Acute Lymphoblastic Leukaemia

Acute lymphoblastic leukaemia or ALL, as it is usually called, affects children between the ages of two and seven and rarely after fifteen. Leukaemia is a generalised disorder of the bone marrow, which is where white blood cells come from. It is in this spongy network of tissue which fills up the cavities of the bones that the uncontrolled reproduction of white cells takes place in leukaemia. What it is that produces the leukaemia remains unknown; it does not seem to be related to a child’s health or diet.

Symptoms

One of the key points of leukaemia is the increased reproduction of the white cells which replicate in such numbers as to swamp the bone marrow, and so interfere with the development of normal cells in the bone marrow. The resulting decrease, particularly in the normal red cells, is responsible for many of the symptoms of leukaemia. Therefore, one of the first signs will be anaemia, which shows that the number of red blood cells has decreased. As a result, the child will be very pale and have much less energy than usual. The other bone marrow cells whose numbers are usually decreased are the platelets. When these cells are severely reduced in numbers, they may make the patient prone to spontaneous bleeding and excessive bruising. Another symptom is that the child is more open to infection, because of the poor quality of the rapidly dividing white cells, which are unable to defend the body against disease-producing organisms.

Diagnosis

An early diagnosis of childhood leukaemia is very difficult because the signs are the same as those of many other minor illnesses through which children always seem to be passing and which are generally treated at home without medical help. No blame can possibly be attached to parents for any delay in taking their child to the doctor for diagnosis. The suggestion that had the child received treatment sooner, the severity of the disease might have been lessened or the child might even have been cured, is incorrect because the long-term response to therapy in childhood leukaemia is not clearly affected by how early the disease is diagnosed. Eventually the diagnosis is made by a simple blood test, which will reveal the true nature of the disease. It is confirmed by removing some tissue from the bone marrow and examining it under a microscope.

Treatment

Acute lymphoblastic leukaemia in children is no longer an incurable disease as a result of the vast progress made in chemotherapy in the past 20 years. Multiple drugs are used simultaneously, the main idea being that, whereas the cancer-destroying effect of the various drugs will be cumulative, each of these drugs will have different but mild side-effects, which will make them much more tolerable. An important feature of this drug treatment is in reduction of the dose of each of these drugs, which will prevent permanent damage to normal tissues.

The price of a cure for a child receiving chemotherapy is a very high one in terms of side-effects. Loss of hair, though temporary, can be very traumatic to a child attending school, because he may become the target of cruel comments from other children. The feeling of tiredness, nausea and vomiting, with total lack of appetite, is also very hard for a child to bear.

The parents’ burden is a heavy one, because of the agony associated with seeing one’s child suffer as a result of the treatment, and with regard to the uncertainty of the prognosis. They will also have to make a very difficult decision if the disease affects a boy. It has been found that the leukaemic cells use the boy’s testes as a sanctuary from where, after months or years, a re-infection may occur. This discovery was made as a result of the vastly superior cure rates in girls than in boys.

To eradicate the disease from the testes, X-ray therapy is used which, of course, will sterilise the irradiated boy for life, meaning that although he will not be impotent and should be able to develop most of the male secondary characteristics and can lead a normal sexual life, he will be infertile and not able to have children of his own. This is a hard decision which parents have to make on behalf of a boy usually only 7-12 years old.

Twenty years ago most children died within a month of the diagnosis. Nowadays 70 per cent of children will live five years and 50 per cent of these are eventually cured, but the combined course of treatment lasts about four years.

Treatment is given in three stages. During the first stage the blood returns to normal. However, the disease may still be present in the brain, spinal cord and testes. Treatment to the brain and testes is usually given by means of radiotherapy, whereas the spinal cord is treated by chemotherapy when the drug is directly injected into the spinal canal during the second stage of treatment. This protective phase is directed at the central nervous system because, as mentioned before, it so frequently offers sanctuary to leukaemic cells. Finally, in the third stage, maintenance drug therapy is carried out for two to four years.

The two drugs usually used in the first stage of treatment are vincristine and predinsone. Vincristine is given once a week by injection, whereas predinsone is taken orally in pill-form, several times a day. Vincristine can cause nerve damage resulting in a feeling of tingling mainly in hands and feet. Predinsone increases the appetite and leads to puffiness of face, which is sometimes described as ‘moonface’, but these changes are only temporary.

By the end of one month’s treatment of ALL, the blood test results usually return to normal and the bone marrow appears free of leukaemic cells.

In the second stage, methotrexate is injected directly into the spinal canal. This spinal puncture, carried out under local anaesthetic, is not actually painful, but it can be somewhat unpleasant. Radiation to the head is quite free of bothersome side-effects except, of course, for the temporary loss of hair.

The maintenance therapy can last up to four years. The drugs, usually given by mouth during this period, are in such moderate doses that they rarely cause any noticeable side-effects. The only upsetting aspect of treatment during this final, though long phase, is the bone-marrow examination needed every two months to check for signs of a relapse.

At the end of these three or four years of treatment, all chemotherapy is stopped, provided that the leukaemia has not recurred. After this the child will be monitored by means of blood tests and/or bone marrow punctures at three to six month intervals.

The chances of a permanent cure following this treatment are excellent. During treatment, the child is much more liable to contract a sore throat, a cold, flu, a cough or other infections, because the immune defence system is depleted, as a result of the effects of the drugs on the white cells. Early treatment of such infections is necessary, because the child becomes very sick with these everyday infections that would not really affect a healthy child at all. Chickenpox in a child with acute leukaemia is particularly hazardous, because it can cause fatal pneumonia.

Parents of children with acute leukaemia must always live in fear that their child will contract one of the childhood illnesses and die. The compromise between being overprotective and isolating the child from friends and schoolmates and allowing him to lead a near normal life is a difficult one for the parents to get absolutely right.

The prognosis in children developing recurrent leukaemia is an unfavourable one. In these serious cases, a bone marrow transplant may have to be considered because it may save about 10 per cent of these children even after the disease has recurred.

It must, however, be remembered by all concerned that a bone marrow transplant is a hazardous procedure which involves whole body irradiation (or a large dose of drugs, which is used to destroy all bone marrow cells, a procedure which can be associated with particularly unpleasant nausea and vomiting. To be successful, normal marrow from a brother or sister has to be transplanted into the sick child’s marrow, where it will grow and produce all the various types of normal blood cells. However, unless the donor is an identical twin, these cells will differ from those of the patient. As a result they will be recognised as foreign by the body’s immune system and cause a mostly fatal graft versus-host disease, which damages organs such as the skin, the gastrointestinal tract and the lungs. The chances of a permanent recovery are only about 50 per cent and are best when the transplant occurs before the disease recurs.

Although the cure rate of ALL continues to improve, a number of children will inevitably die of the disease. When this happens, it is important for the parents to feel, looking back, that they have tried all known treatments and that they had made their sick child’s last few years as comfortable and as enjoyable as was possible. They will always think of him, they will always miss him and they may often cry for him, but they will never have reason to reproach themselves for not having tried harder.

In a recent review of the psychological consequences of childhood leukaemia it was found that 2~30 per cent of parents required psychiatric treatment (mainly for anxiety and depression), that 25 per cent of mothers experienced chronic sexual difficulties and that 20 per cent of parents had serious matrimonial disharmony: such is the emotional burden borne by the anxious relatives of a child with cancer.