What is Birdshot Uveitis?
Birdshot Uveitis or Birdshot Chorioretinopathy
Birdshot Uveitis or Birdshot Chorioretinopathy is an extremely rare eye condition, that manifests itself gradually over time. Usually first appearing in adults around the age of 45.
It is an inflammation of the eye, and is characterized in some cases by the appearance of a cluster of spots on the back wall of the eye. There can be a wide range of other symptoms including:Cloudy Vision, Shadowing of Vision, Loss of contrast and loss of color recognition to name just a few.
In the so called 'classic' case. It looks (under the microscope) like the splatter pattern of the pellets fired from a shot gun cartridge. (hence the name)
The symptoms can also include swelling, light sensitivity and the appearance of detached floating objects in the eye. (floaters)
There is very little information about this rare condition or the side effects of the treatment. What little information there is you will find linked below.
This is a personal chronological account, of the treatment and is not intended as medical advice. These side effects may not occur in every case but do seem to have some common features. Regardless of the treatment method.
It is better to know what to expect, rather than find yourself totally confused in the middle of this treatment process.
The following is Linda's experience.
Linda developed a sleep disorder, as a side effect of the treatment for Birdshot. She gained weight (16 kg or 2.5 stone) in just a few weeks. She developed severe Oedema.
Oedema is the medical term for fluid retention in the body. The build-up of fluid causes affected tissue to become swollen. The swelling can occur in one particular part of the body – for example, as the result of an injury – or it can be more general.
Source: NHS Choices website
A year later we managed to get her into a Papworth Hospital Sleep Clinic for tests and it was confirmed that she was stopping breathing throughout the night.
They issued her with a CPAP machine to help her breath at night. This was a great help as it was helping her to find some much needed rest. We had not been warned and had no idea that the treatment would cause obstructive sleep apnea.
We were trying to understand what this treatment was doing to her. None of the doctors warned us of these side effects.
To be honest, they really didn't know what they were dealing with, and the treatment was a bit of a gamble at best.
Every time we visited a doctor, they had to get the medical books out to look up this disease. And I mean every doctor. Even the consultants.
Birdshot Uveitis Photo
Birdshot Uveitis is an Auto Immune Disease
Birdshot Uveitis, Central Serous Chorioretinopathy or Birdshot Chorioretinopathy. ( Chorio-retin-opathy ) You may find several other variants of these terms. As they are still trying to classify this disease.
It is a serious condition, where the patient could lose their eyesight at any moment, with no warning; unless they take some very strong medicines.
There are several alternative treatments that we were unaware of. Linda's Initial treatment included the steroid Prednisolone and Methotrexate, a powerful anti inflammatory. These are someties used to treat some forms of cancer. (chemotherapy)
Auto Immune Disease
It is not known what causes this disease but some think that the disease could be caused by a virus or a bacteria, which covers just about everything and nothing.
The effects are seen, when the bodies own immune system, starts attacking its own body.
The way the doctors tackled this situation, was to suppress Linda's immune defenses and create a weakened immune system by using these powerful medicines.
This artificially imposed immune deficiency, allows other cells in the body to repair the damaged areas of the body. However if the treatment is stopped, the immune system will attack again, as it sees the body it is supposed to be protecting, as a foreign invader.
The cause of Birdshot Chorioretinopathy is still unknown but has been associated with a marker on the gene (HLA)-A29. People with this gene seem to have a higher risk of developing the disease. Although the evidence is inconclusive at this time.
Birdshot Uveitis Society 2nd Conference 2012
Sleep Apnea Symptoms Develop
Trying to explain this disease to family and friends has been difficult. We really didn't know what Linda was dealing with. Family and friends didn't know what to say or how to respond. Nor did many of the doctors.
Secondly having a compromised or weakened immune system, meant that we really could not expose Linda to anyone, with even a 'cold or flu', as it would knock her off her feet for days or weeks on end.
It is a little disconcerting when a specialist eye doctor reaches for a medical book. How do 'we' explain something that even the medical staff do not understand.
Sleep Study at Papworth Clinic
The sleep apnea developed as Linda's weight increased. She would stop breathing while asleep and her body would convulse and she would wake with a jolt.
She became mentally and physically exhausted and eventually we managed to persuade the doctors that something was seriously wrong.
Linda attended a sleep study overnight at Papworth sleep clinic and they recorded that she stopped breathing 19+ times an hour.
Linda was issued a CPAP machine and began to get some much needed quality sleep.
I wrote this diary for a few reasons.
- To keep track of what was going on
- For Linda to be able to use it to recall the sequence of events as they happened. Her memory was in tatters.
- As a source of information for others.
The more people that know about this rare condition the better. It might help someone.
