Institute of Family Medicine Plus Use Only Fetal Stem Cells
Sickle Cell Disease
Also called: Hemoglobin SS affliction, Sickle cell anemia
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Summary
What is sickle cell affliction (SCD)?
Sickle jail cell disease (SCD) is a group of inherited red blood jail cell disorders. If you take SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in ruby blood cells that carries oxygen throughout the body. With SCD, the hemoglobin forms into stiff rods within the red claret cells. This changes the shape of the ruby-red blood cells. The cells are supposed to be disc-shaped, but this changes them into a crescent, or sickle, shape.
The sickle-shaped cells are not flexible and cannot change shape easily. Many of them outburst apart as they move through your blood vessels. The sickle cells usually only concluding x to 20 days, instead of the normal 90 to 120 days. Your torso may have trouble making enough new cells to supervene upon the ones that you lost. Because of this, you may not accept enough red blood cells. This is a condition called anemia, and it can brand you feel tired.
The sickle-shaped cells tin also stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can't reach nearby tissues. The lack of oxygen can cause attacks of sudden, astringent pain, called pain crises. These attacks can occur without warning. If you get i, yous might need to get to the hospital for treatment.
What causes sickle cell disease (SCD)?
The cause of SCD is a defective gene, called a sickle prison cell cistron. People with the affliction are born with two sickle cell genes, one from each parent.
If yous are built-in with ane sickle cell cistron, it'south called sickle cell trait. People with sickle cell trait are generally salubrious, simply they tin can pass the defective factor on to their children.
Who is at take a chance for sickle cell disease (SCD)?
In the Us, most of the people with SCD are African Americans:
- About 1 in 13 African American babies is born with sickle cell trait
- Almost ane in every 365 black children is built-in with sickle jail cell disease
SCD also affects some people who come from Hispanic, southern European, Centre Eastern, or Asian Indian backgrounds.
What are the symptoms of sickle prison cell disease (SCD)?
People with SCD start to have signs of the disease during the first twelvemonth of life, usually around 5 months of age. Early symptoms of SCD may include:
- Painful swelling of the hands and feet
- Fatigue or fussiness from anemia
- A yellowish color of the skin (jaundice) or the whites of the optics (icterus)
The effects of SCD vary from person to person and tin modify over time. Most of the signs and symptoms of SCD are related to complications of the disease. They may include astringent pain, anemia, organ damage, and infections.
How is sickle prison cell disease (SCD) diagnosed?
A blood test can show if you lot have SCD or sickle prison cell trait. All states now examination newborns as office of their screening programs, so treatment tin can begin early.
People who are thinking about having children can have the test to find out how probable it is that their children will have SCD.
Doctors tin too diagnose SCD before a babe is born. That test uses a sample of amniotic fluid (the liquid in the sac surrounding the babe) or tissue taken from the placenta (the organ that brings oxygen and nutrients to the baby).
What are the treatments for sickle cell disease (SCD)?
The merely cure for SCD is os marrow or stem cell transplantation. Because these transplants are risky and can have serious side effects, they are normally only used in children with astringent SCD. For the transplant to work, the os marrow must be a shut match. Usually, the best donor is a blood brother or sis.
There are treatments that tin assist relieve symptoms, lessen complications, and prolong life:
- Antibiotics to try to prevent infections in younger children
- Pain relievers for astute or chronic pain
- Hydroxyurea, a medicine that has been shown to reduce or prevent several SCD complications. It increases the amount of fetal hemoglobin in the claret. This medicine is not right for anybody; talk to your wellness care provider virtually whether you should take information technology. This medicine is not safe during pregnancy.
- Childhood vaccinations to prevent infections
- Blood transfusions for severe anemia. If you have had some serious complications, such every bit a stroke, y'all may have transfusions to foreclose more complications.
In that location are other treatments for specific complications.
To stay as healthy as possible, make sure that you get regular medical care, live a salubrious lifestyle, and avoid situations that may set off a hurting crisis.
NIH: National Heart, Lung, and Blood Institute
- Living Well with Sickle Cell Disease (Centers for Illness Control and Prevention) Also in Castilian
- Sickle Cell Crisis (Pain Crunch) (Nemours Foundation) Also in Spanish
- A1C Exam and Race/Ethnicity
(National Institute of Diabetes and Digestive and Kidney Diseases) - Five Tips to Assistance Foreclose Infections (Centers for Disease Control and Prevention) Likewise in Spanish
- Hemoglobin C, S-C, and E Diseases (Merck & Co., Inc.) Too in Castilian
- Sickle Cell Affliction and Pulmonary Hypertension (Pulmonary Hypertension Association) - PDF
- Sickle Jail cell Disease Quiz (Centers for Illness Control and Prevention) Besides in Castilian
- Hemoglobin electrophoresis (Medical Encyclopedia) As well in Spanish
- Sickle cell anemia (Medical Encyclopedia) Also in Spanish
- Sickle cell test (Medical Encyclopedia) Too in Spanish
Source: https://medlineplus.gov/sicklecelldisease.html
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