Sickle Cell: Harmony and Me 

My daughter Harmony was diagnosed with sickle cell disease shortly after birth, which was detected through the heel prick test.  At 12 weeks old she was started on a daily dose penicillin V, given twice a day and used to give extra protection against infections. People with sickle cell are more likely to contract pneumococcal infections such as pneumonia and meningitis.

Sickle cell disease is the name for a group of inherited conditions that affect the red blood cells, the most serious type is called sickle cell anaemia. Sickle cell disease mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. In the UK it is particularly common in people with an African or Caribbean family background.

People with sickle cell disease produce sickle shaped red blood cells that can cause problems, because they do not live as long as healthy round blood cells and they can become stuck in blood vessels which can cause painful crisis’s.

Harmony’s first symptoms of the illness started when she as just under a year old when she developed pain and swelling in her fingers. This led to her first hospital admission where she was given IV antibiotics and fluids, pain relief and. Since then she has had different hospital admissions, mainly for pain in her hands, feet and legs. The pain can be of different severity and the length of time that the pain can last, varies between a few hours and sometimes weeks. She has also required three blood transfusions, because her haemoglobin level dropped causing severe anaemia. When this happens, it can sometimes lead to splenic sequestration, this is where all of the blood fills within the spleen and can be fatal, this unfortunately happened to my daughter twice but was detected early so an emergency transfusion was given.

Sickle cell disease is a serious and lifelong condition, although long term treatment can help manage many of the problems associated with it. Sickle cell disease is very variable but most children with it will lead happy and normal lives, however it can still be a serious condition that can have a significant impact on a person's life. It can lead to problems such as strokes, serious infections and lung problems, which can occasionally be fatal.  If both parents have sickle cell trait they are carriers of sickle cell disease, there is a 25% chance of each child they have being born with sickle cell disease. The child's parents often won't have the condition themselves because they are only carriers of the sickle cell trait.

Sickle cell disease is often detected during pregnancy or soon after birth. Screening for Sickle cell is offered to all pregnant women in England to check if there is a risk of a child being born with the condition, and all babies are offered screening as part of the heel prick test. Blood tests can also be carried out at any age, to check for the condition or to see if a person is the carrier of the faulty gene that causes it.

Sickle cell disease is extremely unpredictable and there can be a number of triggers as crisis’s can vary. Dehydration is a major trigger for sickle cell so it is essential to keep hydrated. Other triggers can include:

  • Cold weather

  • Changes in temperature e.g. going from a warm house to cold weather without been properly wrapped up

  • Swimming in cold water

  • Passive smoking

  • Illness/infections

  • Stress

Painful crisis’s is the most common complication in sickle cell:

  • Painful episodes can sometimes be prevented by drinking plenty of fluids and staying warm

  • Pain can often be treated with ordinary painkillers such as paracetamol or ibuprofen, although sometimes treatment with stronger painkillers in hospital may be necessary

  • The risk of infections can be reduced by taking daily antibiotics and ensuring you are fully vaccinated

  • A blood transfusion may be needed if severe anaemia develops

  • A medicine called hydroxycarbamide (hydroxyurea) can reduce the symptoms if they are interfering with your or your child's life

  • Having regular blood transfusions (usually every 3-4 weeks) may be helpful if problems continue or get worse, or there are signs of damage caused by the condition

Stem cell or bone marrow transplants can potentially cure sickle cell disease but they are not carried out very often because of the significant risks involved.

Although sickle cell affects many people throughout the country there is still not enough awareness surrounding it and is one of the things that I have found extremely difficult to understand.

Recently the B Positive choir have started raising awareness about the illness as well as trying to encourage people to donate blood. There is currently a massive shortage of black donors.

For more information about sickle cell, check out the Sickle Cell Society’s website