What is Sickle cell disease?

Sickle cell disease (SCD) is a hereditary blood disorder that is caused by an abnormality in the hemoglobin in red blood cells. Hemoglobin is a protein that is found in red blood cells, and it plays an important role in carrying oxygen to all parts of the body.


Hemoglobin is the substance that creates the red color of red blood cells. Red blood cells contain a large number of hemoglobin molecules. Hemoglobin can transport oxygen or carbon dioxide through your body in the bloodstream. Oxygen is a very important fuel for the cells in your body. You breathe in the oxygen through your lungs, where it crosses into the bloodstream. In the blood, the oxygen binds to the hemoglobin in the red blood cells. The red bloods cells are transported across the body, delivering oxygen to cells all over the body.
When you have sickle cell disease, your hemoglobin is atypical. This abnormal type of hemoglobin is called hemoglobin S (where the S stands for Sickle cell). The problem is that hemoglobin S (HbS) potentially binds to other HbS within the red blood cell, causing the red blood cell to become abnormally shaped, namely the shape of a sickle. These sickle-shaped red blood cells are less flexible than normal red blood cells and cause problems.

Youtube. What is Sickle cell disease?

The possible symptoms

How many of the symptoms you will experience differs from one person to the next. This is because the symptoms depend on various factors, such as the type of SCD, the severity of your anemia or the breakdown of red blood cells, how often you suffer sickle cell crises or infections, and how severe these are. Read more

Youtube. The possible symptoms

Frequently asked question: What could be signs of sickle cell disease?

Symptoms that are consistent with sickle cell disease include anemia and regular painful limbs, abdominal pain, or pain in other body parts. Especially when the country of origin or ancestry makes sickle cell disease likely or when a family member has sickle cell disease, sickle cell disease should be considered as a diagnosis.

Frequently asked question: What is a sickle cell crisis?

A sickle cell crisis is an episode where small blood vessels get clogged up, potentially leading to a lack of oxygen in body tissue, which is referred to as infarction. Especially during infections, but also in case of fever, cold, dehydration, pain, high altitude, oxygen deficiency, or stress, sickle cells can get stuck in the small blood vessels and block blood flow. The sickle cells then basically stick to the wall of small and larger blood vessels. Although these kinds of sickle cell crises can occur spontaneously, they are generally triggered by the circumstances listed above.


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Frequently asked question: When do the first symptoms of sickle cell disease appear?

Since newborns still mainly have fetal hemoglobin (HbF), they will not have any sickle cell disease symptoms when the neonatal screening test is conducted. Newborns do, however, already produce small quantities of HbS. The neonatal screening test will reveal HbS production and this will lead to the diagnosis of sickle cell disease. Over the first months of a child’s life, HbS production will increase, topping out between the 4th and the 6th month. From that moment onwards, sickle cell disease symptoms may start to occur.

Interview: Two sisters with sickle cell disease: Hedya

Queena (22) and Hedya (20) are sisters and they both have sickle cell disease. Both suffer from the most serious form, the SS-form. Queena is studying business economics at Utrecht University of Applied Sciences and Hedya is studying applied psychology at Leiden University of Applied Sciences. They were born in Curaçao. Read more

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