Thursday, July 1, 2010

Fascinomas

Even after thirty years in the ER, the world's oldest ER doc still encounters rare and challenging diseases. A 30 year old Cambodian man with a foot drop clearly wasn't having a stroke. I found no evidence of trauma or metabolic disorders. A nodule lateral to his knee seemed suspicious. A biopsy of this nodule revealed the diagnosis, leprosy. The daughter of a nurse and close friend from the ER, had been in South America during one semester in college. On a trip home she showed me a sore on her foot. Treatment for a bacterial infection proved futile. The diagnosis was leishmaniasis, a parasitic disease spread by sand flea bites in endemic areas.

This week I went in to examine a woman in her seventies with a chief complaint of weakness and just not acting like "herself". Her family was helpful in giving me a sense of her usual health and the medications she takes were in a "bag-o-meds". She was weak, short of breath and her capillary sugar was 400 +. Lab tests showed severe metabolic acidosis. The pH of human blood is 7.4. This patient's pH was 6.67. One of my young partners commented that she should have been dead. She was able to follow instructions and even speak. Her medications included metformin. This is a commonly used medication for type II diabetes. It has an uncommon side effect of causing lactic acidosis. A lactic acid determination confirmed the diagnosis. The normal lactic acid level is < 2. A level of 5 is considered critical. My patient's level was 15. I began appropriate therapy and admitted her to the ICU. My friend Dr L, the intensivist was incredulous when I gave him her lab results. He also felt that she should not be among the living with a pH of 6.67. The patient rapidly improved.

Patients who suffer a cardiac arrest outside the hospital rarely survive. Unless bystander CPR is immediately initiated and advanced life support including entubation, continued CPR and specific medications given within 15 minutes, the patient's brain will suffer irreversible damage. A recent innovation is cooling the patient with cold IV solutions, and ice packs to slow the metabolism and try and preserve brain function. Patient as freeze pop. Sadly the outcome is rarely a full recovery.

A recent 62 year man was brought in to the ER after an unwitnessed cardiopulmomary arrest. His initial rythym on the monitor was asystole, no cardiac electrical activity. The ACLS protocol was begun and continued on route. A weak pulse was obtained, just as he cleared the outer ER doors. A contiunous IV drip of isoprel maintained a reasonable pulse and blood pressure. The chest x-ray showed fluid and a questionable mass in his right lung. The lab results showed a sodium of 118. The normal sodium (NA) is 140. This gentleman died 2 days later because of his anoxic brain injury. His brain was deprived of oxygen for too long.

The low sodium was a mystery but I had a theory. Many cancers, especially lung tumors secrete hormones. ADH (anti-diuretic hormone) is normally secreted during times of dehydration so that the kidneys with preserve water. SIADH (syndrome of inappropriate ADH) occurs when the hormone is produced in the normal water balance state. As water is retained, the sodium in the blood is slowly lowered. When the level gets too low, seizures and cardiac arrythymias may occur.

I arrived home that morning and my wife asked "how did the night go". My response was "no one died". Fascinomas keep me curious and focused. 30 years, 20-30 patients per shift, currently 12 shifts per month (formerly 16-18 shifts per month), you do the math. The challenge of unusual and rare diseases and injuries keep me on my toes and prevent my job from becoming routine. I will continue to post rare-anomas and fascinomas in future blogs.

1 comment:

  1. enjoyed your fascinomas, check out my book "Fascinomas" on Amazon, just published with35 unusual cases.

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