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Ortho Sickle Cell Controls

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Last Updated: 17 November 2020

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General | Latest Info

Sickle Cell disease is a group of inherited red blood cells disorders. People who have Sickle Cell disease have abnormal protein in their red blood cells. In the United States, most people who have Sickle Cell disease are from African ancestry, but the condition is also common in people of Hispanic background. Because disease run in families, couples planning to have children can have genetic testing. Early signs and symptoms of Sickle Cell disease include swelling of hands and feet; symptoms of Anemia, including fatigue, or extreme tiredness; and jaundice. Over time, Sickle Cell disease can lead to complications such as infections, delayed growth, and episodes of pain, called pain crises. Most children who have Sickle Cell disease are pain-free between crises, but adolescents and adults may also suffer with chronic, ongoing pain. Over lifetime, Sickle Cell disease can harm patients ' spleen, brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin. Blood and bone marrow transplant is currently the only cure for Sickle Cell disease, and only a small number of people who have Sickle disease are able to have transplant. There are effective treatments that can reduce symptoms and prolong life. Early diagnosis and regular medical care to prevent complications also contribute to improved well-being. Sickle Cell disease is a life-long illness. Severity of disease varies widely from person to person. People who have Sickle Cell disease have abnormal Hemoglobin, call Hemoglobin S or Sickle Hemoglobin, in their red blood cells. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. People who have Sickle Cell disease inherit two abnormal Hemoglobin genes, one from each parent. Hemoglobin S0 thalassemia Hemoglobin S + thalassemia Hemoglobin SC Hemoglobin SD Hemoglobin SE Hemoglobin SS in all types of Sickle Cell disease, at least one of two abnormal genes causes a person's body to make Hemoglobin S. When a person has two Hemoglobin S genes, disease is called Sickle Cell Anemia. This is the most common and often the most severe type of Sickle Cell disease. Hemoglobin SC disease and Hemoglobin S thalassemia are two other common types of Sickle Cell disease. Hemoglobin SD and Hemoglobin SE are much less common. Sickle Cell disease is a lifelong illness. Severity of disease vary widely from person to person. People with Sickle Cell disease can experience both acute and chronic signs, symptoms, and complications. Your doctor may diagnose Sickle Cell disease based on results from tests to confirm results from various screening tests. Blood and bone marrow transplant is currently the only cure for some patients who have Sickle Cell disease. After early diagnosis, goal is health maintenance to prevent complications and medicines and treatments to manage complications, including chronic pain.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Introduction

Sickle cell disease is the most common serious genetic disorder in sub-Saharan Africa. About 2 % of newborns in Ghana have SCD, and in the general population about 25 % have trait. 1 2 principal genotypes include homozygous sickle cell anaemia, sickle cell haemoglobin C disease, and sickle cell-thalassaemia. Pathologic effects of sickle cell anemia, demonstrated in mineralized connective and connective tissues in other areas of the body, also occur in oral tissues. 3 several oral findings described in literature include uncommon degree of periodontitis, hypomaturation and hypomineralization in enamel and dentin and bone alterations resulting in maxillary protrusion and formation of thick trabecular pattern. 3 4 5 Osteoporosis of alveolar crest has been identified by radiographic examination. 6 7 8 9 Odonkor 10 has shown that changes seen radiographically involving maxillofacial and dental tissues are similar to those observed in cases of rickets, fluorosis and after thyroidectomy. Craniofacial features, such as maxillary protrusion and more forward growth of mandible with significantly retruded maxillary and mandibular incisors referred to in FID Konotey-Ahulu's book as gnathopathy, have also been documented in black American children with SCD. 11 12 in a study of 36 Sicilian patients with SCD, Licciaderllo et al., 13 show that clinical craniofacial manifestations were of moderate severity compared to that observed in black American children. According to Mourshed and Tuckson 14 most frequent oral manifestations of SCA include paleness of oral mucosa, delays in tooth eruption, atrophy of tongue papillae, impaired dentine mineralization, mandibular osteomyelitis, and orofacial pain. Craniofacial alterations in SCD patients occur as a result of hyperplasia and compensatory expansion of bone marrow, resulting in exaggerated growth / protrusion of midface, maxillary expansion, predominance of vertical growth, mandibular retrusion, convex profile, and maxillary protrusion. 7 11 13 15 16-19 Other studies, however, have shown that facial features of SCA patients are similar to those characteristics of patients without SCA and that these patients show no compensatory maxillary expansion. 20 literature is scarce on case reports involving sickle cell patients undergoing orthodontic treatment, however two such cases have been reported by Oluwatosin et al 21 and Pithon. 22 in this report, special measures were taken to ensure that patients do not present in any vaso-occlusive crisis. Measures taken include antibiotic cover before any invasive procedure; scheduling of dental appointments for early morning and care to avoid any emotional stress and multidisciplinary care including physician or haematologist of patient.

