GUIDE OF MEDICAL COLLEGE & HOSPITAL
K C G Medical College, Berhampur
C B Medical College, Cuttack
S S Medical College, Burla, Sambalpur
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- October 15, 1999 --Nitric
Oxide May Play Role In Treatment Of Sickle Cell
Increasing levels of nitric oxide in the blood may
offer new options for treating sickle cell anemia,
the most common inherited blood disorder in the
country. In a study described in this
month’s Journal of Clinical Investigation,
researchers at the Warren Grant Magnuson Clinical
Center at the National Institutes of Health are
examining how nitric oxide acts on hemoglobin, the
oxygen-carrying molecule in red blood cells. Nitric
oxide, a gas produced in many parts of the body,
relaxes the smooth muscle cells in blood vessels. As
the muscles relax, the vessels expand and allow more
blood to pass through. Improving blood flow is a
critical aspect in treating the painful and
potentially deadly complications associated with
sickle cell anemia. The Clinical Center
(CC) study demonstrates that when nitric oxide is
inhaled, it binds to hemoglobin. Once attached, the
gas hitches a ride through the blood stream, and may
dialate vessels as it passes through.
- 3/2/99 -- Sickle
Cell Effects On Brain Found In Very Young Children
Sickle cell disease alters the brains of
very young children long before strokes or learning
disabilities announce brain damage, suggest the
results of a study published in this month’s issue
of the Annals of Neurology. "It
appears that patients may be suffering brain damage
at an earlier age than we thought before," said
R. Grant Steen, Ph.D., an assistant professor of
diagnostic imaging at St. Jude Children's Research
Hospital in Memphis, TN., and one of the authors of
the study. Steen and his colleagues used
a form of magnetic resonance imaging (MRI) not
typically employed in clinical medicine to
demonstrate subtle changes in the brains of children
with the disease who had not shown any
manifestations of neurological damage.
The results promise to spark debate about whether
risky -- but potentially protective -- measures such
as blood transfusions should be employed in younger
patients without severe symptoms of the disease.
Cell Disease in Newborns and Infants
A Guide for Parents --
National Library of Medicine
is sickle cell disease?
Sickle cell disease is an
inherited disorder of the red blood cells. Red blood
cells carry oxygen to all parts of the body by using a
protein called hemoglobin. Normal red blood cells
contain only normal hemoglobin and are shaped like
doughnuts. These cells are very flexible and move
easily through small blood vessels.
But in sickle cell disease,
the red blood cells contain sickle hemoglobin, which
causes them to change to a curved shape (sickle shape)
after oxygen is released. Sickled cells become stuck
and form plugs in small blood vessels. This blockage
of blood flow can damage the tissue. Because there are
blood vessels in all parts of the body, damage can
occur anywhere in the body.
most common types of sickle cell disease are:
- Sickle cell anemia
- Hemoglobin SC disease
- Sickle beta-thalassemia
This booklet can help you
understand sickle cell disease and how it can affect
The best way to help your
baby is to learn as much as you can about the disease,
the problems it can cause, and what you can do to care
for your baby. Talk about this booklet and your baby's
health care choices with your doctor and others who
know about sickle cell disease. Working together, you
can give your child the best possible care.
You will find a description
of the kinds of problems a baby with sickle cell
disease may have on below of
this booklet. Remember when you read it that not all
babies will have all of these problems. At the back of
this booklet, you will find a list of terms often used
by doctors and nurses when they talk about sickle cell
There are several forms of
sickle cell disease. The most common is sickle cell
anemia. Your doctor or nurse will tell you what kind
of sickle cell disease your baby has. Be sure to write
down the name so that you can refer to it if your baby
has to go to a new doctor or clinic.
Babies with sickle cell
disease may have:
- Anemia (a low
number of red blood cells). People with anemia may
- Aplastic crisis. Babies
with sickle cell disease may stop making red blood
cells for a short time. Signs include paleness,
less activity than normal, fast breathing, and
fast heartbeat. A baby with these signs must be
seen quickly by the doctor.
- Hand-and-foot syndrome.
Babies with sickle cell disease may have pain
and swelling in their hands or feet.
- Painful episodes (mostly
in the arms, hands, legs, feet, or abdomen). This
happens when sickle cells plug blood vessels and
block the flow of blood. Doctors call this a
painful episode, event, or crisis.
