25 September 2011; fix some vitamin and mineral dosages.
There is a mailing list about CSR. We can use this mailing list to do a number of positive things such as sharing information about treatments and clinicians who are expert in the field, promoting awareness of the condition, and perhaps lobbying for more research.
As my CSR has now been in remission for a long time, I no longer actively research it nor do I monitor the mailing list. If you have asked a question of the mailing list and not had a satisfactory answer, you can email me direct, with a copy of the message you sent to the mailing list.
To subscribe to the list, send an email to firstname.lastname@example.org. The content and title of the message does not matter. Or you can point your browser at http://health.groups.yahoo.com/group/csreye/ and follow the instructions there. One you have subscribed, you will receive all the messages sent to the list, until you unsubscribe. To send a message to everyone on the list, send an email to email@example.com.
I am not a doctor. This page exists only to share my readings and experiences of CSR. Check anything on this page before acting on it. Consult a qualified practitioner etc.
CSR is a serious condition which can lead to functional blindness, though it usually does not. It should be taken seriously.
CSR is a condition which causes temporary or permanent impairment of vision. The symptoms are loss of sensitivity in dim light, usually in an oval shaped gray or brown area, and blurring or distortion of the visual image. It is a result of the detachment of most of the layers of the retina (the back of the eye) from its supporting tissue as a result of the buildup of fluid. The buildup of fluid appears to be due to one or more small breaks in the retinal pigment epithelium.
Often you will see the term "idiopathic CSR" used, which simply means that the cause is not known.
It affects primarily males between 20-45 and is associated with stress. Some females are affected, usually in the 40-50 age range. Reference 7 Reference 9 Reference 10. For example airline pilots suffer from it at a high rate Reference 15, and some people find that attacks go away if they take a holiday. There have been some attempts to classify different forms of the disease but they are tentative and not linked to different causes or treatments.
There have been reports of a link with Helicobacter pylori infections. This bacterium is linked to stomach ulcers. If true, this may explain the link with cortisol, which in excessive amounts weakens the immune system. Reference 19 Reference 20.
Usually the fluid build up disappears after a few weeks to a few months with little long term damage. During this time the condition often fluctuates wildly on a daily or hourly basis. It particularly gets worse when under stress, tired or ill.
However recurrences are quite common and damage can accumulate over time. Significant, disabling damage can accumulate over time. If the detachment persists for too long the retina is starved of nutrients and can be damaged permanently, with a permanent gray area or a blind spot. The likelihood of recurrences is higher if the initial attack is more severe Reference 15.
There are wide variations in the estimates of recurrence rates. Opinions also vary on the risk of the condition occurring in the other eye. Often patients are told that recurrences are rare, and that there is no need for concern. While this may be true in many cases, there is reason to doubt that this complacent attitude is correct.
A long term study of patients with CSR found that 50% of patients get the severe and extensive form of the disease after 12 years of evolution Reference 1. It may be that the complacent attitude of many clinicians is a result of the fact that they provide no effective treatment, so patients drift away and there is no long term visibility to the evolution of the condition.
You can see what CSR did to my left eye from this picture. The bright areas in the central dark area are scarred areas from the CSR. The central dark area is the area for fine central vision. You can see the blood vessels starting from the left, feeding the whole area.
A detailed explanation of what leads to CSR is not available. However some associations are known:
Some people have been successfully treated with COX-2 inhibiters, in particular Bromfenac (Xibrom). Xibrom penetrates the eye well and this may contribute to its effectiveness. See Reference 22 and Reference 23
COX-2 (cyclooxygenase-2 )inhibiters reduce the effectiveness of some inflammatory hormones. If inflammation is a contributor to CSR then inhibition of COX-2 enzymes may help fix CSR. There are two variants of COX, COX-1 and COX-2. Some anti-inflammatories inhibit COX-1. For example, fish oil (Omega-3 oil) inhibits COX-1. See Reference 24.
It can be difficult to get access to Bromfenac. Some people may then consider taking other COX inhibutors, such as Ibuprofen. However Ibuprofen may not help: 1. It does not reach a high enough concentration in the choroid and the RPE. 2. It is not a specific and powerful COX-2 inhibitor. In contrast, Bromfenac (XIBROM) is a very powerful and specific COX-2 inhibitor.
