Materiały źródłowe

• #1 Sickle Cell Disease – Symptoms | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/sickle-cell-disease/symptoms

  Know when to seek emergency medical care. Sickle cell disease can lead to life-threatening health problems. If you think that you or someone else is having a serious symptom, seek medical care or call 9-1-1 right away. […] Symptoms of sickle cell disease vary from person to person and can change over time. How the disease affects your body affects the type of symptoms you have. […] Serious symptoms of sickle cell disease are emergencies and need treatment right away. Seek care or call 9-1-1 if you or someone else is experiencing: […] Severe pain. A serious pain event is sometimes called a pain crisis, sickle cell crisis, or vaso-occlusive crisis. Most people with sickle cell disease have this often-serious pain. It may occur in children as young as 1 year old. Serious pain linked with sickle cell disease often requires treatment in a clinic or hospital.

• #1 Sickle cell anemia – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

  Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include: […] Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body can’t get enough oxygen. This causes fatigue. […] Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay.

• #1 Symptoms of Sickle Cell Disease (Sickle Cell Anemia)

  https://www.webmd.com/a-to-z-guides/symptoms-of-sickle-cell-disease

  Sickle cell disease changes the shape of the oxygen-carrying molecules (called hemoglobin) found inside red blood cells. […] These events trigger the symptoms of sickle cell disease. […] Early signs in babies are: Extreme fussiness, Painful swelling of fingers and toes which is the definition of a condition called „dactylitis”, Yellow skin and whites of the eyes (also called jaundice or icterus). This is caused by the breakdown of hemogloblin released during the constant turnover of red blood cells. […] With sickle cell disease, you don’t have enough healthy red blood cells. This is a condition called anemia. Symptoms can include: Trouble breathing, Dizziness and lightheadedness, Fast heart rate, Fatigue, Irritability, Pale skin color, Delayed growth in infants, Delayed puberty in teens.

• #1 Sickle Cell Disease – Symptoms | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/sickle-cell-disease/symptoms

  Fatigue (extreme tiredness), shortness of breath, dizziness, and irregular heartbeat. These are symptoms of serious anemia. Life-threatening anemia can be caused by conditions such as a splenic sequestration crisis or an aplastic crisis. […] Fever. If you have a fever of more than 101.3 degrees Fahrenheit, or 38.5 degrees Celsius, seek attention from a healthcare provider and treatment with antibiotics right away. Some people need to be hospitalized for care. […] Chest pain, coughing, fever, and shortness of breath. These symptoms may be suggestive of acute chest syndrome requiring treatment in a hospital, where you may receive antibiotics, oxygen therapy, and/or a blood transfusion. […] Sudden weakness, numbness on one side of the body, confusion, or trouble speaking, seeing, or walking. Call 9-1-1 if you’re showing any of these warning signs of a stroke.

• #1 Sickle Cell Disease (SCD): Types, Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/12100-sickle-cell-disease

  Sickle cell disease symptoms begin to show when a child is about 5 to 6 months old. Signs and symptoms of SCD vary from person to person. Some people have mild symptoms, while others develop more serious complications. Sickle cell disease symptoms include: […] Pain is the most common complication of sickle cell disease. Sickled cells passing through blood vessels can get stuck and block blood flow, which causes pain. You may have an acute pain crisis, which is also called a sickle cell crisis, vaso-occlusive crisis (VOC) or vaso-occlusive episode (VOE). These pain crises may be mild or severe and can start suddenly and last for any length of time. Pain crises most often affect your chest, back, legs and arms. You might also have chronic pain, which is when pain lasts longer than six months.

• #1 Sickle Cell Disease (SCD) Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/205926-clinical

  Sickle cell disease (SCD) usually manifests early in childhood. For the first 6 months of life, infants are protected largely by elevated levels of fetal hemoglobin (HbF); soon thereafter, the condition becomes evident. […] The most common clinical manifestation of SCD is vaso-occlusive crisis, which occurs when the microcirculation is obstructed by sickled red blood cells (RBCs), causing ischemic injury to the organ supplied and resultant pain. Pain crises constitute the most distinguishing clinical feature of SCD and are the leading cause of emergency department visits and hospitalizations in this population. […] Approximately half the individuals with homozygous hemoglobin S (HbS) disease experience vaso-occlusive crisis. The frequency of crisis is extremely variable. Some individuals have as many as 6 or more episodes annually, whereas others may have episodes only at great intervals or have none at all. Each individual typically has a consistent pattern for crisis frequency.

• #1 Sickle Cell Disease in Children – Stanford Medicine Children’s Health

  https://deprod.stanfordchildrens.org/en/topic/default?id=sickle-cell-disease-in-children-90-P02327

  Most children with SCD will start showing symptoms during the first year, often around 5 months. […] Symptoms can include: Anemia. This is the most common symptom. Having fewer red blood cells causes anemia. Anemia can make a child pale and tired. […] Yellowing of the skin, eyes, and mouth (jaundice). This is a common symptom. Sickle cells don’t live as long as normal red blood cells. They die faster than the liver can filter them out. The yellow color is caused by a substance (bilirubin) that is released when the red blood cells die. […] Pain crisis, or sickle crisis. When sickle cells move through small blood vessels, they can get stuck. This blocks blood flow and causes pain. This sudden pain can happen anywhere, but most often occurs in the chest, arms, and legs. […] Acute chest syndrome. This is when sickle cells stick together and block oxygen flow in the tiny vessels in the lungs. This can be deadly.

• #1 Sickle Cell Disease Symptoms & Complications | Vaso-Occlusive Events and Pain Crisis

  https://www.sparksicklecellchange.com/what-is-sickle-cell/symptoms-complications

  The underlying damage caused by acute complications can lead to more serious complications. Even when you are not in pain, ongoing blockages, damage to the blood vessels, and the breaking down of sickled red blood cells over time can lead to progressive and chronic complications including organ damage or even failure. […] Chronic pain is common among people with sickle cell and generally intensifies with age. […] Anemia occurs when sickled red blood cells break apart and die faster than the body can replace them, reducing the amount of oxygen throughout the body. This shortage of red blood cells can also cause fatigue. […] Repeated blockages and long-term damage from sickled red blood cells eventually cause an organ to completely stop working. This occurs in 59% of adults with sickle cell due to chronic complications and can affect multiple organs or systems in the body.

• #1 Sickle cell anemia – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

  Some people with sickle cell anemia also have chronic pain from bone and joint damage, ulcers, and other causes. […] Swelling of hands and feet. Sickle-shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell. […] Frequent infections. The spleen is important for protecting against infections. Sickle cells can damage the spleen, raising the risk of developing infections. Babies and children with sickle cell anemia commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia. […] Delayed growth or puberty. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in babies and children and delay puberty in teenagers. […] Vision problems. Tiny blood vessels that supply blood to the eyes can become plugged with sickle cells. This can damage the portion of the eye that processes visual images, called the retina, and lead to vision problems.

• #1 Sickle cell disease | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/blood-and-lymph/sickle-cell-disease/

  Sickle cell disease leaves people especially children extra vulnerable to infection because their bodies arent as effective at dealing with bacteria and viruses. This leaves them more vulnerable to viruses like the common cold, as well as severe and potentially life-threatening conditions like meningitis. […] Almost everyone with sickle cell disease has anaemia. Haemoglobin is found in red blood cells its the substance that transports oxygen around the body. People with anaemia have low levels of haemoglobin in their blood. […] Theres a wide range of other issues that can be caused by sickle cell disease. These include: a serious lung condition called acute chest syndrome that can cause chest pain, a cough, breathing difficulties and fever, pain in bones and joints, delayed puberty, delayed growth in childhood, kidney or urinary problems, including bedwetting and blood in the urine, gallstones these are stones in the gallbladder that can cause jaundice (yellow eyes and skin) and abdominal (tummy) pain, ulcers on the lower legs (open sores that can be very painful), priapism a painful, persistent erection that can last for several hours, high blood pressure (pulmonary hypertension), transient ischaemic attacks (TIAs) or strokes these happen when blood flow to the brain is interrupted or blocked, enlarged spleen this can cause a fast heartbeat, worsening of anaemia, a swollen tummy, shortness of breath and abdominal pain, vision problems like floaters, worsened night vision, blurred vision, patchy vision and, occasionally, sudden vision loss.