- Severe infections. The
child with sickle cell anemia is at great risk for
serious infections -- such as sepsis (a blood
stream infection), meningitis, and pneumonia. The
risk of infection is increased because the spleen
does not function normally.
- Splenic sequestration
crisis. The spleen is the organ that filters
blood. In children with sickle cell disease, the
spleen can enlarge rapidly from trapped red blood
cells. This condition is called splenic
sequestration crisis and can be life-threatening.
- Stroke. This
happens when blood vessels in the brain are
blocked by sickled red blood cells. Signs include
seizure, weakness of the arms and legs, speech
problems, and loss of consciousness. A baby with
any of these signs must be seen quickly by a
In the United States, most
people who have sickle cell disease are African
Americans. About 1 in 375 African-American children
has sickle cell disease. Hispanic Americans from the
Caribbean, Central America, and parts of South America
also may have the disease. Sickle cell disease is also
found in individuals from Turkey, Greece, Italy, the
Middle East, or East India.
All forms of
sickle cell disease are inherited. Children
inherit genes for the disease from their parents.
Genes are substances within
the father's sperm and the mother's egg that determine
all of the physical characteristics of a baby.
Children inherit the genes for hemoglobin from their
parents. Persons who inherit both normal and sickle
hemoglobin have sickle cell trait. Sickle cell trait
is not a disease and does not change to disease. The
individual sperm or egg from a person with sickle cell
trait may contain either a gene for normal hemoglobin
or a gene for sickle hemoglobin.
- When both parents have
sickle cell trait, for each pregnancy, the chances
are: 1 in 4 that the baby will have only normal
- 2 in 4 that the baby will
have both normal and sickle
- hemoglobin (sickle cell
- 1 in 4 that the baby will
have only sickle hemoglobin (sickle cell anemia).
The inheritance of other
forms of sickle cell disease can be explained by your
All newborn babies should be
tested for sickle cell disease. Many States have
screening programs that test babies born in the
hospital within a few days of birth. A blood sample is
taken from the baby's heel for the sickle cell test,
as well as screening tests for several other medical
If the test shows your baby
might have sickle cell disease, the doctor will do the
test again to make sure. The doctor may ask one or
both parents for blood samples to test. If your baby
has sickle cell disease, the doctor will tell you as
soon as possible.
If your baby has sickle cell
disease, the doctor will help you find the best
medical care for your child. This care could be
provided by your family doctor, a pediatrician
(children's doctor), or a pediatric hematologist
(children's blood specialist), or a special sickle
cell clinic. You also may want to see a counselor who
can talk with you about your chances of having another
baby with sickle cell disease.
Sickle cell disease is not
just a medical problem. You may have many concerns
about your baby and your family--for example, how to
cope with your feelings and how to pay the medical
bills. Your doctor or nurse can talk with you about
your concerns. They also can help you find a local
social service agency to assist you. In many areas
there are sickle cell support groups, as well as
community organizations that offer testing, education,
and support to families affected by sickle cell
The best way to help your
baby is to learn as much as you can about the disease
and to make sure your baby gets the best health care
possible. The child with sickle cell disease has
special needs and must have regular medical care to
stay as healthy as possible. The doctor or nurse will
explain how often to bring your baby for medical care
and what you can do if your baby becomes ill.
By 2 months of age, your baby
should start taking penicillin by mouth twice each
day. It is very important to give the medicine
exactly as the doctor tells you. This will help
prevent life-threatening infections. Penicillin should
be continued until at least 5 years of age.
Also, by 2 months of age,
your baby will get a shot to protect against H.
influenzae, a type of bacteria that causes an
infection which can be dangerous to people with sickle
cell disease. The baby also will need a shot to
protect against hepatitis B, a liver disease. At age
2, your child should receive pneumococcal vaccine.
Your child should have all the other shots that
children normally receive.
Here are some of the most
important things you need to know about caring for a
baby with sickle cell disease:
If your baby has a fever
(over 101 degrees), you must get medical help right
away. A fever in a child with sickle cell disease
can be a sign of serious medical problems. Always take
your baby's temperature when your baby appears sick.