There are other COX-2 inhibitors. One recent example is Vioxx, which has been withdrawn from the market due to side-effects. This case reinforces the notion that medications should only be taken as needed and for as long as needed, no more and no longer. See Reference 25.
There is no standard effective clinical treatment for the condition.
Laser treatment has been used but research results suggest that the treatment does not improve the long term outcomes. The laser therapy effectively 'burns' the leak shut. However the leak may reopen elsewhere. The burned area is permanently damaged from the burn.
Laser can be used to overcome an emergency. For example if an attack is preventing studying for examinations. This may also be useful if an attack goes on for a long time - more than a couple of months, depending on how severe the attack is. A long standing attack can cause permanent damage to the retina and in this case laser treatment may be a good idea as a way to prevent permanent damage.
Recently more refined laser treatments have been used as certain frequencies of light cause less damage than standard frequencies Reference 14. If you are having laser treatment, ask your doctor if they are using these later, less damaging techniques.
A paper on a new laser treatment by Dr Larry Yannuzzi and others was published in Retina Reference 21. According to Dr Yannuzi the new form of laser treatment is much more effective.
Given recent reports of a link with bacterial infection (Helicobacter pylori), it may be worth being assessed for this infection and having treatment as appropriate.
See csrtreat for a detailed discussion of other possible treatments.
See Action Plan for a summary of what you can do.
CSR is associated with stress. Individuals who report being under stress and people who are in objectively stressful occupations such as airline piloting are susceptible to CSR. Stress includes pain, infection, excessive heat and cold, low blood sugar, excessive exercise, hunger and crash diets, jet lag, lack of sleep, psychological stress and also very intensive exercise (normal exercise is OK).
Stress causes the adrenal cortex to secrete cortisol and this appears to be the link between stress and CSR. There has been one study which suggested that the link is instead due to the increased adrenaline that comes with stress but these studies appear to be flawed due to lack of control for blood pressure.
High endorphin levels (a result of pain or excess exercise) or opiate levels (from use of heroin or morphine as drugs or as pain killers) are also associated with CSR. Pain is a form of stress of course.
CSR sufferers have usage rates for tranquillizers much higher than the general population. They are more susceptible to high blood pressure, and are more likely to be users for cortisone steroid medications Reference 2. Cortisone steroid medications mimic the effects of cortisone on the body.
High cortisol levels are often but not always a result of stress. See below for the linkage between cortisol and CSR.
Cortisol is a hormone secreted by the adrenal cortex which assists the body to deal with various stresses. It reduces inflammation and immune system function and triggers the breakdown of protein into sugars.
A certain amount of cortisol is necessary for life. Without cortisol even a small amount of stress will kill you. Addison's disease is a disease which causes low cortisol levels, and which is treated by cortisol replacement therapy.
CSR is associated with high cortisol levels. That is, people with high cortisol levels are more likely to suffer from CSR, and people with CSR generally have high levels of cortisol.
CSR sufferers have high levels of cortisol made by their own adrenal gland (50-80% higher than the average, and outside the normal range) Reference 3.
CSR is also associated with treatment by corticosteriods ("cortisone") for other conditions such as allergy and inflammation. These drugs go under names like Hydrocortisone, Cortisone and Prednisolone/Prednisone. There have been several cases where CSR has recurred during each of several courses of treatment with cortisone drugs and gone away each time the treatment was stopped. Usually, doctors will claim that nasal sprays and skin creams are not absorbed into the body and will not therefore cause adverse side effects. However in a number of cases this information has turned out to be unreliable and CSR attacks have ceased when the treatment was terminated. We have also had cases of people with CSR being treated by uninformed doctors with Cortisone! Recent studies suggest that Cortisone medication even in small doses can cause very serious side effects such as osteoporosis.
CSR is also associated with pregnancy (which generates very high cortisol levels in some cases). The high levels of cortisol in pregnancy and the body's need to protect against this may explain why women are less prone to getting CSR than men.
The association of objective and subjective stress with CSR also points to cortisol because stress raises cortisol levels. In addition, a high level of CRF, the hypothalmic hormone which drives cortisol levels, causes a subjective experience of stressfulness.
The incidence of CSR in people suffering from Cushing's syndrome/disease is about 5%, a very high level Reference 8. Cushing's syndrome/disease consists of very high cortisol levels usually caused by a tumour in the pituitary gland or in the adrenal glands. Again this confirms the linkage between cortisol and CSR, but also suggests that high cortisol is not enough to cause the disease on its own, because only 5% of the Cushing's patients get CSR. Presumably some other weakness plays a part. Most Cushing's patients are women, so whatever protects women from CSR may be factor here too.
