‘On That Fringe of Worry’: One Girl’s Battle With Sickle Cell Ache

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

NASHVILLE – She struggled through the night as she often did, restless with sickle cell pain that felt as if knives were pricking her bones. When morning came, she wept on the edge of her hotel room bed. Her stomach was plagued by a complicated knot of anger, fear, and hope.

It was a gray January morning and Lisa Craig was in Nashville, three hours from her Knoxville, Tennessee, home preparing to see a sickle cell specialist who she hoped could do something so many doctors couldn't were able to: bring their painful illness under control.

Ms. Craig, 48, had bumped into doctors for years about her treatment. Those tensions had only increased as the medical consensus on pain management shifted and regulations on opioid use tightened. Her fear had grown so persistent and exhausting that she sometimes thought she would be better off dead.

She was ready to do just about anything to stop the deterioration of her body and mind – and her hope for that day in January 2019 was based on a Nigerian-born doctor at Vanderbilt University Medical Center who diagnosed the disease, mainly from People affected who had long been treated were of African descent.

That morning she slipped into a cream cardigan and a necklace with a heart-shaped pendant. She played Whitney Houston before getting behind the wheel of her black S.U.V. Her husband in the passenger seat keyed her destination into his phone's navigation system.

"Live as if everything were a miracle," says a framed quote on Ms. Craig's beige living room wall, and that's exactly what she had hoped for.

People with sickle cells, a rare inherited blood disorder caused by a mutation in a single gene, typically experience episodes of debilitating pain as well as chronic pain. Around 100,000 Americans and millions of people worldwide, mostly in Africa, suffer from this disease. Red blood cells that carry oxygen become stiff and curved like crescent moons, clog blood vessels and starve the body for oxygen.

Promising developments in gene therapy have given people with the disease hope that a cure is on the way for a disease that often leads to organ failure and premature death. However, the first such therapy is more than a year after regulatory approval. It will almost certainly be extremely expensive, it cannot reverse the damage to tissues and organs from the disease, and it can be late for people whose bodies are so battered by the disease that they may not survive the strenuous treatment.

Most people with sickle cells are looking for something far more basic: a way to prevent or treat the devastating complications of the disease – stroke, depression, and most importantly, pain.

This search can be difficult, as I learned while following Mrs. Craig for over two and a half years of struggle and heartbreak. I accompanied her to doctor visits, shared meals with her family, analyzed her medical records, attended a therapy session, and accompanied her while she ran errands in Knoxville and relaxed at home. I saw moments of anger, sadness and agony, but also determination, joy and love.

Her efforts to find relief have been hampered by a national opioid epidemic and coronavirus pandemic, as well as the challenges of navigating a medical system that frequently abuses blacks like her. At the same time, doctors changed the way they treated sickle cells as recent research suggested that narcotics might actually make the pain worse.

Ms. Craig believed that doctors tended to stereotype her as drug addicts and did not believe in the extreme end of her suffering.

Racist myths persist in health care, such as the notion that blacks tolerate more pain than whites. Such stereotypes have resulted in poorly cared for black patients, extensive research suggests. This can be especially problematic for sickle cell patients like Ms. Craig, who describe rushing to the emergency room in agony and waiting to be seen, only to be sent home in pain after doctors tell them that her lab results are fine and she shouldn't be suffering.

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

"This is the essence of the problem," said Dr. Sophie Lanzkron, the director of the adult sickle cell center at Johns Hopkins Hospital. “There is no objective measure of the crisis. The gold standard is for the patient to tell you, "I'm in a crisis."

Due to the intensity of the illness and the subjectivity of the treatment, a visit to a new doctor can feel like the cruellest game of roulette. And the weight of that pressure was on Ms. Craig as she parked in Vanderbilt and hobbled into the elevator. Would the doctor help her?

"Chest hurts," she said to her husband.

"You will be fine," he assured her.

The pounding hit the little girl's body. It was in her arms and legs and it often made her sob.

Lisa's parents were amazed. Her mother gave her warm baths and body massages, and often took her to the doctor. But the pain persisted.

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

At the time, widespread screening for the disease in newborns was about a decade away. Lisa was the only person in her extended family who was ever diagnosed.

"That was something unknown," she said.

Her mother was often her protector and pampered her when the pain set in while her father urged her to move on.

Relapses of pain caused her to miss slumber parties, ice skating and lots of school. Despite all the limitations, no one ever questioned whether her pain was real.

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

"Painkillers were given because people thought I was in pain," she wrote in her diary decades later.

