By Brad, CVID, United States, December 28, 2018
Twenty-one letters… all one word
“I don’t want to alarm you, but are you bleeding from anywhere?” Those were the words that came across the phone that would change my life forever. Guardedly, I respond, “Ummm… no?” The P.A. I had seen for the very first time only two days earlier for my physical exam tells me she needs to see me again. I ask her to let me check my schedule, “No, now! I need to see you right now. Get in your car and come to my office.” So much for not alarming me.
I drive to her office, we discuss my CBC results and the fact that all of the values were outside the normal ranges. At some point in the conversation, I mentioned that some 20 years earlier I had been told by a doctor that I had funny red blood cells but not to worry. My P.A. asks me why I had remembered that conversation and I tell her because he had asked me a funny question. She inquired what he had asked me and I told her he had asked me if I had any Mediterranean relatives. “Oh, I know what you have!” Beta Thalassemia Minor, a fairly benign, genetic form of anemia… but we will get back to the “fairly benign” part a little later. Relief! We know what I have and it is not life threatening. There may have been some more information presented to me by the P.A., but I deemed it unimportant as we had our answer on the bleeding question.
My P.A. refers me to follow-up with an Immunologist, which seemed odd, but I agree and went home. Now, some history might be in-order at this time. By this point in my life, I had really stopped seeing physicians for the better part of a decade. I would make the occasionally appearance if my hip flared up from working out, to get the annual physical to get my matching HSA dollars or if I knew I had Strep throat, but aside from that I had long ago thrown in the towel on physicians. I was simply tired of them not really being able to help me. I’d go in with one of my obvious, insidious, reoccurring sinus infections and they’d throw a few days of a worthless antibiotic at me and… nothing. I’m still sick. Doctors are worthless.
So, I’m about 20 minutes into my grilling by the Immunologist and after multiple reviews of both my sexual and drug use history I finally interject, “OK, I have this genetic form of anemia, Beta Thalassemia Minor, why do you think I have AIDS?” He chuckles, “Well, I don’t think you have AIDS, but you are incredibly immune deficient and I don’t know why.” Unclear what he just said, he regains my attention and I focus more intently. He presents my results that I had previously deemed as unimportant with my P.A. and he indicates he wants to do some more blood work.
Twenty-two vials of blood later and several weeks pass. The immunologist’s office calls and I head in to get my results. Much discussion ensues… explanations of the many known forms of immune deficiency, that not all can be definitively confirmed by testing, differences between primary and secondary immune deficiencies, a lot of acronyms, explanations of antibodies, etc., etc. In the end, I’m told something about a genetic form of immune deficiency, something about significantly IgG deficient, significantly IgE deficient, and completely IgA deficient and something about IV infusions and something about something and something else about something and… and… and… I’m lost… I check out. Mentally, emotionally and spiritually, I check out. The physician leaves the room and a member of his staff comes in a few minutes later. She hands me a piece of paper and caringly says, “I’m sorry for your diagnosis.” Sorry? No one had ever apologized to me for a diagnosis before. It left me feeling empty. She had to know something I didn’t know. I went to my car and looked at the piece of paper. Hypogammaglobulinemia. Twenty-one letters… all one word. This could not be good. I’m thirty-nine years old, I have a five year old daughter and my wife is pregnant with our son. I sat in my car and cried.
Now, over the course of the prior year or so, I’d been having some mental health issues. Stress at work was a major factor. There had been a couple of trips to the emergency room with panic attacks. Depression was becoming my constant companion… a companion I had sauntered with several times in my life. My teens had been filled with heavy alcohol and drug abuse, in part, to self-medicate. I’m 20+ years clean and sober at the time of my diagnosis… I’m a one day at a time guy, but a guy who is struggling. I am primed for the two years that are about to follow, but I am unaware just how dark my life is about to become and the people I’m about to pull down with me. I free fall into the abyss that is clinical depression coupled with suicidal ideation. I refer to it as the death spiral. For the next two years, there would be nothing but darkness and the ever-present desire to end my life.
At the time it made so much sense, I’m going to die so I might as well kill myself. Regardless, my kids are going to be fatherless, my wife a widow but I don’t want them to watch me go through this. Hell, I don’t want to go through this. I don’t really know what “this” is, but I do not want to live to find out. My wife, a small animal veterinarian, earns a good salary. My life insurance would take care of the kid’s college savings plans and leave some additional cash. It just makes sense… right?
