Recently I was diagnosed with Myasthenia Gravis which is a neuromuscular autoimmune disorder; fortunately it can be managed with medication and will not affect my career. But I want to review the experience as an example of what happens in modern medicine and the lessons we can draw from it. Like most doctors I have been healthy my whole life and have never been in hospital, except for day surgery. It is invaluable to see life from the other side – that of the patient. On 4/2/19 I developed shortness of breath, meaning I felt like I could not take a deep breath. Leaning forward made it better; lying down made it worse, so much so that I could not lie on my back without struggling to breathe and feeling like I was drowning. My pulse ox at rest was only mildly below normal (94%), so I did what I have always done in the past when sick – nothing. I put one foot in front of the other until it goes away. Yes, the old saying about doctors being the worst patients is largely true.
Unfortunately, it did not go away and gradually got worse. That weekend on 4/6/19 my wife and I went to visit my daughter Jacki in Pittsburgh where she is an intern in a family practice residency with UPMC, having graduated from Hershey medical school the previous year. She noticed my breathing became labored with walking and even with speaking too long, and wanted me to go to the ER for admission. I refused, but I did start on sample asthma inhalers that she had in addition to medicine I take for allergic rhinitis hoping to reduce the shortness of breath. We returned home the following day. Jacki was at that time on an Intensive Care Unit (ICU) rotation and discussed my case with her attending who said that shortness of breath is heart, lung or neuromuscular; it was a broad differential but I felt like I could work through it as an outpatient.
My approach to solving medical problems is embodied in the acronym SPIT (Serious, Probable, Interesting, Treatment). You eliminate the most serious possibility in the differential diagnosis first, as that is what can kill you quickly; then move on to the most probable; and then to the most interesting; using treatment along the way to help rule out one disease or another. Heart was the most serious; lung the most likely; neuromuscular the most interesting. The inhalers were not helping so Sunday night 4/7/19 I started taking high dose prednisone (a steroid) thinking that this might be a viral pericarditis even though my daughter could not hear a heart rub on physical exam; pericarditis improves with leaning forward. I took the steroid for 3 days and stopped the inhalers.
On the morning of 4/9/19 I met with our nurse practitioner LC and reviewed the situation with her. She ordered stat blood work, chest x-ray, abdominal ultrasound, and venous dopplers of the legs. All of which proved normal, or so we thought. Echo and heart stress test were scheduled. I also did a 6-minute rapid walk to see how low my pulse ox would go, but it only dropped from 94%-91%. Hospitalization normally requires a pulse ox below 88%.
That evening after supper my shortness of breath at rest became much worse so I decided to go to the ER in Chambersburg. There I was evaluated by Dr. W who laid me on my back and watched me struggle to breathe as my pulse ox dropped; he then put me back up and ordered blood work, EKG, CT angiogram of the chest and CT of the abdomen. I could not lie on my back, so after some discussion the CT tech allowed me to enter the tube on my stomach. All testing proved normal. At that point Dr. W told me that I did not meet admission criteria, but that he would put me in Observation overnight; I replied that Medicare does not pay for observation and that I had no intention of paying thousands of dollars out of pocket for nothing, especially as I already had an Echo of my heart scheduled for 11:00 am the next morning in Waynesboro. I needed a real work-up by either cardiology or pulmonology or both, and was trying to get it done as quickly as possible in a system plagued with road blocks. The staff at Chambersburg were kind and courteous, trying to do the best they could within the limitations of an unnecessarily complex and inflexible structure. We went home at 4:30 am without being forced to sign out AMA (against medical advice).
At 11:00 am on 4/10/19 I had my Echo done in Waynesboro and the ultrasound tech was able to get good pictures of my heart even though I could not lie back; she then introduced me to the heart stress test technician and I explained my problem. He replied that I could not do the test on my stomach, but that he would discuss the matter with cardiologist Dr. D who had been practicing in Waynesboro forever and knew me well. At 12:30 pm I walked out to the car to go to work when my wife called me back into the house to talk to Dr. D on the phone. I sat down at the kitchen table to get myself together before speaking, but Dr. D could tell that my breathing was labored. We discussed the situation and she told me that my Echo was normal; she did not believe that the problem was cardiac and said that I should go immediately to a tertiary center to get a group of smart people to work me up. She refused to order a heart stress test or cardiac cath. I told her I would think about it, but right now I had patients to see; I went to work.
