My Little Brother Has Sickle Cell Anaemia, What’s That?
Posted on 15. Feb, 2010 by afyakenya in Blog
Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin* S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels.
When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There is currently no universal cure for sickle cell disease.
Hemoglobin – is the main substance of the red blood cell. It helps red blood cells carry oxygen from the air in our lungs to all parts of the body. Normal red blood cells contain hemoglobin A. Hemoglobin S and hemoglobin C are abnormal types of hemoglobin. Normal red blood cells are soft and round and can squeeze through tiny blood tubes (vessels). Normally, red blood cells live for about 120 days before new ones replace them.
People with sickle cell conditions make a different form of hemoglobin A called hemoglobin S (S stands for sickle). Red blood cells containing mostly hemoglobin S do not live as long as normal red blood cells (normally about 16 days). They also become stiff, distorted in shape and have difficulty passing through the body’s small blood vessels. When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease.
What is Sickle Cell Trait?
Sickle Cell trait (AS) is an inherited condition in which both hemoglobin A and S are produced in the red blood cells, always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy.
Inheritance
Sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical traits. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets…one from each parent.
Examples:
If one parent has Sickle Cell Anemia and the other is Normal, all of the children will have sickle cell trait.
If one parent has Sickle Cell Anemia and the other has Sickle Cell Trait, there is a 50% chance (or 1 out of 2) of having a baby with either sickle cell disease or sickle cell trait with each pregnancy.
When both parents have Sickle Cell Trait, they have a 25% chance (1 of 4) of having a baby with sickle cell disease with each pregnancy.
How will I know if I have the Trait?
A SIMPLE PAINLESS BLOOD TEST followed by a laboratory technique called Hemoglobin Electrophoresis will determine the type of hemoglobin you have. When you pass an electric charge through a solution of hemoglobin, distinct hemoglobins move different distances, depending on their composition. This technique differentiates between normal hemoglobin (A), Sickle hemoglobin (S), and other different kinds of hemoglobin (such as C, D, E, etc.).
Medical Problems
Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones.
The sickle cells also block the flow of blood through vessels resulting in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest and abdomen), stroke and priapism (painful prolonged erection). It also causes damage to most organs including the spleen, kidneys and liver. Damage to the spleen makes sickle cell disease patients, especially young children, easily overwhelmed by certain bacterial infections.
Treatment
Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation.
Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusion and surgery all backed by psychosocial support. Like all patients with chronic disease patients are best managed in a comprehensive multi-disciplinary program of care.
Blood transfusions help benefit sickle cell disease patients by reducing recurrent pain crises, risk of stroke and other complications. Because red blood cells contain iron, and there is no natural way for the body to eliminate it, patients who receive repeated blood transfusions can accumulate iron in the body until it reaches toxic levels. It is important to remove excess iron from the body, because it can gather in the heart, liver, and other organs and may lead to organ damage. Treatments are available to eliminate iron overload.
Promising Treatment Developments
In search for a substance that can prevent red blood cells from sickling without causing harm to other parts of the body, Hydroxyurea was found to reduce the frequency of severe pain, acute chest syndrome and the need for blood transfusions in adult patients with sickle cell disease. Droxia, the prescription form of hydroxyurea, was approved by the FDA in 1998 and is now available for adult patients with sickle cell anemia. Studies will now be conducted to determine the proper dosage for children.
Other treatment options in clinical development include new, more convenient options than current therapies to eliminate iron overload caused by repeated blood transfusions.
Regards,
Oduwo Noah Akala
Chairman,
Afya Kenya Foundation.
http://adriananthonys.nexo.com/blog
My daughter is 15 years of age and is in form three, are there doctors who are specialised in Sickle cell a nemia. what are my daughters chances of living a normal life.
My daughter was born with sickle cell anaemia (disease). We’ve struggled with the disease with her being in and out of hospital for many years. She passed away March 7, 2010 at the age of 30 years. She went to school and University of Nairobi. She graduated with a Bachelor of Law from the University of Nairobi. She had just finished her Diploma in Law from the Kenya School of Law. I want to encourage parents with children suffering from sickle cell disease not to give up. There is a new treatment method developed in the United States which can reverse the sickle cell trait. After the death of my daughter I wanna help children suffering from sickle cell disease. Please contact me: Lennox Obuong Telephone NO: 0722 – 833100 or email: obuong3@aol.com
Hi Carol,
I also have a 5yr old with sickle cell. I am so sure with good care he will be okay. What I can advice you is to gather as much information as possible on this condition( I never want to call it a disease) and use that as a weapon to take care of that gal.
At first when I discovered that my child had sickle cell I was so devastated; through reading and sharing, I have realized that there are so many out there who has the same condition only that they are not talking about it.
With proper care…they will lead a near normal life…we need to create a positive attitude in them and instill a lot of discipline in them so that they can learn what to do the right things for themselves.
Any Kenyan caregiver of a sickle cell diva or warrior can reach me through the email…eshimuli@gmail.com…I am ready to share my case.
Thanks
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My heart goes out to all the families afflicted by Sickle Cell Anaemia.My son was diagnozed with Sickle Cell Anaemia at the age of 6 months. Like most parents, I was devastated when we found out;but my husband and I have learned that through a lot of prayer, regular Doctor’s visits and pain managment at home, we can sometimes prevent a crisis before it happens.
Our son is now 18 months old and we currently live in South Africa where he goes for regular blood counts and check ups every 2 to 3 months. We are thinking of relocating to Kenya and I was wondering if anyone can recommend a hospital or Pediatric Hematologist in Nairobi, because I don’t know where to start looking. I would rather have all the information I need before we move back. I would really appreciate any information that anyone can give me. Thank you.
Katila
e-mail: katila23@gmail.com
Dear Katila,
Maybe you have already received help. If not I recommend Dr. Nyambura Kariuki of Gertrude’s Children Hospital or DR. K.C. wafula of Aga Khan Hospital Nairobi.
Best regards
Grace
Hi My names are George Adeka and i have a 5 Year old sickler.
She was diagonised with sickle cell at 4 months.
I would like to encorage everyone not to loose hope.
Sicklers require warmth and support.
I would also like to share with everyone on some herbal treatment called Nicosan. The drug helps manage the condition very well and boost formation of more rounded Red blood cells.
The herbal treatment has to be taken daily and it cutsdown on frequent transfusions and greatly lowers number and duration of painfull crisis.
I get the drugs from Nigeria through post and they have worked fine with me. feel free to call me on 0720 676910 for more details.
r,
Adeka
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