Sickle cell anaemia
Sickle cell anemia is a serious condition that causes red blood cells, which are normally flexible, to stiffen and adopt a crescent shape.
This means they don’t travel through the blood vessels easily, and they can get stuck and form clumps which block up the blood vessels. These blockages are referred to as crises – they’re painful and can damage the organs and make your child more vulnerable to infections.
Sickle cell anemia is an inherited condition. If both a child’s parents have it their children will have it too; if only one parent has it their children will have what’s called the sickle cell trait, which means they carry it and may pass it on to their children. Around 72,000 Americans have sickle cell anemia – it’s most common in those of African-American descent and Hispanic Americans. Prenatal tests such as chorionic villus sampling and amniocentisis can detect sickle cell anemia, and most states mandate newborn screening for the condition (if you think your baby may be at risk but the test isn’t routine in your state, you can request it).
Children born with sickle-cell anemia may not show any signs until they are around four to six months old. The typical symptoms to look out for are weakness and tiredness that cause them to sleep for longer periods than normal (remember, babies usually wake often for feeds). If your baby experiences a blockage he’s likely to exhibit signs of severe pain and cry inconsolably. As your child grows he could have anything from one to 15 of these crises a year. The pain can either be acute or chronic, with acute pain lasting for hours and sometimes days, and chronic pain lasting for days or months. Obviously both of these types of pain will be immensely draining and upsetting for your child so ask his pediatrician how best to ease the pain and help with the symptoms.
If sickle cells become stuck in your child’s lungs it can cause symptoms similar to pneumonia, such as difficulty breathing, chest pain and fever. This is an emergency so if it occurs, call 911 or take your child to the ER. It’s also possible for blood cells to get trapped in the spleen, causing it to become enlarged. In some children, the spleen needs to be removed, compromising the immune system and leaving them at higher risk of infections – monitor your child carefully whenever he is unwell and call your pediatrician straightaway if he has a fever, vomiting or coughing. Aplastic anemia is also a possibility – this occurs when an infection prevents your child’s body from manufacturing red blood cells, causing his red blood cell count to drop dangerously low. Symptoms include lethargy and fainting.
Rarely, older children can have a stroke if sickle cells block one of the blood vessels in the brain, so be observant for signs including severe headache, slurred speech, loss of vision and weakness on one side of the body. Other side effects are jaundice and eye problems.
Treatment
Sickle cell anemia is incurable but you can take steps to reduce your child’s pain and discomfort by giving him painkillers. If your child is having a crisis it’s important to get medical help immediately. Look out for your child complaining of severe chest pains, headache and a stiff neck; high fever, drowsiness; and swelling of the joints in your child’s hands and feet.
The treatment for sickle cell anemia ranges from daily penicillin to reduce your child’s risk of infection, to folic acid supplements to boost production of red blood cells and blood transfusions to treat anemia and prevent strokes. Warm compresses, massage and gentle pressure or rubbing on the painful areas can help.
Try to avoid situations that may trigger off a crisis, such as air travel (high and low altitudes can trigger attacks) and dehydration, also linked to crises. Try to alleviate stress in your child’s life and keep him away from anyone who’s sick, as childhood illnesses can cause a crisis. Although strenuous exercise can lead to a crisis, gentle exercise is very good for your child.
• American Sickle Cell Anemia Association www.ascaa.org
The information on this feature is intended for educational purposes only. If you have any concerns about your health, the health of your child or the health of someone you know, please consult with a doctor or other healthcare professional.