HEIDE, scleroderma, Sjogren's syndrome, osteoarthritis

It was in 1941 in Austria as a three year old that I became very ill. The biggest concern was my kidneys. I was taken to two hospitals where the doctors expressed no hope for my survival. I was moved a third time. My parents heard of a physician in Salzburg who was willing to try against the odds. Dr. Thomas McPherson Brown, whose patient I became in April 1988, suggested that while I overcame my problems in 1941, something very likely stayed behind. My pre-teen years were marred by long bouts with horseness. I had a doctor's certificate exempting me from oral examinations in school. By the age of fourteen, my tonsils were removed and the hoarseness disappeared. In 1960, while preparing to move to the United States, I fainted twice. The stress of moving was blamed. In 1961 in Huntsville, Alabama, I was diagnosed with hypothyroidism. (I have been on 0.1 Synthroid since then.)

In 1963 and 1966 I had uneventful pregnancies and both deliveries were normal. I remember though, that it seemed to take me longer to recover. In 1970 I was diagnosed with mumps even though I had mumps as a child. The glands on the right side were very swollen and I had a high fever for days. Dr. Brown suggested that this very likely had not been mumps but an infection of the parotid gland. Recovery was slow. In 1971, I was diagnosed with viral pneumonia and stayed in the hospital for nine days. It took me months to get over this. In 1972, all five fingers on my left hand started to tingle. Six months later, the right pupil was very much enlarged and did not react to light. The right knee jerk and the ankle jerks were absent with the left knee jerk weak. I was hospitalized for extensive testing. No obvious reason for my problem was found. A diagnosis of Adie's syndrome was given. Along with numerous tests I had a myelogram. Recovery was slow, and for weeks I suffered from sudden, severe headaches and nausea which disappeared instantly upon lying down. This would come back later to haunt me.

The same year, I consulted a leading neurologist in Nashville, Tennessee. After careful examination, he suggested that these problems were frequently seen in immigrants. They came, he said, from socio-economic inferior backgrounds (not true in my case), especially with women who marry above their leve. Imaginary physical and psychological problems start. After enquiring about my husband's occupation (engineer at NASA), my fate was sealed. He told me to write to him in six weeks. He was sure that by that time my symptoms would have disappeared.

By 1978 the tingling in my fingers had progressed to numbness. Once again, I tried to find the cause. This time I was referred to a neurosurgeon at the University of Alabama in Birmingham. The electro-diagnostic study done there showed no sensory potential over the finger-wrist segment in the left median and ulnar nerves. I was advised to proceed with the operation for neurolysis and decompression of the left median nerve at the wrist. The surgery revealed that the nerve was healthy and not trapped, and to this day there is no change. The fingers are still numb.

In 1975, I had a D&C and in 1976 a hysterectomy. I was told that fibroid tumors are the cause for the prolonged bleeding (up to 18 days) every month. No fibroid tumors were found and the uterus was removed. Recovery was slow. (0.625 Premarin for 25 days every month was prescribed then and still is today.) Also in 1976 I flew to Austria to attend my father's funeral. I became very ill with what was diagnosed as the flu. I was able to return to Alabama after a three week stay. I took me weeks to recover. By then I had intermittent dry, bothersome eyes and a dry mouth. My dentist voiced his concern about my inflamed, bleeding gums.

In 1983 my mother died suddenly and tragically. Her death was a tremendous shock to me. I flew to Austria to attend her funeral and to help dissolve her estate. I stayed four weeks and came back totally exhausted. By the fall of 1983 changes appeared on my upper body, arms, neck and face. The dermatologist I consulted over a period of several months diagnosed this as sun damage and the rash in the face as an allergy to the toothpaste. I was given medication to be applied and after several medication changes, I gave up since nothing helped. In 1985 I agreed to gum surgery to help my inflamed, bleeding gums. It took me a long time to get over this with no positive change. That same year I actively started to find out what was wrong with me. At my yearly physical, I was diagnosed with hypertension and medication was prescribed. (Hytrin, Capoten).

