A Living Miracle: My Journey with Pulmonary Hypertension
by LaVerne Cash
My journey with pulmonary hypertension (PH) really started the day I was born. All my life I have suffered with a food addiction, thus a weight problem. In my early teens I was obese, weighing in at 260 lbs at a height of 5ft 11in. My parents had tried to get me to lose weight, but it wasn’t until losing weight became important to me that I was able to do so. My freshman year in high school I watched a fellow obese student trying to do push ups in gym class and realized that, for all practical purposes, I was watching myself. At that point losing weight and keeping weight off became a high priority in my life. By my sophomore year I was down to 170 lbs. What does this have to do with pulmonary hypertension? I’ll give you a hint: think “phen/fen”.
In my youth if I kept some control over my eating and exercised, keeping weight off wasn’t much of a problem. But as I reached my 30’s, I found myself having to eat less and less and exercise more and more to keep my weight down. By the time I turned 35 I was struggling to keep my food addiction and calorie consumption under control enough not to gain weight. I realized I needed help, but, at the time, food addiction wasn’t recognized as a problem. You would have had a hard time getting anyone to take you seriously if you told them you had a food addiction problem.
It was during this time I saw an ad in the local newspaper for a center with a medically supervised program for weight loss, smoking cessation, and various other health issues. I decided to check them out. They did have a weight maintenance plan which appealed to me. As with most programs you had to record everything you ate detailing portion size, fat content, and exercise. I also had to weigh in every week. There were low-fat cooking classes and lifestyle management classes which I found very helpful. Although the urge to eat never went away, the accountability factor alone helped me to keep the eating under control. Also being able to talk with someone who understood my problem helped. One day I went in for my weekly weigh in and was met at the door by the owners. They told me that medical research had found that many addictions are caused by low levels of serotonin. They thought my food cravings could be caused by low serotonin and suggested that taking phen/fen might reduce or even eliminate the cravings entirely. I too had read articles relating addictive behaviors with low levels of certain neurotransmitters, serotonin included. I found their proposition interesting; however, I wasn’t totally convinced, especially given I had no proof that my serotonin levels were low. I asked whether tests were available to measure serotonin. The answer was yes, but, the test was expensive and invasive and that it was easier to treat the symptoms and then determine whether the treatment was effective. I was also told that phen/fen was safe and their clients were monitored more closely at the center than they would be at a doctor’s office. I was still skeptical, but, in light of their promise of close monitoring and against my better judgment, I agreed to take the drugs.
I experienced side effects from the beginning. Within days I began getting weak and lightheaded. A couple weeks later I began having headaches and running a fever. At first I attributed the symptoms to a sinus infection. I postponed going to the doctor because I was embarrassed to admit that I was on phen/fen. I had read that sinus infections usually clear up without treatment in a couple weeks. I thought that if I waited it out, the infection would go away on its own and I would never have to admit to being on the drugs. Two weeks later the sinus infection showed no signs of going away, so reluctantly, I went to the doctor and confessed my use of the phen/fen combo. Both she and the owners of the weight loss center suggested that I go off the drugs until the infection cleared up.
My experiences – and, more importantly, my guilt at being on phen/fen - should have been enough to keep me far, far away from ever taking those drugs again; but, unfortunately, it wasn’t. Enticed by the possibility of not having to fight food cravings, several months later I tried the drugs again. Even without the sinus infection, in short order I became weak, lightheaded, and short of breath after walking short distances or climbing stairs. I was convinced the drugs were the cause. I told the owners of the center prescribing the drugs of my symptoms. They swore there was no way the symptoms could have come from the drugs. During this time I was on sabbatical from my job to attend school full time. A couple of weeks after I started taking the drugs the second time, I passed out in the school library. On my doctor’s advice I stopped taking the drugs. Immediately the symptoms disappeared.
