A Parent handbook for Sickle Cell Disease in Nigeria Part 1: by Brown, Falusi, Jaudes

January 3, 2019by HLF0

What is Sickle Cell Disease?
Sickle cell disease is a disease that affects a special protein inside our red blood cells called haemoglobin. Red blood cells have an important job. They pick up oxygen from the lungs and take it to every part of the body. It is the haemoglobin in these red blood cells that carry the oxygen to different parts of the body. A person with sickle cell disease makes a different kind of haemoglobin called haemoglobin S. This haemoglobin S causes the red blood cells to change their shape. Instead of being smooth and round, the cells become hard and sticky. Their shape looks like a banana or like a sickle, a hand tool used to cut wheat or tall grass. It is this sickle shape of the red blood cells that gives “sickle cell” disease its name. The hard, sticky sickle red blood cells have trouble moving through small blood vessels. Sometimes they clog up these blood vessels and cause other changes so that blood can’t bring oxygen to the tissues. This can cause pain or damage to these areas.

Types of Sickle Cell Disease
There are many different types of sickle cell disease. The most common types are: sickle cell anaemia (SS disease) and sickle C disease (SC disease). sickle beta (ß) thalassaemia disease (S ß thal disease) and sickle cell alpha (a) thalassaemia disease (S a thal disease) are other types but are less common. There are many rare combinations of sickle haemoglobin with other types of haemoglobin. Some are as serious as sickle cell anaemia. Some types of sickle cell disease cause fewer problems than others.

What problems are caused by Sickle Cell Disease?
Sickle cell disease can cause many kinds of problems. Some of the most common problems are:
– Infections
– Pain
– Anaemia (low blood)
– Damage to the body organs
Not everyone who has sickle cell disease will have all of these problems. In fact, many people with sickle cell disease feel well most of the time. However, most people with sickle cell disease will have to deal with these problems during their

One of the most serious problems that people with sickle cell disease have is infections. Infections, like pneumonia, pose a special problem for infants and small children who can get very sick or even die if they don’t get prompt treatment. These infections are caused by problems with the spleen, a large organ in the body that removes damaged red cells and helps fight infections. The sticky sickle cells will clog the spleen so it can’t do its job. This leaves the body open to infections. Thankfully, we can prevent many of the sickle cell infections by giving young children penicillin every day until they are at least five years old, or as recommended by the National Institute of Health (NIH) Consensus Development Conference. There are also many ways to treat infections, especially if they are found quickly. Later sections of this book describe ways to prevent and treat these deadly infections.

The hard, sticky sickle red blood cells can sometimes cause pain. The shape of these cells makes it hard for them to get through tiny blood vessels. The sickle cells can cause changes in the blood that block the blood vessel. This cuts off the blood supply to nearby tissues so that no cells can get through to bring oxygen. Without oxygen, the area starts to hurt. This is the source of the pain that comes from sickle cell disease. Some sickle cell pain can be very strong and needs to be treated in the hospital. Most pain is milder and can be handled at home. There are many ways to treat the pain to make your child feel better. This pain is sometimes called a “crisis”. In fact, most sickle cell pain is not a crisis. Later sections of this book will describe ways to help you deal with this pain.

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Copyright by HLFONLINE. All rights reserved.