Discovering the Problem
Linda first had treatment for the Birdshot condition in 2005. We assumed that it had been cured. The doctors never told her to have regular examinations.
Several years after this first and what we thought successful treatment. Linda's eyesight again was getting progressively worse.
We assumed it was just the aging process, and so did the several opticians that we visited. Most opticians and eye doctors will not recognise this disease. It can take years to be diagnosed.
We didn't make the connection with the earlier episode. It was by a pure fluke that it was discovered this time.
A friend of the family worked at a laser eye surgery clinic and offered us a consultation.
We thought that Linda could have the eye surgery and that would sort out the problem.
We thought that she would no longer need to wear reading glasses. We put off going for weeks as it didn't seem that important.
Eventually we decided to check it out. Thinking it was just a routine thing.
The ophthalmic technician on examining Linda's eyes, said.
'There is something really wrong here, you had better go straight to the hospital'
Linda was seen very quickly at the hospital. This is when we were told that her eyesight could have gone at any moment. Linda had been very lucky.
The doctors do not know what causes it, or where it comes from.
Basically her own immune system is attacking the main arteries at the back of her eyes.
They cannot cure Birdshot but it can be checked and can respond to treatment if caught in time.
Depending on when it is diagnosed. The initial treatment is a course of high dosage steroids Prednisolone and the powerful anti-inflammatory medicine Methotrexate.
These are usually used in the treatment of rheumatoid arthritis, and some forms of cancer.
Linda had to undergo a battery of tests, to make sure she was cancer free. It took two months to complete these tests and her eyesight could have gone at any moment.
These medicines could accelerate any mutant or cancerous cells. It was hoped that the treatment would arrest the deterioration in her eyesight.
Birdshot Treatment Side Effects
- Rapid Weight gain
- Hair loss from head and increased growth elsewhere
- Edema (Oedema UK) abnormal accumulation of fluid below the skin. Formerly known as Dropsy
- Exhaustion Physical & Mental
- Inability to walk for more than a few paces before needing to rest
- Memory loss short and long term
- Inability to concentrate
- Permanently tired
- Sleeping all day
- Sleep deprivation
- Poor quality of sleep
- Sleep Apnea
- Loss of appetite
- Acute Bruising
- Brittle finger nails and teeth (calcium deficiency)
- Fungal growths on skin
- Double vision
- Blurred vision
The above list does not cover all of the side effects that Linda has experienced, and continues to have.
It has been going on for so long now, that we have assimilated many of her side effects into our lives and have adapted to our new reality.
Whether it is from some kind of virus or bacteria is actually irrelevant. We are dealing with the effects and that is about all we can do.
Linda doesn't smoke or drink and has always led a healthy life with a good diet.
The only known treatment, we have were advised. Is to suppress her immune system with the steroid, and to bring down the inflammation in the eyes with the Methotrexate.
We have since discovered that there are alternative treatments.
Not Breathing is Not Normal
Linda became very ill and her sleeping became very erratic and she would often stop breathing.
She often complained. 'I sleep too much, and yet I am always tired'.
We all snore from time to time and it can be annoying but this was way beyond snoring. She just wasn't breathing.
I would nudge her gently when she stopped breathing and thought to myself, this is getting crazy.
When she would stop breathing, after a while, a matter of seconds, her body would convulse and she would breath again.
It was very scary to say the least. Trying to get it through to her and the doctors was almost impossible. They just didn't get it.
More Side Effects
The therapy scrambled her ability to think clearly and she was almost completely non functional.
When the Cure Makes you Sick
The therapy affected her ability to think and remember things. Linda is the kind of woman who remembered everyone's birthdays. Now she couldn't remember even simple things.
Linda has a high IQ and loved to do puzzles. She was noted for her memory in our family.
To be losing this part of her was devastating to her personality.
She had dreadful invasive thoughts at this time and still has some today but they are lessening as she knows it is the therapy and has adapted as best she can to the weekly injections of Methotrexate.
All I could do was to reassure her that it would all come right and she must just try to rest and relax. I can't honestly that I believed it would improve.
As the treatment wore on, she got weaker and weaker. Her mind was almost gone completely. Trying to explain this to people was almost impossible. They just didn't get it.
To see her struggling with this failing mental ability, made me determined to find out what we could about sleep disorders let alone the Birdshot issue. That took a back seat.
Linda couldn't be left alone for safety reasons. Linda would leave things cooking until they burned amongst many other things. She simply forgot what she had done five minutes earlier. Short term memory was gone.
Linda in fact started to set timers so that she would remember that something was happening and she used this method to remind herself.
It wasn't very effective as she would forget what the timer was reminding her of. Which made her feel even worse..
A Problem Shared
Not really knowing what to do or who to ask for advice. I researched and wrote an article on snoring and sleep debt and showed it to her.
