Sickle Cell Care Clinic Provides Relief from Crippling Pain

What’s it like to have the genetic blood disorder known as sickle cell disease? Ask Anais Fernandez, 37, of Miami, who at just three months old was diagnosed with the disease, which restricts the amount of oxygen traveling through the blood stream. It can cause crippling pain and damage to vital organs.

“It’s slowly killing my brain, my lungs, my blood cells, my kidneys, my everything,” Ms. Fernandez says matter-of-factly. “I am slowly just dying from a lack of oxygen in my blood.”

Even worse is the pain caused by a sickle cell crisis, which is what happens when red blood cells abruptly change their shape as a result of what is called oxidative stress. “The pain is excruciating,” she says. “For someone who has never endured a full-blown crisis, it’s almost unimaginable. The location of the pain varies and it can last from a few hours to several weeks or longer.”

(Watch now: What’s it like having sickle cell disease? Anais Fernandez, 37, says the pain can be excruciating but she is grateful for the convenient and compassionate care she gets at Baptist Health South Miami Hospital’s Sickle Cell Care clinic. Video by Carol Higgins, Homestretch Creative.)

Who is at risk for sickle cell disease?

According to the National Institutes of Health (NIH), sickle cell disease is a group of inherited red blood cell disorders that affect hemoglobin, the protein that carries oxygen through the body. The NIH says the disease affects more than 100,000 people in the United States and 20 million people worldwide.

In the U.S., most people who have the disease are of African ancestry or identify themselves as Black:

·      About 1 in 13 Black or African American babies are born with sickle cell trait.

·      About 1 in every 365 Black or African American babies are born with sickle cell disease.

·      About 1 in every 16,400 Hispanic babies are born with sickle cell disease.

Neil Miransky, D.O., a palliative medicine specialist and chief medical officer of pharmacy services for Baptist Health who oversees Sickle Cell Care at Baptist Health South Miami Hospital

The mortality rate of sickle cell disease is high, says Neil Miransky, D.O., who oversees Sickle Cell Care at Baptist Health South Miami Hospital. “According to our most recent data, the average lifespan for someone with sickle cell disease in the U.S. is approximately 42 years for females and 40 years for males,” he says. “This impacts how you see your life and what’s important.”

How does sickle cell disease affect your life?

Sickle cell disease has had a profound impact on Ms. Fernandez’s quality of life for as long as she can remember. Because a crisis can come on suddenly and its duration is unpredictable, patients experience repeated disruptions in their lives that can leave them unable to complete their education, hold steady jobs or simply enjoy daily living.

“Sickle cell disease has kept me from finishing school because I have a lot of pain crises where I’ve been stuck at home or in the ER and I end up missing a lot of classes,” says Ms. Fernandez, who has been on disability since she was 23. “I’ve tried to get certain jobs but unfortunately I’m what they consider an unreliable employee because I don’t know when my crises are going to come.”

What is a sickle cell crisis?

A sickle cell crisis occurs when red blood cells become rigid and sickle-shaped, says Dr. Miransky, a palliative medicine specialist and chief medical officer of pharmacy services for Baptist Health.

“When red blood cells change their shape, they no longer are able to carry oxygen effectively, and so the organs of the body that are supplied by those red blood cells don’t get enough oxygen, which causes pain,” he explains. “In addition, because they change shape, those red blood cells get caught in blood vessels and cause decreased blood flow to areas of the body. This also causes pain.”

How is a sickle cell crisis treated?

Sickle cell pain is “very intense,” according to Dr. Miransky, who says that in a severe crisis, patients frequently have required hospitalization and inpatient care to stabilize them. “During an acute crisis, the pain associated with that is usually sharp and stabbing and aching and it requires high-potency, opioid analgesics.”

Ideally, Dr. Miransky says, these powerful medications should be administered by someone with experience treating sickle cell patients. “You need professionals who are well-trained and educated to support and care for these patients,” he says. “Many physicians are very uncomfortable writing prescriptions for the level of medications needed to manage this condition,” he says.

