Richmond resident Kenny Lane is receiving treatment through the Adult Sickle Cell Program at VCU Health,, and is a resident in its adult sickle cell medical home. Courtesy VCU Health
Richmond resident Kenny Lane is receiving treatment through the Adult Sickle Cell Program at VCU Health and is a resident in its adult sickle cell medical home.
Pain is an inescapable part of life for Kenny Lane.
It’s been so intense at times that he’s had to seek help in emergency rooms.
Lane’s pain stems from a medical condition, sickle cell anemia.
Sickle cell is the most common inherited blood disease in the United States and is often diagnosed through screenings in infancy. It is an inherited blood disorder in which red blood cells gain a sickle shape and become sticky and hard, according to the U.S. Centers for Disease Control and Prevention. The sickle-shaped cells can get stuck in blood vessels, and that limits the flow of blood and oxygen. That in turn may cause pain, damage organs, bring on infections and trigger a stroke. The disease occurs mostly in African Americans in this country at a rate of about 1 out 500 births. That’s about 90,000 to 100,000 Americans, according to the CDC.
Lane, 34, has been contending with intense, awful pain since he was a preschooler. His grandmother would take him to emergency rooms seeking relief. “I used to have a lot of aches and pains in my body, and we didn’t know exactly where the pain was coming from,” he says. “I didn’t learn about sickle cell until I was like 14 or 15 and understand it, what happens with my body and causes me to have so much pain.”
Pain can strike anywhere at any time and be mild to severe. Episodes can be brief or prolonged.
Dr. Wally Smith, director of the VCU Health Adult Sickle Cell Program. Courtesy VCU Health
Dr. Wally Smith, director of the VCU Health Adult Sickle Cell Program
“The pain is just terrible, like somebody took a baseball bat to your shin and started whamming on it, and hours later they still haven’t stopped — just wham, wham, wham, wham. Women who have it sometimes compare it to childbirth and say they’d rather have a baby,” says Dr. Wally Smith, director of VCU Health's adult Sickle Cell Program and the Florence Neal Cooper Smith Professor of Sickle Cell Disease for VCU. There are 700 patients served through the VCU clinic, with people seeking its services from Central and Eastern Virginia, Fredericksburg to Charlottesville, and down to North Carolina.
Sickle cell patients have long been at the front of the line in terms of denial for pain medications, Smith says, and that is especially true now. Medications should be given to a sickle cell patient in acute pain crisis within an hour, but they often have to wait two to six hours to receive them.
Emergency medicine doctors are hypervigilant in dealing with people seeking pain relief medications because of the ongoing opioid epidemic. “The pendulum swung away on pain medications,” Smith says. “Sickle cell patients don’t get the medication they need, especially in an ER and hospital.”
The pain makes it hard for some people with sickle cell disease to manage daily life, maintain regular employment or afford housing.
Treatments vary, depending on symptoms, and include blood transfusions, increasing fluid intake through drinking water or through an IV, and medication for pain. People contending with severe sickle cell disease may receive a drug, hydroxyurea, which the CDC notes may reduce the number of painful episodes, lessen the frequency of hospital stays and reduce the need for blood transfusions. Stem cell or bone marrow transplants may also be considered.
In earlier decades, 20% to 30% percent of children with sickle cell disease died before age 10, which is still true in sub-Saharan Africa. Survival on average was to 18 or 19 years old by the late 20th century. Now, treatments provide a better quality of life and longer lives, with the median life expectancy for someone with sickle cell at 42 for men and 48 for women, with many people surviving well into old age.
Smith dived into sickle cell treatment and research in the 1980s in Memphis, Tennessee. Patients, he says, “were orphans” nobody wanted. Smith and another retired doctor, a hematologist, started a clinic. The patients “were so grateful to have somebody come rescue them, [like we were] starting an orphanage.”
