What Is Sickle Cell Disease
Sickle cell disease is a group of conditions in which red blood cells are not shaped as they should be. Red blood cells normally look like round discs. But in sickle cell disease, they’re shaped like sickles, or crescent moons, instead.
The sickle shaped cells cause problems because:
- They are stiff and sticky and block small blood vessels when they get stuck together. This stops blood from moving as it should, which can lead to pain and organ damage.
- They break down faster than normal red blood cells. That leads to too few red blood cells, a condition called anemia.
The two most common symptoms of sickle cell disease are pain and anemia.
The pain caused by sickle cell disease is called a pain crisis or vaso-occlusive crisis. In a pain crisis:
- Pain may happen in any part of the body.
- Cold, stress, illness, or dehydration can bring on pain but often there is not an obvious trigger.
- The pain may last a few hours, a few days, or sometimes longer.
Sometimes pain can be managed at home. But someone with severe pain might need treatment in a hospital.
Signs of anemia include:
- paleness, often seen in the skin, lips, or nailbeds
- trouble paying attention
- a fast heartbeat
People with sickle cell anemia may have jaundice . This happens because the sickle-shaped red blood cells break down faster than normal cells.
Home Remedies For Sickle Cell Pain
There is no cure for sickle cell pain, but there are treatments that can help manage the pain and other symptoms. Some home remedies that may help include: drinking plenty of fluids, rest, and mild exercise. Over-the-counter pain medications, such as ibuprofen or acetaminophen, may also help. If the pain is severe, your doctor may prescribe stronger pain medications or recommend other treatments, such as hydroxyurea or blood transfusions.
Red blood cells can become crescent-shaped or sickle-shaped in a person with sickle cell disease. Because of the clumping of these cells, pain is caused by a restriction of blood flow. The pain in your back, legs, arms, chest, or stomach may be throbbing, dull, sharp, or stabbing, as well as sharp pain in your leg, arms, chest, or stomach. If you are experiencing severe pain, it may be necessary for your doctor to prescribe strong opioid pain relief such as acetaminophen or ibuprofen. IV fluids, which are delivered via a vein, may be administered as well. When red blood cells in sickled red blood cells block small blood vessels that carry blood to the bones, a sickle cell pain crisis occurs. When you have severe sickle cell disease, you may experience a variety of symptoms. Because the blood cells that form hemoglobin are scurvy, blood vessels restrict blood flow when they clump together or form clumps inside them. When you are dehydrated, stressed, or infected, you may have a pain crisis.
How Are They Prevented And Treated
Preventive antibiotics, especially penicillin, have decreased the rate of pneumococcal disease in children with SCD by 85 percent. Penicillin is safe and beneficial for children up to 5 years old. However, there are some concerns about long-term penicillin treatment.1,12
Universal use of pneumococcal and other standard vaccines has also reduced mortality from infectious diseases. The first pneumococcal vaccine reduced the rate of pneumococcal disease by 93 percent.
Infections may be prevented in people with SCD by:3,8
- Washing hands often
- Making sure food is prepared safely
- Avoiding contact with people who have fevers or colds
- Avoiding exposure to tobacco smoke
- Taking penicillin daily until 5 years old, or as prescribed by your doctor
- Regularly getting the flu and pneumococcal vaccines, and any others recommended by your doctor
Infections are usually treated with antibiotics. Sometimes, blood transfusions will be used, especially to treat an aplastic crisis.3
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How Do Healthcare Providers Treat 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.
Hydroxyurea is an anticancer drug now used to treat sickle cell anemia. In 2017, the U.S. Food and Drug Administration approved hydroxyurea as sickle cell anemia treatment for children age 2 and older, as well as adults. Studies show hydroxyurea:
- Cuts the number of VOC/acute pain crises by 50%.
- Reduces the need for blood transfusions.
- Improves anemia symptoms.
- In children, hydroxyurea eases dactylitis, which causes painful swelling.
