Jamaican pediatrician Dr. Anya McLaren, a specialist in respiratory medicine, has been awarded the Pediatrics Scientific Abstract Award from the American Thoracic Society (ATS). Dr. McLaren received the award for her groundbreaking work in sickle cell disease. Originally from Kingston and now based The Hospital for Sick Children in Toronto, Canada, Dr. McLaren’s research involves the effects of hydroxyurea on the decline of pulmonary function in children suffering from sickle cell. The research found that the drug improves the pulmonary function in these children by more than 33 percent. In her acceptance speech upon receiving the award, Dr. McLaren said it was “tremendously meaningful” for the ATS to recognize the work of her team and hoped that the study will contribute to improving treatment of sickle cell disease in young people. Dr McLaren graduated from St Andrew High School for Girls in Kingston and the University of Toronto. She is a Fellow of the Royal College of Physicians Canada. Dr. McLaren’s mother is Dr. Ingrid McLaren, who is a lecturer an associate dean of the faculty of humanities and education at the University of the West Indies, Mona. Her father is captain Louis McLaren, formerly with the Air Wing of the Jamaica Defense Force and the Sandals Group.
Creating hospital teams devoted to treating pregnant women who havesickle cell disease reduced death rates for those women by almost 90 percent, a study at a major hospital in Ghana showed.
Sickle cell disease is common in West Africa, and among black people in the Americas whose ancestors came from West Africa. It is caused by a genetic mutation that if inherited from only one parent protects against malaria, but if inherited from both parents can be lethal. Red blood cells can collapse into curved “sickle” shapes and clump together to jam capillaries, sometimes causing excruciating pain, shortness of breath and death.
At the Korle-Bu Teaching Hospital in Accra, the capital of Ghana, women with sickle cell disease were about 12 times as likely to die in childbirth as women without it, according to a study presented last month at the American Society of Hematology.
To overcome that, the hospital formed teams of nurses, obstetricians and blood and lung specialists and assigned them to care for all pregnant women with the disease. If the women suffered serious pain or breathing crises, and when they began labor, they got beds in two wards overseen by the team.
With help from an American team led by Dr. Michael R. DeBaun, a pediatric hematologist who directs the Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease, Korle-Bu adopted proven care protocols. Those included giving transfusions before cesarean sections and treating women experiencing chest pain by having them take deep breaths and blow hard, which helps prevent lung collapse.
Because Korle-Bu could not afford spirometers, patients were taught to simply blow up balloons, Dr. DeBaun said. The hospital was given some fingertip sensors that measured how much oxygen was in the blood.
“Previously, patients would be sitting there with air hunger, but no one knew it,” Dr. DeBaun said.
Acute chest syndrome, which resembles pneumonia, was common among pregnant women with sickle cell disease and so serious that “it was previously considered a death sentence,” Dr. DeBaun said.
The sensors cost only about $200 each, so the hospital should be able to afford more, he added.
After the teams and protocols were in place, the hospital’s rate of maternal deaths in childbirth for women with sickle cell disease dropped to 1.1 percent from 9.7 percent. Also, the women suffered only about one-quarter as many episodes of severe chest or joint pain. Deaths of babies also dropped by about one-third.
“It was a real learning curve that allowed us to have this terrific drop in deaths,” Dr. DeBaun said. “And it’s sustained — the teams are still in place and the death rates are still low.”
Atlanta Falcons running back Tevin Coleman, who carries the sickle cell trait, is unsure about playing in the high altitude of Denver next Sunday because it could negatively affect his health.
The blood disorder related to sickle cell disease can cause complications if an individual is under physical stress in extremely hot weather or in environments with low oxygen, such as the high altitude in Denver. Sickle cell trait is a genetic abnormality which can affect red blood cells.
“With my sickle cell, I have no idea if I’ll play there,” Coleman told ESPN.com. “I really don’t know how I’ll feel or how it will be like. I’ve never been to Denver.”
Former NFL player and current ESPN analyst Ryan Clark, who also has the sickle cell trait, had an episode with the Pittsburgh Steelers (2007) where he became gravely ill and lost his spleen and gall bladder after playing in Denver. Clark was then held out of two subsequent games in Denver, including a playoff matchup.
“It does make me scared a little bit, a little nervous, because I’m risking my life,” Coleman said, referring to Clark’s experience. “We’ll have to see. I’ll have to talk to coach [Dan Quinn]. I guess coach will see how I feel when we get there. And I just have to communicate with him, if I am out there [playing], if I’m dead [tired] or can’t breathe. We just have to play it smart.”
