Sickle Cell Anemia:
A Parent's Guide for the School Age Child
Important Facts About Sickle Cell Anemia
 Sickle
cell anemia is the most common serious genetic disease in Black Americans.
About one in every 400 black infants is born with the condition. A person
with this life-long disease has an abnormality of the hemoglobin, which
leads to abnormally shaped red blood cells. All complications of disease
can be traced to these abnormal shaped red blood cells. The cell sometimes
assumes a "sickle" shape which is very rigid. Rigid red blood
cells can become trapped and cause "log jams" within the blood
vessels interfering with normal blood flow. The resulting obstruction can
lead to sudden pain anywhere in the body, as well as damage the body
tissues and organs over time. The altered structure of the red blood cell
causes it to break down more rapidly leading to a chronically low blood
count, or anemia. The manifestations and severity of the disease are
unpredictable. Some children have almost no symptoms while others have
multiple serous problems. Most children have occasional mild symptoms.
Supportive treatment is available for sickle cell anemia, but as yet there
is no cure.
With an incurable disease, prevention of complications and early
treatment are very important. Understanding the special needs of the child
with sickle cell anemia is essential for the child's health and well
being. The intent of this guide is to educate parents and others about how
to provide the best care to the child with sickle cell anemia.
DIAGNOSIS OF A SERIOUS DISEASE IN A HEALTHY CHILD
Children with sickle cell anemia are well most of the time, but certain
complications can occur which are very serious and sometimes fatal
(cause death). It may be hard to believe that a healthy looking child has
a life threatening disease, but this is true when a child has sickle cell
anemia. At first parents may not want to know what can happen with this
disease. They may want to pretend that nothing is wrong until something
happens to indicate otherwise. This is a normal reaction to the unpleasant
news that a child has a serious disease. Besides feeling sad, parents may
feel angry and or guilty. These emotions are very normal and usually
temporary. Eventually parents work through their feelings and want to
learn everything they can about this disease to provide the best care for
their child.
ANEMIA
New red blood cells are produced in the bone marrow every day to
replace old blood cells. In a child with sickle cell anemia a red blood
cell will last about 14 days. A red blood cell will last about four months
in the child with normal hemoglobin. Because the red blood cells do not
last long and because the bone marrow cannot make new blood cells fast
enough, anemia results. Anemia exists when the number of red blood cells
in the circulation is below normal. Sometimes anemia is referred to as a
low blood count. Anemia is common in all individuals with sickle cell
disease. Even though a child's usual blood count is quite low, this causes
few if any real problems.
The blood count in a child with sickle cell anemia can drop suddenly.
When a child who is already anemic has a drop in the blood count, this is
potentially serious. The symptoms of a very low blood count are headache,
irritability, unusual sleepiness, lethargy, rapid heart beat, and pale
color. If the palms of the hands do not have any pink color even when the
hands are warm, the child is pale. A child can have a seriously low blood
count without many symptoms. Sometimes the only symptom is that the child
is less active. If a child has symptoms of a low blood count a doctor
should be consulted immediately. In most cases no treatment is necessary
and the blood count comes up on its own. Sometimes a blood transfusion is
necessary.
Aplastic Crisis
Sign and Symptoms
- Paleness
- Lethargy
- "Not feeling good"
- Headache
- Fever
- Low blood count (anemia)
- Recent upper respiratory infection
- Passing out (fainting)
INFECTION
The child with sickle cell anemia will get colds, sore throats and ear
infections just like other children. These minor infections are not
serious. The potentially serious infections which are more likely to occur
in the child with sickle cell anemia are septicemia (infection of the
blood), meningitis (infection around the brain), pneumonia (infection in
the lungs), and osteomyelitis (infection of the bone).
A with septicemia may mot seem very sick. A fever of 102 degrees or
higher may be the only symptom at first. Other symptoms are unusual
sleepiness, rapid breathing, pale color, vomiting, and diarrhea.