The drugs gave her relief, but they also put her body on a path that made treatment difficult decades later: she needed opioid pain relievers to live comfortably.

A crisis was brewing in society that hampered efforts to treat sickle cell pain: the spread of opioid addiction, in large part triggered by the ruthless, even criminal, marketing of the drugs by large pharmaceutical companies.

Research showed that people with sickle cells were no more likely to become dependent on opioids than other people with chronic pain, and that their drug use had not skyrocketed as in the general population.

In March 2016, the Centers for Disease Control and Prevention published strict guidelines for prescribing narcotic pain relievers, although exceptions were made for sickle cells.

A few months later, Ms. Craig's doctors began reducing the amount of intravenous narcotics she received for pain crises. She argued that the reduced doses didn't work. Your hematologist, Dr. Jashmin K. Patel, told her to take hydroxyurea, a chemotherapy drug that is a standard treatment for the disease. Ms. Craig had tried but had an unusually severe reaction with mouth pain, hair loss, and vomiting, so she stopped. She said she felt the doctor wasn't taking her complaints about the side effects seriously. (Most patients can take the drug successfully.)

"Why are you still bullying me, dear doctor?" Wrote Ms. Craig in her diary on September 17, 2017.

She didn't want a doctor preaching to her, she wrote, but one who would listen because as someone "who is concerned with how MY body deals with this disease, you don't think that my expertise outweighs your expertise."

In the past decade, even some of the best-informed sickle cell specialists have begun to reconsider their reliance on long-term opioid therapy. They found little evidence that sickle cell patients who regularly take opioids experience an improvement in their quality of life. And her concern about long-term narcotics addiction is particularly high among patients like Ms. Craig, who suffered into middle age with a disease that killed those affected in childhood or early adulthood.

Dr. Lanzkron of Johns Hopkins said her patients would "take these ridiculous doses" and "still have the same pain."

"It's terrible treatment," she said.

So the specialists began teaching people with sickle cells how to relieve and tolerate pain using techniques such as therapy, meditation, and hypnosis.

Mrs. Craig had tried everything – warm baths, feet raised, breathing calmly. She hated feeling addicted to pills. Still, she dreaded the way a simple pain felt like a thousand bee stings or a hand smashed into a door.

In July 2018, her need for relief led to a visit to Dr. Patel to conflict. Alarm bells rang in Ms. Craig's head as the doctor, accompanied by a stenographer, entered the room.

Dr. Patel said she was concerned that Ms. Craig was not taking hydroxyurea as intended, according to medical records reviewed by the New York Times. She told Ms. Craig she wasn't going to increase her pain medication, and noted on the file that Ms. Craig had called two weeks earlier to refill.

Ms. Craig said in an interview that she never asked for the medication to be increased and that Dr. Patel twisted her words and ignored her concerns. Neither Dr. Patel still the practice she worked in responded to requests for comment.

Voices grew loud, feelings hurt. Eight days later – on July 18, 2018 – Ms. Craig received a letter from Dr. Patel, in which she said in practice she was no longer welcome to request narcotics before the agreed timeframe. "

After getting out of Dr. Patel's practice, Ms. Craig went to see Dr. Wahid T. Hanna, a senior oncologist at the University of Tennessee Medical Center who treated dozens of sickle cell patients.

Known tensions arose by December 2018. Dr. Hanna became suspicious of her request for narcotics. She had been through the 120 oxycodone pills he had prescribed a month earlier and wanted a refill.

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

"Really, I have no justification as to why you should be in pain," he told her on one of those visits.

"I've always been in pain," she replied, based on a note made by Ms. Craig's husband of the meeting.

Months earlier, Tennessee had put some of the toughest restrictions in the country on doctors prescribing opioids during a deadly epidemic, though there were exceptions for sickle cell patients.

"My question is, with the way the state regulates narcotics and all of that, we might be questioned," said Dr. Hanna. "We could stand on the red flag."

When Ms. Craig was in pain, Dr. Hanna, it could be due to arthritis or the heavy periods she was complaining about. These could be managed without opioids, he said.

"We do this every time I come and I don't get it," said Ms. Craig.

"I'm saying this because we can be interviewed," said Dr. Hanna, and when the authorities asked him if he had seen someone with her type of sickle cell in great pain, "I would normally say I don't." . ”

"You can't 100 percent say that you can't," said Ms. Craig.

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

At that moment, Dr. Hanna in a later interview: "I didn't know whether her pain demands were real or not."

