Crohn’s Disease, Humira, FDA, Respiratory Problems & “BOOP”

2013-03-26 19.12.03

What Exit in New Jersey are you from?

This picture of MY ROOM IN THE EMERGENCY ROOM (”ER”), taken by me on Tuesday, March 26, 2013, from a New Jersey Hospital’s Emergency Room (“ER”), was demanded to be deleted from my phone by that hospital’s security personnel as they escorted me out of the ER, after I was forced to remove my own Intra-Venous Line (“IV”) causing my blood to squirt so high it almost hit the ceiling,  No door, no curtains, no privacy; no HIPAA compliance since I was barely being treated; just a gurney, under an Exit Sign, in a hallway in the ER, where I was essentially IGNORED for 5-7 hours.  My medical problems had dehumanized me to the point where some might say I was now just a lyric in a great Bruce Springsteen song like “Jungleland” where song characters agreed, “they’ll meet ‘neath that giant Exxon [i.e., Exit 🙂 ] sign, that brings this fair city light.”  If that doesn’t typify the stereotype about New Jersey, …

These days, Emergency Rooms Render Triage Medicine on Steroids

Ironically, and in retrospect, this Exit Sign was fitting as a description for a bad experience in the ER of a New Jersey (“NJ”) hospital given all the NJ Turnpike Exit jokes which have come to unfairly define New Jersey in the lexicon of the public’s awareness.  However, please try and understand I am not identifying the specific NJ hospital because I don’t think that’s fair since I must have caught it on a busy night and my condition is extremely complicated such that a bad experience was certainly possible, as it is with ANY emergency room  visit in hospitals all across the United States.  After all, emergency rooms in the United States are designed to render triage medicine.  But due to ERs being overtaxed as sources of Primary Care for many Uninsured patients, the ER triage medicine model is on steroids out of bare necessity to keep serving its respective local communities.  If the New York Yankees slugger Alex Rodriguez is any example of what happens to steroid users over the long run, it is no wonder why our ERs are consistently providing unsatisfactory “consumer” results.  Still, in Forty (40) years of going to hospitals and emergency rooms, this was BY FAR my worst experience in over 200 hospitalizations and ER trips.  But just like there exists a Bruce Springsteen, Jon Stewart and Frank Sinatra to extinguish the stench from every “Jersey Shore” and “Housewives of New Jersey” entertainment industry venture which somehow permeates the mainstream’s consciousness, this experience with a NJ hospital and emergency room does not reflect the typical NJ medical interaction.

The Patient Consumer in a Hospital Emergency Room

Now that I’ve set the stage for this ER debacle and provided some context for my experience, please note this will be a two (2)-part Blog Post with this first one adding the necessary medical context for my ER trip so that you understand the seriousness of my situation and thus why I was so disappointed in how I was treated.  Part 2, which I will post shortly, will detail the “consumer” side of things which prompted me to call my credit card company to cancel the $100.00 ER fee I had to pay pursuant to my Health Insurance Plan.  More specifically, just as I might revoke a credit card charge to a shady automotive repair shop which I thought had ripped me off, I did the same with this $100.00 ER charge as I believed the services rendered to me were almost non-existent and I thought it was unreasonable to pay for being either mistreated or treated like an animal.  I don’t blame any one person in that ER but my experience made me re-think how I must use a hospital ER moving forward.  I hope you come to the same conclusion after reading my story. But before detailing what happened to me in the ER on Tuesday, March 26, 2013, it is important that you understand WHY I went to the ER.  Please trust I will make this part of the journey as interesting and entertaining as possible.

Brief Medical Background regarding my Crohn’s/Lung Condition

In order to best comprehend my contention that I was treated like an ANIMAL inside this NJ Hospital’s ER, it is necessary to share with you some pertinent personal medical details regarding my condition since it is a repeat of something I went through during the beginning of the spring in 2011.  As many of you already know, I have been battling the autoimmune and incurable Crohn’s Disease for almost 30 years.  I was diagnosed in and around 1984 when there wasn’t very many treatment alternatives other than surgery for my case of severe (and aggressive) “Obstructional” Crohn’s Disease.  As a result, as of today’s date, I have endured approximately Twenty (20) + abdominal surgeries and over Two Hundred (200) hospitalizations/trips to the ER.  I have also had several other serious surgeries related to, or caused by, the autoimmune aspects of my Crohn’s including, but not limited to, spine fusion surgery, two (2) cataract surgeries and knee, nasal and toe surgeries.

In approximately 1998, after I believe first being formally approved in 1988 as a “group” of “Anti-TNF Agent” drugs by the Food and Drug Administration (“FDA”), the FDA began approving certain specific brands of these drugs to help place Severe Crohn’s Disease patients into remission.  Without getting too scientific, these  “TNF inhibitors” or “Anti-TNF Agent” drugs caused TNF inactivation and that has proven to be important in controlling  the abnormal inflammatory reactions associated with autoimmune diseases like Crohn’s Disease where the abnormal reaction causes the body’s immune system to attack itself, rather than the cause of the inflammation.  The identification of the role of the “TNF” in the inflammatory response of patients suffering from autoimmune disease and the development of drugs to mitigate or control the TNF response were considered major breakthroughs in the treatment of incurable, autoimmune diseases.  As a group, these Anti-TNF Agent drugs are referred to as “Biologics.”

