‘On That Edge of Fear’: One Woman’s Struggle With Sickle Cell Pain

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NASHVILLE — She struggled through the night as she had so many times before, restless from sickle cell pain that felt like knives stabbing her bones. When morning broke, she wept at the edge of her hotel-room bed, her stomach wrenched in a complicated knot of anger, trepidation and hope.

It was a gray January morning, and Lisa Craig was in Nashville, three hours from her home in Knoxville, Tenn., preparing to see a sickle cell specialist she hoped could do something so many physicians had been unable to do: bring her painful disease under control.

Ms. Craig, 48, had clashed with doctors over her treatment for years. Those tensions had only increased as the medical consensus around pain treatment shifted and regulations for opioid use became more stringent. Her anguish had grown so persistent and draining that she sometimes thought she’d be better off dead.

She was willing to try just about anything to stop the deterioration of her body and mind — and her hope on this day in January 2019 rested in a Nigerian-born physician at Vanderbilt University Medical Center who had long treated the disease, which mostly afflicts people of African descent.

That morning, she slipped on a cream-colored cardigan and a necklace with a heart-shaped pendant. She played some Whitney Houston before sliding behind the wheel of her black S.U.V. Her husband, in the passenger’s seat, punched their destination into his phone’s navigation system.

“Live as if everything is a miracle,” reads a framed quote on Ms. Craig’s beige living room wall, and that’s exactly what she was hoping for.

People with sickle cell, a rare, inherited blood disorder caused by a mutation in a single gene, typically endure episodes of debilitating pain as well as chronic pain. Roughly 100,000 Americans and millions of people globally, mostly in Africa, have the disease. Red blood cells that carry oxygen become stiff and curved like crescent moons, clogging blood vessels and starving the body of oxygen.

Promising developments in gene therapy have given people with the disease hope that a cure is on the way for an illness that often causes organ failure and premature death. But the first such therapy is more than a year from regulatory approval. It will almost certainly be extremely expensive, cannot reverse the disease’s damage to tissues and organs, and may come too late for people whose bodies are so battered by the disease that they might not survive the grueling treatment.

Most people with sickle cell are searching for something far more basic: a way to prevent or manage the disease’s devastating complications — strokes, depression and, above all, pain.

That search can be rocky, as I learned following Ms. Craig over two and a half years of struggle and heartache. I joined her on doctor’s visits, shared meals with her family, parsed her medical records, sat in on a therapy session and tagged along as she ran errands around Knoxville and relaxed at home. I saw moments of anger, sadness and agony, but also determination, joy and love.

Her efforts to find relief were complicated by a national opioid epidemic and the coronavirus pandemic, as well as the challenges of navigating a medical system that often mistreats Black people like her. At the same time, doctors were changing how they treated sickle cell as emerging research suggested that narcotics could actually worsen pain.

Ms. Craig felt doctors were prone to stereotyping her as an addict cadging narcotics and didn’t believe in the extremity of her suffering.

Racist myths persist in medical care, like the idea that Black people tolerate more pain than white people. Such stereotypes have led Black patients to receive poor care, extensive research suggests. That can be especially problematic for sickle cell patients like Ms. Craig, who describe rushing to the emergency room in agony and waiting hours to be seen, only to be sent home still in pain after doctors tell them that their lab results are fine and they should not be suffering.

Biopsies can detect cancer, X-rays a broken bone. But there is no definitive clinical test to determine when a sickle cell patient is suffering a pain crisis.

“This is the essence of the problem,” said Dr. Sophie Lanzkron, the director of the Sickle Cell Center for Adults at the Johns Hopkins Hospital. “There is no objective measure of crisis. The gold standard is the patient tells you, ‘I am having a crisis.’”

The intensity of the disease as well as the subjectivity of treatment mean that a visit to a new doctor can feel like the cruelest game of roulette. And the weight of that pressure bore down on Ms. Craig as she parked at Vanderbilt and hobbled into the elevator. Would the doctor help her?

“Chest hurts,” she told her husband.

“You’ll be all right,” he assured her.

The throbbing pounded the little girl’s body. It was in her arms and legs, and it often made her sob.

