10: Psychological Support of Thalassaemia
Why is psychological support so important?
It is now universally recognized that thalassaemia, like other chronic diseases,
has important psychological implications. The way in which the family and the
patient come to terms with the disease and its treatment will have a critical
effect on the patient’s survival. Without an understanding and acceptance of
the disease and its implications by patients and family, the difficulties of
lifelong transfusion and chelation therapy will not be faced, leading to an
increased risk of disease complications and poorer survival. A key role for
doctors and other health care professionals is to help patients and families
face up to the difficult demands of treatment. Monthly contact with the
thalassaemia centre allows doctors and other members of the team to act as a
reference point for the patient’s overall state of health. This regular interaction
also provides the doctor with a good opportunity to promote the patient’s
physical, emotional and social development.
Management of thalassaemia is based on the therapeutic alliance between doctor and
patient throughout the course of the disease. Because of the objective and diseaseoriented emphasis of medical education, many doctors find it difficult to come to
terms with the psychological demands of treating chronic inherited diseases. This can
be made more difficult for the doctor because patients with thalassaemia often express
strong negative feelings, which can hamper communication. Furthermore, after many
years of treatment, patients and family are often better informed about the illness than
non-specialist clinicians, which some doctors may feel undermines their perceived
role. All of these factors can make honest, in-depth communication, which is vital to
successfully coping with thalassaemia, extremely difficult to maintain.
The psychology of inherited chronic disease
Every genetic disease implies, regardless of its aetiology, a sense of guilt. This
may interfere with the primary parent-infant relationship. As its clinical
manifestations develop in the first year of life, the disease can have also a
negative impact on the parent-child relationship. Moreover, the treatment is
emotionally demanding, as transfusion and chelation therapy require repeated
invasive procedures and hospital visits.
Because thalassaemia major typically “starts” during the first year of life and
requires a monthly follow up, it allows the center doctor to take on many of the
characteristics of the traditional “family doctor,” as guardian of the patient’s
overall wellness – both physical and psychological.
Chronicity is a strong font of emotional problems that intensify at each
significant developmental stage of the patient’s life. Patients can feel that they
are different, limited, or isolated. Their state of mind can shift rapidly from
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depression to anger and vice versa. The doctor must be prepared to accept
this shift and to help them deal with these feelings.
Communication by health care professionals with patients
This should include as far as possible:
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Listening – to be interested in the patient’s emotional and real
experiences
Accepting – both respecting the patient’s point of view and being
sensitive in the timing of personal communication
Sharing – being consistently close to the patient’s positive and
negative feelings as human being.
Understanding – not simply at an intellectual level but at the emotional
one
Maintaining boundaries – giving help and relief, but keeping in mind
his/her role as a physician
Settings and methodology for discussion are important all along the course of
the disease, but are mandatory at crucial milestones in the patient’s
experience:
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diagnosis
first transfusion
start of chelation
puberty
serious complications
This type of interaction can be extremely beneficial for the patient, helping
him/her to cope better with thalassaemia and to maintain a sense of balance. It
can also be extremely rewarding for the physician, both in medical and
emotional terms. If the doctor manages to maintain a constant dialogue, s/he
can often find in thalassaemic people skills greatly surpassing those of their
peers when facing the great challenges of life such as birth/death,
love/loneliness, and possibilities/limits.
Coming to terms with the diagnosis
The day of diagnosis is usually recalled by parents as shocking and
overwhelming. To assist them in coping with this distressing information,
communication about the diagnosis has to be done within a specific setting:
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The room and time should be chosen to provide an atmosphere that
will sustain hopes without deluding or depressing.
The doctor should discuss the diagnosis with both parents together,
allowing ample time to listen to their concerns and respond to
questions.
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Information must be sincere, complete, and repeated as often as
needed. The weight of negative emotions may be so great that
parents may appear confused even after complete information has
been given more than once.
In the months following diagnosis, the discussion must be renewed,
with the same attention to the setting, and preferably with the same
doctor to preserve continuity.
The child has to be included as soon as possible. From as early as three to
five years of age, young patients begin to ask crucial questions about
duration of care and possibilities of recovery. These should be dealt with
sensitively and honestly.
Psychological impact of anemia and transfusion
Serious anemia will cause the patient to feel weak and vulnerable. Maintaining
an adequate haemoglobin level through optimal transfusion therapy (see
Chapter 3) eliminates these symptoms and reduces the patient’s anxiety about
death. However, the decrease in haemoglobin during the transfusion interval
may allow these symptoms to recur. This gives the patient the experience of
instability and doubt about his/her physical capabilities.
