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  1. 1. WHAT IS SICKLE CELL? Origin and Distribution of Sickle Cell Disease Sickle cell trait occurred as a natural mutation of the hemoglobin gene. Sickle trait serves as a protective mechanism against malaria. Malaria is a deadly disease found in countries along the equator. People with sickle cell trait are protected from malaria while those with sickle cell anemia and normal hemoglobin are susceptible to it. Over the years individuals with sickle trait migrated to other continents. Sickle cell disease is seen predominantly among African Americans but is also seen in people of other ethnic groups. These ethnic groups include individuals from parts of the Middle East, Central India, and countries bordering the Mediterranean Sea, especially Italy and Greece. 1
  2. 2. Red Blood Cells Sickle Red Blood Cells 2
  3. 3. It is the inheritance of the sickle genes that causes red blood cell (RBC) abnormality. All complications of Sickle Cell Disease can he traced to changes in the make-up of the RBC. Normal RBC’s are smooth surfaced, enabling them to change their shape to flow through small blood vessels. Under certain conditions (i.e., acidosis, dehydration, infection, and low oxygen. etc.) RBC’s containing Sickle Hemoglobin become rigid, elongated, and sickle shaped. Some RBCs sickle immediately, while others remain normal for hours before sickling. Most RBCs containing Sickle Hemoglobin can sickle and then unsickle. After repeated cycles of sickling and unsickling, the RBC’s become irreversibly sickled. In Sickle C Disease, some RBCs are sickle cells. The C Hemoglobin forms slightly misshaped RBCs but they are of normal size and color. The C Hemoglobin tends to reduce the complications caused by the sickled calls. In Sickle Beta Plus or Zero Thalassemia, a portion of the RBCs are sickle cells. The Thalassemia cells are paler than usual and too small. There is no Hemoglobin A present in Sickle Beta Zero Thalassemia. There is a small amount of Hemoglobin A present in Sickle Beta Plus Thalassemia which tends to minimize the complications caused by the sickled cells. Blood Flow Sickled RBC's can become trapped within the blood vessels and thus interfere with normal blood flow. This obstruction can lead to sudden pain anywhere in the body as well as cause damage to body tissues and organs over time. 3
  4. 4. PAIN Painful episodes are common complications in children with Sickle Cell Disease. When the sickled cells are unable to flow through small blood vessels they obstruct blood flow causing vascular occlusion (vaso-occlusion). Vaso-occluision reduces blood flow to an area of the body resulting in pain. This can occur anywhere in the body, including fingers, arms, legs, ribs, abdomen, and organs such as the spleen, brain, and eyes. 4
  5. 5. During infancy, vaso-occlusive crises (VOC’s) are generally manifested as dactylitis or handfoot syndrome. This is characterized by soft tissue swelling warmth and/or pain in the hands and/or feet due to ischemia (decreased oxygen) in these small bones. Dactylitis call be recurrent hut usually does not occur after two or three years of age. The most common sites of pain in children over two years of age are the long bones, joints, back, and abdomen. VOC’s can vary in duration, intensity. location, and time between episodes. They can be mild moderate, or severe in terms of pain. Sometimes swelling is seen in the area of pain. VOC’s may be preceded by a fever dehydration, trauma, swimming, exposure to cold and/or emotional stress and unknown factors. Infection may occur at the same time. Management of Pain Painful episodes can often be treated and managed at home with regular acetaminophen (Tylenol), ibuprofen, or acetaminophen with codeine and hydration (an extra two to four ounces of water or juice every hour). Rubbing or application of heat with a heating pad or hot water bottle to the painful area may also alleviate discomfort. Sometimes pain is unresponsive to home therapy. During those times, the child should go to the emergency room for intravenous (IV) hydration and pain medication. IV morphine is usually used. Most of the time this is adequate treatment so that home therapy will then be effective. 5
  6. 6. In very severe painful crises, the child may need to be admitted to the hospital for IV therapy. Sometimes the child can be taught to give his or her own pain medication by vein. This is called patient controlled analgesia, or PCA. 6
  7. 7. GENETICS OF SICKLE CELL DISEASE Sickle Cell Anemia (SS) is an inherited blood disorder (autosomal recessive). Approximately one in 400 African American children are born with Sickle Cell Anemia, and about one in ten have Sickle Cell Trait (AS). Sickle Cell Anemia (SS) The two hemoglobin types inherited will determine the shape of the red blood cell (RBC). When both parents have Sickle Cell Trait, there is a l-in-4 chance (25 percent) the baby will have normal hemoglobin (AA), a 50 percent chance the baby will have Sickle Cell Trait (AS), and a lin-4 chance (25 percent) the baby will have Sickle Cell Anemia (SS). These chances remain the same with each pregnancy. 7
