Lymphomatoid Granulomatosis

Fredda said:

I was finally diagnosed with stage 1 LG in February and have been on rituxan for several weeks and am now on combination of rituxan and chemo. There are no other cases like mine that I am aware of in my area and even my doctors have had little experience with this since it is rare and hard to diagnose. Any info would be helpful. What are sympthoms? I have cough, no energy, woozy, and lose voice often.

Hi Fredda,

Where are you located? It is a very rare disease and even the top hospitals in New York City directed us to Bethesda Maryland to part of a clinical study it is about a 4 hour trip for us. My husband is patient #51. I would love to hear more of your story. Did you have a biopsy? We were told down at the NIH that chemo was how they originally treated (and the local oncologist we had originally gone to was going to treat us that way) LYG but they found the route of the problem was the immune system. So that is why we are on interferon only. From my understanding only grade III LYG receives chemo but since it is so rare and each case is different you never know. My husband had a consistent cough, very tired and lost a lot of weight. After a CT and PET scan they had found he had a bunch of nodules on his lungs. He was in remission but had a relapse when we went back to the NIH last week..so his dosage of interferon was just increased. He was 38 years old when he first got sick and just turned 40 this weekend. Would love to hear more of your story.
Sincerely,
Patty

Fredda said:

Dear Patty,
Thanks for your reply. We live in Venice, Florida, coming from Brooklyn and Liberty, NY. I am a 72 year-old lady who had a cough for about one year. Went to my internist, had all kinds of cough medications, prednisone, even went to an allergist thinking I had allergy or asthma. The first chest x-ray came out negative, the next one positive and showed nodules and lesions. I had two needle biopsies which were negative. I did have uterine cancer when I was in my 50's. My son, Peter, is a cancer researcher at the U of Penn and got me into Moffitt Cancer Center in Tampa. I was diagnosed with lymphocytic interstitial pneumonitis. The lung specialist there had me as his first patient. I asked Peter to find me a Doctor who perhaps has seen someone with LIP that I could talk to and find out the prognosis. We were going to Arizona for Thanksgiving last year and Peter made an appointment for me to see a specialist at the National Jewish Research Center in Denver, Colorado. All my test were sent there to be reviewed. The Doctor told me that he did not think the diagnosis was correct. It was on the right side of the spectrum, lymphoma was on the left and he felt I was going towards the left and the only way to have a definitive answer was to go in the have a large piece taken out of the lung which I had done in February in Tampa. Peter also had all the info sent to the NIH in Bethesda as he knew about the trial run, but for us it would be too much for traveling. The NIH concurred with the diagnosis of LYG which is hard to pronounce and spell. I have had pet scans, catscans, etc. and have one in two weeks to see if the infusions are working. The local oncologist that I see locally keeps on telling me that LYG is rare and very hard to diagnose. I do not think there is a definite medication. Interferon was mentioned, but Moffitt decided to start with rituxin-and then chemo. I hope it is working. I am not a computer person and my son who was visiting last week-end set me up with this Cancer Network. I'm glad that he did. Do keep in touch.
Sincerely,
Fredda

Well Fredda..your story sounds so much like ours. I am glad that the NIH confirmed everything for you...we have put our faith in them because quite frankly that is all we have. I have been told the same thing...it is so rare and from our experience we know it is so difficult to diagnose. I have been looking for someone who has this and so glad I did...I will keep thinking about you, and keep me posted as to how you are responding when you go back in 2 weeks. We go back on August 30th and I hope that things are looking up...he will be on the interferon at least another year, and he has already been on it 7 months. He is a trooper though...we have 3 kids and he commutes into NYC everyday by train. I agree when you tell people what you have most do not understand nor can they pronounce it. I feel like I am constantly explaining myself. I hope your son Peter gets to study this disease...maybe he can tell us why some people get this. Take care and talk to you soon.

Patty

Fredda said:

Dear Patty,
I have been thinking about you and wonder if you saw an article in the newspaper "Patient access to lymphoma drug is limited" that appeared in our local newspaper on July, 14th. It was on the front page of the New York Times that same day. A cousin of mine from NYC called my son to tell him about the story. It was interesting and pointed out that a patient has to be an advocate and ask plenty of questions which I'm sure you have done and I know I certainly still do. Let me know about the newspaper article and I will send it to you if you have not read it. When you have a minute, please tell me your husband's symptoms. Does he cough up clear stuff? Does he lose his voice? Does he feel woozy (not dizzy, but unfocused)? You wrote that he works. I retired 4 yeears ago as a retailer and I could no more go into work now because of being tired and woozy. I keep asking the doctor maybe this is because I am older and he says no that it is because of LYG. Any information you can give me would be helpful. I hope your husband responds to his treatment. We are thinking of him. He is Peter's age. Do keep in touch.
Fredda