Lisa’s parents were baffled. Her mother gave her warm baths and body rubs, and took her to the doctor frequently. But the pain persisted.

Then one day in the late 1970s, when Lisa was about 5, her parents drove her from their home in Knoxville to the Mayo Clinic in Rochester, Minn. Doctors ran tests and discovered the cause: sickle cell disease.

At the time, widespread screening for the illness in newborns was still about a decade away. Lisa was the only person in her extended family ever to have it diagnosed.

“That was something that was unheard-of,” she said.

Her mother was often her protector, coddling her when the pain set in, while her father urged her to carry on.

Flare-ups of pain made her miss out on slumber parties, ice skating and plenty of school. But for all the restrictions, no one ever questioned whether her pain was real.

At the East Tennessee Children’s Hospital where she was treated, the rooms were decorated with ocean- or circus-themed wallpaper. Nurses gave her games and puppets and tried to make her smile.

“Pain medication was given because people believed I was in pain,” she wrote in her journal decades later.

The medicines gave her relief, but also set her body on a path complicating her treatment decades later: She needed opioid painkillers to live comfortably.

A crisis was brewing in society that complicated efforts to treat pain caused by sickle cell: the spread of addiction to opioids fueled in large part by reckless, even criminal marketing of the drugs by major pharmaceutical companies.

Research showed that people with sickle cell were no more likely to become addicted to opioids than other chronic pain sufferers, and that their use of narcotics had not skyrocketed as it had in the general population.

In March 2016, the Centers for Disease Control and Prevention released stringent guidelines on prescribing narcotic painkillers, though it carved out exceptions for sickle cell.

A few months later, Ms. Craig’s doctors began cutting back on the amount of intravenous narcotics she was given for pain crises. She argued that the reduced doses were not working. Her hematologist, Dr. Jashmin K. Patel, urged her to take hydroxyurea, a chemotherapy drug that is a standard treatment for the disease, saying it would reduce her pain, according to medical records. Ms. Craig had tried it, but had an unusually severe reaction, with mouth sores, hair loss and vomiting, so she stopped. She said she felt that the doctor wasn’t taking her complaints about the side effects seriously. (Most patients can take the drug successfully.)

“Why do you dear doctor still bully me to take it,” Ms. Craig wrote in her journal on Sept. 17, 2017.

She didn’t want a doctor who preached to her, she wrote, but one who listened, because as someone “who deals with how MY body works with this disease don’t you think my expertise outweighs yours.”

Over the past decade, even some of the best-informed sickle cell specialists have begun reconsidering their reliance on long-term opioid therapy. They have found little evidence to suggest that sickle cell patients who regularly take opioids see their quality of life improve. And their concern about long-term reliance on narcotics is especially high in patients like Ms. Craig, who are living well into middle age with a disease that used to kill its sufferers in childhood or early adulthood.

Dr. Lanzkron at Johns Hopkins said her patients would “end up on these ridiculous doses” and “still have the same level of pain.”

“It’s a terrible treatment,” she said.

So the specialists started trying to teach people with sickle cell how to lessen and tolerate pain with techniques including therapy, meditation and hypnosis.

Ms. Craig had tried everything — warm baths, elevating her feet, steady breathing. She hated feeling dependent on pills. Yet she dreaded the way a simple ache crescendoed to feel like a thousand bee stings or a hand smashed in a door.

In July 2018, her need for relief led to conflict during a visit with Dr. Patel. Alarm bells began ringing in Ms. Craig’s head when the doctor stepped into the room accompanied by a stenographer.

Dr. Patel said she was concerned that Ms. Craig was not taking hydroxyurea as she was supposed to, according to medical records reviewed by The New York Times. She told Ms. Craig that she was not going to increase her pain medication, noting in the file that Ms. Craig had called two weeks earlier for a refill.

Ms. Craig said in an interview that she had never asked for an increase in medication and that Dr. Patel was twisting her words and ignoring her concerns. Neither Dr. Patel nor the practice where she worked responded to requests for comment.