Every two or three minutes, the relentless tape would repeat in my head, “Brad, you should just kill yourself.” I’d pace the first floor hallway eyeing the banister at the top of the steps. How much rope would I need? I would have to tie it off multiple different directions to insure it wouldn’t break when I jump. I would fix the ropes in the door jams of the bedrooms and tie them off to the banister. I’d have to reinforce the banister at both the top and bottom. How much rope would I need? I can’t mess this up. I wondered why automotive manufactures ever invent airbags. It would have been so much easier back in the day. Days of planning turn into weeks, months and years. I was desperately looking for the one reason to justify my end. I was in the death spiral… and I was sucking everything into it with me. I pulled my wife, my kids, my friends down with me. It was clear by this point that there would be no recovery.
I know I’m in trouble… everyone knows I’m in trouble. There are two disability leaves of absence at work due to mental health breakdowns. Upon return from my second leave my boss tells me that this can’t happen again. That was helpful! There is a weeklong sabbatical to a psychiatric hospital. They will only accept me as a day patient because of my PIDD diagnosis… they don’t understand it and they are scared. Twenty-one letters… all one word. I’m so defective I can’t even get accepted in the psych ward! There is medication, changes in medication, further changes in medication and alas, more changes in medication. I’m broken! My wife acknowledges latter she wanted to put the kids in the car and leave. She should have, but I’m so grateful she didn’t because I truly would have had no reason to live.
I meet every Friday morning with a guy I call my spiritual advisor… he is another one of those one day at a time guys. One morning he looks at me, holds a piece of paper in the air and says, “I can’t help you, I don’t know how to help you but I think this guy might be able to help.” On the piece of paper is the name and number of a psychiatrist. Months go by while we shift medications away from the antidepressants towards mood stabilizers. Initially, things get worse as coming off of the antidepressants is difficult at best. The mood stabilizers start to take affect and things, well, start to improve.
I don’t recall the exact moment, but suddenly, something changed. I came to realize that I am going to die, that I have no control over that fact and, most importantly, that it is OK. It seems so obvious now. Rudimentary truths of life that up until that point I had been unable or unwilling to internalize, accept, and embrace have been incorporated into my psyche. In the end, I had to take death, wrap it around me and accept it as a friend. I recognize that the end is likely not going to be pretty for me, but then death is rarely, if ever, pretty. I realize that dying is easy. Now the real dilemma hits home and I’m left with the enormous question of how to live?
So the years went by and I came to understand that I’m incredibly fortunate. I live a fairy “normal” life. I feel like Lou Gehrig or Napoleon returning from Elba… life is good. I fall into a routine with my infusions. My kids are growing up. I’ve tried to repair my devastated relationship with Mary, my wife, as best I can. I’ve lost some friends but come to realize that those events may not been losses after all. That dilemma of how to live life is working itself out. I’m having to work hard at living and when I fail to put the effort forth the results are usually less than ideal. It is beginning to look like I may just survive this thing called life after all.
“Oh my God, Brad, you are blue!” These are the words the nutritionist at the gym uttered as I stumbled into her office and collapsed. I had been on the stair climber and something had happened. My pulse was likely 200+, respiratory rate was rapid and involuntary, chest pain and disorientation… the room was now spinning and closing in on me. It was over I thought, I’ve caused myself to have a heart attack. If I live through this Mary is going to kill me. I’d been going to the gym doing cardio consistently for years but could not run a mile if my life depended upon it. It was the anemia I thought. I just don’t have enough oxygen caring capacity. I had looked at Mary just three weeks earlier and literally spoken the words, “I’m going to get my cardio capacity up if it kills me!” But for now, it’s a one-eighth of a mile ambulance ride across the street to the hospital. It feels as if my understanding with death was dissolving.
