What Is Sickle Cell Disease In Children
Sickle cell disease is a blood disorder that a child is born with. It’s passed down through a parents genes. Children with SCD make an abnormal type of hemoglobin. This is the protein in red blood cells that carries oxygen to all parts of the body. With SCD, the body organs and tissues dont get enough oxygen.
Healthy red blood cells with normal hemoglobin are round and move easily through blood vessels. When a child has SCD, the red blood cells are hard and sticky. They are shaped like the letter C . These damaged red blood cells clump together. They cant move easily through the blood vessels. They get stuck in small blood vessels and block blood flow. This blockage can cause pain. It can also damage major organs.
Sickle cells die sooner than healthy cells. Normally the spleen helps filter infections out of the blood. But sickle cells get stuck in this filter and die. Having fewer healthy red blood cells causes anemia. The sickle cells can also damage the spleen. Without a healthy spleen, children are more at risk for serious infections.
There are several complex types of the sickle cell gene. Some dont cause symptoms or severe problems, but others do. Talk to your childs healthcare provider about the specific form of sickle cell your child has.
Most children with SCD will start showing symptoms during the first year, often around 5 months.
Mycoplasma Infection And Red Cell Auto
There is therefore the need for detailed studies to evaluate the incidence of auto-antibodies and their effect on rheology, sickling, opsonization, neutrophil-sickle cell adherence and vascular occlusion in SCD patients infected by mycoplasma. Meanwhile, it is important to ensure that SCD patients with ACS and mycoplasma infection are promptly screened for cold and warm auto-agglutinins. While keeping the patients in warm environment will largely mitigate the effect of cold antibodies, cautious administration of steroidal immunosuppressive agents maybe necessary to abolish the production of warm auto-antibodies while taking care not to further jeopardize the inherent immune dysfunction due to the background SCD.
How Is The Hemoglobin S Gene Inherited
When the hemoglobin S gene is inherited from only one parent, and a normal hemoglobin genehemoglobin Ais inherited from the other, that person will have sickle cell trait. People who have sickle cell trait are generally healthy.
Only rarely do people who have sickle cell trait have complications similar to those seen in people who have sickle cell disease. But people who have sickle cell trait are carriers of a defective hemoglobin S gene, so they can pass it on when they have a child.
If the childs other parent also has sickle cell trait or another abnormal hemoglobin gene, such as beta-thalassemia, hemoglobin C, hemoglobin D, or hemoglobin E, that child has a chance of having sickle cell disease.
Inheritance pattern for sickle cell disease. The image shows how hemoglobin S genes are inherited. A person inherits two hemoglobin genesone from each parent. A normal hemoglobin A gene will make normal hemoglobin. A hemoglobin S gene will make abnormal hemoglobin.
In the image above, each parent has one normal hemoglobin A gene and one hemoglobin S gene, which means each of their children has:
- A 25% chance of inheriting two normal hemoglobin A genes. In this case, the child does not have sickle cell trait or disease.
- A 50% chance of inheriting one normal hemoglobin A gene and one hemoglobin S gene. This child has sickle cell trait.
- A 25% chance of inheriting two hemoglobin S genes. This child has sickle cell disease.
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What Else Can I Do To Control The Pain
A heating pad, hot bath, rest or massage might help. Physical therapy to relax and strengthen your muscles and joints might lessen your pain. Individual counseling, self-hypnosis and activities to keep you from thinking about your pain might also help.
It’s important for you to have a positive attitude, create a supportive environment, and develop coping skills to help you deal with your disease. Strong family relationships and close personal friends can be helpful. A support group might help you cope with your disease.
Work with your family doctor to set goals for the management of your pain. Becoming more actively involved in your treatment will help you better manage your disease.
Central Nervous System Involvement
Central nervous system involvement is one of the most devastating aspects of SCD. It is most prevalent in childhood and adolescence. The most severe manifestation is stroke, resulting in varying degrees of neurological deficit. Stroke affects 30% of children and 11% of patients by 20 years. It is usually ischemic in children and hemorrhagic in adults.
Hemiparesis is the usual presentation. Other deficits may be found, depending on the location of the infarct.
Convulsions are frequently associated with stroke. Convulsions occur as an isolated event but also appear in the setting of evolving acute chest syndrome, pain crisis, aplastic crisis, and priapism. Rapid and excessive blood transfusion to a hemoglobin level of greater than 12 g/dL increases blood viscosity and can lead to stroke.