• #1 Sickle Cell Disease – Blood Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/blood-disorders/anemia/sickle-cell-disease

  Acute chest syndrome can occur in people of all ages, but it is most common among children. It is usually characterized by severe pain and difficulty breathing. Acute chest syndrome can be fatal. […] In children, acute sequestration of sickled cells in the spleen (sequestration crisis) may occur causing an enlarged spleen and worsening anemia. […] Most people who have sickle cell disease develop an enlarged spleen during childhood because sickled cells become trapped in the spleen. […] Because the spleen helps fight infection, people with sickle cell disease are more likely to develop pneumococcal pneumonia and other infections. […] Poor circulation to the skin may cause sores on the legs, especially at the ankles. […] Blocked blood vessels may cause strokes that damage the nervous system. […] In older adults, lung and kidney function may deteriorate.

• #1 Sickle Cell Disease (SCD): Types, Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/12100-sickle-cell-disease

  Red blood cells die early with sickle cell disease, which can lead to anemia. Anemia occurs when you dont have enough healthy red blood cells to carry oxygen throughout your body. Anemia can cause severe fatigue, along with jaundice, irritability, dizziness and lightheadedness. […] Acute chest syndrome is a life-threatening medical emergency. It can cause lung injury, difficulty breathing and low oxygen to the rest of your body. This complication of SCD occurs when sickled cells block blood and oxygen from reaching your lungs. […] When sickled cells get stuck in a blood vessel, it blocks blood flow to your brain. This makes it harder for your brain to get the oxygen it needs to work correctly. This can lead to a stroke. About 10% of people with SCD will have a symptomatic (clinical) stroke. Strokes are most common in people with sickle cell anemia. […] People with sickle cell disease are at risk for problems related to their heart, lung, kidneys and other organs. This is because blood and oxygen arent reaching them. SCD can lead to multi-organ failure.

• #1

  https://www.beaumont.org/conditions/symptoms-of-sickle-cell-anemia

  Acute chest syndrome. This occurs when sickling is in the lungs. This can be a life-threatening complication of sickle cell anemia. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough. Multiple episodes of acute chest syndrome can cause permanent lung damage. Damage to the blood vessels in the lungs can lead to high blood pressures in the lungs (pulmonary hypertension), a condition that can cause death in adults with sickle cell anemia. […] Splenic sequestration (pooling). This complication is a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life-threatening if not treated promptly. The spleen can also become enlarged and painful from the increase in blood volume. After repeated episodes of splenic sequestration, the spleen becomes scarred, and permanently damaged. By age 8, most children do not have a functioning spleen either from damage related to the sickled cells, surgical removal, or from repeated episodes of splenic sequestration. The risk of infection is a major concern of children without a functioning spleen. Infection is the major cause of death in children younger than age 5 in this population.

• #1 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  In children older than 2 years, the most common initial presentation is a painful episode of a generalized or variable nature, while a slightly less common presentation involves acute chest pain. […] The underlying cause is sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischaemia, pain, necrosis, and often organ damage. […] Symptoms include pain on the left side, swollen spleen (which can be detected by palpation), fatigue, dizziness, irritability, rapid heartbeat, or pale skin. […] Acute chest syndrome is caused by a VOC which affects the lungs, possibly triggered by infection or by emboli which have circulated from other organs. Symptoms include wheezing, chest pain, fever, pulmonary infiltrate (visible on x-ray), and hypoxemia.

• #1 Signs and Symptoms of Sickle Cell Disease | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/signs-and-symptoms-sickle-cell-disease

  If a person has sickle cell disease (SCD), it is present at birth. But most infants do not have any problems from the disease until they are about 5 or 6 months of age. Some children with SCD will start to have problems early on, and some later. Early symptoms of SCD may include: Painful swelling of the hands and feet, known as dactylitis, Fatigue or fussiness from anemia, A yellowish color of the skin, known as jaundice, or whites of the eyes, known as icteris, that occurs when a large number of red cells hemolyze. The signs and symptoms of SCD will vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease. Pain episodes (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 the pain often occurs in the lower back, legs, arms, abdomen, and chest. Many adolescents and adults with SCD suffer from chronic pain. This kind of pain has been hard for people to describe, but it is usually different from crisis pain or the pain that results from organ damage. People with SCD usually have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life threatening. Severe anemia may lead to symptoms that include: Shortness of breath, Being very tired, Feeling dizzy, Having pale skin. The spleen is important for protection against certain kinds of germs. Sickle cells can damage the spleen and weaken or destroy its function early in life. People with SCD who have damaged spleens are at risk for serious bacterial infections that can be life-threatening. Symptoms may include: Chest pain, Fever, Shortness of breath, Rapid breathing, Cough. A stroke occurs when blood flow is blocked to a part of the brain. When this happens, brain cells can be damaged or can die. The symptoms depend upon what part of the brain is affected. Symptoms of stroke may include: Weakness of an arm or leg on one side of the body, Trouble speaking, walking, or understanding, Loss of balance, Severe headache. Brain imaging and tests of thinking (cognitive studies) have shown that children and adults with hemoglobin SS and hemoglobin S0 thalassemia often have signs of silent brain injury, also called silent stroke. Silent brain injury can lead to learning problems or trouble making decisions or holding down a job. Sickle cell disease can injure blood vessels in the eye. The most common site of damage is the retina, where blood vessels can overgrow, get blocked, or bleed. Detachment of the retina can occur. These problems can cause visual impairment or loss. People with SCD can have problems with blood vessels in the heart and with heart function. The heart can become enlarged. In adolescents and adults, injury to blood vessels in the lungs can make it hard for the heart to pump blood through them. This causes the pressure in lung blood vessels to rise. High pressure in these blood vessels is called pulmonary hypertension. Symptoms may include shortness of breath and fatigue. The kidneys are sensitive to the effects of red blood cell sickling. SCD causes the kidneys to have trouble making the urine as concentrated as it should be. This may lead to a need to urinate often and to have bedwetting or uncontrolled urination during the night (nocturnal enuresis). Sickle cell ulcers are sores that usually start small and then get larger and larger. The number of ulcers can vary from one to many. Some ulcers will heal quickly, but others may not heal and may last for long periods of time. Some ulcers come back after healing. Children with SCD may grow and develop more slowly than their peers because of anemia. Pregnancies in women with SCD can be risky for both the mother and the baby. As in other chronic diseases, people with SCD may feel sad and frustrated at times. The limitations that SCD can impose on a person’s daily activities may cause them to feel isolated from others. Sometimes they become depressed.

• #1 Sickle Cell Disease (SCD): Symptoms & Causes | NewYork-Presbyterian

  https://www.nyp.org/pediatrics/sickle-cell-disease

  Complications of sickle cell disease include: […] Acute chest syndrome (ACS) – A life-threatening condition that occurs when sickle cells block blood and essential oxygen from reaching the lungs, causing low-oxygen rates, breathing difficulties, and injury […] Stroke – When sickle cells get stuck in blood vessels and block blood flow to the brain. Stroke symptoms may include seizures, numbness/weakness in the legs and arms, speech difficulties, and loss of consciousness, and may lead to death. […] Pulmonary hypertension – More common in adults, SCD may cause high blood pressure in the lungs, which may result in extreme fatigue and life-threatening breathing difficulties […] Splenic sequestration – Sickle cells may become trapped in the spleen. This can lead to an enlarged spleen and pain in the belly on the left side of the body.

