Four of every 10 people treated in a hospital for pain or other problems caused by sickle cell disease have to be readmitted for treatment again within 30 days, according to a new study published in the April issue of JAMA.

High rates of rehospitalization within 30 days of a previous hospital stay, an indicator of poor-quality post hospital outpatient care for a number of diseases, has only recently gained interest related to sickle cell care. The study showed high rates of rehospitalizations at 30 days, and also at 14 days, which may be a more accepted marker of care quality in sickle cell disease.

“It was important for us to draw attention to the high rate of acute care utilization for people with sickle cell disease. Armed with this knowledge we can focus attention on the need for improved care for those with sickle cell disease” said lead author David C. Brousseau, M., MS, associate professor of pediatrics (section of emergency medicine) at the Medical College of Wisconsin. He also practices at Children’s Hospital of Wisconsin as a pediatric emergency specialist.

“This study is important because it provides benchmark data to evaluate the quality of outpatient management of sickle cell disease symptoms,” said AHRQ’s Director Carolyn M. Clancy, M.D.

The study found that one in three sickle cell disease patients was rehospitalized within 30 days, a rate roughly 1.5 times greater than that of diabetes patients, twice that of heart failure patients, and nearly 10 times greater than the rate for pediatric asthma patients. Moreover, two-thirds of the patients rehospitalized within 30 days were re-admitted within 14 days of their previous hospital discharge, suggesting that interventions to prevent rehospitalizations need to happen during or soon after the hospitalization.

When the researchers analyzed acute care use by age, they found that 18- to- 30 year-olds had over three and a half acute care encounters -- rehospitalizations or treat-and release emergency department visits--per year, a rate markedly higher than the two visits per year from children 10-17 years old. Regardless of age, the patients with Medicaid or other type of public insurance used acute care for sickle cell related reasons more than privately insured and uninsured patients. Publicly insured 18-to-30 year-old patients had the highest rate – nearly five encounters per year.

This same age group also had the highest rate of hospital readmissions within 30 days or 14 days of their previous hospitalization, 41 percent and 28 percent respectively, and the highest rate of emergency department visits within the same time frame, 20 percent and 14 percent respectively.

The study was based on 21,112 sickle cell disease patients in eight states – Arizona, California, Florida, Massachusetts, Missouri, New York, South Carolina and Tennessee – who were hospitalized or treated and released from hospital emergency departments in 2005 and 2006. The state databases are part of the AHRQ-administered Healthcare Cost and Utilization Project.

For details see “Acute Care Utilization and Rehospitalization for Sickle Cell Disease” by Dr. Brousseau, Pamela Owens, Ph.D.,; Andrew L. Mosso, M.S., Analyst,; Julie A. Panepinto, M.D., MSPH, and Claudia A. Steiner, M.D. MPH.

Source: http://www.mcw.edu

Marelle Aguillard kept working even though sickle cell disease had pumped her right arm to twice the size of her left.

The pain was so bad she asked to borrow money to have her right arm amputated. She was right-handed.

Mark Yancy missed a year-and-a-half of college classes because sickle cell had destroyed bone and bone tissue in his hips. At 21, he had his right hip replaced once and his left hip replaced twice.

"The pain (during a sickle cell crisis) is so severe, so excruciating, I can't even describe it," Yancy said.

Jim McGehee Jr. will never know that pain himself but he's seen it from the front row. Aguillard was his wife's nurse, helping her at home after an Alzheimer's diagnosis.

McGehee would drive Aguillard to the hospital, see the pain in her eyes and he mourned her death just two months after his wife, Virginia, passed in 2008.

"You have never in your life seen anyone suffer the way a sickle cell patient suffers during (a crisis)," McGehee said.

So, McGehee and Yancy have joined forces to try to give Memphis-area sickle cell patients a new place to get comprehensive care.

They've been charged to raise $5 million in start-up funds for a comprehensive, 40-bed sickle cell unit to be built on one whole floor of Methodist University Hospital on Union.