In 1986 I went to see an opthalmologist. The agony and discomfort with my eyes were apparent. He diagnosed keratonconjunctivities sicca and told me to use moisture drops as often as every 30 minutes. This made my problem even worse. Unfortunately, he failed to suggest that this condition may be a sign of a much larger problem. Two agonizing years went by. A friend told me to read an article in a magazine. She said that it sounded like everything I have been complaining about. The article was about Sjogren's Syndrome, and I knew then that I had found the root of my problems. I sent for more information to the Sjogren's Syndrome Foundation. It became apparent to me that I have scleroderma also. I was very familiar with scleroderma since I had a very close friend who died from this disease. Through her I knew that there was no viable treatment available for this illness.

A rheumatologist in Nashville, Tennessee made the diagnosis in March 1988. My lab work revealed that I have both anticentromere (1:1280) and antitopoisomerase I (SCL-70). Anticentromere is associated with the CREST syndrome, while antitopoisomerase 1 points to diffuse cutaneous systemic sclerosis. I understand that finding this combination in a patient is unusual at best. By this time, nothing seemed unusual to me as I knew for certain that I was very ill. Extreme fatigue, joint pain, daily low grade fever (99.8), extremely dry, gritty eyes, dry mouth, swollen glands, severe coughing attacks that left me without a voice for several minutes after each attack have taken its toll. I was so sick that there was no way I could have made the 2 hour trip to Nashville by myself.

Upon returning from Nashville, I was told by a friend to contact Kathryn Loftis who had overcome scleroderma by following the antibiotic therapy. Reluctantly I called her. What she told me convinced me that she was handing me a chance, a ray of hope I had not felt for quite some time. Best of all was that this therapy seemed to be safe and the risk minimal. In April 1988 I became Dr. Brown's patient. This was an experience I will never forget. He was the most caring, understanding physician I have ever met. I stayed at the National Hospital for 1 week. Through my daily, long conversations with Dr. Brown, he convinced me that I would get well provided there had been no permanent damage yet. He urged me to understand, though, that the recovery would take a long time and lots of patience. He prescribed 500mg tetracycline and 1mg prednisone on Mondays Wednesdays and Fridays, a 900mg clindamycin IV once every two weeks, and daily vitamin C 1000mg, vitamin E 400 units and 1 Therogran M.

My symptoms intensified greatly. But always, I took this as a positive sign that the medication was attacking what it is supposed to attack in my body. In October 1988 I returned to the National Hospital as Dr. Oliver's patient. I saw Dr. Brown one last time. He wrote my prescriptions for the next 6 months, adding a course of 5 IVs every 3 months to combat the severe increase in Raynaud's activity. It took 2 years and 7 months before I realized any measurable improvement. Through these trying times, I am fortunate to have a family physician here in Huntsville who has reluctantly gone along with the treatment option I have chosen.

By 1990 my Raynaud's problems had intensified to the point where my left index finger stayed dark much of the time. I was referred to an anesthesiologist for stellate ganglion blocks. I had three blocks each year, March 1990, winter 1991 and winter 1992. Since then, while I still have Raynaud's symptoms, these have eased to the point where I can deal with this problem without intervention.

Since 1986 I have had intermittent problems with headaches and a general ill-feeling which could come every 6 to 8 weeks. It always started in the early afternoon and nothing would help but to lie down and sleep until the next morning. By 1991, these bouts came closer together, appearing every few days. These episodes were beginning to worry me greatly. In July 1991, I was cleaning the tile on the bathroom floor by squatting down and applying pressure with my hand. In an instant I went from feeling well to being violently ill with an enormous headache and throwing up. The effect of this was overwhelming. All symptoms disappeared immediately upon lying down. Instantly, it reminded me of my problems after the myelogram. Since the symptoms persisted upon getting up, I was hospitalized and a neurologist was called in. A cisternogram, lumbar puncture, CAT scan and other tests did not reveal the cause. It was determined that spinal fluid was leaking somewhere. The pressure I had put upon my system by squatting down and applying pressure with my hand by cleaning the floor may have reopened the puncture of the myelogram years ago. I was sent home with orders of total bed rest.

