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.
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.
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.’”
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.
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.
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.”
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.”
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.”
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.
Dr. Adetola A. Kassim strolled in, chomping gum. He shook hands with her and her husband.
“So what brings you?” he asked.
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.
‘Too good to be true’
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.”
‘A defeated acceptance’
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.”