McGehee is the chairman and owner of McGehee Realty and Development Co. and a former chairman of the Memphis/Shelby County Airport Authority. Yancy is the major gifts coordinator for Methodist Le Bonheur Healthcare Inc.

Their first task has been to assemble a committee to help raise funds and awareness. While that task is not complete, they've already attracted star power like Memphis Grizzlies point guard Mike Conley and fundraising powerhouses like Meg and Scott Crosby, who raised millions for the Salvation Army's Kroc Center of Memphis.

Should the committee succeed and the sickle cell center be built, it would be the only one like it in the Mid-South.

The Regional Medical Center at Memphis has been the home of the Diggs-Kraus Sickle Cell Center since 1929 and it sees some 2,000 patients per year. The center does have an outpatient clinic just for sickle cell patients, but it does not have specialized inpatient care for them as the new center would.

Sickle cell patients can now only turn to hospitals' emergency departments if they're in the midst of a crisis, or a pain event caused when sickled red blood cells begin to cut off circulation to certain parts of the body, Yancy said.

"It is a crisis, but it's not considered an emergent condition, so you have to wait behind people with heart attacks or strokes," Yancy said.

"So you may have to wait in terrible pain in an emergency waiting room for six to 12 hours. That's not good for the patient and it's not appropriate from a hospital standpoint."

Sickle cell disease

— Is an inherited disorder that causes red blood cells to weaken and become rigid and sickle-shaped

— It causes severe blood clotting

— It affects 1 in 5,000 African-Americans in the United States

— Is predominantly carried by those with lineages going back to tropical or subtropical areas where malaria was present

— Toby Sells: 529-2742

Obituary

Edelle Elizabeth Kennebrew Thompson

Edelle Elizabeth Kennebrew Thompson was born in Tuskegee, Alabama, on February 6, 1944, to Clarence “Joe” Kennebrew of Macon County (Roba) and Mary Woods Kennebrew of Macon County (Shorter). She joined Friendship Baptist Church at an early age and attended Washington Public School. She matriculated to Tuskegee High School, in which she proudly served as their Miss Tuskegee High School. She graduated from her beloved high school in 1961 at the age of sixteen.

She attended Tuskegee Institute where she majored in nursing under the tutelage of the famous Dean Lillian Harvey. She completed her internship in Baltimore, Maryland, at City Hospital and graduated in 1965. During her stay in Baltimore, she met and eventually married Tommie Thompson, in which two off springs were born.

Upon graduation, Nurse Thompson was initially employed by Providence Hospital (the only hospital for blacks during this time period) and was responsible for mentoring student nurses. Over the years, Thompson worked in various capacities of nursing. After several years of living in Baltimore, she began feeling home sick and was led back to Tuskegee, where she followed her father’s footsteps and began working at the Veterans Administration Hospital (VA). She retired from the VA in 1999 and sought a new beginning by moving to Memphis, Tennessee, to watch over her children and grandchild. Being always the extravert, she immediately adapted to the Bluff City and began to work as a Nurse at the Memphis Mental Health Institute until her untimely decline in health.

Ms. Thompson was an active member Alpha Kappa Alpha Sorority, Inc., where she was initiated through Mu Sigma Omega chapter in the fall of 1995.Additionally, she served as an active member Beta Epsilon Omega Chapter of Memphis, Tennessee. She was also a chapter member of the Auburn, Alabama, Chi Eta Phi Nursing Sorority and Sigma Theta Tau Honor Society. She was an active member of St. Augustine Catholic Church in Memphis, Tennessee, and volunteered numerous hours for the Sickle Cell Foundation of Tennessee. Anyone who knew Edelle, knew that she was extremely creative, theatrical, an avid reader that loved modeling, and was extremely proud to be from Tuskegee, Alabama, the pride of the swift growing South.