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Discussion

SCD is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, red blood cells become hard and sticky and look like a C-shaped farm tool called Sickle. 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 blood flow. This can cause pain and other serious problems such as infection, acute chest syndrome and Stroke.


Conclusions

SCD can markedly affect the musculoskeletal system and is responsible for a large burden of morbidity related to spinal pathology in this population. Though information about the relationship between SCD and spine pathology is lacking, it is well understood that the spine undergoes the same disease process as the rest of bones in the body of patient with SCD. These disease processes include vaso-occlusive crises and microinfarction, osteomyelitis, and osteoporosis, all of which lead to both acute and chronic presentations. One key clinical point to remember is that oftentimes these pathologies are difficult to distinguish from each other given overlap in inflammatory markers and clinical symptoms. Nonetheless, majority of SCD spine pathology can be treated conservatively with rest, symptomatic therapy, antibiotics, and orthosis. It is preferable to avoid surgery if possible as these patients have a higher risk of developing perioperative complications, including surgical site infection, and stress of surgery and anesthesia can trigger vaso-occlusive crises in a perioperative setting. If indicate, surgery may be necessary for certain SCD spine pathology. This is often the case in patients who fail to respond to 6 to 8 weeks of antibiotics for spinal abscess. With careful planning and technique of anesthesia and surgery, spine pathology from SCD can be effectively treat.

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* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Conclusions

In the above patient, all pretreatment objectives were realized, namely reduction in overjet and overbite to normal values, relief of mild crowding, and camouflage of class II skeletal pattern. Lip incompetence was also correct; as such, the patient was able to close lips together without any strain on perioral muscles. Our case shows that the quality of life for patients suffering from SCD can be improved for those presented with craniofacial disorders, when they seek dental treatment. Sickle cell patients with craniofacial manifestations should thus be encouraged to seek oral health and advice at an early stage since most of these severe manifestations are easily managed in growing children. In the treatment of patients suffering from SCD, special care must be taken to avoid excessive stress and forces. Care should also be taken when extractions are indicated and as much as possible this should be done in the hospital environment. Extraction of teeth and prescribing of 500mg Amoxycillin three times daily post extraction for one week has also proven successful without any adverse effects and this is routinely used for other sickle cell patients undergoing orthodontic treatment

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

For Parents

Table

ComplicationCauseSymptoms
Acute Chest SyndromeSickle cells clog and block blood flow to the lungs Can occur if your child has pneumoniaCough Chest pain Fever of 100.4F (38C) or higher Shortness of breath or difficulty breathing
AnemiaDecreased red blood cells, which commonly occurs because red blood cells dont live as long in children with sickle cell diseaseShortness of breath Dizziness Weakness Fast or irregular heartbeat Pale or yellow skin
InfectionsDamage to the spleen which normally filters bacteria out of blood caused by sickle cells Risk is higher in infants and children with sickle cell diseaseFever of 100.4F (38C) or higher Chills or shaking
Hand-Foot SyndromeSickle cells clog and block blood flow to the hands and feet Risk is higher in infants and children with sickle cell diseaseSwelling in the hands and feet Fever of 100.4F (38C) or higher
Pain EpisodesSickle cells clog and stop blood flow in small blood vesselsMild to severe pain, usually in the abdomen, arms, chest, lower back, and legs
Splenic SequestrationSickle cells clog and block blood flow in the spleen, causing it to get bigSudden weakness Pale lips Fast breathing Pain in the left side of the belly Fast heartbeat
StrokeSickle cells clog and block blood flow to the brainLoss of balance Severe headache Difficulty talking, walking, or understanding Weakness in the face, arm, or leg on one side of the body
Vision lossSickle cells clog and block blood flow to the eyeBlurry vision, difficulty seeing, or complete blindness

Every state in the US performs newborn Screening test for Sickle Cell disease. If this test finds signs of disease, baby is referred to a pediatric hematologist. If test suggests Sickle Cell Trait, parents are counselled by general pediatrician, and children generally do not need to see a hematology specialist. Early diagnosis, before infants show any symptoms, allows infants with Sickle Cell disease to get early treatment and may decrease their risk of infections and other serious complications. In addition to regular visits with their pediatrician, children with Sickle Cell disease should see a pediatric hematologist at least periodically. These doctors specialize in care of children with Sickle Cell disease.


