Chapter 15Psychological Support in Thalassaemia

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Why is psychological support so important?

It is now universally recognised 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 and quality of life. Without an understanding and acceptance of the disease and its implications, 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 treating physicians and other health care professionals is to help patients and families to face up to the difficult demands of treatment, while maintaining a positive role.

Adherence to treatment is a basic goal, but a general acceptance by the patient of his/her own condition constitutes the key to normal development from childhood to adulthood.

Monthly contact with a local thalassaemia centre from the first years of life allows doctors and other members of the team to act as a reference point for the patient’s overall state of health, including general attitude and well-being. In addition, this regular interaction provides to the whole staff, and in particular to the treating physician a good opportunity to promote the patient’s physical, emotional and social development, taking on several characteristics of the traditional ‘family doctor’ as guardian of the patient’s overall wellness.

The success of management of thalassaemia is based, to a great extent on the establishment of a therapeutic alliance between caring staff and the patient throughout the course of the disease. Because of the disease-oriented emphasis of medical education, many health professionals find it difficult to come to terms with the psychological demands of treating chronic inherited diseases. This can be made more difficult in thalassaemia because patients often express strong negative feelings, which can hamper communication. Furthermore, after many years of treatment, patients and family may be better informed about the illness than non-skilled health professionals—a factor that can undermine the health professionals’ perceived role. Taken together, 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, regardless of its aetiology, implies a sense of guilt that 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.

Chronicity is a powerful source 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 depression to anger and vice versa. Health workers must be prepared to accept this shift and to help patients deal with these feelings, finding a way to their own ‘normalisation’ that implies different individual styles in adult life.

Overall, good treatment facilitates personal development and achievement of targets in life, while poor care makes such development difficult or unpredictable.

Communication: healthcare professionals with patients

Healthcare professionals should seek, as far as possible, to

  • Listen – to be interested in the patient’s emotional and real experiences
  • Accept – to respect the patient’s point of view and be sensitive to the timing of personal communication
  • Share – to be consistently close to the patient’s positive and negative feelings
  • Understand – at an emotional and not simply an intellectual level
  • Maintain boundaries – to give help and relief, but keeping in mind his/her role as a physician

Good communication with healthcare professionals 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 healthcare professional, both in medical and emotional terms. Where a healthcare professional manages to maintain a constant dialogue with his/her patients, s/he will often discover in patients with thalassaemia skills that greatly surpass those of their peers when facing the great challenges of life such as birth/death, love/loneliness, opportunities/limits.

Caring for a ‘normal’ development

Settings and methodology for discussion are important all along the course of the disease, but are mandatory at crucial milestones in the patient’s and parents’ experience: At the onset and during the first period, communication work is carried out with parents, but 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 the duration of care and possibilities of a cure. These should be dealt with sensitively and honestly. Separate interviews with patient and with parents are recommended in the approach to adolescence, while in adulthood individual interviews with the patient are essential.

Communication of diagnosis

As exemplification, it is useful to focus on communication of diagnosis, because it is the natural starting point of the whole course of disease and may mark permanently (positively or negatively) the therapeutic relationship.

In trying to establish an ideal setting, the following points should be considered:

  • The room chosen and time allocated aim to provide an atmosphere that will sustain hope and optimism; and not make the patient feel depressed or misled.
  • The diagnosis should be discussed with both parents together, allowing ample time to listen to their concerns and to respond to their questions, worries and concerns.
  • 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 given to the setting, and preferably with the same doctor to preserve continuity.

The same attention to setting must be provided at any significant step, in order to better support patient/parents in coping with distressing information.

Psychological impact of anaemia and transfusion

Serious anaemia will cause the patient to feel weak and vulnerable. Maintaining an adequate haemoglobin level through optimal transfusion therapy (see Chapter 2: Blood Tranfusion Therapy in β-thalassaemia Major) 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. Moreover, because of the risk of transfusion-transmitted diseases, fears of being contaminated are ever-present and may be intense for real reasons (high risk of transmission) or due to the patient’s emotional state. This fact helps to sustain ambivalence towards therapy.

In any case, the need for periodic transfusions testifies that vital energy comes from other people, implying a dependence at the physical level that can invade the mental level, limiting personal development. In addition, transfusion therapy does not cure; it merely provides a monthly “patch” for the anaemia, giving life and well-being but also (even if safe from infection) causing iron overload, which necessitates additional treatment that is also lifelong.

This combination of advantages and disadvantages of transfusion finds a parallel in patients’ psychological reactions to their treatment.

Regularly transfused patients can experience positive feelings, such as gratitude for receiving life, and negative ones such as fear and anger at being ‘ruined’.