She will be mourned by her devoted children Trevor (Cherry) Kennebrew Thompson, Traci Charis Thompson and her grandchildren James Chad Thompson, Alexandria Elizabeth Sylvia Thompson, and Preston Stills. She also leaves behind sisters-in-law, Barbara (Cliff) Waller, Geraldine Eggleston and nephews Aaron, Devin, and Darron Waller, extended families Ford, Harris, Whitehead, Woodall, as well as numerous, cousins, uncles, aunts, sorors, and friends.

Lane College Spa Night
Friday, February 12, 2010

James C. Thompson (Events Manager) and Trevor K. Thompson (CEO) of the Sickle Cell Foundation of Tennessee provided a booth at the Lane College Spa Night. They provided information on Sickle Cell disease as well as Iron Overload to Sickle Cell consumers as well as their relatives. The event had approximately 150-200 woman in attendance.

Sickle Cell Innovation Encouraging, But Not Silver Bullet

TOM WILEMON | The Daily News

December 17, 2009

Nine out of 10 adults with sickle cell had the disease reversed after undergoing a new modified stem cell transplant regimen developed by researchers, but a cure still remains elusive for most people living with the illness.

Dr. Patricia Adams-Graves, a professor at the University of Tennessee Health Science Center and the medical director of the Diggs-Kraus Sickle Cell Center at The Regional Medical Center at Memphis, said the biggest obstacle is still finding donor matches for transplants.

She noted that in the study about the new regimen, which is in the Dec. 10 issue of the New England Journal of Medicine, only 10 of 112 sickle cell patients could participate. Those 10 had siblings with identical human leukoycyte antigen (HLA) typing.

However, the treatment regimen, which was developed by the National Institutes of Health Clinical Center in Bethesda, Md., is a major step forward, Adams-Graves said.

“I’m so encouraged,” she said. “However, I can’t promise all my patients that they can go have this procedure. For a given individual who doesn’t have a sibling match, this is not available to them currently.

“The good thing is for in the future we know it works. Now, we need to try some different types of matching – donor matches. In the future, to overcome the problem, we might want to go to a donor pool that we call a half-match from a sibling or a parent.”

However, she stressed that much research and innovation is required to broaden HLA donor pools.

Down through the ages

About 1,300 people in the Mid-South have been diagnosed with sickle cell disease, said Trevor Thompson, chief executive officer of the Sickle Cell Foundation of Tennessee. The inherited disease is more prevalent in African-Americans and Latinos with Caribbean ancestry.

“One in nine African-Americans carries the gene as a trait,” Adams-Graves said. “Half of my family has the sickle cell trait and I personally have had three cousins with the disease.”

The disease gets its name from the shape red blood cells take when they lose oxygen and collapse because of abnormal hemoglobin caused by an altered gene. The red blood cells become stiff and sticky, which can result in severe pain, organ damage and stroke.

Dr. Griffin P. Rodgers, a NIH official who was one of the co-authors of the paper, said the trial “represents a major milestone in developing a therapy aimed at curing” the disease.

Bone marrow or stem cell transplants have been more successful in treating children with the disease. St. Jude Children’s Research Hospital in 1983 pioneered bone marrow transplantation as a cure for sickle cell, but it is not an easy cure and the hospital continues its research.

St. Jude has five labs that perform sickle cell research, according its Web site.

Resilience of youth

Similar treatments for adults performed by other research institutions have not been successful. Children have been cured of the disease by undergoing bone marrow transplants after having their own marrow destroyed with chemotherapy – a process that is too toxic for most adults to tolerate.

In the NIH study with adults, toxicity was reduced by partially replacing the bone marrow and augmenting the treatment with low doses of radiation and administering two drugs to suppress the immune system. The drugs helped prevent the new stem cells from being rejected.

There are no plans at this time to bring the new transplant regimen to Memphis, said Adams-Graves. The Diggs-Kraus Sickle Cell Center, which is housed in The MEDplex, does not do stem cell transplants. The biggest difficulty remains finding suitable donors, she said.

