Thursday, February 12, 2009

SICKLE CELL DISORDER

Sickle cell disorder is an inherited disorder of haemoglobin in which there are not enough healthy red blood cells to carry oxygen throughout the body in any person who has inherited a sickle haemoglobin from each parent(e.g Hb SS) or sickle haemoglobin from one parent and another haemoglobin variant from the other parent(e.g Hb SC).

Normally,the red blood cells are flexible and round and they move easily through the red blood vessels to carry oxygen to all parts of the body.In persons with sickle cell disorder,the red blood cells are rigid and sticky and are sickle-shaped;hence,the name "sickle" cell disorder.These irregular-shaped blood cells die prematurely,resulting in chronic shortage of red blood cells.In addition,the sickle-shaped cells can get stucked in small blood vessels,which can slow or block blood flow and oxygen to certain parts of the body.This is why persons with sickle celldisorder suffer pains,poor circulation of blood and oxygen,anaemia,poor resistance to infections and other serious complications.

Sickle cell anaemia is predominant in malaria-infested regions,especially Africa.People with sickle cell anaemia are particularly susceptible to malaria.That means they are easily affected by malaria because they are already anaemic(short of red blood cells and haemoglobin) and malaria makes the anaemia worse.Malaria infection can therefore lead to death from severe anaemia unless treated or prevented promptly.This is why it is believed that people with sickle cell anaemia have a short lifespan.But recent study has shown that with prooper care and management,they can live for as long as possible.

People with sickle cell trait(i.e Hb AS) have better chance of survival because when malaria parasite affects their red blood cells,the cells become sickle-shaped and thereafter the cells loose potassium which causes the parasite to die.The protection afforded by the sickle cell trait keeps the allele for a sickle prevalent in areas exposed to malaria.

SIGNS AND SYMPTOMS
People with sickle cell trait(carriers) have one gene for the disorder.They do not develop the disorder and ususally have no sign nor symptom.
People with sickle cell anaemia have two genes for the disorder and one from each parent.They usually show some signs after four months of age.Some people with sickle cell anaemia have mild symptoms while others have severe symptoms which call for frequent hospitalization.The signs and symptoms of sickle cell include:
HAND-FOOT SYNDROME:swollen hand and feet are usually the first signs of sickle disorder in babies.The swelling is caused by sickle-shaped red blood cells blocking blood flow out of the hands and feet.Hand-foot syndrome is often accompanied by pain and fever.
ANAEMIA:the lifespan of normal red blood cells in people with haemoglobin AA is 120 days(4 months),while the lifespan of red blood cells in people with sickle cell(Hb SS) is about 10 days.As a result,affected people have fewer red blood cells and les haemoglobin than usual in their blood-this is an anaemic condition.Without enough red blood cells in circulation,the body cannot get the oxygen needed to feel energized.This is why anaemia causes tiredness and lower eye-lid appear pale.This pale condition is described as pallor.
PAIN CRISES:Periodic episodes of pain,known as crises is a major symptom of sickle cell anaemia.Pain develops when rigid sickled red blood cells cannot alter their shape and squeeze through small blood vessels.They easily stick together and form a logjam and this obstructs the flow of blood causes pain.The pain vary in intensity and can last for a few hours to several days.If a crises is severe enough,hospitalization maybe needed for painkillers to be injected into the veins.
JAUNDICE:Jaundice is the yellowing of the eyes and skin that occur as a result of liver damage or malfunction.Sometimes,people who have sickle cell anaemia have some degree of jaundice because the liver,which filters harmful substances is overwhelmed by excessive breakdown of red blood cells.
FREQUENT INFECTION:Sickle cell can damage the spleen,an organ that fights infection.This may make one vulnerable to infections.
COMPLICATIONS:sickle cell disorder can lead to a host of complications which includes;
ACUTE CHEST SYNDROME:it is a life-threatening complication that causes chest pain,fever and breathing difficulty.It is similar to pneumonia but caused by a lung infection or trapped sickle cells in the lung.It requires urgent medical attention..Re-occurrence of attack can damage the lung.
STROKE:stroke can occur if sickle cells block the flow of blood to an area of the brain.Stroke is one of the most serious complications of the disorder.Signs of stroke include include seizures,sudden speech difficulties,numbness or weakness of arms and legs and loss of consciousness.Stroke can be fatal so if any of these signs and symptoms is observed in a child,medical treatment should be sought immediately.
ORGAN DAMAGE:sickle cells can block blood flow through the blood vessels,depriving an organ of oxygen and blood.Blood is also chronically low in oxygen in sickle cell anaemia.Chronic deprivation of oxygen-rich blood can damage nerves and organs in the body,including kidney,liver and spleen.Organ damage can be fatal.
OTHER COMPLICATIONS:sickle cell disorder can cause open sores in the leg,called ulcer.Sickle cells can block blood vessels that nourish the skin,causingg skin cells to die.Gallstone is another possible complication.The breakdown of red blood cells produce a substance called bilirubin.Bilirubin is responsible for the yellowing the eyes and skin(jaundice) in people with sickle cell disorder.A high level of bilirubin in the body can lead to gallstone.Men with sickle cell disorder may experience an unwanted and painful erection which goes on for longer than 20 minutes,this condition is known as priapism.Sickle cells can prevent blood flow out of an erect organ.Overtime,priapism can damage the male organ and lead to impotence and sterility in men with sickle cell disorder.

WHAT TO DO
If you or someone in the family has sickle cell disorder,there is need to help in handling the stress of coping with this lifelong disorder.Talk to you doctor about your concerns.If your child has sickle cell disorder,the best way to help is to learn as much as possible about the disorder and make sure the child gets the best health care possible.A child with sickle cell disorder has special needs and requires regular medical care to stay as healthy as possible.The doctor can explain how often the child should be brought for medical care and what to do if he/she becomes ill.You may also let teachers and caregivers know about the child's illness.Help them understand the kind of exercises and situations that are harmful to the child,and teach them to recognize signs of illness and infections.

Taking steps to stay healthy is critcal to anyone with sickle cell disorder.Eating well,getting adequate rest and protecting yourself against infections are good ways to maintain your health and prevent crises.

SELF CARE:if you or someone close to you have sickle cell disorder,the following suggestions can be followed to stay healthy:

  • Take folic acid supplements daily and eat well-balanced diet.Bone marrow needs folic acid and other vitamins to produce new red blood cells.Vitamin B-complex and paludrine can also be taken daily to prevent malaria.
  • Drink plenty of water.Staying hydrated help keep the blood diluted,which reduces the chance of sickle formation.
  • Avoid stress.A sickle crises can occur as a result stress.Know your limit to avoid crises.
  • Avoid temperature extremes.Exposure to extreme heat or cold can trigger the formation of sickle cells.
  • Avoid mosquito bite as much as possible.A treated mosquito net can be used and window net too.
  • Avoid staying in over-crowded places.Stay in a well ventilated place.

PREVENTION

Intending couples should go foe premarital screening(including genotype test) to ensure compatibility.In my opinion,intending couples should go for counselling to be able to make informed decisions so that they can avoid having offspring with sickle cell disorder.And for those who already have it,u guys are wonderfully and fearfully made!

These people need our love and care.Let us support them!Thank you and God bless you all.