GENERAL FACTS ABOUT DESFERAL
How is Desferal supplied?
Desferal is supplied in packages of four 500 mg vials or is available in larger 2 gram vials. [top]
Who is responsible for producing and marketing Desferal?
Novartis Pharmaceuticals, Switzerland. [top]
What is Desferal used for?
Desferal is used for the treatment of iron overload due to frequent blood transfusions. [top]
How does Desferal work?
Desferal works by binding with excessive iron and removing it via the urine and stool. [top]
Is Desferal the only drug that can help me to excrete iron from the body?
Desferal is the only drug available in the US that chelates excess iron from the body. [top]
What are the ways Desferal is administered?
1. Subcutaneously
Using a small needle placed under the skin that allows slow infusion of Desferal through a pump.
2. Intravenously
Through a vein (usually given in a hospital setting)
Through an indwelling catheter/port, an internal device that provides access to a major vein in the body
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What are the different devices used to infuse Desferal?
1. Pump Infusion Driver
The pump is a small infusion driver that can hold a 20cc syringe.
2. Volumetric Pump
Commonly used for intravenous infusions of Desferal through an indwelling catheter/port, this pump is usually portable.
3. I.V. Pump
Thalassemia patients can receive a dose of Desferal intravenously while receiving their blood for the day as a supplement to nightly infusions at home.
4. Balloons
A disposable device inflated by adding medicine, it allows for one-time use and works without a motor or batteries.
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Which way of doing Desferal is best for me?
There are several advantages and disadvantages to each method of administering Desferal. Some of the issues surrounding each method are as follows:
Intravenous Desferal:
1. I.V. Desferal while getting blood
Desferal can be given at the same time one is receiving blood products; however, it is only helpful in removing a significant amount of iron if it is infused over a period of time greater than 8 hours. The time needed for effectiveness is often longer than most transfusions and doing Desferal this way does not take the place of doing your Desferal at night, in between transfusions.
Most transfusion services discourage simultaneous infusion of Desferal and blood products since reactions such as fever may cause confusion as to their source.
2. I.V. Desferal through a port
Desferal given through a port can be given at higher concentrations for a longer period of time and allows Desferal to be absorbed directly into the blood stream. Implantation of a port requires surgery. Proper maintenance of a port is very important because it can become infected quite easily, and serious thought should be given to the risks involved in having a port because of the threat of infections.
Subcutaneous Desferal:
1. Infusion driver for subcutaneous Desferal
With the use of a pump, Desferal can be infused slowly under the skin. This is the most common way Desferal is administered and if done as prescribed is very effective in chelating iron from the body. Subcutaneous Desferal can cause localized irritation and swelling. Also, syringe drivers tend to make noise and are bulky, which can hamper the ability to do Desferal during the day.
2. Balloon – Desferal administered subcutaneously without a pump
The advantages of doing Desferal with a balloon is that it is portable and is small enough to fit in a pocket or can be clipped to clothing without being noticed. This form of disposable pump is expensive and may not be covered by your insurance company.
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WHAT ARE THE THINGS I NEED TO THINK ABOUT BEFORE I DO DESFERAL?
How old do you have to be to start Desferal?
Your doctor will consider several factors beyond just your age, such as weight, number of blood transfusions, diagnosis and iron levels.
Most patients with thalassemia major start Desferal by 3 years of age, but depending on the number of transfusions you may have had, the starting age can vary.
Patients with thalassemia intermedia may not start Desferal at such an early age because of their varying requirements for blood products.
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What is the recommended dosage of Desferal? How many vials should I be using?
The dosage can range from 25 mg/kg bodyweight to 55 mg/kg bodyweight, with the dose of 40 mg/kg bodyweight as average.
The recommended dosage varies according to your age, serum ferritin and degree of iron overload.
Consult your physician on the correct dosage and nights per week you should use Desferal.
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When would my dosage change?
A change in your dosage can be a result of numerous factors:
a change in your serum ferritin level, up or down
a change in your transfusion schedule
a significant body weight change (greater than 20%)
A change in your dosage should not be made without consulting your doctor. [top]
What supplies will I need to do Desferal subcutaneously?