Thompson is an advocate for transitional treatment and cooperative research between St. Jude and the Diggs-Kraus.

“It is actually happening – a strong partnership for a transition program,” Thompson said. “We can always strengthen it, don’t get me wrong.”

St. Jude and Diggs-Kraus have participated in trials of Hydroxyurea, a drug that increases the concentration of fetal hemoglobin.

Thompson, who takes Hydroxyurea, credits the drug for his not having been hospitalized in three years.

Adams-Graves said the center at The MED offers Hydroxyurea.

“We use Hydroxyurea, which is the only drug that targets the molecular level – not quite like the stem cell transplant for a cure – but it does manipulate the gene, increases their fetal hemoglobin and decreases their sickle hemoglobin,” she said. “Sickle cell patients are living longer on that. They do better. They have fewer crises.”

Dying of pride

The center provides patients with comfort measures and treatments for complications of the disease. Adams-Graves said she would love someday to have a stem cell transplant center for adults with sickle cell.

A more immediate wish list is to have additional staff, offer more services and be able to operate with hours beyond 5 p.m.. Her goal is have a more comprehensive approach to helping people cope with the disease.

“Moreover, we feel like transitioning young adults and adolescents over to our side needs to be perfected a little more,” she said.

Thompson and Adams-Graves said more public awareness is needed about sickle cell.

“(My) family branch afflicted with the disease lived in Virginia, (and) were kind of ashamed of it and didn’t talk much about it,” she said. “They were dying, and we thought they would just die of anything – they would die of a blood clot or whatever. Actually, one of my cousins who outlived his siblings with the disease actually called me well after I started taking care of sickle cell and informed me that’s what my cousins died of. It just flipped me out.”

Over the years, Adams-Graves said she has diagnosed patients who did not even know they had the disease until suffering a health crisis late in life.

Nine out of 10 adults with sickle cell had the disease reversed after undergoing a new modified stem cell transplant regimen developed by researchers, but a cure still remains elusive for most people living with the illness.

Dr. Patricia Adams-Graves, a professor at the University of Tennessee Health Science Center and the medical director of the Diggs-Kraus Sickle Cell Center at The Regional Medical Center at Memphis, said the biggest obstacle is still finding donor matches for transplants.

She noted that in the study about the new regimen, which is in the Dec. 10 issue of the New England Journal of Medicine, only 10 of 112 sickle cell patients could participate. Those 10 had siblings with identical human leukoycyte antigen (HLA) typing.

However, the treatment regimen, which was developed by the National Institutes of Health Clinical Center in Bethesda, Md., is a major step forward, Adams-Graves said.

“I’m so encouraged,” she said. “However, I can’t promise all my patients that they can go have this procedure. For a given individual who doesn’t have a sibling match, this is not available to them currently.

“The good thing is for in the future we know it works. Now, we need to try some different types of matching – donor matches. In the future, to overcome the problem, we might want to go to a donor pool that we call a half-match from a sibling or a parent.”

However, she stressed that much research and innovation is required to broaden HLA donor pools.

By Jennifer Thomas
HealthDay Reporter

WEDNESDAY, Dec. 9 (HealthDay News) -- Researchers say that a new method of bone marrow transplantation cured nine out of 10 adult patients with sickle cell disease, an inherited condition that causes bouts of severe pain, organ damage and sharply limits life expectancy.

Adults have typically not been candidates because they were thought to be too sick to handle the high doses of chemotherapy and radiation necessary to prep the body for the procedure, explained senior study author Dr. John Tisdale, a senior investigator in the molecular and clinical hematology branch at the U.S. National Institutes of Health.

Until now, transplantation was generally reserved for more resilient children, whose bodies had not yet suffered as much damage from sickle cell disease.

But the new method allows for a less grueling pre-transplantation routine, one that even adults with severe sickle cell can tolerate.

