Clinical History:

The summer of '86 was hot and dry on the northern plains. Fall was milder than usual. But strange events made everyone uneasy. Migrating birds began flying south much earlier than normal. Arctic snowy owls were seen for the first time anyone could remember. Animals began growing long coats of fur. Then in mid-November an arctic storm dumped large amounts of snow that drifted deep with the howling wind, the temperature dropped to -20 F. and snow continued to fall for a month. Mid-December brought a brief respite of warmer temperatures, and the snow began to melt. However, new storms came and temperatures plummeted, causing the melting snow to freeze into solid sheets of ice that prevented animals from grazing and people from traveling. A blizzard in mid-January lasted 3 days, with temperatures going as low as -60 F. After 100 days of extreme cold, a Chinook wind in February brought warming that melted the snow and ice to reveal thousands of rotting animal carcasses across the land.

Members of the Crow Nation (Apsáalooke) have been driven from the lands they occupied for thousands of years to places that newly arrived homesteaders and ranchers do not want. No native Americans have citizenship rights. The buffalo, a major traditional food source, are nearly extinct. A family of four has a meager existence even in the best of times on land near the Rosebud River. Now, with the onset of a severe winter, they are confined to a sod hut with dwindling supplies consisting of 20 kg of white flour, 20 kg of corn meal, and 5 kg of dried apples.

The infant boy had been born at 38 weeks with weight of 2400 gm (5.3 lb). Now at 12 weeks of age, the baby is only 3600 gm (8 lb) because mother can produce little milk. The baby dies a week later in early December.

Questions:

1. What problem is suggested by these findings?

Marasmus is due to total calorie deprivation (proteins, carbohydrates, fats) with severe wasting (<60% of ideal body weight for age).

2. How could this situation have been prevented?

Sufficient daily caloric intake is required. Infants and children are at greatest risk.

Further History:

The 5-year-old girl weighs 16 kg (35 lb) and begins to develop abdominal enlargement with swollen feet. She becomes weaker with diminished muscle strength. Her normally dark hair appears lighter at the roots. She has areas of flaking skin. Representative serum chemistry values would show total protein 4.9 g/dL and albumin 2.8 g/dL. She develops a fever with productive cough. In early January she leaves the hut to search for firewood, but she cannot see well in the dark and gets lost. She is found frozen to death the next morning.

Questions:

1. What problems are suggested by these findings?

The general features are those of kwashiorkor, or protein calorie deficiency, leading to fatty liver, ascites, and edema. The pulmonary infection and night blindness are features of vitamin A deficiency.

2. How could this situation have been prevented?

Throughout human history, and particularly among persons with a subsistence existence, good sources of dietary protein have been difficult to consistently obtain. This family mainly had carbohydrates in their meager food sources.

3. What are the mechanisms by which these findings are produced?

Dietary protein is required for maintenance of liver function and circulating blood proteins. Growing children need protein. Vitamin A helps to maintain epithelia.

Further History:

By late January they are boiling shoes to eat. The 30-year-old father is becoming weaker. He develops soft and inflamed gingiva with loose teeth, swollen and tender joints, myalgias, poor wound healing following even minor injuries, and purpuric areas on his skin. "Bumps" on his skin represent hyperkeratotic hair follicles with surrounding hemorrhage. Representative laboratory findings would include hemoglobin 8 g/dL, hematocrit 24.2%, MCV 84 fL, platelet count 249,000/microliter, prothrombin time 12 sec (control 12.2), and partial thromboplastin time 25 sec (control 26). He develops a high fever with shaking chills and dies in late January.

Questions:

1. What specific nutritional deficiency is suggested by these findings?

The findings are indicative of vitamin C (ascorbic acid) deficiency with scurvy.

2. How could this situation have been prevented?

The diet needs to include fresh fruits and vegetables that contain vitamin C. Since humans cannot synthesize or store vitamin C, a constant and sufficient dietary source is necessary.

In history, scurvy is illustrated by European voyages of exploration. As the voyages of exploration became longer, ships' crews went without fresh food, including fruits and vegetables, for much longer times. Portuguese, Dutch, and Arab traders were the first to recognize the importance of diet, particularly citrus fruits, in preventing illness on long voyages. Captain James Cook knew that ships of the Dutch East India Company included plenty of sauerkraut (pickled cabbage) in their stores, and their crews did not suffer from scurvy. He included this item in his stores for his voyages of discovery to the Pacific, and his men did not develop scurvy. Scurvy remained prevalent in the English Navy, however, until 1795 when Dr. James Lind, a Scottish physician, recommended that lime juice be issued to all British naval vessels (the sailors subsequently were called "limeys").

3. What is the mechanism by which the pathologic findings are produced?

Vitamin C activates enzymes that hydroxylate procollagen to form collagen. Collagen a principle building block of connective tissues. Poor formation of collagen leads to increased capillary fragility and bleeding into soft tissues. The connective tissues of the gums in the mouth and the joints are also affected. Collagen forms the matrix of bone, so that growing bones in children have weakened matrix that produces deformities (widened epiphysis). Wound healing is dependent upon collagen formation.

4. What laboratory findings may be present?

A CBC will show a moderate degree of anemia with red blood cells of normal size, or reduced size if iron absorbtion is affected by the vitamin C deficiency. Tests of the coagulation mechanism are normal (PT, PTT, platelet count). Vitamin C is not ordinarily measured, as levels of water soluble vitamins are subject to dietary intake and can be variable.

5. In the modern world, who is at risk for these problems?

A full blown case of scurvy is rare since diets contain vitamin supplementation in many products. However, any form of stress increases the requirement for vitamin C, particularly any form of inflammation. Pregnancy also increases the requirement for vitamin C, as does surgery. Vitamin C absorbtion is decreased with achlorhydria and diarrhea.

6. What are good dietary sources?

In a typical American diet, over 90% of vitamin C comes from fruits and vegetables. The heat from cooking diminishes the amount of vitamin C available in foods, so foods prepared by steaming, simmering, or microwaving with minimal water (vitamin C is water soluble) are preferred, as are fruits and veggies served raw. Veggies that are good sources include broccoli, cabbage, peas, potatos (with skins), and tomatos.

Further History:

By early February the 23-year-old mother has a BMI of 15. She has developed erythema with vesiculation and focal desquamation of her hands. She has nausea, vomiting, and diarrhea. When the thaw comes, relatives able to travel with a surviving horse arrive to find her disoriented, confused, and talking incoherently. She cannot carry out activities of daily living -- even if there were "living" at this point.

Questions:

1. What problem is suggested by these findings?

The findings of dermatitis, diarrhea, and dementia suggest niacin deficiency.

2. What are good dietary sources?

Grains, beans, and seeds contain niacin; it can also be endogenously synthesized from tryptophan (assuming there is dietary protein with this amino acid). The niacin in maize (corn meal) is minimal, as is tryptophan, and not readily available for absorption without exposure to an alkaline solution (e.g., processing in an alkaline solution [lime] to make hominy grits or tortillas).

3. What was the result of the Dawes Act passed by the U.S. Congress in '87?

In '87 the U.S. Congress takes full advantage of these events and passes the Dawes Act, which "gives" every native American head of household 160 acres of land. But that is a fraction of what the Apsáalooke previously had (38,000,000 acres) and the fraction no one else wanted, hardly enough to provide the self-sufficiency that was, at least on the surface, intended by the Act. Instead, through various manipulations, the surplus land goes to those with political power.

Native populations of the Americas declined by over 95%, beginning in 1492. This occurred mainly through introduced infectious diseases and malnutrition. No increase in native population numbers occurred until the 20th century.