• #1 Understanding Sickle Cell Symptoms | Sickle Cell Speaks

  https://www.sicklecellspeaks.com/understanding-sickle-cell/symptoms

  People with sickle cell, especially infants and children, are more likely to get infections. […] When sickled cells get stuck in the blood vessels, it blocks blood flow to the arms and legs. That causes swelling in the hands and feet. […] Sickle cell can affect the blood vessels in the eyes. That can lead to long-term damage, including loss of vision. […] Sickle cell ulcers are sores that usually start small and then get bigger. […] Symptoms include pain and problems with walking and joint movement. […] Because of their anemia, children with sickle cell may grow and develop at a slower pace than other children, and they may take longer to reach puberty.

• #1 Sickle Cell Anemia: Types, Symptoms, and Treatment

  https://www.healthline.com/health/sickle-cell-anemia

  Symptoms of sickle cell anemia usually show up at a young age. They may appear in babies as early as 4 months old, but generally occur around the 6-month mark. […] While there are multiple types of SCD, they all have similar symptoms, which vary in severity. These include: excessive fatigue or irritability, from anemia; fussiness, in babies; bedwetting, from associated kidney problems; jaundice, which is yellowing of the eyes and skin; swelling and pain in hands and feet; frequent infections; pain in the chest, back, arms, or legs. […] Delayed growth often occurs in people with SCD. Children are generally shorter but regain their height by adulthood. Sexual maturation may also be delayed. This happens because sickle cell RBCs can’t supply enough oxygen and nutrients. […] The prognosis of the disease varies. Some patients have frequent and painful sickle cell crises. Others only rarely have attacks.

• #1 Symptoms of Sickle Cell AnemiaEnvelope icon

  https://sicklecellanemianews.com/symptoms-sickle-cell-anemia/

  Low red blood cell numbers cause tissues to become deprived of oxygen and nutrients. This may delay growth and development in children, as well as delay puberty in adolescents. Adults with sickle cell disease are usually shorter and thinner than the general population for the same reason. […] Sickle-shaped red blood cells can obstruct vessels that supply blood and oxygen to the eyes. This can damage the retina — the region at the back of the eye that enables one to see — and cause vision problems. These include floaters, blurred vision, reduced vision at night, and possibly vision loss. […] Other symptoms and complications of sickle cell disease may include headaches, dizziness, jaundice (yellow tint in the skin or whites of eyes), gallstones, liver and kidney problems. Patients with sickle cell disease are also at a higher risk of having strokes, developing leg ulcers (painful, open sores) that do not heal, and acute chest syndrome — a serious lung condition that causes fever, chest pain, and breathing difficulties.

• #1 Sickle Cell Disease – Symptoms | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/sickle-cell-disease/symptoms

  Priapism. Having an erection that lasts for 4 hours or more is an emergency. You will need to go to the hospital and see a hematologist (a doctor who specializes in blood conditions and diseases) and a urologist (a doctor who specializes in treating conditions of the reproductive and urinary systems). Priapism can lead to erectile dysfunction, so it is important to get treatment as soon as possible.

• #1 Disease progression in sickle cell disease

  https://ourironwill.com/progression/scd

  Sickle cell disease can cause progressive organ damage. […] Sickle cell disease is a progressive disease that can result in multiple organ damage over time starting in childhood. […] Younger people are at risk of progressive organ damage by the time they reach their early adult years. […] Within the first 10 years of shifting to adult care, there is more rapid worsening in organ damage with a death rate of up to 20%. […] 50% of people have irreversible organ damage affecting at least one organ by the age of 50. […] Iron overload from multiple blood transfusions can add additional stress and damage to the vital organs. The goal for people living with sickle cell disease is to get and stay ahead of progressive organ damage. Work with your healthcare team to make sure that your organ function is being monitored early in childhood and regularly in adulthood according to guidelines.

• #1 SEVERE SICKLE CELL DISEASE – PATHOPHYSIOLOGY AND THERAPY

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2832723/

  Specific complications include pulmonary hypertension, osteonecrosis, chronic renal disease, and cognitive dysfunction. […] Many such patients seem to do well during childhood, but are vulnerable to developing irreversible organ damage due to chronic hemolytic anemia and/or vascular occlusion in young adulthood. […] Improved therapy has dramatically changed the prognosis of sickle cell disease. Once a fatal pediatric illness, it is now a chronic adult disease characterized by poor quality of life with end organ failure and acute intermittent medical emergencies. […] Annual screening with transcranial doppler enables selective chronic transfusions to be implemented that successfully prevent CNS injury. […] The outcomes of children with sickle cell disease have improved dramatically as described above due to better comprehensive care, pneumococcal prophylaxis, in addition to transfusion therapy and hydroxyurea. Currently, greater than 90% of newborns with sickle cell disease can expect to live beyond their 20th birthday. However, this optimistic outlook begins to unravel soon after. There is a rapid progression in organ damage and morbidity with a mortality rate of 5.820% in the first 10 years after transition to adult care. […] By the fifth decade of life 50% of the patients have irreversible organ damage of at least one organ. These complications are the cause for premature mortality with a mean age of death merely 38 years.

• #1 SEVERE SICKLE CELL DISEASE – PATHOPHYSIOLOGY AND THERAPY

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2832723/

  Patients with sickle cell disease have an abnormal hemoglobin that polymerizes under physiologic conditions, leading to the formation of distorted and rigid red blood cells. This in turn causes hemolysis and obstruction of blood flow in the microcirculation, with resultant tissue ischemia and necrosis. Pain and organ injury are the sequelae. […] The most common organ-related complications that characterize sickle cell disease as a severe clinical entity include vaso-occlusive or pain crisis, acute chest syndrome, stroke, and priapism. […] Although the acute events described above cause much morbidity during childhood as well as in adults, the toll of chronic sickling and vascular injury as well as ongoing hemolysis also promotes insidious, silent, clinically inapparent, but progressive organ damage. Such injury involves the lungs, heart, brain, kidneys, bones, and other organs.

• #1 Sickle Cell Disease (SCD) Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/205926-clinical

  In young children, acute chest syndrome consists of chest pain, fever, cough, tachypnea, leukocytosis, and pulmonary infiltrates in the upper lobes. […] 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 neurologic deficit. […] The heart is involved due to chronic anemia and microinfarcts. […] Cholelithiasis is common in children with SCD, as chronic hemolysis with hyperbilirubinemia is associated with the formation of bile stones. […] The kidneys lose concentrating capacity. Isosthenuria results in a large loss of water, further contributing to dehydration in these patients. […] Priapism, defined as a sustained, painful, and unwanted erection, is a well-recognized complication of SCD.

• #1 Sickle Cell Disease (SCD) Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/205926-clinical

  Vascular occlusion can result in avascular necrosis (AVN) of the femoral or humeral head and subsequent infarction and collapse at either site. […] Pulmonary hypertension is a serious complication of SCD, with an incidence as high as 31.8%, and the frequency increases with patient age. […] Dyspnea on exertion and fatigue are the most common initial symptoms of pulmonary hypertension. As pulmonary hypertension progresses, patients may develop peripheral edema and exertional chest pain and syncope, due to right ventricular failure.

• #1 Sickle cell disease: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/sickle-cell-disease/

  Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of signs and symptoms varies from person to person. Some people have mild health issues, while others are frequently hospitalized for more serious complications. […] The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the skin and whites of the eyes (jaundice). Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs, such as the lungs, kidneys, spleen, and brain, of oxygen-rich blood and can lead to organ damage. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension), which can lead to heart failure. Pulmonary hypertension occurs in about 10 percent of adults with sickle cell disease. […] Without treatment, individuals with sickle cell disease often have lifelong health problems.