From that time (early 1994) until late 1995 I noticed that, even though I wasn’t taking the drugs, I had more headaches than normal. I didn’t have a history of migraines, but I had begun having exercise-induced migraine-like headaches and body aches. None of my doctors could explain why. In December of 1995 I came down with what I call “the flu from hell.” I ran a fever and threw up, which is very rare for me, even when sick. Normally I bounce back very quickly after an illness, but this time I didn’t. During the time I was sick I experienced chest pain and ended up having a battery of tests for heart disease, all of which came back normal. I ran a low-grade fever off and on for a couple weeks and was on antibiotics for a sinus infection for two months. The fever finally went away, but the fatigue and the headaches didn’t.
A couple months before my illness, I had completed a grueling year of comprehensive written and oral exams for my PhD. It was generally believed that between the flu and the stress of the preceding year, I was run down and that with time and rest, I would get better.
After several months of little, if any, improvement, my doctor performed a number of additional tests, all of which came back normal. Finally, she told me I had Chronic Fatigue Syndrome (CFS). Early in 1997 my doctor told me of a study from Johns Hopkins linking Chromic Fatigue Syndrome with Neutrally Mediated Hypotension (NMH) and sent me to Hopkins for a tilt table test (the diagnostic tool for NMH).
In April of 1997 I tested positive for NMH (which I would later discover is consistent with PH). A person with NMH has excessive dilation of the blood vessels in the legs. As a result of the dilation, when the person stands, all the blood in the legs pools in the feet rather than being pumped throughout the body. This lowers the individual’s blood pressure. The doctor testing me observed that, although my blood pressure dropped during the test, my heart rate rose indicating my heart was trying to compensate for the drop in blood pressure by working harder to pump blood throughout my body. This was unusual for an NMH patient; normally an NMH patient’s heart rate would fall. (In retrospect, this was the first clue that maybe we didn’t have all the pieces of the puzzle.) I began treatment for NHM, but it didn’t seem to help.
Around this time results of studies linking phen/fen to PH were being released to the public. At this point I became concerned that maybe my symptoms were not NMH or Chronic Fatigue but instead were related to my prior use of phen/fen. My primary care physician sent me for an echocardiogram “to rule out pulmonary hypertension”. The results of the echocardiogram showed that I had some minor valve leakages but nothing serious, so the cardiologist decided I did not have PH. The echocardiogram done two years earlier showed a perfectly healthy heart, no leakage or any other issue major or minor. In retrospect, the signs of PH were there, but the cardiologist was not well versed enough in PH to recognize it.
The treatment for NMH was not as effective as I had hoped and I couldn’t shake the feeling that something else was going on. I had read a book written by a doctor in Annapolis, Maryland, about Chronic Fatigue and the successes he had had treating it. This doctor had CFS himself. His experience had enough similarities to my own that I gave my primary care physician a copy of his book and ask her opinion. She said she had done all she could for me and gave me a referral to this doctor in December 1997.
Although the onset of the worst of my symptoms did fit the pattern for CFS, I still couldn’t shake the feeling that CFS wasn’t my only problem. My instincts were screaming at me that the doctors were missing something and that somehow that “something” was related to my prior use of phen/fen. I told every doctor I saw that I had taken phen/fen and asked whether my symptoms were related. The Chronic Fatigue specialist was no exception. Like all the others, he felt, that I had not been on phen/fen long enough for it to be a contributing factor. Although the prevalent view is that a person needs to have taken phen/fen for at least three months for associated problems to develop, my pulumonologist told me later that he had seen people develop PH after as few as three weeks of phen/fen use.
The difference in the two echocardiograms still concerned me, so I asked about them. I was told that technology had improved since the first echocardiogram was done and that what I was seeing was an improvement in imaging capability from 1995 to 1997. Later it would be discovered that that was not the case but that PH was probably already present and the second echocardiogram showed the progression of the disease during the two years.