Her red blood cell counts were stable and her iron was low – metrics that, as Dr. Hanna said, suggesting that her sickle cell wasn't that heavy. However, experts who treat sickle cells say that iron and hemoglobin levels are not an indication of how severe the disease is.

Nevertheless, Dr. Hanna took her anesthetic dose, encouraged her to use over-the-counter pain relievers, and scheduled her for an iron infusion that told her she was "feeling like a different person."

Days after another disappointing visit to Dr. Hanna in December 2018 sat Ms. Craig on a light green leather couch under a painting of an ocean in her therapist's office and choked back tears.

"To come to terms with someone who belittles me and makes me feel less than I am not worth," she told her therapist.

It was hard enough to control her physical pain, but curbing the mental agony was just as troublesome.

Ms. Craig, a former preschool teacher who speaks with wide eyes, has been unable to work full time since 2005 due to unpredictable pain. She finds a purpose where she can, taking care of her family, occasionally taking on odd jobs, and babysitting for relatives and friends.

She exercised as her doctors recommended, took 15 minutes a day to be "selfish" as a friend suggested, and wrote prayers on brown pieces of paper that she sealed in a jar. She listened as her therapist explained that there was no shame in getting prescriptions for pain relief.

But everywhere the signals were telling Ms. Craig something else: the constant stream of news of the opioid crisis and a heated discussion with an aunt at the family dining table one evening just before her visit to Vanderbilt.

"You can't just come in and just say," Look, this is the drug I'm on. And I know that will work, ”said her aunt Nanette Henry Scruggs, who used to work in a hospital.

"The hospitals keep telling people to be their own lawyer," said Ms. Craig.

Times were changing, her aunt explained, because doctors over-medicated pain sufferers and now risked losing their license.

"You don't understand because you have the disease," said Ms. Scruggs.

"And you don't understand because you don't understand," replied Ms. Craig, her voice full of emotion. "And you're not the one they look at and say," Oh, she's just exaggerating her pain. "If I want to see my own damn legs, that's a problem!"

Many sickle cell patients are frustrated that doctors don't believe that patients know what is working. Often times these are narcotic doses that are much higher than the average person needs. However, the demand for certain drugs can fuel suspicion, compounded by the lack of familiarity many doctors have with sickle cells.

According to a 2015 survey, only one in five GPs said they were comfortable with treating sickle cells. Even hematologists rarely specialize in it, with an emphasis on blood cancers, which are more common.

Ms. Craig complained that sickle cell patients did not seem to get the compassion shown for those with other devastating diseases. Somebody had to change that, she said to her aunt, "and I'll be that someone."

"Sickle cell patients aren't abusing, they're not the main cause of overdoses," said Ms. Craig.

"I'm not saying that," said her aunt, and later added, "she thinks I'm against her."

"I'm not saying you are against me, but you are definitely not standing shoulder to shoulder with me," said Ms. Craig.

Mrs. Craig was excited now, and her husband Jeremy urged her to calm down. He was her champion for a long time, but Mrs. Craig feared that her illness would affect her family. Jeremy, 45, their daughter Kaylyn, 19, and their son Mason, 15, endured her in her weakest and angry form. They accompany her to the emergency room in the middle of the night and wake up when she is walking up and down her one-story brick house in the middle of the night because of pain.

Even so, they always kept an eye out for her. Her husband first learned that she had sickle cells when they were together, and she told him that she was having a pain crisis. He drove her to the emergency room at 2 a.m., kissed her forehead and told her he loved her. She was sold. And because he was white, he was less likely to have the sickle cell mutation, which meant that her children were less likely to have the disease – something she found appealing, too.

For Mr. Craig, just watching his wife suffer was not an option. He was always looking for solutions and thought he'd come up with one as he flipped through his cell phone one evening in her dark living room: marijuana.

"I think you should try," he said.

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

"What if it works?" he asked.

"What if it isn't," she replied. "I'm done talking to you about this whole situation."

"If we go to Washington State," he insisted.

"I'm not going," she said, interrupting him. "It feels like an addict to me."

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

She made the appointment. Just days before the visit, she made her fourth trip to the emergency room in six weeks because of a pain crisis. The doctor gave her Tylenol and four oxycodone tablets intravenously. After four hours, she was still in pain and, as she had often done, left the hospital with no relief.

"I want to be very honest with you and let you know that I'm tired," she wrote to me on Facebook at 1:16 am after coming home from the emergency room. "I feel down from these doctors like I'm addicted."

It was racing, she said, towards "dangerous levels of depression".

