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Alert Update, Sheba Medical Center-Tel Hashomer, Israel There are several exciting breakthroughs and introductions of new protocols defining the critical role of Sheba in the health care of Israel and in medical treatment that will impact patients from around the world. Breakthrough treatment of Fabry's Disease is attempted with promising results at the Sheba Medical Center. Fabry's Disease is an extremely rare, genetically transmitted disease. Fabry's Disease is diagnosed in one out of 40,000 deliveries. Carriers of the disease have a deficiency of a particular enzyme. This deficiency is characterized by abnormal accumulation of waste (neutral glycolipids) in blood vessel wall cells. Thus the disease is termed an "Accumulation Disease." This accumulation of waste causes increasing damage to various body organs. Life expectancy for patients with Fabry's Disease is limited. Most of those afflicted will die in their fifties. Fabry's Disease manifests only in men. Men carry and/or develop Fabry's Disease, while women are carriers of the gene aberration. The first symptoms of Fabry's Disease manifest during childhood or adolescence, appearing as rash-like spots on the skin (1-5 mm diameter), mainly in the pelvic area. Another manifestation is a low diaphoresis level, and in some cases complete lack of diaphoresis or perspiration. Another manifestation is elongation of the eye (cornea, retina, lens and fundus). Other symptoms that manifest are neurological. A neurological symptom, characteristic of the disease, is pain accompanied by a burning sensation at the tips of the extremities. The level of this pain is a reflection of the patient's mental state (stress, pressure, or fatigue). There have been suicides documented among Fabry's Disease patients. Heart diseases such as cardiac insufficiency, blood vessel blockage, cardiac arrhythmia, and stroke are characteristic of Fabry's Disease. In the later stages of the disease renal damage also occurs and is characterized by high levels of albumen in the urine, uremia, and by advanced renal failure. In addition to all of the above, symptoms resulting from damage to the gastric system also manifest. Pain is often experienced after taking nourishment, the patient will experience bouts of nausea and diarrhea, and pancreatic function is lowered. For the first time in Israel, innovative Enzyme Replacement Therapy is offered at the Nephrology Department of the Sheba Medical Center. Professor E. Holtzmann, the Director of the Nephrology and Hypertension Department, reports that patients have experienced no side effects during the course of treatment. Twice a week the patients receive treatment at the Nephrology and Hypertension Department. Professor Holtzmann reports that "patients receiving treatment in our department have shown marked improvement, and have begun to perspire and the palms of their hands are damp." According to Professor Holtzmann, there are approximately 30 patients with Fabry's Disease in Israel today. However, only two of them are being treated at the Sheba Medical Center. Professor Holtzmann says "because Fabry's Disease is a genetically transmitted fatal disease, Sheba plans to invite all branches of the patient's family to the Center and perform genetic screening. Thus we will generate heightened awareness of the disease within the family and be able to identify other family members with a potential for having the disease." Innovative "Bi-ventricular Cardiac Pacemaker" for the Treatment of Heart Failure. Frontline technology in the field of Cardiac Pacemakers is offered at Sheba Medical Center. Recently published medical research supports the use of Bi-ventricular Cardiac Pacemakers in treatment of heart failure. The prestigious medical publication, The New Englande", recently published a research report supporting the benefits of innovative cardiac pacemakers that regulate the left ventricle in cases of heart failure. A number of other research projects presented recently at international conferences also support this approach. For the past two years innovative treatment utilizing this new Bi-ventricular Cardiac Pacemaker has been offered at the Sheba Medical Center, and has proven successful. The standard cardiac pacemaker is an electrical device subcutaneous implanted
in the chest or abdominal area and connected by means of two electrodes usually
implanted intravenously in the heart cavity. The new Bi-ventricular Cardiac Pacemaker
has three electrodes, one of which regulates the left ventricle via the (ventricular)
venous system. Dr. Michael Licks, Head of the ICD Service at the Sheba Medical Center, says that when offering treatment with this innovative cardiac pacemaker Sheba offer patients a quality of life that is unattainable with any other treatment modality. Dr. Licks adds that "patient follow up is carried out to ascertain whether their quality of life has indeed been improved." Looking back on the past two years since this procedure was initiated; the procedure has certainly proven itself. A sixty one year old patient, Mr. Shimon Farrago, had a new cardiac pacemaker
implanted in July 2000. Mr. Farrago says, "Today I am able to breathe freely;
I can walk as much as I want, and I can walk up a number of flights of stairs
without having to stop to rest. Before the procedure I felt like half a man, like
an old rag." He adds that "Dr. Licks from the Sheba Medical Center suggested
implantation of the new cardiac pacemaker as the only answer to my condition,
and since the procedure my condition has indeed improved immeasurably." In a complex and innovative surgical procedure, surgeons at Sheba Medical Center have succeeded in removing an enormous growth from a small child's body. The cystic growth weighed five kilograms, and is certainly one of the largest documented growths surgically removed from a child's body. The seven-year-old child was referred to the Pediatric Surgery Department with a diagnosis of extreme abdominal swelling due to an abdominal cyst. The cyst had been observed during ultra sound scanning performed prior to birth. The baby underwent extensive evaluation following birth, with no confirmed diagnosis. During the past seven years the child was hospitalized in several health centers and underwent repeated inconclusive diagnostic testing. Due to the size of the growth surgeons were unable to make a decision regarding surgery and the child was sent repeatedly for evaluation to try and establish what would be the most effective surgical procedure. Following the last round of extensive diagnostic testing, the child was referred
to the Pediatric Surgery Department at Sheba Medical Center. Surgeons at Sheba
excised the growth in a complex surgical procedure. The team was headed by Dr.
Itamar Avigad, Director of the Pediatric Surgery Department, and included Dr.
Danny Shinhar and anesthesiologist Dr. Ilan Kedar. After having one fifth of his
total body weight removed the child is now feeling well and recuperating rapidly.
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