This medication prevents red blood cells with abnormal hemoglobin from becoming sickled cells. Voxelotor may prevent some red blood cells from being destroyed faster than your bone marrow can replace them. In 2019, the FDA approved voxelotor for sickle cell disease treatment. In late 2021, the FDA approved voxelotor as treatment for children age 4 and older.
L-glutamine helps reduce some of the complications linked to sickle cell anemia. Sickled cells develop over time. L-glutamine helps protect sickled cells from becoming more misshapen. In 2017, the FDA approved Endari for treatment of children age 5 and older and adults.
Take Home Messages From This Study
- Children with SCD should be immunized according to the recommended immunization schedule pdf icon for children with conditions that make their bodies less capable of fighting off infection.
- Children with SCD should receive a daily dose of penicillin, an antibiotic medicine, to help prevent infections until the child is at least 5 years of age.
- More research on better prevention strategies is needed to reduce the higher risk of IPD among children with SCD compared to otherwise healthy children.
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Sickle Cell Patients: Tips For Caregivers
Sickle cell patients require special care due to their condition. They must be monitored closely and treated carefully to avoid complications. There are several things that caregivers can do to help sickle cell patients. First, they should keep the patient hydrated. This can be done by giving the patient plenty of fluids to drink and by providing humidified air if the patient is having difficulty breathing. Second, they should provide pain relief. This can be done with over-the-counter medications, as well as prescription painkillers. Third, they should avoid stress and anxiety. This can be done by providing a calm and supportive environment. Finally, they should monitor the patients progress and contact a doctor if there are any changes in the patients condition.
People who have sickle cell disease should exercise, but extreme activities that make them seriously ill should be avoided. Avoid alcohol and cigarettes- drinking alcohol can cause dehydration, and smoking can trigger a serious lung condition known as acute chest syndrome.
This approach to supporting circulation differs from that oflizumab. This therapy, which differs from hemoglobin therapy in that it aims to reduce blood vessel wall stickiness rather than improving hemoglobin in red blood cells, prevents blood vessel clotting and thus altering blood flow in sickled patients.
In 2011, Chicagoan Iesha Thomas became the Midwests first person to receive a stem cell transplant to treat sickle cell disease.
The Effects Of Sickle Cell Anemia
It is a serious blood disorder caused by a gene inherited from parents. In addition to strokes, serious infections, and lung issues that can be fatal, it can have a variety of serious effects on a persons health. In addition to people of African descent, it is more common among people of other races and ethnicities.
Complications Of Sickle Cell Disease
People with sickle cell disease 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. People with SCD can experience different complications, but some of the common ones are listed below.
Transcranial Doppler Hydroxyurea Use Low For Sickle Cell Anemia
THURSDAY, Sept. 22, 2022 — For children with sickle cell anemia , transcranial Doppler ultrasound screening and hydroxyurea therapy use remains low, according to research published in the Sept. 20 early-release issue of the U.S. Centers for Disease Control and Prevention Morbidity and Mortality Weekly Report.
Laura A. Schieve, Ph.D., from the CDC in Atlanta, and colleagues analyzed data from the IBM MarketScan Multi-State Medicaid Database and examined TCD screening and hydroxyurea use for 3,352 children and adolescents with SCA aged 2 to 16 years.
The researchers found that TCD screening increased 27 percent among children and adolescents aged 10 to 16 years during 2014 to 2019, and hydroxyurea use increased 27 and 23 percent among children aged 2 to 9 and 10 to 16 years, respectively. Only 47 and 38 percent of children aged 2 to 9 and 10 to 16 years, respectively, had received TCD screening in 2019, and 38 and 53 percent, respectively, used hydroxyurea. Usage was highest among children and adolescents with high levels of health care utilization and evidence of previous complications indicative of severe disease for both prevention strategies.
“Even among groups with the highest usage rates a substantial proportion of children and adolescents for whom these interventions are indicated were not receiving them,” the authors write.
Several authors disclosed financial ties to the biopharmaceutical industry.