More than 70,000 Americans suffer from sickle cell disease, and it is especially common among blacks. People with the disease have abnormal, crescent-shaped hemoglobin. The abnormal cells have difficulty passing through small blood vessels, causing blockages and damaging tissues. Over time, the damage can lead to stroke and severe bouts of pain in the chest, arms, legs, chest and abdomen. Sickle cell disease also damages the kidneys, liver and spleen, leaving people, especially children, more susceptible to infection, said Dr. Lanetta Jordan, chief medical officer for the Sickle Cell Disease Association of America.

Treatments include prophylactic antibiotics to fight infections, blood transfusions and hydroxyurea, the only drug U.S. Food and Drug Administration-approved drug for treating sickle cell, Jordan said.

In the new study, Tisdale and his colleagues gave 10 patients ages 16 to 45 with severe sickle cell disease alemtuzumab, a drug used to suppress immune system T-cells; relatively low doses of radiation; and sirolimus, an immune suppressant to fight rejection. Marrow donors were siblings with matched HLA (human leukocyte antigen) markers in their blood.

None of the patients experienced graft-versus-host disease, one of the most common and potentially fatal complications of bone marrow transplants, in which the body rejects the new bone marrow.

After 30 months, all of them are alive, and nine of the patients had successful grafts and are considered cured of sickle cell disease, according to the study.

"It's been transforming for these patients," Tisdale said. "These were the sickest of the sick patients. Some were in the hospital every other week for pain or other crises. Today, some have gone back to school and to work. One patient had a baby."

The last item is important, because in conventional bone marrow transplants, high doses of chemotherapy drugs and radiation typically destroy fertility. However, the lower level of radiation used in the new method does not seem to do this.

The study is published in the Dec. 10 issue of the New England Journal of Medicine.

Conventional bone marrow transplants cure sickle cell disease by first using chemotherapy and radiation to wipe out the person's own marrow, which makes the faulty red blood cells. The marrow is replaced with stem cells from a donor's marrow, which then takes over and begins to produce new, healthy red blood cells.

But when doing the new bone marrow transplants, the researchers noted that not all of the patient's own marrow was wiped out. Some remained and seemed to co-exist with the donor marrow without causing problems, Tisdale said.

"That meant we didn't necessarily have to kill the entire bone marrow of the patient to make this work," Tisdale said, opening the possibility of using an even less toxic means of preparing the body for transplant.

Though most patients in the study are still taking immune-suppressant drugs, researchers hope to eventually wean them off the medications.

Dr. Miguel Abboud, a pediatric hematology/oncology specialist and a professor of pediatrics at the American University of Beirut Medial Center, in Lebanon, said the new protocol is promising, especially since it could eventually include those who don't have an HLA-matched sibling.

"The findings are very significant because adults with very severe sickle cell disease have decreased life expectancy and multiple morbidities but have limited therapeutic options," said Abboud, who wrote an accompanying editorial. "In the past these patients were excluded from transplant studies as they are very poor candidates for high dose chemotherapy regimens. This study makes it possible to offer this subset of patients with severe sickle cell disease stem cell transplants."

Dr. Lakshmanan Krishnamurti, a pediatric hematologist/oncologist at the University of Pittsburgh and director of the Sickle Cell Program at Children's Hospital of Pittsburgh, has done bone marrow transplants in children, also using a less toxic protocol.

"This is an important paper and a big step forward for the field," Krishnamurti said. "Now we are able to say, 'OK, young adults or not so young adults can be transplanted successfully.' That is a very big deal."

More information
There's more on sickle cell disease at the Sickle Cell Disease Association of America.

Saturday, September 26, 2009
The 2nd Annual Health Fair and Sickle Cell Symposium sponsored by the Urevbu Foundation

Wednesday, September 16, 2009
Sickle Cell Walk committee appears on Live
at 9 with Mary Beth & Chris

July 15, 2009
DKSCSSF becomes a member of the SCDAA

The Diggs Kraus Sickle Cell and Social Service Foundation has been accepted to be a member of the Sickle Cell Disease Association of America (SCDAA). The official orientation will occur at the national conference in Orlando, FL on September 29, 2009-October 3, 2009.