Introduction to “Biologics”

My first experience with Biologics was with the drug, “Remicade,” and it worked VERY WELL, at first, but then I needed the drug infused every 6 weeks instead of 8 and then I soon needed twice the dosage, until I started to develop signs I was allergic to it.  My doctors then watched me very closely and pre-medicated me with steroids, as they infused what would be my final dose of Remicade.  Unfortunately, within 48 hours of that Remicade infusion, I developed what can only be referred to as a “Delayed Anaphylactic Reaction” when my throat began closing and I was rushed to the hospital to be pumped up with even more steroids to counteract the allergic effects of the Remicade.  Please note this does not happen to many Remicade patients and I know MANY such Crohn’s Disease patients who are still on Remicade and feeling GREAT.  Anecdotally, I am also aware of many Pediatric Crohn’s Disease patients who do VERY WELL on Remicade.  In my case, however, as the Remicade had staved off what appeared to be an impending serious Crohn’s abdominal surgery, once I stopped the Remicade I soon thereafter turned up in the operating room.

Enter, the Biologic drug, “Humira”

Post operation, my doctors were concerned about me being on no medication to help thwart off any possible Crohn’s recurrences because my Crohn’s Disease had been very aggressive and each time it flared the possibly of surgery was all too real yet I had very little small bowel left.  Therefore, a year or two later I was put on the next “invented” Biologic Agent drug for Crohn’s, namely, “Humira.”  From a layperson’s perspective, and as it was explained to me, the differences between Remicade and Humira appeared to be that Remicade was discovered/developed using mice proteins whereas Humira was more pure and based on human proteins so that a patient allergic to Remicade might have much better success with Humira.  Humira was also an injectable drug as opposed to an infused one so it offered a greater degree of control over one’s life and lifestyle and that made it even more appealing to patients.

However, Humira came with many Medical/Legal Disclaimers about side effects including possible severe Cancers and lethal Respiratory Side Effects.  At the time I started taking Humira (approximately 2005), I assumed these Disclaimers were included in the drug’s labeling because there now existed some historical real patient data and a bunch of pharmaceutical (“Pharma”) lawyers were simply being thorough and candid in provided new Humira patients with as much patient data as possible. These Disclaimers were subsequently substantially strengthened in August, 2009, to reflect the Cancers which were occurring in Crohn’s patients using Humira such that the FDA issued the Pharma-dreaded “Black Box Warning Label” to reflect the possibility of Lymphoma being a side effect of taking Humira.

Humira, the FDA and YouTube

Beginning in November, 2009, some of the most serious changes in the Humira label came when the Black Box of the label was strengthened to include a serious risk of “opportunistic” respiratory infections including “Histoplasmosis” and “Bacterial Sepsis,” along with the risk of lymphoma and other cancers in children and young adults. The FDA even utilized YouTube in an extraordinary measure to convey these risks to Health Care Professionals and to Patients via THIS Video.  The Humira label was subsequently strengthened even further in response to DEATHS which were occurring from rare but fatal Fungal Lung Infections in Crohn’s patients using Humira. The FDA again used THIS Video and YouTube to convey the dangers of these Fungal Infections.  Leukemia was also subsequently added to this list of known serious side effects of taking Humira for Crohn’s Disease.  Despite this pattern of increasing risks of cancers and fatal respiratory side effects, someone in the marketing department of Abbott Laboratories, the manufacturers of Humira, apparently didn’t “get the memo” from the FDA as TV commercials celebrating the success of Humira placing Severe Crohn’s Disease patients into remission increased and started replacing Erectile Dysfunction drug commercials as the most rotated and parodied drug commercials on TV.   WATCH the TV Commercial HERE.

How Humira affected me re: Respiratory Side Effects

This began to affect ME approximately 12-18 months after I started Humira when I began to experience such severe and unpredictable joint pain that it felt as if I were a Voodoo Doll being pricked by a sadistic arch rival from high school.  The sudden onset of the joint pain was often so extreme that I could be walking and talking one minute and then laid out on the sidewalk the next.  However, my Crohn’s Disease seemed to be in remission so I was “okay” with the tradeoff of 50 daily trips to the bathroom now supplanted by being pricked with sharp needles all over my body by a guy named Skippy from High School just because one night I made out with his ex-girlfriend. 🙂  But, Skippy’s presence in my life was quickly replaced by recurring and increasingly intense bouts of Bronchitis and Pneumonia.  I, like most other people with compromised immune systems, had suffered from occasional respiratory infections and whatnot but since starting Humira whenever I got Bronchitis it almost always turned into Pneumonia and then the symptoms would be so bad I had to be hospitalized.  That had never happened before.

It is one thing to be 45 and hospitalized for recurrent Severe Crohn’s Disease but it’s entirely a different story when you begin to cough up blood and run fevers as high as 105 to the point where your gastroenterologist insists you be hospitalized.  This happened several times until I started researching it in the wonderful world of health care social media when I learned that other Crohn’s Disease Humira patients were experiencing the same severe respiratory problems.  The difference was, according to them, when they discontinued the Humira; the respiratory problems soon thereafter disappeared.  Shortly after recovering from my latest hospitalized bout with an epic case of Pneumonia, I asked my gastroenterologist if I could stop the Humira in hopes of alleviating these respiratory problems which were becoming far too frequent and disabling.  He acquiesced but insisted that I soon thereafter start taking the newest Biologic drug called “Cimzia” because he didn’t want me on no possibly “preventative” medication, again, given the aggressive nature of my Crohn’s and due to the fact that I didn’t have much more small bowel to donate to a surgeon.  I agreed.

