Sickle cell anemia - diagnosis and treatment (2023)


A blood test can check the form of hemoglobin that underlies sickle cell anemia. In the United States, this blood test is part of routine newborn screening. But older children and adults can also be tested.

In adults, a blood sample is taken from a vein in your arm. In young children and babies, the blood sample is usually taken from a finger or heel. The sample is then sent to a lab where it is tested for the sickle cell form of hemoglobin.

If you or your child has sickle cell disease, your doctor may suggest other tests to detect possible complications of the disease.

If you or your child carry the sickle cell gene, you will likely be referred to a genetic counselor.

Stroke risk assessment

A special ultrasound device can provide information about which children have an increased risk of stroke. This painless test, which uses sound waves to measure blood flow in the brain, can be done on children as young as 2 years old. Regular blood transfusions can reduce the risk of stroke.

Tests to detect sickle cell genes before birth

Sickle cell anemia can be diagnosed in an unborn baby by taking some of the fluid in the womb (amniotic fluid) surrounding the baby. If you or your partner have sickle cell disease or sickle cell anemia, ask your doctor about this screening.

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Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Treatments might include medications and blood transfusions. For some children and teenagers, a stem cell transplant might cure the disease.


  • Hydroxyharnstoff (Droxia, Hydrea, Siklos).Daily intake of hydroxyurea reduces the frequency of painful crises and could reduce the need for blood transfusions and hospitalizations. But it can increase the risk of infection. Do not take the drug if you are pregnant.
  • L-glutamine oral powder (Endari).TheFDAThis drug was recently approved for the treatment of sickle cell anemia. It helps in reducing the frequency of pain crises.
  • Crizanlizumab (Adakveo).This drug, which is given by injection, may help reduce the incidence of pain crises in adults and children over the age of 16. Side effects can include nausea, joint pain, back pain, and fever.
  • Voxelotor (Oxbryta).This drug is used to treat sickle cell disease in adults and children over 12 years of age. When taken by mouth, this drug can reduce the risk of anemia and improve blood flow throughout the body. Side effects can include headache, nausea, diarrhea, fatigue, rash, and fever.
  • Pain Relieving Drugs.Your doctor may prescribe narcotics to reduce pain during sickle cell seizures.

prevent infections

Children with sickle cell anemia can be given penicillin from about 2 months to at least 5 years of age. This helps prevent infections like pneumonia, which can be life-threatening for children with sickle cell anemia.

Adults with sickle cell anemia may need to take penicillin for their entire lives if they have had pneumonia or surgery to remove the spleen.

Childhood immunizations are important to prevent disease in all children. They're even more important for children with sickle cell disease because their infections can be serious.

Your child's doctor should ensure that your child receives all recommended childhood vaccinations, as well as vaccinations for pneumonia, meningitis, hepatitis B and an annual flu shot. Vaccines are also important for adults with sickle cell disease.

During the COVID-19 pandemic, people with sickle cell anemia should take extra precautions, such as B. Stay isolated at home as much as possible and get vaccinated.

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Surgical and other procedures

  • blood transfusions.These are used to treat and prevent complications such as stroke in people with sickle cell disease.

    In an RBC transfusion, red blood cells are taken from a blood donation and then given through a vein to a person with sickle cell disease. This increases the normal red blood cell count, which helps reduce symptoms and complications.

    Risks include an immune response to the donor blood, which can make it difficult to find future donors; Infection; and excess iron buildup in your body. Because excess iron can damage your heart, liver, and other organs, you may need treatment to lower iron levels if you have regular transfusions.

  • stem cell transplant.In this procedure, also known as a bone marrow transplant, the bone marrow affected by sickle cell anemia is replaced with healthy bone marrow from a donor. The procedure usually uses a matched donor, e.g. B. a sibling who does not have sickle cell anemia.

    Because of the risks associated with bone marrow transplantation, including death, the procedure is only recommended for people, usually children, who have significant symptoms and complications of sickle cell disease. A stem cell transplant is the only known cure for sickle cell anemia.

    Clinical trials are currently underway to treat adult stem cell transplants and gene therapies.

