Death Row Reprieve
Sydney Morning Herald
Friday July 18, 2003
Once considered near fatal, Hodgkin's disease is no longer an automatic death sentence, thanks to medical advances. Ruth Pollard reports.
NEWS that the actor and singer Delta Goodrem was struggling with Hodgkin's disease suddenly hoisted the disease to national attention. But she is not the first famous face to fight the relatively rare lymphoma.
Author Jane Austen is believed to have died from Hodgkin's disease, as did artist Joy Hester and actor Richard Harris.
Indeed, though once considered a near-fatal disease, in recent years it has proved eminently beatable: now it affects only about 130 people in NSW each year.
The change began 40 years ago, as chemotherapy and radiation therapy improved. Survival rates quickly began to rise in some age groups, with early detection, the survival rate after five years is as high as 95 per cent.
The first written description of the disease, also known as Hodgkin's lymphoma, was made in 1666 by Marcelle Malpighi, an Italian professor of medicine. But it was English scholar Thomas Hodgkin's article ``On Some Morbid Appearances of the Absorbent Glands and Spleen" that first documented cases of the cancer. That was in 1832, when a diagnosis meant a death sentence.
Today, with intricate diagnostic scans and improved use of the traditional cancer medicines, radiation therapy and chemotherapy, the cure rate for early-stage Hodgkin's is almost at its pinnacle.
The American Cancer Society puts the one-year survival rate at 93 per cent, the five-year and 10-year rates are 82 per cent and 72 per cent, at 15 years, the overall survival rate is 63 per cent.
Bruce Armstrong, professor of public health at Sydney University and a cancer expert, said the prognosis for Hodgkin's sufferers was not always this rosy. Figures from the United States indicate that from 1960 to 1963 the estimated survival rate across both sexes was 40 per cent poorer than most cancers today.
A decade later the survival rate had jumped to 67 per cent, and it just kept climbing. In some age groups it is now as high as 95 per cent.
``It has been one of the great success stories the other two . . . are acute lymphocytic leukaemia in children and testicular cancer," Armstrong said.
Both successes are attributable to chemotherapy.
In NSW there are 130 new cases of Hodgkin's per year in NSW, he said, translating to about 20 people dying from the disease. About one in every 400 people will get Hodgkin's in their lifetime.
According to Armstrong, the survival rate is best in younger patients, and in women under 45, who are better able to tolerate the aggressive therapy needed to knock the lymphoma out of the system.
And with survival rates as high as they are, Armstrong said further gains would be hard won.
``People are still looking for other infectious agents that might be involved, but so far without much success Epstein Barr virus is where the evidence lies, and maybe that is sufficient to explain its existence."
The deputy director of the Westmead Cancer Research Institute, hemotologist Ken Bradstock, agreed.
He pointed to US research that found an association between Hodgkin's lymphoma and Epstein Barr virus, an infection that causes glandular fever.
Up to 50 per cent of cases appear to be connected to EBV. ``It appears that a portion of Hodgkin's disease is definitely due to Epstein Barr, because the virus is present in the tumour cells what causes the other cases we do not know," Bradstock said.
EBV also causes Burkett's lymphoma, which is endemic among children in Africa.
Armstrong pointed to clusters of Hodgkin's lymphoma occurring in the 1970s in the US, where children at the same school, or in the same class, were developing the disease. A virus like Epstein Barr would be the likely culprit, he said, given at least 12 per cent of all cancers were caused by infection.
The risk of treatment is an issue for most Hodgkin's sufferers one of the downsides of some chemotherapeutic regimes is the risk of developing acute myeloid leukaemia, while radiation therapy puts women at risk of developing breast cancer.
However Armstrong said: ``It is much better targeted than it used to be, so the collateral damage from radiation therapy has been somewhat reduced."
Despite better targeting and shielding in the use of radiation therapy, the danger is still there. This month's Journal of the National Cancer Institute warns that the breast cancer risk increases with increasing radiation doses, and that women undergoing radiation therapy for Hodgkin's disease have an increased risk of breast cancer.
