Breakthrough treatments for paroxysmal nocturnal hemoglobinuria (PNH) offer new hope and better quality of life for patients.
Though he loves to travel, Patrick Hanson usually limits his time away in case he catches a cold or needs a blood transfusion. Recently, however, he travelled to Europe for two weeks. The difference? Hanson is on a new treatment for paroxysmal nocturnal hemoglobinuria (PNH). PNH is a rare disease of the bone marrow that affects the blood and major organs. It can occur at any age, but is usually diagnosed in young adulthood.[1]
PNH occurs when a gene required for producing normal blood cells becomes mutated, creating a family of abnormal blood cells referred to as a PNH clone, which produces defective blood cells.[2] Red blood cell destruction results in lowered hemoglobin levels, otherwise called anemia, which reduces muscular strength and stamina. PNH causes fatigue, weakness, abnormally pale skin, shortness of breath, and an increased heart rate. People with PNH may also be prone to infections and abnormal blood clotting or hemorrhage.[3]
Managing a difficult diagnosis
Hanson had aplastic anemia for many years, which occurs when your bone marrow cannot make enough new blood cells for your body to work normally. Aplastic anemia is a risk factor for PNH. Though the symptoms of the two diseases are similar, Hanson started to notice some new symptoms, including dark-coloured urine. His wife, Nina, did some research and told him to ask his doctor about PNH. After some testing, Hanson was diagnosed with PNH in 2015.
It’s important to educate yourself and to advocate for yourself.
Hanson says with aplastic anemia, he was dependent on blood transfusions to increase his hemoglobin levels and was getting one as often as every week. Transfusions help relieve symptoms by providing blood cells that the bone marrow isn’t producing. Hanson’s PNH treatment helped better manage his hemoglobin levels, but they were still low and would drop every few weeks or months, when he would need another blood transfusion.
Reclaiming health and freedom
“I was always monitoring whether I would need a blood transfusion. If I didn’t have an infection or a cold, my levels would stay up for longer,” he says. “That was why I would only go away for six or seven days at the most, because I would worry that I’d get a cold or a fever, as those drop your blood counts. When I changed medication this year, it changed my life. In a few weeks, I had more energy and I saw colour in my face and hands.” When Hanson went for blood tests, his hemoglobin levels were in a normal range. “They hadn’t been for 20 years, so that was a miracle for me,” he says.
Hanson wants other patients to learn from his experience and find hope in it. He says rare conditions like PNH don’t get a lot of attention and may not be the first thing that comes to mind for doctors when diagnosing a patient.
“It’s important to educate yourself and to advocate for yourself,” Hanson says. “Learn about new approaches and the latest research and speak to your health care provider because it can change your life completely. There is hope.”
Learn more about other patient experiences with PNH.
[1] National Institutes of Health, Paroxysmal nocturnal hemoglobinuria
[2] Canadian Association of PNH patients, About PNH
[3] National Institutes of Health, Paroxysmal nocturnal hemoglobinuria