Three and a half months went by where I improved to the point of being able to be upright several hours at a time, provided I did absolutely nothing but sit. I had to discontinue the IVs of clindamycin after it became apparent to me that the infusion provided enough pressure to my system, causing the leak to open up, resulting in instant, severe headaches and ill-feeling. In October 1991, the neurologist decided to order a procedure called blood patch which was performed by the anaesthesiologist. 10cc of my own blood was infused in the epidural space. The blood forms a clot at the leakage site. Collagen deposition and scar formation will follow within a few weeks. It was an immediate success, and I have been free of headaches since. Four weeks after this procedure, I resumed the clindamycin IVs without any problems.

In 1990 and 1991, I had two episodes of chills and fever each year with no other symptoms or ill feeling. The chills last 2 to 3 hours with the fever as high as 104 degrees. In 1992 these episodes increased to four and in 1993 to nineteen. In the summer of 1993 I was sent to a physician specializing in infectious diseases. Extensive testing was ordered. No cause was found. To me it feels as if the immune system is getting rid of something in my body that should not be there. I always feel so much better after each episode and much more energetic. I have improved a lot this year and feel more stable. I suspect that these chills and fever episodes have something to do with this, too.

I have been following the antibiotic therapy now for five and half years. For the last four months I have continuously felt much improved. For years I tried to shield my dry and miserable feeling eyes from any wind without much relief. The thought of sitting at the beach with constant strong winds blowing was out of the question. The mere thought of this would throw me into total panic. Today I can read, write and enjoy the wonders of nature again. I have travelled the long, bumpy road back for quite a few miles now. I could not have done it without the support and understanding of my family and friends. To my dear friends in and out of Grissom High School who have persistently pointed me in the right direction, many thanks for caring and for your support. My biggest thanks goes to my husband, Erich, who undying love, support and extreme patience have provided me the foundation to travel the long road back. He never wavered, and therefore, neither could I.

Update 1994 to early 1998

When I was asked in early 1994 to write about my experience with the antibiotic therapy related to my rheumatic problems, I was convinced then that one day I would go into remission. While this day has not come just yet, I am still convinced that this therapy is the correct treatment at this time. I have followed it for 10 years now. While the improvement is slow, nevertheless, it is significant.

To continue my accounting of the twists and turns I have taken, I have to go back to the chills and fever episodes. These started in 1990. By 1994, I had 13 episodes, in 1995 I recorded 16, in 1996 - 4, in 1997 - 4, and none so far in 1998 (it is now early May) for a total of 64 episodes. In Oct. 1995, I was referred to an internist here in Huntsville who has the reputation of solving tough cases. He contacted the infectious disease doctor I had seen in 1993, and their joint opinion was that I should discontinue IV and oral antibiotics to exclude the possibility of drug fever. I knew that this was not the cause, but went along with it for one month. I was advised to elevate the top mattress of my bed at least 8 inches and to take 20 mg Prilosec daily. Stool studies such as C.Difficile were ordered because of the chronic antibiotic usage along with echocardiogram, pulmonary function studies, chest X-rays and lab work. All tests were normal. The final diagnosis was gastroesophageal reflux secondary to Scleroderma and CREST syndrome with aspiration pneumonia resulting in chills and fever. I was advised that in the event the fever episodes persist, to undertake further testing such as gastroscopy metric studies as well as PH studies to consider possible surgical intervention, but only if oral medication, diet and bed elevation do no longer control the problem. After taking Prilosec for 2 years, my medication was changed to 2 Zantac 150 mg each twice a day. I have not seen a gastroenterologist yet, but have the best intentions to do so.