• #1 Sickle Cell Disease (Anemia): Symptoms, Treatment, and Life Expectancy

  https://www.medicinenet.com/sickle_cell/article.htm

  Symptoms in adults typically are intermittent pain episodes due to injury of bone, muscle, or internal organs. […] Infants with sickle cell anemia do not develop symptoms in the first few months of life because the hemoglobin produced by the developing fetus (fetal hemoglobin) protects the red blood cells from sickling. This fetal hemoglobin is absent in the red blood cells that are produced after birth so that by 5 months of age, the sickling of the red blood cells is prominent and symptoms begin. […] Adolescents (preteens and teens) and young adults more commonly develop the following symptoms: Leg ulcers, Aseptic necrosis, Eye damage. […] Pain crises in persons with sickle cell anemia are intermittent painful episodes that are the result of inadequate blood supply to body tissues. The impaired circulation is caused by the blockage of various blood vessels from the sickling of red blood cells. The sickled red blood cells slow or completely impede the normal flow of blood through the tissues. This leads to excruciating pain, often requiring hospitalization and opiate medication for relief. The pain typically is throbbing and can change its location from one body area to another. Bones are frequently affected. Pain in the abdomen with tenderness is common and can mimic appendicitis. Fever frequently is associated with the pain crises.

• #1 Understanding Sickle Cell Symptoms | Sickle Cell Speaks

  https://www.sicklecellspeaks.com/understanding-sickle-cell/symptoms

  Sickle cell is present from the start of life you are born with it. Infants usually dont have symptoms in the first few months of life because the fetal hemoglobin produced before birth protects the red blood cells from sickling. A baby may start to have symptoms by six months of age. If you notice any symptoms, be sure to discuss them with your doctor. […] Most of the symptoms of sickle cell are related to complications of the disease. Some, such as fatigue, anemia, pain episodes and bone problems, can occur at any age. […] Teens and young adults may develop leg ulcers, avascular necrosis (death of bone tissue due to lack of blood supply), acute chest syndrome, and eye damage. […] Adults generally have pain crises caused by an injury to a bone, muscle or internal organs. […] Some common symptoms you might have to manage with your sickle cell include:

• #1

  https://www.nhs.uk/conditions/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 for days or weeks, an increased risk of serious infections, anaemia (where red blood cells cannot carry enough oxygen around the body), which can cause tiredness and shortness of breath. […] Some people also experience other problems, such as delayed growth, strokes and lung problems. […] Sickle cell disease is a serious and lifelong health condition, although treatment can help manage many of the symptoms. […] 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.

• #1 Sickle Cell Disease Symptoms & Complications | Vaso-Occlusive Events and Pain Crisis

  https://www.sparksicklecellchange.com/what-is-sickle-cell/symptoms-complications

  Sickle cell is a progressive, lifelong illness that has long-term complications and affects everyone differently. Its symptoms are unpredictable and can be severe, varying from person to person, as well as for an individual over time. […] Pain crises, also known as a sickle cell crisis, are unpredictable and extremely painful, lasting anywhere from a few hours to a few weeks, and are the most common reason for hospitalization. Frequency, duration, and intensity of pain crises can vary with age. […] Although sickle cell is present at birth, most newborns don’t experience problems until they are ~5 months old. […] It’s important to note that although a person may not be experiencing symptoms, the silent damage of sickle cell to your body and organs is progressive, and frequency of symptoms may increase with age.

• #1 Symptoms of Sickle Cell Disease (Sickle Cell Anemia)

  https://www.webmd.com/a-to-z-guides/symptoms-of-sickle-cell-disease

  Pain is an unpredictable symptom of sickle cell disease. The deformed blood cells can get stuck in and block your blood vessels. When this happens, oxygen-rich blood cannot reach key organs and tissues. The result is a sudden pain attack, called a pain crisis. […] A pain crisis can last anywhere from hours to weeks. If its severe, youll need to seek emergency care at a hospital. The number of pain attacks varies from person to person. Some people have them often. Others have only a few every now and then. Most kids are pain-free between attacks. But many teens and adults have long-term pain. […] Some types of the disorder, like sickle cell anemia (also called hemoglobin SS), cause more severe symptoms than other types. Some people have only mild symptoms while others may have dramatic flares that require medical care. And your experience may change.

• #1 Sickle Cell Trait: Symptoms, Diagnosis, Treatment And More

  https://www.healthline.com/health/sickle-cell-trait

  Sickle cell disease (SCD) is a group of inherited blood disorders. People with SCD have two copies of a gene that affects hemoglobin, an essential component of red blood cells. […] SCD affects the form and function of your red blood cells. People with SCD have hemoglobin, the oxygen-carrying component of red blood cells, that does not function properly. […] These red blood cells cant live as long as healthy red blood cells and can block your blood vessels. This causes serious health problems such as: anemia, pain, lung problems, delayed growth, increased risk of developing infections, stroke. […] Most people with SCT dont have any symptoms. But in certain stressful conditions, healthy red blood cells in people with SCT can become sickle-shaped, causing symptoms linked to SCD. […] Some of the severe symptoms and complications linked to SCT include: hematuria (blood in urine), chronic kidney disease, fetal loss and other pregnancy complications, muscle breakdown, severe pain, renal papillary necrosis (a serious kidney disorder), splenic infarction (blocked blood flow to the spleen causing tissue death), sudden death due to exertion.

• #1 Sickle Cell Trait – Hematology.org

  https://www.hematology.org/education/patients/anemia/sickle-cell-trait

  Most people with sickle cell trait have no symptoms and will not have any health complications. […] Occasionally people with sickle cell trait can have blood in their urine. Under extreme conditions such as high altitude, severe dehydration, or very high intensity physical activity, red cells can become deformed or sickled. Complications include muscle breakdown (rhabdomyolysis), reduced blood supply to the spleen (ischemia/infarction), or increased pressure in the eye (glaucoma) following eye injuries. Finally, a very rare form of kidney cancer (renal medullary carcinoma) has been associated with sickle cell trait.

• #1 Sickle cell anemia – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882

  Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Treatments might include medicines and blood transfusions. For some children and teenagers, a stem cell transplant might cure the disease. Gene therapies also are being developed that may offer cures for people with sickle cell disease. […] Hydroxyurea reduces the frequency of pain crises and might reduce the need for blood transfusions and hospital stays. […] Children with sickle cell anemia might receive penicillin from about 2 months old to 5 years old, or longer. This medicine can help prevent infections, such as pneumonia, which can be life-threatening to children with sickle cell anemia. […] Red blood cell transfusions are used to treat and prevent complications, such as stroke, in people with sickle cell disease. […] A stem cell transplant can cure sickle cell anemia. […] People who are successfully treated with gene editing therapy no longer have symptoms of sickle cell disease.

• #1

  https://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/sickle-cell-disease-in-children.aspx

  Sickle cell disease may cause damage to the spleen, kidneys, lungs and brain. Once damaged by sickled cells the spleen may not be able to filter bacteria from the blood as well as in other children. […] As a result, infants and children with sickle cell disease have a compromised immune system. This means they are more likely to have certain infections which may be fatal. […] Sickle cell disease is a chronic disease, meaning it does not go away. Early diagnosis and starting antibiotics shortly after birth for those with severe forms of sickle cell have greatly improved childhood survival. […] The U.S. Food Drug Administration (FDA) has approved medications that can help prevent sickle cell complications in children. […] Transfusions are often required by children with sickle cell disease either urgently for treatment of an acute complication or they may be given on a monthly schedule to prevent future complications. […] This treatment can cure sickle cell disease. […] The Food and Drug Administration (FDA) approved new therapies (exa-cel and lovo-cel) to treat sickle cell disease in people age 12 years and older especially those who often have serious problems from sickle cell.