Septicemia is more common in young children, but does occur in older
children with sickle cell anemia. Early treatment is the best hope for
recovery from this sometimes fatal complication.
Meningitis is very similar to septicemia in its symptoms and can also
be fatal. In addition, a child with this problem is usually very
irritable.
Pneumonia can be mild with very few symptoms or it can be quite
serious. The symptoms are high fever, rapid breathing, shortness of
breath, chest pain, abdominal pain, and cough.
Osteomyelitis causes fever, pain, swelling and or redness over a bone.
A child will often have a fever of 102 degrees. If this complication
occurs and treatment is delayed, serious and permanent damage to the bone
or joint can occur.
All the infections named here are treatable and complete recovery is
possible. It is also true that even with treatment, permanent disabilities
and sometimes death can result. The child with symptoms of a serious
infection should be seen by a doctor as soon as possible. Early
recognition and treatment of infection offers the best chance for complete
recovery.
FEVER
Fever is a normal response of the body to infection. Fever is usually
due to a minor infection, but it can be the first symptom of a very
serious infection. When fever first begins, it is impossible to tell how
serious the infection is. The child with sickle cell anemia is more
susceptible to serious infections such as septicemia (infection of the
blood or blood poisoning). If the child does have septicemia, treatment
must be started early to save his life. Fever can be the first symptom of
septicemia, so it is important for parents to know what to do when their
child seems sick and has a fever.
Whenever a child seems sick, the temperature should be checked with a
thermometer and always have one at home or with them if they are away from
home.
1) IF THE TEMPERATURE IS 103 DEGREES OR HIGHER take the child
immediately to a facility that gives emergency care. Be sure to tell the
doctor that the child has sickle cell anemia.
2) IF THE TEMPERATURE IS 102 DEGREES give the child medication for
fever and check the temperature again in one hour. If the child's
temperature is the same or even higher, then take the child to see a
doctor. If the temperature is 101 degrees or less it is safe to watch the
child at home for a few hours. During this time the temperature should be
taken every 2 hours. If the fever rises to 102 degrees again, then take
the child to a doctor.
3) IF THE CHILD CONTINUES TO HAVE TEMPERATURE AT OR ABOVE 101 DEGREES
FOR OVER 24 HOURS it is best to call or see a doctor.
4) WHEN A CHILD HAS FEVER AND OTHER SYMPTOMS such as pale color,
trouble breathing, unusual sleepiness, chest pain, severe cough, abdominal
pain, diarrhea, or vomiting, it is best to call or see a doctor soon.
5) SUDDEN WORSENING OF ANY ILLNESS IS REASON ENOUGH TO CALL OR SEE THE
DOCTOR.
6) IT IS IMPOSSIBLE TO KNOW HOW HIGH THE FEVER IS BY FEELING THE SKIN.
The temperature must be taken with a thermometer. Do not give medication
for fever before checking the temperature with a thermometer.
Fever medication makes a child feel better and reduces fluid losses
from the body, but it does not treat the cause of fever. Tylenol is not
needed unless the fever is over 102 degrees or the child is uncomfortable.
There are other things to do when a child has a fever. Dressing the
child in light clothing, keeping the room about 70 degrees, and giving a
lukewarm bath may help lower the fever. If the child is sleeping, use only
light covers. Anything which causes shivering should be avoided because it
will make the fever rise. A child with fever needs to drink more fluids.
If a child has a temperature of 103 degrees or higher, it is not safe to
wait and see if the fever comes down. The child needs to be seen by a
doctor right away.
