orbital pseudotumor

This is Kristine, Rachel’s mom.

Rachel spent her 13th b’day in the hospital this last March.  I go back and forth with feelings of anxiety and optimism that my daughter’s suffering may or may not end soon.

Rachel’s symptoms began in Feb. 08 with eye pain and slight swelling. The pediatric ophthalmologist thought it might be residual symptoms of a sinus infection. After several days of antibiotics the pain and swelling were worse.  Another doctor (a pediatric neuro-ophthalmologist) in the same group suspected it may be something more serious and ordered a CT Scan which ruled out a tumor but confirmed inflammatory orbital pseudotumor. He put her on prednisone and pred-forte eye drops (the drops were to treat the swelling in the anterior chamber of the eye caused by iritis-also extemely painful). The pain was greatly relieved within a day or two.  He tried to wean her off the steroids over a period of a month but when she got down to 15mg. a day the pain came back with a vengeance. This time both eyes started swelling and the pain (unrelieved by Tylenol or ibuprofen) was so bad that her her body’s response was to start vomiting.  The doctor perscribed something to stop the vomiting so she could at least take her prednisone, but nothing worked. So she ended up in the hospital for a couple of days to get her stablized and start her on IV prednisone and pain killers. The transformation was almost instantaneous.  An MRI (whose image didn’t turn out because of her braces…) and another CT scan still showed pseudotumor (only in her left eye, but she had a lot of pain and some swelling in her right eye).  The doctors also wanted to rule out any other underlying auto-immune disorders that could cause these problems. Thankfully, all the blood work showed her to be clear of any other disease. 

The ophthalmogist decided to work closely with a pediatric rheumatologist, who is much more familiar working with steroids.  He convinced us to try another medication, Cellcept, along with the prednisone. It’s an anti-rejection drug given to people who’ve had organ transplants. His hope was that he could wean her off the the prednisone a lot sooner.  Since then we’ve had lots of dr. visits and Rachel’s had to deal with all the negative side effects of the prednisone: acne (which has been the most difficult for her to deal with), intense moodiness (hard for the rest of the family to deal with), hair growth on her face, and difficulty falling asleep and concentrating (I’m so grateful we homeschool–I can’t imagine how a child deals with all the effects and disruptions of this disease and still keep up with a regular school schedule).  I also forgot to mention that the Dr. put her on Prilosec to protect her stomach from the prednisone and also 400mg of ibuprofen for pain.

Well, two months later, her prednisone dose was down to 12.5mg and the pain and swelling began to return.   The rheumatologist says the Cellcept isn’t doing what he hoped it would and says not to lower the prednisone dose any further.  He now says he wants to switch her to Remecade, an IV infusion (which I’ve been told can take 4-5 hrs. from the beginning to the finish of the process) every 6-8 weeks.  I’ve also read that this is a drug given mostly to people with Chron’s disease or rheumatoid arthritis. 

We’re a family that’s dealing with the anxiety of having a child suffer from the pain and side effects of a disease that no one knows very much about.  I want others to know our story so they might find some encouragement that they’re not alone. And maybe they can also find some treatments that work for them (hopefully some doctors out there are also reading this blog).

It’s good for me to write it down. Thanks for taking the time to read this. 

Kristine

I had visited hospital for the o.p.d. treatment for three years and last November
I finally decided to try to have biopsy, because even after taking predonisone my O.P.Ds have come back,and the result showed “malignant lymphoma!!!!!!”,
God it is NOT pseudo but MALIGNANT!!!
It’s MALT type lymphoma,so radio therapy is quite effective.

I strongly recommend to try biopsy if your O.P.D. repeats too many on and off.

hello everyone! i would like to share my experience with OP since my treatment has been different that what i’ve seen posted here. first of all, i am a 27 year old nursing student in chicago, and trying to juggle such a demanding schedule with such a demanding disease has just really sucked! i think my friends and family are sick of hearing about my stupid eye so i’m glad i found this blog.

i was diagnosed almost 2 years ago on thanksgiving day. a week before thanksgiving in 2004 i woke up and my eye was swollen shut. i went to my internist who immediately sent me to an opthamologist who put me on antibiotics (they were thinking infection at the time). over the next couple of days i got better, then i woke up on thanksgiving at 1am and my eye was swollen again (i was still taking the antibiotics). i was in too much pain to wait till a decent hour so my brother drove me to the ER and i was put on IV steroids. the opthamology residents were called in and diagnosed orbital pseudotumor. the IV steroids worked immediately and i was discharged the next day. i had no idea what i was in for. i think i assumed i would just take prednisone for awhile and that i would be better forever. according to the opth., most OP pts are treated with a few months of steroids and that is the end of it. not for me!