During my first visit to the Chronic Fatigue specialist’s office, I presented the doctor with an exhaustive list of every symptom I had, my complete medical history in terms of what tests and procedures had been done, and when they were performed, the results, treatment (if any), and my perceived effectiveness of the treatment. The doctor reviewed the data and determined that I indeed had Chronic Fatigue Syndrome and began treatment. My condition seemed to improve with treatment but not as much as I had expected; I still couldn’t shake the feeling that something was being missed. The whole premise of this doctor’s treatment was that Chronic Fatigue Syndrome did not have a single root cause but had many different sources occurring concurrently and in different combinations with different patients. Medical science at this point has found many of the sources of Chronic Fatigue Syndrome, but not all. The fact that I continued to believe that something was being missed was not outside the realm of possibility for this condition. I had faith that this doctor could diagnose and treat the various specific abnormalities in my body. However he had one very strong drawback. He did not participate in any health insurance plans and his rates were very expensive.
The fact that a year and a half later I felt much better in my mind, was an indication that I did have some elements of Chronic Fatigue; however, the symptoms I had prior to 1995 (headaches, lightheadedness, and body aches) were still there and, if anything, were getting worse. I believed that these persistent symptoms were consistent with NMH. After all, if the blood isn’t circulating throughout the body, then the various parts of the body, especially the brain, aren’t getting the oxygen they need – so, of course, there will be aches and lightheadedness. NMH is a frequently occurring component of Chronic Fatigue. When I complained that NMH symptoms were still giving me problems, the CFS specialist recommended ephedrine. Ephedrine is a vasoconstrictor as well as bronchodilator. For a while it made me feel a lot better. I attributed this to the bronchodilator properties of the drug allowing more oxygen to be transferred to the blood. The vasoconstrictor properties would have further constricted already constricted arteries, eventually making the condition worse, not better. Indeed, over the course of time my condition did get worse. As a result of some hormonal problems I suffered following a hysterectomy in 2002, my primary care physician took me off ephedrine. I couldn’t tell much of a difference on or off the drug.
By late 2004 I couldn’t walk across a parking lot without getting winded and having to stop for breath. Going up a flight of stairs would almost make me pass out. At this point I knew I could not live with this anymore. Something had to be done or I would be unable to work or support myself. I had seen one doctor after another for almost ten years and test after test came back normal. Other than Neurally Mediated Hypotension, nothing was proven to be wrong with me.
In 1995, when I first became ill, my blood pressure averaged 100/70. Slowly over the years I my blood pressure had been creeping up. When it reached a level of 120/80 (which is considered good), my doctors claimed the increase in blood pressure was the result of the medication correcting my NMH. I didn’t buy it. If the medication was correcting my NMH, why didn’t I feel better rather than worse?
As my blood pressure crept up, so did my weight because I couldn’t exercise to keep it down. By late 2004 my weight had gone up to 230 lb, the highest it had been since high school; and my blood pressure had increased to a point that even the doctors were becoming concerned. Feelings aside, I decided it was time to take off some of the weight I had gained, hoping that getting rid of the excess baggage would help. I started a weight-loss program at the local hospital. This decision probably saved my life.
December 31 2004, New Year’s Eve: I went to the weight-loss center for my entrance exam. My blood pressure was 160/100! The attendants suggested that I see my doctor if it continued to be that high. As I thought about it on the way home, I knew I needed attention and I needed it now! My regular doctor’s office was closed for the holiday, so I went to Patient First. After a long wait, I was finally able to see a doctor. He hooked me up to an EKG and found something he didn’t like. In no time I was on oxygen, had an IV in my arm, and was taken in an ambulance to the hospital.
I was in the hospital for two days undergoing the usual battery of tests for heart patients. It didn’t take them long to discover that I had not had a heart attack. This time the echocardiogram revealed the problem. My right ventricle had enlarged to almost twice its normal size, indicating pulmonary hypertension. The question remained “Why?” My cardiologist assured me that she would find the answer to that question.
The next month was among the scariest in my life. Everything I had been told or read about PH was not good. Test after test came back negative. Again nobody could find a reason for pulmonary hypertension. My thoughts were along the lines of, “Yeah, yeah, here we go again.” For ten years there was never anything found to be wrong, and yet I seemed to get sicker and sicker. It was very frustrating and very depressing. I’m single, live alone, and am dependent on myself for my income. What would I do if I couldn’t work anymore, which was exactly where I was headed.