Cortisol is a powerful chemical and has numerous adverse effects in excess. It is therefore recommended in the drug manuals that cortisone medication be taken in as small a dose as possible for as short a time as possible. A partial list of the adverse effects of cortisol follows:
It should be noted however that Cortisone mediation can be a life saver, for example with asthma, and in controlling some forms of short term but dangerous inflammation. A judgement needs to be made in each case of the dangers vs the benefits.
The body's control of cortisol levels is complex. The PVN area within the hypothalamus secretes a substance called CRF (corticotropin releasing factor). This is picked up by the pituitary gland which then secretes ACTH (adrenocorticotropic hormone). In turn this causes the adrenal cortex to secrete cortisol.
The hypothalamus acts as the body's stress detector and drives cortisol production in this way as a response to stress.
Negative feedback mechanisms exist between the body's cortisol levels and the pituitary and hypothalamus to keep the cortisol levels within reasonable bounds in normal circumstances. The negative feedback is more effective in controlling high cortisol that results from psychological stress than high cortisol that results from physical causes such as blood loss or illness.
One unfortunate fact about the body is its tendency to resist any change. For example if you go in a diet the body increases subjective hunger and decreases metabolic rate, thus 'helping' you not to lose weight. The same thing applies with many of the techniques to reduce cortisol levels. They may work for a while but then the body adapts and levels return towards the original values. So it may be necessary to keep revising your strategies.
I originally thought that this happened to me after I gave up coffee, but later I tracked the problem down to excessive working hours (12-14 hours * 7 days a week) and the fact I had taken up drinking decaffeinated coffee again.
According to http://qualitycounts.com/fptheanine.htm, "Chief among the supplements with documented cortisol-controlling effects are Phosphatidylserine, Beta-sitosterol, Magnolia bark, Theanine, Epimedium, Ashwagandha and Passionflower".
Sites such as http://www.cortislim.com/faq.htm have products that are claimed to reduce cortisol levels.
Drugs like caffeine and nicotine tell the hypothalamus that you are under stress, leading to increased cortisol levels, as well as increased adrenaline levels Reference 11. The increase in cortisol levels from consuming 4-5 cups of coffee per day may be of the order of 50-60% and the increase is highest in people who are already prone to high levels of cortisol. There are anecdotal reports that other stimulants are also associated with CSR attacks eg ephedrine (found in decongestants and herbal weight loss preparations).o
Stress increases cortisol levels. This includes all the forms of stress described above. However the body is better able to control cortisol levels that result from psychological stress than those that result from physical stress.
Cortisone steroid drugs mimic the effect of high cortisol levels.
There is a lot of evidence that a stressful childhood leads to a permanently increase susceptibility to stress and increased CRF levels from the hypothalamus leading to higher cortisol levels. The stresses in childhood can range from separation from the mother through to physical and sexual abuse, hunger and disease. This can sensitize the hypothalamus and make it "trigger happy".
While many CSR sufferers live objectively stressful lives as pilots etc, others suffer from high levels of subjective stress although their lives do not appear to be inherently stressful. For these people, it is not entirely clear why they should have high levels of subjective stress.
This may be due to an inherent metabolic tendency to over-produce CRF, ACTH, adrenaline and cortisol which produce subjective feelings of stress via brain receptors for those hormones.
Another explanation is that a stressful childhood can lead to an over-reactive hypothalamus, leading to the production of adrenaline and cortisol in relatively normal circumstances. There is considerable animal research to suggest effect this does occur. If you were ill when very young, or if you were physically psychologically abused, this may be the problem.
It is also possible that the person may have poor strategies for dealing with stresses. Studies have suggested that many CSR sufferers have 'Type A' personality which describes a tendency to be unable to relax. I would caution against assuming that the cause is psychological though. In the past many conditions thought to be of psychological causes have turned out to have significant or dominant physical causes, for example severe depression and schizophrenia. In a sense then, psychological explanations are the explanation of last resort for the clinician who has no answers, and often amounts to a strategy of 'blame the patient'. The so-called 'schizophrenogenic mother' from the 1960s, who was supposed to cause her children to become schizophrenic by her poor parenting, is a notorious example of this syndrome.