Cimzia

Literally a few weeks after stopping Humira, my lungs felt normal and it seemed I had gotten back the strength to run, play tennis, chase women, etc.  I didn’t want to start the “Cimzia” until I knew for sure if stopping the Humira made that healthy difference in my lungs.  To truly trust such a conclusion, however, I needed to be methodical in my approach.  I therefore abstained from taking Cimzia until I was sure I had conducted a “controlled experiment.”  I also wasn’t so eager to jump back into the world of Biologics and thus took my time beginning the Cimzia.  After several months going medication “commando,” I was convinced I had isolated the Humira as the probable culprit for my hospital-grade respiratory problems so I was enjoying getting back to playing tennis and chasing women.  I started the Cimzia in and around 2009 after my tennis game had improved and I was again content that I was clueless about women.

Suffice it to say, the medical progression to Cimzia was analogous to changing from Remicade to Humira and it was also injectable so my lifestyle was improving and I was optimistic about my future.  But after only a VERY short while on Cimzia, the unmistakable “Skippy” joint pain and respiratory effects I had experienced while on Humira had come back, and this time they came back in spades.  It was unclear to me if my respiratory problems were caused by the cumulative effects of being on Humira for a few years or if they were triggered by an allergic reaction to the Cimzia, which I had just started.  (Subsequent to my respiratory problems relevant to this Blog Post, in July, 2012, the FDA again turned to YouTube to distribute THIS Video with their Black Box Label Warning for Cimzia due to DEATHS that were occurring from Fungal Lung Infections.)   

The Beginning of BOOP and Severe Lung Problems

It was spring, 2011 and I began to come down with strange spikes of 105 fever which would last but a few hours and go away on its own.  Then I developed Bronchitis which turned into Pneumonia and that began my “Darkness on the Edge of Town” as I had to go to local New Jersey hospital emergency rooms because my breathing became seriously labored (and I lived nearby in NJ).  The first few times I was admitted for tests and to determine the most effective antibiotic to treat my severe respiratory symptoms but soon it appeared I possibly had a strain of Pneumonia that was resistant to drug therapy.  In time, however, one of the antibiotics seemed to work and I was discharged but only to head directly into NYC to see my 30-year Crohn’s Disease doctor who is the most experienced and smartest physician I have ever encountered.  Yes, I had a pulmonary problem but given the respiratory problems associated with the aforementioned Biologics I had taken for my Crohn’s Disease, I knew this current medical fiasco was predicated on, or somehow connected to,  my Crohn’s Disease treatment history.  To that end, he first tested if I was allergic to the Cimzia but I was NOT.  Nevertheless, given my rather extreme and recurrent respiratory problems, I had no choice but to discontinue the use of ALL Biologics.  I was again going medication “commando” with my Crohn’s Disease and my NYC doctor was very worried.

Having recovered from a dangerous case of Pneumonia for which I was hospitalized in NJ several times, one day I walked a few feet to the mail box to pick up my mail and my sudden shortness of breath caught me by surprise and I passed out.  When I awoke, I couldn’t breathe and talk at the same time.  Trying to take a deep breathe was like sucking as hard as possible on a toothpick-sized straw.  I got scared very quickly and called my Internist who recommended I see a certain local respected NJ pulmonologist.  That doctor was kind enough to see me that day and as I struggled to breathe walking only the 50 feet from my car to his office, he merely tested my blood/oxygen levels and deemed me FINE.  I almost had to use sign language to communicate with him as I couldn’t catch my breath from that 50 foot walk but he just smiled and said I must have a heart problem so I should see a Cardiologist. From my perspective, it felt as if I had brought my dry cleaning into the dry cleaning store yet the owner of the dry clothing store was telling me I had to bring my jacket and shirt into the bagel store across the street in order to get it cleaned and pressed.  Let’s just call this doctor the “dry cleaner” because unfortunately he was a recurring character in this 2011 story.

The 2011 BOOP Emergency Room Visit

Luckily a friend of my family is a cardiologist and he saw me immediately after the dry cleaner had somehow misconstrued my simultaneous inability to talk and breathe as the new pulmonary endurance standard for the Olympic athlete.  The cardiologist did not concur with the dry cleaner and was SERIOUSLY CONCERNED about my inability to multi-task in this manner and after confirming my heart was fine he ordered me to go to the emergency room at a prominent NJ hospital very close to where I lived at the time.  This was also the same hospital I had been in and out of with my recurrent respiratory problems and seemingly drug-resistant Pneumonia so everyone knew me but I’m not so sure they were happy to see me as I was very scared and frustrated.  The anxiety which accompanies the inability to breathe is very much like the feeling a 9-year old Little Leaguer gets the first time he collides with the catcher and has the “wind knocked out of him.”  It feels as if you’ll never be able to breathe again and all you can do is wait for your body to “reset” itself.  But at 9 years old, you are unfamiliar with this process so it’s frightening.

At 48 years of age, I felt the same way each time I had to re-visit an emergency room and go through my medical history with a nurse.  My inability to consistently breathe and convert the process to an involuntary bodily function was not “kicking in” and talking only made it worse. Fear of the unknown is as traumatic at 48 as it was at 9 years of age although ice cream seemed to help when I was a kid.   Now it was just successfully flirting with an attractive nurse all the while lying to myself that she’s laughing at my jokes because I’m very funny. But what jokes?  I couldn’t carry on a conversation about my medical history AND also breathe.  Since I’m no Brad Pitt, without those jokes I was again, “running into the catcher.”