1. Make sure you have all of the necessary supplies listed below. Obtain these from your pharmacist or home healthcare agency beforehand.
sterile water
20cc syringe
needle for syringe
butterfly needle or thumbtack needle
extension tubing
alcohol swabs
tape or tegaderm
infusion device
2. Make sure your pump is set to the correct rate that correlates with the number of hours required to do your Desferal. The operating manual, which came with your syringe driver pump, explains how to determine the rate at which you should set your pump. The rate takes into account the size of the syringe you are using (10cc, 20cc), the volume to be infused and the number of hours of the infusion [top]
What supplies will I need to do Desferal I.V. at home?
A nurse will instruct you on how to access yourself using the proper sterile technique.
After you are accessed, make sure you have on hand:
gloves
extension tubing
alcohol swabs
saline for flushing
Heparin for flushing
tubing caps
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How many hours should I do the pump? When should I do my Desferal?
Desferal works best if done continuously, because it has a cumulative effect within the body.
It has been suggested that Desferal be infused over a 10-12 hour period of time.
Because Desferal requires continuous infusion it has been suggested that Desferal be done at night, when it is least intrusive.
Keep in mind that infusing Desferal for less than 8 hours may significantly decrease its effectiveness.
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Where on my body is the best place to infuse subcutaneous Desferal?
Areas that are recommended are the stomach, upper arms, thighs, and buttocks.
Try to find an area that is free from swelling and bumps from previous use of Desferal for that night’s infusion. Using an area of the body that is inflamed is not only painful, but can hinder the absorption of Desferal into the body.
Rotation is the key to minimizing the amount of irritation that occurs due to the continuous use of Desferal. [top]
Why is rotation of sites important?
Welts and swelling in the skin can disrupt the absorption of Desferal in the tissues and thereby decrease the amount of iron getting excreted out of the body. It is best to use a different part of the body each time you do Desferal so that the inflamed area has a chance to recover from the last infusion. [top]
What changes will occur during my infusion of Desferal?
Beneficial Changes:
Removal of excess iron from the liver and other organs prevents complications such as liver and heart disease.
Use of Desferal helps in the development of normal puberty and increases the likelihood of becoming pregnant or being able to father a child.
If you are severely iron overloaded, you will notice with time a slight change in your skin color. As the iron leaves your body the skin becomes lighter in the face, hands, feet and elsewhere.
Adverse Changes:
Short-Term/Immediate
Swelling, redness and irritation at the site of infusion
Some leaking of blood or fluid from the needle site
Dark, reddish color to your urine and stools. This change in color is not harmful and indicates removal of iron.
Long-Term
Disturbances of vision – blurred vision, loss of vision, problems with color vision, night blindness
Disturbances of hearing – ringing in the ears, hearing loss
Disturbances of bone development and normal growth
(If you experience vision and hearing loss you should consult your doctor)
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When should I not use Desferal?
If you have a contraindicating medical problem(s):
1. Changes in vision or hearing pending evaluation
2. Allergic reactions to Desferal, especially if severe, i.e. anaphylactic shock
If you have fever over 101 F:
If you have a temperature of above 101 F, you must discontinue use of Desferal and notify your doctor. Having a temperature may be an indication of a bacterial infection. Bacteria can utilize Desferal in the body to grow rapidly and bacteria tend to thrive in iron-rich environments.
If you have fever, consult with your doctor and remind your doctor about Yersinia, a strain of bacteria that is particularly harmful to patients on Desferal. Do not restart Desferal until you have consulted with your doctor or until you have finished your course of antibiotics.
If you are taking certain medicines:
1. Your doctor needs to be aware if you are taking medications that contain prochlorperazine, such as Compazine, a drug used for anti-nausea and psychosis.
2. If you are taking antibiotics for a bacterial infection, you should consult with your doctor before starting Desferal with the exception of taking penicillin prophylaxis following a splenectomy.
Sometimes when taking Vitamin C:
Vitamin C supplements should not exceed 200 mg a day and should only be taken at the start of infusion. Vitamin C should not be taken at all on days when you are not using Desferal.
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PREGNANCY AND DESFERAL
Can you use Desferal if you are pregnant?
Desferal may be harmful to the unborn child and therefore consideration needs to given to stopping Desferal if you are thinking about having a child. Since the effect of Desferal on the fetus is not known with certainty, many doctors recommend stopping Desferal during pregnancy, at least until the completion of the first trimester. However, this concern must be weighed against the degree of iron overload which may occur. Before making any changes to your Desferal regimen or stopping Desferal altogether, consult with your doctor. [top]
Can you use Desferal if you are trying to get pregnant?
