tips for patients and families - leben mit …...of iron. this is about 100 times what is normally...
TRANSCRIPT
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Living with transfusionsTips for patients and families
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Blood – a liquid organ and the elixir of life for which there is no substitute
The flow of life – things you need to know about blood
Blood – emergency medication #1
Who needs blood transfusions?
How a transfusion is done
What you can expect on transfusion day
Risk: transfusion-induced iron overload
Iron – crucial, but also a potential poison
Which patients are at increased risk of iron overload?
Symptoms of iron overload
Diagnosing iron overload
When is treatment needed?
Effective treatment with iron chelating agents
Three treatment options
Important – taking medication regularly with no gaps
My experience: Bergit Kuhle
Tips for living with transfusions and iron overload
Important questions and answers
Glossary
Online Service Center
More important information, contacts and addresses
Hotline
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Contents
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Foreword
A blood transfusion is a treatment that is applied
individually, with planning and great care.
Along with its effects of prolonging life and improving general
well being, doctors and nurses need to keep a close eye on
negative side-effects, e.g. iron overload after approx. 20-25
transfusions, possible allergies being triggered, and potential
build up of anti-bodies in the recipients blood.
After about 450 blood transfusions, spread over eight years,
I am a “living proof” of these reliable checks. But above all
I am so very grateful to the 450 donors, for donating blood
for me, and also to those who will do so in
the future.
Bergit Kuhle, dependent on blood transfusions for the last eight years
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Blood – a liquid organ and the elixir of life for which there is no substitute
“Blood is a quite peculiar juice.”
5 – 7 liters of blood
8 % of body weight
The blood circulation system is the biggest
transport system in the body. Over a pipeline
measuring 96,000 kilometers, it supplies the body
and billions of cells with oxygen and nutrients.
This makes blood one of the most valuable liquids
in the world. The poet and playwright Goethe knew
this, when he had Mephistopheles say to Faust:
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The flow of life – things you need to know about blood
approx.
5–7 liters of blood
2,000 times a day
Adults, depending on their size and weight have
between 5 and 7 liters of blood in their body,
representing about 8% of their body weight.
This volume of blood flows round the body
approx. 2,000 times per day – that is about
10,000 liters of blood that flow through the
aorta each day.
96,000 km
1 minute
Blood takes about 1 minute to flow
round the body when you are resting,
and when you are physically active, just
20 seconds.
If you lined up all the blood
vessels end-to-end, they would
stretch for 96,000 km
or 2.5 times round the world.
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Blood – emergency medication #1
Blood is an important part of
healthcare
The effect of blood cannot be
substituted by any medication, so
blood stocks and blood transfusions
are vital for treating severe illnesses or
as part of major operations.
In the past, the donor’s blood was
given to the recipient unchanged,
while today the important components
in the blood are separated and stored.
Most blood stocks are concentrates of
red corpuscles, known as erythrocyte
concentrates or “ECs”.
Regular blood transfusions are part of
many patients’ routine. They are performed depending on the
blood values and on their general condition and quality of life.
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Who needs blood transfusions?
Treatment with erythrocyte concentrates and possibly other
blood elements is needed especially for
chronic blood diseases, that lead to a reduction in blood
generation or a reduced lifetime for the red corpuscles, such
as anemia or blood marrow diseases like MDS (myelodys-
plastic syndrome) and thalassemia.
acute blood loss, for example after
severe accidents or during operations.
Most transfusions of red
corpuscles (erythrocytes) are
actually given to patients with
some form of blood cancer and
chronic anemia.
Prof. Dr. med. Wolf-Karsten HofmannClinic DirectorIII. Medical ClinicHematology and Internal OncologyUniversity Medicine Mannheim
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Blood transfusions are usually given on an out-patient
basis.
For a blood transfusion, it is important that the donor and
recipient bloods are compatible, as blood groups and rhesus
factors need to match.
Before the blood transfer, therefore, a series of tests are
carried out by the doctor or qualified medical staff to ensure
this compatibility is present.
Only once all the tests are in order, is the blood product batch
connected to the vein access catheter.