Anyone know a good Dry Cleaner?

The ER head physician was also familiar with my case and if memory serves me well, she didn’t know what to do for me since an apparent well-known pulmonologist at the hospital who was the on-call pulmonary specialist that evening had already deemed my lungs as being FINE.  (My luck, this was the dry-cleaner. I was not impressed, or in agreement, with his conclusion and I respectfully made that known from the get-go.  After all, it was counter-intuitive to think I did not have a respiratory problem when I couldn’t breathe and my heart had checked out just fine.)  Thankfully, routine ER flat x-rays showed something strange in my lungs and despite the cost to the hospital of performing a CT Scan on me and the associated blow to the dry cleaner’s ego, they had to perform more diagnostic tests to confirm the dry cleaner’s diagnosis that my respiratory system was just fine and that my inability to breathe was being caused by some other medical problem.  As I also recall, the cardiologist I had seen suggested that I be tested for a Blood Clot in my lungs due to the recurrent Pneumonia I had been dealing with so that test was also conducted.

The Definitive May, 2011 BOOP CT Scan

It was a rather intense and long emergency room visit but when the CT scan results came back I went from being some pain-in-the-ass patient to what they were referring to as a VERY SICK YOUNG MAN.  I knew that because I looked at my patient chart and saw that written in one of the margins.  If I only knew who wrote it I would have demanded an explanation and fired that doctor but I was unable to do so.  Based on the physician personalities I had to deal with, it could have been one of five doctors.  I didn’t think it was very compassionate to write such a mendacious statement which would affect the opinions of all health care professionals who would subsequently read my patient chart.  But I also knew I was perceived to be very sick because they had now formally admitted me to the hospital.  Having finally found some peace and quiet in my own room, I had briefly fallen asleep only to be woken up by a small army of doctors surrounding my bed, which included the infamous dry cleaner.  His face was located within striking distance of my left foot and I felt like doing a “Karate Kid” on his face but an authoritative thoracic surgeon sharpened my focus to eye level when he said he needs to perform lung biopsy surgery on me ASAP while I am still breathing and not on a Ventilator.  Huh?

The Journey to the Correct BOOP Diagnosis Begins

He was very nice and I liked him immediately even though he told me I might have lung cancer.  I’m funny like that; I evaluate “new” people by their sincerity rather than by the content of their dialogue.  I believe in the old adage to only believe what you see and not what you hear.  I also won’t play poker with a guy named “Doc” and I won’t eat at a joint call “Mom’s.”  I look for “tells” that might come in handy later.  Anyway, this thoracic surgeon explained to me that they saw a great deal of damage in my lungs and needed to find out what was going on so they could devise a treatment plan.  Normally they would simply perform an endoscopic-type procedure called a bronchoscopy and go in through my mouth but in this case they needed larger biopsy sizes and the doctor again explained that I might soon be on a ventilator so they needed to formally operate.  That word “ventilator” kept coming up almost as if they were talking about someone else.  All they while, the dry cleaner was nodding his head in approval of everything said by this erudite and experienced thoracic surgeon and I felt like looking at him and saying, “Really?  Where the “f**k” was your diagnosis when I presented at your office not being able to breathe and talk at the same time yet you deemed me fit as a fiddle?”

Patient Engagement kicks in

Instead, my brain woke up and I engaged the thoracic surgeon in an intellectual discussion about how I came into the hospital with Crohn’s Disease but now might have Lung Cancer having never been a smoker?  He then started asking me if I had ever been a coal-miner or if I had lived in certain parts of Ohio and it began to feel like I was being PUNKED.  Back then I was an Entertainment Attorney and a Film Producer and the closest I had come to a coal mine was watching a documentary about one on TV.  He apologized for the grim nature of his questions but he explained that they suspect I had “foreign materials” in my lungs and they had to figure out how that happened.  That’s when I recalled all the Humira Black Box Label Warnings and I knew I needed to somehow get my NYC Crohn’s doctor into the conversation.  When I brought up the possibility of the Crohn’s drugs possibly contributing to whatever lung problem I had, that’s when the dry cleaner decided to speak and he incorrectly, but very confidently, explained that one thing had nothing to do with another.  I just smiled because it beat crying as I knew dealing with this problem was going to be two-fold.  That is, I was going to have to deal with the medical problem AND I was going to have to obtain my medical care from a pulmonologist who understood this connection and I assumed finding that person was going to be difficult to do.

The Lung Biopsy Surgery for BOOP

The lung surgery went fine but the post-operative debriefing with me was surreal.  I did NOT have Lung Cancer but the thoracic surgeon admitted to me that he had seen damage to my lungs he had never seen before and he had been operating for MANY years.  I admired his candor and liked him even more for being straight with me but I was worried.  Again, there were at least 8 doctors surrounding my bed and the conclusion they were prepared to make, subject to clarification of the biopsied parts of my lungs being confirmed by a Pathologist, was that I had a rare form of Pneumonia called “Bronchiolitis Obliterans with Organizing Pneumonia,” better known by its acronym, “B.O.O.P.” Once the word BOOP came out of the doctor’s mouth I could tell by looking at the faces of the other doctors that they had never treated a BOOP patient before and were only familiar with it from a textbook.  My suspicions were confirmed when the dry cleaner said to the bagel store owner (another clueless pulmonologist at my bedside) that 40 mgs of Prednisone for one year should do the trick.  Given my vast experience unsuccessfully taking 40 mgs of Prednisone for my Crohn’s Disease and my substantial difficulties breathing, I remember thinking, “That might work for his dog, but it was not going to work on me.