• #2 Sickle Cell Disease (SCD): Types, Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/12100-sickle-cell-disease

  Sickle cell disease symptoms begin to show when a child is about 5 to 6 months old. Signs and symptoms of SCD vary from person to person. Some people have mild symptoms, while others develop more serious complications. Sickle cell disease symptoms include: […] Pain is the most common complication of sickle cell disease. Sickled cells passing through blood vessels can get stuck and block blood flow, which causes pain. You may have an acute pain crisis, which is also called a sickle cell crisis, vaso-occlusive crisis (VOC) or vaso-occlusive episode (VOE). These pain crises may be mild or severe and can start suddenly and last for any length of time. Pain crises most often affect your chest, back, legs and arms. You might also have chronic pain, which is when pain lasts longer than six months.

• #2 Sickle Cell Disease (SCD): Types, Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/12100-sickle-cell-disease

  Red blood cells die early with sickle cell disease, which can lead to anemia. Anemia occurs when you dont have enough healthy red blood cells to carry oxygen throughout your body. Anemia can cause severe fatigue, along with jaundice, irritability, dizziness and lightheadedness. […] Acute chest syndrome is a life-threatening medical emergency. It can cause lung injury, difficulty breathing and low oxygen to the rest of your body. This complication of SCD occurs when sickled cells block blood and oxygen from reaching your lungs. […] When sickled cells get stuck in a blood vessel, it blocks blood flow to your brain. This makes it harder for your brain to get the oxygen it needs to work correctly. This can lead to a stroke. About 10% of people with SCD will have a symptomatic (clinical) stroke. Strokes are most common in people with sickle cell anemia. […] People with sickle cell disease are at risk for problems related to their heart, lung, kidneys and other organs. This is because blood and oxygen arent reaching them. SCD can lead to multi-organ failure.

• #2 Sickle Cell Anemia: A Parent’s Guide for the School Age Child | Texas DSHS

  https://www.dshs.texas.gov/newborn-screening-program/sickle-cell-disease/more-about-sickle-cell/parents-guide

  Anemia exists when the number of red blood cells in the circulation is below normal. Sometimes anemia is referred to as a low blood count. Anemia is common in all individuals with sickle cell disease. Even though a child’s usual blood count is quite low, this causes few if any real problems. The blood count in a child with sickle cell anemia can drop suddenly. When a child who is already anemic has a drop in the blood count, this is potentially serious. The symptoms of a very low blood count are headache, irritability, unusual sleepiness, lethargy, rapid heartbeat, and pale color. If the lips and fingernails have no pink color even when the hands are warm, the child is pale. A child can have a seriously low blood count without many symptoms. Sometimes the only sign may be that the child is less active or sleeping all the time. If a child has symptoms of a low blood count a doctor should be consulted immediately. In most cases, the blood count comes up on its own and no treatment needed. Sometimes a blood transfusion is necessary.

• #2 Symptoms of Sickle Cell Anemia

  https://www.everydayhealth.com/sickle-cell-anemia/guide/symptoms/

  Sickle cell anemia causes hemolytic anemia, which occurs when the body destroys sickled red blood cells. […] Symptoms of hemolytic anemia can include: A racing or irregular heartbeat, A yellow tint to the skin and whites of the eyes, known as jaundice, Cold hands and feet, Confusion, Urine that’s darker than usual, Difficulty concentrating, Headache, Insomnia, Leg cramps, Pale skin, Shortness of breath, particularly with exercise, Weakness or decreased stamina, Fatigue. […] Emergency symptoms of severe anemia include the following: Severe weakness, Breathlessness, Extremely rapid heart rate. […] Sickle cell anemia is the most severe type of sickle cell disease, causing anemia symptoms, pain crises, jaundice, and swelling of the hands and feet. […] Some symptoms may resemble those of other types of hemolytic anemia, and the complications can affect many organs, vision, breathing, and sexual function.

• #2 Sickle cell anemia – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

  Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include: […] Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body can’t get enough oxygen. This causes fatigue. […] Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay.

• #2 Sickle Cell Crisis: Symptoms, Causes, Treatment, Prevention

  https://www.webmd.com/a-to-z-guides/sickle-cell-crisis

  The most common sign is pain that might be dull, stabbing, throbbing, or sharp, and seems to come out of nowhere. How severe it is and how long it lasts varies with different people and different crises. Some people have crises here and there, while others may have them every month. […] You might feel the pain anywhere in your body and in more than one place, but its often in your: Arms and legs, Belly, Chest, Hands and feet (more typical in young children), Lower back. […] You may also have: Breathing problems (shortness of breath or pain when breathing or both), Extreme tiredness, Headache or dizziness, Painful erections, Weakness or a hard time moving some parts of your body, Yellowish skin color (jaundice). […] The stuck cells slow or even totally block blood flow, so some parts of your body dont get the oxygen they need. That can cause intense pain that lasts anywhere from a few hours to a few weeks.

• #2 Sickle Cell Disease Symptoms & Complications | Vaso-Occlusive Events and Pain Crisis

  https://www.sparksicklecellchange.com/what-is-sickle-cell/symptoms-complications

  Sickle cell is a progressive, lifelong illness that has long-term complications and affects everyone differently. Its symptoms are unpredictable and can be severe, varying from person to person, as well as for an individual over time. […] Pain crises, also known as a sickle cell crisis, are unpredictable and extremely painful, lasting anywhere from a few hours to a few weeks, and are the most common reason for hospitalization. Frequency, duration, and intensity of pain crises can vary with age. […] Although sickle cell is present at birth, most newborns don’t experience problems until they are ~5 months old. […] It’s important to note that although a person may not be experiencing symptoms, the silent damage of sickle cell to your body and organs is progressive, and frequency of symptoms may increase with age.

• #2 Sickle Cell Disease (SCD) Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/205926-clinical

  Aplastic crisis is a serious complication caused by infection with parvovirus B19 (B19V). […] Splenic sequestration occurs with highest frequency during the first 5 years of life in children with HbSS, but can occur at any age in individuals with other sickle syndromes. […] As HbS replaces HbF in the early months of life, problems associated with sickling and RBC membrane damage begin. The resulting rigid cells progressively obstruct and damage the spleen, which leads to functional asplenia. This, along with other abnormalities, results in extreme susceptibility to infection. […] During childhood and adolescence, SCD is associated with growth retardation, delayed sexual maturation, and being underweight. […] Infants with SCD may develop hand-foot syndrome, a dactylitis presenting as exquisite pain and soft tissue swelling of the dorsum of the hands and feet.

• #2 Adult Sickle Cell Disease Symptoms & Treatment | UPMC – Pittsburgh PA

  https://www.upmc.com/services/sickle-cell/conditions/sickle-cell-disease

  Although the pain is most often acute lasting for a relatively short time, such as hours to several days some people experience chronic pain. Chronic pain can endure for weeks or months. […] Repeated bouts of sickle cell crises can permanently damage organs. This damage occurs more frequently in adults than in children. […] Some adults with sickle cell disease may develop complications and experience associated symptoms. […] Common symptoms include: Labored breathing, Tachycardia (rapid heartbeat), Fever, Changes in mental status (i.e. confusion).

• #2 Sickle Cell Anemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia

  Sickle cell anemia is a severe form of the inherited blood disorder, sickle cell disease. Symptoms are fatigue, infections and painful tissue damage. […] Common symptoms include: Fatigue from anemia: Babies with sickle cell anemia may seem unusually fussy and irritable. Frequent infections: The condition affects your spleen, which weakens your immune system so you’re more likely to develop infections. Pain: Sickle cell anemia causes tissue damage because your tissues don’t get enough oxygen. Tissue damage hurts, so you may have pain in your arms, legs, chest and back. It may start as an ache that gets worse or come on suddenly, causing excruciating pain. Painful swelling in hands and feet: This is one of the first symptoms of sickle cell anemia in babies. Yellow-colored eyes and skin from jaundice: Your liver filters red blood cells. In sickle cell anemia, fast-dying sickled cells release bilirubin that builds up in your system to cause jaundice.

