Pediatric Dental Health

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Sickle Cell Disease
Sickle cell disease is an inherited, chronic, blood disease in which the red blood cells become crescent-shaped and function abnormally. The sickle-shaped blood cells block the smallest vessels in the body's organs, thereby reducing their supply of oxygen, and causing severe damage to the organs. Sickle cell disease is caused by an abnormal type of hemoglobin - hemoglobin S.

Sickle cell disease affects one out of every 600 African-Americans, and occurs in 0.15% of African-American newborns. Eight percent of black Americans have sickle cell trait, which does not usually cause medical problems. In the United States, patients with sickle cell disease survive into their fifth and sixth decades of life.

Research on sickle cell disease was initiated at the end of World War II. In 1945, Dr. William B. Castle discovered that the sickled red blood cells of patients affected by the disease were only found in the veins, that is, in the deoxygenated blood. This meant that the sickling occurred after the red blood cells had released the life-giving oxygen. This gave future researchers a clue as to where the ultimate cause of sickle cell disease might be found. In 1949 Dr. Linus Pauling, America's leading physical chemist of the time, published a landmark scientific paper entitled "Sickle Cell Anemia, a Molecular Disease." In his famous paper, he and his research team demonstrated that the hemoglobin of sickle cell anemia patients had a different electrical charge from that of healthy people. This meant that the hemoglobin molecule was somehow different in these sick patients. Hemoglobin is the molecule in red blood cells which binds to oxygen, and then transports the oxygen to distant parts of the body. Dr. Pauling's discovery was very significant, because it showed that a very small change in the molecular structure of a protein could lead to the development of a devastating disease. In 1957 Dr. Vernon Ingram used a new technique (paper chromatography combined with electrophoresis) for separating different proteins. Using his new protein separation technique, he determined that there was only one amino acid which was different in the hemoglobin molecule of sickle cell patients. He found that the amino acid, valine, was substituted in place of the usual amino acid, glutamic acid, in the beta chain of the hemoglobin molecule. His new discovery would later be shown to be an example of single nucleotide polymorphism.

Sickle cell disease is chronic illness, punctuated by multiple crises, which can become life-threatening at any time. A sickling crisis in children can be caused by lack of oxygen in any part of the body, dehydration, infection - which can lead to acidosis, fatigue, or stress. Death in children who have sickle cell disease can be caused by infection, acute chest syndrome, splenic sequestration, or other causes.

Signs Of Sickle Cell Disease In Children:

The vaso-occlusive crisis is the most frequent sign in children. In infants, it appears as a painful swelling of both hands and feet (hand-foot syndrome), and is often the first sign of sickle cell anemia in children.
Later on in life, these painful vaso-occlusive crises occur in the hip, knee, chest, and back joints.
The acute chest syndrome. This is an acute respiratory tract illness, which is associated with fever, chest pain, and respiratory distress. It tends to affect children who are older than three years of age.
Episodes of abdominal pain, often excruciating.
Sequestration crisis. This is a painful increase in the size of the spleen, due to a blockage of the blood vessels in the spleen. This leads to acute hyperspleenism.

What The Dentist Needs To Do:
Consult with the child's primary pediatrician.
Request the child's most recent lab test results.
Schedule the dental appointment in the morning, when the child is well-rested.
Prescribe additional antibiotics, before and after invasive dental treatment, to prevent infections.
Recommend adequate fluid intake before the dental appointment.
Consider using nitrous oxide to reduce anxiety during dental treatment.
Initiate a strict preventive dental program for the child. This will help prevent dental disease and infections.

A recent study published in The New England Journal of Medicine found that the acute chest syndrome was the leading cause of death in patients with sickle cell disease. The most common causes of death for the 538 patients with sickle cell disease in the study were pulmonary fat emboli and bronchopneumonia.
Vichinsky EP, et al.: Causes and outcomes of the acute chest syndrome in sickle cell disease. The New England Journal of Medicine. 22 June 2000; 342(25) 1855-1865.

Does Your Child Need A Mouthguard?
An estimated 25 million youths in the United States participate in competitive school sports, and 20 million children and adolescents play organized out-of-school sports. Sports-related dental trauma continues to occur, but there is something we can do about it.

Properly designed and custom fabricated mouthguards are essential in preventing athletic oral-facial injury. The use of mouthguards reduces the likelihood of tooth fractures and tooth dislocations. Mouthguards protect against jaw fractures by absorbing the energy of a traumatic blow to the chin. They also protect against soft tissue lacerations, and against bruising of the lips and cheeks. Finally, mouthguards reduce the likelihood of concussions, cerebral hemorrhage, and unconsciousness during athletic activities.

In the early 1930's boxers began using rubber mouthguards to protect their teeth and lips during fights. Later, in the 1950's, the American Dental Association began promoting the use of mouthguards among athletes. In 1973 the National Collegiate Athletic Association required players to wear mouthguards during games. In 1995 the American Dental Association amended its recommendations for oral-facial protection to include all sports participants who were at risk for injury. Currently all states mandate the use of mouthguards during high school football, ice hockey, men's lacrosse, field hockey, and amateur boxing.

The American Dental Association estimates that mouthguards prevent approximately 200,00 injuries each year in high school and collegiate football. A 1989 study by Mc Nutt et al. found that 75 percent of oral-facial injuries occurred among athletes who did not wear a mouthguard. Hard tissue (dental) trauma was found to be 60 times more likely for athletes who wore no mouthguard than for those who did. In a 1997 study by Kumamoto et al., athletes learning to slam-dunk a basketball while not wearing a mouthguard suffered severe dental injuries as a result of entangling their teeth in the net.

Now, if mouthguards are so great, why don't more children, adolescents, and college students wear them? The main complaints about mouthguards include: discomfort, loose fit, difficulty breathing, and difficulty speaking. All of these problems, however, are found only in over-the-counter mouthguards, which 90 percent of athletes use. A properly fitted mouthguard is protective, comfortable, resilient, tear resistant, ordorless, tasteless, causes minimal interference with speaking, has an excellent fit, and is sufficiently thick to protect the teeth. Only your dentist can provide your child with a properly fitted mouthguard.

The Four Types Of Mouthguards:
Stock Mouthguards (OTC). Not recommended, due to lack of retention and lack of protective qualities.
Boil And Bite Mouthguards (OTC). Also not recommended, because they do not cover and protect all of the back teeth during impact. In addition, they are too thin to be protective.
Vacuum-Made Mouthguards. These are OK. They are made by the dentist on a stone cast of the athlete's teeth. A thermoplastic material is adapted to the stone cast using a special vacuum former. The vacuum-formed mouthguard is then trimmed and polished.
Pressure Laminated Mouthguards. These are the best! They are custom-made, multiple-layered mouthguards. High heat and pressure is used to produce a laminated mouthguard having two or three layers of mouthguard material. This type of mouthguard will provide a superb fit and great protection for the athlete.

How To Take Care Of The Mouthguard:
Rinse the mouthguard with cold water or mouth rinse before and after each use.
Brush and clean the mouthguard periodically with a soft toothbrush and toothpaste.
Store and protect the mouthguard in a carrying case, and keep the mouthguard wet during storage.
Avoid contact with hot water, high temperatures, and direct sunlight to minimize distortion of the mouthguard.
Periodically check the mouthguard for holes or tears, and replace as needed.
Bring the mouthguard to each dental visit so that your dentist can check its condition and dental fit.

A recent article in General Dentistry discusses mouthguards:
Ranalli DN: Sports dentistry in general practice. General Dentistry. March-April 2000; 48(2)158-164.

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