Tae W. Kim M.D.
Texas Children's Hospital, Houston Texas
What is Sickle Cell Disease?
Sickle Cell Disease represents a collection of medical problems related to an inherited genetic disorder. Normal red blood cells contain a protein called hemoglobin A. The hemoglobin in the red blood cell is responsible for holding onto the oxygen in the red blood cell, until it is time to release the oxygen to different parts of the body. The red blood cells in sickle cell disease contain an abnormal protein called hemoglobin S. This hemoglobin S may cause the red blood cell to change shape from a normal round shape to abnormal sickle shape under such conditions as low oxygen, infection or other body stress.
How would I know if my child has sickle cell disease?
Sickle cell disease is not contagious; it is a genetic disorder, which is passed on to the children of families with backgrounds from Sub-Saharan Africa, the Mediterranean countries, and India. If your child was born in the United States, there is a very good chance your child's newborn screening included the blood test for the sickle cell gene.
Every state has a newborn screening center from which your pediatrician or your child's primary care physician can obtain the results of the newborn screening test. Newborn screening tests are especially important for those children of African American background and for those children, whose parent's genetic background is unknown. Eight percent of the African American population has sickle cell trait, and 0.2% has sickle cell anemia.
What is the difference between sickle cell trait and sickle cell anemia?
Normal red blood cells contain hemoglobin A and some other minor types of hemoglobin. Children with sickle cell trait have red blood cells mainly containing hemoglobin A and some hemoglobin S. Children with sickle cell anemia have red blood cells with mainly hemoglobin S. There are other forms of abnormal red blood cells referred to as the thalassemias, which can affect the severity of your child's blood disorder. These are very rare, and should be discussed thoroughly with your child's physician.
My child has sickle cell disease and is scheduled to have surgery with anesthesia, should I be worried?
No. Anesthesiologists are aware of the interaction between anesthesia and the medical problems associated with sickle cell disease. It is best to check with your anesthesiologist and alert him/her to your child's condition and inquire about any specific information the anesthesiologist may need to properly care for your child.
What can I do as a parent to ensure my child is properly prepared for surgery and anesthesia?
It is very important to have a note from the hematologist concerning your child's sickle cell status, recent illnesses, hospitalizations, laboratory results and history of blood transfusions in anticipation of your child's surgery. Also, like any other child coming for surgery, please follow the preoperative instructions provided by your doctor.
Your child may be at a higher risk for a sickle cell crisis if he/she is denied liquids for a prolonged period. Therefore, try to make arrangements for minimizing this time by discussing it with your surgeon and/or the anesthesiologist. Some hospitals require intravenous fluid hydration prior to surgery, and if this is the case, it may be best to let your child know ahead of time and explain the reason for the intravenous fluids.
Will my child need a blood transfusion?
This really depends on your child's type of sickle cell disease, severity of anemia and complexity of the operation. Children with sickle cell trait (AS) are treated in the same manner as any other child with normal hemoglobin (AA). However, children with sickle cell anemia (SS) tend to have chronic anemia (low blood count) and require periodic blood transfusions. Unfortunately, the exposure to multiple blood transfusions may make it difficult to find compatible blood for your child, and therefore advance planning is necessary with your hematologist, the surgeon and anesthesiologist.
What should I expect after the operation in the recovery room?
If your child has sickle cell anemia, they will probably receive oxygen on arrival to the recovery room, and depending on how your child is recovering, the extra oxygen may be continued for a longer period of time. Intravenous fluids will be continued and pain medication will be provided as needed. When your child has met the discharge criteria, he/she may go home if they are an outpatient, or be discharged to their hospital room.