How a transfusion is done
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What you can expect on transfusion day
You will be given the transfusion
logbook by your clinic, or you can
order one free of charge from
www.leben-mit-transfusionen.de
The patient needs to inform the doctor administering
the transfusion if they have any symptoms of anemia
such as e.g., shortness of breath, dizziness, muscular pain
when walking upstairs, headaches or feeling weak.
Enough time needs to be allowed for the treatment:
A blood transfusion should not be performed too quickly,
an average input time of about two hours should be
allowed for 2 x 250 ml erythrocyte concentrate. Reading
or listening to music can help to pass the time.
After the transfusion is complete, you should remain
under observation for at least another 30 minutes.
A transfusion logbook helps to keep you up-to-date on
the number of blood transfers.
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After a series of transfusions, however, there is a risk of
iron overload which is explained further below.
Potential incompatibilities and risks of a blood
transfusion
Any problems should be reported to the medical staff
immediately. Especially shortness of breath, weak
circulation, breaking out in a sweat, dizziness, nausea or
headaches may be symptoms of hidden complications,
that may make rapid medical treatment necessary or even
mean breaking off the transfusion.
These kind of effects are the exception. In most cases
blood transfusions are tolerated without any issues.
Not every worsening of your conditions during a
transfusion is necessarily caused by it.
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For many patients, regular blood transfusions may be vital to
their survival of certain illnesses, e.g. anemia, but they can
also be the prime cause of so-called secondary iron
overload:
Normally the level of iron in the
body is in equilibrium: with a
balanced diet around 1 to 2 mg
of iron are ingested each day
with your food. Regular losses
of iron through the skin (sweat),
kidneys and intestines amount
to about 1 to 2 mg per day.
Every erythrocyte concentrate contains about 200 to 250 mg
of iron. This is about 100 times what is normally absorbed from
food each day.
As the body has no way to actively secrete the iron, it builds up
with every blood transfusion and iron deposits are made within
the body. These insoluble iron compounds can cause severe
damage to organs such as the heart and liver.
Risk: transfusion-induced iron overload
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Any patient who receives regular transfusions needs to be
aware that even around 15 to 20 transfusions can lead to
iron overload that can have life-threatening consequences if
left untreated.
Genetically caused problems with the metabolism can also lead
to the body becoming overloaded with iron. But these are not
covered in this brochure.
Imbalances after multiple transfusions
1 blood product batch 200–250 mg
daily reduction 1 mg
Iron intake Iron secretion
Physiological reduction 1–2 mg/day
increases during menstruation, increased consumption
when growing
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Life-critical trace element..
Iron is a life-critical trace element and is important for
transporting oxygen and the metabolism. To be able to make
best use of iron, under normal circumstances a system of finely
regulated intake, transport and storage mechanisms ensures a
balanced level of iron.
...and toxic heavy metal
On the other hand, iron is also a toxic heavy metal. Free iron
favors chemical reactions in which oxygen radicals are created.
These radicals can attack and damage proteins, membranes
and even the genetic DNA material.
Iron – crucial, but also a potential poison
Iron in the bloodto attach oxygen
Iron in the liveras stored iron
Iron in the musclesto transport oxygen and in other proteins
Iron in bone marrowto synthesize blood
Foods containing iron
Loss of iron in old cells and in women during menstruation
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The patients mainly affected are those who suffer
from illnesses where repeated blood transfusions are
necessary. These include:
Myelodysplastic syndrome (MDS)
In MDS there are incomplete and dysfunctional precursors
of red corpuscles (erythrocytes) in the bone marrow, where
the blood is synthesized. This means that the overall number
of functional red corpuscles falls dramatically. This implies a
reduction in the red blood coloring (hemoglobin); anemia is
the result. This leads to symptoms like shortness of breath,
limited strength and headaches, and in two thirds of cases it
needs to be corrected by regular blood transfusions.
In Germany, it is mainly older people who are affected by
myelodysplastic syndromes and so also by iron overload.
Which patients are at increased risk of iron overload?