If you are trying to get pregnant consult with your doctor on this issue. [top]
Can you use Desferal while breastfeeding?
Desferal in breast milk is not a concern for the baby because the baby cannot absorb it. [top]
MIXING AND MAKING DESFERAL
How should I store my Desferal before I mix it?
Store Desferal in a safe place, out of the reach of children.
Keep medication away from heat and direct light.
Keep Desferal at room temperature. Do not refrigerate it.
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Is Desferal available premixed?
Desferal can be premixed for subcutaneous use by the pharmacist; however, the most common form of premixed Desferal is used for intravenous infusion.
Desferal that comes premixed for I.V. purposes remains stable for up to two weeks, but should be kept in similar storage situations as Desferal you mix yourself, in a cool dry place away from light or extreme heat. [top]
If I premix subcutaneous Desferal how should I store it and how long is it good for?
Premixing Desferal for subcutaneous use, while convenient for the patient, has some drawbacks.
Premixed Desferal should not be kept for more than a week.
A significant concern when premixing is the loss of sterility. Patients should be careful, as contamination can cause localized infections.
The utmost precautions must be taken in order to ensure that the syringes remain as sterile as possible, i.e. recapping syringe, storing syringe safely, etc.
Once premixed, Desferal should be kept away from extreme heat and cold, as well as light.
Desferal should never be used if kept for more than one week or if the mixture appears cloudy or crusty.
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My pharmacy sends me Desferal in 2000 mg vials and I use a dose that is less than that or more than 2gm, but not divisible by 2. How do I mix my Desferal?
If your dosage of Desferal is 2500 mg or 3000 mg, you can still use the larger bottles; however, you need to store the extra medication for your next use.
If you have a dosage of 3000 mg and you mix your Desferal with 2 cc of sterile water per 500 mg, you can mix one 2 gm vial with 8 cc of water and another 2 gm vial also with 8 cc of water. After the Desferal has had a chance to dissolve, draw up the liquid in the first 2 gm vial completely. Only draw up half the liquid (4 cc) from the second vial. Then store the second vial with the remaining mixture in a cool, dry, dark place for use the next day. Do not use the premixed Desferal after one week.
The same procedure applies to dosages that are half dosages, such as 2500 mg. Instead of withdrawing 4 cc, you would withdraw only 2 cc and could use the premixed vial for 3 more days. [top]
How do I mix Desferal and prepare the pump?
1. Gather all of your supplies.
2. Make sure your pump is set correctly.
3. Draw the water for injection into the syringe after attaching a needle to the syringe.
4. After cleaning the rubber stopper of the Desferal vial with alcohol, inject the desired amount of water into the vial.
5. Roll the drug in the palm of your hands or across the table in order to dissolve it, or allow vials to sit until the Desferal has dissolved. Do not shake the vials.
6. Draw the dissolved drug into the syringe.
7. Attach the extension tube to the syringe. Connect the tubing to the needle and fill the empty tubing with the liquid from the syringe.
8. Place the syringe into the infusion pump.
9. Clean the skin thoroughly with alcohol before you insert the needle.
10. To begin infusion, insert the needle under the skin of the abdomen, buttocks, upper arm or thigh.
11. When inserting the needle into the skin, be sure to insert the needle into a fold of skin created by pushing your skin together.
12. Adjust the needle so that it is secure by taping it down.
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Why should I not shake the vials when making Desferal?
Desferal should not be shaken when trying to dissolve it in liquid because shaking can cause air bubbles to form in the vial, thereby trapping the Desferal and decreasing the concentration of the Desferal drawn out of the vial.
Rolling or letting the vials sit is the best way to dissolve the drug into solution. [top]
I have run out of sterile water. Can I mix Desferal with regular saline in order to do it subcutaneous?
No. [top]
What amount of sterile water is best to mix with Desferal when doing it subcutaneous and why?
It is best to mix each vial with about 1 - 2 ml of sterile water since this amount will permit the Desferal to dissolve more easily. [top]
ADMINISTERING DESFERAL
What happens if I get a blood return in my needle when I stick myself?
