Symptoms Of Sickle Cell Disease
People born with sickle cell disease tend to have problems from early childhood, although some children have few symptoms and lead normal lives most of the time.
The main symptoms of sickle cell disease are:
- painful episodes called sickle cell crises, which can be very severe and last up to a week
- an increased risk of serious infections
- anaemia , which can cause tiredness and shortness of breath
Some people also experience other problems, such as delayed growth, strokes and lung problems.
What Is Sickle Cell Anemia
Sickle cell anemia is a form of the inherited blood disorder, sickle cell disease. Sickle cell anemia affects your red blood cells, turning them from round flexible discs into stiff and sticky sickled cells. Sickled cells keep red blood cells from doing their job, which is carrying oxygen throughout your body. Sickled cells also dont live as long as normal red blood cells. As a result, you dont have enough healthy red blood cells and you develop anemia, the condition that gives sickle cell anemia its name.
In the past, babies born with sickle cell anemia rarely lived to be adults. Now, thanks to early detection and new treatments, about half of all people who have sickle cell anemia live into their 50s. People who have sickle cell anemia still face potentially life-threatening medical complications. However, healthcare providers have treatments that reduce the risk of complications and ease symptoms when they happen.
Managing Your Child’s At
You can take steps to help your child cope at school with the special needs of having sickle cell disease.
- Help your child keep up in school.
Make arrangements with teachers or a tutor to help your child keep pace with classmates when illness causes absences from school.
- Talk with your child’s teachers about washroom breaks.
Explain that children with sickle cell disease may need to use the washroom more often than other kids. They also need more water than the other students. Not drinking enough water can raise the chance of a sickle cell crisis.
- Give school staff members instructions about what to do in an emergency.
Educate teachers and other school employees about the signs and symptoms of sickle cell disease that need urgent medical care. Write down the instructions and list who to call in an emergency.
- Take steps to help your child safely exercise and play at school.
- Have your child drink plenty of fluids before, during, and after exercise. Lack of fluids can cause cells to sickle.
- Encourage your child to get regular rest breaks during vigorous exercise.
- Remind your child to stay warm. Exposure to cold air, wind, and water can trigger a sickle cell crisis. Dress your child in warm layers of clothing for cold-weather activities. Your child should avoid swimming and playing in cold water.
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What Is The Life Expectancy Of Sickle Cell Anemia
The life expectancy of persons with sickle cell anemia is reduced. Some patients, however, can remain without symptoms for years, while others do not survive infancy or early childhood. Nevertheless, with optimal management patients can now survive beyond the fourth decade.
Most patients suffer intermittent pain crises, fatigue, bacterial infections, and progressive tissue and organ damage. Impaired growth and development is the result of the physical and emotional trauma that is endured by children with sickle cell anemia.
Causes of death include bacterial infection , stroke or bleeding into the brain, and kidney, heart, or liver failure. The risk of bacterial infections does diminish after three years of age. Nevertheless, bacterial infections are the most common cause of death at any age. Therefore, any signs of infection in a person with sickle cell anemia must be reviewed with a doctor to prevent damage and save lives.
The sickle cell gene is not a “black gene.” It just happens to disproportionately occur in the black population. When a black person who carries a sickle cell gene has children with a non-black person, the children may inherit the sickle cell gene regardless of race. There are also people of all races who carry the sickle cell gene.
How Is Fatigue Treated
Fatigue is hard to treat because many factors can contribute to feelings of exhaustion. Also, very little research has been done on treatments that reduce fatigue in people with SCD.3
Fatigue is usually treated by reducing the impact of anemia. These steps may include:8
- Eating a balanced diet
- Developing a healthy sleep routine
- Planning your day to include rest periods
- Drinking plenty of water.
- Trying to do light exercise every day
- Asking for help when you need it with childcare, shopping, chores, or driving
- Avoiding injuries that might cause bleeding or bruising.
- Making time for distracting or relaxing activities, such as meditation, yoga, and reading.
Treatments that reduce the frequency of pain crises, such as hydroxyurea, may be helpful if pain is disrupting your sleep. Severe anemia caused by an infection or enlarged spleen may be treated with blood transfusions.5
Addressing stress, depression, and anxiety may also help. Counseling, support groups, and stress management techniques may improve your mood and energy levels. Sometimes, medications for depression or anxiety may also help combat fatigue.
Talk to your doctor about the best way to treat fatigue for you. They may refer you to physical therapists, nutrition counselors, or other specialists. They can also discuss how certain medications may help or hurt your energy level.
