Guess who’s in the waiting room? the charge nurse sings out in a high-pitched voice as she walks into the emergency department. Without waiting for our guesses, she answers herself: Livinia!
A chorus of doctors’ and nurses’ voices erupts from the work station.
Her again?
What a wimp.
But I just saw her here two weeks ago.
Oh, no--not Livinia!
I’ll admit it--this is unbecoming conduct for health professionals talking about a patient, particularly one with a serious chronic disease. And if you were to meet the person whose presence provoked this outburst, you’d be even more appalled. Livinia Johnson is a perfectly pleasant, well-mannered and well-dressed 29-year-old black woman who works as a computer programmer and is a voracious reader of science fiction in her spare time.
But Livinia Johnson also suffers severe recurring bouts of pain from the distressingly common and untreatable condition called sickle-cell anemia. As she’s wheeled by the glass windows of my station, a grimace on her face and a sci-fi book in her hand, I am filled with a mix of familiarity and dread. Here we go again, I say to no one in particular.
Although research offers hope of someday curing sickle-cell disease with bone marrow transplants or gene therapy, for the moment all we doctors can do is offer Band-Aids: treat an infection, try to make a dent in the incredible pain. It’s medicine at its least satisfying--for doctor and patient. It’s why seeing Livinia here again frustrates us. It’s also why she’s always here.
In sickle-cell disease the normally supple, round red blood cells harbor a defective protein that distorts and locks them into an unusual shape--the shape of a boomerang. These defective, sickled cells are unusually stiff and fragile and have a shorter life span than normal red blood cells. Thus people with the disease are almost always anemic, and the scarcity of oxygen-ferrying red blood cells means they live with fatigue and shortness of breath.
In addition, the stiffened cells can’t squeeze through small blood vessels as deftly as normal cells. From time to time they get stuck, blocking blood flow to an organ, muscle, or bone. Starved for oxygen, the tissues begin to suffocate, and as their cells die they give off signals that the nerves interpret as pain. But this is no ordinary pain. It’s the same kind of pain--from the exact same cause--experienced by someone undergoing a heart attack. Some have likened its severity to the pain of childbirth.
Livinia’s pain is usually in her lower back, stomach, or knees. This time is no exception: the nursing note on her chart says she’s complaining of severe stomach pain.
Hi, Dr. Rosenthal, she greets me wearily from the stretcher when I walk into the room. How’s your little girl?
She’s fine, thanks, Livinia, I answer. How are you? Not so great, I guess, if you’re here.
Over the seven years of our sporadic doctor-patient relationship, Livinia has seen me through medical training, marriage, and pregnancy and is now watching me snail my way up the academic chain. I have seen her change boyfriends, move away from her parents’ home, finish college, find a job. A few years ago she asked me to call her by her first name.
But now she’s not much in the mood for small talk. She tells me that her pain started during the night and even though she took the narcotic pills she had at home, she couldn’t stand it anymore. I was taking Percocet all night long, but my stomach’s getting worse and worse, she says, her eyes filling with tears.
Quickly, I go through a series of questions--questions I’ve asked and she’s answered well over a dozen times:
Any fever? (No.)
Drinking and eating okay? (Fine until last night.)
Any sign of infection--cough, sore throat, burning sensation when urinating--before you got the pain? (No.)
Although these painful crises can arise out of the blue, they’re often precipitated by some sort of physical stress, such as a cold or other viral infection or dehydration, which can make it harder for the already- crippled red cells to flow though the blood vessels. However, that’s not the only reason for watching out for an infection. In a patient with sickle-cell disease the spleen--which normally helps the immune system by filtering bacteria from the blood--is often damaged by repeated bouts of vessel clogging and so has difficulty fighting off certain germs. So when there are signs of infection--such as a fever or an extremely high white blood cell count--these patients are almost always hospitalized.
