Since a lot of people now ask me questions about the nature of my partner Donald's illness, what it involves and how it can be treated, I've created this to help provide an explanation. The term 'leukaemia' refers to any cancer of the lymphatic system, of which there are several. Donald has acute lymphoblastic leukaemia, so that's what I'll be discussing here.
Acute lymphoblastic leukaemia is a cancer of the lymphatic system which affects neutrophyls. Neutrophyls are the type of white blood cells which actually attack and combat invading bacteria, after other white blood cells have located, identified and tagged those invaders. In this type of leukaemia, the body produces neutrophyls which divide too fast and never properly mature. These cancerous cells are not capable of combating infection. Furthermore, because they soon become produced in such great quantities, they prevent the body from producing healthy neutrophyls. The cancerous cells travel around the body and collect in lymph nodes, in places like armpits and the glands in the throat, often causing swelling. Occasionally, this swelling has a dramatic negative effect on the patient's health by itself. However, most people with acute lymphoblastic leukaemia do not die from the disease itself, just as people do not die directly from HIV; they are usually killed by bacterial infections which can easily destroy a body with no defences. Even very minor infections and infections which do not normally attack humans can be fatal in this situation.
Acute lymphoblastic leukaemia usually occurs in children. Donald was nineteen when he contracted it, which is very unusual. It is a very rare condition in anybody.
The practice of using chemotherapy originally developed when doctors observed that people who had cancer when they went to war, and who were subsequently exposed to mustard gas in the trenches, often came home with their cancers diminished or less advanced than would have been expected. Since then, chemotherapy research has been endeavouring to find ways of attacking cancerous cells as specifically as possible, doing the minimum amount of damage to the rest of the body. If the genetic structure of a particular type of cell can be mapped, and the difference between a healthy cell and a cancerous cell of that type identified, it is sometimes possible to engineer a retrovirus, an infection which attacks and destroys the cancerous cells without hurting the healthy ones. Advances have recently been made in this area with regard to brain cancer. Research of this type is taking place for acute lymphoblastic leukaemia, but as yet it has produced no practical results.
Chemotherapy for acute lymphoblastic leukaemia concentrates on wiping out the body's neutrophyls - ideally, this will result in all the cancerous ones being destroyed, so that after the treatment finishes healthy neutrophyls are able to grow in their place. Because this means that a patient undergoing such chemotherapy has a damaged immune system, such patients have to be kept in isolation rooms, where the chances of infections reaching them are minimised, and they are treated with heavy doses of intravenous antibiotics as soon as there is any sign of infection. As far as I am aware, Donald is as yet the only person in the world to have completed a year's course of inpatient chemotherapy (his first year of it) without once contracting a single bacterial infection. Almost everyone catches something; the hope is that the antibiotics can destroy the infection before it causes serious damage or proves fatal.
Because this chemotherapy is still not very accurate, other cells are destroyed as well as the neutrophyls. This means that the patient also becomes more vulnerable to fungal and viral infections. Lack of red blood cells can cause anaemia and lack of platelets can cause haemophilia, though these rarely become serious problems, since they can be treated by giving the patient new red blood cells or platelets from a donor's blood. White blood cells cannot be provided in this way because they would recognise the patient as foreign (having different DNA from themselves) and go on the attack. After each bout of chemotherapy the patient has to sit around and wait for the white blood cells to grow back.
As chemotherapy essentially consists of poisoning the body, it makes most patients feel quite ill; some describe it as like having a permanent hangover. Certain types of chemotherapy can cause a burning sensation in the veins when they are being administered. Nausea is common, though usually quite treatable. Headaches occur, and patients tend to feel exhausted all the time. Stamina is often damaged in the long term.
Remission is achieved when no further cancerous cells can be found in the patient's blood or bone marrow. Every patient treated for acute lymphoblastic leukaemia receives at least three months' initial chemotherapy to try and ensure that remission has occurred. It only takes one surviving cancerous cell to start the whole illness over again. Sometimes, cancerous cells hide in the fluid around the brain and the spine, where they are harder to treat and harder to identify. Radiotherapy is usually applied to the head in an attempt to decrease the likelihood of these cells surviving.
Radiotherapy is a pretty straightforward process which involves irradiating cells so that they die; radiation has a greater effect on fast-growing cells than on slow-growing ones, so in this type of situation it is more inclined to kill cancerous cells than healthy ones. Unfortunately, exposing other types of cell to radiation means that there is an increased chance of other types of cancer developing at some point in the patient's life. This is also a risk with chemotherapy, as some of the chemicals used in that process can cause other cells to mutate. A patient who has been treated by either of these processes needs regular check-ups to try and catch any other developing cancers early while they can be treated as effectively as possible. Check-ups are also needed to ensure that the patient has not come out of remission. This goes on for the patient's entire life, though the danger decreases with time.