What is sickle cell disease?

Normal, healthy red blood cells are round and carry oxygen throughout the body. If your child has sickle cell disease, their red blood cells become hard, sticky, and shaped like letter C. These sickled red blood cells can get stuck in small blood vessels, slowing or stopping the flow of blood and oxygen to parts of the body, such as legs and arms, lungs, heart, and brain. This decreased blood flow can cause pain, lead to infections, and make it difficult for your childs organs to function properly. Sickled red blood cells also only live 10 to 20 days, compared to 90 to 120 days for normal red blood cells. Because of this, your child may not have enough red blood cells. This is called anemia. Symptoms of anemia may include: paleness Tiredness Dizziness Headache Shortness of breath Fast heartbeat

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

For Coaches

Hydroxyurea has established a role as a safe and effective treatment for SCD. Hydroxyurea increases total and fetal Hemoglobin in children with SCD. Increase in fetal Hemoglobin retards gelation and sickling of RBCs. Hydroxyurea also reduces levels of circulating leukocytes, which decrease adherence of neutrophils to vascular endothelium. In turn, these effects reduce the incidence of pain episodes and acute chest syndrome episodes. In 2008, National Institutes of Health Consensus Development Conference concluded that strong evidence supports efficacy of Hydroxyurea in adults to decrease severe painful episodes, hospitalizations, number of blood transfusions, and acute chest syndrome. Although evidence for the efficacy of Hydroxyurea treatment for children is not as strong, emerging data is encouraging. In meta-analysis of literature from 2007, Strouse et al studied efficacy, effectiveness, and toxicity of Hydroxyurea in children with SCD and found that fetal hemoglobin levels increased from 5-10 % to 15-20 %; Hemoglobin concentration increased modestly but significantly; hospitalizations decreased by 56-87 %; and frequency of pain crisis decrease. A Phase III multicenter international clinical trial in 38 children with SCD found that Hydroxyurea treatment can lower elevated cerebral blood flow velocities, which have been linked to Stroke risk. After mean of 10. 1 month transcranial Doppler ultrasound showed that mean velocity had decreased 15. 5 cm / sec in patients receiving Hydroxyurea but had increased 10. 2 cm / sec in those receiving observation only. Post hoc analysis according to treatment receive show that after 15 months, conversion from conditional to abnormal cerebral blood flow velocities occurred in 50 % of patients in the observation group but none of those in the Hydroxyurea group. In December 2017, FDA approved Siklos to reduce the frequency of painful crises and need for blood transfusions in children 2 years of age and older and adolescents with Sickle Cell Anemia who have recurrent moderate to severe painful crises. Siklos is the first Hydroxyurea formulation to be FDA-approve for pediatric SCD. Approval was based on data from ESCORT, open-label single-arm trial that enrol 405 pediatric patients with Sickle Cell Disease from 2-18 years of age. The median change in fetal hemoglobin levels was 0. 5 g / dL in 63 patients at 6 months and 0. 7 g / dL in 83 patients at12 months after initiation of treatment. After 12 months of treatment, drug exhibits the ability to increase fetal hemoglobin in all patients, and decreases the percentage of patients who experienced at least one vaso-occlusive episode, one episode of acute chest syndrome, one hospitalization due to SCD, or one blood transfusion. Hydroxyurea is usually prescribed by a hematologist, using rigorous selection criteria. Indications for Hydroxyurea include the following: patients receiving Hydroxyurea require frequent blood testing and monitoring, with special attention to development of leukopenia and / or thrombocytopenia. Good continuous doctor-patient relationship and rapport must exist to ensure that potential toxicity is identified at its onset. Hydroxyurea is a potentially leukemogenic and carcinogenic agent.