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More information

  • blood transfusion
  • bone marrow transplant

Request an appointment at the Mayo Clinic

lifestyle and home remedies

If you take the following steps to stay healthy, you can avoid complications of sickle cell anemia:

  • Take folic acid supplements daily and choose a healthy diet.The bone marrow needs folic acid and other vitamins to make new red blood cells. Ask your doctor about a folic acid supplement and other vitamins. Eat a variety of colorful fruits and vegetables and whole grains.
  • Drink plenty of water.Dehydration can increase the risk of sickle cell crisis. Drink water throughout the day and aim for about eight glasses a day. Increase the amount of water you drink when you exercise or spend time in a hot, dry climate.
  • Avoid extreme temperatures.Exposure to extreme heat or cold can increase the risk of sickle cell crisis.
  • Exercise regularly, but don't overdo it.Talk to your doctor about how much exercise is right for you.
  • Use non-prescription drugs with caution.Use pain relievers like ibuprofen (Advil, Motrin IB, Children's Motrin, others) or naproxen sodium (Aleve) sparingly, if at all, because of the potential effects on your kidneys. Consult your doctor before taking over-the-counter medications.
  • Do not smoke.Smoking increases the risk of pain crises.

coping and support

If you or someone in your family has sickle cell anemia, here are some things you can consider to help manage it:

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  • Find someone to talk to.Living with a chronic illness is stressful. Consider seeing a psychologist, counselor, or social worker to help you cope.
  • Join a support group.Ask your doctor about family support groups in your area. It can be helpful to talk to others who are facing similar challenges as you.
  • Find ways to deal with the pain.Work with your doctor to find ways to control your pain. Painkillers do not always eliminate all pain. Different techniques work for different people, but it might be worth trying heating pads, hot baths, massage, or physical therapy.
  • Learn more about sickle cell disease to make informed decisions about care.If you have a child with sickle cell disease, learn as much as you can about the disease. Ask questions during your child's appointments. Ask your healthcare team to recommend good sources of information.

Prepare for your appointment

Sickle cell anemia is usually diagnosed through genetic screening done when a baby is born. These test results will likely be shared with your GP or pediatrician. He or she will likely refer you to a doctor who specializes in blood disorders (hematologist) or a pediatric hematologist.

Here you will find information to help you prepare for your appointment.

What you can do

Make a list of:

  • your symptoms,including any that seem unrelated to the reason you scheduled the appointment and when they started
  • important personal information,including family history and whether someone has sickle cell disease or has the trait
  • Questions to askyour doctor

If possible, bring a family member or friend with you so that you can remember the information given to you.

If you have sickle cell disease, you should ask your doctor the following questions:

  • What is the most likely cause of my child's symptoms?
  • Are there any other possible causes?
  • What tests are required?
  • What treatments are available and which ones do you recommend?
  • What side effects are common with these treatments?
  • Are there any alternatives to the primary approach you propose?
  • What is my child's prognosis?
  • Are there any diet or activity restrictions?
  • Do you have brochures or other printed materials I can have? Which websites do you recommend?

Don't hesitate to ask further questions.

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What to expect from your doctor

Your doctor will likely ask you questions, including:

  • When did you notice your child's symptoms?
  • Were they continuous or occasional?
  • What, if anything, seems to improve symptoms?
  • What, if anything, seems to make it worse?

By Mayo Clinic staff


How is sickle cell anemia diagnosed and treated? ›

Sickle cell anemia is usually diagnosed through genetic screening done when a baby is born. Those test results will likely be given to your family doctor or pediatrician. He or she will likely refer you to a doctor who specializes in blood disorders (hematologist) or a pediatric hematologist.

What are 3 treatments for sickle cell anemia? ›

Healthcare providers typically treat sickle cell anemia with blood transfusions, antibiotics to treat infections and medications that reduce symptoms caused by sickle cell anemia complications. These medications may include hydroxyurea, voxlelotor, L-glutamine therapy and crizanlizumab.

What is the diagnosis of sickle cell anemia? ›

Blood test and genetic tests

Genetic testing can help determine which type of sickle cell disease you have or can help confirm a diagnosis if results from blood tests are not clear. Genetic testing can also tell whether you have one or two copies of the sickle hemoglobin gene.

What test is most useful in diagnosing sickle cell disease? ›

The best way to check for sickle cell trait or sickle cell disease is to look at the blood using a method called high-performance liquid chromatography (HPLC). This test identifies which type of hemoglobin is present. To confirm the results of HPLC, a genetic test may be done.

What is the first treatment for sickle cell anemia? ›

Research and Treatment

1960s - Blood transfusions first used to treat sickle cell. Blood transfusions are used to treat patients experiencing sickle cell disease complications.

What is the new treatment for sickle cell anemia? ›

“The new therapy, called LentiGlobin, is a gene-based therapy where doctors harvest the patient's own stem cells and add to them a corrected gene that makes non-sickle hemoglobin,” Dr. Rifkin-Zenenberg, the principal investigator of the gene therapy study.

Do you treat sickle cell anemia with iron? ›

People with SCD may begin iron chelation therapy after 1 to 2 years of transfusions or when iron levels hit a certain level. Doctors may monitor your iron levels by measuring the amount of ferritin (a protein that stores iron) in the blood using a blood draw.