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Thalassemia (Mediterranean anemia)
This form of anemia is caused by a hereditary failure to build
hemoglobin correctly. The beta-thalassemia major variant is
usually severe. It is found mainly among inhabitants of the
Mediterranean region, the Middle East and South-east Asia.
Sickle cell anemia
Sickle cell anemia is also a hereditary disease. It is signaled
by changes to the hemoglobin. This affects mainly people of
African descent, but also inhabitants of the Mediterranean
region.
Every year, 10/25 is International MDS Day.
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Iron overload develops gradually and is initially non-
specific. Symptoms often only appear at an advanced stage
of the illness.
Storing the surplus iron in the organs can in turn lead to
damage to the heart muscle, heart weaknesses, heart
arrhythmia, liver damage such as cirrhosis of the liver and liver
carcinoma, diabetes mellitus, under-active thyroid, growth
disorders, depression and other disorders (see Fig.).
Symptoms of iron overload
Iron overload does not hurt, but can
cause damage to organs over time.
Therefore the doctor providing treatment
must keep in mind the likelihood of iron
overload caused by transfusion and
diagnose it by means of laboratory tests
and possibly other scanning procedures. Prof. Dr. med. Norbert GattermannSenior DoctorClinic for Hematology, Oncology and Clinical Immunology, University Clinic Düsseldorf
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Frequent damage to organs from iron deposits in the heart, liver and glandular tissue
Pancreas
Diabetes
Gonads
Inactive gonads
Stunted growth, infertility
Pituitary gland
Thyroid glands
Under-active
Liver
Cirrhosis of the liver
Heart
Damage to the heart muscle and
impact on the heart functioning
The damaging role of surplus iron in the bone marrow is currently being researched.
Bone marrow(sternum/pelvis)
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Diagnosing iron overload
Blood tests: Early diagnosis of iron overload is of great
importance to the patients’ life expectancy: The earlier that
atypical values are detected, the earlier successful treatment
can be started to protect the organs.
The following blood readings should be checked regularly: Serum ferritin (iron store) Hemoglobin Erythrocytes (red corpuscles) Inflammation parameter Liver readings Pancreatic enzyme
Serum ferritin should not exceed 1,000 ng/ml, as this can
cause damage to organs.
Other diagnostic methods
A serum ferritin level of over 1,000 ng/ml indicates iron
overload, but does not provide any indication in which organ
the iron has been deposited.
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Liver biopsy: The liver is the organ with the greatest
capacity to store iron, and is therefore always damaged by
severe iron overload. Even if the serum liver values are not
unusual, tissue samples can show if iron overload is present
which has already led to damage to liver cells.
MRT: As puncturing the liver involves a certain risk of
bleeding and infection, the iron deposits in the liver are more
and more being detected with the help of imaging processes
such as magnetic resonance tomography (MRT). This can
show iron overload in the liver very reliably.
FerritinFerritin is the normal iron storage molecule that is found both in the organs and in the blood. If there is a lot of iron in the body, then a lot of ferritin will be created. Normal serum ferritin levels are below about 300 ng/ml (nanogram per milliliter; a nanogram = one billionth of a gram). Levels of over 1,000 ng/ml indicate iron overload. But it must also be borne in mind here that an inflammation reaction can also increase the ferritin levels. Therefore that must first be ruled out before determining iron overload.
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ECG and ultrasound: The heart should be checked using
ECG (electrocardiogram) and ultrasound (echocardiography),
to detect changes early.
SQUID: A special procedure to demonstrate the iron
concentration in the liver and spleen is known as SQUID
biomagnetometry. This allows a precise determination of
the iron content in the tissue without having to carry out a
biopsy. This method is only in use at the Hamburg-Eppendorf
university clinic.
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If transfusion treatment has led to iron overload that is high
enough to create damage to tissues, then the excess enriched
iron needs to be removed by medication, using so-called iron
chelating agents.
Treatment guidelines help to determine what treatment is
needed. In these, a chelate treatment is recommended
for serum ferritin levels above 1,000 ng/ml or when organ
damage from iron is already present. It also needs to be taken
into account which secondary symptoms are present, that
might make the patient especially liable to iron overload.