Psychological aspects of chelation therapy

Treating staff should be very familiar with the emotional aspects of chelation, as compliance with therapy determines prognosis (see Table 1).

In general, chelation is a psychologically demanding therapy, because:

  • Iron chelation does not cure but rather treats the main complication of the basic therapy (transfusion), as ‘the patch of another patch’.
  • Like transfusion, it is a reminder of one’s illness, and even more on a daily basis.
  • Optimal chelation starts during the first years of life.
  • 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 treating staff-patient relationship and a belief in long-term benefits.

Subcutaneous chelation

Parenteral treatment 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’.

Time and movement restrictions related to the use of the pump generate feelings of being different and restricted.

Parents may:

  • 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:

  • Adopt attitudes of out-and-out refusal, feeling ‘tortured’ instead of cared for.
  • Exploit any opportunity or excuse to skip a day’s infusion.
  • Repeatedly select the same sites for needle insertion, so trying to reduce the body image damage.

Physicians may:

  • Bargain’ with the patient, under-prescribing desferrioxamine, more for psychological reasons than on the rationale of iron balance.
  • Tacitly encourage non-compliers to treatment in order to avoid the development of negative psychological states.

While the underlying motivation for all the above reactions or attitudes 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.

Oral chelation

Oral administration simplifies significantly many practical aspects of chelation management with Deferrioxamine. For some patients (and some healthcare professionals), a shift to oral chelation may seem ‘the solution to every problem’. In fact, however, oral chelation only helps with the issues of daily ‘holes’ to the skin and consequent damage to body image. Patients taking oral chelators must still face the daily feeling of being different and will still lack the means to immediately and quickly assess the impact of treatment and in this context it will remain difficult for some patients even on oral chelation to maintain adequate compliance.

Recommendations

  • Define and resolve the practical aspects of optimal chelation (see Chapter 3: Iron Overload).
  • Avoid judging, reprimanding or threatening the patient.
  • Pay due attention to the psychological aspects of the disease, as underestimating these undermines the effectiveness of the treating staff-patient relationship with an increased risk of treatment failure.
  • Be involved in supporting, instead of simply insisting or prescribing.
  • 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 six years of age. The early initiation of self-management limits overprotection and stimulates autonomy in the young patient. It also gives relief to 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 well-being.

Psychological impact of complications

During adolescence or young adulthood various complications may occur. The psychological implications of such complications lie in their degree rather than their onset. In general, asymptomatic complications do not require medication and do not interfere heavily with quality of life. However, when a serious complication such as heart disease or diabetes appears, the patient undergoes a period of psychological readjustment. S/he has 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.

Table 2 outlines the impact of the most common complications (at moderate/severe stage) on patient emotional balance.

In contrast to the past, recent advances in iron chelation therapy have led to a dramatic progress in survival, in saving patients from acute life-threatening heart failure, and generally in improving quality of life. Treating staff must maintain a positive perspective and support a patient’s sense of hope.

Even in very serious cases, it is still possible to deal with suffering by sharing and working together to find ways of accepting new limits inherent to a given situation.

Challenges for the adult patient

If the disease is fully compensated, physical conditions allow the patient with thalassaemia to make his/her choices of adult life without any restrictions or limitations. Even in this ideal state, however, at a psychological level young adults with thalassaemia can run into more difficulties than peers in coping with the tasks of adult life, particularly those implying independence and responsibility.

Beginning a new job or an important loving relationship can increase feelings of inadequacy and fragility. Sometimes an emotional crisis occurs and psychological support may be required.

Treating staff should accompany the patient along his/her life path, while respecting his/her fragilities, sensitivity, and supporting resources. The most common mistakes on the part of healthcare professionals is that they are being overprotective or disinterested. On the other hand, special care should be taken in preventing intrusions into the patient’s privacy.

Summary of psychological goals

In terms of the psychological care of the patient, healthcare professionals should aim to:

  • Provide information that promotes understanding of the illness
  • Help patient and parents to talk and to express feelings about the illness
  • Help the patient to accept the illness and to take care of him/herself
  • Maintain realistic hopes
  • Facilitate a ‘normal’ lifestyle and encourage self-esteem
  • Support the full development of an adult life

Putting these goals into practice requires health professionals to be:

  • Open-minded about psychological aspects of having and treating inherited disease
  • Trained in normal psychosocial development from childhood to adulthood
  • Sensitised to the special issues of this chronic hereditary disease
  • Available to accompany and support the patient throughout his/her life path

It is clearly not possible for a health professional to provide all the above support if the organisation of his/her healthcare system does not provide him or her with the opportunity to work with patients on a long-term basis. The ‘rotation’ of experienced professionals to different wards 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 that allows for the successful delivery of optimal and comprehensive care.