Survival has been extended into adulthood through measures including a wide implementation of screening for strokes in childhood, providing prophylactic treatment with antibiotics in children with sickle cell before age 6 to ward off pneumonia, meningitis or overwhelming blood disease, Smith says.
Transfusions help, too, in preventing strokes. A downside, though, is that transfusions work for a while but can lead to complications, such as the development of antibodies in a patient to a point where they can no longer receive a transfusion. “We have to be more conservative with transfusions,” Smith says.
VCU’s clinic is involved in medication research, including development in 2019 of the drug Voxelotor. The clinic also helps in moving people with sickle cell from pediatric care to adult care.
People with sickle cell disease are living longer, but that has meant more are transitioning into adult care, and that may be problematic for several reasons, Smith notes. Often, medical insurance is available to cover children with sickle cell, but patients are unable to afford insurance as an adult. They move, they may stop taking medications, they may not have a doctor. The transition is a time of increased risk for hospitalizations, emergency medical visits and death for people with sickle cell.
“That’s a risky time,” Smith says.
A New Hope
Sickle cell disease kept Lane out of many school activities, he says; there were a lot of exercises he was unable to do in physical education, for instance.
He’s says he’s gone through hell, dealing with pain, and was without a home.
But now he’s taking part in an adult sickle cell medical home program through VCU Health. Lane has an apartment through the program, which provides people with a place of their own and connection with social services that they need to help them manage their medical conditions as well as overall life skills.
The adult medical care village serves several patients down on their luck, Smith says. It began with a program to help military veterans coping with substance abuse by giving them a place to stay and helping them in their treatment and life, and it has been adapted for people in similar straits with sickle cell disease. The goal is to get them stabilized and thriving to the point they can graduate in a year or two and manage their own affairs.
“We are proud of what we have been able to do for a few very lucky patients,” Smith says.
Participants are paired with a liaison available around the clock who helps with everything from coordinating care and providing advice on managing pain to life skills such as helping a participant in paying bills. Patients are carefully monitored and screened.
VCU Health reports that hospital admissions declined 10% in 2019 for participants in its adult housing program programs and that inpatient services were used 1,096 fewer days. Those stats represent a $1.18 million savings.
Lane notes that he was once told that he wouldn’t live beyond age 18, but here he is, 34 and taking control of his life. He says now that he’s no longer in that situation and is living in his apartment, he feels that he’s done a tremendous job since he’s been on his own.
“I am blessed,” he says. “I’ve always kept the positive. I’ve always told myself, ‘Kenny, you have to beat it.’ ”
Smith is also upbeat, noting that there are a number of clinical trials of drugs in the pipeline, about 40 medications in the works. About 2,000 people, mostly children, have received bone marrow transplants to alleviate sickle cell disease, according to Smith. Gene therapy is “still very much experimental and not readily available,” he says, with about 20 to 30 people with the disease having received gene treatments.
He also notes a study underway that looks at ways to curb the death rate from sickle cell disease, ways to smooth the transition from pediatric to adult care and programs to provide peer mentorship to ease the transition.
“This is the golden age of treating sickle cell disease,” he says.
CAPSULES
Health and medicine news and notes
- Children’s Hospital of Richmond at VCU tied for 10th out of 21 children’s facilities rated in the mid-Atlantic region in U.S. News & World Report’s annual assessment of children’s hospitals. The rankings were released Tuesday. The Richmond facility also earned national recognition in the assessment of medical specialties, ranking No. 28 in Urology, No. 30 in Pulmonary and Lung Surgery, No. 42 in Nephrology, and No. 50 in cancer treatment. Other Virginia children’s facilities earning regional honors in include the University of Virginia Children’s Hospital at No. 7, and Inova Children’s Hospital in Falls Church in a six-way tie at No. 13.
- Enjoy your three to five cups of coffee a day without the guilt: Coffee is associated with a lower mortality risk, or so says a research fellow at the National Cancer Institute in a feature earlier in the week in The New York Times.