  8. 8. Sickle Hemoglobin C Disease Sickle Hemoglobin C (SC Disease) is a milder form of Sickle Cell Disease. The baby has inherited two (2) abnormal hemoglobins, hemoglobin S and hemoglobin C. Approximately one in l,000 black babies are born with SC Disease. Hemoglobin C Trail (AC) occurs in about one in 40 black babies. If one parent has AS and the other AC, there is a 1-in-4 chance (25 percent) the baby will inherit AA, AS, AC, or SC Disease. AS and AC are carrier states, not disease conditions. 8
  9. 9. Sickle Beta Zero Sickle Beta 0 Thalassemia Sickle Beta Zero Thalassemia (ST0) is clinically similar to Sickle Cell Anemia. STO occurs in approximately one in 10,000 black babies. STO is treated the same as Sickle Cell Anemia. When one parent has Sickle Trait (AS) and the other parent has Beta Thalassemia Zero Trait (AT) there is a l-in-4 chance the baby will have normal hemoglobin (AA), Beta Thalassemia Zero Trait (AT, Sickle Beta Zero Thalassemia (STO), or Sickle Trait (AS). These chances remain the same for each pregnancy. Sickle Beta Plus Thalassemia Sickle Beta Plus Thalassemia (ST+) is the mildest form of Sickle Cell Disease. ST+ occurs in approximately one in 4,000 African American children. When one parent has Sickle Trait (AS) and the other parent has Beta Thalassemia Plus Trait (AT+), there is a l-in-4 chance (25 percent) the baby will have normal hemoglobin (AA), Sickle Trait (AS), Beta Thalassemia Plus Trait (AT+), or Sickle Beta Plus Thalassemia (ST +). These chances remain the same for each pregnancy. 9
  10. 10. Hemoglobin C Disease Hemoglobin C Disease, a condition found mostly in the African American population (approximately one in 6,000), consists of a minor abnormality of the hemoglobin. When both parents have C Trait (AC), there is a 1-in-4 chance (25 percent) that the baby will have normal hemoglobin (AA) or Hemoglobin C Disease (CC), and a 50 percent chance the baby will have Hemoglobin C Trait (AC). These chances remain the same for each pregnancy. 10
  11. 11. SELF CARE Treatment Analgesia, hydration, antibiotics and oxygen are used. Sometimes a blood transfusion is necessary if the blood count or if the chest syndrome is severe. Blood Transfusion Most often a blood transfusion is given to raise the blood count until the body can start making its own RBC’s again. Brothers and sisters with Sickle Cell Anemia should have their blood count checked since Parvovirus B19 is very contagious and they may be at risk of an aplastic crisis too. 11
  12. 12. CRITICAL HEALTH CONDITIONS Acute Splenic Sequestration Crisis The spleen is normally a small organ located on the upper left side of the abdomen under the rib cage. When sickle cells are trapped in blood vessels inside and leading out of the spleen, the normal flow of the blood is blocked. Blood stays inside of the spleen instead of flowing through it. This is called sequestration, as a result, the blood count falls and the spleen gels very large and is easy to feel. If the spleen suddenly enlarges with a significant drop in the blood count, this is a serious and potentially life-threatening problem. When the spleen gradually gets larger over several weeks, the blood count does not change much, so it is not as serious. Any enlargement of the spleen is of concern and must be watched for changes. Parents should know how their child’s spleen normally feels, so that whenever the child seems sick they can check the spleen to see if it is bigger. If the spleen suddenly becomes larger, the child should he checked by a physician immediately. If the blood count is dangerously low from sequestration, blood transfusion may he 12
  13. 13. necessary. Recurrent episodes are common, and a splenectomy (removal of the spleen) is sometimes required. Babies and young children with Sickle Cell Anemia are at greatest risk of splenic sequestration. After age five years, the spleen becomes smaller and in most cases it cannot enlarge any more. Children with Sickle C Disease usually experience this complication after the age of five years. 13
  14. 14. Infections Infection is the major cause of death in children with Sickle Cell Anemia under the age of five years. The spleen functions as part of the body’s defense against infection by serving as a filter to remove bacteria from the blood stream. The sickle RBC’s damage the spleen by about four months of age so that the spleen does not function normally. This can allow bacteria to grow in the blood stream and cause septicemia, which can be fatal. Children under the age of five years are at highest risk for septicemia. Streptococcus Pneumonia, (also called the pneumococcus) and Hemophilus influenza are the two bacteria most likely to cause septicemia in the child with Sickle Cell Anemia, ninety percent of the infections occur before the age of three years. Thirty-five percent (35 percent) of children with Sickle Cell Anemia who get pneumococcal sepsis die from the infection. Signs & Symptoms Fever* - 101 F degrees or higher Coughing Vomiting and or Diarrhea