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Fatigue Can Be Disruptive
It may be true that everyone is tired these days. People work too many hours, stay up too late and spend a lot of time online. But their tiredness is nothing like the fatigue you have with sickle cell. To help with fatigue, it is important to practice a good sleep routine, which means building healthy habits that support sleep. Some of these habits include:
- Limiting naps to less than 30 minutes
- Limiting caffeine to the morning
- Having a bedtime routine that you follow every night
- Making your bedroom pleasant, dark, and as free of distractions as possible
Signs Symptoms And Prevention
Because of the microcirculatory distress, a telltale sign or symptom of a potential sickling collapse is cramping. Specifically to sickle cell trait, cramping occurs in the lower extremities and back in athletes undergoing intense physical activity or exertion. In comparison to heat cramps, sickling cramps are less intense in terms of pain and have a weakness and fatigue associated with them, as opposed to tightly contracted muscles that lock up during heat cramps.
A sickling collapse comes on slowly, following cramps, weakness, general body aches and fatigue. Individuals with known positive sickle cell trait status experiencing significant muscle weakness or fatigue during exercise should take extra time to recover and hydrate before returning to activity in order to prevent further symptoms.
A collapse can be prevented by taking steps to ensure sufficient oxygen levels in the blood. Among these preventative measures are proper hydration and gradual acclimation to conditions such as heat, humidity, and decreased air pressure due to higher altitude. Gradual progression of exertion levels also helps athletes’ bodies adjust and compensate, gaining fitness slowly over the course of several weeks.
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When Should I Call The Doctor
Get emergency medical care right away if your child has any of these problems:
- fever of 101°F or higher
- pain that isn’t getting better with medicine
- chest pain
- severe stomach pain or swelling
- shortness of breath or trouble breathing
- extreme tiredness
- skin that’s yellow or very pale
- sudden change in vision
- weakness or trouble moving part of the body
- slurred speech
- loss of consciousness
- numbness or tingling
What Is Sickle Cell Trait
Did you know theres more than one way to inherit sickle cell trait? Learn how it is inherited »pdf icon
People who inherit one sickle cell gene and one normal gene have sickle celltrait . People with SCT usually do not have any of the symptoms of sickle cell disease , but they can pass the trait on to their children.
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Outlook For Sickle Cell Disease
Sickle cell disease varies between individuals from mild to serious, but most people with it lead happy and normal lives.
Mild sickle cell disease may have no impact on a person’s day-to-day life.
But the illness can be serious enough to have a significant effect on a person’s life.
It can lead to health problems like strokes, serious infections and lung problems, which can occasionally be fatal.
Overall, the life expectancy for someone with sickle cell disease tends to be shorter than normal, but this can vary depending on the exact type of sickle cell disease they have, how it’s treated and what problems they experience.
Sickle Cell Trait And Chronic Kidney Disease
In patients with heterozygous sickle cell trait, approximately 40% of their RBC Hb is HbS and the rest is normal HbA. In general these patients have normal Hb levels and do not have symptoms of hemolysis or vaso-occlusion. However, there have been reports of catastrophic vaso-occlusive crises and sudden death in young people with sickle cell trait under extreme adverse conditions such as excessive exercise or exposure to severe hypoxia. In addition, renal medullary carcinoma is more common in patients with SCT than SCD, though the reasons for this are unclear. Non-malignant microhematuria and macrohematuria are reported more frequently in patients with sickle cell trait than in the general population, and older patients exhibit a loss of urinary concentrating ability. Patients who co-inherit sickle cell trait and adult polycystic kidney disease have a more rapid decline to ESRD than family members with APKD who do not carry an HbS gene. Whether having sickle cell trait alone is a risk factor for progressive CKD has been debated, but a recent study in the United States using data from five large prospective studies of African Americans concluded that patients with SCT had an odds ratio of developing incident CKD of 1.76, an OR of experiencing a decline in renal function of 1.32, and an OR of having albuminuria of 1.86 compared with noncarriers.23,53,54
Dwomoa Adu, Akinlolu O. Ojo, in, 2020
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Preventive Measures For Managing Adult Sickle Cell Disease
Here are some other ways you can manage your adult sickle cell disease to help prevent complications.
To reduce the risk of infection:
- Take prophylactic antibiotics, such as penicillin
- Stay up to date on certain vaccinations, especially:
- Haemophilus influenzae vaccine
- Pneumococcal conjugate vaccine
- Pneumococcal polysaccharide vaccine
Be sure to stay hydrated and take a folic acid vitamin supplement every day. The body needs folic acid to produce red blood cells.
Other Sickle Cell Disease Treatments And Support Services
- Bone marrow and stem cell transplants Although these therapies have the potential to cure sickle cell disease, they are currently not an option for the majority of people with sickle cell disease. Frequently, people with sickle cell disease cannot locate well-matched stem cell donors.