That night I once again worsened after eating supper. I labored to breathe while sitting on the couch watching television. The shortness of breath did not relent and at 9:00 pm my wife and I packed and left to go to the ER at the Hershey Medical Center hoping for admission and work-up. I was evaluated in the ER by Dr. N with my daughter listening on the phone; Dr. N ordered EKG, chest x-ray and blood work, all of which were reported as normal. She then told me that I did not meet admission criteria but that she would put me in Observation. I again refused observation and explained why. She replied that, “Things get crazy here at night and I need to Rock N Roll.” In other words, you are not going to die tonight, so don’t waste my time. I explained that at minimum I needed a cardiac work-up; she said that lung was more probable; fine then I’ll take a lung work-up. She arranged for both the cardiac team and the pulmonary team to see me in the morning. This would not have happened had I not been a doctor. This is why it takes months and usually a myasthenia crisis before the diagnosis is made. I knew I was in trouble but could not get the work-up I needed.
Around 4:00 am on 4/11/19 I insisted that my wife go to the Hilton Garden Inn as she was exhausted and we would be getting no sleep in hospital. I told her I would call when then the cardiac team arrived to evaluate me. She slept at the hotel and Hilton graciously did not charge us for the room. I did not call her as I did not think I would need her while being examined. That proved to be a mistake as she is a registered nurse with more than a decade of in-hospital experience; more on that later.
Around 9:00 am the intern for the cardiac team arrived. I explained to her that I feared that I had become an interesting patient and that an interesting patient needs an interesting doctor or at least an interested doctor. She replied that I would be seen by Dr. Wm. D a very experienced cardiologist; unfortunately he proved to be neither interesting or interested. He concluded without testing that not only did I not have a heart problem, but in fact he diagnosed me with restrictive lung disease due to obesity as the cause of my shortness of breath. He asked the team to leave the room while he attempted to fat shame me. He recommended that I add another blood pressure medication (losartan/hydrochlorothiazide) and a statin for cholesterol (even though I have a listed allergy to atorvastatin causing a rise in liver enzymes) and as a parting gift he ordered 40 mg of IV Lasix (a diuretic). I questioned all of this and pointed out that my shortness of breath had come on suddenly, and that there was no evidence of congestive heart failure so how would Lasix help me. He assured me that he had seen this all before and that I was wrong about how quickly it had come on; “trust me the Lasix will make you feel better.” “Well, what can it hurt?” I said – famous last words. The nurse came in and pushed the Lasix dose taking about 15 to 20 seconds; he also insisted that I urinate in a container to measure the result even though I told him that Dr. Wm D was not coming back and was not interested in my urine output. The encounter with Dr. Wm D was bizarre and disturbing. I was hoping for better luck with pulmonology.
Almost immediately after getting the IV Lasix, in came the intern for the pulmonology team; he had graduated from Hershey with my daughter. After his assessment he asked if there was anything he could do for me before I left Hershey. “Yes, I need a pulmonary function test.” “I am sorry but it is unlikely that will be done as we only get paid for it outpatient; but I will ask the attending.” He was right. The pulmonology team came in shortly led by Dr. H and essentially told me that I would have to be evaluated as an outpatient. Another worthless exam. But now I had to urinate – urgently. I struggled to get to the bathroom and to get my penis into the measuring container, cutting the urethra and causing blood and urine to fall onto my sweat pants before catching the rest in the container. I then put the container on the large plastic waste basket next to the toilet, not noticing that the top of the waste container was slightly curved. The urine container fell to the floor spilling all over the place. I explained to the nurse what had happened and asked for my discharge papers; I apologized to the cleaning lady, called my wife to pick me up and left.
I got into the car around noon and my wife said, “My God you stink of urine! What happened?” “Take me to the hotel to change and tell our staff that Hershey Cardiology says I am an old, bald fat guy that just needs to exercise and lose weight to feel better. It might reassure them.” I own WFMA, having bought out my partners, and have 25 employees who depend upon me for their jobs. I almost never call in sick. The staff is panicked. Something is wrong and there is no explanation as yet. LC and other staff make it clear that they are not buying the Hershey Cardiology diagnosis. I have to get to the bottom of this quickly. I jump to interesting – neuromuscular disease. I Google “shortness of breath in neuromuscular disease”. Up comes an article on ALS (Lou Gehrig’s Disease) that says that up to 10% of patients can present with shortness of breath as the first symptom, including the inability to lie flat. I call Jacki and she calls her friend Dr. AA, chief resident at NYU (New York University) Neurology. He says that there are 3 possibilities: an atypical presentation of Guillaume Barre (7% chance of death), an atypical presentation of Myasthenia Gravis (5% chance of death), or an atypical presentation of ALS (say goodbye). He says I need to make an appointment as soon as possible with Dr. S, chief of the ALS clinic at Hershey. Because I am a doctor I was able to get past the Praetorian Guard protecting Dr. S and an appointment was scheduled for 4/18/19. I also scheduled an appointment with local pulmonologist Dr. J for 4/17/19 as my experience with Hershey Pulmonology did not inspire confidence. To be continued . . .