For Athletes

What is Sickle Cell Trait? Sickle Cell Trait is a descriptive term for a hereditary condition in which an individual has one normal gene for Hemoglobin and one abnormal gene for Hemoglobin. 1 Hemoglobin is a protein in red blood cells in your body responsible for oxygen transport. Approximately 99 % of oxygen transport in blood is bind to hemoglobin inside red blood cells. 2 Sickle Cell Trait is generally a benign condition that DO not develop or change over time. In general, it is a condition that does not affect the life span of an individual. As individuals with Sickle Cell Trait get older, they may become unable to concentrate urine normally. This is not usually an issue for high school or college age athletes. 1 Sickle Cell Trait is not a barrier to exercise or participation in sport. 3 Knowing your Sickle Cell Trait status is vital to preventing exertional sickling event. First, check with your parents as they may have this on record from your birth. If you cannot find information this way, simple blood test at your doctor's office can tell you whether you are positive or negative for Sickle Cell Trait. What is Sickle Cell Anemia? Sickle Cell Anemia is a hereditary condition in which an individual has two abnormal Hemoglobin genes. 1 Sickle Cell Anemia is a lifelong disease that can cause painful episodes sometimes requiring hospitalization. Sickle Cell Trait will not develop into Sickle Cell Anemia at any time. Carriers of Sickle Cell Trait usually DO not have any of the clinical symptoms of Sickle Cell Anemia; however, they may pass this onto their children. 4 Complications of Sickle Cell Trait for Athletes Although Sickle Cell Trait will not turn into a disease, it is possible to have clinical symptoms when exercising under extreme conditions of physical exertion and / or low oxygen levels. 1 Athletes with Sickle Cell Trait are potentially more vulnerable to gross hematuria, splenic infarction, exertional heat stroke, severe muscle breakdown and potentially even Sudden Death when participating in strenuous exercise, often under intense environmental conditions. 1 3 5 managing your Condition NCAA recommend that athletes with Sickle Cell Trait be knowledgeable of their conditions and take the following precautions 1 3. Set your own pace Gradual acclimatization to heat, humidity and altitude Use adequate rest and recovery between intense conditioning sets Limit all-out exertion of any kind to less than 2-3 minutes Be excuse from Performance tests that require extreme exertion for longer than 2-3 minutes Stop activity immediately if any signs or symptoms occur Stay well hydrate at all times, especially in hot and humid conditions Manage asthma appropriately Suspend exercise if feeling ill or while experiencing fever Have access to supplemental oxygen as need Seek proper medical attention when experiencing unusual distress DO not participate during acute illness, especially one involving fever screening and Communication Complications from Sickle Cell Trait are often preventable. The first key to prevention, in any case, is communication.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Topic Overview

Pain is a long-lasting problem for people who have sickle cell disease. Bouts of severe pain can last for hours to days and are difficult to treat. Pain can be exhausting for caregivers as well as for person in pain. A Pain management plan can help people cope with chronic pain and with pain caused by sickle cell crisis. Your doctor or pain treatment specialist can help you make a pain management plan. This plan includes instructions on how to treat mild pain at home. And it tells you when you need to go to hospital if your pain gets worse. Clarifies your and your doctor's preferences ahead of time. Explain your needs to other health professionals who care for you. It offers you some predictability and control during times of crisis.


What is sickle cell disease (SCD)?

Sickle cell disease is a group of inherited red blood cell disorders. If you have SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With SCD, hemoglobin forms into stiff rods within red blood cells. This changes the shape of red blood cells. Cells are supposed to be disc-shaped, but this changes them into a crescent, or sickle, shape. Sickle-shaped cells are not flexible and cannot change shape easily. Many of them burst apart as they move through your blood vessels. Sickle cells usually only last 10 to 20 days, instead of the normal 90 to 120 days. Your body may have trouble making enough new cells to replace ones that you lose. Because of this, you may not have enough red blood cells. This is a condition called anemia, and it can make you feel tire. Sickle-shaped cells can also stick to vessel walls, causing blockage that slows or stops the flow of blood. When this happen, oxygen can't reach nearby tissues. Lack of oxygen can cause attacks of sudden, severe pain, called pain crises. These attacks can occur without warning. If you get one, you might need to go to hospital for treatment.


What is sickle cell trait?

Sickle cell trait occur when a person inherits sickle cell genes from just one parent. It's not the same as sickle cell disease, in which a person inherits two sickle cell genes, one from each parent. People with sickle cell disease have just one kind of hemoglobin, which turns their normal, round red blood cells into abnormally curved shapes. But people with sickle cell trait have both normal hemoglobin and hemoglobin S, so they rarely have symptoms of sickle cell disease. But they are carriers and can pass sickle cell genes to their children.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Cause