Posted September 2022
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Sickle Cell Crisis Treatment
There is no one definitive treatment for sickle cell crisis, as the condition can vary greatly from person to person. Treatment typically focuses on managing pain and other symptoms, as well as preventing and treating complications. Medications, home remedies, and lifestyle changes can all be used to help ease symptoms and prevent sickle cell crisis.
Millions of people around the world are affected by a blood disorder known as sickle cell disease. Due to this condition, red blood cells stiffen and change shape, resulting in blood vessel obstruction. Suppressed blood cells have the ability to block blood vessels supplying blood to the brain and lungs. When the organ becomes damaged or inflamed, it will suffer pain and inflammation. A sickle cell crisis can occur as early as 6 months of age in humans. The pain can affect even the most sensitive parts of our bodies. People suffering from SCD describe the pain as difficult to describe.
How Anemocheck Mobile Can Help Those With Sickle Cell Anemia
If youre someone with sickle cell anemia who receives regular blood transfusions, AnemoCheck Mobile can be a great tool for your treatment. Regular transfusions could result in excess iron, which can lead to organ damage, digestive issues, and stomach ulcers.
And because we know iron is present in hemoglobin, a great way to check your iron levels would be with AnemoCheck Mobile. Simply take a fingernail selfie and youll have an instant reading.
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Infection And The Pathophysiology Of Scd
Deoxygenation of HbS alters the structure of the -chain of hemoglobin, resulting in reduced solubility, hemoglobin polymerization, and diminished membrane flexibility . Distortion of globin chains and exposure of intracellular heme iron intensifies intracellular oxidant stress, cell membrane damage, and erythrocyte dehydration . Vascular occlusion, tissue ischemia, and a cascade of systemic inflammation activate endothelial cells to interact with erythrocytes, activated leukocytes, the coagulation cascade, and activated platelets . Potent vasoconstrictors are liberated from the endothelium in response to injury . These events result in endothelial dysfunction, inflammation, and tissue ischemia, which correlates with SCD symptom severity . The clinical presentations of SCD-specific complications and serious infections can overlap. Care of the critically ill SCD patient must address infection while supporting the underlying pathophysiology of the disease to prevent or mitigate SCD-related complications. These interactions are summarized in Figure 2.
Chronic endothelial inflammation and dysfunction cause progressive vital organ system deterioration over time. Relevant organ system considerations for critical care management are summarized in Figure 3.
Why People With Sickle Cell Disease Are At Risk For Infections
Sickle cell disease starts damaging the organs in the first year of life. One of the first organs affected is the spleen. The spleen is a small organ on the left side of the abdomen underneath the ribcage. The spleen filters the blood and removes old, damaged red blood cells.
The spleen is also an important part of the immune system. It has two immune functions. It filters out bacteria, particularly a type of bacteria that has an outer covering that helps protect it from the body’s immune system. It also produces lymphocytes, white blood cells that help make antibodies to infections or in response to immunizations.
People with sickle cell disease have sickle-shaped blood cells. These cells can stick together and pool in the spleen, which damages it. The damage occurs repeatedly and spleen function is lost before the age of 5. This loss of function significantly increases the risk of infection.
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Whats It Like To Live With Sickle Cell Anemia
New treatments are helping people with sickle cell anemia live longer and with better quality of life. The U.S. Centers for Disease Control and Prevention has the following suggestions for living well with sickle cell anemia:
Living with medical complications
Sickle cell anemia is a chronic illness with symptoms that change over time. For example, children and adults have to cope with pain from acute chest syndrome or VOCs. Theyre at increased risk for stroke. As people grow older, theyre likely to develop new complications such as chronic lung disease and painful leg ulcers.
If you or your child has sickle cell anemia, ask your healthcare provider what you can do now and what you should expect. Theyll likely plan regular tests to monitor signs and symptoms so they can diagnose and treat complications as early as possible.
Studies show people with sickle cell anemia are constantly trying to manage pain that many times affects their quality of life. Sometimes, they have acute pain that happens when sickled cells block blood flow. This is VOC and is a medical emergency. Other people have chronic pain pain that lasts for more than three to six months. If you have chronic pain, consider working with a pain management specialist. They can recommend different ways you can manage pain.