Children with sickle cell disease may have various anatomic and physiologic abnormalities that involve the CNS even if they appear to be neurologically healthy. These silent brain infarcts occur in 17% of patients and may be associated with deterioration in cognitive function, with effects on learning and behavior these infarcts may increase the potential risk for clinical and subclinical damage to the CNS.
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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 .
Chronic endothelial inflammation and dysfunction cause progressive vital organ system deterioration over time. Relevant organ system considerations for critical care management are summarized in .
When Should I Seek Immediate Care
- You feel like you cannot cope with your pain, or you feel like hurting yourself.
- You have behavior changes, a seizure, or faint.
- You have a fever.
- You have abdominal pain, nausea, or vomiting.
- You have a different or worse headache.
- You have new weakness or numbness in your arm, leg, or face.
- You have new pain in any part of your body.
- Your urine is dark and you are urinating less than usual or not at all.
- You are dizzy, lightheaded, or faint.
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What Are The Treatments For Sickle Cell Disease
The only cure for SCD is bone marrow or stem cell transplantation. Because these transplants are risky and can have serious side effects, they are usually only used in children with severe SCD. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister.
There are treatments that can help relieve symptoms, lessen complications, and prolong life:
- Antibiotics to try to prevent infections in younger children
- Pain relievers for acute 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 blood. This medicine is not right for everyone talk to your health care provider about whether you should take it. 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 as a stroke, you may have transfusions to prevent more complications.
There are other treatments for specific complications.
To stay as healthy as possible, make sure that you get regular medical care, live a healthy lifestyle, and avoid situations that may set off a pain crisis.
NIH: National Heart, Lung, and Blood Institute
What Is Sickle Cell Disease
Sickle cell disease is a condition in which red blood cells are not shaped as they should be. Red blood cells look like round discs. But in sickle cell disease, they’re shaped like sickles, or crescent moons, instead.
These sickle shaped cells get stuck together and block small blood vessels. This stops blood from moving as it should, which can lead to pain and organ damage.
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Treatments During The Covid
If you take medications such as Oxbryta and Adakveo , you might not be able to receive these treatments if you are exposed to or get COVID. Most other SCD treatments are OK to continue if you are exposed to or get the virus.
If you are concerned about being exposed to COVID when going to and from your appointments, your doctor may permit telehealth visits.
Hydroxyurea is one of the primary treatments for SCD. The medication may reduce the need for blood transfusions at a time when there is a shortage of donated blood. Whats more, even when blood donations are available, going to the clinic for a blood transfusion carries the risk of potential exposure to COVID.
Pain is common with SCD. In some cases, people who would ordinarily be hospitalized for treatment with intravenous pain medications might be treated with oral medications to avoid going to the hospital and risking exposure to the virus.
Transfusions or exchange transfusions to prevent strokes, acute chest syndrome, or painful erections should be continued during the pandemic. The exception would be if the pandemic has caused shortages that make the treatment unavailable.
For those who are not being treated with Oxbryta, therapy might be considered for those who have low hemoglobin levels and who have difficulty with transfusions because they have antibodies against some antigens in blood products.
What Causes Sickle Cell Anemia
Is there an identifiable cause of rth diseases? Gene defects make up the root cause of inherited diseases like s gene, is an inherited disease caused by a defect in a gene. In order for a child to be born with sickle cell disease, they have both parents with the same gene, one from each mother and father. inherit just one copy of a gene, they are believed to be carriers of the disease and thus healthy.
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Sickle Cell Disease And Risk Of Exposure To Sars
People who have SCD often require more frequent medical care than people who do not have the disease. The increased need for medical care translates to a higher likelihood of being exposed to people who are infected with SARS-CoV-2.
There have not been enough studies looking at how many people with SCD get COVID compared to the general population, but visits to the pharmacy for medication, hospitalizations for pain crises, and transfusions may increase their chances of exposure.
Potential Genetic Therapy Treatments For Sickle Cell Disease
Researchers at the NHLBI are exploring ways genetic therapies may help develop new treatments or find a cure for sickle cell disease. Genetic therapies aim to treat or cure conditions by adding new DNA or changing existing DNA.
Watch this video to learn about how genome editing works and how it could be used to treat sickle cell disease.
Genetic therapy involves either restoring a faulty or missing gene or adding a new gene that improves the way the cell works. Researchers take blood or bone marrow from a patient and modify their stem cells in a laboratory using genetic therapies.