When is treatment needed?
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Iron chelating agents are used to possibly prevent iron
overload or to treat it effectively. The iron chelating agents
bind iron molecules and create a compound, depending on the
preparation used, that can be excreted with the stools or urine.
Effective treatment with iron chelating agents
Medication (iron chelating agent)
Iron
Iron chelateThe compound is passed out in the stools
The principle of iron chelation
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Currently in Germany and the EU three iron chelating
agents are approved, which differ in their areas of
application as well as in the type and frequency of
ingestion.
Deferoxamin
Deferoxamin was for many years the standard medication
for iron overload therapy, that was administered five to seven
times per week via an eight to twelve hour infusion using a
pump in the subcutaneous fatty tissue. This is an enormous
nuisance, as chelate treatment continues for years and is
only successful if applied continuously.
Deferiprone
For a long time the only available oral iron chelating agent
was Deferipron. The active ingredient was taken three
times a day as a tablet or a juice and is restricted to specific
cases such as thalassemia major. In addition, with this active
ingredient there is a risk of serious side effects such as e.g.
reduction in a sub-group of white blood cells, leading to
substantially reduced resistance to infection.
The principle of iron chelation
Three treatment options
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Deferasirox
The iron chelating agent using the active ingredient
Deferasirox makes treatment much simpler, because it
needs to be taken just once a day. Excess iron is removed
effectively and the effect continues for over 24 hours.
As Deferasirox penetrates all cells, it removes excess iron
from all organs and can therefore be used for stabilizing
or improving heart functions.
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The need for transfusions may reduce
Removing the excess iron is a measure that prolongs life
expectancy and the therapeutic benefit of iron chelation
therapy is well proven by studies.
In addition, it has also been demonstrated that the treatment
can also reduce the need for transfusions.
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It must be noted that only a small proportion of the iron is ever
available to chelating molecules. Therefore the chelating agent
must be present as constantly as possible and the treatment
should never be interrupted.
Important – taking medication regularly with no gaps
Treatment with an iron chelating agent needs to be
adjusted to the level of the existing iron overload
as well as the frequency of transfusion treatments.
Patients who receive a high level of extra iron
from frequent blood transfusions need higher
doses of the iron chelating agent than patients who only
occasionally receive a transfusion.
Prof. Dr. med. Norbert GattermannSenior Doctor
Clinic for Hematology, Oncology and Clinical Immunology
University Clinic Düsseldorf
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Tip: Turn taking the medication into a ritual – for the
sake of your health
So that taking your medication does not get lost from
sight on busy days, you should link it to a daily ritual
such as when you get ready for bed.
Things that are highly visible, like smiley faces that
you can stick onto things you use frequently, such as
your mobile phone or your wallet, are ideal. Colorful
reminder notes placed everywhere you look, e.g. on
the mirror, the fridge etc. are also a very good way to
remind yourself.
Always be aware, above all, that by regularly taking your
medicines you are making a major contribution to the
success of your treatment – and therefore to retaining
your health and quality of life.
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Bergit Kuhle felt
when she had just turned 48 that
something was wrong. The usual
jogging route with her friends just
got harder and harder, she was
exhausted and tired.
On her 49th birthday in 2006, after more than a year of
uncertainty, she was diagnosed with “myelodysplastic
syndrome” and had to learn to accept her new limits, inexorably
imposed by the illness.
Suddenly everything changed … Various treatments, more frequent stays in hospital and long
waiting periods became the norm, and affected her quality of
life. Her life and all her conversations with her husband
and family were dominated totally by her illness. The
illness dominated the daily life of the whole family.
My experience
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Bergit has been dependent on transfusions
since 2008, and despite the now frequent transfusions she
has learned to fill the free spaces that the illness still allows her
with the best possible quality of life. Removing the excess iron
overload from her body with mediation has become part of her
daily ritual.
Managing her life despite MDS is something that Bergit
Kuhle therefore feels very strongly about, and she wants
to share her experience with all MDS patients.
She is the contact person for the MDS Patient Interest
Group (MDS Patient IG), under the umbrella of LHRM e.V.