Glad to hear from you again. I just read that article online and it was really interesting...put it in as a favorite so I can refer back to if I ever need to. When my husband was at the height of his illness which was about Jan/Feb 06 he was still working but when I think back to how sick he was and I did not realize it, but he continued to work and I do not know how he did it. The doctors were shocked too. He had a persistant cough pretty much all day long, back pains that would take his breath away (sometimes I felt like I needed to call 911), weakened voice, he was definitely a bit "out of it" and the biggest thing was he had lost about 25 pounds in one month. We were expecting our 3rd child at this time that I never really noticed any of it which so upsets me....I think I got myself wrapped up in the pregnancy and my other 2 kids. It was not until I was in the hospital after having our son that I told him to go to the doctor...my other 2 were in school and I was in the hospital it was the perfect time. That is where our life went crazy..we went from shear happiness to devastating news. We were told he had Stage IV Metastatic Colon Cancer, that proved wrong after a colonoscopy. Then we went to active TB, cancer that we can not locate,etc. After 2 open lung biopsies (my son was only 4 weeks old by then!)..he was diagnosed with Wegener's Granulomatosis...he was treated for 7 months with chemo, and after that something still was wrong and the biopsies were sent to Sloan Kettering and the NIH which confirmed his real disease. Eventually he would not have been able to work if we did not act quickly, and my job was to be his advocate. I would not stop until I got answers that made sense...when we were told he had colon cancer I would not accept it. He was 38 with no family history, no cancer markers etc...but they told us they were 98% positive...we left that oncologist real quick. I am hoping and praying everyday that this treatment will work...the interferon is so tough on him, and the whole family but we know that this is our only option. I hope you are responding as well, and I think of you often. I am off to the Philly area today to visit old college roommates, and my parents are both from Brooklyn (they are 71)...what a small world!
Take Care...talk to you soon. Patty

sembe said:

Hello, I'm from England, and my 34 yr old son also has LG. It was such a shock when he was diagnosed with it last month. He'd had pneumonia last January, where his lungs were seen to be covered in "white spots". He didn't take too long to get over the pneumonia, but the doctors kept him off work for 10 weeks. During this time he went to hospital to give blood and have x-rays on his lungs, which still showed these white spots. So then he went to another hospital in March for a lung biopsy and a camera down his throat. Then, come June, the consultant in the hospital sent him to one in London for further blood tests and x-rays, who sent him to another London hospital a week or so later!!! So he's been to 4 different hospitals! This last one treats cancer only, and they're treating my son as if he had low-grade lymphoma and he's been on alpha interferon for the last month, We've been told there are only about 5 in 100,000 people who have LG, so it's that rare and I wish I knew how he got it in the first place. He worked out in the cold just before he caught pneumonia, so we think his immune system went up the creek when he got cold, and we're not sure whether it really was pneumonia or the start of the LG. It really is wonderful to hear him laughing, like when he's watching something on the TV. Will write more soon.

Regards - sembe

Hi Sembe
I wish I knew how my husband got LYG too...it is a real mystery. How did they finally diagnosis your son? Was it through a lung biopsy? How is he handling the interferon? My husband is really tired most of the time but is still managing to work. He was on 10 million units for 7 months, but we had a setback a couple of weeks ago so he is now on 15 million units and should be on that for at least a year. How long did they tell your son he would be on it?
Regards,
Patty

sembe said:

Hi Patty and Fredda

The consultants said they'd diagnosed LYG by using a whole picture scenario, which I think meant looking into all of the x-rays, the ultrasounds, the blood specimens, the camera picture and lung biopsy, but I think it was the latter which finalised their diagnosis, but didn't confirm it til Tony got to the cancer hospital. Tony had a cold for 3 weeks, over Christmas, where he was coughing and sneezing, then came the pneumonia, otherwise he'd had no symptoms at all, but the cold and cough might have carried on, which after a couple of months, would have sent him then down to the doctors, and the x-rays etc might have started up from then. Tony's on 10 million units too, like your husband Patty; one person said for possibly up to 20 months, another said up to 12 months, so they're not sure yet. He's going up to London again next Tuesday, to get some more interferon - the flu symptoms side effect when he first started interferon seem to have gone now, but he's been told he'll get more tired than he's feeling at the moment. Otherwise he seems fine, and is working like your husband.
I hope the rituxin and chemo are working Fredda, and yes, the tiredness that the doctor confirmed to you was because of LYG does seems to be right. Patty - can your husband remember a time when he got very cold, like Tony did, Fredda, did you ever get very cold at any time? How LYG starts is just guesswork really, because even the doctors don't know what causes LYG.