June 5, 2009
Moore Twins raise more than S1600.00 for DKSCSSF

Autumn and Adrienne Moore hosted a "Party with a Purpose" to benefit the Diggs Kraus Sickle Cell and Social Services Foundation. This is the Second Annual Party with a Purpose in which the twins raise funds for a cause. This year they decided to support Sickle Cell because their friend Morgan Harris suffers from the disease. Approximately 60 teens were in attendance and received information concerning Sickle Cell Disease. The event was held at the Whitehaven Link Golf Course Club House.

Mentor/Mentee Cookout
Saturday, May 16, 2009

The Mentor/Mentee Cookout was a collaboration with Memphis city Schools CONNECT mentoring program and adolescent consumers from St. Jude Children’s Research Hospital. In order to address our common goals of celebrating and implementing mentorship programs, the two organization integrated resources in order to have a successful cookout. We had approximately 150-200 people in attendance through out the day.

We provided free food to all participants. Additionally, we provided Face painting, Rock Climbing Wall and a slide down the obstacle giant slide as activities. Furthermore, we provided prizes for Mentors to compete with their Mentee in horse shoes, sack races and three-legged races. Additionally, Hula Hoops and Karaoke activities were hit for all ages!

Sickle Cell consumers attended the event to get screened for Iron Overload. We had 19 Sickle Cell Consumers screened for Iron Overload within four hours. This number exceeded our expectations.

April 11, 2009

January 12, 2009

Novartis Pharmaceutical Corporation selected the Diggs-Kraus Sickle Cell and Social Services Foundation to be a 2009 Be Sickle Smart Partner. The foundation was selected based on recommendations from the National Sickle Cell Disease Association of America, patient demographics and commitment to sickle cell disease patients and health professional education in your area. Novartis Pharmaceuticals Corporation established the Be Sickle Smart (BSS) in 2006. Be Sickle Smart is a disease awareness community health-education program, created to raise awareness and understanding of Sickle Cell Disease (SCD) and Iron Overload (IO) among sickle cell disease patients and healthcare professionals. Building on past program successes, in 2009 they are expanding their efforts to 10 cities in order to help more patients by having a greater emphasis on patient education efforts and healthcare professional programming. The 10 cities/regions are: Baltimore/D.C., Baton Rouge, Cleveland, Houston, Jackson, Memphis, Miami/Ft. Lauderdale, New York City, Raleigh/Durham and SF/Oakland.

The 2009 Be Sickle Smart program highlights include:

• BSS Patient & Caregiver Educational Materials

• BSS Nurses Speakers Bureau

• Physician Speakers Bureau

• Serum Ferritin Screening

• Patient Education Symposium with celebrity/PR support

NIH State-of-the-Science Conference Statement on Hydroxyurea Treatment for Sickle Cell Disease

Trevor K. Thompson was a contributor to the National Institute of Health Consensus Conference on Hydroxyurea. The NIH Consensus Development Conference on Hydroxyurea for Sickle Cell Disease was webcast live February 25-27, 2008. The webcast is archived and available for viewing free of charge at http://consensus.nih.gov/sicklecell.htm. Below is the report.

The Sickle Cell Disease Walk-A-Thon in Memphis originated in 2007. It has been developed to create a greater awareness in the Memphis community of Sickle Cell Disease and to raise money for patient care, social services, and research. All proceeds from the Walk-A-Thon will directly benefit people living with Sickle Cell. The ultimate goal is to improve the quality of life for individuals living with this debilitating disease. The 3rd Annual Sickle Cell Walk is scheduled for September 19, 2009, at Overton Park in Memphis, Tennessee at 8:00 AM. More details to come in the near future.