(Leukemia aid Rhine-Main area) that supports shared activities
for improving early diagnosis, raising public awareness of rare
diseases and the need to develop effective treatments.
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For all patients it is the quality of life that
must be at the top of the list, says Bergit Kuhle. No MDS
patient should have to do without a necessary treatment.
Long-term, the MDS Patients Interest Group is seeking a
greater right of consultation with health policy bodies for the
MDS Patients Interest Group - the MDS Patients Interest
Group, together with many other MDS interest groups in other
European countries, signed a petition on the approval of an
MDS medication and submitted it to the European Medicines
Agency (EMA) in London. Since June 2013 that medication
has now been approved for MDS patients across the EU. The
MDS Patient IG is a founder member of the MDS Alliance, an
international network of MDS patient organizations.
Quality of life is the most important thing
Not that this is the result of our initiative”, says Bergit
Kuhle, “but you cannot begin to imagine what an
important step is it when the voice of the patients
starts to be heard in this area.
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Bergit Kuhle wants to encourage all patients to talk to their
doctors and ask questions – about possible treatment options,
about the risks and results of treatment, about how to avoid
side-effects, about the pros and cons of taking part in ongoing
medication studies, about professional psycho-oncological or
psychosocial support options, and about self-help groups and
contacts.
Patients who take responsibility live longer, of that Bergit
Kuhle isconvinced. “It is important that we as MDS patients
follow the available treatments as defined, and are open to new
methods of treatment, and keep up our physical and mental
strength for the battle against this disease.”
Bergit KuhleBest regards
It is unbelievable and amazing the progress that has
been made relating to MDS in medicine over the last
few years. Even if the medication does not achieve
the desired effect for all patients, at least there are
grounds for hope.
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!
Tips for living with transfusions and iron overload
Nutrition
A strict low-iron diet is not necessary. But you are
encouraged to eat a healthy, fiber rich, mainly plant-
based diet with plenty of fruit and vegetables. Meat
and offal contain a lot of iron, and therefore should be
eaten sparingly.
Also be careful with multi-vitamin products, cornflakes
and muesli mixes: they can have added iron.
Black tea, coffee or large quantities of calcium-rich foods
such as milk, drunk with a meal, help to block iron. But
orange juice helps with iron intake.
It’s better to give up alcohol: basically you should not drink
alcohol because iron deposits in the liver make that organ
more susceptible to the damaging effects of alcohol and
other recreational poisons.
Other helpful tips on nutrition can be found at
www.leben-mit-transfusionen.de
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Prof. Dr. med. Wolf-Karsten HofmannClinic Director
III. Medical ClinicHematology and Internal Oncology
University Medicine Mannheim
When increased iron levels are found,
normally there is a thorough laboratory review
and further diagnostic tests.
For this, it is very important that the patient acts
responsibly and precisely follows the measures
prescribed by the doctor and discusses any problems
these cause immediately.
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Practice sport, but don’t push yourself
too hard
Regular exercise is important, but you need to respect your
body’s limits and avoid both extreme physical effort and also
stress.
Regular visits to the doctor to maintain health
Doctor’s appointments must be kept regularly. In most
cases this is a long-term treatment, during which your doctor
checks the state of your health, i.e. lab tests, regular heart
and liver checks, and then adjusts the treatment accordingly.
Tips for living with transfusions and iron overload
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For patients with cirrhosis of the liver and high ferritin
levels (over 1,000 ng/ml) a liver ultrasound scan should
be performed regularly. The most important data should
be documented regularly and continually in the transfusion
logbook.
All these measures are highly important to support
your health and to maintain or even improve your
quality of life.
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1. How long does a transfusion take? The transfusion of blood products is performed intravenously (usually a vein in the arm) and normally lying down, although it can also be performed sitting up. It takes about 45–60 minutes per pack of blood products (= 250 ml erythrocyte concentrate) and normally two packs are administered in each transfusion session. The speed of the flow is set by the doctor or nursing staff and depends on how well the individual patient can handle the transfusion. Including laboratory tests before or after, and waiting time, you normally need to plan to allow four to six hours for each visit.
