Sickle Cell Disease: Course of Illness

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Sickle Cell Disease: Course of Illness




Guest:  Dr. Sherron Jackson – Pediatrics - Hematology / Oncology

Host:  Dr. Linda Austin – Psychiatry


Dr. Linda Austin:  I’m Dr. Linda Austin.  I’m talking, today, with Dr. Sherron Jackson who is Associate Professor of Hematology and Oncology; hematology is the study of blood, and works in the Children’s Hospital of the Medical University of South Carolina.  Dr. Jackson, in another podcast, we talked about the genetics of sickle cell anemia, but in this segment, let’s turn to talking about the clinical experience, the health experience, of the child with sickle cell anemia.  What are some of the first symptoms that a parent might observe in their child?


Dr. Sherron Jackson:  Certainly.  We mentioned the development of sickle cells, the abnormally-shaped red blood cells and how they have a tendency to become clumped together and stuck in the small vessels in the body and in the various organs.  The consequence of this jam of cells is a problem with delivery of oxygen to the tissues.  The tissues, often, then, become injured or damaged, put out chemicals that cause pain receptors to be stimulated, so the patients recognize pain.  And, interestingly, in newborns and, in fact, infants, up until about six or seven months of age, there may not be any symptoms of sickling at all.  And this is wonderful because of the presence of fetal hemoglobin. 


The hemoglobin that we have as babies and as young infants does not cause a cell to sickle.  It ends up protecting, actually, the baby from some of the effects of the sickling.  So, we may not see any signs of pain or injury to the tissues in the first six or seven months of life.  As a baby gets older, the fetal hemoglobin converts to sickle hemoglobin.  And, after you have a majority of sickle hemoglobin in the body, in the bloodstream, then the cells do have the tendency to take on the sickle shape.  So, we talk about a painful episode in a young infant, it might manifest as hand-foot syndrome, we call it, where the fingers and toes and the tops of the hands and feet become swollen and painful and tender. 


This pain crisis, we call it, or painful episode, may last a few days.  As the children get older, the pain moves more to the long bones, the bones of the arms and legs.  There may or may not be local swelling, but the patient can tell you they’re having pain in the leg.  It’s difficult to walk.  It’s difficult to use that extremity because of the pain.  And, then, as teenagers and adults, the pain becomes more centralized and the trunk is involved, so it would be the bones of your chest, which would include your sternum and ribs and the bones of your lower back, as well as the bones of the pelvis and hips.  So, we see a change from the periphery of the body, the distal parts of the body, and pain becomes more centralized as patients get older.


Dr. Linda Austin:  Now, how do you, as a pediatric hematologist, help a child through a crisis?  What can you do to make it more comfortable?


Dr. Sherron Jackson:  The main focus of treating a crisis is pain management.  We have a variety of techniques to use.  The most obvious ones would be pain killers, medications that take away pain.  And we use common medicines, like Tylenol; acetaminophen is the other name, or Motrin, and ibuprofen, its other name.  These are medicines that are over-the-counter.  They come in liquid form or tablet form.  And they would serve as the first line of pain management, as far as medications are concerned, in our patients.  The more severe pain episodes may require IV, intravenous, pain medication.  We talk about morphine, a very powerful narcotic.  This is often the medication that’s required to manage bone pain.  The other thing that helps is increasing the fluid intake, by taking fluids orally or using IV tubing to administer fluids, and any additional measures to make the patient comfortable. 

Some patients require a hot pack.  Some require soothing music and a dark room, a quiet place, a quiet environment.  We use all of these measures together to try to make the patient more comfortable.


Dr. Linda Austin:  What happens to the form and frequency of these crises as that child goes into late adolescence and adulthood?


Dr. Sherron Jackson:  The typical pattern, we mentioned that in infants, the amount of sickle hemoglobin is low, as the children get older, their percentage of hemoglobin increases, and the more sickle hemoglobin you have, potentially, the more sickling episodes you would have.  And, remember that the blood cells travel to every part of the body, so that the symptoms begin to show up, or manifest, in other organs than just the bones.  So, as a patient becomes a teenager, we expect they may have sickling episodes in the lungs that look like pneumonia, behave similar to pneumonia, with respiratory symptoms, coughing, pain in the chest, shortness of breath, a lower oxygen level, haziness on the chest x-ray.  We would think it’s pneumonia, but it actually represents a sickling event in the lungs, and these episodes are more likely to occur as patients become teenagers and adults.


Dr. Linda Austin:  So, then, overall, does the illness tend to get worse with time, or better with time?  What do you usually see?


Dr. Sherron Jackson:  In most cases, because the sickling process has been ongoing, even silently, we say, because patients are not complaining everyday of pain or injury to the different organs, the sickling event is going on even if the patient is not complaining or symptomatic.  So, we would expect that as the patients become adults, the frequency of their sickling episodes, the severity of the episodes, would increase, and that the damage to the organs begins to show up now as young adults.


Dr. Linda Austin:  So, is the life expectancy altered by this, then?


Dr. Sherron Jackson:  As a result, the life expectancy is shorter than typical adults.  The patients with the most common form of sickle cell, we call it SS, typically, would live to be about 45 years old, instead of 70 years old, as other adults would live.  And that’s the consequence of injury to the organs that’s been taking place for forty years.


Dr. Linda Austin:  I see.  So, no wonder you think genetic counseling is so important.


Dr. Sherron Jackson:  That’s the reason counseling is important, and for couples to make a decision before they have a baby, to know if there’s the possibility their child might have sickle cell disease.  With newborn screening, we’re identifying the babies as soon as their born, within the first week or two of birth, and we’re able to take care of the children better because we know who they are.  We know which babies and which children are at risk for complications.  Before we did newborn screening, many children died in their young childhood from infections and sepsis, and we didn’t even know they had sickle disease.  So, now the life expectancy is better and longer because we’re identifying the babies at birth, and we want couples to know if they’re at risk for having a baby with sickle disease.


Dr. Linda Austin:  Dr. Jackson, thank you so much for talking with us today.


Dr. Sherron Jackson:  Thank you very much.


If you have any questions about the services or programs offered at the Medical University of South Carolina or if you would like to schedule an appointment with one of our physicians, please call MUSC Health Connection:  (843) 792-1414.

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