ACETAMINOPHEN (tylenol or tempra) Dosage
Chart
| Age |
Weight |
| Liquid
| Chewable Tabs
| Junior Tabs |
| 4-5 yrs
| 36-47 lbs
| -
| 11/2 tsp
| 3 tab
| - |
| 6-8 yrs
| 48-59 lbs
| -
| 2 tsp
| 4 tab
| 2 tab |
| 9-10 yrs
| 60-71 lbs
| -
| 21/2 tsp
| 5 tab
| 21/2 tab |
| 11 yrs
| 72-95 lbs
| -
| 3 tsp
| 6 tab
| 3 tab |
| 12-14 yrs
| 96 lbs
and over
| -
| -
| -
| 4 tab |
PAINFUL EPISODES
Painful episodes occur in children with sickle cell anemia as a
complication of their disease. These episodes are more common in older
children, but sometimes happen in babies. Most often the pain seems to be
in the bone, but occasionally it occurs in other areas. These episodes
usually are not dangerous and may last for several hours to several days,
to sometimes a week or ten days. Pain medicine can make a person more
comfortable until the episode passes. There is no medicine which prevents
painful episodes.
WHERE IS THE PAIN?
Most often painful episodes seem to be in the bone, but occasionally
the pain will occur in the chest and abdomen. A complication that may
occur in boys with Sickle Cell Anemia is a prolonged, painful erection
called priapism.
This may take several hours or days to resolve. If the erection is not
better after 3 hours, notify the doctor. Sometimes admission into the
hospital is necessary for pain control.
WHAT IS THE CAUSE OF THESE PAINFUL EPISODES?
The exact cause of these episodes of pain is unknown, but it is thought
that red blood cells become trapped causing "log jams" inside a
blood vessel and interfere with normal blood flow. If blood flow is
reduced in even a small area of the body it can cause pain. Sometimes
swelling is seen in the area of pain. In babies swelling often occurs in
the hands and feet. Older children can have swelling in the arms and legs.
Swelling usually does not mean that something is seriously wrong, but in
rare cases swelling and pain are caused by infection in the bone. A child
with swelling other than hands and feet should be seen by a doctor.
WHAT CAN BE DONE TO EASE THE PAIN?
Discomfort is usually eased by taking medication for pain and drinking
plenty of liquids. A child may refuse to use the part of the body which
has the pain. If a child will not stand or walk it is best not to force
him. As soon as the pain is better he will be active again. Other measures
which may help the pain are rest, and an application of warmth such as a
heating pad. If a child cannot be made comfortable at home with medication
by mouth, then it may be necessary for the pain to be treated with
stronger medication in the hospital.
HOW LONG DOES THE PAIN USUALLY LAST?
Pain usually lasts only a few hours, although it may last several days up
to a week or 10 days. If the pain continues for more than 4 days the
parent should call the doctor or sickle cell clinic for advice.
HOW DO YOU KNOW WHETHER THE PAIN IS FROM THE SICKLE CELL ANEMIA OR
FROM SOMETHING ELSE?
If the child does not have other symptoms of illness, the pain is probably
due to the sickle cell anemia. If the child has fever of 102 degrees or
above, an infection could be present, and should be seen by a doctor. If
the child has pain in the chest or abdomen with cough, rapid breathing,
shortness of breath, pale color, or high fever, he should be seen by a
doctor as soon as possible.
CAN TOO MUCH ACTIVITY CAUSE A PAINFUL EPISODE?
Some parents worry that their child is too active and will cause himself
to develop a painful episode. the child that is active is a healthy child.
Active behavior should not be discouraged. A child with sickle cell anemia
needs to be treated as normally as possible especially when he is acting
normally! However, certain conditions may precipitate a painful episode;
cold weather, swimming in an unheated pool, injury, emotional distress,
and unknown factors.
CAN ANYTHING PREVENT PAINFUL EPISODES?
There is not enough known about the cause of the painful episodes to
prevent them. Several types of medicines have been tried, but nothing is
effective. There is no known nutritional factor which affects the
development of painful episodes. Children with sickle cell anemia who eat
a balanced diet usually do not need extra vitamins. Research may show us
ways to prevent pain in the future.
ENURESIS & NOCTURIA
Enuresis (incontinence of urine during the day) and nocturia (bedwetting
at night) can occur in the child with sickle cell anemia from about age 3
years into adulthood. It is more commonly seen in boys than in girls.
