Sorry about the length.
I’m a 48 yr female (DOB ’59); history: asthma, allergies, eczema, psoriasis.
1. at 17 yrs of age, center (small) of my left eye vision during exam; ophthalmologist exam noted no evidence of disease; myopic
2. 29 yrs: dry eyes
3. At about 31 yrs, pregnant, 2nd trimester, severe migraine aura, optic neuritis, no evidence of MS; smaller, less frequent migraine auras after birth up until present time (miscarriage 2 yrs prior)
3. New ionternist wanted follow up on optic neuritis; opth. doc did visual evoked response- found significant latency in both eyes; MS discussion took place
3. Breast cancer ’94-St. II, 3.5 cm tumor, 6 round CAF; BRCA2; siblings with BC, melanoma, non-small cell lung, basal skin
4. Few years after chemo, treated by an alternative practitioner for vitamins and foods avoidance. Allergies and energy helped. Latent VER results resolved during this time.
5. GI doc visit-esophageal spasms that started during chemo; hydrochloric acid test did not induce the spasm; yet I was told I had GERD since that area was irritated; on my own, I learned that mashed potatoes and garlic induced spasms – just avoided them.
4. ’05 5 uterine polyps removed
5. Injections for allergies have been given since approx ‘97/’98; “pan allergic”.. good results; still taking them
6. ’07 dx’d EDS III (Ehlers-Danlos type III) ; daughter diagnosed first (’06)
7. sought med. attention upon loss of peripheral vision in l. eye summer ‘07; had migraine aura 1 month before (been a long time since I had one); there was aching in each eye
opth. doc visit: ’07 Optic neuritis Dx discounted; new dx: AZOOR due to retinopathy in left eye (see below); r. eye exam normal
CAR (Cancer Associated Retinopathy) blood testing is currently being done –considered doubtful since retinopathy is only in one eye
8. Misc: heart murmur noted since early teens; structure in spine deemed ‘atypical” hemangioma (’00 by MRI’s 6 months apart… no growth); low ferritin (with usually low normal hematacrit) that is now in low norm. range ) daughter (EDS III) also has this problem
9. onco every 6 months (CA125 done); oopherectomy needs to be done but doc wating on eye problem resolution;
10. I fatigue easily - more muscular fatigue than sleepy; have joint aches; heart fluttering for a few years, infrequent but significant pain in the area of my heart; EKG and cardiac echo done recently but don’t know the results; recent exercise stress test which was good but legs fatigued
Everyday, my eyes ache and, when it happens, my left eye seems bigger. The left is usually aches worse than my right. I feel I have dry eyes since they feel gritty and get red. When the ache comes, my vision starts to degrade a bit. Text msgs on my cell phone bother my eyes … last time caused mild double vision after.
I have dry skin, low, normal blood pressure, and can have a dry mouth. I’ve used Biotene mouth wash and toothpaste since chemo.
Below is the paragraph from my recent exam with an ophthalmologist. I am not satisfied with the way the exams were done or reported. In the last year of so, I’ve noticed a mild dizziness when I switch my head from front looking forward and then 90 degrees to the side. For a long time now, I get dizzy when I look up and then straight ahead. During a scan CAT scan, I suffered vertigo and nausea from staying in the position for so long.
I am not comfortable with the diagnosis of AZOOR. Even though I saw a neuro-opthamologist and a retinal specialist, ultimately, I’ve ended up with a diagnosis that isn’t clear while I have other dx’s that are high risk or rare. I do not have a secure feeling about where I will be 6 months down the road. I also have a prophylactic oopherectomy waiting to be performed (got cancelled by doc last summer and then eye problem started). Any advice on how to proceed … through either the alternative or allopathic systems… is appreciated.
Here is the paragraph from the ophthalmologist from well known hospital and then some blood chemistry done by my primary:
“The Neuro-Ophthalmological examination revealed visual acuities with glasses were: 16/13 OD; 16/100 +1 with pinhole improvement to 16/50 +2OS. Amsler grid testing was normal OU. Pupils were equal in size and showed an afferent papillary defect OS. The external examination of the eyes and orbits was normal. The lids were in normal position. The examination of extraocular motility revealed no strabismus, and full dictions and versions of both eyes. Persuit and saccadic fundtion was normal. There was no nystagmus. Slit lamp examination revealed normal conjuctiva, cornea, anterior chamber and iris. Thee were 1 + cells in the anterior vitrous OS. The crystalline lenses were normal. Applanation tonometry 1030 hours) revealed pressures of 12 mm OD; 13 mm OS. Dilated stereoscopic funduscopy revealed a normal appear optic nerve OD and mildly pale optic nerve OS, with cup-disc ratios of 0.2 OD; o.3. The retinal arterioles are definitely narrowed (diffusely) OS. A large infer-nasal arteriole appears white, possibly with thickened a wall (i.e., ghost vessel). Two distinct areas of perivascular, linear pigmentation were evident OS. A large plaque of chrorio-retinal pigment atrophy/hypertrophy is evident inferotemporally.