The Post-Operative Discussion & firing the Dry Cleaner

As the discussion continued and I began to absorb my new reality.  It felt as if my head was swirling around 360 degrees because I am quite familiar with Prednisone and its myriad of side effects from my Crohn’s Disease and being on it for a year would be catastrophic in so many ways.  Further, Prednisone never seemed to work on my Crohn’s Disease, as demonstrated by the high number of surgeries I had to endure, and I was equally perplexed by the relatively low daily dosage of 40 MGs because that dose never worked for me when I had inflammation in my small bowel and here I was not able to breathe and talk at the same time so I asked for an explanation.  The straight-shooting thoracic surgeon explained there was a mathematical formula to figure out the daily dose and then the overall dose would be carefully weaned down in calendar quarters over the course of said one year.  But he then acknowledged that given my medical background, my Crohn’s Disease, an already compromised immune system and an ineffective history with Prednisone, 60 MGs was, in his opinion, the more appropriate daily dose.  He them tempered that opinion with a declarative statement that he would not be “treating” the BOOP but the gentlemen standing around my bed would be and therefore it is their call.

Confronting the Doctors who didn’t LISTEN to me

I thanked the thoracic surgeon but said he was not entirely accurate because I want the dry cleaner out of my room and off my case because he will not be treating me.  That pretty much set the stage for my relationships with the NJ pulmonologists who had seen me to-date and who had completely missed this BOOP diagnosis.  Please understand that missing a diagnosis never bothered me if I felt the doctor was trying to help me.  Even mistakes don’t bother me if the intent was to help me and I am not too badly damaged as a result.  In this instance, however, I was seriously annoyed at the callous manner in which my symptoms were ignored by each of these doctors.  It was as if they knew better but chose to rely on statistics that given my NY/NJ/LA  background, I couldn’t possibly have BOOP or “foreign materials” in my lungs.

But with my history with Crohn’s Disease, Biologics and the FDA’s rather pronounced Black Box Label Warning for Remicade, Humira and Cimzia, they should have been asking me different questions when I first presented at their respective offices and/or in the emergency room with repeated serious bouts with Pneumonia.  In my experience of almost 30 years with an autoimmune disease like Crohn’s, I have built up tremendous credibility with doctors because I know my body and I don’t like being sick.  Yet, because BOOP is so “rare,” these NJ pulminologiosts just assumed I was crazy or somehow seeking attention.  Now I had the opportunity to vent my frustration with them and for the purposes of going into battle with BOOP with a a sound mind I felt it necessary to share my disappointment in them, with them.  Much like a star basketball player “playing” the refs in one game anticipation of his next more important game or series, I was also doing this to ensure that I would be treated differently going forward.

Besides, in my mind I was already at the Lincoln Tunnel on my way to see my trusted NYC Crohn’s doctor so he could get in the loop and recommend a NYC pulmonologist who he could work with in getting me through this most unexpected of nightmares courtesy of the combination of my Crohn’s Disease, Abbott Laboratories, Humira and the FDA.

My NJ Pulmonologist – Personality & Bedside Manner of a Handball

It took a few days to heal from the lung surgery and it was VERY PAINFUL so all I could do was have people make phone calls for me to my NYC doctor since I could barely speak.  As soon as I was strong enough to get into NYC, I met with him and he gave me the name of a female NYC pulmonologist and I made a projected appointment with her while I followed the directions of a different NJ pulmonologist (i.e., the “bagel store” owner from the NJ hospital who also surrounded my hospital bed but only listened to the thoracic surgeon share his findings so we never actually spoke while I was hospitalized).  As I was to find out after consulting more closely with him, he had a passive-aggressive personality and not only did he NOT acknowledge the connection between my Crohn’s, having taken Humira and developing BOOP,  but he had the personality and bedside manner of a handball.  Clearly, I had no palatable options at the time so I tried to stay positive while I battled a possibly life-threatening condition with the hope that my NYC doctors would figure out the best solution to my problem.

Notwithstanding this doctor aggravating me to no end with his lack of confidence in anything I contributed to the conversation, but his patronizing and passive-aggressive style of feigning listening to me was insulting my intelligence.  Yet, I knew there was nothing to do but take the 60 MGs of Prednisone until I was better situated with a NYC pulmonologist who would collaborate with my NYC Crohn’s doctor.   So, I just feigned my interest in his every word but just made sure he never deviated from the 60 MGs of Prednisone protocol.   I was approximately one-month into the 60 mgs of Prednisone regiment when my appointment with the NYC pulmonologist came up.  Coincidentally, this prickly and rude NJ pulmonologist managed to insult my mother so disrespectfully during one of my last appointments that I stopped him from talking about me (she was temporarily out of the room) and respectfully requested that he apologize to my Mom upon her return to the room.  He looked at me like I had just delivered his lunch and was asking for a $100 tip.  Accordingly, he blew me off yet I persisted.  He apologized to her. At that moment of his patronizing apology to my Mom, I put all my trust in the NYC pulmonologist who I hadn’t even met yet.  If my NYC Crohn’s doctor recommended her, she was going to be good enough for me.