Crankiness Rapid breathing Pale Color Unusual sleepiness Trouble Breathing A fever may be the ONLY initial sign of septicemia. Other potentially serious infectious which are more likely to occur in the child with Sickle Cell Anemia are meningitis, pneumonia and osteomyelitis. Any infection in the child with Sickle Cell Disease is an emergency. Infection is treatable and complete recovery is possible only if it is recognized and treated early enough. However, even with treatment, permanent disabilities and even death can result. Penicillin is often prescribed prophylactically twice daily to help fight infection. Septicemia can still occur even if penicillin is taken regularly. The child with SC Disease or Sickle Beta Plus Thalassemia is not at as high a risk for septicemia as the child with Sickle Cell Anemia or Sickle Beta Zero Thalassemia. Penicillin is not always recommended for these children. Pneumococcal and Hemophilus influenza vaccine should be given to children with Sickle Cell Disease to help boost their immunity. 14
  15. 15. Acute Chest Syndrome Acute chest syndrome is a common cause of hospitalization in children with sickle cell disease. It is clinically similar to pneumonia. Acute chest syndrome can be fatal in the child with sickle cell disease. Acute chest syndrome is the result of sickling in the lungs. It is believed that sickled cells clump up in the small blood vessels in the lungs or move there from some place else in the body. This may be triggered by a lung infection like pneumonia. Acute chest syndrome may develop right before, during, or after an episode of pain in the abdomen or bones Signs & Symptoms • • • • • • Chest pain Fast breathing and/or retractions Congested pneumonia-like cough Abdominal pain Fever Trouble breathing 15
  16. 16. Aplastic Crisis An aplastic crisis results from an infection caused by Parvovirus B19. It causes production of RBC’s to be shut down for about 10 days. This means that RBC’s are not being made during this period. Because the RBC's in children with Sickle Cell Anemia live only 10 to 15 days (compared to 120 days in children who do not have Sickle Cell Anemia), the blood count (hemoglobin and hematocrit) drops very rapidly to a dangerously low level during the infection. Aplastic crisis usually occurs in children under the age of 16 years. It occurs in the general population but can only be noticed in those people with chronic hemolytic anemia (e.g. Sickle Cell Anemia). Recurrences of aplastic crisis are rare. 16
  17. 17. Stroke A stroke is a sudden and severe complication of sickle cell anemia. The most common cause of a stroke in children with sickle cell anemia is cerebral infarction (b1ockage of the oxygen supply to the brain by sickled cells). Strokes occur in six to 12 percent of individuals with sickle cell anemia, more commonly between three and 10 years of age. A stroke may occur with a painful episode or an infection, but in most cases there are no related illnesses. Although recovery from a stroke may be complete in some cases, frequently the stroke can cause brain damage, paralysis, convulsions, coma and even death. Repeat strokes occur in at least 60 percent of the children who have already suffered one stroke. A repeat stroke causes greater brain damage and increases the risk of death. To prevent recurrent strokes, blood transfusions are often given at four or five week intervals. It is not known how long these transfusions must be given to prevent another stroke from occurring. Transcranial Doppler Ultrasounds (ultrasounds of the brain vessels) are performed on sickle cell patients (SS, SS- Beta Thalassemia) to determine if they are at risk for developing a stroke. 17
  18. 18. Gallstones and Jaundice Gallstones in children with sickle cell disease are the result of elevated bilirubin excretion due to the increased hemolysis. Gallstones are found in about 30 to 50 percent of children with sickle cell anemia. They may be symptomatic or asymptomatic. Most physicians monitor the child with asyniptomatic gallstones and do not recommend a cholecystectomy (removal of the gallbladder) until symptoms occur. Elective cholecystectomy may be indicated when gallstones are symptomatic (chronic right upper quadrant pain, nausea, vomiting, and fullness after meals). The complications of gallstones can include passage of stones causing colic, common bile duct obstruction, cholecystitis, and rarely, pancreatitis. Often the eyes of children with sickle cell anemia may appear yellow or jaundiced. This is due to the accumulation of a waste product (called bilirubin) from the increased RBC hemolysis associated with sickle cell disease. It is a benign complication. 18
