This is from a friend -- I thought it worth reading.
Occasionally, after specific things have been rattling around in our heads for awhile, there may be a fortunate moment when at some connections are made. I got lucky, I hope.
As I was contemplating and yes, that's as far as it got, repairing a closet door, it occurred to me that a recent PH Specialist visit which resulted in an outcome similar to that experienced 14 times by a PH patient described elsewhere, may not have been the disaster that it first seemed. Although the physician's impression was incorrect, ("I am not sure you have PH"), I most certainly recognize that he is an exceptional doctor. He is widely recognized as being an expert in certain PH areas and was a very impressive individual. Besides, I am not qualified to evaluate his medical skills, and will not attempt to do so. My disagreement is with the scope of the evaluation. Pulmonary function test and the six-minute walks are standardized screening and even confirmation features of nearly every pulmonary hypertension assessment conducted in the United States and elsewhere. They formed the basis for his opinion.
While statistically sound, these instruments do not address the many different types of PH, resulting in all too common diagnostic delays, disease progression and needless suffering. Clinical indications which can be quantified exist but are not usually detected during standard evaluations, such as presenting problems which are intermittent in terms of symptom intensity, as in sporadic pulmonary hypertension. Chronic thrombeombolic PH, that's where the spinner stopped on my wheel, and does not necessarily respond to standard evaluation as would idiopathic PH, and so on... I cannot state unequivocally how many different type/classes of PH have been identified, but my last count was between 8 and 10, which is quite likely an underestimate.
A subjective issue about which I have received multiple comments from other PH patients involves the use of outward personal appearance as a yardstick for overall health status. At least initially. Many of the replies to "my, you look great today!" are quite humorous and very creative. And ironically, most folks, regardless of their illness, do not wish to show up at their doc for help only to hear about how wonderful they appear! PH in particular is insidious in its tendency to hide beneath a superficial facade of decent health.
Diagnostically, right heart catherization is the gold standard, as we all know. Yet even it can be fickle. Today's advanced diagnostic imaging is imperfect. Indispensable of course, but imperfect nonetheless. During my all too common sleepless PH nights, I have reviewed volumes of nationally respected research generated by quality physicians at well-respected institutions, and there is a trend developing toward a more comprehensive clinical assessment. Including not only quantifiable information, the scope is expanding to include what are considered subjective measures, such a quality of life, extent of patient support system and daily functioning, ability to accomplish goals, as well as sleep quality and pain management.
After all, if we could all participate in a six-minute walk and the other function tests once a week for a year, if PH existed, it would be hunted down eventually. The alternative is more inclusive evaluation, drawing from the leading work in multiple areas relative to PH as a class of diseases. In my particular case, employing additional heart catherizations as a diagnostic instrument has been avoided for three years due to "risky" primary and secondary diseases. Consequently, the aforementioned recent, expectation-laden journey to a well-respected teaching hospital, famous for it's experts, resulted in what was perceived by some (and oh, yes I was a member at first) as basically the end of the story. This rendering, these thoughts, helped me to resolve any doubts and get on with the daily struggle to deal effectively with PH.
Don't get me wrong; while I chewed this dilemma every which way and back again, I wasn't having one of those rare 'good days', as I did during the PH assessment. I recall thinking during a pulmonary function test; I know I'm going to hit a home run on this thing, because expiration is not my major challenge. But during a 24/7 02, and too much TV day, when fatigue steam rolls me, I would have fumbled getting any inspiration worth reporting. I would have fallen asleep and missed the game. Excepting emergencies, timing an evaluation for a highly symptomatic day appears to be a poor approach; at least for me.
Which brings me back to that finely considered repair for my closet door, when the realization that the presence of other, perhaps more credible evidence was laying out on my coffee table. I raced, hobbled, banged my 02 bag against myself, but did reach my objective. The haste was necessary lest I forget why I went looking in the first place (oh the PH moments!). My prey was the fall 2009 Issue of the Pulmonary Hypertension Association Newsletter, "Pathlight". Slightly off-task but worth mentioning. I read elsewhere in a PHA publication: "When you can't breathe, nothing else matters." Absolutely no way I can conceive to improve on that sentiment.