- Support services for adults with sickle cell disease We offer counseling to address emotional and social complications. We also provide treatment for the overuse or abuse of narcotic pain medications used to alleviate the intense pain that occurs during pain crises.
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Why Does Sickle Cell Disease Cause Fatigue
Fatigue is an important symptom of SCD. Young people with SCD report being tired, having low energy, wanting to sleep, and being unable to perform many daily activities. They express fatigue-related concerns more often than pain-related concerns. But much less research is done on fatigue than on pain in SCD.1,4
People with SCD experience fatigue because of anemia. This happens when sickle cells die early and there are not enough healthy red blood cells to carry oxygen throughout the body.5
SCD also causes acute and chronic pain because of blocked blood flow. Adults with SCD who experience worse pain have lower energy levels. This is because pain crises can lead to poor sleep quality. People with SCD commonly experience stress, depression, and anxiety. These may also contribute to fatigue.1,6,7
We need more research to understand how anemia, pain, and depression are connected to fatigue in SCD. We also do not know how other personal factors like age, sex, socioeconomic status, treatments, and disease severity contribute to fatigue.1
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
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.
Voxelotor
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 therapy
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.
Crizanlizumab-tmca
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What Is Sickle Cell Disease
Sickle cell disease is a genetic red blood cell disorder. It changes normal, round red blood cells into cells shaped like crescent moons. Sickled cells can get stuck in blood vessels and block them, which stops oxygen from getting through. That can cause a lot of pain and can harm organs, muscles, and bones.
How Do You Get Sickle Cell Anemia Is It Inherited
Sickling of the red blood cells in patients with sickle cell anemia results in cells of abnormal shape and diminished flexibility. The sickling is promoted by conditions associated with low oxygen levels, increased acidity, or low volume of the blood. These conditions can occur because of injury to the body’s tissues, dehydration, or anesthesia.
Certain organs are predisposed to lower oxygen levels or acidities, such as when blood moves slowly through the spleen, liver, or kidney. In addition, organs with particularly high metabolism rates promote sickling by extracting more oxygen from the blood. These conditions make these organs susceptible to injury from sickle cell anemia.
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Fatigue And Quality Of Life
For the third aim, we examined the relationships between fatigue and quality of life. All of the fatigue measures significantly and negatively correlated with all 8 subscales of the SF-36, indicating that greater fatigue was associated with lower quality of life . The strengths of these relationships were also moderate to strong, with correlations ranging from r = â0.34 to â0.74.
How Is Sickle Cell Trait Diagnosed
A simple blood test can show whether you have sickle cell trait. During pregnancy, a woman can have a test to find out if the baby will have either sickle cell disease or sickle cell trait. In Canada, infants at high risk for sickle cell disease may be screened before they go home from the hospital. Some college athletic programs screen student athletes to find out if they have sickle cell trait.
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Can Healthcare Providers Cure Sickle Cell Anemia
Right now, allogenic stem cell transplantation is the only way healthcare providers can cure sickle cell anemia and other forms of sickle cell disease. Healthcare providers typically recommend transplantation for people who have severe complications such as stroke, acute chest syndrome or recurring VOC/acute pain crises.
Sickle Cell Trait Can Take A Sudden Deadly Turn
Many people who carry sickle cell trait dont know it. Yet, it can be just as deadly as sickle cell anemia.In the United States, about 80,000 people have sickle cell anemia, but more than 12 million have sickle cell trait. Both disorders target certain ethnic groups, including South and Central Americans, Cubans, Saudi Arabians, Asians, Mediterraneans, and American Indians, but the disorders disproportionately strike African Americans. About 1 in 400 African Americans have sickle cell disease. About 1 in 10 African Americans have sickle cell trait.
What causes sickle cell disordersBoth sickle cell anemia and sickle cell trait are genetic disorders. People with sickle cell anemia have inherited homozygous sickle hemoglobin genes from both parents their blood type is called hemoglobin SS. People with sickle cell trait have inherited a normal hemoglobin gene from one parent and a sickle hemoglobin gene from the other parent their blood type is called hemoglobin AS.Sickle cell anemia is a general term for several genetic disorders caused by red blood cells that are sickle-shaped because of deformed hemoglobin. A chronic condition, sickle cell anemia causes life-threatening signs and symptoms, such as an enlarged spleen, muscle pain, liver scarring, pneumonia, and bone and joint pain. The typical lifespan for those with sickle cell anemia is 40 to 60 years.
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How Is Sickle Cell Disease Diagnosed
Sickle cell disease and sickle cell trait usually are found at birth with a blood test during routine newborn screening tests. A second blood test will confirm the diagnosis.
Sickle cell disease also might be diagnosed before a baby is born with a test on the amniotic fluid or with a sample of tissue from the placenta.