Mrs. Craig fidgeted, sweat beading around her lips, forehead and eyes. It was January 18, 2019, and she was finally sitting in an exam room in Vanderbilt.

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

"So what's in it for you?" he asked.

For half an hour, Ms. Craig led him through their arduous journey: hip replacements, seizures, blood clots. Pain crises usually started just before their periods, she told him, and he said researchers were looking at whether there was a link between sickle cell pain and menstruation.

Dr. Kassim, director of the adult sickle cell program at Vanderbilt, is from Nigeria and has specialized in the treatment of the disease for more than 20 years. While listening to her medical history and symptoms, he pondered the mystery of treating her.

"What you've had over the years is an interplay of your illness with other chronic health problems," he told her. "I'll think about it carefully because you're a little complicated."

He told Ms. Craig that he would need tests to find out the underlying causes of her chronic pain. For example, did she have arthritis? Since hydroxyurea had so many side effects for her, he wanted to try another drug, Endari.

And he wanted to treat her pain with economical use of anesthetics. He feared she was prone to hyperalgesia, a condition where prolonged opioid use can alter patients' nerve receptors and actually cause more pain.

In many ways he repeated Dr. Hanna. She had to take less narcotics. Sickle cells probably weren't the cause of some of her pain. But he never asked if she was hurt. He listened. He came up with a plan.

"You can't just come in one day and be like a cowboy," said Dr. Kassim in a later interview. "You have to gain their trust and start raising them slowly."

After leaving his office that day, Ms. Craig leaned her head on her husband's shoulder. "I think we should have come here a long time ago," she said.

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

"Keep switching between Aleve and extra strong Tylenol," advised Karina L. Wilkerson, a nurse at Dr. Kassim's office, she in an email, prescribed a muscle relaxant and told her, "Rest, heat, and humidity."

Days later, the pain was so relentless that Ms. Craig went to the emergency room and was given a dose of intravenous anesthetic.

It felt like history was repeating itself. She tried to wean herself off opioids, relying mostly on over-the-counter medications, using heat and ice, but it didn't work.

"I feel like a junkie," she said in an interview with a cracking voice.

The pain returned a day after leaving the hospital. Four days before her next visit to Dr. She sent Kassim another message to ask if there was anything left to be done and made sure not to request any hydrocodone. One nurse wrote that she could be prescribed more muscle relaxants, but "we cannot fill anesthetics for you before your appointment."

Ms. Craig felt like she was back where she'd started. Dr. Kassim was kind, attentive, and knowledgeable, but she was still in pain.

"Part of me knew we would be in that position again," 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 reunion after the death of a relative. When she was settled in for the evening, a family friend brought two children she'd agreed to babysit, and she prepared for the inevitable outcome of a busy day: pain.

In the past, she would have taken a hydrocodone maintenance dose earlier in the day. But she had Dr. Having seen Kassim for more than a year, and although the pain continued to gnaw at her, she began to follow his advice. She had been paying close attention to Facebook groups and news from medical journals with the latest developments in sickle cells. Her perspective changed in her 48th year fighting the disease.

She had realized that no matter how much hydrocodone she took or how well her doctor knew about the disease, her pain would not go away – and that the medical consensus against the use of narcotics had shifted.

"It's like acceptance defeated," she said.

In the early hours of the morning after the family reunion, she began to feel pain. Her hips throbbed. She tried to sleep on her left side, then on her right. She lay on her back and lifted her feet. Nothing worked.

Still, she held back with the narcotics. Most people with sickle cells remember a crisis when their pain was "a million fold 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 this edge of fear."

She held back until about 11 a.m. when she was taking a hydrocodone. It was enough relief to keep her out of the hospital – exactly the kind of progress that Dr. Kassim wanted from her.

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

But the spread of the coronavirus has disrupted their plan.

Dr. Kassim told Ms. Craig during a visit last February that he wanted her to do an M.R.I. to better understand the underlying causes of their pain. But the pandemic hit, and she couldn't get these pictures until December. It showed some of the pain triggers that Ms. Craig needs to control: a bulging disc in her back and arthritis in both hips and her left shoulder.

She stopped physiotherapy for fear of getting Covid-19 but plans to leave now that she has been vaccinated. She has tried to endure the pain and avoid the hospital, but not always successfully. Last June there were three visits in a week and a five hour wait during a visit in September.

Over the past year she has become more determined trying to raise awareness and support for people with the disease in Knoxville. She had masks printed with the words "sickle cell" on the front. She decided to live with the disease and not suffer from it.

"It's just my life," she said. "The one I got."

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