Voices were raised, feelings hurt. Eight days later — on July 18, 2018 — Ms. Craig got a letter from Dr. Patel saying she was no longer welcome at the practice, “because of your lack of cooperation in your medical treatment, non-compliance with treatment recommendations and frequent narcotic requests before agreed time-frame.”

After she was kicked out of Dr. Patel’s practice, Ms. Craig went to Dr. Wahid T. Hanna, a veteran oncologist at the University of Tennessee Medical Center, who had treated dozens of sickle cell patients.

By December 2018, familiar tensions arose. Dr. Hanna grew suspicious of her request for narcotics. She had gone through the 120 Oxycodone pills that he had prescribed a month earlier and wanted a refill.

On several visits, Dr. Hanna repeated a refrain as if he were saying it for the first time: He was puzzled that she had pain because she had a generally less severe version of sickle cell.

“So really, I don’t have any justification why should you have pain,” he told her on one of those visits.

“I’ve always had pain,” she replied, according to a recording Ms. Craig’s husband took of the meeting.

Months earlier, Tennessee had enacted some of the nation’s most stringent restrictions on doctors prescribing opioids during a deadly epidemic, though there were exceptions for sickle cell patients.

“My question is, with the way the state is regulating the narcotics and all that, we could be questioned,” Dr. Hanna said. “We could be red-flagged.”

If Ms. Craig had pain, Dr. Hanna said it might have been from arthritis or the heavy periods she complained of. Those could be managed without opioids, he said.

“We do this every time I come, and I’m not understanding,” Ms. Craig said.

“I’m saying this because we can be questioned,” Dr. Hanna said, and if the authorities asked him whether he saw a lot of pain in someone with her kind of sickle cell, “I’d say usually I don’t.”

“You can’t say 100 percent that it’s not possible,” Ms. Craig said.

“I want to take care of you, but I want to do it right,” he said.

In that moment, Dr. Hanna said in a later interview, “I did not know whether her pain requirements were genuine or not.”

Her red blood cell count was stable and her iron was low — metrics that, Dr. Hanna said, suggested that her sickle cell was not that severe. But experts who treat sickle cell say that iron and hemoglobin levels do not indicate how severe the disease is.

Still, Dr. Hanna reduced her narcotic dosage, encouraged her to use over-the-counter pain medicines and scheduled her for an iron infusion, which he told her would make her “feel like a different person.”

Days after another disappointing visit to Dr. Hanna in December 2018, Ms. Craig sat on a light green leather couch beneath a painting of an ocean in her therapist’s office, choking back tears.

“Putting up with somebody belittling me and making me feel less than is not worth it,” she told her therapist.

It was difficult enough to control her physical pain, but reining in the mental anguish proved equally troublesome.

A former preschool teacher who speaks with wide-eyed animation, Ms. Craig has not been able to work full time since 2005 because of her unpredictable pain. She finds purpose where she can, taking care of her family, picking up the occasional odd job, babysitting for relatives and friends.

She exercised as her doctors advised, took 15 minutes a day to “be selfish” as a friend suggested and wrote prayers on brown slips of paper that she sealed in a jar. She listened as her therapist explained that there was no shame in trying to get prescriptions to relieve pain.

But all around, the signals told Ms. Craig otherwise: the constant stream of news about the opioid crisis and, one evening shortly before her Vanderbilt visit, a heated discussion with an aunt at the family dining room table.

“You can’t just come on in there and just say: ‘Look, this is the drug I take. And I know this’ll work,’” said her aunt, Nanette Henry Scruggs, who used to work at a hospital.

“The hospitals tell people all the time to be your own advocate,” Ms. Craig said.

Times were changing, her aunt explained, because doctors had overmedicated pain patients and now risked losing their licenses.

“You don’t understand it because you have the disease,” Ms. Scruggs said.

“And you don’t understand it because you don’t,” Ms. Craig fired back, her voice straining with emotion. “And you’re not the one that they look at and go, ‘Oh, she’s just exaggerating her pain.’ When I want to saw my own freaking legs off, that’s a problem!”