When should I go to the emergency room?
Sickle cell anemia may cause serious medical conditions. Go to the emergency room if you have the following symptoms:
- Extreme fatigue.
Managing Infection In A Person With Sickle Cell Disease
At home, do not give fever reducers, such as acetaminophen or ibuprofen , for a fever of 100.4 degrees F or higher. Although these medicines can lower the bodys temperature, they can mask infection symptoms.
If you take your child to the St. Jude clinic or local emergency room, the staff will take a blood sample to test for infection. The blood test will help the doctor find the cause of infection.
After the blood sample is taken, the staff will give your child antibiotics. Antibiotics should be given after the blood sample is taken. If the antibiotics are taken first, it will be harder for the doctor to find the cause of infection. The staff might give other treatment depending on the cause, location, and severity of the infection.
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$1m Grant Will Help Scd Patients In Western Us Get Covid
Adults with SCD, particularly older patients, were reported to be at a higher risk of severe disease when infected with SARS-CoV-2, the virus that causes COVID-19. In the first waves of the pandemic, COVID-19 seemed to affect mostly adults, while children in general were spared infection.
However, viruses can acquire mutations that change their behavior over time. When this happens, scientists consider it a new variant.
One of the most recent variants of SARS-CoV-2 called Omicron has been considered less severe for adults in general, but has resulted in a higher number of children hospitalized.
How Is Sickle Cell Disease Treated
Sickle cell disease is a lifelong condition. Treatment helps people with sickle cell disease avoid problems and stay active. A treatment plan includes:
- Immunizations and daily doses of penicillin to help prevent infection. Kids with sickle cell disease should get all recommended vaccinations, including the pneumococcal, flu, coronavirus , and meningococcal vaccines.
- Folic acid supplements, which can help kids make new red blood cells.
- Medicines to help manage pain when it does happen.
A doctor may recommend other treatments for a child with sickle cell disease, such as:
- hydroxyurea, a daily medicine that makes the cells less sticky. This helps decrease the frequency and intensity of painful episodes and many other problems. It is strongly recommended for many children with sickle cell disease.
- blood transfusions for severe anemia or to treat or prevent some problems
- voxelator , a daily medicine to reduce sickling and increase the number of red blood cells by helping them hold on more tightly to oxygen
- crizanlizumab , a medicine given by IV infusion that can help make red blood cells more slippery and can reduce pain crises
- L-glutamine, a daily medicine taken by mouth to reduce pain
Stem cell transplant is the only proven cure for sickle cell disease. Transplants are complex and risky but often very successful. They’re currently an option only for some patients.
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What Are The Signs And Symptoms Of Medical Conditions Linked To Sickle Cell Anemia
Sickle cell anemia symptoms typically start when babies are 5 to 6 months old. As they grow older, most people with sickle cell anemia have increased risk for developing new medical conditions. Some of these conditions are life-threatening. But by learning about conditions and symptoms, people with sickle cell anemia can seek help at the first sign of trouble so healthcare providers can treat the condition.
Healthcare providers may call this condition acute pain crisis. VOC, or acute pain crises, is the most common reason why people with sickle cell anemia go to the emergency room or need to spend time in the hospital. Symptoms include:
- Sudden intense pain.
- Pain can be sharp or stabbing.
- VOC may affect any part of your body, but typically affects your abdomen, lower back, arms and legs.
Living with VOC is one of the more difficult aspects of having sickle cell anemia. Among other symptoms and complications, people who have sickle cell anemia may feel depressed or anxious because theyre dealing with certain stigmas associated with the condition.
Stigma and sickle cell anemia
Healthcare providers sometimes call VOC the invisible illness because people who are having a pain crisis many times dont have symptoms other than sudden excruciating pain thats only eased with opioid painkillers.
Acute chest syndrome
- Trouble breathing.
People who have sickle cell anemia may have mild, moderate or severe forms of anemia. Symptoms include: