Treatment - Some commonly used Medicines
Research carried out at The University of Birmingham Medical School into vasculitis has shown that damage to organs can occur early on in the course of disease. Therefore, in the beginning, aggressive treatment is used in the short term that is directed towards trying to switch off the mechanisms that are causing the inflammation. Then a maintenance therapy will be started, which often overlaps for a while with the initial treatment given. This has to be continued for a long period of time, to allow healing to take place and prevent flares recurring.
Drug regimes are prescribed according to the severity of presenting symptoms. For example, if your main problems are localised to the skin, you may be treated with mild therapy or low dose steroids. But if you present with kidney problems, a high dose of cyclophosphamide will usually be started.
Early, aggressive treatment has improved the course of this disease. Unfortunately, it needs to be given when the patient is feeling at their worst. So, not only do they feel poorly from the effects of the disease, but also they have to cope with a treatment that may make them feel (temporarily) more ill than the disease itself. This is like many forms of cancer therapy.
The following is a list of commonly used drugs that may be given either in hospital or as an outpatient:
Cyclophosphamide
This is a powerful but very effective drug. It is the drug of choice for the aggressive treatment that is needed to bring about a remission. How it works is not entirely clear, but it is known that it damages the cell's DNA and interferes with the cell's ability to multiply in some way.
It may be given by a drip or in the form of tablets depending on what the individual needs. Side effects are normally related to the dose that is given, i.e. the higher the dose, the more likely there will be some side effects. However, as everyone reacts differently, it is wise to take things on an individual basis.
Drugs are routinely given to counteract the known common side effects of cyclophosphamide. For example, bladder-protecting drugs are given to counteract the irritating nature of this drug on the bladder. It is therefore also important to drink at least 3 litres of fluid per day and to pass urine often, in order to keep the bladder healthy. However, your doctor may advise you to drink less than this if the kidneys are not working very well.
Sickness is another commonly reported side effect; so anti-sickness tablets are routinely given to prevent this from happening. Hair loss tends to occur when higher doses of the drug are given. This can be very distressing at a time when your self-esteem is most probably low anyway. However, it usually grows back after the high dose treatment is stopped. Also, the NHS has a good wig service and their products actually look like real hair!
Whilst taking cyclophosphamide it is advisable to use effective contraception. This is because it may cause damage to the unborn child. Also, some patients may become less fertile (or infertile). The risks of infertility increase with age, number of treatments and pre-existing disease. However, all patients should have the opportunity to discuss issues about fertility and contraception before treatment is started.
For those who may be at risk from infertility and would like further information regarding what services are available, the Assisted Conception Unit has produced a helpful booklet. The booklet also contains many useful addresses of related patient support organisations. (NB. It is not possible at the moment to store unfertilised eggs. Eggs have to be artificially fertilised before they can be frozen.) Males at risk from infertility should consider sperm storage.
For a copy of the booklet
"An introduction to the Assisted Conception Unit" please contact:
The Assisted Conception Unit
Birmingham Women's Hospital
Edgbaston
Birmingham B15 2TG
Tel: 0121 627 27 00
Fax: 0121 627 27 01
email: info@acu.co.uk
URL: www.medweb.bham.ac.uk/repromed
Prednisolone
This is a corticosteroid (steroid), which is also used to treat a wide range of other conditions. (It is NOT the same as anabolic steroids.) It is a very useful and effective treatment for vasculitis because it helps to reduce inflammation. It also can depress the immune system. Often this drug is used at high dosage for a short time (days or weeks) to treat active disease, but patients with vasculitis may also need it in lower doses to keep the disease under control as part of a long-term plan.
The dose given does vary tremendously, but don't be alarmed if you are put on what may seem to be a very high dose during a flare. It may be necessary to increase the dose to get the symptoms under control.
Steroids are not an ideal long-term therapy, but if your doctor thinks that you need them then you should be told about the possible long-term side effects. No drugs are without risks, but you can do a lot to minimise these by talking to your doctor/nurse. For example, with steroids the golden rule is to keep as active as possible. Weight bearing exercises will help to minimise bone loss associated with these types of drugs. Drugs to prevent osteoporosis, indigestion or certain infections are often added.
Steroids like prednisolone should not be stopped without consulting your doctor. Stopping prednisolone quickly can be dangerous as the body gets used to it. Normally this is avoided by reducing the dose gradually, under the doctor's supervision, when it is appropriate and safe to reduce the dose.
When you are started on steroids you will be given a steroid treatment card. Always carry this with you as it gives instructions regarding your medication and who to contact in case of an emergency.
Azathioprine
This is an immunosuppressant (i.e. a drug that suppresses the immune system thereby reducing inflammation.) It can be used to enable the doctors to reduce the amount of steroids taken or may even be used in place of steroids. Azathioprine is often used longer term.
Whilst on this drug, your doctor will usually want you to have regular blood tests to monitor how much your immune system is being suppressed and to make sure that it isn't being suppressed too much.
Methotrexate
This drug is an antimetabolite i.e. it interferes with how the cell processes nutrients and other substances. It also acts as an anti-inflammatory drug and because it suppresses the immune system your doctor will want to monitor this with regular blood tests.
PLEASE NOTE
All of these drugs are designed to modify the immune system so that inflammation is reduced. Because of this, your ability to fight infections may be significantly lowered. You can help yourself by avoiding people who are known to have infections, and by seeking help sooner rather than later if you feel that you have developed an infection.
All drugs that are used in the treatment of vasculitis require some degree of monitoring. This regular monitoring is vital to minimise possible side effects and to record your progress. Regular blood tests are an important way of plotting progress and also pre-empting any changes in blood counts that may be a result of the drug therapy.
It is the responsibility of the doctor who prescribes the treatment that you are on to arrange the appropriate blood tests. It is vital that the person who takes the responsibility for monitoring the blood and your general health knows what to look out for.
You have a part to play in keeping yourself well and monitoring your progress. Try to keep as fit as possible, watch your weight and take notice of the changes in your body. Any unexplained rashes, ulcers, hair loss etc. must be reported to your doctor or specialist nurse.
Many Departments offer a Shared Care Card. This is a booklet that gives a list of contact numbers, a brief description of the treatment planned and the monitoring requirements. It is held by the patient, who takes it to appointments with the GP and the hospital team. This is so that everyone knows what has been going on. Of course, this only works if the information is up to date. It is often left to you (the patient) to remind the team to update it.
Your disease may require you to take a combination of drugs, and this can seem confusing at first. So it is important to ask your doctor or nurse to explain what your drugs are for. This may also then help you to feel more in control.
Occasionally you may need to be given certain drugs as a "one off" therapy to quickly control the inflammation. For example, "Plasma exchange" is used-prescribed in a course of seven-fourteen Treatments. In basic terms, it removes antibodies and proteins from the blood. It has been useful in providing an immediate relief in situations where patients are not responding fast enough to cyclophosphamide and steroids. Low blood pressure has been reported as a side effect, but as the treatment is carried out in hospital, the effects will be closely monitored.
Newer therapies are being developed all the time. Also, older drugs that have been previously used for other conditions, such as thalidomide, have had a good deal of success in reducing symptoms for patients with Behcet's disease.
General note about side effects
All the drugs mentioned may give you some side effects. However, the number and severity of these side effects will be influenced by the amount of the drug taken and, different individual's reactions to a particular drug. It is important to inform your doctor of any side effects experienced, so that dosages can be adjusted and medicines prescribed to counteract the side effects.
It is also important to remember that even though the side effects may be unpleasant, it is even more important to get the inflammation under control so that eventually tablets can be reduced and in many cases, stopped altogether. It is important to think of the long-term benefits and reason for taking the tablets i.e. to reduce the inflammation and prevent damage.
It is an acknowledged problem that at the moment the only medicines available to treat vasculitis can themselves cause problems. However, much work and research is being carried out to try to discover treatments that are less damaging. Without treatment of any kind, vasculitis can be life threatening in its severest form, and so strong drugs are sometimes needed to get it under control.
If you do feel that you are getting side effects, then that is the time to consult your doctor. It is quite common to get a few effects when you first starting taking any drugs, for example, feeling sick. But persisting with the drug for a while to see if the sickness goes away may be the best option rather than trying another drug.