my first round of prednisone lasted about 4 months, and after i was done i noticed my lacrimal gland was really swollen still so i did another round of steroids. by then i was sick of the side effects and my quality of life had seriously diminished because i hated the way i looked and i was always in a bad mood and hungry so i was referred to another opth. who specializes in inflammatory disorders of the eye. luckily this doctor is at the same university where i go to school. he decided to try a round of methotrexate which is a chemo drug that is also used for inflammatory problems like RA and does not have the side effects of prednisone. i was on this for 10 months, and i had moderate control of my OP but it never completely went away. some days i would still have pain, and my right eye always sticks out a little. methotrexate is a pretty serious drug and i regret even going on it because now i’m worried about secondary cancers although i was assured that the risk is low (it also made some of my hair fall out).

then last summer while i was still on methotrexate i noticed my vision was getting blurry. the doctors decided it was time for something else so they recommended a short course of low dose radiation to destroy the tumor. the radiation oncologist wanted a biopsy to confirm that what i have is in fact OP and not cancer but i declined it basically because it would have delayed the radiation and i just wanted this ordeal to be over with, and my opth. said that the MRI was consistent with OP and not a cancerous tumor. the radiation oncologist and i went back and forth about it and he finally agreed to do the radiation without the biopsy. so i went off methotrexate, started a small dose of prednisone again to control the pain while the radiation was taking effect, and finished my radiation course this past monday.

so far i see no results but i was assured that radiation can take awhile to work after the treatment is completed. this is EXTREMELY frustrating because i am still in pain, my eye looks freakish, the radiation drained all of my energy, and my eyebrows are starting to fall out (i was told this is expected and that they grow back). if this doesn’t work i will be so upset because this is my last option. i have spoken to 2 different radiation oncologists at 2 different hospitals and both said this would definitely work and probably put me in remission for good. if it doens’t work then i definitely have to have a biopsy because the OP may have converted to lymphoma and i may need more radiation at a higher dose. i’m really scared that i will be in pain forever and i am nervous about all the medications i have put into my body and what they will do later on. the radiation was low dose but i was told that i will almost definitely develop a cataract from it, which isn’t a big deal i guess, but i would hate for the radiation not to work and now i have a cataract on top of a huge eye.

this thing has taken over my life and i’m ready to be done with it, so i am trying to stay optimistic that the radiation will kick in within the next couple of weeks. today my eye looks puffier than normal but they said the radiation itself caused my eyelid to puff up and will go away.

so this is my story in a nutshell, i wanted to share it because i noticed that many people on this blog have been treated with only prednisone and have had this a lot longer than i have. steroids are not the only treatment out there, and if they didn’t work the first or second time, go and see a radiation oncologist and get his/her opinion. i have read many studies on this and it works in up to 80% of cases in people with steroid relapse and study participants were followed for years after the radiation. it is too soon to tell if radiation worked for me but i will post again in a few weeks with my progress.

unfortunately radiation is extremely expensive so for people who don’t have insurance it wouldn’t be much of an option. i would also recommend, if possible, to switch to an opth. who specializes in inflammatory disorders because regular opth will just keep continuing prednisone. unfortunately there aren’t many and they seem to be located in only urban areas at large university medical centers.

- Here is my journey with Orbital Psuedotumors (OP)
- I was diagnosed with OP about 25 years ago
- Sent to an eye specialist (Dr. X) for a quarterly checkup for about three years – no medication prescribed
- I was not satisfied with the progress, or lack of progress
- Dr. X recommended performing a biopsy which I agreed
- The biopsy results were negative (basically a cluster of non-malignant tissues) and I was sent away
- After approximately five years with Dr. X, he sent me to Dr. Y (head of eye department) at a downtown hospital
- I visited Dr. Y on a regular basis for examinations (check eye sight, some ancient measuring tool to measure the swelling to determine growth…) Again, no medication prescribed at all.
- Dr. Y sent me to numerous specialists within the hospital, including the psychology department, to determine whether the OP is related to other parts of the body malfunctioning. The conclusion is no.
- Over the years, I have had two biopsies with Dr. Y and the results were both negative.
- A little over two and one half years ago, I contracted influenza. In addition to the typical symptoms, I had difficulties breathing with non-stop coughing. My General Practitioner prescribed steroids tablets for 10 days. After 10 days, the swelling in my eyes have decreased significantly. Once I stopped taking the steroids tablets, the swelling came back.
- During my next visit to Dr. Y, I asked him to prescribe me steroids to reduce the swelling.
- Dr. Y is strongly against using steroids for the OP. He explained with excruciating details on the negative side effects of using steroids. He believes the risks outweigh the benefits. I was concerned about taking steroids.
- However, he recommended performing another biopsy which I agreed
- Note: The biopsy was performed with a local anesthetic and I was wide awake during the operation. It takes about two weeks to recover from a biopsy.
- The standard procedure, after the biopsy, is to visit the doctor in approximately two weeks to review results.
- My understanding is the doctor receives two reports from the biopsy. The first report is a summary of results with additional details to follow.
- During my visit with Dr. Y to review the results and remove the stitches from the biopsy, he had the summary report and it was the ‘usual’ stuff… I was on my way out the door when the detailed report was faxed to his office. He took a quick glance & summoned me back to his office and closed the door. I looked at his face while he was reading the report. And, at this point I had a dreadful feeling that something was not right. He broke the news, it was cancerous. Wow, it’s very difficult to describe what is going through my head when I heard the ‘C’ word. I can write a book to describe all my thoughts and feelings at that moment in time. I thought about the kids, family, financial situation, insurance, work, and so on…. (Hey, maybe I will write a book).
- I was put through a very aggressive schedule, at a cancer hospital, to treat the swelling in my eyes. The radiation treatments started a few weeks before Christmas. The treatments simply drained me. I needed to put on a brave front for the rest of the world. I was hurting physically and emotionally.
- I really don’t think I’m the same person after all the treatments. My hair is much thinner now; I need to wear eye glasses; my eyes are dry…. I believe I’m more susceptible in catching influenza. I started to get the annual flu shot.
- I’m now a patient of Dr. Z at the cancer hospital with biannual checkups. Psychologically, I look at life much differently than I did three years ago (that will be in my second book). I am much more focused on spending time with the family. And, the career is not as important anymore.
- I will be celebrating my 30th birthday soon (this will be in chapter two of my book).