Formulation: The ERG confirmed the presence of a retinopathy. The clinical examination reveals evidence of inflammatory. the cause of this inflammatory retinopathy is not certain; I agree with Dr. ___(retinal specialist) that the most likely diagnosis is AZOOR.
We discussed the potential for future inflammatory attacks, with additional visual loss. Her right eye could become involved in the future. I have recommended that she return for examination 2 – 3 times per year, assuming she does not develop any new visual symptoms.”
[right eye involvement and loss of vision was NEVER discussed but this is what is written… also CAR testing not mentioned]
“I have recommended that she discuss some of her non-visual symptoms with her other doctors, especially the chest pain.”
From the primary care doc:
ANA HEP2 (Titer): 1:40
ANA HEP2 (Pattern): DIFFUSE
ANA ML (Titer): TRACE
ANA ML (Pattern) 0
ANA (quant): ANA SCREEN POSITIVE, showing non-specific pattern of no clinical significance
Well for 48 you've had more than your share of medical problems. Acute Zonal Occult Outer Retinopathy (AZOOR) is not an area that I have neither expertise or experience in. I am therefore unable to help you. I can say that there are many diseases that can only be diagnosed with the passage of time. The prototypes are multiple sclerosis, degenerative neurological disease and autoimmune/collagen vasuclar disease. There are parallels to that game "Wheel of Fortune" when they show the word puzzle and there are only blanks no-one can make out the answer, as more and more letters become visible it becomes easier to make out the answer, when only a very few letters have not been exposed everyone in the audience knows the answer. That's the way a lot of disease work also.
You may be able to find a neurology or neuro-ophthalmology website that may be able to help you. remember its much better to have an uncertain disease but minimal symptoms than the certainty of the diagnosis being 100% but untreatable or a really terrible disease. Give me uncertainty anyday.
JCH III MD
Anyone that goes into neuro-ophthalmology is usually highly intellient, doesn't mind plowing in the field of uncertainty and often ends up doing watchful waiting.
Thanks for you candid reply. I'm certainly am not surprised by your answer.
I think my case takes a good ... available ... internist. That is the person who should be coordinating the specialist and doing the watchful waiting. I have a bright internist but his style is to do the minimal. All the coordination of going to the neuro-ophth, retinal specialist, geneticist, and cardiologist (whom I uncovered at a children's hospital & taking adults... specializing in Marfan's, familial syndromes, and connective tissue disorders)... all of that I did myself. That doesn't seem right. Since I am not an MD, I am afraid I am not up for the "job" and will make a mistake.
I am changing to a physician who requires a membership fee. In return, she takes no more than 400 patients. I know she is good, well connected, and respected. She used to be my internist before she went "concierge".
I learned a long time ago that the cost benefit ratio puts me at a disadvantage. And the deeper I get into specialty practices, the less anyone wants to know about anything to do with me that is outside their specialty. I'm not blaming anyone... it's just the state of health care today.
I can do watchful waiting ... as long as it is done well. Uncertainty has turned too many times into a terrible disease and, in some cases, has caused deaths in my family. Uncertainty was just a name for the waiting period until one got VERY sick. I know what you are saying ... but I have trouble thinking in those terms. Mine has been a "Wheel of Misfortune"... no ones fault but it takes a doc to say, "this one needs a special attention." That's tough to find.
Thanks again. If you know of anyone who likes to play with a complex case, just tell them to write to me.
These are some thoughs. If you are near a world class tertiary treatment center such as the Mayo Clinic you might want to be evaluated there.
As for "the state of health care today" if you live in the USA be thankful. Read some of the many posts from patients in Canada and England that are waiting months to up to a year to see even one specialist and may of the tests you have done cannot be obtained there. The cancer survival rate for breast cancer in the USA and the detection is the best in the world.
I'm in Boston and my husband (and I was) in health care industry. Mayo would certainly be a good option for getting a second opinion to counter the one in Boston.