Your doctor will tell you what to do if your baby has
If your baby is sick, you
must get medical help right away. Any
sign of illness in a child with sickle cell disease
can be serious. Your baby needs to see the doctor
quickly if the baby:
- Is breathing fast or
having a problem with breathing
- Coughs frequently
- Is cranky and cries more
- Screams when touched
- Is very tired or has
- Is very weak
- Does not want to eat
- Has diarrhea
- Has fewer wet diapers
- Has pain or swelling in
- Has swollen hands or feet
- Has pale blue or grey lips
If any new doctor or health
care provider sees your baby for any reason, explain
that your baby has sickle cell disease.
A good diet is very important
for all babies. Ask your doctor or other health care
provider about the right foods and liquids for your
baby. Make sure your baby drinks plenty of liquids.
Find out if your baby also should have vitamins or
Make sure your baby does not
become overheated or chilly. Keep your baby warm. Cold
baths or cold air can slow the baby's blood flow and
You should always feel free
to ask any question about sickle cell disease and how
it affects you and your family. Here are some
questions you may want to ask the doctor, nurse,
counselor, or social worker. There is room at the end
of the list for you to write your own questions.
- What does my baby have?
How did he or she get it?
- What do I have? How did I
get it? How will it affect me and my family?
- How often does my baby
need to see you?
- What medicine does my baby
need? What do I need to know about giving it?
- What should my baby eat
- Is there anything my baby
should not do?
- How can I tell if my baby
- What should I do, and who
should I call, if my baby gets sick?
- What other help is
available to my family?
To learn more about sickle cell
disease and how to cope with it, contact:
- National Association for
Sickle Cell Disease
- 3345 Wilshire Boulevard
- Suite 1106
- Los Angeles, CA 90010-1880
- Telephone: 1-800-421-8453
Additional organizations that
can provide help include:
- California State
Department of Health
- Childrens Medical Services
- Sacramento, CA 95814
- Telephone: 916-654-0499
- Cincinnati Comprehensive
Sickle Cell Center
- Children's Hospital
- Cincinnati, OH 45229
- Telephone: 513-559-4200
- Clinical Center
- 9000 Rockville Pike
- Building 10, Room 1C255
- Bethesda, MD 20892
- Telephone: 301-496-2563
- Education Programs
- 1 West Campbell Ave,
- Campbell, CA 95008
- Telephone: 408-374-1210
- Howard University
- Comprehensive Sickle Cell
- 2121 Georgia Ave
- Washington DC 20059
- Telephone: 202-806-7930
- March of Dimes
- Birth Defects Foundation
- 1275 Mamaroneck Avenue
- White Plains, NY 10605
- (For faster service, look
in the telephone book for a local March of Dimes
chapter in your area)
- Mid-South Sickle Cell
- Le Bonheur Children's
- Memphis, TN 38103
- Telephone: 901-522-6792
- Mississippi State
Department of Health
- Genetics Division
- P.O. Box 1700
- Jackson, MS 39215
- Telephone: 601-960-7619
- National Maternal and
Child Health Clearinghouse
- 8201 Greensboro Drive
- Suite 600
- McLean, VA 22102
- Telephone: 703-821-8955
- New York State Department
- Newborn Screening Program
- Wadsworth Center for
Laboratories and Research
- P.O. Box 509
- Albany, NY 12201-0509
- Telephone: 518-473-7552
- Northern California
- Sickle Cell Center
- San Francisco, CA 94110
- Telephone: 510-428-3651
- Texas Department of Health
- Newborn Screening Program
- 1100 West 49th St
- Austin, TX 78756-3199
- Telephone: 512-458-7111
This is not a complete list.
Check with your state or local health department or
sickle cell agency for other sources of information.
Your doctor, nurse, or other
caregiver may use these terms in talking with you
about sickle cell disease and your child.
- Acute chest syndrome.
- A serious condition caused
by infection or trapped red blood cells in the
lungs. Fast or difficult breathing, chest pain,
and coughing are signs of acute chest syndrome in
the child with sickle cell disease. A child with
acute chest syndrome usually will have to go to
the hospital for treatment.
- A reduced number of red
blood cells. Anemia occurs in persons with sickle
cell disease because sickled red blood cells do
not live as long as normal red blood cells. A
child with sickle cell disease cannot make red
blood cells fast enough to keep up with the rapid
breakdown, so the person with sickle cell disease
has fewer red blood cells than normal and is
- Aplastic crisis.