2. Why do you have to wait so long on the day of the blood transfusion before you finally get given the blood? Careful checks of the blood products before the transfusion take quite a bit of time, and are intended to ensure the patient’s safety. In addition, emergency cases where blood is required unexpectedly, take priority.
Each stored blood product first needs to be warmed and its quality checked visually. Before the transfusion, the blood group and rhesus factor of the recipient are determined, so that the doctor carrying out the transfusion can select a blood product with matching donor blood. Then there is the so-called cross-matching test, to make sure there is no rejection reaction. In addition, in the second test run, we look for anti-bodies in the recipient. If all the tests are clear, then the blood product is approved. Immediately before starting the transfusion we carry out the bedside test at the patient’s bedside, to make sure there were no mistakes.
Important questions and answers
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3. What is a blood bank? A blood bank stores and holds blood products ready for use. This means that patients with anemia or who have acute loss of blood, e.g. due to an accident, can be supplied quickly.
University clinics always have large blood banks. But even smaller hospitals have a blood store.
Larger blood banks, like that of the Red Cross are responsible as a central facility for making sure that there is always an adequate quantity of blood products available, and that the way they are extracted and used complies with the latest scientific and technological state of the art.
4. What quality standards do blood products need to meet? Security is our top priority. The main priority in extracting and producing blood products is to make sure the donation does not present any health risk and that the patient is protected against infections (such as e.g. HIV, hepatitis B or C, CMV virus) being transferred. Every blood production center has to follow strict guidelines, and is subject to regular inspections and checks by the responsible supervisory authorities.
Around four million blood donations are made in Germany each year. There is a high demand for blood products. One reason is the short length of time they can be stored, just 42 days (for erythrocyte concentrates). They cannot be frozen, only chilled.
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Important questions and answers
5. What criteria does the doctor use to decide if and when a blood transfusion is performed? The basic rule is: for all decisions about transfusions, the clinical condition of the patient is at least as important as the hemoglobin count. The decision should never be based on a lab result alone, rather the overall condition of the patient needs to be evaluated. If you feel bad and tired, and recognize that you have anemia symptoms, you should tell your doctor clearly and talk about the possibility of a transfusion.
6. How is the hemoglobin level measured? The blood needed to measure the hemoglobin (Hb) level is usually taken from a vein in the patient’s arm. The elbow is often used to collect the blood. A few milliliters of blood is all that is needed for the test. The blood collection tube has a blood thinner added, that helps to keep the collected blood liquid. In the laboratory we check the hemoglobin concentration. Modern hemoglobin detectors can also measure the hemoglobin level using a finger sensor.
7. What units are used to express my hemoglobin count? The hemoglobin count is usually give as g/dl, but many clinics have now moved over to using the new unit of mmol/l. The ratio can be calculated as: g/dl x 0.6206 = mmol/l.
Always ask which unit is being used each time. Often the unit is not given when speaking, which means that the numbers can cause patients a lot of confusion. Tip: Note the approximate level at which you generally need a transfusion, and memorize the unit for that.
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8. Why do patients with heart or circulatory problems often get a blood transfusion earlier than other patients? Lower concentrations of hemoglobin place stress on the heart. People whose hearts are performing poorly (cardiac insufficiency) or who suffer from other prior conditions such as coronary disease, will often suffer from symptoms such as shortness of breath, circulatory weakness and dizziness even at higher hemoglobin levels than people with healthy hearts. If someone suffers from heart disease, then they may need a transfusion earlier than someone with a healthy heart. But it is always an individual decision when a transfusion should be performed.
9. Why do the gaps between transfusions get shorter, needing blood more often? If you need frequent transfusions, then you will probably find that your body can, with time. make do with a lower HB count than normal. Otherwise, you may find that the time between transfusions gets shorter. Initially you maybe needed blood only once every four to six weeks, but perhaps the frequency increased at some point to e.g. every two weeks. Why does this happen? This is most likely due to the course of your underlying illness, or to the creation of antibodies, so that the level of foreign blood corpuscles falls more quickly.
