“Oh, I get it. You think there’s a chance that sports records could trump the doctor’s oath to care for his patient,” showing the slightest irritation with Larry.
“No, not really. I’m not trying to make light of the situation. I seem to have a strange coping mechanism that immediately searches for humor whenever possible. I apologize for offending you with my stupid remark, but these days when we say ‘immune system’ I’m already thinking about AIDS problems.”
“Apology accepted. The only connection with AIDS—and it isn’t a connection at all—is that we wouldn’t have half the knowledge about the immune system if the acquired immune deficiency syndrome had not been studied so aggressively during the past few years. That’s how we came to better understand the body’s natural defense systems. For what it’s worth, AIDS patients typically have problems with their T cells, but you’re just fine in that category. Your problem relates directly to B cells. The immunoglobulins break down into at least five subparts: A, D, E, G, and M. These letters rearranged spell ‘gamed’ if you want a way to remember them. Your treatment will be infusion with immunoglobulin G antibodies, which we abbreviate ‘IgG,’ ‘IgA,’ and the others are not readily available for replacement therapy, so patients like you do without for the time being.”
“Your tests reveal deficiencies in all categories,” the doctor continued. “IgG is the biggest single constituent by percentage of the antibodies involved in your defense system. Your numbers are below normal in all categories. The good news is we can replace IgG nicely with infusion therapy.”
“Sounds like I’ll have to remember to get a booster shot once in a while,” Larry piped up, again, still short of anything approaching realistic assessment of his problem.
“Since I’m referring you to a specialist, it’s idle for me to say much on this score, however, if this is the problem I think you have, you and I will be meeting a minimum of thirteen times a year. More likely, your care locally will be followed by the oncologist who also serves as our immunologist here in our medical group. But you do have company out there. The frequency of this diagnosis is about one out of fifty thousand in the population, although the actual prevalence of cases, a number that includes undiagnosed and untreated cases, may be one in twelve hundred, thanks to results from studies available from the Immune Deficiency Foundation in Maryland.”
“What happens,” Larry blurted out, “to me if I don’t do anything and just don’t get all excited about it? Look, I’ve been reasonably healthy so far. If this is so bad, why haven’t I developed problems before now,” he added, still showing usual patient preoccupation with denial and unwillingness to get realistic about the diagnosis offered.
“Fair question. Answer number one: without treatment you’ll most likely die of pneumonia, sometime. Answer number two: you’ve been reasonably healthy so far because of your relative youth. That advantage is pretty much used up now at your age, and if you’ve had any stress in your life, and most of us have, depending on what and when, your system could have been weakened further. Even dietary habits play a role.”
Larry visibly turned somber and distant. No more smart coping remarks. In fact, nothing was said for moments. Larry rushed to search his mind for examples of stressors, and it took no time to identify several: the senseless murder of his wife whom he cherished, later followed by his discovery of Maria but losing her consortium after the birth of their child. Even though Maria and Lupe were still living in the Central Valley, just not within his reach, it was something he still had not reconciled in his head and heart.
Moments passed and Dr. O’Connor broke the silence. “Larry, I didn’t mean to pile so much on you in this initial session. I know it’s a lot to absorb, so let’s keep it as simple as possible. As far as a referral, I’m thinking UCSF for the simple reason that researchers there may have a slight edge in the field, but don’t quote me, because I have no basis for making that statement. Stanford or UC Davis would also be perfectly satisfactory for your purposes as well. I’m certain USC Medical School would take a look at you. What’s your preference?”
“UCSF, because they don’t have a football team,” and he laughed a hearty chortle that loosened up both men at that point.
“Okay, it’s done,” Dr. O’Connor confirmed. “We’ll make the arrangements from here and then let you know as soon as possible. Remember, what I have said here is only an EWAG,” he intoned seriously.
“I’m not familiar with medical terms. I’m afraid I don’t understand,” stammered his confused patient.
“That’s not medical jargon. It’s an engineering term from my undergraduate days before medical school. It simply means ‘educated wild-ass guess’,” and O’Connor let loose his own patented howl to cap the meeting. As he departed his office to see other patients, he turned to his patient: “We’ll take very good care of you, I promise!”
A week later, Larry was on his way to the specialist. He didn’t feel comfortable trying to find his way through the streets of San Francisco, so he took a cab from his downtown hotel to Parnassus Avenue. Ironically, the driver was a recent immigrant from Russia who depended on Larry for directions to the hospital. Puzzling through the tricky intersections, they eventually found the high-rise buildings and vast sprawling campus of the University of California Medical School–San Francisco. The arches of the Golden Gate Bridge appeared close enough to touch, that is, when the fog wasn’t obstructing their view. Typical weather for “the city” in August—cold, windy, and foggy, punctuated occasionally with a brief splash of sunshine to remind the locals they were indeed living in California. This afternoon was credible evidence of Mark Twain’s memorable quip: “The coldest winter I ever spent was a summer in San Francisco.”
