What is sickle cell disease (SCD)?
Sickle cell disease (SCD) is an inherited blood disorder. It is not contagious. Most children with SCD are of African ancestry, but children of Middle Eastern, Mediterranean and South Asian ancestry are also affected.
Signs and symptoms of sickle cell disease
The two main characteristics of SCD are a long-standing anemia and repeated episodes of vaso-occlusion.
- Anemia is a result of increased breakdown of red blood cells. Your child may appear pale and have yellow eyes from time to time. They may become tired sooner than their peers when doing an activity.
- Vaso-occlusive episodes are blockages of the blood vessels anywhere in the body by deformed red blood cells. This causes a lack of oxygen in the affected area of the body. Symptoms depend on where the blood vessels are blocked.
The most common symptom of a vaso-occlusive episode is bone pain. Any bone can be affected, including the arms, legs, back and skull. These episodes, commonly called pain crises, are unpredictable. Some children do not feel well before the actual onset of pain and can let an adult know.
Possible triggers for a pain crisis include:
- exposure to cold and very hot temperatures
Some pain crises happen without a known reason.
Preventing pain crises
You can help prevent a pain episode by:
- giving your child 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
- sending an extra sweatshirt and socks to school in case your child becomes wet during recess or at any time
- recognizing fever as a sign of infection and having your child 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, though, children may still have a pain crisis.
What to do if your child with sickle cell disease is unwell
- First, have your child sit or lie down in a quiet place.
- Take their temperature right away.
- If their temperature is higher than 38°C under the armpit, or 38.5°C in the mouth, take them to the closest Emergency Department.
- If their temperature is less than 38°C, do a pain assessment.
- Is there any sign of breathing problems? If yes, take them to the emergency department. If no, do a pain assessment.
- For younger children, examine your child’s abdomen. If the spleen feels enlarged, take your child to the nearest emergency department.
Doing a pain assessment
- Ask your child about their level of pain on a scale of 0 to 10, with 0 being no pain and 10 being “the worst pain ever”. Ask where the pain is located.
- If the pain is less than 7/10, give them pain medication as prescribed by their doctor.
- If the pain is limited to one or two areas, you can apply a warm pack to the site of pain. Do not use ice packs.
Children (5 to 8 years of age)
Ask your child: "If 0 is no pain and 10 is the worst pain you can imagine, what are you feeling now?"
Older children (8 years of age and older)
Ask your child: "If 0 is no pain and 10 is the worst pain you can imagine, what are you feeling right now?"
- Morphine _____ mg. Repeat dose every four hours.
- Before each dose, take your child's temperature. If his temperature is higher than 38°C under the armpit, go to the nearest emergency department right away.
- Acetaminophen _____ mg. Repeat dose every four hours.
- Ibuprofen _____ mg. Repeat dose every six hours.
- Lactulose _____ mL x one dose.
- Give your child plenty of fluids. Do not give cold fluids.
- Gently massage the area.
- Apply heat to the area.
Tips for using heat
- Use disposable, instant hot packs. Be sure to follow the instructions on the package.
- Use warm blankets from the dryer.
- Use warm baths.
- Apply heat in 20 minutes intervals – 20 minutes with heat, 20 minutes without.
- Do not leave babies and young children unattended with heat. Use heat with children 3 years of age and older.
- If the area becomes painful or uncomfortable or if a local skin reaction develops, remove heat immediately.
- Have your child practise deep breathing.
- Encourage your child to do relaxation exercises.
- Use distractions (movies or music).
- Use imagery – have your child imagine a favourite place or memory.
With SCD, the spleen function is not as good at destroying the cell coating of some bacteria. If your child is under the age of 5, they should be on preventative antibiotics. Also, they should be receiving additional vaccinations to prevent infections caused by the pneumococcal and meningococcal bacteria.
Fever in a child with SCD is considered an emergency and requires prompt treatment with antibiotics. Fever is a sign of infection.
A thermometer must be available at home for assessing the temperature of your child if they are feeling unwell. Go to the nearest emergency department promptly if your child's temperature is higher than 38°C under the arm and higher than 38.5°C by mouth.
