Sickle cell disease is an inherited (passed from parent to child) disorder that affects the body’s red blood cells. In this disease, defective hemoglobin (a substance that carries oxygen in the blood) causes the red blood cells to change shape (into a sickle) when oxygen is released to tissues. Normal red blood cells are round and are able to move through small blood vessels in the body to deliver oxygen. In sickle cell disease, a chemical change in hemoglobin causes the substance to form long rods in the red blood cell as the hemoglobin releases oxygen. These rigid rods change the shape of the red blood cell into a sickle shape. The disease gets its name because the faulty blood cells are shaped like sickles used to cut wheat. When the deformed cells go through blood vessels, they become stiff, distorted, clog the blood flow, and can break apart. Individuals with sickle cell disease have hemoglobin "S," which may be homogenous or also be in combination with other abnormal hemoglobins (Hemoglobin C; Beta thalassemia trait or other rare Hemoglobin variants). Others without this disease have hemoglobin "A", which is the normal adult hemoglobin. Sickle cell disease is found most often in African-Americans and Africans. However, other ethnic groups also can have sickle cell disease. In many states, the law requires newborn babies to be tested for sickle cell disease, regardless of ethnic background. Abstract source

Several diseases can be spread when people have sex. They are called sexually transmitted diseases (STDs). These diseases are common in teenagers and young adults. Some teens today are having sex at an early age. They may even have more than one sex partner, and they are not always using protection. For these reasons, STDs are a growing problem in people under the age of 25. Teens and young adults with sickle cell disease can make and have babies. If you choose to have sex and you do not want to have children, you must use birth control. Both males and females need to think about birth control. The only form of birth control that will prevent both pregnancy and STDs is a condom with a spermicide. A condom looks like a long balloon that is placed over a man’s penis. Spermicide is a foam, cream, or jelly, which contains chemicals that kill sperm. Abstract source

Moving from pediatric (children’s) care to adult care can be one of the hardest changes you will face as a patient with sickle cell disease. As a family you must find a balance between concerned parents who want to handle all the details and teens who want to be completely independent. Many issues must be considered when moving from a pediatric hematologist (sickle cell doctor) to a hematologist who treats adults. This booklet will help you address some of these issues. Feel free to ask the St. Jude staff questions you might have about making this transition (change). At the back of the booklet are several pages for you to write notes and questions. You will also find a glossary of terms that you might encounter during this time of change.   Abstract source

Living with chronic pain can be devastating, and effective pain management is important to getting your life back. It is essential that you and your doctor discuss treatment options, carefully considering all of the risks and benefits. Some medications, such as prescription opioids, can help relieve pain in the short term but also come with serious risks and potential complications—and should be prescribed and used carefully.   Abstract source

Sickle cell disease is an inherited disorder of the red blood cells. There are several different types of sickle cell disease. Normal red blood cells are shaped like disks and can move normally through the blood vessels. In sickle cell disease, the iron-rich protein called hemoglobin forms strands inside the blood cells. These strands cause the cell to be sickle (U-shaped). Sickle cells cannot move normally through the blood vessels, because they are stiff and sticky. They block blood flow in the blood vessels, keeping blood and oxygen from reaching the tissues. Blocked blood flow causes pain that might last hours or days. It can also damage many major organs in the body. Children, especially young children, with sickle cell disease are also at higher risk of serious infection. Abstract source

Splenic sequestration is a problem with the spleen that can happen in people who have sickle cell disease. Splenic sequestration happens when a lot of sickled red blood cells become trapped in the spleen. The spleen can enlarge, get damaged, and not work as it should. When the spleen doesn't work well, a person is more likely to have serious, life-threatening infections with certain types of bacteria. If splenic sequestration happens suddenly, it can be a life-threatening emergency. Abstract source

If a person with sickle cell disease is infected with parvovirus, the virus that causes fifth disease in children, an aplastic crisis may develop. Bone marrow suddenly stops producing red blood cells, which results in sudden and severe anemia. During this time, a person will often feel tired, have pale skin, and be short of breath.Blood transfusions might be done to treat an aplastic crisis. After a few days, the bone marrow usually recovers on its own. And red blood cell production returns to its usual rate.Most aplastic crises occur in children. This condition only occurs once in any person. Abstract source

Stem cell transplantation is a potential cure for sickle cell disease. Stem cells can be found in bone marrow. Bone marrow is the substance in the center of your bones that produces red blood cells. A person with sickle cell disease has bone marrow that produces red blood cells with defective hemoglobin S. But if that bone marrow is replaced with healthy bone marrow, a person's body may start to produce normal hemoglobin. Stem cell transplants require bone marrow from another person (donor). This is called an allogeneic stem cell transplant. Before the transplant, bone marrow stem cells are taken from someone who has closely matching bone marrow, usually a healthy brother or sister. The child who has sickle cell disease is then treated with drugs that destroy his or her bone marrow cells. After that, the donated bone marrow stem cells are injected into a vein. After the process is complete, the donor's bone marrow begins to replace the recipient's bone marrow. These new cells restore the immune system and make normal red blood cells.   Abstract source

Sickle cell disease turns normal, round red blood cells into misshaped cells that look like sickles or crescent moons. The sickle-shaped cells can get stuck in blood vessels, blocking blood flow and causing severe pain. The sickle-shaped cells also can harm organs, muscles, and bones. It is a lifelong condition that causes anemia and puts your child at risk for bacterial infections. Sickle cell disease is passed down in families. Your doctor also may recommend that other family members get tested for sickle cell disease. Your doctor may treat your child with medicines. Some children get blood transfusions or a bone marrow transplant. Managing pain and preventing bacterial infections are important parts of your child's treatment. Follow-up care is a key part of your child's treatment and safety. Be sure to make and go to all appointments, and call your doctor if your child is having problems. It's also a good idea to know your child's test results and keep a list of the medicines your child takes. Abstract source

A sickle cell test is a blood test done to check for sickle cell trait or sickle cell disease. Sickle cell disease is an inherited blood disease that causes red blood cells to be deformed (sickle-shaped). The red blood cells deform because they contain an abnormal type of hemoglobin, called hemoglobin S, instead of the normal hemoglobin, called hemoglobin A.Sickled blood cells are destroyed by the body faster than normal blood cells. This causes anemia. Also, sickled cells can get trapped in blood vessels and reduce or block blood flow. This can damage organs, muscles, and bones and may lead to life-threatening conditions.The best way to check for sickle cell trait or sickle cell disease is to look at the blood using a method called high-performance liquid chromatography (HPLC). This test identifies which type of hemoglobin is present. To confirm the results of HPLC, a genetic test may be done. Abstract source