My Fight with Histiocytosis X

Before I start, if you or someone in your family has been diagnosed with Histiocytosis you can find help with the good people at the Histiocytosis Association of America. Your can find a link to their homepage at the bottom of this page. Thank you!

I first felt that something was wrong the end of November and beginning of December 1999. I had pain in my back that "wandered" along my back from one side to the other. It got so bad that I made an appointment with my family doctor. He said it was just muscle pain and would probably go away.

The following weekend I was on a trip to London and Helsinki to setup some business deals for a friend of mine. During lunch on Saturday in London I had to cough and I thought when I did that my left lung exploded. The pain was terrible, but I did not say anything because I did not want to be possibly hospitalized in London! I continued on to Helsinki, dealing with the pain as best I could. Upon returning from Helsinki late that Sunday evening I was glad to get home. First thing Monday morning I was again in the waiting room of my family doctor.

After describing to him what had happened, he mad an x-ray of my lungs. Needless to say, things did not look good. The x-ray picture of my lungs looked like what I call "salt and pepper". My doctor then said I would have to immediately be referred to a lung specialist and made me an appointment for later that same day! After a few additional tests and more x-rays at the specialist it was determined that I needed to be admitted to a lung clinic not far from our home in Steinach.

Three days later I was admitted into the clinic. After more tests, the doctors decided they had to perform a transbronchial biopsy. A transbronchial biopsy is a procedure of inserting a long "tube" through the mouth or nose into the bronchial area and "plucking" out a piece of the lung for further testing. Unfortunately, the test was inconclusive because the transbronchial biopsy could not take such a small piece of lung that was affected. It was, being shortly before Christmas, decided that I would return on the 2nd of January 2000 to the clinic for a Thoracotomy to determine exactly was disease I had.

Upon returning to the clinic on the morning of the 2nd of January, I was again subjected to more tests and in the afternoon the doctors came to my room to describe the procedure they were going to perform the next day. The procedure was to be a Thoracotomy with a wedge excision. Basically they made an incision from under the armpit to just below the nipple and then spread the ribs apart to get to the lung and take the biopsy. After I was finished in the operating room, I was admitted to the intensive care ward where I would stay for the next three days.

The doctors had determined the Thoracotomy showed that I had pulmonary Langerhans cell Histiocytosis X (LCH), a rare disease most commonly diagnosed in young children. Why the disease afflicts adults has not been determined! The problem with the diagnosis was the pain that I was having. As the lungs do not transmit pain the doctors, or at least my family doctor, said the pain was in my head. For the next two years I put up with a continuous level of pain equal to a real bad tooth ache, and pain spikes which felt like hot needles being jabbed into my rib cage.

Every three months I was admitted to the clinic to see the how pulmonary Histiocytosis X was evolving. After approximately one year there was a spontaneous healing of the lungs. The doctors were happy with the progress I had been making. But, the pain was just as bad if not worse. Finally in 2002 my family doctor agreed that we should do a MRI. The MRI results showed that the Histiocytosis had attacked the spinal column starting in the neck and going down into the back and was slowly dissolving the bone - what is called Eosinophilic Granuloma. Now we knew were the pain was coming from.

I am now 54-years-old and have been fighting this disease for five full years. In addition to the treatments and appointments with medical doctors, I have also spent another 6-week stay at the pain clinic and I am still seeing my psychiatrist to help me through these difficult times.

If you are interested in the rest of my story in more detail then read on. If you also suffer from LCH and would like to contact me you can do so under the "Contact me" page.

The beginning:

I first began having uncomfortable pain late November 1999. I made an appointment with my family doctor thinking it was probable that I was having just some muscle spasms in my back. After a preliminary examination and an x-ray, I was referred to a lung specialist the same day in a near by city. After taking his own x-rays, the specialist was also at a loss to give me a concrete diagnosis. Both x-rays should white flecks or spots in my lungs. The specialist wanted to send me to a lung clinic right away in the next small city. So on January 4, 2000 I underwent a Thoractomy in the clinic. The results came three or four days later and I was diagnosed with pulmonary Langerhans cell Histiocytosis (LCH).

The only thing I was told at the beginning was that I had a very rare disease. So with me in the hospital recuperating from my surgery my wife started researching LCH over the internet. And what she found was very troubling to say the least. With this new knowledge we were able to talk intelligently with my attending physician in the hospital about the disease and what was in store for me. Furthermore, it was disturbing to learn that about 1 in 560,000 adults will be diagnosed with LCH each year! Because LCH is such a rare disease, it has been very frustrating in finding a physician with first-hand knowledge in treating LCH. It is usually treated like cancer or at least in my case. Very few physicians have experience with this disease and my family doctor once told me that I "knew as much about LCH as most doctors."