The cabbie stopped directly in front of Larry’s destination, and he exited the taxi after paying the driver. The breeze was maybe fourteen or fifteen knots and slightly bit his face. Refreshing, he mused as he tried to forget the 100-plus degree temps in the Valley this time of the year. He pulled his windbreaker closely around his shoulders and zipped it to the top. Much better. He began to get his bearings as he visually toured his surroundings. It was a very pretty setting for such a large urban health center.
By now, he was aware that the entire medical community of San Francisco was beleaguered by the huge number of AIDS-related cases in desperate need of treatment. But he realized the research done for the benefit of AIDS patients also helped discovery and treatment of symptoms of his condition, that he would soon learn was called “common variable immune deficiency,” or CVID for short, which is a subcategory of his general diagnosis of Primary Immune Deficiency Disease, or PIDD. This insight did not settle in immediately, however, as he had so much to absorb over the coming months. Understanding of his own diagnosis and the development of his lifetime treatment regimen would consume a good bit of his concentration for many of the ensuing days. Ultimately, he would come to learn that his suffering was a ticket to deeper appreciation and greater empathy of the plight of many others whose problems had never seemed to matter or even interest him very much.
Larry pulled out his instructions and found the directions to the office of his specialist who was located inside a tall building across the street from the hospital. This was a very busy institution, unlike anything he had experienced in the past. People of all descriptions were everywhere. The ones moving the slowest were likely patients, while the ones nearly running everywhere were professors and students of medicine, nursing, and a huge assortment of allied professions, including research.
Once he had located the office of his doctor, he was redirected by a receptionist to still another floor in the same building to fill out numerous forms aimed at getting his medical history and insurance coverage information completed and into his chart. Thank God, his company had a strong group health program for all the employees. As it would turn out, Larry would learn soon a lot about health care reimbursement programs and their administration, another area previously untap
ped as a consequence of his stellar health history.
Totally unaware of the protocol and the plan for his introduction to care at UCSF, Larry spent a good portion of the morning and half the afternoon giving blood samples, having his vitals checked and re-checked, and blowing into a hose for a respiratory technician who seemed overly interested in pushing him beyond his limits. Well, what the hell, all in the name of good science. It was later in the afternoon, about a quarter past four, when he was invited into Dr. Aaron Weiss’s office to make the personal acquaintance of his doctor. First impressions are important, and his feelings about Dr. Weiss were nothing short of sensational. Larry listened carefully as the doctor explained the diagnosis and treatment plan.
“Mr. Martorano,” the doctor intoned amicably, “I’m satisfied that based upon the results of all our studies today as well as your history reflected in your chart that I reviewed, that you have the condition we refer to as primary immune deficiency disease or PIDD as we abbreviate the term. In so many words, a portion of your body’s natural immune system is no longer functioning. Medical science has yet to discover the cause of this anomaly, although we are most suspicious of genes from your ancestors in your case. If we thought, for example, that something outside your gene pool was the cause, we would substitute the term ‘acquired’ for the word ‘primary’ that we now use for your diagnosis. Frankly, most of our cases of primary immune deficiencies are diagnosed during childhood. If your situation was extremely serious, and that’s not said to minimize your diagnosis by any means, you might be a ‘bubble baby,’ an extreme and singular example of the most difficult case. In such situations, the patient is destined to live in a highly controlled environment to minimize exposure to harmful bacteria and viruses, since that type of patient has essentially no natural protection afforded by his or her immune system.
“Acquired immune deficiency is the term we use if we conclude that the condition is caused by factors outside your body. For example, many times cancer patients develop acquired immune deficiency as a result of the treatment modalities they undergo. You have not been treated for cancer, to my knowledge, and we can rule out that possibility. We think that stress and prolonged emotional upset are very taxing on the immune system. I am always suspicious of short illnesses that result in death. I can’t prove it yet, but my theory is that if an otherwise healthy person is lost in a relatively short time, then some unexplained force overcame the immune system first without discovery. Then death follows shortly.
“But, let’s focus on you for the moment. Your nurse will be giving you a booklet that explains the disease. It’s a useful explanation. You are attentive, and I’m already convinced that we will make a good team working on your illness.”
“Sorry, doctor, for interrupting, but isn’t there a treatment I can complete in a few months and be on my way,” and Larry leaned forward with an eager and expectant look.
“I wish I could say yes to that question, but it’s not possible. Your diagnosis is permanent. The best that medicine has to offer at this stage of development are treatment plans involving something we call infusion therapy, but I’ll have more to say about that in a moment. I don’t want to get off track in my introduction of the subject.
“You see we have tested your immunoglobulin levels for comparison purposes, and I’m talking about the number of antibodies found in normal immune systems. AIDS patients have a much different issue. They have problems with their T cells, which is definitely not your problem, thankfully. To make it even more interesting—or complicated—there are at least five subsets of immunoglobulins found in persons with full complements of antibodies. I don’t want to overwhelm you in this initial meeting. I will say that your treatment will involve replacement of immunoglobulin G; very little can be done about replacement of the remaining portions, depending on your particular numbers. And it’s worth noting that your treatment is preventative rather than curative. This notion focuses on the fact that you are antibody deficient rather than diseased. With any luck, we’ll keep you free of disease through this preventive treatment, and you’ll live a long and productive life.”
