The author describes sickle cell disease(SCD) as a genetic condition, which is inherited when a child receives twosickle cell genes – one from each parent. Sickle cell occurs when the red bloodcells are rigid and sickle-shaped, which causes them to stick together andblock oxygen to the body. A large number of infants are born with SCD annually;today every newborn is screened for the sickle cell gene. In this article theauthor covers the following topics pain, acute chest syndrome (ACS), infection,stroke, and available treatment options. Fisher mentions that adults andchildren with SCD must be continuously checked for pain with the use of theappropriate pain scale. There are several natural pain treatment approachessuch as, acupuncture, physical therapy, massage, hydration, behaviormodification, deep breathing, imagery, and distraction.
Medicated treatmentincludes administration of IV fluids, opioids or nonsteroidal anti-inflammatorydrugs and oxygen if hypoxia is present. One of the conditions is Acute ChestSyndrome (ACD), it is a rapid deterioration in respiratory function, and it is causedby vaso-occlusion in the vessels of the lungs. Due to severe pain and usingheavy narcotics, there is a large possibility for the decreased respiratoryeffort, which may lead to ACD. Those with ACD are recommended to use anincentive spirometer to promote deep breathing, and blowing efforts for youngerages.
ACD is the most common cause of death amongst young adults.Infection, it is one of the major conditionsthat cause death in SCD patients called Streptococcus preumoniae sepsis. Thepatients who experience fever should be treated as an emergency and requireintervention as soon as possible, including blood cultures, complete bloodcount, chest radiographs, IV fluids, and IV Antibiotics. The author describes Transcranial Doppleras one the great imagining tools to determine whether a child with SCD is atrisk of a stroke. This tool is used regularly and is only to be used forchildren. If the stroke is suspected, the patient must be stabilized to patientto prevent any brain damage, and an exchange transfusion should be performed rightaway. A Chemotherapeutic agent that has beenshown to decrease vaso-occlusive episodes and incidence of ACS is called Hydroxyurea.
Hydroxyurea therapy is one of theavailable pain treatments, which results in less need for blood transfusion andis not used in the acute state. The bone-marrow transplant is rarelyused; due to high death rate from complications in children who receive a bonemarrow transplant. Finding a match through the Bone Marrow Registry isextremely difficult.