Following the surgery, my next two years were filled with doctor appointments and experimenting with different pain medication. I was trying to find a cure or at least some kind of therapy for LCH. What really was bothering me was the cause of the sometimes intense pain I was having. Most doctors said either they didn't know or that the pain was "all in my mind" because the lungs do not transmit pain. In the Fall of 2002, my family doctor suggested that a high-resolution MRI might help explain what was causing the pain. The MRI showed that I was not suffering from pulmonary Histiocytosis, but instead the disease was dissolving my spinal column (Eosinophilic granuloma) away. My pain was being caused by the spinal column nerves being left exposed. Epidermal morphine patches (Durogesic) and other pain pills (Katadolon and Oxygesic) were providing little relief.

All during this time I was trying to cope as best I could. But with all of the appointments and doctors and drugs and pain, it was becoming too much for me to handle. You might wonder at all of the things that go through ones mind after being diagnosed with an incurable disease. I felt myself coming to a point that I was afraid I would not be able to handle my situation any longer. That is when I made the decision to make an appointment with a psychologist. I knew I needed the kind of professional help that you can't get from family and friends. It was the best thing I could have done! I have been with the same psychologist through these years and I know that without him things could have been a lot worse!

So with the new MRI in hand I made an appointment at the University Clinic in Wuerzburg, Germany. My new doctor at the clinic and I decided that I would begin a 26-week chemotherapy (Vinblastin) treatment at the clinic. During this time, I also had Cortisone (Mercaptopurine 50mg cont. and pulses of Prednisone 40mg.) therapy and we also decided that I would have radiation treatment, 18 times (30 Gy) for the worse damaged vertebrae in my back. These treatments were followed by six-month check-ups to monitor my progress. Unfortunately, the treatments did not provide the desired relief from my symptoms. In addition to LCH, I was also now diagnosed with Osteoporosis. I have experienced abnormal loss of teeth, my eyesight has gotten worse, and I also suffer from dizziness and memory loss.

In early fall of 2004, I was admitted for a six-week stay at a special pain clinic in Bad Mergentheim, Germany. I experienced some relief through a variety of treatments I received, including daily treatments of hot mud packs, lying in hot sand, magnetic field therapy, massages, and injections of pain medication directly along the spinal column and shoulder blade (20-25 injections per sitting). I also met with a psychologist. We tried relaxing techniques and hypnosis. Upon leaving the clinic I did feel better, not much better, but still better than. Unfortunately, two weeks after returning home, the pain returned and I was experiencing more pain.

It was that time again for another MRI and the results were not good. The damage had spread further along my spine. So my family doctor and I decided that I should undergo another chemotherapy. With no desire to return to the university clinic, I searched for a doctor to perform the chemotherapy. My search led me to a physician who was not only head of a hospital chemotherapy unit, but also had experience in treating LCH or who had at least heard of it! Under this doctor's guidance, I began another 26 weeks of chemotherapy (Vinblastin, Ribomustin and Prednisolon) and a drug, Zometa, which was to help stabilize my spine, in January 2005.

Despite the chemotherapy, another high-resolution MRI in June revealed even more destruction of my neck and back vertebrae. In October 2005, I was again admitted to the hospital for a bone marrow biopsy to learn if the disease had spread further.  The test results were negative so at least something was positive.  During the follow-up examination before my release, my doctor discovered that I have a faulty Aorta valve in my heart.  This diagnosis helps to explain the drowsiness and shortness of breath that I had been experiencing.

Therapy

Having the results of the MRI, I was sent to the University Clinic in Wuerzburg, Germany for further tests and possible treatment. To get a more information on the damage of my spinal column, my doctor at the university clinic sent me to a special orthopedic clinic also located in Wuerzburg. The damage turned out to be significant and the first thing the orthopedic doctors decided was to fit me with a hard plastic corset. Their worry was a possible fracture of the spinal column and the corset was designed to protect me rather than to provide support. I was also fitted with a neck brace which was to guard against whip lash and to keep me from turning my neck left and right to quickly. Surgery was on my damaged vertebrae was ruled out due to the fact there were too many affected areas.

At my next appointment to the university clinic, we decided that I would begin chemotherapy and massive doses of Cortisone, which would last 26 weeks as well as 18 sessions of radiation therapy of the most damaged vertebrae, fourth to the sixth, in my back. Needless to say, it was a long 26 weeks! The radiation treatment was hardest on me not so much from the treatment itself, but the driving to Wuerzburg every day for 18 days straight. We live a good hour from Wuerzburg, so it was an hour to, then the treatment, then an hour back home. Also the days when I had chemotherapy were long. The round trip time of two hours plus the four hours of chemo were just too much.