Coleman was pulled from the end of an August practice as result of extreme heat.
“I was just dehydrated,” Coleman said. “I’ll probably get a lot of fluids in me and an IV while I’m in Denver. I will be hydrated so that helps a lot. That will be a step that I take.”
Coleman plans to do more research on the matter before the trip. The Falcons could simply limit his reps in the game.
“I know myself, and I know how it will affect me, but I’ll do a little more research of how it is in Denver because, like I said, I’ve never been there,” Coleman said. “I’ve never been nowhere with high altitudes.”
There is no doubt Coleman would be disappointed if this causes him to miss the game.
“It would bother me quite a bit,” he said. “I’d be real upset because my team is going to be out there, and I want to compete and battle for my team. Watching them play, you just want to be out there and ball with them. I’d be really disappointed if I can’t play, but I’d understand because things could happen.”
Coleman has been a major factor in the Falcons’ 3-1 start while sharing carries with starter Devonta Freeman. Through four games, Coleman has 129 rushing yards and four rushing touchdowns on 40 carries. He also has 13 receptions for 181 yards.
The Falcons currently have just two running backs on the active roster in Freeman and Coleman. Terron Ward is on the practice squad but could be elevated if Coleman is unable to play. That would also mean a corresponding roster move.
The Falcons already have to tweak the roster with cornerback Jalen Collins, who is expected back after he was suspended the first four games for violating the league’s policy on performance-enhancing substances.
Sickle cell anemia, or sickle cell disease (SCD), is a genetic disease of the red blood cells (RBCs). Normally RBCs are shaped like discs, which gives them the flexibility to travel through even the smallest blood vessels. However, with this disease, the RBCs have an abnormal crescent (“sickle”) shape. This makes them sticky and rigid. They can get trapped in small vessels and block blood from reaching different parts of the body. This can cause pain and tissue damage.
SCD is an autosomal recessive condition. You need two copies of the gene to have the disease. If you have only one copy of the gene, you are said to have sickle cell trait.
Part 2 of 8
What Are the Symptoms of Sickle Cell Anemia?
Symptoms of sickle cell anemia usually show up at a young age. They may appear in babies as early as 4 months old, but generally occur around the 6-month mark.
While there are multiple types of SCD, they all have similar symptoms at different levels of severity. These include:
excessive fatigue or irritability (from anemia)
fussiness (in babies)
bedwetting (from associated kidney problems)
jaundice (yellowing of the eyes and skin)
swelling and pain in hands and feet
Part 3 of 8
What Are the Types of Sickle Cell Disease?
Hemoglobin is the protein in RBCs that carries oxygen. It normally has two alpha chains and two beta chains. The four main types of sickle cell anemia are caused by different mutations in these genes.
Hemoglobin SS Disease
Hemoglobin SS disease is the most common type of sickle cell disease. It occurs when you inherit copies of the hemoglobin S gene from both parents. This forms hemoglobin known as Hb SS. As the most severe form of SCD, individuals with this form also experience the worst symptoms at a higher rate.
Hemoglobin SC Disease
Hemoglobin SC disease is the second most common type of sickle cell disease. It occurs when you inherit the Hb C gene from one parent and the Hb S gene from the other. Individuals with Hb SC have similar symptoms to individuals with Hb SS. However, the anemia is less severe.
Hemoglobin SB+ (Beta) Thalassemia
Hemoglobin SB+ (beta) thalassemia affects beta globin gene production. The size of the red blood cell is reduced because less beta protein is made. If inherited with the Hb S gene, you will have Hemoglobin S Beta thalassemia. Symptoms are not as severe.
Beta-zero thalassemia is the second type of beta thalassemia. It has similar symptoms to Hb SS anemia. However, sometimes the symptoms of beta-zero thalassemia are more severe. It is associated with a poorer prognosis.
People who only inherit a mutated gene from only one parent are said to have sickle cell trait. They may have no symptoms or reduced symptoms.
Part 4 of 8
Who Is at Risk for Sickle Cell Anemia?
Children are only at risk for sickle cell if both parents carry sickle cell trait. A blood test
called a hemoglobin electrophoresis can also determine which type you might carry.
People from regions that have endemic malaria are more likely to be carriers. This includes people from:
Part 5 of 8
What Complications Can Arise from Sickle Cell Anemia?