Table

ComplicationCauseSymptoms
Acute Chest SyndromeSickle cells clog and block blood flow to the lungs Can occur if your child has pneumoniaCough Chest pain Fever of 100.4F (38C) or higher Shortness of breath or difficulty breathing
AnemiaDecreased red blood cells, which commonly occurs because red blood cells dont live as long in children with sickle cell diseaseShortness of breath Dizziness Weakness Fast or irregular heartbeat Pale or yellow skin
InfectionsDamage to the spleen which normally filters bacteria out of blood caused by sickle cells Risk is higher in infants and children with sickle cell diseaseFever of 100.4F (38C) or higher Chills or shaking
Hand-Foot SyndromeSickle cells clog and block blood flow to the hands and feet Risk is higher in infants and children with sickle cell diseaseSwelling in the hands and feet Fever of 100.4F (38C) or higher
Pain EpisodesSickle cells clog and stop blood flow in small blood vesselsMild to severe pain, usually in the abdomen, arms, chest, lower back, and legs
Splenic SequestrationSickle cells clog and block blood flow in the spleen, causing it to get bigSudden weakness Pale lips Fast breathing Pain in the left side of the belly Fast heartbeat
StrokeSickle cells clog and block blood flow to the brainLoss of balance Severe headache Difficulty talking, walking, or understanding Weakness in the face, arm, or leg on one side of the body
Vision lossSickle cells clog and block blood flow to the eyeBlurry vision, difficulty seeing, or complete blindness

Your childs blood has many important jobs. It brings oxygen and nutrients to their organs, carries cells to fight off infections, and carries waste to their kidneys and liver to be filtered out of their body. In other words, healthy blood is vital to your childs health. Plasma: liquid part of blood, which transports blood cells throughout your body Platelets: cell fragments that can form clots, which help stop wounds from bleeding White blood cells: cells that help keep the body protect against infections Red blood cells: cells that help carry oxygen to the body and bring carbon dioxide back to lungs when your child has sickle cell disease, their Red blood cells are damage.


What is Sickle Cell Disease?

Children with sickle cell disease are at increased risk of infection and other health problems such as anemia, so early diagnosis and treatment are important to keep your child healthy. Penicillin: This medication prevents bacterial infections. Most children with sickle cell disease take penicillin until the age of 5. In some cases, your child may need to take it longer. Pain medication: These medications treat pain. For mild or moderate pain, Ibuprofen or other nonsteroidal anti-inflammatory medications are used. For severe pain, your child may need opioids. Hydroxyurea: This medication decreases or prevents sickle cell complications. Immunizations: Vaccines prevent infections, such as influenza, meningitis, and pneumonia. Blood transfusion: Blood transfusions treat severe anemia. Exchange transfusion: Exchange transfusion removes and replaces sickled blood with donor blood or plasma. This may be needed for children with severe sickle cell disease or complications. Stem cell transplant. Stem cell transplant replaces bone marrow with healthy blood-forming cells. This is a rare treatment option, but may cure sickle cell disease. Transplant is only used in severe cases.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Symptoms

If a person has sickle cell disease, it is present at birth. But most infants do not have any problems with disease until they are about 5 or 6 months of age. Every state in the United States, District of Columbia, and US territories requires that all newborn babies receive screening for sickle cell disease. When a child has sickle cell disease, parents are notified before the child has symptoms. Some children with sickle cell disease will start to have problems early on, and some later. Early symptoms of sickle cell disease may include: yellowish color of skin, known as jaundice, or whites of eyes, known as icterus, that occur when large number of red cells undergo Hemolysis Fatigue or fussiness from anemia Painful swelling of hands and feet, known as dactylitis signs and symptoms of sickle cell disease will vary from person to person and can change over time. Most of the signs and symptoms of sickle cell disease are related to complications of the disease.