Two articles I had read seemed not only to be relative to my recent experience, but to the slow but certain transition to a system of PH diagnostic techniques which reaches beyond intrinsically institutional factors. The first, from "Phenomenal Lives", written by a PH patient who was frustrated by multiple misdiagnoses, vividly described the process of delay after delay before diagnosis and treatment were attained. In this case, 14 years transpired before a diagnosis was attained. Her spirit undiminished, this incredibly resilient PH patient is now a stout advocate for PH education, awareness and research. The second article, the one that pulled me in like a hungry rainbow trout, was under "Meet the Doctor", and composed by PHA Medical Education Program Associate Christa Donald and Kaitlyn Benneville, Former PHA Web Services Intern. It features Dr. Stephen Mathai of Johns Hopkins Hospital, and after reading about his approach and objectives and hopes, the establishment of a more comprehensive PH diagnostic evaluation system seems like just a matter of time. Time, however, is and will remain unconcerned and unhelpful for most PH patients. Only continued research and determination will unlock the keys to both diagnosing and treating this terrible disease. "Dr. Mathai and his colleagues are attempting to improve the outcome for these patients. He is also interested in developing new tools to measure progress beyond the six-minute walk and lung function tests. Dr. Mathai believes scientific measures should also reflect a patients quality of life, looking at such areas as depression and anxiety levels, sleep quality and the like".
Whatever the cause, I am very grateful my thoughts collided where they did this evening. While my closet door remains neglected, I have a small measure of peace of mind that might convey more strength on a rough day, or two consecutive hours of restorative sleep. Hope is both a very fragile burden and a headstrong, immovable force for change. It is where the inherent discrepancies meet that I believe true progress occurs. And it seems to be happening, thanks to the members of organizations like the Pulmonary Hypertension Association, American Heart and Lung Associations, Dr. Mathai, countless researchers, and all the undeniably tough, determined PH patients who do not give up, and our physicians who never settle, never stop learning.
So, that likely over-describes my little epiphany, It was a blessing to finally gain more perspective, as PH can consume so much thought and energy. However, I could not drag myself off to some uncomfortable sleeping position and likely visions of the miserable closet door until I provided heartfelt gratitude for those PH patient fighters. An unsung hero. I met Merle Reeseman through the PHA Help Line and we have been friends ever since. And what a Blessing, indeed. She inspires many and asks for absolutely nothing in return. Her encouragement allows me to consider hope as something other than deferred despair. Also, I would be remiss not to acknowledge my Critical Care/Pulmonary Medicine doc, who has been effectively treating my PH for about six years. He also helped to save my life during a massive pulmonary embolism in 2006. Humble and dedicated to his patients, this outstanding physician has established such an impressive record that folks come from all over the U.S. to study under him. My PCP is a compassionate young woman who is not intimidated by dealing with tough quality of life issues and pain management. I consider myself both blessed and very lucky to be involved with these physicians.
Following my appointment with the PH specialist, I was admittedly quite discouraged. And a good month of learning and introspection and conversations were required before my tiny epiphany occurred. Perhaps now I'll address the home repair issues, but I anticipate continuing to try to improve my understanding of PH whenever and wherever possible. Finally, (yep, I'm nearly finished!), I would like to reach out to the previously described, constantly misdiagnosed PH patient, who indicated in her article that she often wondered and struggled with the idea that God had given her PH and all its' many demons, and it was seemingly too much to bear. May I just suggest, without imposing my personal beliefs, that perhaps God was not responsible for the PH, but for the strength and determination which have enabled her to obtain a proper diagnosis and deal with the debilitating disease.
And the battle continues....
Rod ~ PH Patient