10. What does the hematocrit level actually mean? The hematocrit shows the ratio of all blood cells to the total blood volume. As the red corpuscles make up the vast majority of the blood cells, the hematocrit allows us to calculate the share of red corpuscles in total in the blood. The Hb count and hematocrit always change in step with each other. The oxygen carrier, hemoglobin, is contained only in the red corpuscles.
Many thanks to Ms Bergit Kuhle for her wonderful help in putting together
these questions and answers
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Agranulozytose A condition in which the granulozytes, a sub-group of the white corpuscles (leukocytes) have fallen sharply below the level of 500 cells/μl of blood.
Anemia A lack of red corpuscles (see erythrocytes) and/or a reduction in the hematocrit (percentage ratio of cells to blood volume) and/or lack of red blood pigment (see hemoglobin). We refer to anemia when the hemoglobin level is below 11 g/dl.
Bedside test Rapid test at a patient’s bedside to check the blood group compatibility between the donor (blood product) and the recipient.
Blood corpuscles Collective name for the cells contained in blood, i.e. the red corpuscles (see erythrocytes), white corpuscles (leukocytes) and blood platelets (thrombocytes).
Blood products Donor blood with added stabilizers, that is transfused to a recipient either unchanged (whole blood) or after appropriate processing (e.g. as an erythrocyte concentrate).
Blood serum Blood fluid without any of the cell components, that, unlike blood plasma, has also had the fibrin removed, and therefore cannot clot.
Bone marrow The red bone marrow, also called hematopoietic marrow, is where all the types of blood cells are synthesized.
Glossary
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Chelating agent Molecule that can bind with other molecules into a chemical compound (chelate compound). Chelating agents that create compounds with iron ions are referred as iron chelators (see below).
CRP The C-reactive Protein (CRP) is a protein that is created in the liver It is an indicator of inflammation, i.e. when the CRP count in the blood rises this is evidence of inflammation somewhere in the body.
Echocardiography Ultrasound scan of the heart.
Electrocardiogram (ECG) Procedure to record the heart’s electrical activity.
Erythrocytes Red corpuscles. Disc shaped blood cells, that contain the red blood pigment (see hemoglobin) and use it to transport oxygen from the lungs to the tissues. Reference levels for your erythrocyte count are around 5.5 million/μl of blood (men) or around 4.5 million/μl of blood (women).
Erythrocyte concentrate Transfusion pack filled with red blood corpuscles. These products are not whole blood.
Ferritin A protein that stores iron, that occurs both in organs and in the blood. Normal ferritin levels in blood serum are below about 300 ng/ml. Levels of over 1,000 ng/ml indicate iron overload.
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Hematological Center Locations in both hospital and out-patient environments that use blood products (e.g. hospitals, doctor’s surgeries etc.).
Hemochromatosis Disease that stores too much iron.
Hemoglobin Red blood pigment that contains iron, found in the red blood corpuscles (see erythrocytes). Well-functioning hemoglobin is essential for the erythrocytes to be able to transport oxygen. The reference levels for men are around 13.5–17.5 g/dl and for women 12–16 g/dl.
Imaging procedure Using ultrasound, X-rays or magnetic resonance tomography (MRT) to display changes inside the body.
Iron (Fe) Metal trace element, core component of red blood pigment (see hemoglobin). Iron can exist in either a divalent (Fe2+) or trivalent (Fe3+) form. Divalent iron is absorbed in the small intestine and bound to transferrin (see below).
Iron chelating agent A medication that can remove excess iron from the blood and from tissues.
Iron stores Deposits of iron in the liver, spleen and bone marrow, who collect iron that is not needed to synthesize hemoglobin (see below) and is not bound to transferrin (see below).
Glossary
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Leukocytes White blood corpuscles that are responsible for immune defenses. They can be found in the blood, the bone marrow, lymphatic organs and other body tissues.
Magnetic resonance tomography (MRT) An imaging procedure that allows, among other things to see if iron is being deposited in the liver.
