Sickle Cell Disease – by – Dr. Melanie Kirby
What you need to know about your student with Sickle Cell Disease.
Sickle Cell Disease (SCD)is an inherited blood disorder.It is not contagious. Children affected are of African ancestry in the majority, but also are of Middle Eastern, Mediterranean and South Asian ancestry.
The disorder is characterized by a long standing anemia and recurrent episodes of vaso-occlusion.
Anemia is a result of increased breakdown of red blood cells. Children may appear pale and have yellow eyes from time to time.
The other cardinal feature of SCD is vaso-occlusive episodes, that is, blocking of the blood vessels anywhere in the body by deformed red blood cells. This presents most commonly as bone pain. Pain can start suddenly while doing an activity, or afterwards. This can be severe pain. Symptoms seen depends on where the blood vessels are blocked. If, for example, a blood vessel going to the brain is blocked, the child will show symptoms of a stroke ie, weakness of one side of the body. If the blood vessel is blocked going to a leg bone, the child will present with pain in the leg.
PAIN
Pain is caused in part when red blood cells change shape, become distorted and block very small blood vessels. When the red cells are blocked from delivering oxygen to the bone of an arm or leg, for example, that bone lacking oxygen will hurt. Any bone can be affected including arms, legs, back, and skull. The episodes are episodic and unpredictable as to when they occur. Some children have a prodrome and verbalize when that they do not feel well prior to the actual onset of pain.
Triggers for pain crisis include:
• infection
• stress/fatigue
• dehydration
• exposure to cold and very hot temperatures
• unknown reasons
Pain crisis can be prevented by:
• giving children lots of fluids to drink so they are not thirsty
• dressing them in a few layers of warm clothing in the winter when they are leaving home
• recognizing fever as a sign of infection and having them seen immediately by a doctor
• Avoiding vigorous exercise without the ability to take breaks and drink fluids, especially during hot days.
Even with these measures children may still have a pain crisis.
What should you do in the classroom?
First have the student sit or lie down in a quiet place.
Take the temperature.
If temperature is greater than 37.5 C under the armpit, or greater than 38 Cin the mouth , call the parent immediately to take the student to the ER. If less than 37.5C then do a pain assessment.
Is there any sign of breathing problems? If yes, call an ambulance. If no, move to do a pain assessment.
Pain assessment:
Ask the student what level on a scale of 1 to 10 his pain is and where it is located.
If the pain is less than 7/10 and the parents have given the school teacher/nurse a supply of pain medication, the child can have a dose given as prescribed on record.
If no medication is at school to treat pain or if greater than 7/10, then the parent should be called to pick up the student.
A warm pack can be applied to the site of pain if localized to one or 2 areas until the child is picked up.
FEVER
Children with Sickle cell Disease are at greater risk of life-threatening infections than their peers. The reason for this is in part due to the fact that their spleen function is not as good at destroying the cell coating of some bacteria. Fever is considered an emergency requiring prompt treatment with antibiotics.
A thermometer should be available at school for assessing the temperature of children if they present unwell while at school. A temperature under the arm of greater than 37.5 degrees C requires that the child be seen promptly at an ER. The parents can be called to pick up the child or if they are not reachable in a given time (perhaps an hour) then the child should be taken to the ER.
A note regarding medications to reduce fever. These will reduce fever, but will not affect the infection that is causing the fever. It is preferred that they not be used as this can lead to a false sense of security when the fever falls and the infection is not treated, or fever not taken seriously.
FLUID REQUIREMENTS AND BATHROOM PRIVELEDGES
Children with SSD excrete increased volumes of urine. Their kidneys are unable to concentrate urine, and as a result make increased volumes of urine compared with their peers. Bathroom privileges are a must whenever the student needs to go. Attempts to hold urine will result in accidents and embarrassment to the student and the potential for future teasing.
