Patient information: Thalassemia (The Basics)

What is thalassemia? — Thalassemia is a condition that affects the red blood cells. The red blood
cells are the part of the blood that carries oxygen. Normally, red blood cells carry oxygen to all
of the organs in the body.

In thalassemia, the body doesn’t make enough red blood cells. When a person has too few red
blood cells, it’s called “anemia.”

Thalassemia is a life-long condition that people are born with. It is caused by an abnormal gene.
If people get this abnormal gene from both their mother and father, it’s called “thalassemia
major.” If people get this abnormal gene from only 1 parent, it’s called “thalassemia trait.”
Thalassemia trait doesn’t usually cause any of the symptoms of thalassemia major.

There are many different types of thalassemia. This article discusses a serious form called “beta
thalassemia major.”

What are the symptoms of thalassemia? — Symptoms usually start after a baby is a few months
old. Symptoms can include:

 Pale skin
 Acting cranky or upset
 Not growing as much as expected
 Swelling of the belly
 The skin or white part of the eyes turning yellow
 The bones of the face or skull being wider than normal

Is there a test for thalassemia? — Yes. A blood test can show if someone has thalassemia.

How is thalassemia treated? — People with thalassemia and severe anemia are treated with blood
transfusions. A blood transfusion is when a person gets blood that was given (donated) by
another person.

Although blood transfusions help treat thalassemia, they can also cause problems. That’s because
the donated blood has iron in it. When people get a lot of blood transfusions, their body gets too
much iron. Too much iron can damage the heart and liver.

People getting blood transfusions for their thalassemia need treatment to get rid of the extra iron
that builds up in their bodies. Treatment to get rid of this extra iron is called “iron chelation.”
Doctors can use different medicines for iron chelation.

Thalassemia can sometimes be cured with a procedure called a “bone marrow transplant.” This
procedure involves replacing the cells in the bone marrow (the inside part of bones) with healthy
cells. These healthy cells come from another person (the donor). But not everyone with
thalassemia can have this procedure. That’s because it can be done only if a person and his or her
donor meet certain conditions.

Some people with thalassemia will need surgery to remove an organ called the spleen (figure 1).
That’s because the spleen removes red blood cells from the blood, which can make anemia
worse.

What can people with thalassemia do to stay healthy? — People with thalassemia should:

 See their doctor for regular follow-ups, and follow his or her instructions about tests and
treatment.
 Avoid taking vitamins with iron in them.
 Take a vitamin called folic acid (folate), if the doctor or nurse recommends it.

What if I have a child with thalassemia and want to get pregnant? — If you have a child with
thalassemia, it’s possible that your future children will have thalassemia, too. Talk with your
doctor to find out how likely it is that your future children will have the disease.

If you do get pregnant, you can choose to test your unborn baby for thalassemia. This can be
done in different ways.

INSULIN

 Gather injection supplies, including insulin bottle(s), syringe, alcohol swab.

 Wash hands with soap and water.
 Clean the rubber top of the insulin bottle(s) with alcohol swab. New bottles of insulin may have
a hard plastic cap that must be snapped off to reveal the rubber top.
 Remove the syringe cap. Pull on the plunger to draw air into the syringe, equal to the amount of
insulin to be given. For example, if 10 units of insulin will be given, draw back 10 units of air.
 Insert the needle into the insulin bottle and depress the plunger, injecting the air into the bottle.
 If clear and cloudy insulin are used, inject air into the cloudy insulin first, followed by the clear
insulin. Leave the needle inside the clear insulin bottle.
 Hold the bottle with one hand and turn it upside down, keeping the needle inside the bottle.
 Slowly pull the plunger back to draw the correct insulin dose into the syringe.
 Remove bubbles from the syringe, if needed. Bubbles are not harmful if injected
subcutaneously, but they can decrease the amount of insulin in the syringe and potentially
affect blood glucose levels.
 Draw up insulin slowly and steadily. Bubbles form in the syringe if it is drawn up too quickly. If
bubbles are seen, push the insulin back into the bottle and slowly re-draw. Do this as many
times as needed until the bubbles are gone.
 Draw two more units of insulin into the syringe than needed. If bubbles are seen, flick the
syringe with the middle finger and thumb to make the bubbles rise. Once the bubbles are at the
top, push the extra two units of insulin back into the bottle. Don't forget to push the extra
insulin back into the vial - even if there are no air bubbles in the syringe.
  If clear and cloudy insulin are used, it is only possible to remove bubbles from the clear insulin,
which is drawn up first.
 If cloudy insulin is also needed, it must first be mixed. Gently turn the bottle up and down 20
times to mix it. Inject the needle into the cloudy insulin bottle. Carefully turn the bottle upside
down (with the needle in place) and withdraw the dose needed slowly and carefully to avoid
forming bubbles.
 Withdraw the needle from the insulin bottle and replace the needle cap.