Genetic therapies that modify a persons own hematopoietic stem cells may provide a cure for people who have sickle cell disease and do not have a well-matched donor. Modified stem cells can be injected into the blood, then the cells travel in the bloodstream to the marrow spaces inside the bones. Once inside the bone marrow, the cells can produce healthy red blood cells that do not sickle.
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Common Symptoms Of An Infection
- Fevertemperature of 100.4 degrees F or higher
- Cough, chest pain, trouble breathing
- Swelling, tenderness, and redness in the skin or in the area over a bone or joint
Families of patients with sickle cell disease should watch for fever. A body temperature of 100.4 degrees F or higher is a medical emergency. Call the H clinic right away at 901-595-5041 if your child has a fever of 100.4 degrees F or higher.
After 5 p.m. and on weekends and holidays:
Crizanlizumab For Sickle Cell Crisis
Monoclonal antibodies to prevent sickle cell crises represents the promise and difficulties of high tech medical treatment.
Crizanlizumab is a monoclonal antibody treatment to reduce the risk of a sickle cell crisis. You can tell because the name ends in mab. There are a lot of mab drugs coming onto the market in the last few years. One might be tempted to think that this is a new technology, but it was first developed in 1975. There was a long road of development from the first monoclonal antibodies to the current crop of treatments, and it is a good reflection of the relationship between cutting edge science and medicine.
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What Is The Most Common Cause Of Sickle Cell Crisis
Cold air, wind, and water may increase a persons risk of developing a crisis by triggering red blood cell inflammation in their exposed areas. You should exercise with care if you want to be healthy. You are advised to rest after exercising for at least 15 minutes to relieve fatigue. Drink a good amount of fluids as soon as you feel dizzy.
Rate Of Sickle Cell Disease In Uganda
The data compiled on sickle cell disease in Uganda has not been updated since the early 1970s. The deficiency of data is due to a lack of government research funds, even though Ugandans die daily from SCD. Data shows that the trait frequency of sickle cell disease is 20% of the population in Uganda. This means that 66 million people are at risk of having a child who has sickle cell disease. It is also estimated that about 25,000 Ugandans are born each year with SCD and 80% of those people don’t live past five years old. SCD also contributes 25% to the child mortality rate in Uganda. The Bamba people of Uganda, located in the southwest of the country, carry 45% of the gene which is the highest trait frequency recorded in the world. The Sickle Cell Clinic in Mulago is only one sickle cell disease clinic in the country and on average sees 200 patients a day.
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What Causes Sickle Cell Disease In A Child
Sickle cell is present at birth. It is inherited when a child has 2 sickle cell genes, 1 from each parent.
A child who has only one sickle cell gene is healthy. But he or she is a carrier of the disease. If two carriers have a child, there is a greater chance their child will have sickle cell disease.
Once parents have had a child with sickle cell disease, there is a 1 in 4 chance that another child will be born with sickle cell disease. There is also a 1 in 2 chance that a child will be a carrier, like the parents.
What Medicines Can I Use At Home To Control My Pain
Some over-the-counter medicines might help relieve mild pain. Taking acetaminophen or aspirin might help. Medicines like ibuprofen or naproxen sodium might help if you can safely take these medicines. However, talk to your doctor before you take any medicine for your pain.
If you have moderate to severe pain, your doctor might prescribe a mild narcotic like codeine. This medicine is often given with aspirin or acetaminophen. You take this medicine regularly, around the clock, rather than waiting for the pain to return before taking your next dose.
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Enhancing Healthcare Team Outcomes
Patients with sickle cell crises are best managed by an interprofessional team that also includes ICU nurses. The key is rapid hydration and pain control. In addition, oxygenation should be monitored. It is important to find and treat the trigger of the crisis to prevent a recurrent crisis.
Despite optimal treatment, the quality of life of most patients with sickle cell is poor, marked by repeated admissions. The pharmacist should ensure that the patient is compliant with hydroxyurea because it has been shown to reduce the adverse effects of the disease.
What Medications Treat Sickle Cell Anemia
Sickle cell pain crisis
- Pain medications, often narcotics, will be given.
- IV fluids are an important part of therapy.
- Infection: If the physician diagnoses or suspects a bacterial infection, antibiotics are prescribed.
- Anemia: If there is a significant decrease in the red blood cell count, a red blood cell transfusion may be needed.
- Hydroxyurea may be used to increase the amount of Hgb F and decrease the amount of Hgb S, which can decrease the long-term risks of sickle cell crises.
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