Following the chemo, Cortisone and radiation treatments, I returned every three months, later every six months, to be seen by the university doctors to see if the disease was progressing or if it was at a stand still. The Histiocytosis the thought was under control but because of the chemo and Cortisone therapies I had developed Osteoporosis. At the end of 2004 I stopped going to the clinic in Wuerzburg.

In an effort to get control of my pain, I was admitted to a special pain clinic in Bad Mergentheim, Germany. For the next six weeks, I received daily treatments designed to reduce the pain symptoms which included hot mud packs, massages, magnet therapy, lying in hot sand, water gymnastics and daily injections of medication to numb the nerves. These injections, 15 to 25 at one sitting, were left and right along my spinal column and along the right shoulder blade. The medication was supposed to give six to eight hours of relief, but for me the pain relief only lasted between two and four hours! My medication was also changed which was later instrumental in obtaining definite pain relief (more under the medication section).

After being released from the clinic I went home feeling much better. The pain I felt was under control and that I would be able to get back to leading a normal life. I wrong I was! The pain relief lasted only approximately three weeks and I was almost in the same condition as before going to the clinic.

In December 2004 it was time for another control MRI. This MRI revealed a new outbreak of the disease and after consulting with my family doctor, we decided than renewed chemo and possibly radiation therapy was needed. This time I did not return to the University Clinic in Wuerzburg but decided to go to the district hospital in Mellrichstadt (only 18 miles away). Additionally I selected this hospital due to the fact that the doctor treating me has some experience with Histiocytosis!

After a long consultation and the usual tests, well usual for me, we decided that I would begin another 26 week chemotherapy treatment starting in January, 2005. My doctor felt that the chemotherapy I received in the university clinic in 2002 was a "catch all" treatment not really designed to be effective in treating the Histiocytosis. The new therapy would be stronger and hopefully more effective. (Note: My first chemo and radiation treatments did not accompany strong side effects such has hair loss and vomiting). Now I was a little worried about side effects but there was nothing I could do, just grin and bear it!

I was to receive the chemo therapy (Vinblastin, Ribomustin, Prednisolon and Zometa) every three weeks giving the body to catch up. After the first treatment I developed a vein infection in my arm which was quite painful. Before the start of the second treatment I told the nurse about the infection so we switched arms. Beginning with the third bottle of chemicals (four altogether) my arm began to burn and we had to insert a needle into another vein to finish the treatment. After consulting the doctor it was determined that I would have to have a port or shunt surgically inserted under the skin. With this done, I had no further problems with the chemical infusions.

Following the 26 week therapy in late June, 2005 I underwent again a MRI examination to see the effect of the chemotherapy. The results were disappointing! The damage on the vertebrae had gotten worse instead of getting better or at least bringing the disease to a stand still. I don't know who was more disappointed, me or my doctor.

In order to plan the next steps in my therapy my doctor wanted to get a second opinion and an appointment was made for me in a large clinic in Nuernberg, Germany. Before I left for my appointment I discussed with my family doctor about the necessity of going to Nuernberg. We both, in the end thought that it would probably be a waste of time. And a waste of time it was. The doctor in Nuernberg told me what I have known all along: there was nothing he could do for me! I have had all the tests possible, have had Cortisone, chemical and radiation treatments. Because the disease is so rare there is no cure and the damage to my spine is irreversible.

I have applied to return to the pain clinic in Bad Mergentheim with my health insurance provider and am waiting to hear from them. Maybe a return visit will help ease the constant pain and give me a few days of pain relief.

Medication

Needless to say I have "run the gauntlet" with pain medication. At the very beginning I was taking oral opiates, such as Oxygesic, to help ease the pain. But as my body got used to the medication and the pain was getting worse I advanced to transdermal Morphine (Durogesic) patches.

The first prescription was the minimal dosage of 25µ. Within four hours I was feeling the side effects of the morphine. At first I began sweating profusely then the cold sweats and then vomiting. After a call to my family doctor, I was given a medication (drops) which would ease the side effects until my body became used to the morphine. It wasn't long until the dosage had to be increased to 50µ, the 75µ until finally I was taking the largest dosage of Durogesic 100µ.

My pain was still not under control and along with the morphine patches, my doctor and I were trying different combinations of oral pain medication. In addition to the patches, oral pain medication, I was also taking an anti-depressant, Doxepine which helped me get to sleep at nights.