Livinia doesn’t have a fever. I take some blood to count her white cells and a few other things, but there is no sign whatsoever of infection. Therefore there’s no need to admit her to the hospital; Livinia should be able to weather this crisis in the emergency room with just some pain medicine, oxygen, and fluids. We give oxygen so that the suffocating tissues have the best possible chance of grabbing some of the vital gas. We give lots and lots of fluids to keep the blood thin and flowing as freely as possible and to prevent dehydration.
The reason for the narcotics is obvious. What do we normally give you for pain? I ask Livinia as the nurse slips an oxygen mask over her face and struggles to get an intravenous line in her arm. She has had so many needles in the past that her veins are deeply scarred.
Demerol, 125 milligrams, and Vistaril, 50 milligrams. All sicklers, as we call these patients, know by heart the pain cocktail that works best for them.
Okay. Just hang on for a few minutes. I’ll go write an order and the nurse will give you a shot.
The work station is filled with doctors and nurses when I sit down to order Livinia’s drugs.
You’re giving her 125 milligrams of Demerol? one doctor asks as he peers over my shoulder. I don’t know. She sure looks good to me.
I think she’s faking, a nurse weighs in. Out in the waiting room she was writhing in pain, but when I just passed by her room she was reading.
I’ve already written the order, but their comments give me pause. I decide to sneak a quick peek at Livinia’s card before handing the order sheet to the nurse.
One of the most often used pieces of equipment in the emergency room is a green plastic file box overflowing with dog-eared four-by-six- inch index cards. Each card contains the name of a patient who has been treated repeatedly in our emergency room for chronic pain, along with the drugs (generally narcotics) and doses (generally large) that have been employed to fight that pain. Each line documents a separate visit; some of the patients’ records are now five or six cards long. And while a few of the patients suffer from migraines and a couple suffer from a bad back, the lion’s share are sicklers.
Livinia’s cards detail her decades of pain. I flip through to the last entry: Demerol 125 mg/Vistaril 50 mg. Just as she said. I feel guilty for checking--after all, if a suffering patient told me he normally took 125 milligrams of an antibiotic for an ear infection or 125 milligrams of a heart medicine for chest pain, I would give it posthaste. So why must sickle-cell patients run into this wall of doubt when they’re in pain and asking for help?
Part of the problem is that doctors and nurses are trained as scientists--and there is still no way to measure scientifically the agony caused by sickled red blood cells. I can look into an ear and see a reddened drum. I can scan an EKG and determine that a heart needs more blood. But when Livinia tells me she’s hurting, there is no blood test I can do, no body part to poke or probe or palpate to convince me that this is indeed the case.
All I can gauge is her reaction. Some of the nurses feel that because Livinia can sit up and read while she’s in the emergency room, she can’t really be hurting. As for me, well, I’m not sure. Does Livinia’s reading necessarily mean she’s not in agony? Perhaps the plot diverts her attention from the searing pain within. But when I give the drug order to the nurse, she tells me she’ll give the injection after she’s finished a few other chores and then ribs me, calling me a pusher and an easy mark.
The dilemma of how to treat sickle-cell patients in crisis is compounded by the fact that the drugs that help them are almost all potentially habit-forming narcotics. Throughout our education, doctors and nurses are warned about the addictive potential of these medications; we’re admonished to use them sparingly. But sicklers need them often, and they need a lot.
Certainly those of us who work in an emergency room have seen more than a handful of patients who were probably addicted to the shots, or at least really enjoyed their brief forays to the hospital. That became quite obvious three years ago when a powerful non-narcotic pain reliever called Toradol came on the market. We tried, at my hospital, to use it to dampen the pain of sickle-cell patients in crisis. While a few patients said it did indeed help, others refused even to try it, immediately claiming Toradol allergies.
In the United States, sickle-cell disease is found almost exclusively in blacks. (It’s found in nonblacks in the Middle East and India.) And in this emergency room, because of both the nature of the disease and the nature of the neighborhood we’re in, it’s seen most often in young, poor blacks--the very same population in which we most worry about narcotic addiction in the first place.