SCD can cause severe complications. These complications appear when the sickle cells block vessels in different areas of the body. Painful or damaging blockages are called sickle cell crises.
Anemia is a shortage of RBCs. Sickle cells are easily broken. This breaking apart of RBCs is called chronic hemolysis. RBCs generally live for about 120 days. Sickle cells live for a maximum of 10 to 20 days.
Hand-Foot Syndrome occurs when sickle-shaped RBCs block blood vessels in the hands or feet. This causes the hands and feet to swell. It can also cause leg ulcers. Swollen hands and feet are often the first sign of sickle cell anemia in babies.
Splenic sequestration is a blockage of the splenic vessels by sickle cells. It causes a sudden, painful enlargement of the spleen.
Delayed growth often occurs in people with SCD. Children are generally shorter but regain their height by adulthood. Sexual maturation may also be delayed. This happens because sickle cell RBCs can’t supply enough oxygen and nutrients.
Neurological complications include seizures, bleeding in the brain, or even coma. They are caused by brain blockages. Immediate treatment should be sought.
Blindness is caused by blockages in the vessels supplying the eyes. This can damage the retina.
Skin ulcers in the legs can occur if small vessels there are blocked.
Heart Disease and Chest Syndrome
Since SCD interferes with blood oxygen supply, it affects the blood vessels. Over time, this can lead to an enlarged heart and subsequent heart disease. This can also lead to pain known as chest syndrome. High blood pressure (hypertension) and stroke may also develop.
Priapism is a lingering, painful erection that can be seen in some men with sickle cell. This happens when the blood vessels in the penis are blocked. It can lead to impotence if left untreated.
Gallstones are one complication not caused by a vessel blockage. Instead, they are caused by the breakdown of RBCs. A byproduct of this breakdown is bilirubin. High levels of bilirubin can lead to gallstones. These are also called pigment stones.
Part 6 of 8
How Is Sickle Cell Anemia Diagnosed?
All newborns in the United States are screened for sickle cell disease. Prebirth testing looks for the sickle cell gene in your amniotic fluid.
In children and adults, one or more of the following procedures may also be used to diagnose sickle cell.
Detailed Patient History
This condition often first appears as acute pain in the hands and feet. Patients may also have:
severe pain in the bones
painful enlargement of the spleen
ulcers of the legs
Your doctor may want to test you for sickle cell anemia if you have any of the symptoms mentioned above.
Several blood tests can be used to look for SCD:
blood counts can reveal an abnormal Hb level in the range of 6 to 8 g/dL
blood films may show RBCs that appear as irregularly contracted cells
sickle solubility tests look for the presence of Hb S
Hb electrophoresis is always needed to confirm the diagnosis of sickle cell. It measures the different types of hemoglobin in the blood.
Part 7 of 8
How Is Sickle Cell Anemia Treated?
A number of different treatments are available for SCD:
Antibiotics like penicillin may be given from 2 months of age to 5 years. They help prevent pneumonia in sickle cell patients.
Supplemental oxygen is given through a mask. It makes breathing easier and improves oxygen levels in the blood.
Pain medication is used to relieve the pain during a sickle crisis. You may need over-the-counter drugs or strong prescription pain medication like morphine.
Hydroxyurea (Droxia, Hydrea) helps to increase production of fetal hemoglobin. It may reduce the number of blood transfusions.
Immunizations can help prevent infections. Patients tend to have lower immunity.
Surgery may be used to treat persistent and painful erections.
Bone marrow transplant has been used to treat sickle cell anemia. Children younger than 16 years of age who have severe complications and have a matching donor are the best candidates.
Blood transfusions can also treat sickle cell anemia. Packed red cells are removed from donated blood and given to patients. This improves transport of oxygen and nutrients.
There are things you can do at home to help your sickle cell symptoms:
Use heating pads for pain relief.
Take folic acid supplements, as recommended by your doctor.
Adequate fruits, vegetables, and whole-wheat grains are important and can help your body make more RBCs.
Drink more water to reduce the chances of sickle cell crises.
Exercise regularly and reduce stress to reduce crises, too.
Support groups can also help you deal with this condition.
Part 8 of 8
What Are the Long-Term Outcomes of Sickle Cell Disease?
The prognosis of the disease varies. Some patients have frequent and painful sickle cell crises. Others only rarely have attacks.
Sickle cell anemia is an inherited disease. Talk to a genetic counselor if you are worried that you might be a carrier. This can help you understand possible treatments, preventive measures, and reproductive options.