During my visit to the pain clinic in Bad Mergentheim I was slowly taken off of the morphine patch Durogesic and later switched to Transtec patches. The Transtec patches were basically the same as the morphine patch, but act differently in association with the nerve endings. Also, I was prescribed a new drug "Lyrica", an anti-epileptic medication. I am not epileptic but the new drug, is also used to "calm" the nerve cells. Additionally, I was taking Katadolon, also a strong pain medication.

During my second chemical therapy I had to quite using the Transtec patches because the patches were causing severe skin irritation. So it was back to the morphine patch Durogesic which did not conflict with the chemo.

Currently, I am taking Durogesic morphine patch, Lyrica and Katadolon to provide some pain relief. In addition I take Doxepin, an antidepressant, to help me get to sleep at nights. The Doxepin also helps keep the level of the pain medication at a higher level during the night. I have only started using the Palladon the first week of August and unfortunately, the pain relief is not very good.

Continuing on.........

As I last left you, I was waiting on the doctors from my health insurance to approve my application to go again to the special pain clinic where I was in 2004. Much to my surprise it was approved without any question or delay! I contacted the clinic and made arrangements to be admitted on the 15th of May, which would give me time to finish up with my soccer tournament site (www.nizza-cup.de).

I took the train to Bad Mergentheim, where the clinic is located, with a small suitcase and backpack. I had sent my two larger suitcases with the post office, which made it easier to travel. Things had not changed much at the clinic "Arkauwald", and I was glad to learn that I would have the same doctor and the same psychologist for my stay. Since they already knew what my problems were, it was easier to start my therapies. I became a nice large room at one of the back corners of the clinic, which as it turned out, was very quiet, near the therapy area and most importantly, near the dining area.

I started the very next day with physical therapies to help get the tightness out of the muscles in my back, shoulders and arms. If you are fighting continuous pain as I am, then you change your posture to ease the discomfort as much as possible. After a time, the muscles become hardened even more and contribute more pain and discomfort. I took muscles relaxers, layed in hot sand and clay and received massages. After a time, I could tell that some of the muscle pain I was having was much better. Also, we began with injections (20 - 25 times!) of a strong muscle relaxer direct to the left and right of my spine and along the right shoulder blade. We also began experimenting with my medication, trying to find the right mix which would help ease the pain. The medication which worked back in 2004 did not work this time and in the end I was not wearing the morphine (40 times stronger) patch anymore, but was taking an oral medication 80 times stronger than morphine!

After six weeks I decided that it was time to go home. I did learn from my doctor, that a colleague of his had started a "day clinic" in a large hospital in Schweinfurt (30 min. drive) and that I should contact her to discuss the possibility of continuing my therapy.

I took his advice and made an appointment in Schweinfurt for the beginning of August. After a physical examination and a review of my MRI's and therapy history, my new doctor said that she did not agree with the medication I was taking because it only gave me short-term pain relief. She prescribed me a new medication, Palladon, which she believed would work better than that which I had been taking. It was also decided that after four weeks of taking Palladon I would again see her to see how the medication was working and eventually changing the dosage. Also we discussed the pain therapy offered by the hospital. It is a day clinic four weeks long, Monday thru Friday, 8 a.m. till 2 p.m., in groups of eight patients combining physical and physiological therapy as well as pain medication. Driving back and forth from Steinach and Schweinfurt is practically impossible for me and using public transportation would not be much better. I would have the leave the house at 5:30 a.m. to be there by 8:00 a.m.

At this time, we have increased the dosage of my medication four times and I am trying to figure out how I could make it work to attend the clinic in Schweinfurt. If it does not work out, then I will have to try and find a new clinic for next year. I do not believe that I will go back to the clinic in Bad Mergentheim again because I could see the cut backs which had been made due to the changes in the German heath system. Being afflicted with a rare disease sure is depressing!

The Palladon was not helping my pain and my family doctor and I have increased the dosage of my medication four times! What I was not told was that Palladon causes problems with urinating and bowel movements. It got so bad that the time in between me having to urinate was 25 hours! The bowel movements were even longer. On a Sunday evening after being able to finally urinate I saved a sample which my wife took to our family doctor who lives in town. A short time later they called and said I needed to get to the hospital right away and wanted to call an ambulance for me! I opted out for the ambulance but instead my wife drove me to the hospital.

I was admitted immediately and after the initial examination the doctors discovered a fist-sized cyst in my left buttocks which would have to be surgically removed. I was then scheduled for surgery first thing on Monday morning. I was another week in the hospital. Needless to say I quit taking Palladon that evening and have not been back to the pain clinic in Schweinfurt since!

If you are using or have used Zometa, please read my report on Osteonecrosis!