Since prednisone seems to be the one drug (a necessary evil, I think) that is used the most when treating orbital pseudotumor, I’m curious to hear what side effects you all experience when on it. Also, what was the highest dosage you were prescribed and for how long?

For me, the side effects that I experienced were:

• Insomnia
• Weight gain
• Swelling in the legs
• Skin clearing up
• Thin skin
• Thicker hair
• Getting hot easily
• Camel hump
• Moon face
• Increased appetite
• Immune system down/susceptible to infections

The weight gain has been particularily bad this most recent flare-up. For those that don’t know, prednisone will kill the signal, to the brain, that your stomach is full. At higher dosages of pred, I would feel hungry all the time! Even if I’ve already ate my usual amount of food on my plate, I would still feel hungry! Doubled with the retaining of water, you gain pounds quickly. Prior to this flare-up, I had lost about 60lbs that I had gained in 2004. That was due to using Phentermine, which is an appetite suppresent. My family doctor prescribed it to me to help me lose the additional pounds. Unfortunately, I gained back most of the weight. I will probably get my doctor to prescribe me another appetite suppressant again. I think I developed a tolerence to the Phentermine after a certain point.

Another thing I noticed after my pred dosage reaches 10mg is that my hair starts to fall out and my skin breaks out really badly. My hair grows a little thick at the higher dosage (thankfully, I don’t have the facial hair problems they say that can develop as a side effect). It really freaked me out when my hair was falling out a lot faster and in noticeable amounts after my 2nd flare-up.

The “thin skin” part, I found out when I had my eyebrows waxed for the first time. When the strips were pulled off, some skin came off with it. Not a lot but enough to freak out the girl that was doing the waxing. She asked if I was on any medications and I told her I was on pred and explained what it was. She said I shouldn’t get my eyebrows waxed since my skin seemed pretty thin.

Anyhow, I want to hear your experiences! Please leave a comment and tell me!

Hello,
My name is Chaz and I am a 33 yr old female in Atlanta currently dealing with Bilateral Orbital Psuedotumors. I was first diagnosed in 94-95 with orbital psuedotumor and was given a Zpack (a week supply of prednisone 40mg) and sent on my way with the Dr telling me that the reoccurrence of them would be slim to none.
Well, I have very sensitive eyes so anytime I would get constant headaches or eye swelling I would go to the eye doctor to see if my “slim to none” psuedotumor had come back. For about a year and a half, I would have minor symptoms and the dr I was seeing at the time would dismiss it as allergies, even though I would ask him to check for the p.t.’s (psuedotumors) Finally, I started getting very bad headaches again the end of 2001. I went to my regular doctor and told him of my previous history and how my eye doctor dismisses them as allergies. He decided to send me for an MRI to be on the safe side and in the mean time put me on migraine meds. Once he got the MRI results back and compared them to the ones from before, he said that the left muscle behind my eye was larger than the right side. He sent me back to my eye doctor with this (sort of as proof that I needed treatment for p.t.’s) Once again, my eye doctor disregarded it as me needing reading glasses (at 28, I DON’T Think so!) and had me fitted for contacts and told me to get a pair of 1.25 or lower reading glasses to wear with my contacts when working on the computer. Well, this was the end of the year, somewhere around Septemberish. Then in November I went back complaining (except this time my left eye was starting to swell up) and yet again, he dismissed it as allergies and advised me to take some Benadryl and it would go away. Well, around the end of Feb 2002, my left eye was still a little puffy, but the right eye started swelling up. By the end of March it had swollen completely shut and stayed that way for about a month (yes, even taking the Benadryl) so I started on my journey to find a doctor smart enough to tell me something better than “Oh, it’s allergies, take Benadryl!” (I think Benadryl was paying off my previous eye doctor! lol) And so my saga begins….