Lymphomas are the fifth most common form of cancer, and Hodgkin's represents less than a quarter of lymphomas overall, the NSW Leukaemia Foundation says.
Experts say Hodgkin's disease can start almost anywhere in the body that lymph nodes are present. Found in the neck, underarm, groin, chest, abdomen and pelvis, lymph nodes make and store infection-fighting white blood cells, called lymphocytes.
If left untreated the disease can be fatal, penetrating blood vessels and spreading to other parts of the body.
Classic symptoms include painless swelling in the lymph nodes in the neck, underarm or groin, unexplained recurrent fevers, night sweats, unexplained weight loss and itchy skin.
Bradstock said new ways of measuring the extent of the disease had a huge effect on the success of treatments.
``Twenty years ago, a person would have been treated, for example, with radiotherapy, which is highly effective but sometimes it missed some parts of the disease.
``We didn't have . . . the fancy nuclear technology that we have today PET scans and CAT scans provide a better way of looking at the extent of the disease."
There are no new miracle treatments for Hodgkin's but there are better drugs with fewer side effects.
Bradstock said: ``Most patients will be treated with combinations of chemotherapy and radiation therapy, and the treatment is tailored to the person's disease."
``We ration the treatment depending on how much disease is there, with the aim of reducing the side effects and improving recovery time."
For those with extensive Hodgkin's disease, who would have received a bad prognosis in the past, there are new cocktails of old chemotherapy drugs.
``It is a more intelligent use of the old drugs," Bradstock said.
Hodgkin's disease responds well to treatment, because the cells of the immune system appear to be more prone to die when exposed to therapy.
That differed, Bradstock said, from other cancers like breast cancer, where the tumour cells appeared to have more inherent resistance to treatment.
Recurrences do occur, especially in advanced Hodgkin's disease.
``Recurrence is variable the bottom line is, there is a really high response rate to the treatment so most people will get a complete recovery," he said.
Although seen in all ages, Australian Institute of Health and Welfare figures show Hodgkin's lymphoma most commonly occurs in people in their 20s and 30s.
It is often the fact that the disease targets this age group those in the prime of their lives that captures people's attention, NSW Leukaemia Foundation spokeswoman Gabrielle Prest said.
``People are at a stage of their life where things like careers and starting families are foremost on their minds, which makes rehabilitation after therapy so important.
``They must consider issues like employment, insurance, a future family and fertility, because of the side effects of therapy these are the key psychosocial issues people battle with."
Prest said women may choose to harvest eggs, and men may bank their sperm to assist with fertility in the future.
But one of the major hurdles many face is employment discrimination. Prest said the foundation had regular reports of people unable to find work after being unemployed for up to a year while receiving treatment.
``Employers are scared about employing them because they do not understand the nature of the disease," she said.
``We even had one employer who refused to consider hiring someone because they thought Hodgkin's was infectious."
There is one key message Prest is keen to push early detection means early
treatment, and even better survival rates.
A GOOD PROGNOSIS
Number of new cases and deaths from Hodgkin's disease. Australia, 1992-1997
NEW CASES DEATHS
FIVE-YEAR RELATIVE
SURVIVAL
(%)
AGE MALES FEMALES MALES FEMALES MALES
FEMALES
0-4 184 151 12 8 95.7
95.1
5-9 265 274 14 21 96.1
92.4
10-19 211 189 28 7 87.9
95.6
20-29 169 90 19 13 88.5
86.6
30-39 107 65 34 17 68.5
77.9
40-49 134 93 68 39 56.3
62.0
50-59 94 93 59 55 51.1
52.6
60-69 32 40 28 33 21.8
23.4
All ages 1198 996 264 193
82.6 84.4
SOURCE: AUSTRALIAN INSTITUTE OF HEALTH AND WELFARE CANCER SURVIVAL IN AUSTRALIA
2001
© 2003 Sydney Morning Herald
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