Radiotherapy tends to exhaust patients much more dramatically than chemotherapy does. It causes nausea and dizziness, and it can result in a sunburn effect on the skin. These sensations usually persist for a few days after it has been administered. Other minor side effects include problems with eating, as the skin of the mouth is often damaged, making rough-textured foods painful to chew and swallow, but this problem usually goes away after a few weeks.
After remission has been achieved, an alternative to continued chemotherapy is a bone marrow or peripheral stem cell transplant. Continued chemotherapy takes about three years and offers, the first time it is used, a fifty to sixty percent chance of a permanent cure. A bone marrow transplant from a sibling, if the patient has a sibling who is a close enough genetic match, can offer as much as a seventy five percent chance of a permanent cure. A transplant from an unrelated but matching donor can now be almost as effective. Also possible is a transplant from the patient's own tissue; this can be removed while the patient is in remission and treated to make sure it is absolutely free of cancerous cells. It can then be transplanted back into the body. This latter procedure, also known as an autograft, causes the least complications, because there is no possibility of rejection, but it offers a considerably lower chance of a cure.
There are two ways to transplant bone marrow - either directly, or via the peripheral stem cells which float around in the blood and are able to develop into new bone marrow. As yet, the medical profession is uncertain as to which of these options offers the best benefits for the patient, though Donald would have preferred stem cells; he received bone marrow. No surgery is necessary for the transplant to take place. The cells are infused into the patient's blood through a standard intravenous drip and find their own way to where they are supposed to be.
A transplant is a risky procedure, as it involves completely destroying the patient's immune system for a period of weeks. A patient in this condition is far more vulnerable to infection than a patient whose immune system has been suppressed in the ordinary process of chemotherapy. Infections develop faster and can easily be fatal. For this reason, patients undergoing transplants are kept in a still stricter form of isolation, and have minimal contact with the outside world. Even after they are released from hospital they remain in critical condition for several months, a condition similar to full-blown AIDS. Thirty percent of patients die during these early stages. The chances are slightly improved for younger, fitter patients, and slightly worse for patients who have already undergone a lot of chemotherapy.
Unless the transplanted tissue is the patient's own, some degree of tissue rejection generally occurs. Because the patient has just been given a new immune system, it is actually this new bone marrow which starts attacking the patient, rather than the other way round. This is known as graft versus host disease (GVHD). Where it is controllable, which is usually the case, this can be a good thing, as it means that the new white blood cells hunt down and destroy any remaining cancerous cells which they can find. To decrease the danger of rejection, a post-transplant patient must remain on immunosuppressant drugs for several months. Sometimes GVHD can remain problematic for a patient's entire life, even decades after treatment. It can cause thickening of the skin (which in turn can restrict the throat, making breathing difficult), thickening of lung tissue and various other complications.
New bone marrow in a post-transplant patient can take some time to start producing the other (non immune-system) blood cells which the body needs. For this reason, patients have to return to hospital at least twice a week for the first few months to determine whether they need to receive red blood cells and platelets which have been donated by other people.
Post-transplant, a patient can expect lifelong complications. The levels of radiotherapy involved destroy the spleen, which ordinarily stores up lymphocytes to release them when the body encounters an infection; without one, the patient must remain on a mild antibiotic such as amoxycillin for the rest of hir life. The absence of a functional spleen also makes red blood cells less efficient, creating anaemia-like symptoms, most notably reduced stamina. For several months after the new immune system has begun to function the patient will remain very vulnerable to infection, so diet and social interaction must be heavily restricted. Energy levels will usually be low. About six weeks after the radiotherapy, the patient will usually experience a week or two of feeling intensely sleepy all the time; this is normal, and will pass, though it's still important to keep doctors informed of every little change. Finger and toe nails will be fragile for a couple of months, and will sometimes break off completely. Eyes and skin will tend to be dry and require drops and moisturiser. Approximately a year after the transplant, childhood vaccinations must be re-administered; until this time, common childhood diseases can be extremely dangerous, and the patient is advised to try and avoid contact with anyone under the age of fourteen. Some post-transplant patients go on to return to work and live mostly normal, productive lives; others, inevitably, will require some degree of personal care throughout the time remaining to them. One notable survivor is the opera singer Jose Carrera, who came through a transplant sixteen years ago, when they were much cruder and more likely to be fatal than they are now.