What is the main cause of sickle cell anemia? ›

People who have sickle cell disease inherit two faulty hemoglobin genes, called hemoglobin S — one from each parent. A person has sickle cell trait when the hemoglobin S gene is inherited from only one parent and a normal hemoglobin gene — hemoglobin A — is inherited from the other.

What is a nursing diagnosis for sickle cell? ›

Based on the assessment data, major nursing diagnosis for the patient include: Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels. Risk for infection. Risk for powerlessness related to illness-induced helplessness. Deficient knowledge regarding sickle cell crisis prevention.

Can you be diagnosed with sickle cell anemia later in life? ›

You can only get it if both of your parents carry a certain gene trait and pass it on to you. If you're not born with it, you can't get it later in life. Sickle cell disease affects red blood cells. It makes them hard and “sickle” shaped instead of soft and round.

What age is sickle cell anemia diagnosed? ›

People with sickle cell disease (SCD) start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications of SCD are different for each person and can range from mild to severe.

What is the best medication for sickle cell? ›

Medicine for sickle cell pain

Hydroxycarbamide can lower the amount of other blood cells, such as white blood cells and platelets (clotting cells), so you'll usually have regular blood tests to monitor your health. If you have regular episodes of pain, you may also be able to have a medicine called crizanlizumab.

What drugs should sickle cell patients avoid? ›

Morphine is considered the drug of choice for the treatment of acute sickle cell pain (Table 2), whereas meperidine (Demerol, Sanofi-Synthelabo) should be avoided because of the increased risk of seizures in patients with renal dysfunction, which can occur in patients with SCD.

How does sickle cell disease affect daily life? ›

Fatigue, bone aches, and headaches were the 3 most commonly reported symptoms (41%, 38%, and 25%, respectively). Patients who experienced these symptoms were more likely to respond that the disease had a high impact on their lives or wellbeing. Fatigue was the most common symptom in patients before the survey (65%).

How much is treatment for sickle cell anemia? ›

“Our findings suggest that the lifetime out-of-pocket cost of medical care is about $44,000 for people with SCD,” explained study author Kate Johnson, PhD, of the Comparative Health Outcomes, Policy & Economics (CHOICE) Institute, Department of Pharmacy, University of Washington.

Did the FDA approve a new treatment for sickle cell disease? ›

FDA Approves Oxbryta™ (Voxelotor), the First Medicine Specifically Targeting the Root Cause of Sickle Cell Disease.

What is the difference between iron deficiency anemia and sickle cell anemia? ›

Iron deficiency is mainly prevented by maintaining a balance between iron intake, iron absorption and excretion. Individuals with sickle cell disease have an adequate iron source, potentially from increased red cell turnover and from repeated blood transfusions.

What are the 4 major symptoms of sickle cell anemia? ›

Symptoms and complications may include:
  • Anemia. Because sickled cells are short-lived or destroyed, there are less red blood cells available in the body. ...
  • Pain crisis, or sickle crisis. ...
  • Acute chest syndrome. ...
  • Splenic sequestration (pooling). ...
  • Stroke. ...
  • Jaundice, or yellowing of the skin, eyes, and mouth. ...
  • Priapism.

What problems do sickle cells cause? ›

The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications (health problems) such as infection, acute chest syndrome and stroke.

How long does sickle cell patients live? ›

Low Life Expectancy with Sickle Cell Disease

Thus, their life expectancy is reduced compared with that of the general population. A recent study estimated the life expectancy of adults with SCD to be 54 years, which is approximately 20 years shorter than that of normal adults without SCD.

What is the first nursing intervention for sickle cell crisis? ›

1. Keep the patient oxygenated. During a vaso-occlusive crisis, sickle cells clump together and are not able to carry oxygenated blood to the rest of the body. The administration of oxygen will help increase oxygen and tissue perfusion.

What is the prevention of sickle cell anemia? ›

Sickle cell anemia is an inherited blood disorder. Because it's a genetic condition someone is born with, there is no way to prevent the disease, so scientists are constantly investigating ways that the disease can be stopped before it passes to the next generation.

How can you prevent sickle cell crisis? ›

Try to:
  1. drink plenty of fluids, particularly during hot weather – dehydration increases the risk of a sickle cell crisis.
  2. avoid extreme temperatures – you should dress appropriately for the weather and avoid sudden temperature changes, such as swimming in cold water.

Is sickle cell anemia a serious disorder? ›

People with sickle cell disease produce unusually shaped red blood cells that can cause problems because they do not live as long as healthy blood cells and can block blood vessels. Sickle cell disease is a serious and lifelong health condition, although treatment can help manage many of the symptoms.

Can sickle cell be misdiagnosed? ›

Screening programs that diagnose sickle cell aneimia without first demonstrating sickle hemoglobin in both parents will consistently misdiagnose several more benign hemoglobin states as sickle cell anemia.

