The Psychological Aspects
The Psychological Aspects
As with any other chronic illness, patients with thalassaemia major face considerable challenges. The physical demands of the disease, as well as a lack of public awareness in many affected countries, mean that both patients and their parents and families experience emotional difficulties in trying to cope with thalassaemia. At various times in their lives, patients may experience many emotions, such as:
- Fear of death
- Lack of confidence
- Feelings of being unloved
- Feelings of being overprotected
- Low self-esteem and many others.
The infant patient - In the early years, a child feels the pain and discomfort of treatment for thalassaemia, even though he or she will not understand what is happening to him/her. However, this is also the time a child develops trust and confidence in whoever takes care of him/her. It is therefore crucial that parents accept the situation as early as possible and begin to build a strong, healthy relationship with the child that includes helping it through treatment. Difficulties tend to emerge more clearly in toddlers, when the child begins to seek greater autonomy, doing things by itself and taking initiative. It is at this point that the child begins to understand that it is restricted in what it can do and - perhaps more pointedly - cannot do what other children of its (his/her) age do. When the child reaches school age, the situation becomes more difficult. In addition to the pain and complications of treatment, an awareness of being different - particularly of looking different, when patients have experienced bone changes and/or delayed growth due to late start of or inappropriate medical care- can have a serious, detrimental effect on a young patient's sense of well being. It is at this stage that patients tend to be most forceful in demanding explanations. Here, parents and physicians play an important role in explaining the disease and, very importantly, in encouraging the child to feel confident. If they succeed without being overprotective (particularly in the case of parents), leading to other negative aspects, the seeds will have been sown for a confident child to grow into a confident adolescent and a confident adult.
This is perhaps the most difficult period - a time when young people are particularly vulnerable as they negotiate the difficult transition from youth to adulthood. It is often at this time that the prospect of a lifetime spent managing thalassaemia becomes most stark but it is also the time the patient needs to recognize that he/she has the power to control his/her quality of life. Rebelling against required behaviour is just as normal amongst teenagers with thalassaemia as it is amongst other teenagers. But for patients with thalassaemia, the risk is that that rebellion will take the form of refusing to comply with treatment, particularly iron chelation therapy. Parents, siblings and medical staff, including doctors, nurses and psychologists, all play a crucial role in providing support to teenage patients with thalassaemia to feel confident and happy and to comply to treatment, reminding them of the dangers of not carrying out their doctor's recommendations. Some doctors use examples of happy, healthy and successful older patients who comply with treatment, in an effort to encourage and support teenage patients.
Once into adulthood, patients face new challenges. Of course, by adulthood, patients have often developed the additional confidence and hope they lacked as children. Like their parents and family, patients will have become stronger and wiser, themselves serving as examples to younger patients and their families. Nonetheless, as patients begin to make lifestyle and career decisions, perhaps moving into long-term relationships or considering higher education, employment, marriage and the possibility of starting a family, the demands of treatment can become particularly irritating. Patients therefore continue to need strong encouragement to keep to treatment regimes - for themselves and for those around them. Additional strains also emerge if a patient finds out that he or she is unable to become a mother or father. Concerns about mortality continue to be a major source of distress, particularly in adult patients who are more aware of the medical complications of the disease and who may have lost friends to the disease. Each of the challenges appearing at different ages and times requires the constant support and encouragement of family, friends and medical staff and very importantly, the positive attitude and effort of patients themselves. How well, confident and mature a patient with thalassaemia major grows to be is related to the level of support he/she receives - as well as the realization that without the patient’s own active involvement, no battle can be won. Provided that appropriate treatment is available and affordable and adequate support is provided, most patients are able to counteract to a great extent the negative aspects of living with a chronic disease, and can fulfil almost all their ambitions and make their wishes and dreams come true. Unfortunately, most of the countries heavily affected with thalassaemia are in the developing world, where the support the national health authorities, parents and medical staff can provide to patients is constrained by very limited resources, poor health structure and other health priorities. One very important role of TIF is the promotion of national thalassaemia associations and the provision of constant encouragement and support to national health authorities of affected countries, in order to make them recognize the problem and the tremendous impact this disease will have on their public health if not appropriately controlled.
Similar negative and positive emotions are experienced by the parents who in addition may feel responsible or guilty for their child's health condition. Most are in shock when their child is first diagnosed. In countries with very little or no knowledge about the disease and its treatment, parents are also unprepared for the intensive home care that is an essential part of their child's long-term prognosis. A diagnosis of thalassaemia may also place considerable strain on a couple's relationship sometimes leading to separation or divorce. On the other hand, the illness may also bring parents closer together, determined to protect and support their child. Equally, however, parents may focus a great deal of their attention on caring for the child diagnosed with thalassaemia, putting a strain on relationships with other members of the family, particularly other children.
The feelings experienced by both patients and parents, combined with the painful reality of long-term treatment that goes far beyond blood transfusion and iron chelation, can often lead to behavioural problems. As a result of the social stigma attached to thalassaemia and other genetic hereditary diseases, many parents may be unwilling to discuss the diagnosis with others, keeping it secret even from close family members. Even now, in countries where the level and quality of public awareness is high and clinical advances have increased the life expectancy and quality of life of patients with thalassaemia, patients and parents and often the whole family still experience these feelings - at least until they learn to accept that they are going to live with thalassaemia, learn how to cope with the disease. A period of grieving by both parents and patients is therefore expected, and should be allowed until strong negative emotions subside and the disease and its care become integrated into their everyday life. The support of professionals during this period may be extremely valuable. The sad part of the story, however, is seen in affected countries with limited resources where patients and parents learn about the disease and its treatment and realize their very limited opportunities to address the problem. This leads to life-long distress, despair, frustration, depression and social isolation. Patients in these countries die at very young ages, often undiagnosed or misdiagnosed, and inappropriately treated or not treated at all.
It is important for parents with an affected child to search for as many sources of information as possible about thalassaemia - in libraries, on the Internet, and from other parents who have children with thalassaemia. National Thalassaemia associations and the Thalassaemia International Federation can play a key role, providing parents with an important source of information and support, helping to strengthen their confidence in helping their child to live and live well with the disease. Parents who are well informed about the disease, its treatment and prevention are best able to support their child and to avoid, if they wish, having a second affected child. Doctors are a very important additional source of information, as well as of guidance and reassurance. The role of the nurse is also pivotal. In some cases, the nurse/patient relationship may be different to that of the doctor/patient. Due to the often lengthy and regular periods of time spent with the patient, the nurse may often be the first one to pick up on specific problems a patient may be experiencing. Furthermore, due to the informal surroundings of the transfusion unit, the nurse frequently comes into contact with the patient's family and friends, the people most likely to be aware of treatment difficulties the patient may have. Often these people will volunteer such information more readily than the patient. It is essential, however, that this information is relayed back to the doctor so that treatment changes can be made. One of the most important areas of the doctor's and nurse's role concerns treatment compliance. Many parents and patients suggest that the most difficult aspect of the medical care in thalassaemia major is beginning iron chelation therapy. Having successfully adapted to monthly transfusions, patients and parents are now faced with yet another hurdle. The medical and nursing staff can often ease this transition considerably, resulting in a more relaxed and successful treatment programme.
Patients with thalassaemia major can certainly marry and have children. Whether their children will be healthy will depend on the thalassaemia status of their partner -- i.e. whether they are healthy, a carrier or a patient themselves.