  19. 19. Priapism Priapism is a persistent, unwanted erection of the penis that is often extremely painful. Specific causes are unknown. Acute episodes often begin during sleep or following sexual activity, hut frequently there is no identifiable event or cause. There is no current therapy to prevent episodes of priapism. There is no way to predict who will develop priapism and impotence. Those individuals experiencing repeated episodes are encouraged to avoid long periods of bladder distention, dehydration, and extended sexual activities. Priapism may present in the following ways: Stuttering There may be repeated, reversible painful erections occurring over several hours (the penis becomes erect, the erection goes away, then becomes erect again, etc.). There are no problems with sexual functioning. Prolonged There may be a prolonged, painful erection that does not go away for more than several hours. This can last up to several days or weeks. This type of priapism needs attention by a doctor. Severe priapism can lead to partial or complete impotence. Sometimes a blood transfusion and liberal analgesics are given during the episode. Persistent There maybe a persistent penile erection that may last for weeks to years. This type of priapism is usually painless. It usually develops after a long episode of prolonged or stuttering priapism (as described above). Sexual functioning is often impaired. 19
  20. 20. TREATMENT Hydroxyurea Hydroxyurea (Hydrea) is a drug used to treat cancer. Recently, it has been found to benefit some patients with sickle cell disease. The benefit occurs by increasing fetal hemoglobin (hemoglobin F). Hemoglobin is a protein in red blood cells. Hemoglobin carries and delivers oxygen to the tissues of the body. Fetal hemoglobin is a special hemoglobin produced by babies before they are born. It protects against some of the effects of sickle cell disease. During the first four to six months of life, fetal hemoglobin decreases and sickle hemoglobin increases. This is why infants with sickle cell disease do not have painful crises during the first four to six months of life. Some children and adults maintain higher levels of fetal hemoglobin. They also have fewer problems from their sickle cell disease. Research studies are now reporting that drugs like hydrea can reduce some of the problems associated with sickle cell disease. Hydrea stimulates the production of hemoglobin F. Hydrea has been used in several recent research studies. It has been shown to decrease the number and severity of acute painful episodes when taken regularly. Pain occurs when blood vessels are blocked by clumps of sickled cells (vaso-occlusion). One study showed a 50% decrease in intensity of pain and the frequency of episodes. Hydrea does not help pain once the episode begins. Hydrea also decreases the number of white blood cells produced. White cells often stick to the sickled cells increasing the intensity of the painful episode. Some studies also suggest that the function of the spleen may improve while taking Hydrea. One function of the spleen is to fight bacterial infections. Research studies in adults indicate that Hydrea must be taken for four to six months before improvement occurs. Research studies show promise for the use of Hydrea in children. However, its’ long term benefits and risks are not yet fully known. Laboratory studies in animals indicate Hydrea may cause genetic changes, increase the risk for cancer, and/or increase birth defects. For this reason, it is recommended that patients taking Hydrea not conceive children or breast-feed. Sickle cell patients have been treated with Hydrea beginning in the mid 1980s, and have not shown any increased risk of birth defects or cancer, but this will need continued monitoring. Hydrea is generally well tolerated. The most common side effect is decreased white blood cells and platelets. Less common are nausea, vomiting, diarrhea, constipation, hair loss, rash, nail and skin discoloration, fever, weight gain, leg ulcers and bleeding. Occasionally, kidney or liver function is decreased. These side effects usually disappear when Hydrea is stopped. Short-Term Effects The blood-related, short-term effects of hydroxyurea are dose-related and can be predicted on the basis of its mechanism. These are intrinsic to the therapeutic effect of hydroxyurea. They include a decreased leukocyte count (leukopenia), decreased platelet count (thrombocytopenia), decreased erythrocyte count (anemia), and decreased reticulocyte count (fewer newly formed erythrocytes). A decrease in leukocyte count may predispose the patient to infection, and a decrease in platelet count may predispose the patient to bleeding; these blood cells are therefore monitored regularly 20
