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September is Sickle Cell Disease Month: CDU’s Dr. Samuel Shacks Cautions Parents of Sufferers to Beat the Heat
By Sentinel News Service
Published September 18, 2015

DRThis sweltering September heat has driven bedroom thermometers almost to 97 degrees, not to mention the humidity to 63%– numbers that Angelenos are struggling with. But what about the child with Sickle Cell Disease (SCD) that is often outside playing in the sun or cooped up inside hot steamy rooms?

This sweltering September heat has driven bedroom thermometers almost to 97 degrees, not to mention the humidity to 63%– numbers that Angelenos are struggling with. But what about the child with Sickle Cell Disease (SCD) that is often outside playing in the sun or cooped up inside hot steamy rooms?



“Hydration is something that is of great concern in patients with Sickle Cell Disease and heat only makes the need for hydration greater,” said Dr. Samuel James Shacks. Dr. Shacks has been studying Sickle Cell Disease since his days of running the Sickle Cell Clinic in the late 1980s through the 1990s, when he taught for almost three decades at Charles R. Drew University of Medicine and Science (CDU). “Water is great, they don’t need Gatorade or anything like that.” Dehydration, coupled with the anemia and the resulting lower oxygen levels in a person’s blood may worsen red blood cells sickling. Conversely, keep children warm in cold weather, because cold air, wind, and cold water can also trigger a sickle cell crisis.


Dr. Shacks, also known for being the first African American to earn a PhD degree from UC Irvine–before he earned his MD there–hails from Pine Bluff, Arkansas, so he knows about those dog days of summer heat. His PhD is in Molecular Biology and Bio-Chemistry. To please his mother, he became a pediatrician and taught at CDU, gravitating toward research and treating patients with Sickle Cell Disease.


“My colleague, Dr. Stephen Taylor and I, took responsibility for the Sickle Cell Disease Clinic with 125 enrollees. It allowed me to complement the work that I was doing as President, board of directors of the Sickle Cell Disease Research Foundation, (now known as the Sickle Cell Disease Foundation of California.)



The semi-retired pediatrician spent much of his focus on children who suffered with the most serious cases of sickle cell anemia, touched by the levels of pain they had to endure when their red blood cells would form crescent shapes impeding their course through their arteries and veins, causing unbearable discomfort.


What is Sickle Cell Disease?


“It’s a hereditary condition involving an abnormality in the structure of the hemoglobin technically called a “Hemoglobinopathy,” explained Dr. Shacks. “Hemoglobin is the molecule that carries oxygen. In its natural state we are able to deliver oxygen to all of the areas of the body, to the tissues and things that keep us going.


“However, if you have the severe form of the disease, then you have a process where you have crescent shaped red blood cells that are formed. Those red blood cells are a result of the change in the solubility of the hemoglobin molecule itself. That means that you will have blockage of very small arterioles and capillaries leading to a painful crisis because they are not going to get the oxygen to those areas and they will have more in the way of lactic acid buildup. For most people that go out to jog and do things like that, the burn that they feel is due to lactic acid buildup, so they stop. But for Sickle Cell Disease sufferers, there is no stopping the pain.” Of course there are treatments to manage the pain and best practices to keep those episodes at bay.


What symptoms does the parent or guardian look for?


“Thank God, we finally got to the point where there is Universal Testing for newborns. Parents will be notified just as parents of babies born with Tay Sachs and other hereditary diseases are,” said Dr. Shacks.


He said one of the things that happens to very young children is called dactylitis, which is a child having more pain and changes about the joints of the extremities–fingers and toes–that will cause them quite a bit of discomfort. They may notice swelling and edema that goes along with the bony changes. These changes usually occur in someone with the severe form of the disease.


Respiratory difficulty: Kids with Sickle Cell Disease are 100 more times likely to have pneumonia than a normal child.


Heart disease: There is a known syndrome with Sickle Cell that effects the heart. Of course we are talking about Sickle Cell Anemia, so sufferers have more in the way of a burden put on the heart because there is an increased need for blood to move around due to the lack of sufficient numbers of red blood cells.


Brain impairment: These children can also have things occurring in the brain. “You have to consider, is this a child sitting around not doing anything?” asked Dr. Shacks. “’Does this child actually have intellectual deficits associated with the disease? Or is there something else that is going on there?’ What I am getting at is this disease affects every part of the body,” he said.


“You have to know your child in terms of behaviors and look for signs that may indicate that you should be seeing a physician trained in hematology and not just a general doctor. Don’t sit at home and say ‘it will go away.” You’ve got to get out there and get help. A time delay isn’t warranted. A hematologist should be the overseer of this particular disease.”


Be an advocate for your child to receive appropriate pain medications:

“Often when African Americans come in to doctors’ offices they are seen as drug seeking. As a result, SCD patients haven’t always received the high quality narcotic pain relief that they should have. There is good evidence showing that if the narcotics are being prescribed for pain, patients don’t become addicted. They use it as it is intended to be used: therapeutically. You can’t just give them Tylenol and send them home. You’ve got to treat the pain because it is a chronic pain. You must bring it to an endpoint so they can get to the next level. The treatment will be combined with effective care and therapy for their anemia,” he said.


Tips for dealing with Sickle Cell Disease by Dr. Shacks:



  • Be very concerned about what stress factors trigger your child’s crises. Too many crises can lead to stroke, even in children.


  • Monitor exercise. The military has learned that there are severe consequences to strenuous exercise that can be fatal to people who just carry the trait.


  • Find a heated pool: For water sports and swimming you want to find a heated pool. You do not want your child splashing in a pool with a lower temperature that can precipitate a crisis.


  • Children with Sickle Cell shouldn’t live in a bubble but they should have oversight. Do not just drop your child off and leave them without some conscious guidance.


  • Skin abnormalities: Again, Sickle Cell Disease effects every part of the body including the skin. Be watchful for ulcers, particularly leg ulcers, excessive dryness or a rash that looks like a “slapped cheek.” The “slapped cheek” can be due to a specific viral infection.


The good news is that many living with sickle cell disease are living well past their former life expectancy. “If they do what all of us should be doing, and take care of themselves, eat right and exercise moderately, they will live well into advanced years,” said Dr. Shacks.


About Charles R. Drew University of Medicine and Science

CDU is a private, nonprofit, nonsectarian, medical and health sciences institution. Located in the Watts-Willowbrook area of South Los Angeles, CDU has graduated more than 575 medical doctors, 2,700 post-graduate physicians, more than 1,200 physician assistants, 615 nurses and hundreds of other health professionals. CDU’s mission is to develop a diverse group of health professional leaders who seek social justice, promote wellness, provide care with excellence and compassion and are uniquely qualified to transform the health of underserved populations through outstanding education, research and clinical services in the context of community engagement. For more information, visit www.cdrewu.edu/.


Categories: Health

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