Regards - sembe

Hi Sembe,

I do not remember my husband sick atany time...it all started in January/February so it was cold around here then, but he never seemed sick. The only thing he was doing besides work was redoing the nursery in my house so he was taking down sheetrock and putting up new ones. He lost about 20 pounds in one month without trying, was coughing, and was having difficulty breathing...he was always very healthy. Robert was on 10 million units for 7 months, and now they are saying at least another 12 months on the 15 million units. Did they tell you what grade your son has I, II, or III? My husband is grade II. I am so glad that I found someone else dealing with this disease...it is comforting to know there are others out there going through this too.
Regards,
Patty

sembe said:

Hi Patty - Tony's in the middle of Grade I and II and is going back to the hospital to give blood again on 21 August, and collect some more interferon which evidently can only be used within 4 weeks and has to be kept in the fridge doesn't it?
I'm hoping the doctors will tell Tony how he's doing, and I might phone them up myself to find out.
Is Robert going for a check-up etc on 30 August? How long was he in remission and how did you know he'd had a relapse? Did Robert go straight onto the interferon after having had chemo and when he was in remission, was he still taking the interferon? It's nice to be able to talk to somebody who understands and I wonder how many others currently have LYG.

Regards - sembe

Hi Sembe,

Yes interferon does have to be kept in the fridge. Robert goes on the 30th for a check-up..everytime he goes he gets a complete cat scan and bloodwork. He was in remission for 7 months and was still taking the interferon. We were told that once you are in remission you should be on the interferon for 12 months after that. The only reason he was on chemo in the first place was he was misdiagnosed with something else...so the chemo was for that, not for the LYG. When we went for the check up in July they found a new spot on his lung...that is how we found out he relapsed. They take blood just to see how his white blood cell count is. There were no other signs...he did not feel sick at all. It was a real blow to us because he was supposed to be done the interferon this November...but now since he relapsed we are looking at atleast another year.
Robert is in a clinical trial at the National Institute of Health here in the states..he is currently patient #51....they told us we would have a better chance of winning the lottery than getting LYG.
Thanks so much for talking with me...how is Tony handling the interferon now? I hope OK.

Regards,
Patty

sembe said:

Hi Patty

Tony's injected the interferon 15 times now, the last 4 times being at 10 MU. He's still dubious when taking it, and holds the needle while he builds himself up to put it in because of the pain. He seems ok, other than tired.

I've told Tony that if I had a pound (sterling) for every tear I'd cried since his diagnosis I'd be a millionaire - it seems so unfair and I bet you feel the same about Robert, especially about the relapse as well. At the moment the doctors are only asking Tony for blood samples, and will be giving him a scan at 3 months, but I wish they'd do this every month. I hope the scan will be every month when he gets to the remission stage as a relapse could go undiagnosed for quite a few weeks otherwise. Was Robert's relapse spotted early?

I'm on holiday until the end of August and will write as soon as I'm back.

Regards - Shirley

Hi Sembe,

Tony will become an old pro at it real soon...as bad as that sounds. I have cried almost everyday for a year now, it seems so unfair. I try to hold myself together for my kids but they know that someday Daddy will be fine.
Robert was getting cat scans every 6 weeks at first, and when they switched it to 3 months is when they saw the spot. They did catch it very early since they said the spot was extremely small. He will know have cat scans every 4-6 weeks, and gets blood every week, where when he was in remission we were down to every 3 weeks.

I hope you enjoy your holiday and look forward to talking to you when we get back. Hopefully you and I will both have good news.
Take care,
Patty

Fredda said:

Dear Patty and Sembe,
I have been thinking of you both and have just read through all the blogs again. I just had another catscan and met with my oncologist. The scan was much improved compared to the last one and showed that some of the nodules or lesions have disappeared while others have reduced in size. I am going through another regiment of rituxin and chemo until the end of September and then we will go from there. LYG is not a disease that is caught in the sense of a cold, but has to do with an irregularity of the B-cells that are part of the immune system in the blood stream. In the case of Robert, Tony and myself, the problem with the B-cells manifested itself in the lungs causing the problems that we have. And Patty, I too had to have a colonoscopy to assure the Doctors that the irregular cells in my lungs were not caused by a metastisized colon cancer. I do hope that both Robert and Tony will have good results. You are both in my thoughts. Will keep in touch.
Fredda

Hi Fredda,

So glad to hear from you again, and so glad to hear that your scans are improving. I hope it all continues. Robert goes back to the NIH this Thursday and I am really hoping for good news...don't know how I will handle hearing anything else. I will keep you posted and keep praying.

Patty