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| The Infant & Young Child With Sickle Cell Anemia | |
THE INFANT AND YOUNG CHILD WITH SICKLE CELL ANEMIA
TABLE OF CONTENTSFever Sickle cell Anemia is an inherited blood disease which is particularly serious for infants and young children. About one in every 400 black babies is born with sickle cell anemia. A person with this lifelong disease has abnormally formed red blood cells. All complications of disease can be traced to changes in the makeup of the red blood cell causing it to assume a "sickle" shape, making it unusually fragile, and sometimes very rigid. These red blood cells 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 damage to body tissues and organs over time. Supportive treatment is available for sickle cell anemia, but as yet there is no cure except for a very expensive and experimental bone marrow transplant. The young child with sickle cell anemia has special needs. If these needs are understood by those caring for the child, then complications can be prevented or treated early. These complications are discussed here in detail to help parents and others provide the best possible care to the child with sickle cell anemia. Many young children with sickle cell anemia are rarely sick, but certain complications can occur which are very serious and sometimes fatal (cause death). It is hard to believe that a healthy looking baby has a life-threatening disease, but this is true when a child has sickle cell anemia. At first parents may not want to know what can happen with this disease. They may want to pretend that nothing is wrong until something happens to indicate otherwise. This is a normal reaction to the unpleasant news that a child has a serious disease. Besides feeling sad, parents may feel angry and/ or guilty. These emotions are very normal and usually temporary. Once the disappointment is accepted, parents are able to see the importance of understanding this disease and recognizing early symptoms of serious complications. Complications that can be fatal are: (1) septicemia (infection of the blood), and (2) acute splenic sequestration (sudden enlargement of the spleen and rapid drop in the blood count). Both of these medical emergencies can occur suddenly. Treatment is available for both septicemia and splenic sequestration, but it must be started in time. In the sections which follow, more detailed information is given about these and other problems which can occur in the infant and young child with sickle cell anemia. FEVERFever is normal response of the body to infection. Fever is usually due to a minor infection, but it can be the first symptom of a very serious infection. When fever first begins, it is impossible to tell how serious the infection is. The child with sickle cell anemia is more susceptible to serious infections such as septicemia (infection of the blood or blood poisoning). If the child does have septicemia, treatment must be started early to save his or her life. Fever can be the first symptom of septicemia, so it is important for parents to know what to do when their child seems sick. Whenever a child seems sick the temperature should be checked with a thermometer. Parents should know how to read a thermometer and always have one at home or with them if they are away from home.
Fever medication makes a child feel better and reduces fluid losses from the body, but it does not treat the cause of fever. Medication is not needed unless the fever is over 102 degrees or the child is uncomfortable. There are other things to do when a child has a fever. Dressing the child in light clothing, keeping the room about 70 degrees, and giving a lukewarm bath may help lower the fever. if the child is sleeping, use only a few covers. Anything which causes shivering should be avoided because it will make the fever rise. A child with fever needs to drink more and should have such things as water, juice, or a soft drink frequently. If a child has a temperature of 103 degrees or higher, it is not safe to wait and see if the fever will come down. The child needs to be seen by a doctor right away. THE SPLEEN
