November is Pulmonary Hypertension Awareness month. This year marks the ten-year anniversary of a national initiative to create awareness for what once was an obscure and terminal illness. Today millions of dollars are committed to the search for a cure!
Six years ago, my sister Renie, died at the age of forty-five due to the progression of this respiratory disease. She was an attractive, vibrant and upbeat young woman seemingly with her entire life ahead of her. Little did she know that on July 15, 1998, her doctor would diagnose her breathlessness and fatigue as Primary Pulmonary Hypertension, (PPH) and prescribed twenty-four hour oxygen therapy. Very little knowledge about PPH was available at that time. It is so rare and without a known cause, that statistically it occurs in about two persons per million each year.
However, the symptoms, shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells and fainting, results in PPH being frequently misdiagnosed. It often progresses to the late stages before discovery. The greatest numbers of PPH cases occur in women from ages 20 to 40; however, men and children can also develop this disease. There is no known cure for PPH so the goal of treatment is to control symptoms.
Renie was not a candidate for a complete heart and lung transplant that could have increased her life expectancy. Unfortunately, this procedure involves another set of obstacles in obtaining a donor. Therefore, her treatment involved large oxygen machines to use at home and the portable type for excursions. Around the clock oxygen therapy greatly diminished the quality of her life. Many everyday tasks such as bathing and preparing a meal left her gasping for her next breath, if somehow the oxygen flow became blocked. Trips to the grocery store, cleaners and even doctor visits were an ordeal if enough portable oxygen was not anticipated. (Being stuck in traffic could cause full-scale panic!)
Initially, Renie worked from home a couple of days a week. As the disease progressed, she reduced the number of days that she went into her office until gradually she worked from home full time. Doctor visits for my sister were numerous. Fortunately, she had the loving support of her mother, daughter, twin sister and other siblings to accompany her to these visits and other trips when needed. Even with the deadly prognosis before her, Renie was determined to enjoy her remaining time on earth. Her prayer was to see her daughter graduate from high school and settle into college. Always with a positive and upbeat attitude, she made it her mission to call her nieces and nephews with encouragement for their continued educational success.
Life was too valuable to her to waste with self-pity and depressive spells. It was her faith in GOD that helped her during lonely times when she was confined at home. Scripture promises in the Book of Psalms and gospel music were tools she used to buoy her spirits. She often quoted, “Yea, though I walk through the valley of the shadow of death, I will fear no evil; for You are with me; Your rod and Your staff, they comfort me.” (Psalm 23:4, KJV)
Diagnosed each year in the United States are an estimated 500 to 1,000 new cases of PPH. Additional treatments have been developed and approved over the last six years since my sister’s death. People are doing so much better, living longer and enjoying a higher quality of life. Today there are more opportunities for those with a PPH diagnosis to reach out to others. Supplementary information amassed and disseminated through the National Organization of Rare Diseases (NORD), about healthy diet choices, stress reduction, rest, and relaxation tips are all strategies made available for coping day to day with PPH.
Today there are numerous groups have formed for the Pulmonary Hypertension community to share info, relate personal experiences and seek prayer. There are an increased number of books, publications and websites now from those managing all of the challenges of this disease than were available during the last years of my sister’s life. Support groups like the Pulmonary Hypertension Association have mobilized to raise funds for research via concerts, conferences, golf tournaments, walk-a-thons and other social events.
Although Renie lived a little over three years after her diagnosis, today there are cases of those living twenty years and beyond. With the heightened involvement of the public to combat this disease, there is hope and encouragement for those living with PPH today!