- Occurs when a child's bone
marrow temporarily stops producing red blood
cells. A child with aplastic crisis may appear
pale and be tired and less active than usual.
- Tiny blood vessels where
sickle-shaped blood cells may get trapped and
- The biological units that
are passed from both parents to a child. Genes
determine all of the child's characteristics --
for example, hair, eye, and skin color, foot size,
height -- and whether the child will have sickle
cell disease or another inherited disease.
- Haemophilus influenzae.
- A type of bacteria that
causes infection and can lead to serious problems
in the child with sickle cell disease. Babies must
receive a special vaccine beginning at 2 months of
age to protect them from this condition.
- Hand-and-foot syndrome.
- Pain and swelling of the
hands and feet caused by sickle-shaped red blood
cells that plug blood vessels in the hands and
feet. Often this will be the baby's first problem
caused by sickle cell disease.
- A molecule found in red
blood cells that carries oxygen from the lungs to
other parts of the body.
- Pain event or painful
- Pain caused by plugging of
blood vessels by sickled blood cells. Pain is most
often felt in the arms, legs, back, and abdomen.
The pain may last only a few hours or as long as a
week or two. The pain may be mild or so severe
that pain medicine is needed. The number of pain
events a person has may vary greatly.
- The presence of infection
in the blood stream.
- Sickle cell anemia.
- The most common form of
sickle cell disease. Other types of sickle cell
disease include hemoglobin SC disease and sickle
beta-thalassemia; there are also other, less
common types of sickle cell disease.
- Sickle cell disease.
- A group of inherited
disorders in which anemia is present and sickle
hemoglobin is produced.
- Sickle cell trait.
- The condition in which a
person has both normal and sickle hemoglobin in
the red cells as a result of inheriting a normal
hemoglobin gene and a gene for sickle hemoglobin.
Sickle cell trait is not a disease and does not
change to sickle cell disease. Persons with sickle
cell trait may pass the sickle gene to their
- Sickled cells.
- In children with sickle
cell disease, hemoglobin molecules in red blood
cells stick to one another and cause the red cells
to become crescent or sickle shaped. Sickled cells
cannot pass easily through tiny blood vessels.
- Splenic sequestration
- Occurs when a large
portion of the child's blood becomes trapped in
the spleen. Early signs include paleness, an
enlarged spleen, and pain in the abdomen.
- Streptococcus pneumoniae.
- A bacteria that causes a
very serious type of pneumonia in children with
sickle cell disease. Twice daily doses of
penicillin by mouth, starting at about 2 months of
age, can help to prevent this life-threatening
infection in children with sickle cell anemia and
The information in this
booklet was taken from the Clinical Practice
Guideline on Sickle Cell Disease: Screening,
Diagnosis, Management, and Counseling in Newborns and
Infants. The guideline was written by a panel of
experts sponsored by the Agency for Health Care Policy
and Research. Other guidelines on common health
problems also are being developed.
For more information about
guidelines, or to order extra copies of this booklet,
- Agency for Health Care
Policy and Research
- Publications Clearinghouse
- P.O. Box 8547&127
- Silver Spring, MD 20907
Or call 1-800-358-9295 (for
callers outside the US, only: 301-495-3453) weekdays,
9 a.m. to 5 p.m., Eastern time.
- Department of Health and
- Public Health Service
- Agency for Health Care
Policy and Research
- Executive Office Center,
- 2101 East Jefferson Street
- Rockville, MD 20852
- AHCPR Publication No.
- April 1993
The Center for Sickle Cell
2121 Georgia Avenue NW
Washington, D.C. 20059
Sickle Cell Disease
Association of America
3345 Wilshire Boulevard, Suite 1106
Los Angeles, CA 90010-1880
(310) 216-6363; 800-421-8453; FAX: (310) 215-3722
Comprehensive Sickle Cell Center
PO Box 109
Grady Health System
80 Butler St.
Atlanta, Georgia 30335
Donor Cord Blood Program at Children's Hospital
Oakland serves families nationwide who wish to save cord
blood for their children, when there is a specific
medical indication for banking (such as sickle cell
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