When all the testing was done, I was told I had Primary Pulmonary Hypertension, meaning PH without an apparent cause. I was sent to Johns Hopkins for a right heart catheterization. Prior to the catheterization I met with Dr Reda Girgis of the Pulmonary Hypertension program. He talked to me about PH, explained the various treatment options, told me what to expect during the procedure, and answered my questions. During our conversation he explained to me that one of the things that would be done during the catheterization would be to have me breathe nitric oxide. He told me the nitric oxide probably wouldn’t have any effect; the reason they had me do it was that in 10% of the population of PH sufferers the increase in blood pressure in the lungs is the result of the blood vessel spasms in which the vessels constrict. In those cases, exposure to nitric oxide causes the vessels to open up and the blood pressure to temporarily go down. That was a best-case scenario, but it rarely happened.
A week later, following my catheterization, Dr. Girgis came out to discuss the results. He said “Remember that lucky 10% I talked to you about last week? You are in it!” I don’t remember the exact numbers, but there had been a significant reduction in the blood pressure in my lungs when they exposed me to nitric oxide. Dr. Girgis put me on gradually increasing amounts of calcium channel blockers. Once I reached the maximum dose, he wanted to repeat the catheterization to determine the effectiveness of the calcium channel blockers. He also indicated, that if the calcium channel blocker brought the blood pressure down enough, the damage to my heart might reverse to a certain extent.
I started my medication, and, before I had increased to anywhere near the maximum dose, I was able to easily walk up stairs again. At my second catheterization the blood pressure in my lungs had gone down from 50 to 33. Dr Girgis said that normally he would be pleased with that decrease, but in my case he thought he could get the pressure down even more and increased my medication.
For two months after my hospitalization I was on extended medical leave from work. My supervisors were trying to talk me into going out on disability. I didn’t want to do that because I knew getting it would be a major struggle, usually taking no fewer than two years. I couldn’t support myself in the time it would take to get disability approved, and I didn’t meet the age or the time in service requirements for regular retirement. Fortunately, within a month of starting treatment, I was able to go back to work. Ten months from the time of my hospitalization I was enjoying my first cruise, walking all over the ship, and having a great adventure when we ran into 35 mph winds from hurricane Wilma. The rough seas made a most of the crew and passengers sick, but for me it was fun.
As of this writing, I have continued to do well with the calcium channel blockers. In April of 2006 a cardiologist at Hopkins examined me. He said that, if he didn’t have my history in front of him, he would never have guessed I had PH. An echocardiogram taken during the same period revealed partial healing of my heart. There was normal function of the left heart and the enlargement of the right ventricle was significantly reduced. A repeat right heart catheterization in November 2006 showed that the blood pressure in my lungs was only mildly elevated. I now walk one to two miles most days of the week. I walk up two flights of stairs to my office every day; I have gotten back into aerobics once a week, am able to work a full time job, and am active in my church.
I could probably write a book about my thoughts, feelings, and lessons learned during this ordeal (and maybe some day I will), but for now I will share a few of the most memorable. The most significant factor in all this is that I am a Christian. My faith in God is what kept me going. Throughout this whole experience God has been right there with me and has met all my needs. As a result, my faith has strengthened and, because of what I have been through, I feel I am a better person.
The years between 1995 and 1997, when I thought I was dealing with CFS, were was almost as frightening as when I found out I really had PH. I had read about people with CFS who had to quit work because they couldn’t function or couldn’t even get out of bed. I wasn’t quite that bad - yet. The best way to describe how I felt was that I perpetually had that rundown; “I don’t really feel bad but I don’t really feel good” feeling one usually has when they are coming down with something. I felt this way all the time. As I mentioned, earlier I am dependent on myself for my income. Quitting work was not an option for me; yet my energy levels were so low that it was all I could do to go to work, come home, and do the bare essentials. Because I had always been so active outside of work, others couldn’t understand why I suddenly had no energy for activities. When I tried to explain about CFS, I was told, “Well I’m tired, too.” They didn’t understand. Many nights I went to bed praying “God, cure me, or take me but don’t leave me this way.”