So I went to the New York Eye and Ear Infirmary today and waited about four hours total to see a doctor. When you are uninsured, the physician world becomes almost unbearable. Anyway, so after showering and everything today, my eye didn’t look too bad at all. This was not a good thing. Because I was taking some left-over Prednisone (I know this is bad), the swelling around my eye is not that noticable. My left eye merely looks like it would after a night of crying. Basically, the docs stated that it didn’t look too bad, that they didn’t want to put me on Prednisone, and scheduled an appointment for me with their “Plastics” department. I’m not sure what their “Plastics” department is, but they stated that they deal with OP or IOI all of the time and that I should procure my previous medical records related to the 2001 flare-up and then they’d move forward. The problem enlies in that the appointment is not until the last week of June and I’m scared that now that I’m off the Pred, my eye will just swell again. Oh well, what are you going to do? TGIF!!

Hello All,

My name is Jayne and I am a 31 year old female currently living in NYC. I came across a blog by JoAnn who started this blog for us and thought it would be a great outlet for those of us with this mysterious disorder to exchange experiences and gather information. If you’ve suffered with OP, you know as well as I that there’s extremely limited information regarding the disorder. My hope with this blog is that we can work together to become better informed and help each other cope with the physical and emotional effects of OP.

My experience with OP started about eight years ago in 1998. I was in Las Vegas visiting my brother and sister when overnight, my eye swole shut accompanied by massive pain. You all know the process. It got so bad that I went to an ER in Vegas only to be told I had a massive sinus infection. When I got back home (Mississippi at the time) I went to doctor after doctor all of whom finally decided it wasn’t my sinuses but had no idea what it was. I even had an MRI with a neurologist. They couldn’t diagnose it and I basically lived with it for several months and it eventually subsided on its own. The more I discover about the disorder, I guess I am lucky because there are many of you out there that have it much worse than I and could potentially lose your eye/vision without a diagnosis.

Anyway, life was great until about 2001. I thought it was some fluke thing and hoped it was over. I was now living in Indianapolis, IN after taking a job in publishing out of college. It came on again and thankfully, this time around, the primary care physician I had at the time recognized the disorder and sent me directly to an ophthalmologist. My ophthalmologist knew what it was and I was on Prednisone that afternoon. Ohhhhhhhhhh Prednisone, the drug us OP sufferrers love to hate. I was on Prednisone for several months and eventually weaned off just in time for my summer vacation.

So, here we are in May of 2006 and it’s back! It’s just such a pain in the arse. I hate the drugs, I hate looking like I have a lazy eye, I just plain hate this thing. I am hoping we can all get together and talk about our personal experiences. Tomorrow morning I am going to the NYC Eye and Ear Infirmary to seek treatment. I’m sure I’ll be on Prednisone yet again come tomorrow afternoon and temporarily happy because my eye will look and feel normal, but the rest of my body will be weak, hungry, unable to sleep, and just plain hormonal due to the drugs. Oh, and not to mention the potential hair loss, weight gain, osteoperosis, and basic havoc Prednisone racks on our bodies. There’s got to be something else out there and I look forward to conversing with you all in the hopes of finding it.

Best,
Jayne

I found an interesting article that I think relates to a new treatment therapy for Orbital Pseudotumor patients.

Jayne

I am stupid.  Yes, I really am.  I completely mis-interpreted the directions my opthamologist gave me with regards to taking my dosage of prednisone!  I thought he had said, “Decrease by 10mg (from 60mg) every day till you hit 10mg and then take 5mg every day till your next appointment with me.”  Uh, what he really said was decrease by 10mg every week!

I went for a week at 5mgs and my eye swelling back up and the muscle pressing on my sinuses.  The sinus pain was BAD and left me really grouchy all last week!  I stayed in bed most of the time, just wishing I could gouge my eye out to stop the sinus pain.  *sigh*  Finally, I called the doctor on Monday and after I explained my problem, he said, “Nooooo, it’s 10mg a week!!!!  Go back up to 40mgs now!”

Argh!  I immediately took the correct dosage and went to bed.  I felt much better after an hour and the sinus pain was totally gone.  Damn, a whole fucking week WASTED!  I got nothing accomplished, all because of this!  Next time, I’m having my doctor write down the directions!