Prepared by : Abdullah Rababah CI- BB & OPD NED-KFMC
the most common form of sickle cell disease(SCD).SCD is a serious disorder in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that thered blood cells are shaped like a crescent.
SCD*Normal red blood cells are disc-shaped and look likedoughnuts without holes in the center.*Sickle cells contain abnormal hemoglobin called sicklehemoglobin or hemoglobin S. Sickle hemoglobincauses the cells to develop a sickle, or crescent shape.*Sickle cells are stiff and sticky.
Overview*Sickle cell anemia is one type of anemia. *Anemia is a condition in which your blood has a lowerthan normal number of red blood cells. This conditionalso can occur if your red blood cells dont containenough hemoglobin
Overview*Normal red blood cells live about 120 days in thebloodstream and then die BUT the abnormal sicklecells usually die after only about 10 to 20 days..*Sickle cell anemia is an inherited, lifelong disease.People who have the disease are born with it.
Other Names for Sickle CellAnemia-HbS disease-Hemoglobin S disease-Hemoglobin SS disease-Sickle cell disease-Sickle cell disorders-Sickling disorder due to hemoglobin S
Sickle Cell Trait*People who inherit a sickle hemoglobin gene from oneparent and a normal gene from the other parent havesickle cell trait. Their bodies make both sicklehemoglobin and normal hemoglobin.
Example of an Inheritance Patternfor Sickle Cell Trait
Sickle cell anemia is inherited.Genetics2 copies of the genefor Hb (each parent)HbS –RecessiveS=SickleA=Normal
What Are the Signs and Symptomsof Sickle Cell Anemia?*The signs and symptoms of sickle cell anemia vary.Some people have mild symptoms. Others have verysevere symptoms and often are hospitalized fortreatment.*Sickle cell anemia is present at birth, but many infantsdont show any signs until after 4 months of age.*The most common signs and symptoms are linked toanemia and pain. Other signs and symptoms arelinked to the diseases complications.
Signs and Symptoms Related toAnemiaThe most common symptom of anemia is fatigue .Other signs and symptoms of anemia include:-Shortness of breath-Dizziness-Headaches-Coldness in the hands and feet-Pale skin-Jaundice
Signs and Symptoms Related toPain-This pain is called a sickle cell crisis. It often affects thebones, lungs, abdomen, and joints. -It occurs when sickled red blood cells block blood flow tothe limbs and organs. This can cause pain and organdamage.-Can be acute or chronic, but acute pain is more common.- Acute pain is sudden and can range from mild to verysevere. The pain usually lasts from hours to as long as aweek or more.
Cont . Of Crisis :Almost all people who have sickle cell anemia havepainful crises at some point in their lives. Some havethese crises less than once a year. Others may havecrises once a month or more. Repeated crises candamage the bones, kidneys, lungs, eyes, heart, andliver.
Notes :You can control some factors. For example, the risk ofa sickle cell crisis increases if youre dehydrated .Drinking plenty of fluids can lower the risk of a painfulcrisis.You cant control other factors, such as infections.Painful crises are the leading cause of emergency roomvisits and hospital stays for people who have sickle cellanemia.
Complications of Sickle CellAnemia-Acute Chest Syndrome-Multiple Organ Failure-Gallstones-Hand – Foot Syndrome-Splenic crisis leads to infection-Pulmonary hepertension-Stroke and Eye problem-Priapism
How Is Sickle Cell AnemiaDiagnosed?A simple blood test, done at any time during a personslifespan, can detect whether he or she has sicklehemoglobin. However, early diagnosis is veryimportant.In the United States, all States mandate testing for sicklecell anemia as part of their newborn screeningprograms
Cont.Doctors also can diagnose sickle cell disease before birth.This is done using a sample of amniotic fluid or tissuetaken from the placenta.
How Is Sickle Cell AnemiaTreated?The goals of treating sickle cell anemia are to relievepain; prevent infections, organ damage, and strokes;and control complications (if they occur).1.RBCX2.Treating pain3.Preventing complications and Treating if occurs.
New TreatmentsBlood and Marrow Stem Cell TransplantThe stem cells used for a transplant must come from aclosely matched donor. This limits the number ofpeople who may have a donor.The transplant process is risky and can lead to seriousside effects or even death.
New TreatmentsStudy & Research -Gene TherapyGene therapy is being studied as a possible treatment forsickle cell anemia. Researchers want to know whether anormal gene can be put into the bone marrow stem cells ofa person who has sickle cell anemia. This would cause thebody to make normal red blood cells.Researchers also are studying whether they can "turn off" thesickle hemoglobin gene or "turn on" a gene that makes redblood cells behave normally.
New MedicinesDecitabine. Like hydroxyurea, this medicine prompts thebody to make fetal hemoglobin. Fetal hemoglobin helpsprevent red blood cells from sickling and improves anemia.Decitabine might be used instead of hydroxyurea or addedto hydroxyureaAdenosine A2a receptor agonists. These medicines mayreduce pain-related complications in people who havesickle cell anemia5-HMF. This natural compound binds to red blood cells andincreases their oxygen. This helps prevent the red bloodcells from sickling.
How Can Sickle Cell Anemia BePrevented?can’t prevent sickle cell anemia, because it’s ………………
Living With Sickle Cell Anemia1.Adopt or maintain a healthy lifestyle-Follow a healthy diet.-take folic acid .-regular physical activity to stay healthy .-enough sleep and rest.-Stop smoking and alcohol
Cont.2.Take Steps To Prevent and ControlComplications-Ongoing Care-Emotional Issues and Support
Cont.3.Learn Ways To Cope With Pain-Work with your doctor to find ways to manage your pain.-heating pad, taking a hot bath, resting, or getting amassage.-Physical therapy might help ease your pain by helping yourelax and strengthening your muscles and joints.-find that activities that keep your mind off the pain, such aswatching TV .