Cell Disease: Course of Illness
Guest: Dr. Sherron Jackson – Pediatrics - Hematology / Oncology
Host: Dr. Linda Austin – Psychiatry
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?
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.
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
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.
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?
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.
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.
Linda Austin: What happens to the form
and frequency of these crises as that child goes into late adolescence and
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.
Linda Austin: So, then, overall, does
the illness tend to get worse with time, or better with time? What do you usually see?
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.
Linda Austin: So, is the life expectancy
altered by this, then?
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.
Linda Austin: I see. So, no wonder you think genetic counseling is
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.
Linda Austin: Dr. Jackson, thank you so
much for talking with us today.
Sherron Jackson: Thank you very much.
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