• #2 Signs and Symptoms of Sickle Cell Disease | Hematology-Oncology Associates of CNY

  https://www.hoacny.com/signs-and-symptoms-sickle-cell-disease

  If a person has sickle cell disease (SCD), it is present at birth. But most infants do not have any problems from the disease until they are about 5 or 6 months of age. Some children with SCD will start to have problems early on, and some later. Early symptoms of SCD may include: Painful swelling of the hands and feet, known as dactylitis, Fatigue or fussiness from anemia, A yellowish color of the skin, known as jaundice, or whites of the eyes, known as icteris, that occurs when a large number of red cells hemolyze. The signs and symptoms of SCD will vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease. Pain episodes (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 the pain often occurs in the lower back, legs, arms, abdomen, and chest. Many adolescents and adults with SCD suffer from chronic pain. This kind of pain has been hard for people to describe, but it is usually different from crisis pain or the pain that results from organ damage. People with SCD usually have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life threatening. Severe anemia may lead to symptoms that include: Shortness of breath, Being very tired, Feeling dizzy, Having pale skin. The spleen is important for protection against certain kinds of germs. Sickle cells can damage the spleen and weaken or destroy its function early in life. People with SCD who have damaged spleens are at risk for serious bacterial infections that can be life-threatening. Symptoms may include: Chest pain, Fever, Shortness of breath, Rapid breathing, Cough. A stroke occurs when blood flow is blocked to a part of the brain. When this happens, brain cells can be damaged or can die. The symptoms depend upon what part of the brain is affected. Symptoms of stroke may include: Weakness of an arm or leg on one side of the body, Trouble speaking, walking, or understanding, Loss of balance, Severe headache. Brain imaging and tests of thinking (cognitive studies) have shown that children and adults with hemoglobin SS and hemoglobin S0 thalassemia often have signs of silent brain injury, also called silent stroke. Silent brain injury can lead to learning problems or trouble making decisions or holding down a job. Sickle cell disease can injure blood vessels in the eye. The most common site of damage is the retina, where blood vessels can overgrow, get blocked, or bleed. Detachment of the retina can occur. These problems can cause visual impairment or loss. People with SCD can have problems with blood vessels in the heart and with heart function. The heart can become enlarged. In adolescents and adults, injury to blood vessels in the lungs can make it hard for the heart to pump blood through them. This causes the pressure in lung blood vessels to rise. High pressure in these blood vessels is called pulmonary hypertension. Symptoms may include shortness of breath and fatigue. The kidneys are sensitive to the effects of red blood cell sickling. SCD causes the kidneys to have trouble making the urine as concentrated as it should be. This may lead to a need to urinate often and to have bedwetting or uncontrolled urination during the night (nocturnal enuresis). Sickle cell ulcers are sores that usually start small and then get larger and larger. The number of ulcers can vary from one to many. Some ulcers will heal quickly, but others may not heal and may last for long periods of time. Some ulcers come back after healing. Children with SCD may grow and develop more slowly than their peers because of anemia. Pregnancies in women with SCD can be risky for both the mother and the baby. As in other chronic diseases, people with SCD may feel sad and frustrated at times. The limitations that SCD can impose on a person’s daily activities may cause them to feel isolated from others. Sometimes they become depressed.

• #2 Sickle Cell Anemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia

  Sickle cell anemia symptoms typically start when babies are 6 to 9 months old. Symptoms change over time as your body makes more abnormal (sickled) cells. […] Sickle cell anemia can cause serious and sometimes life-threatening complications. For example, people with sickle cell anemia often need emergency medical care or are admitted to the hospital because they experience complications like acute chest syndrome (ACS) or vaso-occlusive crisis (VOC). […] Acute chest syndrome (ACS) is the most common complication of sickle cell anemia. It’s also the most common cause of death and the second most common cause of hospital admission. It happens when sickled cells clump and clog blood vessels in your lungs. Symptoms include: Sudden chest pain. Cough. Fever. Trouble breathing. […] Vaso-occlusive crisis (VOC): This is when sickled cells block your blood vessels. In VOC, you can have excruciating pain that affects your arms, legs, lower back and belly.

• #2

  https://www.beaumont.org/conditions/symptoms-of-sickle-cell-anemia

  Acute chest syndrome. This occurs when sickling is in the lungs. This can be a life-threatening complication of sickle cell anemia. It often occurs suddenly, when the body is under stress from infection, fever, or dehydration. The sickled cells stick together and block the flow of oxygen in the tiny vessels in the lungs. It resembles pneumonia and can include fever, pain, and a violent cough. Multiple episodes of acute chest syndrome can cause permanent lung damage. Damage to the blood vessels in the lungs can lead to high blood pressures in the lungs (pulmonary hypertension), a condition that can cause death in adults with sickle cell anemia. […] Splenic sequestration (pooling). This complication is a result of sickle cells pooling in the spleen. This can cause a sudden drop in hemoglobin and can be life-threatening if not treated promptly. The spleen can also become enlarged and painful from the increase in blood volume. After repeated episodes of splenic sequestration, the spleen becomes scarred, and permanently damaged. By age 8, most children do not have a functioning spleen either from damage related to the sickled cells, surgical removal, or from repeated episodes of splenic sequestration. The risk of infection is a major concern of children without a functioning spleen. Infection is the major cause of death in children younger than age 5 in this population.

• #2 Sickle Cell Disease in Children | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/s/sickle-cell-disease-in-children.html

  Splenic sequestration (pooling). The spleen becomes enlarged and painful when sickle cells get stuck and build up there. Fewer red blood cells are able to move. This can cause a sudden drop in hemoglobin. It can be deadly if not treated at once. […] The symptoms of SCD may look like other disorders or health problems. Always see your child’s healthcare provider for a diagnosis. […] Complications of SCD include: Long-term anemia. This may lead to delayed healing and delayed growth and development. […] Pain crisis, or sickle crisis. In severe cases, your child may need treatment in a hospital. […] Acute chest syndrome. Over time, many episodes of acute chest syndrome can cause lasting (permanent) lung damage. […] Splenic sequestration (pooling). When red blood cells build up in the spleen, it becomes enlarged and painful. The spleen can be damaged and scarred after many episodes of splenic sequestration. […] Stroke. If the blood vessels to the brain are blocked, a stroke can occur. Serious long-term problems may result. A child who has had a stroke is more likely to have another one. […] Infections. Babies and children with SCD have a higher risk for infections.

• #2 Sickle Cell Disease (SCD): Symptoms & Causes | NewYork-Presbyterian

  https://www.nyp.org/pediatrics/sickle-cell-disease

  Complications of sickle cell disease include: […] Acute chest syndrome (ACS) – A life-threatening condition that occurs when sickle cells block blood and essential oxygen from reaching the lungs, causing low-oxygen rates, breathing difficulties, and injury […] Stroke – When sickle cells get stuck in blood vessels and block blood flow to the brain. Stroke symptoms may include seizures, numbness/weakness in the legs and arms, speech difficulties, and loss of consciousness, and may lead to death. […] Pulmonary hypertension – More common in adults, SCD may cause high blood pressure in the lungs, which may result in extreme fatigue and life-threatening breathing difficulties […] Splenic sequestration – Sickle cells may become trapped in the spleen. This can lead to an enlarged spleen and pain in the belly on the left side of the body.