I was too young to be on the beneficial side of the cost benefit ratio when diagnosed with BC. Detection would not have even been used. An earlier diagnosis could have only come through the art of medicine and not through a reimbursement matrix. It was only found at my persistence and vigilance... after 3 years. At that time, my father's history did not matter on the clinical "side of the street". (I mean this quite literally because I could cross the street and meet researchers/part time clinicians who were well aware that it did ...but the studies weren't published yet). At this time, the big debate over should mammograms be given at 40 or 35 was going on. I went to 2 breast surgeons who said I did not need to have a mammogram. One told me it was more important to have a less stressful way of life. I know she meant well.... but...wow.
The numbers you refer to are for the majority. One thing that I learned during BC treatment was that my profile was deviant from the norm. All the treatment info I was being given was based on a 62 year old women. So I needed to ask my doctors to think in terms of my profile and use the art of medicine versus what each health care community concurs is best for a 62 year old women. Consequently, my request to have a bilateral mastectomy and to use CAF instead of the combo with metheltrexate (???Name of protocol) was honored (after 2 second opinions). A few years later is was recommended as the treatment of choice for BRCA patients. (admittedly, 6 rounds of CAF was overkill... but that was the best we knew at that time).
I've had all very good doc's but I've been in strange situations where I've pushed for a consideration of a particular treatment and gotten some "I did not tell you that" statements and guidance. It's sort of scary but that is the way it is.
Right now, I find myself in the EDS "boat" and some surprisingly similarities are occurring. Dr. McDonnell at the NIH is studying connective tissue disorders and has recently published about other things that EDS patients have. I've taken some of that in order to get my daughter's doctor to consider giving her a pneumo-vac. I just want to remain active and healthy.
Due to what I know about my history, I think the attenuated a. vessels are a separate issue from the problem with my rods and cones. The latter, I believe has been coming on for years. But I am not hearing that being considered by any one. I am looking forward to the appointment with the cardiologist... hopefully for some peace of mind.
BTW, I fixed my ache problem in my eyes: I have dry eyes. It was not as bad as the the time I had it years ago so I did not recognize it. My vision improved and the ached disappeared with eye drops. I wish either the general ophthalmologist, the retinal specialist, or the neuro-ophthalmologist could have at least provided me with a hint of self help. I spent 25 hours and 4 dilations over 2.5 months and I did not even come away with help with dry eyes. That is the disadvantage of being amongst specialists. The can get distracted by the obvious.
I know this sounds negative but you would have to be in my situation and experiencing what I do in health care. It's quite challenging to navigate.
On a more positive note, I would like to say that my daughter's orthpaedic doctor made a phenomenal call on the EDS diagnosis. Her case is mild but could have been made so much worse. He diagnosed it early enough so that operations weren't done to correct problems. Other EDS'rs have not been so lucky and suffer from less than successful orthopaedic interventions until they are diagnosed. It was a tough call but through his patience and quite artful way of doing medicine.... he got it. That in turn has helped me to get a diagnosis and access to specialized PT/OT group and a pain clinic. So we do benefit by being in Boston.
Thanks for your candid reply and for being on this site. You sound like you enjoy your work and I think that is a big part of quality medical care. I am sure your patients have benefited.
I am interested in talking/emailing with other people who have AZOOR/AIBSE I was just diagnosed after 9 months of thinking I had optic neuritis.
laurie at ***@**** if you contact me please put big blind spot in subject
My daughter is 23 & recently has found that her eyes would have water in them in the corners & when she wiped it, it was bloody...what exactly is that & what can she do about it.???? To her knowledge she did not have any trauma to her head area & she doesn't have a cold. please let us know. thank you . A concerned mom
CAR - negative
Sjogren's testing - negative
Some IL??? testing (don't have it in front of me right now).. but it was negative
HLA B27 positive
SBO 07/08; clean pathology on all; 7 weeks out and feeling fine, surprisingly... and appreciative of that; pre-op found 5mm x 9mm nodule on chest x-ray; CT scan says it scarring but finds a 3 mm in left lung; radiologoist recommends 3 month CT scan fo9llow-up... I think they have "big guns" for equipment over at MGH and that they just can see too much... II am thinking enough is enough... However, I will go with the pulmonologist's recommendation (he's is a critical care specialilst).
rheumatologist visit - does not see anything at the moment (sort of that watchful waiting approach); this one admittedly did not know much about EDS ; she mentioned a local rheumatologist who deals excusively with fibromyalgia and thought he was the best bet in the area... otherwise she recommends going out of state; I mentioned I had psoriasis for about 17 years; told her I go through phases of joint pain and muscle spasms... last year was an eventful phase and now things are doing pretty well.... just a shoulder and hip; she recommended a neurologist and getting my left foot looked at (big toe joint and toe has become malformed/deformed due to a bunion that went out of control healing from torn ankle ligaments).