The spleen is normally a small organ located under the rib cage on the left side of the body. It functions as part of the body's defense against infection by removing bacteria (germs) from the bloodstream. In the child with sickle cell anemia the spleen is not able to remove bacteria, so bacteria can grow in the bloodstream and cause septicemia (infection of the blood). WHAT CAUSES THE SPLEEN TO BECOME LARGER?When sickled cells are trapped in blood vessels leading out of the spleen, the normal flow of the blood is blocked. Blood stays inside the spleen instead of flowing through it. This is called sequestration. When this happens, the spleen is very large and it is easy to feel. Sometimes sequestration is painful. Medication can be given to ease the discomfort. IS SPLENIC SEQUESTRATION A SERIOUS PROBLEM?If the spleen enlarges suddenly with a large 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 as 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 usually 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 be checked by a doctor as soon as possible. If the child also has symptoms of a low blood count, then the situation becomes an emergency. WHAT ARE THE SYMPTOMS OF A LOW BLOOD COUNT?A child with a low blood count is likely to be irritable, unusually sleepy, and pale with a rapid heart beat. If the fingernails do not have any pink color even when the fingers are warm, the child is pale. A child can have a seriously low blood count without many symptoms. Sometimes the only symptom is that the child is less active. AT WHAT AGE DO PROBLEMS WITH THE SPLEEN USUALLY OCCUR?Babies and young children are at greatest risk of splenic sequestration and septicaemia. Complications can occur as young as 2 months of age, but usually occur between the ages of 6 months and 5 years. As children get older the spleen becomes smaller. Eventually it cannot enlarge anymore. CAN SPLENIC SEQUESTRATION HAPPEN MORE THAN ONCE?Yes. A child who has one episode of splenic sequestrations is likely to have other episodes. WHAT IS THE TREATMENT FOR SPLENIC SEQUESTRATION?If the blood count is dangerously low, then blood transfusion is the treatment. If a child has several episodes, surgery to remove the spleen (splenectomy) may be recommended. WHAT CAN BE DONE TO HELP CHILDREN WITH SICKLE CELL ANEMIA FIGHT INFECTION SINCE THE SPLEEN DOES NOT WORK?Prevention and early treatment of infection is the best defense against serious complications. The child who is sick should be carefully watched for symptoms of serious infection. A fever of 102 degrees or higher should always be considered a symptom of possible septicemia (see fever section). Some children are given penicillin twice daily to prevent infection. Pneumococcal vaccine (a shot) is given to boost immunity. INFECTIONThe child with sickle cell anemia will get colds, sore throats and ear infections just like other children. These minor infections are not serious in the child with sickle cell anemia. The potentially serious infections which are more likely to occur in the child with sickle cell anemia are septicemia (infection of the blood), meningitis (infection around the brain), pneumonia (infection of the lungs), and osteomyelitis (infection of the bone). A child with septicemia may not seem very sick initially. A fever of 102 degrees or higher may be the only symptom at first. Other symptoms are unusual sleepiness, rapid breathing, pale color, vomiting, and diarrhea. Septicemia is the major cause of death in young children with sickle cell anemia. Early treatment is the best hope for recovery. Meningitis is very similar to septicemia in its symptoms. In addition a child with this problem is usually very irritable. Pneumonia can be mild with very few symptoms or it can be quite serious. The symptoms are high fever, rapid breathing, shortness of breath, chest pain, and cough. Osteomyelitis causes fever, pain, and swelling over a bone. At first it is quite similar to a painful episode, but a child usually seems sicker with an infection of the bone. All the infections named here are treatable and complete recovery is possible. It is also true that even with treatment permanent disabilities and even death can result. The child with symptoms of a serious infection should be seen by a doctor as soon as possible. Early recognition and treatment of infection offers the best chance for complete recovery. WHY IS PENICILLIN SO IMPORTANT?The infant and young child with sickle cell anemia is more susceptible to septicemia (infection of the blood). Recent scientific studies indicate that penicillin may prevent fatal cases of septicemia. When given every 12 hours (twice each day), penicillin can kill bacteria before they grow and cause septicemia. WHAT IF A CHILD DOESN'T GET PENICILLIN FOR A FEW DAYS?A child who is not taking penicillin may be going without important protection. About 12 hours after a dose, the penicillin is almost gone from the body. It is best to refill the penicillin before it runs out. CAN SEPTICEMIA DEVELOP EVEN IF THE PENICILLIN IS GIVEN REGULARLY?Yes, it is possible since a few bacteria are resistant to penicillin. A child who is taking penicillin who develops a fever of 102 degrees or higher needs to be watched carefully. If the fever is not down in 1 hour, the child should see the doctor (see Fever). WILL THE PENICILLIN LOSE ITS