Many sickle cell patients feel frustrated that doctors don’t believe patients know what works. Often, that’s narcotic doses much higher than the average person requires. Yet asking for specific medications can fuel distrust, compounded by many doctors’ lack of familiarity with sickle cell.

Only one in five family physicians said they were comfortable treating sickle cell, according to a 2015 survey. Even hematologists rarely specialize in it, with a greater focus on cancers of the blood, which are more prevalent.

Ms. Craig lamented that sickle cell patients did not seem to get the sympathy given to people with other devastating illnesses. Somebody needed to change that, she told her aunt, “and I’m going to be that somebody.”

“Sickle cell patients are not abusing, are not the major cause of people overdosing,” Ms. Craig told her.

“I’m not saying that,” her aunt said, later adding, “She’s thinking I’m against her.”

“I’m not saying you’re against me, but you’re definitely not standing shoulder to shoulder with me,” Ms. Craig said.

Ms. Craig was now worked up, and her husband, Jeremy, urged her to calm down. He has long been her champion, but Ms. Craig worried her disease was a drag on her family. Jeremy, 45, their daughter, Kaylyn, 19, and their son, Mason, 15, have endured her at her weakest and angriest. They accompany her on middle-of-the-night emergency room runs and wake up when she paces their single-story brick home in the middle of the night because of pain.

Still, they have always looked out for her. Her husband first learned that she had sickle cell when they were dating and she told him that she was having a pain crisis. He drove her to the emergency room at 2 a.m., kissed her on the forehead and told her he loved her. She was sold. And because he was white, there was a lower chance that he would carry the sickle cell mutation, meaning it was less likely that their children would have the disease — something she also found appealing.

For Mr. Craig, simply watching his wife suffer was not an option. He always looked for solutions and thought he’d come up with one as he scrolled through his cellphone one evening in their dim living room: marijuana.

“I think you should try it,” he said.

Ms. Craig waved him off, but he insisted that it would be safe to try in states where it was legal.

“What if it works?” he asked.

“What if it doesn’t,” she replied. “I’m done talking to you about that whole situation.”

“If we go to Washington State,” he insisted.

“I’m not going,” she said, cutting him off. “To me, that feels like an addict.”

Still, she was desperate for help as her relationship with Dr. Hanna deteriorated. A social worker suggested she consult specialists at Vanderbilt.

She made the appointment. Just a few days before the visit, she made her fourth trip to the emergency room in six weeks for a pain crisis. The doctor gave her intravenous Tylenol and four oxycodone tablets. After four hours, she was still in pain and left the hospital, as she had many times, without relief.

“I want to be extremely honest with u and let you know I am tired,” she wrote to me on Facebook at 1:16 a.m., after getting home from the emergency room. “I feel beaten down by these doctors as if I am an addict.”

She was hurtling, she said, toward “a dangerous level of depression.”

Ms. Craig fidgeted and sweat beaded around her lip, forehead and eyes. It was Jan. 18, 2019, and, at last, she sat in an exam room at Vanderbilt.

Dr. Adetola A. Kassim strolled in, chomping gum. He shook hands with her and her husband.

“So what brings you?” he asked.

For half an hour, Ms. Craig guided him through her arduous journey: hip replacement, seizures, blood clots. Pain crises usually came right before her period, she told him, and he said that researchers were exploring whether there was a link between sickle cell pain and menstruation.

Dr. Kassim, who heads Vanderbilt’s adult sickle cell program, is a native of Nigeria who has specialized in treating the disease for more than 20 years. As he listened to her medical history and symptoms, he contemplated the riddle of treating her.

“What you’ve had over the years is an interplay of your disease with other chronic health problems,” he told her. “I’m going to think about it carefully because you’re a little complicated.”

He told Ms. Craig that he needed to run tests to figure out the underlying causes of her chronic pain. Did she, for instance, have arthritis? Since hydroxyurea had so many side effects for her, he wanted to try another drug, Endari.

And he wanted to manage her pain with sparing narcotic use. He worried she was susceptible to hyperalgesia, a condition in which prolonged opioid use can alter patients’ nerve receptors and actually cause more pain.