Myelodysplastic syndrome (MDS) A group of illnesses are referred to as myelodysplastic syndrome (MDS) which result in the production of one, two or all three types of blood cells is interrupted (erythrocytes, leukocytes, thrombocytes). A growing number of incomplete or (dysplastic) deformed cells occur in the bone marrow, which has a negative effect on the regular blood production.
Neutropenia This refers to the reduction of neutrophil granulocytes in the blood, one of the most important sub-groups of the white corpuscles (leukocytes).
Oral Taking medication by mouth.
Serum creatinine Laboratory parameter that reflects kidney function. Creatinine is a waste product produced by the body, that is eliminated via the kidneys. If kidney function worsens the level of creatinine rises.
Serum ferritin (iron store) See ferritin.
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Sickle-cell anemia A hereditary form of anemia that is indicated by a change to the red blood pigment. Sickle-cell anemia is very rare in northern Europe and does not always have to be treated by blood transfusions.
SQUID biomagnetometry Special non-invasive method to determine quantitatively the concentration of iron in the liver and spleen. Is only performed at a few specialized centers around the world. SQUID stands for Superconducting Quantum Interference Device.
Thalassemia (Mediterranean anemia) A form of anemia caused by malformation of the hemoglobin pigment. There are two types, alpha and beta thalassemia. Thalassemia major is a beta thalassemia. The major type is the worst type of hereditary anemia, requiring lifelong transfusions.
Thrombocytes Thrombocytes or blood platelets are one of the cellular components of blood. They are very small, disc-shaped cells with no nucleus. Thrombocytes are important for blood clotting as they stick to each other when blood vessels are damaged and create a plug at the site of the injury. If there are not enough thrombocytes in the blood, or if these cells malfunction, then severe bleeding can occur. This means that if you are injured, you bleed more and for longer. If the platelet count falls below 150,000/μl, then we refer to thrombocytopenia.
Glossary
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Transaminases Occur mainly in the liver. If the organ is damaged, these enzymes are released into the blood.
Transferrin A protein that is created in the liver, that can store trivalent iron (Fe3+) and that is used in the body to transport iron.
Transfusion Transfer of blood or blood products from a donor to a recipient.
Ultrasound scan Imaging method that uses sound waves above the upper limit of human hearing. Ultrasound scanning of the heart is referred to as echocardiography.
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Important information on the Internet about transfusions
and iron overload
The Internet address www.leben-mit-transfusionen.de
provides special information for patients with myelodysplastic
syndromes (MDS), other forms of anemia or iron overload. This
Internet site is a service offered by Novartis Pharma GmbH.
Patients can find here explanations about the causes,
diagnosis and treatment of iron overload. In addition, these
public service pages should help to encourage patients: they
show ways of living with MDS without having to sacrifice quality
of life.
In addition, under the “Services” heading on this website
you can sign up for a newsletter, in either digital or
printed format.
Online Service Center
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www.mds-patienten-ig.org
An MDS patients support group within LHRM e.V. www.blog4blood.de
A blog for patients with haemotological illnesses to
share experiences www.mds-net-de.org
MDS Deutschland e.V. www.leukämie-hilfe.de
Deutsche Leukämie- & Lymphom-Hilfe e.V. www.LHRM.de
Leukämiehilfe RHEIN-MAIN e.V. www.mds-register.de
German study register for myelodysplastic syndrome www.kompetenznetz-leukaemie.de
German leukemia skills network Centers to measure liver iron: In certain centers liver
iron can be measured free of charge.
More important information, contacts and addresses
Hotline
For questions on medical-pharmaceutical topics you are
welcome to contact our colleagues at our Novartis Info-
service. They offer skilled advice and extensive information
about our medicines and their therapeutic use.
Monday to Friday from 8 a.m. to 6 p.m.
Tel.: 01802 232300*
Fax: 0911 27312160
email: [email protected]
* Telephone service (EUR 0.06 per call from a German land-line;
max. EUR 0.42 per minute from a German cellphone)
Novartis Pharma GmbHRoonstraße 25, 90429 Nürnberg Tel.: 0911 273-0, Fax: 0800 20100020 www.novartis.de
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