The hematological profile among the internists of the area read the necrology of the patient in the local newspaper in December, 1999. He wondered what had happened to his local and political neighbor. In the post-Christmas sales, he met the widow and the son. They also wondered and wished information and discussion. Thee profile requested the patient chart of his department, 7 cm thick. Records from other departments were not requested.
The patient was born 1922. His production during 77 years was impressive. The profile indeed suspected that the patient was a case of advanced workaholism. The charts suggested solid sufferings during his last 25 years. Pain and anxiety had, nevertheless, not impaired production.
The patient history contained some traffic accidents, joint disorders, episodes of supraventricular tachycardia. In the period 1975-80, he developed a disabling fatigue. He could fall asleep while talking business over the phone. Then he woke up, phone in hand, notes flooded with saliva.
Neurologists informed the patient that he suffered from "narcolepsy". However, it is not known whether he was prescribed amphetamine or like-acting drugs.
About 1977, after the "narcolepsy" episode, the patient visited an internist, who found mild anemia, decreased basal secretion of hydrochloric acid in the stomach, and a poor response to stimulation of gastric secretion by pentagastrin. Ten years later, serum gastrin was elevated, about 600 ng/L, reference range 0-130. The body stores of iron were also poor.
The patient was relieved by parenteral hydroxocobalamin (Behepan). However, his fatigue returned 3-4 weeks after each injection. Nevertheless, his internist stuck to the official recommendations of the time, maintenance therapy with parenteral hydroxocobalamin 1 mg each third or fourth month.
The hematological profile met the patient in the labor party meeting of their quarter in the beginning of the 1980´ies. In the coffee break, the patient lamented over devastating fatigue during the last two months of the interval between hydroxocobalamin injections. The profile suggested that the patient should ask his internist for oral high-dose cyanocobalamin instead of parenteral therapy. He received Behepan tablets, 2 mg daily, and appeared satisfied and worked in his usual tempo.
In June 1993, the profile met the patient in the lobby of the hospital. The patient was in a bad shape, walked with short trotting steps, gait range 10-30 meters on flat ground. He had a pathological methionine load with a 4-hour peak of 57 umol/L, reference range <40.
During the 18 months that followed, the patient had intensive treatment with cobalamin, folic acid, and pyridoxin. The gait range improved to 200-300 meters on level ground. A note in the chart stated that the hemoglobin value had increased 30 g/L within the reference range. Overt iron deficiency developed and was treated with parenteral iron preparations. The methionine load sas normalized. The observations were interpreted as a partial response to therapy.
However, the patient tapered slowly off, became chair-ridden, stopped eating. In the autopsy protocol four years later, it is stated that the patient died from familial amyloidosis with polyneuropathy. A fighter had finished.
Updated juli 13, 2000