If you find that there is blood in the tubing after you insert the needle, take out the needle immediately and find a new site. [top]
What can I do to minimize the pain and irritation of the needle stick and infusion of liquid?
Some patients use a topical anesthetic called Emla Cream to minimize the pain due to needle stick. Emla Cream works by placing a small amount of cream on the intended site about one hour prior to infusion. [top]
What do I do if I get painful swelling and irritation at the site of infusion?
Before administering Desferal:
Use Hydrocortisone. A small amount of hydrocortisone added to the mixture of Desferal may minimize swelling and irritation.
After infusing Desferal:
Place warm compresses on the affected area.
Rotation of sites may minimize the amount of pain and soreness.
Try a larger volume of sterile water mixed with Desferal – not to exceed 2 ml.
Change your needle type. There are several different types of needles available on the market that could make a difference in the amount of irritation and swelling that occurs where you do Desferal, such as:
Butterfly needles - small needles of varying gauges with wings. This needle should be inserted at 45-degree angle to the skin so that it does not lie directly under the skin, but lies deep in the subcutaneous tissue.
Thumbtack needles - small needles in shape of a thumbtack with two grippers on top the needle. This type must be inserted straight down, directly into subcutaneous tissue.
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Why can’t I infuse Desferal faster than prescribed by my doctor? Why can’t I do Desferal at a higher dose than prescribed by my doctor?
Infusion of Desferal faster than is recommended does not give Desferal a chance to accumulate within the body and therefore is less effective in excreting iron than Desferal infused over the proper period of time.
Infusing Desferal at too high a dose can damage the eyes and ears, which can lead to permanent vision and hearing loss. [top]
What should I do if I miss a dose?
A person with thalassemia can live a healthier, longer life if he or she removes as much excess iron from the body as possible. This means doing your Desferal exactly as prescribed. You should follow the plan that you and your doctor came up with regarding the dosage and number of days a week you do Desferal. Let your doctor know if you are having trouble maintaining the prescribed regimen. [top]
If my urine or stools aren’t dark, am I still excreting Iron?
Probably. The amount of iron in your urine can be measured precisely by collecting urine while the Desferal is being infused and for 12-16 hours after it's finished. [top]
My serum ferritin does not seem to be coming down. Does that mean Desferal is not working?
It is important to look at the trend in ferritin levels rather than a single value in order to determine whether your iron load is changing, because ferritin values can increase and decrease for many reasons.
There are other tests that can be done which measure the amount of iron in the body. These tests are often done in conjunction with serum ferritin and can provide a more accurate picture of iron overload.
1. Liver Biopsy
Laboratory values are obtained from the liver sample that corresponds to the degree of iron burden in the liver. These values can vary according to the laboratory where analysis was done and the sample size. The liver biopsy can also determine whether liver scarring (fibrosis) or cirrhosis is present.
2. SQUID
The SQUID is a method for measuring the amount of iron in the liver without the need for a biopsy. At present the availability of the SQUID is extremely limited.
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What can I do to increase my output of iron?
1. Vitamin C
Some patients who use Desferal take Vitamin C, which has been found to help iron excretion while giving Desferal. The dosage recommended is 100-200 mg a day given when you start your Desferal infusion.
2. Tea
Drinking tea with meals can reduce the amount of iron that is absorbed. This is particularly helpful with Thalassemia intermedia, since most of the iron overload is from absorbing too much iron from your diet.
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Will I always have to do Desferal?
As long as you have iron overload you will need to do Desferal in order to get out the excess iron in your body. [top]
Is there research being done to improve methods of chelation?
Research is being conducted all over the world to improve the administration of Desferal and to develop new drugs that can be given orally or are more effective than Desferal. [top]
I am having trouble with compliance. What should I do?
Set goals for yourself in terms of how many nights you are going to do your Desferal and then reward yourself for completing that goal.
Remember that compliance is rigorous and that it is normal to feel discouraged.
There are several places that you can turn to for support, encouragement and motivation that will help you to remain compliant:
1. Other thalassemia patients
Members of the Thalassemia Action Group (TAG), the patient support arm at the Cooley’s Anemia Foundation, are always available at 1-800-935-0024.
2. Doctors, nurses and support staff at your hospital
3. Your friends
4. Your family
This questionnaire was compiled with the gracious help of Dr. Alan Cohen.
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