Do sickle cells return to normal? ›

When you have sickle cell disease, your body's red blood cells become crescent- or sickle-shaped because they do not receive enough oxygen or become dehydrated. Once oxygen is restored, your cells return to their normal round shape, but over time they become fragile and stay in the sickle shape.

How long does a person with sickle cell anemia? ›

Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells typically die in 10 to 20 days, leaving a shortage of red blood cells (anemia). Without enough red blood cells, the body can't get enough oxygen and this causes fatigue. Episodes of pain.

Who is at high risk for sickle cell anemia? ›

Risk Factors

Sickle cell disease is more common in certain ethnic groups, including: People of African descent, including African-Americans (among whom 1 in 12 carries a sickle cell gene) Hispanic-Americans from Central and South America. People of Middle Eastern, Asian, Indian, and Mediterranean descent.

Can stress cause anemia? ›

Sustained stress is another cause of anaemia. Excessive stress hinders the manufacture of hydrochloric acid in your body, which is very important for the integration of iron and proteins. The deficiency of iron is equal to lack of haemoglobin and thus, anaemia.

Why can't sickle cell patients take iron? ›

Too much iron is extremely dangerous and causes damage to blood vessels, red blood cells, liver, hormone producing glands and heart. It is very difficult to know what damage due to iron overload in sickle cell patients because the sickle cell disease itself causes organ damage to the same organs affected by iron.

What is the most common problem experienced by patients with sickle cell disease? ›

Sickle cell disease (SCD) is a multi-organ blood and blood vessel disease, with pain (both acute and chronic) being its most prominent aspect. Individuals with SCD experience recurrent but unpredictable episodes of debilitating acute pain that, over time, evolve into daily chronic pain.

How can I help someone with sickle cell? ›

Hang out, listen to music, talk about what's going on at school, or do homework together. If you can't be there in person, find another way to talk. Encourage healthy habits. Everyone should eat well and stay hydrated, but it's especially important for someone with sickle cell disease.

What are the emotional effects of sickle cell anemia? ›

Mood is an important consequence of SCD. People with SCD commonly report low self-esteem and feelings of hopelessness as a result of frequent pain, hospitalisations, and loss of schooling (in children) and employment (in adults). These accounts could indicate depressive symptoms.

Can sickle cell anemia be treated or cured? ›

Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they're not done very often because of the significant risks involved. Stem cells are special cells produced by bone marrow, a spongy tissue found in the centre of some bones. They can turn into different types of blood cells.

How and when is sickle cell diagnosed? ›

Sickle cell disease is usually detected during pregnancy or soon after birth. Blood tests can also be carried out at any time to check for the condition or to see if you're a sickle cell carrier and are at risk of having a child with the condition.

What happens if you test positive for sickle cell? ›

It means your hemoglobin is normal. A positive test result may mean you have sickle cell trait or SCD. If the test is positive, your doctor will probably order a second test called hemoglobin electrophoresis. This will help determine which condition you have.

Can you live a normal life with sickle cell anemia? ›

People with sickle cell disease can live full lives and enjoy most of the activities that other people do.

Does sickle cell anemia go away? ›

The blocked blood flow through the body can lead to serious problems, including stroke, eye problems, infections, and episodes of pain called pain crises. Sickle cell disease is a lifelong illness.

What is the main cause of sickle cell disease? ›

People who have sickle cell disease inherit two faulty hemoglobin genes, called hemoglobin S — one from each parent. A person has sickle cell trait when the hemoglobin S gene is inherited from only one parent and a normal hemoglobin gene — hemoglobin A — is inherited from the other.

What should sickle cell patients avoid? ›

avoid very strenuous exercise – people with sickle cell disease should be active, but intense activities that cause you to become seriously out of breath are best avoided. avoid alcohol and smoking – alcohol can cause you to become dehydrated and smoking can trigger a serious lung condition called acute chest syndrome.

Does sickle cell count as a disability? ›

Sickle cell anemia requires ongoing treatment, medicines, and hospital stays. If your sickle cell anemia is so severe that it prevents you from working, you may be struggling financially. Because sickle cell anemia is a type of physical disability, you may qualify for Social Security disability (SSD) benefits.


1. Treatment for Sickle Cell Anemia
(Nucleus Medical Media)
2. Sickle Cell Anaemia, Causes, Signs and Symptoms, Diagnosis and Treatment.
(Medical Centric)
3. Sickle Cell Disease - What It Is, Symptoms, Risks, Diagnosis & More
4. Sickle Cell Anemia Nursing | Symptoms, Pathophysiology, Sickle Cell Crisis & Trait
5. SICKLE CELL DISEASE | Clinical Features | Diagnosis | Treatment | Harrison
(Medi - Lectures)
6. How Is Sickle Cell Disease (SCD) Diagnosed? What You Need To Know


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