Medicines such as acetaminophen and ibuprofen will reduce fever but will not affect the infection that is causing the fever. Using them can lead to a false sense of security or to the fever not being taken seriously. Do not give your child these medicines to treat a fever.
Fluid requirements for children with sickle cell disease
Children with SCD excrete higher volumes of urine compared with their peers because their kidneys cannot concentrate urine.
When a child produces more urine than usual, they must also increase their fluid intake. This is particularly important in SCD, as dehydration can trigger pain crises. When a child with SCD is dehydrated, blood cells also become dehydrated and change shape, causing blood vessel blockage and acute pain. Make sure your child has easy access to water at all times.
Sports and extra-curricular activities for children with sickle cell disease
Children with SCD should be encouraged to participate fully to the best of their ability and according to their tolerance level. As a result of low hemoglobin level (anemia), your child will tire faster than their peers with physical activity. Encourage your child to try out for school teams and participate in gym but with more frequent rest periods and increased hydration.
It is a good idea for you to talk to your child’s physical education teacher about the physical discomforts associated with SCD and any symptoms your child has had in the past with physical activity. Try to reach an understanding with the physical education teacher regarding expectations for your child. Children with SCD require exercise like all children and should be encouraged to participate to their tolerance level. Do not restrict your child from all physical activity.
Special precautions for swimming
Your child can participate in swimming classes. However, they should limit the time in the pool to 30 minutes. After getting out of the water, they must dry off and change into dry clothing right away. They should not run around in cold, wet swimsuits as their body temperature will fall, potentially resulting in bone pain.
Special precautions for hot weather
When exposed to very hot temperatures, such as during a sporting event in the summer, your child can participate but with frequent rest breaks and increased hydration.
Special precautions for winter activities
Your child should be allowed to participate in winter activities, 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 - 5°C, your child should be excused from participating.
Academic performance for children with SCD
Most children with SCD will perform as well as their peers, so expectations for your child should be similar to those of other children at the same age. Having a chronic illness at times results in reduced self-esteem and lack of confidence. Encouraging your child is essential.
There are several reasons why some children with SCD may have exceptionalities.
Missed school days
Your child may miss school because of:
- scheduled clinic visits
- inpatient hospitalizations to treat the severe bone pain associated with SCD
- less severe painful bone crises being treated at home
This means your child may have less instructional time. It has been proven that school attendance is directly related to academic performance. As a parent, you could speak with your child’s teacher about setting up a homework buddy program for your child. A “buddy” would be assigned to your child to collect handouts and notify them of important future deadlines. Also, many teachers have websites where they post homework assignments and future dates for both parents and students. Ask your child’s teacher to provide make up work when your child has missed time at school. Maintain open communication with your child’s teachers.
One child in five with SCD has so-called “silent strokes.” These are small strokes in the frontal area of the brain that go undetected unless an MRI is performed. The frontal area of the brain controls executive functioning. This affects your child’s ability to focus, organize, plan and memorize. As children move to the higher grades they may experience increasing difficulty.
One in 10 children with SCD will have overt strokes. These result in diminished academic performance and various degrees of physical disability. Most overt strokes can be prevented with a chronic transfusion program, and when children with SCD are identified early in life.
If your child is finding school work challenging
If your child is having challenges academically, neuropsychological testing should be undertaken and appropriate IEP intervention done on an individual basis. This would require your written consent.
Children with SCD are expected to have a life expectancy well into the 6th and 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.
Special situations where parents should call 911
If any of the following occurs, call 911 right away:
- difficulty breathing
- loss of consciousness
- severe headache
- difficulty speaking or slurring of speech
- weakness of limbs
- seizure activity
- fever greater than 39°C
- unexplained lethargy/sleepiness
- persistent vomiting
- recognition of an enlarged spleen.
- Sickle cell disease (SCD) is an inherited blood disorder.
- The two main characteristics of SCD are 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.
Vaso-occlusive episodes are blockages of the blood vessels by deformed red blood cells.
- Infection, fatigue, and dehydration are possible triggers for a pain crisis.
- Do not use ice packs to treat pain.