Add all this up and it becomes way too easy for jaded doctors and nurses to dismiss a young sickle-cell patient as a faker just out to get drugs. In fact, there is so much bias against sicklers that some hematologists say they dread having to admit their patients to the hospital.
Here is this wonderful man who works hard despite having a terrible disease, I remember one such doctor commenting to me as he signed in a 30-year-old sickle-cell patient with pneumonia. He’s a successful, well-respected professional who hates taking pills unless he’s in real trouble. And then he comes into the hospital and he’s treated with disdain and suspicion, like a junkie. It’s so unfair.
Two hours after her first dose of narcotics, Livinia calls to me as I walk by her room. She says she’d like another shot; she’s in pain again. It’s a little soon, but it’s not unusual for a sickler to need two or more shots. They’re rarely rid of their pain with just one. And Livinia is wincing as she sits up in bed--though she still has that damn book open in her lap. As I write the order for more pain medicine, I can’t help hoping that she puts it away before the nurse arrives.
After I’ve ordered her second shot, her lab work comes back. Her white blood cell count is slightly up--though not high enough to indicate infection. The rise is no doubt due to the tissue inflammation that is causing her pain, and to the stress of being in such pain. Her creatinine test of kidney function is 1.8. Creatinine is a waste product of metabolism that’s cleared from the body through the urine--if there’s a lot left in the blood, it means the kidneys aren’t working right. Normally a person Livinia’s age would have a level of below 1, but 1.8 isn’t bad for her. People on dialysis, by comparison, often have creatinine levels in the 8 to 10 range. But it is an indication that those sickled red cells have damaged her kidneys over the years, and it’s something to keep an eye on. Her hematocrit--the percentage of red cells in her blood--is 28 percent, way below the normal spread of 37 to 48 percent but again normal for a person with this dread disease.
Livinia is actually pretty lucky for a sickler. Many have had to watch their internal organs die piece by piece, bit by bit. One young woman I see frequently has severe heart failure--those sickling blood cells have too often cut off circulation to the muscles of her heart. Another patient has two artificial hips: the curling cells shut off blood flow to the joints, killing the bone. We often wind up with two or three sicklers in the emergency department at once, and they often compare notes. It must scare Livinia to hear the stories. Or to stop hearing them. Though sickle- cell patients are living longer than ever, infections and blockages of major organs like the heart, lungs, and kidneys still leave them with a shorter than normal life span.
If only Livinia’s pain would break, I would be able to send her home. But that’s not always possible. About half the sicklers with painful crises end up getting admitted. We have a loose policy: three rounds of narcotics or eight hours of treatment and then patients either go home or come in. On the wards they will continue to cycle between pain and narcotics. Some hospitals--though not mine--now use patient-controlled narcotic pumps. With the push of a button, sickle-cell patients may give themselves a dose of painkiller. It spares grown men and women who are already sick the further humiliation of begging for medicines and waiting while in pain.
Seven hours after she first walked in the door, Livinia shuffles toward the nurses’ station. I watch her--she’s one of those patients who have been around hospitals long enough to know just how to carry an IV bag. She is clearly feeling better, but as she pokes her head into the work station she says, I think I need one more shot. Then I’ll be ready.
Does she still have pain? Should I give her another shot? Does she just want another injection as an insurance policy, to make sure the pain won’t return when she gets home? Wouldn’t I want one last shot if I were in her situation?
I have no reason to believe that Livinia is addicted: she’s working and on stable doses of drugs. And anyway, I’ve decided for myself that I would rather give narcotics to someone in the emergency room who’s not really in physical pain than withhold drugs from someone who’s truly hurting.
So I write the order for another injection. An hour later Livinia is at the door, dressed and ready to go. She thanks me for her care. I wish her good luck in her quest for a job promotion. I like her. I believe she was in pain. I know she has a bad disease. And yet I make sure to update her card in the battered green box: Demerol 125 mg/Vistaril 50 mg, times 3. No infection. Discharged with 25 Percocets to go. Probable painful crisis.
