When Janice Wheeler discovered a lump under her right jaw, she thought it was a blocked saliva gland. She ignored it, and it seemed to go away. One year later the lump reappeared and this time she went to her GP who referred her to hospital for a biopsy. The diagnosis of non-Hodgkin's lymphoma came as a shock to her but, unlike King Hussein of Jordan, who succumbed to the disease on Sunday after many attempts at treatment, two years after chemotherapy Janice remains well and free of disease.
'I never really thought of myself as ill. I had chemotherapy every three weeks for four months and felt rotten for about three days each time. But you can stick anything if you know the end is in sight. I had treatments all through the summer and I wasn't working at the time so I took up drawing and, apart from the few days when I felt extremely tired, actually had a great summer,' says Wheeler who's now 62. Non-Hodgkin's lymphoma has the dubious distinction of being the fastest-increasing cancer in the UK after lung cancer in women, and malignant melanoma. However, new treatments now coming into use promise to dramatically improve the prognosis of the disease.
The reason for NHL's inexorable rise remains ill understood. Part of it is because NHL is particularly common and virulent among patients with AIDS and others whose immune systems are suppressed by chemotherapy or after transplants.
However, King Hussein's 11 children will be concerned to learn that a family history of NHL increases an individual's risk by three to four times. Older people are more at risk of developing the cancer and it is uncommon below the age of 30, although, tragically, children can develop a rare form of it. There have also been suggestions that people who work with pesticides and those who use hair dyes are at increased risk, though the evidence is weak.
Lymphomas are cancers of the lymph system which sends thin, branching tubes throughout the body carrying a clear, watery fluid called lymph. Lymph contains white blood cells (lymphocytes) which fight infection. Small, bean-shaped organs called lymph nodes or glands are positioned along the tubes and these nodes make and store a range of infection-fighting cells.
Lymphomas come in two types, Hodgkin's and non-Hodgkin's, based on the way the cancer cells look under the microscope. King Hussein apparently had NHL for many years, making it likely that he had an 'indolent' lymphoma which causes fewer symptoms and spreads less rapidly than an 'aggressive' type.
Initially, NHL can be hard to diagnose because the symptoms can be attributed to general tiredness, viral infection or even the menopause. It causes painless swelling in the lymph nodes of the neck, underarms or groin, unexplained fever, drenching night sweats, tiredness, unexplained weight loss or itchy skin.
Treatment depends on age, general condition and whether the lymphoma is confined to one area of the body, or has spread throughout it. This is determined by CT scans and further tests.
Treatment now falls into three main types: radiotherapy, chemotherapy and biological therapy which uses the body's own immune system to fight the cancer. According to Professor Grant Prentice, consultant haematologist at the Royal Free Hospital in London, new therapies mean the majority of patients with aggressive NHL can now expect that treatment will halt the disease.
'Fifty to 70 per cent of people with NHL will go into remission after chemotherapy. The problem is that less than half remain in remission forever, so the disease can recur.' Research is now concentrating on three key areas and there is great optimism among experts that better cure rates can be achieved, with fewer side effects than current treatments allow, says Prentice.
If chemotherapy doesn't work, patients can be given manufactured antibodies - copies of another fighting component of our immune system - which attach themselves to particles on the lymphoma cells and destroy them without damaging normal cells. This form of therapy is marketed under the name Rituxan and, according to Prentice, is the most rapidly selling anti-cancer drug of all time.
'It produces excellent results in over 50 per cent of cases but it is probably not curative, it just increases the chance of remission,' says Prentice.
A further development is to attach a radioactive substance, like radioactive iodine, to the antibody so that the lymphoma cells are attacked by both the antibody and the radioactive substance.
The third field of research is in bone marrow transplant - the final approach tried with King Hussein. 'In severe cases, we need to give very high doses of chemotherapy or radiotherapy to eliminate lymphoma cells. This also knocks out normal bone marrow cells which are needed to make blood cells. Cells from a donor can replace the bone marrow cells and can also have a useful effect of stimulating the patient's immune system to attack any remaining diseased cells.' But, the immune response can also lead to rejection of the donor bone marrow cells, which is a potentially fatal complication for the patient.
A new drug, fludarabine, works very well in helping to keep this immune reaction under control and means that in future bone marrow transplantation is more likely to succeed and less likely to kill the patient.
These developments are too late for King Hussein of Jordan, but they will, hopefully, change the long-term outlook for others with NHL, and allow Janice Wheeler a long, happy, disease-free retirement.