The Concept of “The Second Opinion” – it’s like the Seinfeld “Reservation”

Having already made up my mind that I had to change doctors, I told this NJ Pulmonologist  that I was getting a second opinion in NYC and there was a good chance I would stop seeing him because I needed a doctor to work with my NYC Crohn’s doctor and he clearly did not believe in that Crohn’s Disease connection.  I was trying to be as non-confrontational as possible but then he did something I have never seen a doctor do up until then, or since.  He asked me if HE could talk to this NYC pulmonologist BEFORE I SAW or SPOKE TO HER so he could bring her up-to-speed.  His suggestion was so bizarre that it caught me off guard but my instincts formed words and they sounded very much like this:  “Doctor, the whole point of getting a Second Opinion is to seek objective input into a situation that would benefit from ANY additional clarification.  Therefore, any communication between you and this potential new NYC pulmonologist would taint her perspective and put me back at square one under your care, and with all due respect, I no longer want to be under your care.”

Then I smiled as I caught myself since I had revealed that he was actually being territorial about me being his patient.  Did he really care about me or did he just not like to lose patients?  I wished the former but knew it was the latter based on his actions, especially the way in which he treated my Mom.  Then I got annoyed at his passive-aggressive approach and I explained to him the example the comic Jerry Seinfeld uses when describing the concept of the “Reservation.”  I was trying to lighten up the moment so he would drop it but he actually took me serious and said he watched “Seinfeld” and asked, “What was I referring to?”  I just laughed and firmly told him he is NOT to contact this NYC pulmonologist (as I had already given him her name).  He then seemed to admit “defeat” and said I will need to get all the films and reports organized so that the NYC pulmonologist could make the most informed decisions “and that could take a while,” as if he were rooting against me making any progress by going to see a NYC pulmonologist.

I sarcastically told him his “negativity and clear interest in my best medical interests are duly noted” but I had already complied each and every diagnostic test including the films, reports and all the blood work.  He looked at me as if I had just run the 4-minute mile in Clogs.  He apparently was so accustomed to being “in control” that he was amazed a patient actually asserted himself.  I genuinely thanked him for his help up until that point but did tell him that our personalities did not mesh and I need them to, in order to be treated, especially to take on a battle like this one.  I then left him and his God-complex for some Pizza joint as being on 60 MGs of Prednisone for one month is like being pregnant.  You eat, what you want to eat, when you want, and where you want.

Meet my NYC Pulmonologist

I very much liked the NYC Pulmonologist and from the get-go she and my NYC Crohn’s doctor were on the same page regarding the BOOP having a significant tie to my Crohn’s and the Humira or other Biologics.  I tend to see Humira as the primary culprit of my respiratory problems because I was an avid athlete all my life until I started taking Humira.  It was also during the time on Humira that I began to be hospitalized like a 90-year old for what should have been simply bedridden Bronchitis events.  In any event, the only known effective course of treatment for BOOP was the 60 MGs of Prednisone for one year tapered over calendar quarters, so there wasn’t much for this new NYC pulmonologist to do other than monitor me but there was something about her bedside manner and genuine care for my plight which simply made me feel good.  This intangible quality would become incredibly significant because after a few months on the Prednisone my body began to deteriorate even further.

For example, I contracted a serious case of regular Pneumonia for which I had to be hospitalized twice and I also had Pleurisy for which the pain was “off the charts.”  Luckily, I’ve seen a well-respected Pain Management Doctor for several years due to all of my medical maladies and he was able to control my pain.  It wasn’t just the Pleurisy; it was the contrasting feeling that the pressure of a Piano was always on my chest when I tried to breathe and occasionally I would get dagger-like pains in my lungs from the Pleurisy. With the Pleurisy, the daggers became unmanageable, even with medication. The Prednisone’s typical but myriad of side effects also caused extreme joint pain and by the fourth month I had gained 45 pounds so everything was intensified.

The Turning Point – August, 2011

Throughout the entire time, the NYC pulmonologist stayed in close contact with my NYC Crohn’s doctor and my blood count was monitored routinely.  But, when I got regular Pneumonia after four (4) months of being on such a high dose of Prednisone and had to be hospitalized in NYC for the second time (approximately August, 2011), it was clear something had to be changed as my breathing and pain were not getting better yet the side effects from the Prednisone were causing tremendous additional systemic medical problems. One night during that August, 2011 stay at Mt. Sinai Hospital in NYC, while I tried to fend off the Migraines caused by the Prednisone to get some sleep, the NYC pulmonologist stopped by to check up on me.  I was VERY frustrated with my lack of progress battling the BOOP and the massive dose of Prednisone was playing games with my mind.  This prompted me to ask the doctor if there was anything else I should be doing?  I personalized the situation and asked her, what she would do, if she were me?  She replied that the ONLY thing I hadn’t done, which she would do, was obtain the actual Biopsy Slides from the New Jersey Hospital where the lung biopsy was performed so that the Pathology Department at Mt. Sinai Hospital could render a second opinion.

She even offered to make a few phone calls on my behalf to get the ball rolling since my lung power would be exhausted after making just one phone call.  In that regard, she came back the next day with the information I needed and even though it was going to be VERY EXPENSIVE, I had to do it because it didn’t make scientific sense that my body wasn’t responding to the Prednisone given the clear BOOP diagnosis.  Although, some people don’t respond to the steroid treatment and they either die or the BOOP becomes chronic.  I wasn’t crazy about either option but I was intent on doing all I could to help the doctors, help me.  But it was going to take a few months for the actual slides to be transferred and reinterpreted so my actions would not reveal any beneficial information for quite a while.   This is when the doctors brought in a NYC oncologist because they were trying to figure out if a different class of drugs would work on my BOOP, which I was told was a “T-Cell” disease.