Because of the quantity of drugs, blood products and other fluids which a patient undergoing any of these forms of treatment needs to receive, damage often occurs to the veins, which harden up and resist injections and canulae. For this reason, patients are sometimes surgically implanted with Hickman lines, tubes which attach to the jugular via the chest and progress down into the vena cava, with two protruding orifices (one for fluid going into the bloodstream, one for blood coming out to be tested). Hickman lines are essential for patients undergoing transplants. They used to represent quite a high risk of infection, so Donald and I opted to avoid them the first time he was ill; the second time, we had a lot less choice, on account of the state of his veins, but the surgical techniques involved have improved a lot over recent years, and we were quite comfortable with the result. It restricted his movement a bit, but not much, and it was safe for him to leave the hospital with, though naturally it made it unsafe for him to hold the baby whom we were living with at the time. It was inserted under a local anaesthetic and was similarly removed once the main part of his course of treatment was over.
Treatment with chemotheramotherapy or a transplant is not always successful. If the cancer reappears, it must be beaten back into remission. After this, a second course of treatment is sometimes possible, though a third course is extremely unlikely to actually cure the disease, and can only buy the patient a little more time, averaging one to five years. Because of the level of dangerous radiotherapy necessary to make it possible, a transplant can only be carried out once, either the first or second time that the cancer occurs.
If you want to improve the survival chances of Donald or of any other patient with acute lymphoblastic leukaemia, there are several things you can do.
Patients with this type of illness require a lot of donated blood whilst undergoing treatment. Donating blood to increase the hospitals' supply could save their lives. In the UK, of course, the blood transfusion service still rejects gay and bisexual male donors and women who have slept with bisexual men, on account of the panic which took place after the government's first AIDS warning campaign when many members of the public were under the impression that all gay people had HIV. Naturally, if you have or think you might be likely to have a serious disease of that type, you shouldn't donate blood. However, if you are not in that position but might be excluded otherwise because of your sexuality or sexual history, I shall break with my usual policy at this point and urge you to lie. Donated blood is desperately needed by the medical profession to help many people who could die without it, leukaemia patients among them.
Many people do not realise that it is also possible to donate platelets, which help blood to clot. Chemotherapy and radiotherapy kills platelets, so patients are constantly in need of transfused ones. Giving platelets is easier for the donor (it won't cause tiredness or dizziness), and can be done as often as every two weeks.
The other valuable thing which you can do to help people with acute lymphoblastic leukaemia is to join the bone marrow donors' register. This can be arranged by your own doctor, and initially requires just one simple blood test. You don't need to die to donate your bone marrow. It can be extracted while you are alive and healthy by means of a simple operation; a local anaesthetic is normally used (unless the donor would vastly prefer a general anaesthetic) and marrow is syringed out of the hip. It will feel sore and bruised for a week or two, but nothing worse than that; new bone marrow will grow there to replace it very quickly.
Donating stem cells (which can be done via the same register) is even easier. The donor simply has to sit about for four hours while blood is removed from hir via a tube. This blood is centrifuged to separate and remove the stem cells, then piped back into the donor. However, since stem cell donation has only been taking place for about five years, it is not yet known whether there may be long-term effects on the donor.
The bone marrow donors' register is international; your agreement to donate could save a life anywhere in the world. The more people who join the register, the better are the chances which every acute lymphoblastic leukaemia patient has of staying alive. The chances of a successful genetic match for bone marrow donation are influenced by genetic heritage to some degree. White caucasians are vastly in the majority on the donors' list at present, and doctors are particularly keen to urge potential donors from other races to come forward.
Many people with diseases of this sort do not have anybody to take care of them or to visit them in hospital, where they may be isolated for long periods of time. This can have seriously damaging psychological effects (which, in turn, reduce the patient's overall chances of survival). Many hospitals have community initiatives whereby they ask members of the public to come forward and volunteer to visit and befriend these isolated patients. If you would be interested in helping in this way, your GP, local hospital or local branches of cancer charities can give you the appropriate information.
Direct contributions towards research and towards the provision of emergency financial support for cancer sufferers can be made by donating money to any major cancer charity. Some cancer charities support specific projects, such as providing holidays for children recovering from chemotherapy. If you look around, there's quite a bit of choice as to what specifically you want to donate to.
If you can't help directly in any of these ways, remember that you can always spread the word, and perhaps find other people who are able to do so. It really is vital to raise awareness of these issues.
Donald has already undergone experimental treatment, and would be prepared to extend this in the right circumstances. If you are a professional involved in the treatment of acute lymphoblastic leukaemia I would be interested to hear from you - you can mail me at jennie@innocent.com - especially if you have any news for us about current research developments or any new suggestions to offer. I endeavour to keep this page up to date and would be grateful if you could inform me of any inaccuracies.
Last updated 13th January, 2005