Complications

Acute pain crisis Acute pain episodes, or crises, can occur without warning when Sickle cells block blood flow and decrease oxygen delivery. People describe this pain as sharp, intense, stabbing, or throbbing. Severe crises can be even more uncomfortable than post-surgical pain or childbirth. Pain can strike almost anywhere in the body and in more than one spot at a time. But pain often occurs in several places: abdomen arms, chest, lower back legs, high altitudes Dehydration, or not drinking enough fluids Illness Stress Temperature changes, but often people do not know what triggers, or causes, crisis. Chronic pain Many adolescents and adults with Sickle cell disease suffer from chronic pain. This kind of pain has been hard for people to describe, but it is usually different from crisis pain or pain that results from organ damage. Chronic pain can be severe and can make life difficult. Its cause is not well understood. Severe anemia People with Sickle cell disease usually have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life threatening. Severe anemia in infants or children with Sickle cell disease may be the result of events: aplastic crisis. Aplastic crisis is usually caused by parvovirus B19 infection, also called fifth disease or slap cheek syndrome. Parvovirus B19 is a very common infection, but in Sickle cell disease, it can cause bone marrow to stop producing new red cells for a while, leading to severe anemia. Blood cells get trapped in the spleen. A Spleen is an organ that is located on the upper left side of the belly. Spleen filters your blood and destroys old blood cells. In people who have Sickle cell disease, red blood cells may get trapped in the spleen, making it enlarge quickly. With red blood cells trapped in the spleen, fewer are available to circulate in blood, and this can set off severe anemia. Large spleen may also cause pain on the left side of the belly. A parent can usually feel enlarged spleen in the belly of his or her child. Splenic sequestration crisis and aplastic crisis most commonly occur in infants and children who have Sickle cell disease. Adults who have Sickle cell disease may also experience episodes of severe anemia, but these usually have other causes. No matter the cause, severe anemia may lead to symptoms that include: fatigue Dizziness Pale skin Shortness of breath chlamydia Haemophilus influenza type B Meningococcus Mycoplasma pneumoniae Pneumococcus Salmonella Staphylococcus Acute Chest syndrome Sickling in blood vessels of lungs can deprive persons lungs of oxygen. When this happen, areas of lung tissue are damaged and cannot exchange oxygen properly. This condition is know as Acute Chest syndrome. In Acute Chest syndrome, at least one segment of the lung is damage. This condition is very serious and should be treated right away in hospital. Acute Chest syndrome often starts a few days after a painful crisis begin. Lung infection may accompany Acute Chest syndrome.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Exams and Tests

If one member of a couple has sickle cell disease or sickle cell trait, other member should be tested before becoming pregnant. This test requires a blood sample, which screens for presence of hemoglobin S, hemoglobin C, or beta-thalassemia. If one or both members of a couple carries hemoglobin S gene or another abnormal hemoglobin gene, couple may want to meet with genetic counselor before becoming pregnant to learn more about their chances of having a child with sickle cell disease. Your doctor can help you find genetic counselor to discuss genetic test. Pulmonary hypertension is a severe, common problem for people who have sickle cell disease. It can be detected early with an echocardiogram, painless method of measuring blood flow.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Complications

Babies with sickle cell disease may see a hematologist, doctor with special training in blood diseases such as sickle cell disease. For infants, first sickle cell disease visit should take place before 8 weeks of age. If someone was born in a country that does not perform newborn screening, he or she might be diagnosed with sickle cell disease later in childhood. These people should also be referred as soon as possible for special care. All people who have sickle cell disease should see their health care providers every three to 12 months, depending on the person's age. Your doctor or medical team can help to prevent problems by taking certain steps: educating families about disease and what to watch out for. Examining people Giving medicines and immunizations Performing tests to prevent infection in sickle cell disease, spleen does not work properly or at all. This problem makes people who have sickle cell disease more likely to get severe infections. Penicillin in Children who have sickle cell disease, Taking penicillin two times a day has been shown to reduce the chance of having severe infection caused by pneumococcus bacteria. Infants need to take liquid penicillin. Older children can take tablets. Many doctors will stop prescribing penicillin after a child has reached the age of 5. Some prefer to continue this antibiotic throughout life, particularly if person has hemoglobin SS or hemoglobin S0 thalassemia, since people who have sickle cell disease are still at risk. All people who have had surgical removal of spleen, called splenectomy, or past infection with pneumococcus should keep taking penicillin throughout life. Vaccines People who have sickle cell disease should receive all recommended childhood vaccines. They should also receive additional vaccines to prevent other infections. Influenza. All people who have sickle cell disease should receive influenza shot every year at the start of flu season. This vaccination should begin at 6 months of age. Only inactivated vaccine, which comes as a shot, should be used in people who have sickle cell disease. Meningococcus. Child who has sickle cell disease should receive meningococcal vaccine at 2 4 6, and 12-15 months of age. Child should receive booster vaccine three years after this series of shots, then every five years after that. Pneumococcus. Even though all children routinely receive vaccine against pneumococcus, your children's doctor may recommend a second kind of vaccine against pneumococcus. This second vaccine is given after 24 months of age and again five years later. Adults who have sickle cell disease who have not received any pneumococcal vaccine should get a dose of PCV13 vaccine. They should later receive PPSV23 if they have not already received it or if it has been more than five years since they do. Person should follow these guidelines even if he or she is still taking penicillin.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

Sources

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions.

* Please keep in mind that all text is machine-generated, we do not bear any responsibility, and you should always get advice from professionals before taking any actions

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