Like anyone who is facing a crisis, my first thoughts were, “Why me, God?” Immediately, my thoughts went to all the prayer meetings I had attended over the years, and the prayer requests for individuals with various life threatening illnesses, people who had been in serious accidents, people who had lost loved ones and other traumatic events. At one time or another we all probably ask the question, “Why me, Lord?” With that perspective my next question was, “Why not me, Lord?” What makes me so special that I deserve to not suffer?” The answer was, “Nothing.” Even God’s Son suffered and died on a cross. If God’s own Son was not immune to suffering how could I expect to be? My prayer changed to, “God, I know You are in control. I know You aren’t going to put me through anything that You and I together can’t handle; but God please give me some kind of reassurance that I’m going to be okay whatever form you choose.”
My reassurance wasn’t long in coming. Although I have resided in Maryland for the past 22 plus years, I’m originally from North Carolina. I received a package in the mail one day from some friends in North Carolina who, at the time, were gospel recording artists. It was their new CD. Gospel music has always been uplifting to me; but, in the words of the final song on that CD, God gave me the reassurance I asked for just as sure as he had been sitting in my living room with me and talking to me.
“It seems you’ll never over come these trials in your life, but don’t give up there’s always hope to help you win the fight. ‘Cause friend I’m here to tell you the God I serve is great. He’ll take you through what lies ahead ‘cause he makes no mistakes” (from No Mistake written by Mark Willett performed by Day Three). A writer whom I had never met, and probably never will, had put into words exactly what I was feeling. It was like God was telling me “I know life seems kind of hopeless now, but trust Me I know what I’m doing.” Until then it had never occurred to me that, out of my illness, God had a plan for good.
As for my original prayer, God didn’t cure me but He didn’t take me either. Instead He has shown me how to cope with my disease. He’s provided for my needs. For instance, after I was released from the hospital in 2005, I still had many doctors’ appointments and tests at the hospital. Since I couldn’t walk very far without getting lightheaded or out of breath, I was concerned about having to walk from the far end of the parking lot. Although I probably could have gotten away with parking in a handicapped spot, I didn’t have a handicapped sticker, so I didn’t feel comfortable doing that. For every appointment I had until I began treatment, I managed to find a parking space near the front without having to park in a handicapped parking place. And about the time I would need something from the store, a friend would call to say he or she was going out and did I need anything. When I was lonely or down, someone would call to see how I was doing. For as long as I had a need, somebody was there. And I learned how to ask for help when I needed it.
Currently all of my doctors agree that I am a Pulmonary Hypertension success story. To what do I attribute my success? I give all the credit to God. He is the one who did it. He is the great physician. Granted, I’ve had some good doctors working on my case since my diagnosis in 2005, but God gave them their skills and put me in the right place at the right time to have access to their expertise. If you think about it, I could have been died a long time before I was diagnosed PH. Even the doctors at Hopkins say the signs of PH were there in 1997. In 1997 the five-year prognosis for a person with PH was not good. Doctors generally told patients to get their affairs in order. Yet I not only survived PH, I survived PH while being treated for a condition that was the exact opposite as PH. God kept me alive all that time. That’s the first miracle in this story.
There is a second miracle. After all the struggles and all the difficulty getting a correct diagnosis, I still managed to end up in that lucky, but rare, 10% for which calcium channel blockers are effective. Of that 10% for whom calcium channel blockers work, only half of them do well with calcium channel blockers after the first year. It’s been two years since I started calcium channel blockers, and I’m still doing well. That is the third miracle.
Presently my status is similar to that of a cancer patient in remission. I may stay in remission for as long as I take my medication and never have a problem again. Or I may come out of remission tomorrow and at some point have to face the decision whether or not to have a lung transplant. But that is in God’s hands. He has looked out for me so far, and He will look out for me through the rest of my journey until I’m home with Him. In the meantime, I am grateful for the miracles He has performed to get me where I am now and intend to live every day He gives me to the fullest.