Forty-five year old male collapsed at the gym complaining of chest pain and shortness of breath… or something close to that was barked out as they rolled me through the double doors at the ER. Yes, this is the phrase that will get you the attention you always hope you will get at the ER, but never seem to receive. No one stuck a clipboard in my hands, told me to go have a seat and fill it out. No HIPPA form, no insurance card and driver’s license necessary. “We’ll be right with you.” Nope, this is pure action. One nurse is on my arm drawing blood, another is sticking electrodes to my hairy chest, and the third is running through the boilerplate list of questions. Medical history… why yes, I do have one! I start blurting out my conditions and medications. I can see the nurses are looking at one another inquisitively. As is the norm, they are about to get a lesson in genetic conditions. “Can you spell that for me?” Why yes I can, twenty-one letters… all one word. EKG’s, troponin levels, ER physician and, well, everything is normal? I’m told they will hold me another four hours and rerun the tests. Mary and the kids show up, I tell them to go home and I will call them when they can come pick me up.
Four hours pass and my chest is still sore but I’m ready to go home. The final EKG is normal and we are just waiting for that troponin level to come in so I can call Mary and go home. Elevated? Then, the ER physician speaks the words, heart attack. A Heparin drip, another ambulance ride, another hospital, ICU, and more nurses follow. “Can you spell that for me?” Why yes I can, twenty-one letters… all one word. I settle in for a sleepless night. In the morning it is an angiogram.
The ultrasound technician comes in and does his thing first thing in the morning. He is rolling his cart out of the ICU room while the Cardiologist is walking into the room. “Anything relevant,” I hear the Cardiologist inquire. “Aortic valve,” the technician responds. The Cardiologist introduces himself and explains the procedure all the while providing me statistics that don’t feel very reassuring. They roll me into the room and inform me that they perform the procedure radially now which I find more amiable than working, well, down there. Then they inform me that they still prep the groin in the event that radial access isn’t good enough. Are those clippers? The nurse anesthetist, Susan, with pretty eyes above her mask never diverts her attention from our conversation. Good thing because while I’m still unclear if I have Mediterranean relatives, I’m pretty sure I have some Irish lineage.
I’m back in my ICU room following the angiogram and Mary and the Cardiologist walk in together. The Cardiologist, in a very confident and nonchalant manner proclaims, “Good news, not a heart attack, arteries look good, no blockages, we are going to have to replace your aortic valve… no big deal.” No big deal, I think?
My cardiac surgeon would not proceed until both my Immunologist and a Hematologist signed off on the surgery. My Immunologist’s biggest concern was pneumonia. We would have to closely monitor/manage for pneumonia, but he didn’t hesitate to approve. Now, Hematology was a different story. Mechanical heart valves are designed for nice, big, round, slippery red blood cells. Due to my anemia, my red blood cells are ugly, vary greatly in size, are almost square with pointy corners and, as such, are not very slippery. After a long exam and consultation with the only Hematologist my cardiac surgeon trusted, she approved surgery stating that I would, “probably be fine.” I knew “probably” was the best I could hope for given my condition.
Prior to surgery, one of my wife’s twenty-something colleagues asked me if I was scared. I emphatically said, no! She looked at me questioningly and I told her that no matter what happened, it was going to be OK. I was going to be OK. Don’t get me wrong, the five weeks between my cardiac event at the gym and the surgery were intense, but very little fear crept into my life.
I encountered very few complications post-op, but it was a challenging four to six weeks none-the-less. After 6 months I was pretty much back to “normal.” I feel my experiences had prepared me for the challenges that open heart surgery presented… or as much as anything could have prepared me.
Today, I live with two conditions that the CDC designate as rare which means they occur in less than 1 in 50,000 people. Aortic valve stenosis occurs in approximately 1 in 50 people. The odds of having all three conditions is 1 in 125 billon while it is estimated that in the history of the world there have onmly been 107 billion people to have ever lived. I live life as best I can. Some days are better than others. I say I’m good with my health status about 93% of the time. There are still times where the realities can seem overwhelming, but they occur in small doses and I have the tools to be able to manage those low points. It isn’t always pretty, but much like death, life isn’t always pretty.
I’ve met some great physicians over the years and I’ve met some physicians who seem beaten down by the profession. The healthcare field as a whole is woefully unprepared to deal with mental health issues. It isn’t their fault, but it is their responsibility to address. I’ve learned to pick my physicians like I pick my friends… are they good people and do I think they can put up with me and all the baggage I bring with me? I still give lessons in genetic conditions to both physicians and nurses alike. “Can you spell that for me?” Why yes I can, twenty-one letters… all one word.