As a teacher I know the written word sometimes has more impact.
A glimmer of understanding, eventually seemed to trickle through the fog, to her failing mind. If this sounds a bit dramatic. It was.
My internet research also showed me just how little is known about this dreadful disease.
We were at this time just dealing with the sleep issue. I thought we could find out more about the underlying disease when Linda was more stable.
We have since searched and found very little information. What we did find is linked at the bottom of this article.
I kept showing her the hub on snoring. Comments came in but no advice.
What this hub did highlight for a lot of people was just how widespread the snoring problem is and how potentially lethal.
Linda was still trying to deal with and come to terms with the fact that the Birdshot may make her blind.
This may sound odd but it was fortunate that Linda couldn't make the connection that the treatment she was on also had the potential to kill her. It has damaged several of her organs.
This is the proverbial rock and a hard place and her decision making was virtually non existent.
Linda was booked into the sleep clinic overnight and hooked up to all sorts of machines.
She was highly monitored all through the night by the dedicated staff and recording machines.
She awoke a few times and found nurses adjusting the devices and machines and they just smiled, as Linda had a mask on and couldn't talk.
All night she drifted in and out of sleep. What the nurses had been doing on their visits were taking readings and adjusting the air flows on the machines to suit Linda's needs.
Data Collected and Assessed.
The next day the Doctor told her in a very calm and caring way, that she did indeed have sleep apnea and that it was a potentially terminal condition that must be treated right away.
This doctors calm professional approach in contrast to the Birdshot doctors approach reassured her that it would be good for her to try a breathing device at home.
Linda was a little horrified by the prospect of having to be hooked up all night to a breathing apparatus but conceded that she would try it.
Psychologically it is very hard to accept that you need this kind of thing when you have always thought of these devices are for 'other' people with serious diseases.
She may still lose her eyesight. The treatment can be lethal. The side effects cause other life threatening conditions. i.e. Sleep apnea, Liver failure, Kidney failure amongst many other side effects.
Trying to explain this to people has been difficult to say the least. The only outwards sign that something is wrong is in Linda's weight gain and that she has no energy.
Some well meaning people have suggested all kinds of weird suggestions. They just cannot grasp what is happening to her.
The realization took time to penetrate her foggy thinking processes, that she was indeed one of those people that needed assistance to sleep safely.
They issued her with a C-pap machine and from the first nights use, she has slept soundly with no problems. The C-pap machine forces air under pressure into her nose and so she sleeps soundly. It works by creating a continuous positive airway pressure delivered via a mask.
The Results were Incredible and Immediate
The immediate effect was startling. She awoke the next morning and was up and alert, she had been sleeping until midday and later, prior to using the C-pap.
Within a week she was moving around the house and just generally alive. Her memory was improving too. As she is now getting a good nights undisturbed sleep.
The effects of the therapy while still draining her energy, at least now she had some energy to drain. Linda has more energy.
She still cannot do too much but it is amazing to watch her coming back from the brink. She is one tough cookie.
Linda was fading fast
Linda was upset that she wouldn't get to see her grandchildren grow up. We thought we were going to lose her.
She is getting stronger every day. She will be on the methotrexate and the C-Pap machine, probably for the rest of her life.
Peterborough Hospital Retinal OCT Scans
Update on the Effectiveness of the Treatment
These medicines have given her a weakened immune system which seems to have allowed the body to start to repair itself. It is taking a very long time.
It's been almost five years (updated 14 Nov 2015)
The treatment is also be damaging other vital organs. So she has to have regular blood tests and liver function tests.
The problem with this kind of rare condition, is that virtually nothing is known about it, and treatments seem to be experimental at best.
In Linda's case the most prominent symptoms appeared by attacking her eyes, it may also have been attacking other organs that were not so apparent.
Her Liver was also shown to be damaged.
Methotrexate is often used for the relief of rheumatoid arthritis. Linda began the treatment in early March 2011 on a dosage of 40 mg of the steroid Prednisolone.
Followed by the introduction of 25 mg of Methotrexate in June, combined with Folic Acid, at the same time, as the reduction of the steroids.
Linda also has to take Ad-Cal (calcium), Alendronic Acid, Buccastem (Anti Sickness Pills), Hydro-cortisone, (Fungal cream), and a constant supply of Antibiotics to deal with the recurring urinary tract infections.
There is a fine balancing act between these chemicals. Linda has to have regular blood tests to check several indicators. Liver function, bone marrow and white blood cells.
Going back to the end of the first month on the treatment.
The medicines started to kick in with a vengeance. The effect was that Linda was immediately bedridden. It just got worse from then onward.
We really didn't understand what was going on and had the good sense to re-read the instructions, that came with the medicines.
The list of side effects, told us that there may indeed be a loss of energy, memory loss and depression.