Enuresis and nocturia can occur daily, intermittently or occasionally.
Current therapies used for bedwetting such as restricting fluids before
bedtime does not appear to make a difference in the bedwetting. It is
important to remember the child is not wetting the bed intentionally. When
a child wets the bed he feels badly. The best approach to bedwetting is a
casual one - don't make a big deal out of it, don't spank, punish or yell
at your child - this only serves to make him feel ashamed of a behavior
which he has no control over.
YELLOW EYES
Occasionally the eyes of a child with sickle cell disease may appear
yellow. Usually the yellow eyes result from the build up of by-products
from the increased destruction of red blood cells. Sometimes the eyes may
appear yellow at the time of a painful episode or other illness; other
times there maybe no associated problem. Yellow eyes usually does not mean
that there is something seriously wrong with your child. They do not need
to stay home or be sent home from school.
GROWTH AND ADOLESCENCE
It is important for the parent and child to know that there can be a delay
as long as three years in reaching full adult development. The adolescent
with sickle cell anemia may still look and feel like a child while his or
her friends are developing adult characteristics. Reassurance that they
too will grow up and mature will be necessary to relieve anxious feelings.
Girls with sickle cell anemia can and do become pregnant. Serious
complications can happen, therefore pregnancy should be planned and under
the supervision of a doctor especially knowledgeable in the management of
women with sickle cell anemia. Several different birth control methods can
be used successfully by women with sickle cell anemia.
SCHOOL
The education of the child with sickle cell anemia is just as important as
it is for any child. Sickle cell anemia alone does not affect learning
ability. Motivating and helping a child to achieve his or her potential in
school is important because that child has a future. Extra encouragement
and help will be needed when illness interferes with school. Keeping up
with school work is important. These efforts can make a great difference
in educational achievement. The child with aptitude for it should be
prepared to attend college in order to qualify for employment that relies
upon mental rather than physical skills. The child who does not want to
attend college should be encouraged to train for a career that will not
require strenuous physical activity.
LIVING A NORMAL LIFE
Just because a child has a chronic illness does not mean he or she cannot
live a normal life. A child with sickle cell anemia has very few
limitations. No special diet is needed, just well-balanced meals. There is
no reason to isolate a child from others to keep him or her from catching
colds or other minor infections. Exercise and play should not be
discouraged.
- With the exception of karate, participation in sports activities is
not limited.
- Physical endurance may be less because of the anemia, so a child may
tire more easily.
Children will learn their own bounds for physical endurance and will
usually stop and rest when they need it. Discipline should be the same for
the child with sickle cell anemia as it is for other children in the
family. Treating a child with sickle cell anemia in a normal manner is an
important part of helping a child to develop a healthy identity as a
person.
THE INHERITANCE OF DISEASE
Sickle cell anemia is only one of many diseases that are inherited.
Inherited diseases are passed from parents to children through genes.
Genes are what make the physical characteristics of a person such as
height, eye color, features of the face, and blood type. Every kind of
gene comes in twos with one gene inherited from the mother and one gene
inherited from the father. When a person has sickle cell trait
there is one gene for normal hemoglobin and one gene for sickle
hemoglobin. Because there is one gene for normal hemoglobin the person
does not have the disease. The child with sickle cell anemia has 2
genes for sickle hemoglobin and this causes the disease.
There is a one in four chance or 25% chance that a child will be born
with sickle cell anemia when both parents have sickle trait. When
predicting the chances of having another child with disease, it is
important to remember that it is possible to have another child with
sickle cell anemia with each pregnancy.
Parents usually want to know if their other children could have sickle
cell anemia. This can be learned by blood testing and is a good idea when
one child is already known to have disease. Parents need to know that
future children can also have disease.
General Guidelines
- No cure, complications treatable
- Discourage "sick person" identity
- No special diet
- Discipline same
- Stress importance of school work
- No restrictions
- Regular follow up by medical team
- Encourage normal lifestyle (realistic goals)
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