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Image by Betty Pace
A case series published in Blood indicates that some patients with mildly symptomatic sickle cell disease (SCD) can live long lives if they
comply with treatment recommendations and lead a healthy lifestyle.
The paper includes details on 4 women with milder forms of SCD who survived beyond age 80.
“For those with mild forms of SCD, these women show that lifestyle modifications may improve disease outcomes,” said author Samir K. Ballas, MD, of Sidney Kimmel Medical College at Thomas Jefferson University in Philadelphia, Pennsylvania.
Three of the women described in this case series were treated at the Sickle Cell Center of Thomas Jefferson University, and 1 was treated in Brazil’s Instituto de Hematologia Arthur de Siqueira Cavalcanti in Rio de Janeiro.
The women had different ancestries—2 African-American, 1 Italian-American, and 1 African-Brazilian—and different diagnoses—2 with hemoglobin SC disease and 2 with sickle cell anemia. But all 4 women had what Dr Ballas called “desirable” disease states.
“These women never had a stroke, never had recurrent acute chest syndrome, had a relatively high fetal hemoglobin count, and had infrequent painful crises,” Dr Ballas said. “Patients like this usually—but not always—experience relatively mild SCD, and they live longer with better quality of life.”
In addition, all of the women took steps to maintain and improve their health and had long-term family support. Dr Ballas said these factors likely contributed to the women’s long lives and high quality of life.
“All of the women were non-smokers who consumed little to no alcohol and maintained a normal body mass index,” he said. “This was coupled with a strong compliance to their treatment regimens and excellent family support at home.”
Family support was defined as having a spouse or child who provided attentive, ongoing care. And all of the women had at least 1 such caregiver.
Treatment compliance was based on observations by healthcare providers, including study authors. According to these observations, all of the women showed “excellent” adherence when it came to medication intake, appointments, and referrals.
As the women had relatively mild disease states, none of them were qualified to receive treatment with hydroxyurea. Instead, they received hydration, vaccination (including annual flu shots), and blood transfusion and analgesics as needed.
Even with their mild disease states and healthy lifestyles, these women did not live crisis-free lives. Each experienced disease-related complications necessitating medical attention.
The women had 0 to 3 vaso-occlusive crises per year. Two women required frequent transfusions (and had iron overload), and 2 required occasional transfusions. One woman had 2 episodes of acute chest syndrome, and the second episode led to her death.
Ultimately, 3 of the women died. One died of acute chest syndrome and septicemia at age 82, and another died of cardiac complications at age 86. For a third woman, the cause of death, at age 82, was unknown. The fourth woman remains alive at age 82.
As the median life expectancy of women with SCD in the US is 47, Dr Ballas and his colleagues said these 4 women may “provide a blueprint of how to live a long life despite having a serious medical condition like SCD.”
“I would often come out to the waiting room and find these ladies talking with other SCD patients, and I could tell that they gave others hope, that just because they have SCD does not mean that they are doomed to die by their 40s . . . ,” Dr Ballas said. “[I]f they take care of themselves and live closely with those who can help keep them well, that there is hope for them to lead long, full lives.” 
alongside a normal one
Image by Betty Pace
A case series published in Blood indicates that some patients with mildly symptomatic sickle cell disease (SCD) can live long lives if they
comply with treatment recommendations and lead a healthy lifestyle.
The paper includes details on 4 women with milder forms of SCD who survived beyond age 80.
“For those with mild forms of SCD, these women show that lifestyle modifications may improve disease outcomes,” said author Samir K. Ballas, MD, of Sidney Kimmel Medical College at Thomas Jefferson University in Philadelphia, Pennsylvania.
Three of the women described in this case series were treated at the Sickle Cell Center of Thomas Jefferson University, and 1 was treated in Brazil’s Instituto de Hematologia Arthur de Siqueira Cavalcanti in Rio de Janeiro.
The women had different ancestries—2 African-American, 1 Italian-American, and 1 African-Brazilian—and different diagnoses—2 with hemoglobin SC disease and 2 with sickle cell anemia. But all 4 women had what Dr Ballas called “desirable” disease states.
“These women never had a stroke, never had recurrent acute chest syndrome, had a relatively high fetal hemoglobin count, and had infrequent painful crises,” Dr Ballas said. “Patients like this usually—but not always—experience relatively mild SCD, and they live longer with better quality of life.”
In addition, all of the women took steps to maintain and improve their health and had long-term family support. Dr Ballas said these factors likely contributed to the women’s long lives and high quality of life.
“All of the women were non-smokers who consumed little to no alcohol and maintained a normal body mass index,” he said. “This was coupled with a strong compliance to their treatment regimens and excellent family support at home.”
