Sickle cell disease: What to do if your child is unwell

What is sickle cell disease (SCD)?

Sickle cell disease (SCD) is an inherited blood disorder. It is not contagious. SCD is more common in people with an African or Caribbean background, but children of Middle Eastern, Mediterranean and South Asian ancestry are also affected.

Sickle cell shape

What to do if your child with sickle cell disease is unwell

Temperature

1. First, have your child sit or lie down in a quiet place.
2. Take their temperature right away.
3. If their temperature is higher than 38°C under the armpit or 38.5°C by mouth, take them to the closest emergency department.
4. Check if there is any sign of breathing problems? If yes, take them to the emergency department.
5. For younger children examine your child's abdomen. If the spleen is felt to be enlarged take your child to the emergency department.
6. Finally, if their temperature is less than 38°C and there is no sign of breathing problems then do a pain assessment.

Doing a pain assessment

1. Ask your child what level their pain is 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.
2. If the pain is less than 7/10, give them pain medication as prescribed by their doctor.
3. If the pain is localized to one or two areas, a warm pack can be applied to the site of pain. ICE PACKS SHOULD BE AVOIDED.

Children five 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 now?"

Pain assessment numerical/visual analogue scale

Pain management

Medications

1. Morphine _____ mg. Repeat the dose every four hours.
2. Before each dose, take your child's temperature. If their temperature is higher than 38°C under the armpit or higher than 38.5°C by mouth go to the nearest emergency department right away.
3. Acetaminophen _____ mg. Repeat the dose every four hours.
4. Ibuprofen _____ mg. Repeat the dose every six hours.
5. PEG3350 (RestoraLAX/Lax-A Day/ClearLax). Take _____ g by mouth daily if needed.

Physical

There are also physical strategies that your child can try to help reduce their pain. These include:

1. Give your child plenty of fluids. Do not give your child cold fluids.
2. Gently massage the area.
3. Apply heat to the area.

Tips for using heat

You can use heat with children three years of age and older. Do not leave young children unattended with heat.

If the area with heat applied to it becomes painful, uncomfortable or a local skin reaction develops, remove the heat immediately.

To apply heat you can:

• Use disposable, instant hot packs. Be sure to follow the instructions on the package.
• Use warm blankets from the dryer
• Use warm baths
• Apply it in 20-minute intervals

Psychological

There are also psychological strategies that your child can try to help reduce their pain. These include:

• deep breathing
• relaxation exercises
• distractions (movies or music)
• imagery

What to do when your child with sickle cell disease has a fever

Children with SCD are at greater risk of infections than their peers. This is in part because with SCD, the spleen does not function properly and it is not as good at destroying the cell coating of some bacteria. They should be receiving additional vaccinations to prevent infections caused by the pneumococcal and meningococcal bacteria. If your child is under the age of five, they should be on preventative antibiotics.

Fever in a child with SCD is considered an emergency and requires prompt treatment with antibiotics. Fever is a sign of an infection.

A thermometer must be available at home for assessing the temperature of your child if they are feeling unwell. A temperature higher than 38°C under the arm or higher than 38.5°C by mouth requires that they be seen promptly at an emergency department.

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.

Correspondingly, 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.

Special situations where parents should call 9-1-1

If any of the following occurs, seek immediate medical attention or call 9-1-1 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