EFFECTIVENESS?No, penicillin remains very effective over many years. some bacteria are resistant to the penicillin, but this is due to other factors. If an infection develops, other antibiotics can be used for bacteria that are resistant to penicillin. WILL THE PENICILLIN PREVENT COLDS TOO?No. Because colds are caused by viruses and not bacteria they are not affected by penicillin. Children with sickle cell anemia recover from minor illnesses without special problems. PAINFUL EPISODESPainful episodes occur in children with sickle cell anemia as a complication of the disease. These episodes are more common in older children, but sometimes happen in babies. Most often the pain seems to be in the bone, but occasionally it occurs in the abdomen. These episodes are not dangerous and will disappear usually in several hours or sometimes after several days. WHAT IS THE CAUSE OF THESE PAINFUL EPISODES?The exact cause of these episodes of pain is unknown, but it is thought that red blood cells become trapped inside a blood vessel and interfere with normal blood flow. If blood flow is reduced in even a small area of the body it can cause pain. Sometimes swelling is seen in the area of pain. In babies, swelling often occurs in the hands and feet. Older children can have swelling in the arms and legs. Swelling usually does not mean that something is seriously wrong, but in rare cases swelling and pain are caused by infection in the bone. A child with swelling other than hands and feet should be seen by a doctor. WHAT CAN BE DONE TO EASE THE PAIN?Discomfort is usually relieved by taking medication for pain such as acetaminophen or ibuprofen. Even with relief from most of the pain, a child may refuse to use the part of the body which has the pain. If a child will not stand or walk it is best not to force him. As soon as the pain is better he will be active again. Other measures which may help the pain are plenty of fluids, rest and application of warmth such as a heating pad. If a child is not comfortable at home with medication by mouth, then it may be necessary to treat the pain with stronger medication in the hospital. HOW DO YOU KNOW IF THE PAIN IS FROM THE SICKLE CELL DISEASE OR FROM SOMETHING ELSE?If the child does not have other symptoms of illness, the pain is probably due to the sickle cell disease. If the child has fever of 102 degrees or above, an infection could be present. The child who has pain in the chest or abdomen, cough, rapid breathing, shortness of breath, pale color, or high fever in addition to pain in the bones should be seen by a doctor as soon as possible. CAN TOO MUCH ACTIVITY CAUSE A PAINFUL EPISODE?Some parents worry that their child is too active and will cause himself to develop a painful episode. The child who is active is a healthy child. Active behavior should not be discouraged. A child with sickle cell disease needs to be treated as normally as possible, especially when he is acting normally! CAN ANYTHING PREVENT PAINFUL EPISODES?Not enough is known about the cause of painful episodes to prevent them. Several medicines have been tried, but nothing is effective. No nutritional factor is known to affect the development of painful episodes. Children with sickle cell anemia usually do not need extra vitamins. Future research may show us ways to prevent painful episodes.
THE INHERITANCE OF DISEASESickle cell anemia is only one of many diseases that are inherited. Inherited diseases are passed from parents to children through the genes. Genes are what make the physical characteristics of a person such as height, eye color, features of the face, and blood type. Every kind of gene comes in twos with one gene inherited from the mother and one gene inherited from the father. When a person has sickle cell trait there is one gene for normal hemoglobin (A) and one gene for sickle hemoglobin (S). Because there is one gene for normal hemoglobin the person does not have a disease. The child with sickle cell anemia has 2 genes for sickle hemoglobin and this causes disease. Parents usually want to know if their other children could have sickle cell anemia. This can be learned by blood testing and is a good idea when one child is already known to have disease. Parents need to know that future children can also have disease. There is one in four chance or 25% chance that a child will be born with sickle cell anemia when both parents have sickle trait. When predicting the chances of having another child with disease it is important to remember that it is possible to have four children who all have sickle cell anemia. When both parents have sickle cell trait, each baby has a one in four chance of having sickle cell disease. Copyright & Disclaimer
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