  21. 21. during therapy. The effect of hydroxyurea on blood is temporary and reversible. If leukocyte or platelet counts are too low, the dose of hydroxyurea is reduced or hydroxyurea therapy is discontinued. Careful monitoring of blood-related laboratory tests and dose adherence will usually prevent these side effects. Another possible short-term effect among men taking hydroxyurea is decreased sperm production, which may be temporary and reversible. Data are limited. No large studies are available of sperm production among men receiving hydroxyurea for sickle cell disease. We are not aware of any reports of an increase in birth defects among the offspring of men who take hydroxyurea. Hydroxyurea seems to cause dryness of the skin and darkening of the skin and nails (hyperpigmentation); this may also be a long-term side effect. Leg ulcers are common in adults with sickle cell disease. In a randomized clinical trial comparing hydroxyurea and placebo, hydroxyurea did not seem to affect the development of leg ulcers in people with sickle cell disease. Gastrointestinal tract symptoms were no more common among people receiving hydroxyurea for sickle cell disease than among those not receiving hydroxyurea. Long-Term Effects The potential long-term effects of hydroxyurea are birth defects in the offspring of people receiving the drug, growth delays in children receiving the drug, and cancer in both children and adults who have received the drug. These long-term harms may be permanent and irreversible, but they are not yet proven. There have been concerns about the potential for hydroxyurea to cause birth defects in humans because it has caused birth defects in experimental animals. Pregnant rats and mice given hydroxyurea in very high doses have an increased number of offspring with birth defects. However, the number of birth defects among the offspring of women who received hydroxyurea during pregnancy does not seem to be increased. The long-term effects of hydroxyurea on children exposed to the drug in utero are unknown. Nonetheless, because of concerns about the potential of hydroxyurea to cause birth defects, the drug is generally not prescribed to pregnant women. Men and women who are receiving hydroxyurea are advised to use contraception. Women who are trying to become pregnant or who do become pregnant while taking hydroxyurea should stop taking the drug. Children 5 to 15 years of age who have sickle cell disease and receive hydroxyurea show a growth rate similar to that of peers with sickle cell disease who are not receiving hydroxyurea. Hydroxyurea has an excellent and long-standing safety profile in the treatment of myeloproliferative disorders, although cases of leukemia and other types of cancer have been reported in patients who have received hydroxyurea for other blood conditions. Most of these conditions are blood disorders, such as polycythemia vera or essential thrombocytosis, and these conditions can progress spontaneously to leukemia. This makes it difficult to determine whether hydroxyurea itself causes leukemia. Cases of leukemia and other types of cancer also have been reported among both children and adults who have taken hydroxyurea to treat sickle cell 21
  22. 22. disease. These cases are rare and seem to be no more common than among the general population. The risk for cancer does not seem to differ for people with sickle cell disease who have received hydroxyurea and those who have not. Because both patients and providers have identified side effects as a concern that limits the use of hydroxyurea, more information on the incidence and severity of these side effects is essential for both patients and providers to make informed choices. These data could come from a registry of patients with sickle cell disease. Nevertheless, the currently available data are reassuring with respect to the risks for both short- and long-term harms of hydroxyurea. The natural history of sickle cell disease results in frequent, severe pain episodes and permanent damage to the eyes, brain, heart, lungs, kidneys, liver, bones, and spleen. Hydroxyurea reduces the frequency and severity of pain episodes. The risks of hydroxyurea are acceptable compared with the risks of untreated sickle cell disease Hydroxyurea is an important major advance in the treatment of sickle cell disease. Strong evidence supports the efficacy of hydroxyurea in adults to decrease severe painful episodes, hospitalizations, number of blood transfusions, and the acute chest syndrome. Although the evidence for efficacy of hydroxyurea treatment for children is not as strong, the emerging data are encouraging. The current data on the risks of both short- and long-term harms of hydroxyurea therapy are reassuring, and the risks of hydroxyurea use in adults are acceptable compared with the risks of untreated sickle cell disease. 22
  23. 23. Bone Marrow Transplant Bone marrow transplantation has been used here at the University of Michigan and nationally to cure patients with sickle cell disease. However, it is usually reserved for patient with more sever sickle cell disease including frequent pain crises (usually requiring medical attention), multiple acute chest syndromes, and history of stroke. Once a patient is deemed eligible for bone marrow transplantation for sickle cell disease, bone marrow typing (a simple blood test) studies are performed on the patient’s family (including siblings) to determine if there is a “bone marrow match”. 23
  24. 24. GROWTH AND DEVELOPMENT FEMALES Females with sickle cell anemia maintain a lower average height and weight than those females with normal hemoglobin. This lower than average height and weight continues until late adolescence. Puberty is usually delayed by several years. Menarche (beginning of the menstrual period) is also delayed. It is important to reassure the adolescent that she will eventually catch up with her peers. 24
