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What Are The Symptoms Of Sickle Cell Disease?

Sickle cell disease changes the shape of the oxygen-carrying molecules (called hemoglobin) found inside red blood cells. Red blood cells  are usually round and flexible. This allows them to glide smoothly through your bloodstream. But when you have sickle cell disease, the abnormal hemoglobin creates stiff, sickle-shaped red blood cells.

If you have this disease, these abnormally shaped red blood cells can cause a number of health problems. For example:

The cells get stuck in the smaller arteries and block blood flow to organs and tissues.

The cells die sooner. Sickle cells live about 10 to 20 days. Healthy red blood cells live from 90 to 120 days. Red blood cells may burst open (hemolyze). Even though your body constantly makes new red blood cells to replace the old ones, sickle cell disease may prevent your body from keeping up with the demand.

These events trigger the symptoms of sickle cell disease.

Usually, they first appear when a child is about five to six months old. Early signs in babies are:

Extreme fussiness

Painful swelling of fingers and toes which is the definition of a condition called "dactylitis"

Yellow skin and whites of the eyes (also called jaundice or icterus). This is caused by the breakdown of hemogloblin released during the constant turnover of red blood cells.

With sickle cell disease, you don't have enough healthy red blood cells. This is a condition called anemia. Symptoms can include:

Sickle cells can cause problems anywhere in your body by blocking blood flow. If you block the blood vessels to the eyes, you lose vision, to the skin - you get ulcers, to the bones - you get necrosis (bone death) and eventually osteoporosis. Recurrent infarctions of the spleen, which has very high blood flow and whose job it is to remove damaged red blood cells, results in a non-working spleen. This leads to an increased risk of infections. 

Pain is an unpredictable symptom of sickle cell disease. The deformed blood cells can get stuck in and block your blood vessels. When this happens, oxygen-rich blood cannot reach key organs and tissues. The result is a sudden pain attack, called a pain crisis.

The pain may feel sharp, stabbing, intense, or throbbing. Some people with sickle cell disease say it's worse than childbirth or the pain after surgery.

You may have pain anywhere in your body and in more than one place. The most common areas are:

Arms

Belly

Chest

Legs

Lower back

A pain crisis can last anywhere from hours to weeks. If it's severe, you'll need to seek emergency care at a hospital. The number of pain attacks varies from person to person. Some people have them often. Others have only a few every now and then. Most kids are pain-free between attacks. But many teens and adults have long-term pain.

Everyone is different. Some types of the disorder, like sickle cell anemia (also called hemoglobin SS), cause more severe symptoms than other types. Some people have only mild symptoms while others may have dramatic flares that require medical care.  And your experience may change. You'll want to work with your (or your child's) doctor to best understand the disease, what to watch out for, and how best to take care of the condition.

Sickle Cell Disease Directory

Sickle cell disease is an inherited red blood cell disorder. People with sickle cell disease can have abnormally shaped red blood cells. The misshapen cells can get stuck in blood vessels, blocking blood flow and causing problems. Follow the links below to find WebMD's comprehensive coverage about why sickle cell disease occurs, what sickle-shaped cells look like, complications of sickle cell disease, and much more.

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Sickle Cell Disease: A Global Call To Action

Reducing the burden of Sickle Cell Disease (SCD) requires substantial financial and political commitment to improving data-collection, diagnosis, treatment and training - doing so will positively impact the lives of millions of patients and families worldwide - says a new Commission published in The Lancet Haematology journal.

The Commission publishes shortly after another study in The Lancet Haematology journal provided the first estimates of the full global mortality burden of SCD, revealing a strikingly high contribution of SCD to all-cause mortality that is not apparent when each death is assigned to only a single cause . The study suggests there were 376,000 global SCD-related deaths in 2021, compared to 34,400 cause-specific deaths.

With over half a million babies born with SCD in 2021, the Commission highlights how newborn screening for SCD can lead to babies with the disease receiving life-changing treatment before symptoms develop and calls for all babies worldwide to be tested for SCD by 2025 to prevent long-term complications of the disease.

The Commission also shines a light on the unequitable treatment of SCD. Penicillin, methods to protect against malaria, the drug hydroxyurea and blood transfusions all have good evidence to show they reduce deaths and long-term consequences of SCD – however access and use of these treatments and reduction methods is poor, particularly so in low and middle-income countries where most people with SCD live. There is a shortage of healthcare and scientific professionals with expertise in SCD, as well as a lack of trials aimed at developing novel treatments. This problem is particularly severe in most of sub-Saharan Africa and India, and the Commission argues there is an urgent need for trials specifically designed for people in these countries.

The Commission says that in the context of increasing global inequalities, partly driven by racism, previous calls for action on SCD have been largely ineffective. There is an urgent need for all people with SCD to be given access to minimum specific health care no matter where they live and for funding programmes for research in all aspects of SCD to be prioritised and increased.

Dr Frédéric Piel, Imperial College London & chair of the Commission, says: "Whereas the majority of major causes of death are decreasing, the number of deaths due to sickle cell disease is increasing globally. The costs required to reduce the risk of sickle cell disease is beyond the reach of most individuals in sub-Saharan Africa and India where the disease is most prevalent - it needs to be directly funded by governments. With adequate engagement of governments, the changes identified in our Commission are achievable and will improve the lives of people with sickle cell disease throughout the world."

In a patient viewpoint published alongside the Commission, Lwimba Kasongo, a sickle cell disease patient and advocate from Zambia, says: "I grew up in a supportive family, but nothing could have prepared me for the stigma and mockery I experienced at school and as an adult. I lived in fear because I was told that I would not live long, I made no plans, and had no dreams or hopes for the future. In junior high school, I told my parents that I would not go to college as I thought I was going to die at age 16 years. I spent my teenage years isolated and with very little social life, as I was frequently in and out of hospital. I felt the pain of being different, added to the constant physical pain crises and the dependency on medicine and blood transfusions; I wondered why I was born and what the meaning of my life was."

Source:

Journal reference:

GBD 2021 Sickle Cell Disease Collaborators. (2023) Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the Global Burden of Disease Study 2021. The Lancet Haematology. Doi.Org/10.1016/S2352-3026(23)00118-7.

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