Having said that, there are a lot of effective stress management techniques available. These can be effective if psychological stress is the problem, but probably not if there is a physical problem such as pain or illness. See below.
If you have high cortisol levels but do not have the tumour characteristic of Cushing's disease, your condition is likely to be labelled as "pseudo-Cushing's syndrome". The term is unfortunate because the high cortisol and the damage that results is just as real as in the 'real' thing. Most people develop some tolerance to cortisol over time, so the symptoms are not so extreme - difficulty controlling weight around the middle, a tendency to fluctuating blood sugar levels, high cholesterol and blood pressure, lack of energy, etc. Pseudo-Cushing's syndrome is often assumed to be due to excessive alcohol consumption or psychological depression or stress. However a recent study has established that a significant proportion of the population has a genetic predisposition to high cortisol levels, so it may not all be in your mind after all.
A detailed discussion of pseudo Cushing's syndrome is available in csrpseudo.html
The condition is fairly unusual so can be difficult to find someone who is fully qualified to treat it, or even to diagnose it. It took four optometrists and three opthalmologists 12 years to correctly diagnose my case, for example. It is quite common for serious and obvious illnesses to go undiagnosed for years. A recent study found that the average time to diagnose Cushing's Syndrome, a very serious illness sometimes associated with CSR, was 3 1/2 years.
There are several conditions that can be confused with CSR, such as Age Related Macular Degeneration and diabetic retinopathy, and these are more common. It is important to have the diagnosis confirmed and tests are available to verify the leakage of fluid that is characteristic of CSR.
A retinologist, not just an ordinary opthalmologist, can diagnose the condition. Because the condition initially causes a quite subtle loss of vision, an opthalmologist may even tell you that there is nothing wrong. This in fact happened to me, and to others.
You should then be able to get your cortisol tested without too much trouble to confirm the link with high cortisol in your case, unless you are using cortisone medication or are already aware of a high cortisol level. I strongly recommend taking the test, and doing the right test - a 24 hour urine cortisol test. The blood test is basically useless for diagnosis of high cortisol levels because the levels fluctuate too much from hour to hour. It is like measuring how much time you spend watching TV by taking one photograph of the living room.
In theory the best person for dealing with the high cortisol is an endocrinologist. However the levels of cortisol in CSR are generally not caused by a tumour and so do not qualify as a case of Cushing's syndrome which is quite a rare condition - although it is important to rule this out! Cushing's syndrome is the classic high cortisol disease. So an endocrinologist may diagnose pesudo Cushings and tell you not to drink so much.
As anti-cortisol drugs can have adverse side-effects, you are likely to find a high degree of reluctance to provide any treatment.
This is very frustrating. Doctors prescribe cortisone treatments quite freely and often do not advise patients of the severe side effects that can follow. On the other hand there is a high degree of reluctance to prescribe anti-cortisol treatments even when cortisol levels are high, in the name of 'accepted clinical practice'.
The other problem with endocrinologists is that in most cases of CSR the root cause of the high cortisol seems to be the hypothalamus which is part of the brain, and so is not in the jurisdiction of the endocrinologist. The brain is also far more complicated than the endocrine system.
Potentially a neurologist may be able to help if, as appears often to be the case, the condition is driven by an overactive hypothalamus. However you will probably find an even greater reluctance to do anything about the hypothalamus than is the case for the adrenal gland and its cortisol. The hypothalamus is very complex and plays a critical role in many vital body functions.
On the positive side, practitioners will most likely find your condition interesting, at least until they realize they cannot help you much.
Disclaimer: This is what I am doing. You are an adult and have to verify this information, make your own decisions and monitor the results, preferably under the supervision of a competent medical advisor. If you are or may be pregnant, this all applies doubly so. Having said that, all but one these actions are based on scientific evidence or personal experience of benefit, usually both.
I want to emphasise that I think the whole is greater than the sum of the parts with this plan. It is not much good trying one of another thing for a while. It is important to throw everything at the problem.
If an attack goes on for too long (ie more than 1-2 months) you may want to consider the newer laser treatments and/or the COX-2 inhibitors discussed above.
The references listed below are in standard 'academic' format. If you want to follow them up you can do two things. First, get on to Biomednet and search for them. You can then read the abstracts (summaries). Second, you can go to your local college library and see if they have the journal concerned, or alternatively you can buy the article using the links from biomednet.
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