“Okay, I think I’m beginning to get the idea. I take a bottle of this cell replacement medication with me when I go home and call you once every six months for a status report?” Larry announced rather too positively.
“Hold on! I’m just getting to the treatment program,” voiced a tired and minimally vexed doctor at the end of a long day, almost snapping at his patient. Larry was at once not only sullen but sorry and disappointed in himself. He knew better and offered a meager but sincere apology to Dr. Weiss.
“It’s not about hurt feelings and apologies. I simply want to get you up to speed on this antibody deficiency challenge and provide you with enough information to prepare yourself for a lifetime of specific treatments that average once every three or four weeks.”
“For the rest of my life?” Larry heard himself saying almost in a whimper. What is going on? he demanded silently of no one but himself and offered a more sorrowful and heartfelt apology to the exhausted physician.
“Look,” Dr. Weiss continued in as relaxed a mode as he could muster, “there’s one more test that will be ready for me to review by 5:30 this afternoon. I know your home is a long way from here. I’ll stay a bit longer this evening so you won’t have to come back for an extra meeting with me. Why don’t you take a break, maybe grab a snack in the cafeteria, and come back here at 6:00. I’ll be ready to wrap it up and let you know what comes next.”
Larry found himself out in the hallway, then on the elevator, and then on the street without giving the quick trip a second thought. Not a very smart approach as it would turn out, because the patients were on their own to find their way around this institution with its myriad facilities that included examining, treatment, and research rooms of all kinds. He realized the doctor had mentioned getting a snack, yet eating was of no interest. Once on the street, he found the weather vastly improved; the sun was shining, not a cloud in the sky, or at least there weren’t any visible from his position. He shuffled nowhere in particular, aimlessly, before he was attracted to an open patch of manicured grass near the entrance to his building. Flopping down nonchalantly, he took a seat in the middle of it. He was feeling relaxed enough, yet he was not able to shake the agonizing feeling of incompleteness. He sensed the presence of something he had never experienced with this acuity before. He could not concentrate and felt helpless to fashion a plan of counterattack to wipe out the aggressor bugs, as he could if he were dealing with a choice of pesticides to apply to a cotton field.
He was not feeling sorry for himself, since he had not reached that predictable stage quite yet. His head was rushing with thoughts, but his body was most sluggish. He turned to face a person he suddenly realized was watching him from a short distance. As he looked at the individual, he determined her gender but could not make identification. The sun was lowering in the western sky over her head, which interfered with his view of her face.
“You don’t recognize me, do you?” said the person, who stepped onto Larry’s perch and settled in next to him.
“Oh, now I see,” he said, mostly to himself, and he remembered she had helped check him in to see the doctor at the beginning of his life-changing day.
“I’m very sorry to seem so out of it,” he continued. “Sure, I remember you—from Dr. Weiss’s office, right?”
“Yes, that’s correct, and you owe no apologies. You’re going through so much right now. Mind if I sit here a moment?” Without waiting for approval or an invitation, Chelsea Whiting, RN, settled in to offer support in this time of Larry’s deep hurt and need.
“It’s a lot to handle in a short time. Are you okay? Can I help in any way?” she offered.
“I … I … I really can’t seem to figure out just what to think,” Larry admitted. “I’m usually in control and take charge without a second thought. Now things are totally upside down. My nerve endings seem to have gone numb.
”
“Nothing unusual about that,” Chelsea volunteered, “all of our patients hit the wall at some point. You’re precocious, it seems. Want to talk about it or ask any questions?”
“Thanks, for the offer, but I’m embarrassed to say that I really don’t know what to ask, even if it seems like a good idea. Do you folks see lots of patients for this problem?” he said, starting to regain some ability and interest in trying to refocus his curiosity.
“Yes, we do, but not huge numbers, mind you. I’ve heard estimates that immune system disorders like yours affect one out of fifty thousand people. That may be accurate or just an estimate based on local demographics, yet it seems consistent with our experience with the numbers we have around here.”
“Do the patients … are we patients … how do they manage with this situation?” now getting into the conversation with Chelsea.
“Most of our patients do very well once they achieve a solid understanding of their condition and what it requires to stay as healthy as they can,” she said, trying to sound encouraging.
“Really. It sounds as though there is some risk or at least a challenge involved in going forward with treatment or maintenance or whatever.”
“That’s right to some extent. You see, everyone is just a little different from the next patient. There’s no good reason to explain all the possible complications, since we’ll know more about your case once you’ve finished a couple treatments. Some people, as you might expect, have problems with needles, and they may involuntarily resist the infusion procedure. Permanently installed infusion parts are sometimes required. Others take the needle but need other meds to tolerate delivery of the immunoglobulin. You know: rashes, headaches, and such. But I have to brag a little, our nurses are up to the challenge, and we are able to find a proper rhythm of treatment for just about every patient.”
(2012) The Court's Expert Page 10