Histoplasmosis Test

My 30-year NYC Crohn’s doctor also had me tested for the often fatal Lung Fungal Infection, Histoplasmosis, just to ensure that EVERYTHING possible was being tried to help me.  It turned out to be a simple Urine Test but it took my doctor quite a while to figure that out from a very challenging case he had a year before mine.  This is why I am always so open about my medical challenges as Crohn’s Disease and its treatments can cause some hard to identify medical problems and I want doctors and patients to LEARN from all I must go through.  To that end, I am thankful to that patient who essentially “taught” my Crohn’s doctor what to look for in case of Histoplasmosis.  Thankfully, he survived and was diagnosed and treated in time.

My foray into Chemotherapy and Cytoxan w/ the RIGHT Doctors

Since my NYC Crohn’s doctor knew me so well for 30 years, it was his decision to discontinue and wean me off the Prednisone as fast as was medically safe to do so.  But at the same time he organized a “sit-down” with the NYC pulmonologist and the oncologist and they decided to try a several month course of monthly infusions of a chemotherapy drug, “Cytoxan.”  While the mere word “oncologist” scared me, I also very much liked this oncologist and I also understood why they were considering the Cytoxan after they explained the “T-Cell” analogy to me.  Since I trusted the three (3) doctors with my life, there was no decision for me to make as they made it for me.  I think I learned during the 2011 BOOP ordeal that the best advocate a patient can be for him or herself is in selecting the “right” doctors for him or her.  That doesn’t always mean the “best” doctors but it should always be the doctors best suited to the patient/person and to the task at hand.

I was so sick that I needed some degree of autonomy since I was too ill to be involved on a day-to-basis.  The combination of a Crohn’s Disease expert who knew me for almost 30 years, a compassionate Pulmonologist who understood the systemic connection between my Crohn’s and the BOOP and a very experienced Oncologist who also had worked closely with the two (2) other doctors rounded out a medical team whose sum was much larger than its parts.  I had no time to search for the “best;” I only had the energy to find a core of caring and creative doctors who would listen to me and observe how I responded to each and every treatment. Having achieved that, my work was completed.  I was in their hands and that made my responsibilities going forward very simple.  I just had to be positive and “open” to healing.   The only concern they had was of the unknown side effects the Cytoxan might have on my Crohn’s Disease but since I couldn’t breathe, was in tremendous pain and was having difficulties staying positive about my future, I just did what I was told.  “Damn the Torpedoes” as Tom Petty might have told me.  I then had the first Cytoxan infusion while hospitalized at Mt. Sinai Hospital and was released a few days later once the Pneumonia and Pleurisy were improving.

Perspective – A Patient Tool for Healing

I was soon off the Prednisone and had my second Cytoxan infusion in the office of the NYC oncologist.  He also tested my blood count regularly and I was starting to improve.  Before the second infusion of Cytoxan, I had to mentally map out a plan to try and breathe if I was able to get off the couch and walk 15 feet to the kitchen.  However, a week or so after the second infusion of Cytoxan, breathing wasn’t always on my mind because it was gradually getting back to its “new normal.”  This got even better a week or so after the 3rd infusion but then I started to get severe Crohn’s Disease pain and actually had a dangerous small bowel obstruction for which I again had to be hospitalized at Mt. Sinai Hospital in NYC.  This is when I knew my “perspective” had to be altered because I had dealt with MANY Crohn’s Disease situations and will likely have to deal with many more in my lifetime but not being able to breathe was something which could have killed me so I tried to look at my situation as a lucky one, albeit with some Crohn’s complications.

Dueling BOOP Pathology Reports

I don’t recall if this hospitalization at Mt. Sinai was after the 3rd or 4th infusion of the Cytoxan but it was during this hospitalization that the Pathology Department at Mt. Sinai Hospital had finally received the Lung Biopsy specimens/slides and had issued a written pathology report.  On the day I was to get the next Cytoxan infusion, which would have been my 4th or 5th, the NYC doctors came into see me and told me they had to discontinue the Cytoxan because the Pathologists at Mt. Sinai Hospital did not entirely agree with the opinion of the Pathology Department at the New Jersey Hospital which had initially diagnosed me with BOOP.  More specifically, and it really was a hyper-sensitive analysis, the pathologists at Mt. Sinai Hospital agreed that there were nodules or particles of BOOP in my lungs but not enough to mathematically classify my condition “as BOOP.”

On the one hand, me and my doctors were not very “affected” by this differing analysis because the administration of the Cytoxan had CLEARLY gotten my lungs better when the traditional form of treatment for BOOP (i.e., 60 MGs of Prednisone for 1 year) was ineffective and caused me so many other serious systematic medical problems for which I had to be hospitalized.  But not knowing exactly how far I had recovered from the BOOP, especially since it can be chronic and come back, as it has now in 2013, I wanted at least one more infusion of Cytoxan but the NYC doctors concurred in their opposition given the new Mt. Sinai Hospital Pathology Report, my obvious improvement from the Cytoxan and with all due consideration and respect for the potential toxicity of the Cytoxan such that giving me any more than just the right amount would be increasing the risk of systemic harm to my body.