Another problem is that many healthcare professionals are not well educated on sickle cell disease and sometimes patients seeking pain treatment in an emergency department are looked at as if they’re drug addicts seeking opioid painkillers.

“First of all, I’m Hispanic,” says Ms. Fernandez, who is part of a nonprofit organization called ASAP (Advanced Sickle Cell Advocacy Project). “Sickle cell is predominantly found in Blacks and African Americans so when I go to the ER and tell them I have sickle cell and need high-potency pain medications, they’re like, ‘No, you don’t. You couldn’t possibly have sickle cell.’ And I’m like, ‘Who lies about this? This is my life. This is not something I chose to have.’”

What are the benefits of going to a sickle cell care clinic?

One day a fellow “warrior” – sickle cell patients call themselves warriors because they never give up in their fight against the disease – told Ms. Fernandez about the new Sickle Cell Care clinic in South Miami. There, specially trained doctors and nurses provide prompt, compassionate care in a comfortable setting so that patients in crisis can receive the treatment they need without having to go to an emergency department.

Because sickle cell patients are very prone to infections, being in a hospital or an emergency department comes with some risk, Ms. Fernandez says. But South Miami Hospital’s Sickle Cell Care clinic helps her avoid those settings. “I haven’t been going to the hospital as much since I started coming to the clinic. It’s helped me stay out of the ER a lot more.”

Dr. Miransky says there are other advantages offered by the Sickle Cell Care clinic. “Instead of encountering a doctor or nurse in the ER who doesn’t know you and is reluctant to administer the medications you know you need, our care team already has an extensive history with you and is ready to provide comprehensive and compassionate care whenever you need it,” he explains. “Also, we work with each patient to develop a unique care plan that can be customized throughout the course of their disease.”

What is it like at South Miami Hospital’s Sickle Cell Care clinic?

Ms. Fernandez says she feels right at home visiting the Sickle Cell Care clinic. “They don’t treat me like a number or a statistic. I feel like they actually do listen and care, and they treat me like family. I come in here with hugs and kisses for all my doctors and nurses.”

The clinic is also very private and comfortable, Ms. Fernandez adds, noting that no one in severe pain wants to be in a treatment room along with several other patients who are also in severe pain. “You have your own room, you have your own bathroom, and if you’re in really, really bad pain and can’t get comfortable in the chair, there’s a bed if you need it. And if anything goes wrong, you don’t have to leave and go to a hospital because the hospital is right there. I love that.”

While receiving treatment, all of the patient’s vital signs are closely monitored so that their pain medications can be adjusted as needed. “Some of those pain meds are very strong and can make you really woozy and drop your blood pressure,” Ms. Fernandez says.

She also appreciates the clinic’s flexibility. “If I call them in the morning and say I’m not feeling well and need to come in, they always try and find a way to get me in, even if I didn’t have an appointment. And I can be there all day if I need to. As long as I’m doing okay, they’re not trying to rush. If they feel I need to go to the hospital, then they’ll tell me, ‘Okay, we’re going to have to put you in the ER now,’ and they wheel me over there.”

What do patients say about Sickle Cell Care in South Miami?

Dr. Miransky says the Sickle Cell Care program defines its success by the feedback it receives from patients like Ms. Fernandez. “Our patients tell us the clinic is making a difference in their lives,” he says. “Patients who were having frequent hospitalizations have gone without any. Patients who have frequent crises are noticing a decrease in their occurrence. And patients in crisis are receiving the pain control and disease modification they need to come out of crisis so that the disease is not negatively impacting their life.”

Ms. Fernandez says the clinic has helped her better navigate her occasional crises and she is grateful for the care she receives there. “Giving in to sickle cell disease would be the easy thing to do,” she admits. “But at this point in my life, I don’t have anything else to lose except my life and I’m going to fight for that. Because warriors fight until the very end and they never give up.”