• #2 Sickle Cell Anemia: Types, Symptoms, and Treatment

  https://www.healthline.com/health/sickle-cell-anemia

  Symptoms of sickle cell anemia usually show up at a young age. They may appear in babies as early as 4 months old, but generally occur around the 6-month mark. […] While there are multiple types of SCD, they all have similar symptoms, which vary in severity. These include: excessive fatigue or irritability, from anemia; fussiness, in babies; bedwetting, from associated kidney problems; jaundice, which is yellowing of the eyes and skin; swelling and pain in hands and feet; frequent infections; pain in the chest, back, arms, or legs. […] Delayed growth often occurs in people with SCD. Children are generally shorter but regain their height by adulthood. Sexual maturation may also be delayed. This happens because sickle cell RBCs can’t supply enough oxygen and nutrients. […] The prognosis of the disease varies. Some patients have frequent and painful sickle cell crises. Others only rarely have attacks.

• #2 Understanding Sickle Cell Symptoms | Sickle Cell Speaks

  https://www.sicklecellspeaks.com/understanding-sickle-cell/symptoms

  People with sickle cell, especially infants and children, are more likely to get infections. […] When sickled cells get stuck in the blood vessels, it blocks blood flow to the arms and legs. That causes swelling in the hands and feet. […] Sickle cell can affect the blood vessels in the eyes. That can lead to long-term damage, including loss of vision. […] Sickle cell ulcers are sores that usually start small and then get bigger. […] Symptoms include pain and problems with walking and joint movement. […] Because of their anemia, children with sickle cell may grow and develop at a slower pace than other children, and they may take longer to reach puberty.

• #2 Sickle Cell Retinopathy

  https://www.asrs.org/SickleCellRetinopathy

  Patients with retinal involvement of sickle cell disease often do not have any eye symptoms. However, when symptoms are present, patients describe: […] Symptoms of sickle cell disease commonly include painful vaso-occlusive crises, as well as pneumonia and strokes. […] Vision loss is less common in patients with HbSS disease. […] This form tends to affect the retina more frequently, and patients have higher rates of vision loss. […] Patients will sometimes note blind spots as a result. […] This can cause significant vision loss.

• #2

  https://www.beaumont.org/conditions/symptoms-of-sickle-cell-anemia

  Stroke. This is another sudden and severe complication of people with sickle cell anemia. The misshapen cells can block the major blood vessels that supply the brain with oxygen. Any interruption in the flow of blood and oxygen to the brain can result in devastating neurological impairment. Having had one stroke from sickle cell anemia, a person is more likely to have a second and third stroke. […] Jaundice, or yellowing of the skin, eyes, and mouth. Jaundice is a common sign and symptom of sickle cell disease. Sickle cells do not live as long as normal red blood cells and, therefore, they are dying more rapidly than the liver can filter them out. Bilirubin (which causes the yellow color) from these broken down cells builds up in the system causing jaundice. […] Priapism. A painful obstruction of the penis by sickle cells. If not promptly treated, it can result in impotence (an inability to have an erection) and possibly infertility.

• #2 Sickle Cell Disease Symptoms & Complications | Vaso-Occlusive Events and Pain Crisis

  https://www.sparksicklecellchange.com/what-is-sickle-cell/symptoms-complications

  The underlying damage caused by acute complications can lead to more serious complications. Even when you are not in pain, ongoing blockages, damage to the blood vessels, and the breaking down of sickled red blood cells over time can lead to progressive and chronic complications including organ damage or even failure. […] Chronic pain is common among people with sickle cell and generally intensifies with age. […] Anemia occurs when sickled red blood cells break apart and die faster than the body can replace them, reducing the amount of oxygen throughout the body. This shortage of red blood cells can also cause fatigue. […] Repeated blockages and long-term damage from sickled red blood cells eventually cause an organ to completely stop working. This occurs in 59% of adults with sickle cell due to chronic complications and can affect multiple organs or systems in the body.

• #2 Disease progression in sickle cell disease

  https://ourironwill.com/progression/scd

  Sickle cell disease can cause progressive organ damage. […] Sickle cell disease is a progressive disease that can result in multiple organ damage over time starting in childhood. […] Younger people are at risk of progressive organ damage by the time they reach their early adult years. […] Within the first 10 years of shifting to adult care, there is more rapid worsening in organ damage with a death rate of up to 20%. […] 50% of people have irreversible organ damage affecting at least one organ by the age of 50. […] Iron overload from multiple blood transfusions can add additional stress and damage to the vital organs. The goal for people living with sickle cell disease is to get and stay ahead of progressive organ damage. Work with your healthcare team to make sure that your organ function is being monitored early in childhood and regularly in adulthood according to guidelines.

• #2 Sickle Cell Anemia: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/4579-sickle-cell-anemia

  For most people, sickle cell anemia is a chronic illness. That means they’ll need medical care and support all their lives. Sickle cell anemia symptoms can be mild, moderate or so severe that they’re life-threatening. […] As you get older, you may develop different and more serious medical problems that happen when organ tissues don’t receive enough oxygen. People with sickle cell anemia are at increased risk for stroke and lung, kidney, spleen and liver damage. […] Thanks to early diagnosis and treatment to ease complications, people with sickle cell anemia may live into their 50s. Some people with the disease may live much longer.

• #2 Sickle Cell Disease (SCD) Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/205926-clinical

  Vascular occlusion can result in avascular necrosis (AVN) of the femoral or humeral head and subsequent infarction and collapse at either site. […] Pulmonary hypertension is a serious complication of SCD, with an incidence as high as 31.8%, and the frequency increases with patient age. […] Dyspnea on exertion and fatigue are the most common initial symptoms of pulmonary hypertension. As pulmonary hypertension progresses, patients may develop peripheral edema and exertional chest pain and syncope, due to right ventricular failure.

• #2 Sickle cell disease – Wikipedia

  https://en.wikipedia.org/wiki/Sickle_cell_disease

  Attacks of pain, anemia, swelling in the hands and feet, bacterial infections, stroke. […] Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain (known as a sickle cell crisis) in joints, anemia, swelling in the hands and feet, bacterial infections, dizziness and stroke. […] The probability of severe symptoms, including long-term pain, increases with age. […] All of the major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can be damaged from the abnormal functions of the sickle cells and their inability to effectively flow through the small blood vessels. […] When SCD presents within the first year of life, the most common problem is an episode of pain and swelling in the child’s hands and feet, known as dactylitis or „hand-foot syndrome.” Pallor, jaundice, and fatigue can also be early signs due to anaemia resulting from sickle cell disease.

• #2 Sickle Cell Disease in Children – Stanford Medicine Children’s Health

  https://deprod.stanfordchildrens.org/en/topic/default?id=sickle-cell-disease-in-children-90-P02327

  Most children with SCD will start showing symptoms during the first year, often around 5 months. […] Symptoms can include: Anemia. This is the most common symptom. Having fewer red blood cells causes anemia. Anemia can make a child pale and tired. […] Yellowing of the skin, eyes, and mouth (jaundice). This is a common symptom. Sickle cells don’t live as long as normal red blood cells. They die faster than the liver can filter them out. The yellow color is caused by a substance (bilirubin) that is released when the red blood cells die. […] Pain crisis, or sickle crisis. When sickle cells move through small blood vessels, they can get stuck. This blocks blood flow and causes pain. This sudden pain can happen anywhere, but most often occurs in the chest, arms, and legs. […] Acute chest syndrome. This is when sickle cells stick together and block oxygen flow in the tiny vessels in the lungs. This can be deadly.

• #2 Infants & Toddlers with Sickle Cell | Children’s Hospital Pittsburgh

  https://www.chp.edu/our-services/cancer/conditions/sickle-cell/resources/infants-and-toddlers

  Fever every episode of fever of 101 degrees Fahrenheit (38.4 Celsius) needs medical evaluation immediately. Do not wait. With temperatures of 99-100 Fahrenheit, call for advice. Do not give Tylenol, Motrin or other medicines to reduce a fever call for advice. […] Problems with the spleen (splenic sequestration) symptoms include: paleness, fussiness, irritability, increased sleeping, rapid breathing, enlarged abdomen. Prompt medical evaluation is necessary. […] Dactylitis (Hand/Foot Syndrome) symptoms include: swelling and discomfort in hands or feet. This condition may be treated at home if it is not accompanied by a fever. Call for advice. […] Call to be seen right away if: Fever 101F (38.4C) or higher, Color very pale, Behavior isn’t acting like usual self; won’t wake up, Stomach vomits/has diarrhea more than once, Color jaundice (eyes or skin look yellow), Hands/Feet swelling, tenderness, pain, Nose runny or stuffy nose, Chest cough without fever or chest pain.