 Pick a soft fatty area to inject – tops of thighs, belly, bum and triceps (not always recommended
for children or thinner people)
 Raise a fold of fatty flesh slightly between your thumb and fingers - leaving plenty of space
between to put the needle in
 Put the needle in – if you are particularly slim, you may need to put the needle in at a 45 degree
angle to avoid injecting into the muscle
 Push the plunger, to inject the dose, relatively slowly
 After the dose has been injected, hold the needle in for a good 10 seconds to prevent too much
insulin from escaping out
 If any blood or insulin escapes, wipe this with cotton wool or a tissue
 Ensure that the used needle into a sharps bin is deposited into a sharps bin

Insulin should be kept in the fridge between 4 and 8 celsius

Managing hypoglycaemia
dietary and lifestyle advice e.g. - regular intake of starchy carbohydrates to stabilize the glucose
levels

advice regarding management during

 illness - blood glucose should be measured more frequently since higher doses of insulin are
oftennecessary
 alcohol - there is increased risk of delayed hypoglycaemia with alcohol and therefore should be
advised to eat while consuming alcohol o eating out - adjust the dose and timing according to
the occasion
 missed injections - occasional missed dose will not cause any problems
 managing insulin treatment during travel, holiday and religious festivals involving fasting

Rotate your injection sites to avoid ‘lumpy’ skin

To detect lipohypertrophy, injecting sites should be both inspected and palpated, as some lesions can be
more easily felt than seen. Healthy skin can be pinched tightly together, while areas of lipohypertrophy
cannot.
The site should be changed at each injection (rotated) to reduce the risk of lipohypertrophy developing.

A simple way to reduce this risk is to systematically rotate the site where the insulin is injected.

There are four main injection sites - abdomen, thigh, arm and buttocks.

Divide the injection site into quadrants (or halves when using the thighs, buttocks or arms). One
quadrant should be used per week and moving always in the same direction, either clockwise or anti-
clockwise, keeping the injections at least 2cm apart.

Factors that can speed up absorption and so increase risk of hypoglycaemia

 Warm/hot environment, increasing blood flow to the injection area;

 Rubbing or massaging the area;
 The injection being delivered into a deeper layer of skin.

Factors that may slow down insulin absorption and so cause a potential increase in blood
glucose

 A cold environment, reducing blood flow to the injection area;

 Unhealthy injection sites, for example those that are bruised or scarred.

Methotrexate
 Methotrexate is used to treat various conditions.
 Methotrexate is available as 2.5mg and 10mg tablets- it is important NOT to get these mixed up.
 You will normally be asked to take folic acid tablets along with methotrexate.
 Usually doses of methotrexate are given WEEKLY.
 You will need regular blood tests while taking methotrexate.
 You should be given a booklet to record your blood test results and any change in dose.
 You must report all symptoms and signs suggestive of infection, especially sore throat.
 You must tell your doctor if you are planning to start a family.
 Before you start taking methotrexate your doctor will arrange a chest x-ray and do some blood
tests.