Among many other side effects. Linda seemed to be having all of them. It gave us little reassurance, that it was the treatment causing these side effects.
The doctors did not believe that she could possible be having all of these side effects. They didn't visit her at home.
Looking back we should have insisted that they visit to see for themselves just what was going on. They were wrong and never bothered to investigate.
This has been an ongoing issue with many of the doctors we have seen. They seem to have little or no conception of the impact of these side effects on the patient and their families.
The effects began gradually to worsen over the next few months.
Trying to explain to people was fruitless, as we really wasn't sure what was going on ourselves either.
Sleep issue become apparent.
By the time we got to Papworth, Linda was literally on her last legs. She slept many hours per day and would only get up for short periods.
The effect on Linda's mind, was to cause her to be become depressed. To be told that you are going to lose your sight is bad enough.
Then that the only treatment available will make you very ill and could potentially kill you.
Linda was entitled to be depressed.
Linda has always been an upbeat positive person. To see this downturn was very disturbing. Having had depression my whole life. For once it was actually helpful. At least here was something I knew about and could help her with.
Linda went through some very dark moments. She is working through it without having to take anti depressants. She doesn't want to take yet more medicines.
Recently we returned to the doctor, for the results of the treatment. Unfortunately it has not been completely successful.
Both of her eyes arteries have been damaged.
Linda's right eye has responded better than the left eye, which is still leaking (known as logging). The doctor has now injected the steroid, into the eye socket itself. If this doesn't work, then the next option, is an injection of steroid, directly into the eye.
This particular doctor has not got the best bedside manner. She just blurts things out, without much thought for the patient. Linda began to cry waiting for the injection in the eye socket. The nurse comforted her, while the doctor prepared the injection.
I said to Linda 'it will be okay, don't worry' at which point the doctor asked, why Linda was crying? I was incensed but said calmly. 'it's not everyday, that you are told, you are going blind', and that the 'treatment she has been on for almost a year has done nothing but made things worse'
It seemed to dawn slowly on the doctor, that Linda was a human being. Not just a bunch of symptoms. She reassured Linda that they were going to make sure she didn't lose her sight.
In all fairness to this doctor.
The treatments available on the NHS are limited. Doctors want to be able to 'fix' problems. When they cannot it must be challenging for them too. Linda sank into another depression after hearing this news.
Linda knows she is depressed, and is positive about coming out of it. We have started our fight back. Linda is tough and she is a fighter.
Thank you for reading. I know this is an unusual hub. It is long and we do appreciate your determination and support.
If just one person reads this and it rings enough bells in their head that they get themselves checked out. Then this hub has served more than it's purpose.
Keeping this online diary helped in so many ways and I have tried to list them as I wrote it.
Update 19th April 2012.
Finally some very good news. The arterial holes in Linda's eyes are finally responding to and appear to be healing up. See photo above.
The artery looked like a fishing net before, full of holes. They are healing. A huge thank you to everyone who has helped over these very difficult months and years.
A very special heartfelt, thank you to Lisa, the family friend without whom we may never have rediscovered the Birdshot was back.
Update 18th March 2013
Linda is getting stronger everyday and is able to get around a bit better. She still has a problem if she comes into contact with anyone with a cold of flu.
The depression is restricted to just a couple of days a week (it is induced by the Methotrexate) and she is getting her head around it.
That a drug that is supposed to heal can make you feel so bad is more than a bit disturbing.
We have taken to wearing surgical masks when we have visitors. I know it sounds funny but it seems to cut down on infections.
Good News at last
Today 15th November 2015 we attended our first conference run by the Birdshot Uveitis Society.
It is their 3rd annual conference to date.
We met some wonderful people and have had some great insights into the whole issue. There were doctors, researchers and many patients with Birdshot.
What we learned is that there is still very little in the way of research being carried out and there is still precious little known about the disease.
There are a few dedicated people around the world (a handful of researchers) doing what they can to try to find some answers with virtually no funds or support.
We have found some highly motivated, kind and understanding people (patients) that are working hard to find a solution.
We share a common enemy and felt an immediate camaraderie with these fellow sufferers. Finally we are not alone. We now have allies and have just begun to fight.
Treatments, Side Effects and Support Networks
- Birdshot Retinochoroidopathy | Doctor | Patient UK
Birdshot Retinochoroidopathy - PatientPlus. Information written by our expert authoring team, consisting of independent GPs, doctors working for pr...
- Uveitis - NHS Choices
Find everything you need to know about Uveitis including causes, symptoms, diagnosis and treatment, with links to other useful resources.
Papworth Hospital on the map
This content is for informational purposes only and does not substitute for formal and individualized diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed medical professional. Do not stop or alter your current course of treatment. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.
© 2011 Micheal