The need for periodic transfusions testifies that vital energy comes from other
people, which implies dependence. This therapy does not cure; it merely
compensates as a monthly “patch” for the anemia, giving life and well-being
but also carrying the damaging by-products of viruses and iron, which
necessitate additional treatment. This combination of advantages and
disadvantages of transfusion finds a parallel in patents’ psychological
reactions to their treatment. Multiply transfused patients can experience both
positive feelings such as gratitude and negative ones of being contaminated or
ruined.
Psychology of chelation therapy
The doctor should be very familiar with the emotional aspects of chelation, as
compliance with therapy determines prognosis.
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Chelation is a psychologically demanding treatment as it is “the patch
of another patch”; it partially treats the complications of another
treatment (transfusion).
Like transfusion, it is a reminder of one’s illness, but on a daily basis.
Optimal chelation starts during the first years of life.
It implies a small act of aggression, either self-directed or inflicted by
the patient’s loved ones.
Skin punctures from the needles cause body image damage. The
patient may feel “as full of holes as a colander.”
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Time and movement restrictions related to use of the pump generate
feelings of being different and limited.
Effectiveness of the drug cannot be checked quickly and directly by
the patient. So compliance is a function of trust; that is to say, it
reflects the quality of the doctor-patient relationship and a belief in
long-term benefits.
These emotionally distressing aspects of chelation may give rise to a
counterproductive tendency on the part of the patient with thalassaemia, the
parents, and even the treating physician to tacitly encourage non-compliance
with therapy in order to avoid negative psychological states.
Parents may:
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Not yet have overcome the shock of diagnosis. Administering the
infusion can be painful since they feel responsible for their child’s
discomfort.
Use chelation as a control tool when the child reaches adolescence.
Patients may:
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Realise only after many years of poor chelation how harmful iron
overload can be.
Allow themselves a state of ‘denial’ of the links between poor
compliance and mortality.
Adopt attitudes of out-and-out refusal, feeling “tortured” instead of
cared for.
Exploit any opportunity or excuse to skip that day’s infusion.
Allow feelings of low self-esteem to harm their attitudes to self-care
and self-treatment.
Repeatedly select the same sites for needle insertion, allowing local
reactions to become more frequent and severe.
Physicians may:
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“Bargain” with the patient, prescribing a lower dose or frequency of
desferrioxamine than indicated for the amount of iron loading.
Be rigidly inflexible, insisting on prescribing and judging, reprimanding
or even threatening the patient.
While the underlying motivation for these reactions is generally a desire to
provide relief from the patient’s discomfort and to make him/her feel better, the
long term effects of such behaviour are harmful for the patient’s physical
health and emotional well-being.
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Recommendations:
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Define and resolve the practical aspects of optimal chelation (see
Chapter 5).
Pay due attention to the psychological aspects, as underestimating
these undermines the effectiveness of the doctor-patient relationship
and treatment failure increases.
Promote the shift from parent to patient management as early as
possible. Many patients with thalassaemia can begin to take control of
their medication regimen from 6 years of age. The early initiation of
self-management limits overprotection and stimulates autonomy in the
young patient. It also gives relief to the parents and ultimately
improves the quality of life of the whole family.
Encourage patients to feel a sense of reward for achieving mutually
agreed therapeutic goals.
Remember that long-term high compliance fosters good capability and
self-reliance and is a key positive factor in maintaining emotional wellbeing.
Psychological impact of disease complications
In thalassaemia, a serious complication such as cardiopathy or diabetes may
appear during adolescence or young adulthood. The patient undergoes a
period of psychological readjustment, as s/he must try to integrate the hopes,
enthusiasm and wishes typical of youth with a damaged physical state and
medical features typical of old age. In such a situation, the inadequately
supported patient may feel “hopelessly ruined”, giving up on health and
continued therapy.
Even in very serious cases, it is still possible to deal with suffering by sharing
and working together to find ways of accepting the new limits set by the
situation. The doctor should encourage the patient to keep on caring, even
when the complication suggests a poor prognosis for survival.
Summary of psychological goals
These are:
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To provide information which promotes understanding of the illness
To help the patient and parents to talk and express feelings about the
illness
To help the patient accept the illness and take care of himself
To maintain realistic hopes
To facilitate a “normal” lifestyle and to encourage self esteem.
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To put in practice these tasks, the doctor should be:
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Open minded about psychological aspects of having and treating
inherited disease
Trained in normal psychosocial development from childhood to
adulthood
Sensitized regarding special issues of this chronic hereditary disease
Available to accompany and support the patient all along his life path.
It is clearly not possible for a doctor to provide the latter support if the
organisation of the healthcare system does not allow him or her the
opportunity to work with patients on a long-term basis. The ‘rotation’ of
experienced doctors to different centres, or the moving of patients to a
different clinic at an arbitrary age, can seriously undermine a patient’s
psychological well-being, treatment and prognosis. Appropriate psychological
support therefore not only requires motivated and able clinicians, but also
presupposes an organisational structure which allows the successful delivery
of optimal and comprehensive care.
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