On the flip side, when one produces more urine than usual, there is a need to increase the fluid intake. This is particularly important in SSD, as dehydration can be a precipitating cause for the onset of acute painful episodes. When the patient is dehydrated, blood cells also become dehydrated and change shape with resultant blood vessel blockade and acute pain. We recommend that the student be allowed to have a water bottle at his/her desk so that they will have easy access to water. This will reduce the disruption of leaving the classroom for the water fountain.
SPORTS AND EXTRA-CURRICULAR ACTIVITIES.
Students with SSD should be encouraged to participate fully to the best of their ability and to their tolerance level. As a result of a low hemoglobin level (anemia) they will tire faster than their peers with physical activity. They should be encouraged to try out for school teams, participate in gym, but with more frequent rest periods and increased hydration. They should be allowed to participate up to their tolerance level.
Special precautions:
a) Swimming:
Students with SSD can participate in swimming classes. They should however limit the time to one half hour and when out of the water immediately dry off and change into dry clothing. They should not run around in cold, wet swimsuits as their body temperature will fall and they potentially start having bone pain.
b) Hot weather:
When exposed to very hot temperature e.g. during a sporting event in the summer, they can participate but with frequent rest breaks and increased hydration.
c) Winter activities:
Students should be allowed to participate, but they should be dressed well in layers appropriate for the temperature of the day. Areas of increased loss of body heat such as fingers, toes, head and ears should all be well covered. At temperatures less than minus 5 degrees C students with SSD should be excused from participating.
ACADEMIC PE RFORMANCE
Most children with SSD will perform as well as their peers and so expectations for students who have SSD should be similar to those of their peers. Having a chronic illness at times results in reduced self esteem and lack of confidence. Encouragement of the student is essential.
There are several reasons why some students with SSD may have suboptimal academic performance. Firstly, there are school absences related to inpatient hospitalizations necessary to treat the severe bone pain associated with SSD. There are also scheduled clinic visits for several tests required for good health.
One in 5 children with SSD has so called silent infarcts. These are small strokes in the frontal area of the brain which are undetected unless an MRI is performed to look for them. The frontal area of the brain is responsible for executive functioning and this affects a student’s ability to focus,organize, plan and memorize work required.
One in 10 children with SCD under the age of 20years old will have overt strokes. These result in diminished academic performance, and various degrees of physical disability. Thankfully most overt strokes can be prevented with a chronic transfusion program when the children are identified early in life. When a student is identified to be having difficulties academically, neuropsychological testing should be undertaken and appropriate IEP intervention done on an individual basis.
Whenever a teacher is concerned about a student’s health , with the parents written permission they should seek out the health care team involved with the student as a resource. Children with SCD are expected to have a life expectancy well into the 7th decade, so they should be prepared for the work force in a manner that is suitable for the adult with SCD. As a result of the anemia, most adults will not be able to pursue careers which involve heavy sustained physical activity. It is therefore essential that they be steered away from such careers and into less physically demanding jobs. This makes achieving a good education even more important.
HOW CAN A TEACHER HELP THE STUDENT WITH SCD ACHIEVE ACADEMICALLY AND SOCIALLY IN THE CLASSROOM,
1. Learn about Sickle cell Disease and how it has impacted your student
2. Provide make-up work for students who have missed days from school due to illness.
3. If your student is not performing optimally, suggest a Neuropsychological assessment so an IEP can be done to optimize learning.
4. Have free and open communication with the student’s parents so you can work as a team to optimize performance.
5. Dispel any myths or rumors about your student that may be in the classroom e.g. reasons for the student’s eyes being yellow.
SPECIAL SITUATIONS WHERE THE TEACHER SHOULD CALL 911
If any of the following occurs, the emergency services should be called:
Difficulty breathing
Loss of consciousness
Severe headache
Difficulty speaking or slurring of speech
Weakness of any limbs
Seizure activity
Fever of greater than 39 degrees.
Unexplained lethargy/sleepiness
Persistent vomiting