Let me put it this way:  You know you have a complicated medical problem when you engage very smart and well-intended physicians into a discussion where you are essentially begging them to give you MORE chemotherapy because you want to make sure you fully treat a very dangerous lung condition so that it doesn’t come back.  They listened, I listened, but in the end it was their decision and my breathing was SO MUCH BETTER that I  mentally embraced their decision and start focusing on my Crohn’s Disease small bowel obstruction.  At that exact moment in time, the episode of BOOP, or whatever it was, was over in my mind and I was transferred to the floor at Mt. Sinai Hospital which was exclusively for Crohn’s, Colitis and Inflammatory Bowel Disease (“IBD”) patients.  Even without the actual physical transfer from a pulmonary floor at Mt. Sinai Hospital to a Crohn’s Disease/Inflammatory Bowel Disease floor, I had to make that “transition” in my mind because now I had to contend with some very serious Crohn’s Disease issues and I wasn’t going to be given any sympathy just because I had just somehow navigated my way out of a very dangerous Lung Condition thanks to the knowledge, talent, experience and sheer determination of three (3) very smart physicians.  This was approximately November, 2011.

Medical Background Epilogue

The BOOP and my Lungs were manageable although the capacity of my lungs was clearly limited or damaged either by the episode of BOOP, cumulative damage from the BOOP and/or damage from taking the chemotherapy drug, Cytoxan.  The Crohn’s Disease problems got much worse and I was still going medication “commando” as there was no medication I could take to treat the Crohn’s now that all Biologics were off the table for me.  Long story short, after MANY diagnostic tests in close consultation with my NYC Crohn’s doctor, we found a new Crohn’s Disease surgeon who looked at all the tests, was not at all intimidated by the complexity of my case and he used the tests to map out a surgical strategy that would judiciously treat the diseased parts of my small bowel and also excise all adhesions that were adding to the small bowel obstructions.  It, just like the BOOP episode, was VERY complicated but this new surgeon operated on me in June, 2012, when I stayed at Mt. Sinai Hospital for Seventeen (17) days.   Most people have 21 feet or so of their small bowel but after this last 2012 surgery, I now have only 6 ½ feet.  That “short bowel syndrome,”  in and of itself, causes many absorption and lifestyle challenges but at least I can still eat and I don’t have any sort of stoma (or “bag” for those unfamiliar with the technical terms for such things.)

My NYC Crohn’s doctor thinks the Crohn’s Disease issues may have been exacerbated by the 3-5 monthly infusions of the Cytoxan I took to “beat the BOOP” but he doesn’t think it caused it.  We’ll never know.  However, two (2) casualties as a result of taking the Cytoxan are that my Testosterone Levels decreased to the point where they were almost microscopic.  I had to take Testosterone supplements to help boost it back up as I was experiencing EXTREME FATIGUE and a variety of “man problems.” It is still VERY LOW and barely falls within the “range” of where it should be whenever I get a blood test but all “man issues” are no longer problems.  I can’t afford the Testosterone Supplement so for now, and the foreseeable future, that is my reality.   The other casualty is that my stamina when trying to merely walk on a treadmill or taking a walk in the sun has been greatly diminished.  In fact, a few months ago I tried walking very slowly on a treadmill and I passed out and that brings us back to the road to my Nightmare March 26, 2013 Emergency Room Experience at a New Jersey Hospital as: “The BOOP is back and it’s stone cold sober as a matter of fact.”

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MAW PPP Dec 21 2012

6 responses to “Crohn’s Disease, Humira, FDA, Respiratory Problems & “BOOP”

  1. Great article on the challenges of negotiating the dangers of ERs, hospitals, and the like. The human body is an amazing self correcting dynamo until something unbalances it….We are only beginning to understand the complexity and enormity of our surviving each dayl

  2. Wow! Your story is unbelievable and so true! Please check out our non profit site http://www.nibdinkids.com and my daughter’s story.
    Thank you for your courage it is inspiring!
    Lenore Shapiro
    NIBD

  3. Michael A. Weiss

    Thanks for the kind words. Part 2 will be posted later today or tomorrow and it gets into the 2013 ER experience. I’m glad people are “absorbing” this for what I intended it to be, that is, a chronicling of my experience in a system in which the Patient can ever stop being an Advocate.

  4. Elizabeth Rankin

    Michael:
    You are an inspiration to the “patient, ” patient world of complicated medical treatment and its politics. I faced only a minute smidgeon of your struggles, yet I related to your frustration. Anyone reading your story must understand it is up to the patient themselves to become the project manager to find and get the treatment they need, by organizing their own medical team. Anyone who has to rely on drugs is going to face problems sooner or later. Your story should be used in all medical schools and post teaching courses to reveal how bad the “state and art” of medicine is or has become.

  5. Michael, What an amazing compilation of your history.How you have managed to fight off the ‘dry cleaners’ and get re-directed. Why does the system listen to them, I really feel they are the biggest battle we have [yes have met a couple of his cousins myself] for some reason their diagnosis or lack of, is taken as the gospel truth, when obviously just looking at you would have told a totally different story. I am glad that part 2 is coming, and look forward to reading it. AND amazed that with all that has gone on you are up to writing anything let alone such a moving and monumental story.
    I also look forward to the day, when the system allows for the complicated patient, to recieve prompt and proper care, insteadstrad who gets parked in corridors, stuffed in ward rooms, and that seems to be the amount of treatment we get.Then total amazement if one dares to decide to leave the ignored room, and go home to get better,

  6. Michael, you tell a great story with clarity and humor! I can’t wait for part two…between now and then, please know that I hope and pray that you are feeling better each day.
    PS – 8A

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