me the ability to hope, and it was that hope that allowed me to trust God’s plan for my life. Confusion, heartache, and anger were natural responses. But regardless of my human emotions, there was something more to cling to. I chose to cling to the hope of God’s promises in the saving grace of Jesus Christ. For me, it has brought me closer to the glory of God and gave me a sense of profound peace. A peace that truly surpasses all understanding and guards my heart and mind in Christ Jesus. It’s indescribable.
Thank you, AA-MDS International Foundation for your support and research and letting me share my story.