  25. 25. MALES Males with sickle cell anemia maintain a lower average height and weight than those males with normal hemoglobin. This lower than average height and weight continues until late adolescence. Puberty is usually delayed by several years. It is important to reassure the adolescent that he will eventually catch up with his peers. Retinopathy Retinopathy can occur in children with sickle cell disease. Sickle cells can damage blood vessels in the retina and vision can be affected. Retinopathy is more common in adolescents with Sickle Hemoglobin C Disease. It is recommended that after the age of 10 years, children with sickle cell disease have periodic ophthalmology examinations. 25
  26. 26. REPRODUCTIVE CARE How does pregnancy affect sickle cell disease? Some women have no change in their disease during pregnancy. However, sickle cell crises (painful events) may still occur in pregnancy and may be treated with medications that are safe to use during pregnancy. Pre-existing kidney disease and congestive heart failure may worsen during pregnancy, even with proper treatment. How does sickle cell disease affect pregnancy? The risks for pregnancy depend on whether the mother has sickle cell disease or sickle cell trait. Generally, women with sickle cell trait are not at increased risk for problems, however, they may experience frequent urinary tract infections. It is also important to remember that, unlike sickle cell anemia, a woman with sickle cell trait can have iron deficient anemia while pregnant and may need iron supplementation for this reason. The ability of the blood cells to carry oxygen is especially important in pregnancy. The sickling and anemia may result in lower amounts of oxygen going to the fetus and slowed fetal growth. Because sickling affects so many organs and body systems, women with the disease are more likely to have complications in pregnancy. Complications and increased risks for the mother may include, but are not limited to, the following: • • • infection, including urinary tract (especially kidney) and lungs gallbladder problems including gallstones heart enlargement and heart failure from anemia Complications and increased risks for the fetus may include, but are not limited to, the following: • • • • • miscarriage intrauterine growth restriction (poor fetal growth) preterm birth (before 37 weeks of pregnancy) low birthweight (less than 5.5 pounds) stillbirth and newborn death How is sickle cell disease managed in pregnancy? Although expectant mothers with sickle cell trait are not at higher risk for pregnancy complications, the baby may be affected if the father also carries the trait. Testing of the baby's father is recommended prior to pregnancy, or at the first prenatal visit. If the baby's father has sickle cell trait, amniocentesis (a procedure used to obtain a small sample of the amniotic fluid) or other methods of prenatal diagnosis may be offered to help determine if the fetus has the trait. 26
  27. 27. Early and regular prenatal care is important for pregnant women with sickle cell disease. More frequent prenatal visits allow for close monitoring of the disease and of fetal wellbeing. General pregnancy care includes a healthy diet, prenatal vitamins, folic acid supplements (a B vitamin), and preventing dehydration. Some women may benefit from blood transfusions to replace the sickled cells with fresh blood. These may be done several times during the pregnancy to help increase the blood's ability to carry oxygen and decrease the number of sickled cells. It is important for women who receive blood transfusions to be screened for antibodies that may have been transferred in the blood and that may affect her fetus. The most common antibodies are to the blood factor Rh. Fetal testing may begin in the second trimester and include: • ultrasound (to measure fetal growth) • nonstress test - measures fetal heart rate in response to fetal movement. • biophysical profile - a test that combines an ultrasound with the nonstress test. • Doppler flow studies - a type of ultrasound which use sound waves to measure blood flow. During labor, intravenous (IV) fluids are given to help prevent dehydration. Most women will receive extra oxygen through a mask during labor and a fetal heart rate monitor is often used to watch for changes in heart rate and signs of fetal distress. There are no special recommendations for the type of delivery for women with sickle cell disease and most women can deliver vaginally, unless there are other complications. Written and Developed by Debra A. Vedro, MSN, RN, CPNP And Rebecca A. Morrison, MSN, RN, CPNP Children's Medical Center of Dallas, Dallas, Texas Illustrated by Mary Ann Zaplac, MA In Coordination with The Texas Department of Health Supported in part by project # MCJ-481005 from The Women and Children Program (Title V, Social Security Act), Heath Resources and Services Administration, US Department of Health and Human Services Reprinted by Permission from the Texas Department of Health 3/01/02 27