This story is true and much of it is documented. It is the actual experience I have had during the last year or so with my wide array of medical doctors in New Mexico.
In October 2003 my PCP said that he heard a sound in my chest that might be an indication of Atrial Fibrillation (AF). He referred me to a cardiologist, who examined me, said he heard a murmur and prescribed an Echocardiogram. He also prescribed the drug Warfarin that is a blood thinner used to treat the effects of AF.
The results of the Echo showed a mean Pulmonary Artery Pressure (PAP) of 70 mmhg. At a meeting, I was told I had Severe Primary Pulmonary Hypertension (PPH), at that it was fatal. I had not heard of this before. I was scheduled for a Right Heart Catheterization (RHC) and referred to a pulmonary specialist (PS) for lung tests. The PS scheduled me for 2 MRI’s, a chest x-ray, a sleep study, and a breathing test in his office. The 2 MRI’s were the same but to be done at different places as he didn’t trust results sometimes. I passed the breath test as normal. I asked him how bad a PAP of 70 was and he said that I asked too many questions. All other tests were negative except sleep study.
Then the RHC. It showed a mean PAP of 33. It also stopped me from urinating. I ended up going to the emergency room that night a having a catheter installed. They told me to have my PCP remove it in a few days. The PCP said he didn’t do that. I had to hire a new PCP and he removed it but I still couldn’t pee, and when I strained, my nose would bleed. I thought of Elvis. I had to go back and get another catheter. After 1 month I was back to normal.
I had a lot of questions about my disease and prognoses. PCP said “don’t worry and stay off the internet.” My PCP said to not be nervous because I was bothering my wife. I had just had a death sentence. He continues to make sport of me because I was what he says was “overly concerned.” I spent a lot of time on the internet, especially PHA and learned a tremendous amount about PH and AF. My first revelation was that PPH was a rare disease that it is diagnosed in only about 4 people per million each year. Additionally, most of these are young women and children. Since I am a 70-year-old male, they probably only see one person like me diagnosed in a town of this size once every 20 years or so if at all. Better chance of winning the lottery.
That made me feel better but I still had the sleep test ahead. This is a dreadful experience itself. After 2 of these, the Sleep Pulmonologist said I had a mild case of sleep apnea which causes SPH and that my SPH was mild. He prescribed the use of a CPAP machine to alleviate the stopped breathing during sleep. The prescribed pressure of 5 mmhg for the CPAP is minimum confirming the diagnoses of mild SA.
I at no time have had any symptoms except for arthritis. I am not short of breath or do I have any other symptoms of PH or AF.
I found one of my best sources of info on PPH to be the patient posting on the PPA web site. Typically, A “newbie” would get on and say she was told she had PPH on the basis of an echocardiogram and ask what should she do. They always told her not to trust the results of the Echo. Also that regular cardiologists and pulmonologists knew little of this disease and to find a PPH specialist. There are very few in America. Almost always when they got an RHC, The PAP was quite different than Echo said but not as much as mine. From reading these brave women’s postings I became more aware I didn’t have PPH. Especially since I had no symptoms.
The Sleep Pulmanologist that discovered the Sleep Apnea showed me the charts from the tests. He said, pointing at a chart, that my heart rate was 95 which was too high and that would cause eventual heart failure. I didn’t have the heart to mention that he was looking at the SO2 saturation which should be about 95. My normal heart rate is 60, which the test really showed. He also said that my breathing ability test was bad which the other pulmonologist and internet sources said was great. I hired him anyway since he was the only game in town and I too thought I should have a CPAP machine that would eventually cure me or at least stop progression.
The bright side is that I have lost 40 pounds, I walk 1.5 miles every day without fail, The CPAP machine has many advantages including lowering systemic BP and generating an enzyme (lepton) that aids in losing weight. I never fail to use it while sleeping.
This was a traumatic experience and shows that there is little knowledge of PPH and SPH out there.