Family support was defined as having a spouse or child who provided attentive, ongoing care. And all of the women had at least 1 such caregiver.
Treatment compliance was based on observations by healthcare providers, including study authors. According to these observations, all of the women showed “excellent” adherence when it came to medication intake, appointments, and referrals.
As the women had relatively mild disease states, none of them were qualified to receive treatment with hydroxyurea. Instead, they received hydration, vaccination (including annual flu shots), and blood transfusion and analgesics as needed.
Even with their mild disease states and healthy lifestyles, these women did not live crisis-free lives. Each experienced disease-related complications necessitating medical attention.
The women had 0 to 3 vaso-occlusive crises per year. Two women required frequent transfusions (and had iron overload), and 2 required occasional transfusions. One woman had 2 episodes of acute chest syndrome, and the second episode led to her death.
Ultimately, 3 of the women died. One died of acute chest syndrome and septicemia at age 82, and another died of cardiac complications at age 86. For a third woman, the cause of death, at age 82, was unknown. The fourth woman remains alive at age 82.
As the median life expectancy of women with SCD in the US is 47, Dr Ballas and his colleagues said these 4 women may “provide a blueprint of how to live a long life despite having a serious medical condition like SCD.”
“I would often come out to the waiting room and find these ladies talking with other SCD patients, and I could tell that they gave others hope, that just because they have SCD does not mean that they are doomed to die by their 40s . . . ,” Dr Ballas said. “[I]f they take care of themselves and live closely with those who can help keep them well, that there is hope for them to lead long, full lives.”
alongside a normal one
Image by Betty Pace
A case series published in Blood indicates that some patients with mildly symptomatic sickle cell disease (SCD) can live long lives if they
comply with treatment recommendations and lead a healthy lifestyle.
The paper includes details on 4 women with milder forms of SCD who survived beyond age 80.
“For those with mild forms of SCD, these women show that lifestyle modifications may improve disease outcomes,” said author Samir K. Ballas, MD, of Sidney Kimmel Medical College at Thomas Jefferson University in Philadelphia, Pennsylvania.
Three of the women described in this case series were treated at the Sickle Cell Center of Thomas Jefferson University, and 1 was treated in Brazil’s Instituto de Hematologia Arthur de Siqueira Cavalcanti in Rio de Janeiro.
The women had different ancestries—2 African-American, 1 Italian-American, and 1 African-Brazilian—and different diagnoses—2 with hemoglobin SC disease and 2 with sickle cell anemia. But all 4 women had what Dr Ballas called “desirable” disease states.
“These women never had a stroke, never had recurrent acute chest syndrome, had a relatively high fetal hemoglobin count, and had infrequent painful crises,” Dr Ballas said. “Patients like this usually—but not always—experience relatively mild SCD, and they live longer with better quality of life.”
In addition, all of the women took steps to maintain and improve their health and had long-term family support. Dr Ballas said these factors likely contributed to the women’s long lives and high quality of life.
“All of the women were non-smokers who consumed little to no alcohol and maintained a normal body mass index,” he said. “This was coupled with a strong compliance to their treatment regimens and excellent family support at home.”
Family support was defined as having a spouse or child who provided attentive, ongoing care. And all of the women had at least 1 such caregiver.
Treatment compliance was based on observations by healthcare providers, including study authors. According to these observations, all of the women showed “excellent” adherence when it came to medication intake, appointments, and referrals.
As the women had relatively mild disease states, none of them were qualified to receive treatment with hydroxyurea. Instead, they received hydration, vaccination (including annual flu shots), and blood transfusion and analgesics as needed.
Even with their mild disease states and healthy lifestyles, these women did not live crisis-free lives. Each experienced disease-related complications necessitating medical attention.
The women had 0 to 3 vaso-occlusive crises per year. Two women required frequent transfusions (and had iron overload), and 2 required occasional transfusions. One woman had 2 episodes of acute chest syndrome, and the second episode led to her death.
Ultimately, 3 of the women died. One died of acute chest syndrome and septicemia at age 82, and another died of cardiac complications at age 86. For a third woman, the cause of death, at age 82, was unknown. The fourth woman remains alive at age 82.
As the median life expectancy of women with SCD in the US is 47, Dr Ballas and his colleagues said these 4 women may “provide a blueprint of how to live a long life despite having a serious medical condition like SCD.”
“I would often come out to the waiting room and find these ladies talking with other SCD patients, and I could tell that they gave others hope, that just because they have SCD does not mean that they are doomed to die by their 40s . . . ,” Dr Ballas said. “[I]f they take care of themselves and live closely with those who can help keep them well, that there is hope for them to lead long, full lives.”