What you need to know
Children with SCD need 24 hour access to medical facility that can provide urgent evaluation and treatment of any acute illness. Hydration and anti-inflammatory agents and pain medications (nonsteroidal anti-inflammatory and narcotic analgesia) are helpful.
Up to date immunizations are very important. A yearly check up and studies as needed should occur in the child’s Medical Home. Yearly vision screening can be done at the school. The school should contact the children's primary care doctor before allowing them to join in sports.
No special diet is required for sickle cell disease, although a well balanced diet is important. If a child with sickle cell disease (SCD) is eating poorly it is important to let parents know. Hydration is very important. Children with sickle cell disease should be allowed unrestricted access to drinking water. Caffeine causes blood vessels to restrict so should be avoided.
Be aware, or ask a parent, if the child has a medical alert bracelet.
NOTE: It is important to be sensitive to cultural differences in diet.
Pain is caused by sickled red blood cells which are stiff and inflexible. They get stuck in the small blood vessels and become trapped causing “log jams” inside a blood vessel. This leads to tissues and organs being deprived of oxygen rich blood and poor blood flow. If blood flow is reduced in even a small area of the body, it can cause pain and sometimes swelling. Typically, younger children often complain of pain in extremities. Older individuals more commonly experience pain in the head, chest, abdomen, and back.
Pain can be anywhere and can vary in length, location, intensity, and time between episodes.
- Pain medication and hydration is important for treatment.
- Pain crises are the most frequent cause of sickle cell disease-related hospital admissions as well as school and work absences
Anemia is caused by the altered structure of the red blood cells. This change causes the cells to break down more rapidly, leading to anemia. Anemia can lead to shortness of breath, fatigue, and delayed growth and sexual development in children. The rapid breakdown of red blood cells can lead to jaundice (yellowing of eyes and skin).
Dactylitis is one of the earliest signs of SCD in infants and children. Dactylitis is pain and/or swelling of the hands or feet, sometime referred to as “hand-foot syndrome.”
Sickling events lead to restriction in the blood supply (ischemia) and variable degrees of the destruction of red blood cells. This can lead to multi-organ damage and chronic pain. Organs can include: bones, lungs, liver, kidneys, brain, eyes, and joints.
When sickle cells are trapped in the blood vessels inside and leading out of the spleen, the normal flow of blood is blocked. This can lead to the spleen being engorged or over-filled with blood cells. This occurs in 10-30% of children with SCD. Children with spleen issues may experience abdominal pain, nausea, and vomiting. Signs of spleen problems include: irritability, unusual sleepiness, appearing pale, weakness, rapid heartbeat, big spleen, pain on the left side of the abdomen.
This is painful and unwanted erections which often start in childhood and often occur in the early morning hours.
Some infections that may occur in individuals with SCD are:
- Septicemia (infection of the blood)
- Meningitis (infection around the brain) with pneumococcal and other bacteria
- Pneumonia (infection in the lungs)
- Osteomyelitis (infection of the bone)
- The most common cause of death in children with SCD is Streptococcus pneumonia sepsis. Death risk highest in the first few years of life.
- Most children with SCD are vaccinated against these organisms and begun on prophylactic penicillin which decreases the incidence of infections.
Acute chest syndrome (similar to pneumonia) is caused by red blood cells trapped in the lungs. This is a form of acute lung injury in patients with SCD and a major cause of mortality. It can develop rapidly. Signs of acute chest syndrome include chest pain, fast breathing and or retractions, congested “pneumonia-like cough,” abdominal pain, fever, trouble breathing.
Aplastic crisis is caused by an infection caused by Parovirus B19. This infection causes red blood cell (RBC) production to be shut down for 10 days. The RBC in children with SCD only live for 10-15 days compared to normal red blood cells that live 120 days. Therefore with this infection, the blood count drops. Signs of aplastic crisis include; paleness, lethargy, not feeling good, headache, fever, low blood count, recent respiratory infection, and passing out.
Presenting signs and symptoms include; severe headaches, marked dizziness, visual changes, acute onset of weakness in a limb or one side of face, sudden inability to produce speech, or a seizure. It occurs more often in children than adults. The peak occurrence is between 2-9 years. The most common cause of stroke in children is cerebral infarction or a blockage of oxygen supply to the brain by sickled cells.
Bedwetting may occur from age 3-adulthood in some individuals. It is more common in boys.
What you can do
Children who have SCD should have access to the nurse's office as requested.
Avoidance of climate extremes is important. Be aware of signs of infection. Any infection in a child who has SCD is an emergency. Prompt evaluation and treatment of underlying infections is essential. Symptoms might include: fever, coughing, vomiting, diarrhea, crankiness, rapid breathing, pale color, unusual sleepiness, trouble breathing.
A plan to deal with mild-to moderate episodes of pain should be in place. The main treatment for pain episode is supportive. Pain episodes are managed with a multi-model approach (i.e. warmth, massage, distraction, acupuncture, bio-feedback, and self-hypnosis).
School Personnel should realize that prophylactic antibiotics (including penicillin in children), may need to be taken at school even when no infection is present.