In reading about CAR testing, it can be false-negative. My neuro- doctor said it could be relied upon but I am not seeing it so simply stated in the literature. I find that if there is an unexplained retinopathy such as mine (even if it is unitlateral), a CT scan should be performed since it is usually lung cancer causing it and if nothing found there, a few other investigations should be done. I would think that at minimum,a CT scan should have been recommended (which I finally got due to pre-op---but 9 months later). Further pursuit would be a matter of individual approach, I would think. So I guess there is disagreement in the field on a neg CAR report. Is this correct?
Positive CAR results are first associated with NSC lung carcinoma, then breast, ovarian... and there is another... I believe it is melanoma. All for which I have an higher risk of. There is also a case in the literature of a CAR positive patient who had a carcinoma of the uterus. I have my uterus and just found out that a previous physican did an endometrial abaltion after a polypectomy (the former of which he did not advise me of before nor after the procedure). I had no bleeding problems so I am wondering why this was done. I asked the gyn to take a look at the polyp area (there were 5) because I was having the same sensations again She said she could not because of the EA). Well, back to CAR: if it were to be endometrial CA causing the retinopathy, I would not show the normal sign for early detection, bleeding, due to the EA. Exceedingly small chance of CAR due to EC but it is just one more tidbit in the picture. Dr. did a D & C during BSO operation and took washings while doing BSO so things must be fine for now, I guess.
So question: what is your opinion on the CAR testing... can it be relied upon? What would you do with a patient who has a negative result but high risk for the most likely cancers that can cause a CAR?
I am set to have my eye situation reviewed by another doctor who runs a lab associated with MIT. I detest all the testing (ERG's, etc) however, I will only be dilated for one day. I show up at 8 and leave at 6. So one bad day and it is over with. I will also have another opinion. I am told he is not full of the "warm and fuzzies" but he is complete and information. (I don't care... I'll bring a stuffed animal if I need one : ).
I am hoping this doctor can shed some light on CAR and also talk to me about nutritional factors that may be involved. See, when I had optic neuritis years ago and cancer, I saw a biochemist who practiced alternatively. I am not one to usually do this, but I felt like the walking dead and needed something which I wasn't getting from my standard doctors. I did very well by seeing him. My allergies got better and I felt human again, no bone pain and had energy. My migraine auras went away. The optic neuritis never returned. Some of what he prescribed for me (for example, D3) is what said to be necessary for many people for an assortment of reasons. He told me at the time that it was more of a hormone than a vitamin. He also prescribed magnesium and selenium. At that time, he would not prescribe Vit E because it acted too much like an estrogen and that was not good for me. Much of this was for the cancer prevention benefit but also because of my eye problem.
Is there anyone studying nutritional factors that contribute to eye disease such as retinopathy or demylienation? The bio-chem person has moved further away from me so access is difficult.
Dr. Hagan -- I realize that AZOOR is not something you specialize in. However, I've looked at the credentials of some of your colleagues in your practice. Some probably know the opthamologists and neuro-opth's I am seeing. It's a small world when it come s to neuro-opthamology. Feel free to share my info. Perhaps someone has a connection with someone who is studying this rare sort of disorder. I don't have easy access to literature... nor do I pretend to understand all that I can access. So anything that might point me in an area that could be considered would be great. The same to the community at large.
BTW, I do believe that penetrance (s) can be influenced by other things. It's interesting that science is just being reported on the existence of an epi-genome (as if the genome wsn't a big enough project! : ) It supports my intuitive sense, correctly or incorrectly, that my body needs more that the average body to run correctly My immune system sort of has a "Type A" personality. Seriously, though, I've been told I have a hyper immune system that doesn't know when to quit. It just makes sense to me that a more work laden "machine" needs more fuel or better fuel. Hence, I think my penentrance is high because I am not taking in enough ... producing it myself... in order to keep every "mistake" from doing the least damage and to support that which works well. That's why I saw the bio-chem practitioner. I am entered in a sort of Medical Olympics and can't get all I need from the basic 3 meals a day. Just an FYI
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