In 1994, I was forced to deal with the deterioration of cartilage between the joints of the right and left great toes. MP joint fusion of the right toe and 6 months later of the left toe was performed. Before the surgery, I was advised that in case the screws presented any problems, they could be removed. After about 8 months following each surgery bone spurs formed at the bottom of each fused joint. This was very painful, and walking was once again very difficult. In June 1996, the screws were removed as well as the bone spurs. No more problems have occurred since then.

In Sept. 1996, while on vacation in Austria, I fractured the neck of the femur - right hip. While getting out of the car, I was not aware that my right foot was in my purse strap. As soon as I stepped out of the car, I landed on my right side. I spent the next 12 days in the hospital in Salzburg where a plate was inserted to stabilize the hip and facilitate the healing process. By March 1997, despite physical therapy, walking was cumbersome and again painful. Around the plate area an infection and swelling had formed. I was given 2 steroid shots, which resulted in good improvement. This was followed up by 2 more shots about 4 weeks later. Walking never was without problems no matter how hard I tried.

In Jan. 1997, I lost my sense of taste. Sweets tasted like cardboard might taste and everything else was diminished or strange tasting. Despite medication (Nystatin) and assurances that this could easily be rectified, nothing changed. Three months went by when I was advised to change to Kid's Crest toothpaste. Within one day, my taste came back.

In Oct. 1997, I had surgery on my right thumb to remove very painful calcium deposits, which have become infected; and in March 1998 an arthroplasty on the CMC joint - right thumb was performed.

While I have felt that over the past 3 years or so I did not make much progress with my rheumatic problems, I knew for certain that I had taken a turn for the worse in 1997. By winter of 1997, some of my symptoms, such as Raynaud's activity, had increased and my blood pressure, which had returned to normal in 1992, was very much elevated again. Red spots (telangiectasia) appeared on my face in great numbers. In addition, I had excessive, drenching day sweats which I have not had before.

In January 1998, it was decided to remove the plate from my thigh/hip. While I have had several surgeries over the last 3 years and reacted to these very predictably and about the same, the reaction to this surgery was quite a surprise. After the plate was removed, I immediately felt a sense of well being with increased energy. This was very unexpected. It took me a while to realize that some of the increased symptoms had eased up and that the drenching day sweats had disappeared. When this unexpected improvement persisted, I started to wonder if the screws/plate could have been the reason for the downward spiral in my recovery. I was told that this could be a possibility.

While feeling better, I still faced the fact that I needed to find a rheumatologist with extensive experience with the antibiotic therapy. My search ended when I heard of Dr. Al Robert Franco of Riverside, California. He came highly recommended by Pat Ganger. I decided to attend the patient conference April 18 and 19, 1998 and had an appointment with Dr. Franco April 20, 1998. The ANA which was 1/640 just 7 months prior was 4 times as high - 1/2560. In the 10 years I have been tracking my lab work, it never was that high. He changed my medicine from 100 mg Doxycycline Mo-Wed-Fr and IV of Clindamycin 900 mg once a month to 100 mg Minocin twice a day Mo-Wed-Fr and increased the IV to 1200 mg Cleocin once a month. (RF 52.9; CRP (3.0 -; Sjogren's SSA 96.9)

While reading through my story which is given above, I am struck by the repeated lines of "Recovery was slow" and "It took me a long time to get over this." This, I am glad to say, is a thing of the past. Through all the surgeries I have had since 1994 (7 total), recovery and healing were right on track which is quite a change from before I started the antibiotic therapy. Although I do know and appreciate the value of having a first rate surgeon (Thanks, Dr. Horn. You did good!!), I do realize that I must have sufficiently improved in order for my system to respond to these extra demands in such a great fashion. It may be too soon to tell if the hardware which I carried for over 3 years may have been the cause for some of my increased problems. I do hope, however, that this had something to do with it. I am confident that Dr. Franco can bring me back on track and that things will be getting better.

Heide Engler
Huntsville, Alabama - (256) 881-1926

You can reach me at EEHE@aol.com


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