• #2 Sickle Cell Disease Symptoms, Types and Complications

  https://www.froedtert.com/sickle-cell-disease/symptoms

  End-Stage Organ Disease: Decreased blood flow can lead to organ failure, potentially affecting the kidneys, liver and heart. […] Eye Disease or Blindness: Sickled blood cells can damage the fragile blood vessels in the back of the eye, leading to retina damage called retinopathy. This can lead to blindness. […] Heart Murmur or Enlargement: Restricted blood flow can lead to heart problems, such as murmur (an unusual sound that can mean a heart disorder) or an enlarged heart because your heart has to pump harder with SCD. […] Infections: The spleen fails in SCD patients at a young age, decreasing the body’s ability to fight infections. […] Stroke: Children with SCD between the ages of 6 and 10 are at high risk of stroke. Older patients have a lower overall risk of stroke, but they have a higher risk of dying from a stroke. Children with SCD tend to experience symptoms on an episodic basis. As patients enter adulthood, they tend to have chronic (occur all the time) symptoms and organ dysfunction (organs don’t work as they should).

• #2 Adult Sickle Cell Disease Symptoms & Treatment | UPMC – Pittsburgh PA

  https://www.upmc.com/services/sickle-cell/conditions/sickle-cell-disease

  Sickle cell disease is an inherited blood disorder usually diagnosed at birth. Most people with the disease begin to show symptoms by 4 months of age or shortly thereafter. […] Adult sickle cell disease can cause the same signs and symptoms as in children. […] Although the signs and symptoms of sickle cell disease vary from person to person, most people experience anemia-like symptoms and pain. […] Common symptoms include: Fatigue (feeling weak and unusually tired), Dizziness, Headaches, Cold hands and feet, Jaundice (yellow tinted skin or whites of eyes), Unusually pale skin and mucous membranes (tissue inside the nose, mouth, and elsewhere inside the body). […] Individuals with sickle cell anemia often experience sudden, intense pain throughout the body. These vaso-occlusive crises occur when sickle cells block the proper flow of blood to the extremities (i.e. arms and legs) and organs.

• #2 Symptoms of Sickle Cell Disease (Sickle Cell Anemia)

  https://www.webmd.com/a-to-z-guides/symptoms-of-sickle-cell-disease

  Pain is an unpredictable symptom of sickle cell disease. The deformed blood cells can get stuck in and block your blood vessels. When this happens, oxygen-rich blood cannot reach key organs and tissues. The result is a sudden pain attack, called a pain crisis. […] A pain crisis can last anywhere from hours to weeks. If its severe, youll need to seek emergency care at a hospital. The number of pain attacks varies from person to person. Some people have them often. Others have only a few every now and then. Most kids are pain-free between attacks. But many teens and adults have long-term pain. […] Some types of the disorder, like sickle cell anemia (also called hemoglobin SS), cause more severe symptoms than other types. Some people have only mild symptoms while others may have dramatic flares that require medical care. And your experience may change.

• #2 Sickle cell disease is a genetic disorder that causes lifelong suffering – here’s what you need to know

  https://theconversation.com/sickle-cell-disease-is-a-genetic-disorder-that-causes-lifelong-suffering-heres-what-you-need-to-know-243827

  Symptoms and complications of sickle cell disease start in the first year of life and progress in severity. The disease reduces the quality and duration of life of patients in the UK, those with sickle cell disease have a life expectancy of 67. […] Worldwide, life expectancy is below 50 and many children with sickle cell disease in sub-Saharan Africa die before the age of five.

• #2 Sickle Cell Trait – Hematology.org

  https://www.hematology.org/education/patients/anemia/sickle-cell-trait

  Most people with sickle cell trait have no symptoms and will not have any health complications. […] Occasionally people with sickle cell trait can have blood in their urine. Under extreme conditions such as high altitude, severe dehydration, or very high intensity physical activity, red cells can become deformed or sickled. Complications include muscle breakdown (rhabdomyolysis), reduced blood supply to the spleen (ischemia/infarction), or increased pressure in the eye (glaucoma) following eye injuries. Finally, a very rare form of kidney cancer (renal medullary carcinoma) has been associated with sickle cell trait.

• #2 Sickle Cell Trait: Symptoms, Diagnosis, Treatment And More

  https://www.healthline.com/health/sickle-cell-trait

  The best way to prevent complications of SCT is to avoid situations that may trigger severe symptoms, such as strenuous exercise, scuba diving, or mountain climbing. Athletes with SCT must be cautious about exerting themselves and ensure they stay cool, rested, and hydrated. […] Rare but severe risks include pregnancy complications, muscle breakdown, and even sudden death. In more mild cases, you can manage symptoms at home.

• #2 Sickle Cell Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/sickle-cell-disease-in-children

  Sickle cell disease is the name of a group of inherited blood disorders that is characterized by chronic anemia, periodic episodes of pain and other complications. […] Anemia is the most common feature of sickle cell disease. Anemia may cause fatigue, paleness, yellowing of the skin and eyes (jaundice), or shortness of breath. […] Pain is the most common symptom of sickle cell disease. Some people have few or no severe pain episodes a year while others have 15 or more. Pain may last a few hours to a few days, and, in some cases, it can last for weeks. Some people have chronic, almost daily pain. […] Complications of sickle cell disease may be acute or chronic. […] Acute complications include anemia, pain, infection, splenic sequestration, aplastic crises, dactylitis (hand-foot syndrome), acute chest syndrome, stroke, and priapism. […] Chronic complications related to sickle cell disease include gallstones, pulmonary hypertension, joint disease, eye problems, kidney problems, and neuropsychological effects.

• #2 Children with Sickle Cell, Disease Symptoms, Causes and Treatments | Children’s Hospital Los Angeles

  https://www.chla.org/sickle-cell-disease

  Sickle cell disease can lead to serious health problems, such as blood clots, that may be life-threatening and require immediate medical care. […] Pain Crisis (Vaso-Occlusive Crisis): This is the most common complication of SCD and often requires hospital treatment. Sickle cells block blood flow, causing sudden and intense pain that can last for days or longer. […] Sickle cell disease can be managed, and advances in treatment have significantly improved the quality of life and life expectancy for affected children. Although SCD remains a serious condition, modern therapies have reduced many of its life-threatening complications. Today, more than 95% of children with SCD in the U.S. live well into adulthood.

• #3 Sickle cell anemia – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

  Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include: […] Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body can’t get enough oxygen. This causes fatigue. […] Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay.

• #3 Sickle Cell Disease Symptoms & Complications | Vaso-Occlusive Events and Pain Crisis

  https://www.sparksicklecellchange.com/what-is-sickle-cell/symptoms-complications

  Sickle cell is a progressive, lifelong illness that has long-term complications and affects everyone differently. Its symptoms are unpredictable and can be severe, varying from person to person, as well as for an individual over time. […] Pain crises, also known as a sickle cell crisis, are unpredictable and extremely painful, lasting anywhere from a few hours to a few weeks, and are the most common reason for hospitalization. Frequency, duration, and intensity of pain crises can vary with age. […] Although sickle cell is present at birth, most newborns don’t experience problems until they are ~5 months old. […] It’s important to note that although a person may not be experiencing symptoms, the silent damage of sickle cell to your body and organs is progressive, and frequency of symptoms may increase with age.

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