In many ways, he was echoing Dr. Hanna. She needed to take fewer narcotics. Sickle cell probably was not the cause of some of her pain. But he never questioned whether she was hurting. He listened. He laid out a plan.

“You can’t just come in one day and be like a cowboy,” Dr. Kassim said in a later interview. “You’ve got to win their trust and begin to slowly educate them.”

After she left his office that day, Ms. Craig leaned her head on her husband’s shoulder. “I feel like we should have come here a long time ago,” she said.

Three months after her first visit with Dr. Kassim, pain radiated through her lower back, left hip, elbows and knees. She was out of hydrocodone, and her next refill was more than a week away.

“Continue alternating between Aleve and extra strength Tylenol,” Karina L. Wilkerson, a nurse practitioner in Dr. Kassim’s office, counseled her in an email, prescribing a muscle relaxer and telling her: “Rest, heat and hydrate.”

Days later, the pain was so unrelenting that Ms. Craig went to the emergency room and got a dose of intravenous narcotics.

She felt as if history was repeating itself. She was trying to wean herself from opioids, to rely mostly on over-the-counter meds, to use heat and ice, but it was not working.

“I feel like I’m a junkie,” she said in an interview, her voice cracking.

The pain returned a day after she left the hospital. With four days until her next visit to Dr. Kassim, she sent another message to ask whether there was anything more to be done, careful not to request hydrocodone. A nurse wrote that she could be prescribed more muscle relaxers, but “we cannot fill any narcotics for you before your appointment.”

Ms. Craig felt as if she was back where she started. Dr. Kassim was friendly, attentive and knowledgeable, yet she was still enduring pain.

“A part of me knew we’d be back in this position,” she said, “that it was too good to be true.”

One day last May, Ms. Craig had spent a lot of time on her feet at a family gathering after a relative’s death. As she settled in for the evening, a family friend dropped off two children she had agreed to babysit, and she braced for the inevitable result of a busy day: pain.

In the past, she would have taken a hydrocodone earlier in the day as a maintenance dose. But she had been seeing Dr. Kassim for more than a year, and although pain continued to gnaw at her, she was starting to buy into his advice. She had paid close attention to Facebook groups and news from medical journals with the latest developments on sickle cell. In her 48th year battling the disease, her perspective was changing.

She had come to realize that no matter how much hydrocodone she took or how well versed her doctor was in the disease, her pain did not disappear — and that the medical consensus had shifted against relying mainly on narcotics.

“It’s like a defeated acceptance,” she said.

In the wee hours of the morning after the family gathering, she began to hurt. Her hips throbbed. She tried to sleep on her left side, then her right. She lay on her back and elevated her feet. Nothing worked.

Still, she held off on the narcotics. Most people with sickle cell remember a crisis when their pain was “at a zillion and you were sitting in that emergency room, waiting for them to call you, and all you wanted to do was pass out,” she said. “We live on that edge of fear.”

She held off until about 11 a.m., when she took a hydrocodone. It provided enough relief to keep her out of the hospital — just the kind of progress Dr. Kassim wanted from her.

He sought to address the underlying triggers of her pain: sickle cell, worn joints, her menstrual cycle, nerve damage and prolonged opioid use. The main thing, he said, was to stabilize her quality of life. That goal motivated her.

But the spread of the coronavirus has interfered with their plan.

Dr. Kassim told Ms. Craig during a visit in February of last year that he wanted her to get an M.R.I. to better understand the underlying causes of her pain. But the pandemic hit, and she was not able to get that imaging until December. It revealed some of the pain triggers that Ms. Craig will have to get under control: a bulging disk in her back, and arthritis in both hips and her left shoulder.

She held off going to physical therapy for fear of catching Covid-19, but is now planning to go since she has been vaccinated. She has tried to tolerate the pain and avoid the hospital, but not always successfully. There were three visits in a week last June and a five-hour wait during a September visit.

Through the past year, she has grown more resolute, trying to raise awareness and support for people with the disease in Knoxville. She had masks made with the words “sickle cell” printed across the front. She has resolved to live with the disease, not suffer from it.

“It’s just my life,” she said. “The one I’ve been dealt.”