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Farber's disease

Farber disease (also known as Farber’s lipogranulomatosis or ceramidase deficiency) describes a group of rare autosomal recessive disorders that cause an accumulation of fatty material in the joints, tissues and central nervous system. The disorder affects both males and females. Disease onset is typically in early infancy but may occur later in life. Children who have the classic form of Farber’s disease develop neurological symptoms within the first few weeks of life. These symptoms may include moderately impaired mental ability and problems with swallowing. The liver, heart and kidneys may also be affected. more...

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Other symptoms may include vomiting, arthritis, swollen lymph nodes, swollen joints, joint contractures (chronic shortening of muscles or tendons around joints), hoarseness and xanthemas which thicken around joints as the disease progresses. Patients with breathing difficulty may require insertion of a breathing tube. Most children with the disease die by age 2, usually from lung disease. In one of the most severe forms of the disease, an enlarged liver and spleen (hepatosplenomegaly) can be diagnosed soon after birth. Children born with this form of the disease usually die within 6 months.

There is no specific treatment for Farber’s disease. Corticosteroids may be prescribed to relieve pain. Bone marrow transplants may improve granulomas (small masses of inflamed tissue) on patients with little or no lung or nervous system complications. Older patients may have granulomas surgically reduced or removed.

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AIDS in Africa: puzzling, harrowing pictures
From Townsend Letter for Doctors and Patients, 11/1/05 by Marcus A. Cohen

The descriptive statistics for Africa indicate how poor African countries are. With poverty on the African scale, malnutrition, an unambiguous cause of compromised immunity, is widespread. In tropical Africa, AIDS and HIV sero-positivity are virtually synonymous with regions where malaria is endemic.... In some African medical practices unsterilized needles and shared syringes are used on a scale which would be intolerable in industrialized countries. Pathogenic and other contaminants are thereby transmitted in blood transfusions and inoculations with penicillin and other injected drugs and vaccines. To this can be added the officially unacknowledged but widely known drug abuse problem in many African countries. There is also a huge incidence of all forms of sexually transmitted diseases. Most of these are treated by an injection, facilitating transmission of several pathogens when done with non-sterile equipment. All of this, combined with inadequate medical care, contamination and shortage of water and food, huge population movements and the diseases which accompany political revolution and war, contribute strongly to the increase of AIDS-defining diseases in Africa.

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Craven BM, et al, Time consistency and the development of vaccines to treat HIV/AIDS in Africa, Economic Issues, Vol. 8, Part 1, 2003.

Out of Africa is the title of a memoir by the Danish author Isak Dinesen (Baroness Karen Blixen, 1885-1962). Dinesen wrote in English, with a brilliance equaled by just two other modern literary masters whose native language was not English; Joseph Conrad (1857-1924, Polish), and Vladimir Nabokov (1899-1977, Russian). Her memoir (published in 1938), recounts the years she spent as owner/manager of a coffee plantation in Kenya (1914-31); and the landscapes and Africans she recreates--seen through the eyes of a European colonist--have a nostalgic, idyllic glow about them.

Spin published a two-part report in 1993 (March, April), also titled "Out Of Africa." Celia Farber, the reporter, had begun covering AIDS in the late 1980s. She wrote her report after traveling through central Africa with Joan Shenton, an award-winning British documentary filmmaker, (1) and Dr. Harvey Bialy, a molecular biologist and scientific editor of the journal, Bio / Technology, who'd spent several years lecturing in microbiology at the university level in Nigeria. Shenton was researching a new documentary for Dispatches, a British TV program that had regularly questioned the medical establishment's position on AIDS.

"We wanted to see it all with our own eyes," said Farber, "to see how the real picture matched up with the picture we'd been given." (2) I'll venture that Farber had read Dinesen's memoir (and/or seen the 1980s film based on the book), and her reuse of the title was ironic; the latter-day African scenes and people that passed before her eyes had nothing in common with Dinesen's Africa. Here's Farber's description of the Rakai District, Uganda:

"We were the only car on the road. Joan and I, seated in the back, stared out the car windows, silenced by the sight. It was as if the whole place had been shredded--a chaos of dust and debris, rotting wooden shacks, garbage, people in rags, children in rags. The poverty in Uganda was crushing, total, and unrelenting. As we drove deeper and deeper into the Rakai District, the 'AIDS epicenter of the world,' all this talk of HIV and T-cells and safer sex started to seem a little absurd. We got out of the car and surveyed what looked like a swamp, with a pipe emerging from it. This was, it turned out, the surrounding villages' water supply. It was also where the sewage was deposited. People looked listless, malnourished. Many of the children had swollen bellies, the telltale sign of malnutrition.

"'Don't ask them what they eat,' advised one doctor we spoke to. 'Ask them how often they eat.'

"The nearest hospital was miles away. There were no cars; the only means of transportation were donkeys and the occasional bicycle. The Ugandan government sets and enforces fees for medication, which most people can't afford. It became clear to us that most people living in the Rakai District had no access to health care whatsoever. Malnutrition, filthy water, diseases left untreated--and the WHO [World Health Organization] had come in with 'AIDS educational programs,' instructing people how to use condoms?" (3)

Celia Farber, Joan Shenton, and Dr. Harvey Bialy are among the journalists and scientists anathematized for questioning the medical orthodoxy's holiest tenets in AIDS: that HIV is the condition's primary cause, and that antiretroviral drug cocktails extend survival.

Still, the number of "heretics" has increased since Farber published her eyewitness account of AIDS in Africa. Today, it includes several Nobel laureates and other distinguished medical and scientific figures. That these HIV-AIDS heresies are spreading has goaded members of the medical ultra-orthodox to suggest charging the dissenters with genocide and criminally prosecuting them. (4)

Orthodox media, represented by such pillars of the national press as The New York Times, have merely censored data and information contrary to the medical establishment line on AIDS; they continue to consider it irresponsible to run stories that might induce people at risk to disregard HIV and shun AZT and protease inhibitor cocktails. Farber's courageous investigation of central African AIDS should have come out in a "paper of record" like The New York Times, where the raw truths of her picture of AIDS in Africa might have jolted some readers. Instead, it got rather marginal, less influential exposure in Spin.

In her first report, Farber observed that "there is very little in the way of hard data or reliable figures coming out of Africa. The apocalyptic scenarios have been extrapolated from anecdotal and incomplete evidence."

She remarked, "Our picture of AIDS in Africa was in large part fueled by the idea that AIDS, or HIV at least, originated there. This theory," explained Farber, "was based on a few reports that a virus similar to HIV had been found in African blood samples dating as far back as the 1950s. A virus said to be 'closely related' to HIV was isolated in the African Green Monkey and before long, the theory evolved that HIV had somehow crossed species, jumping from monkeys to humans through some unidentified mode of transmission. This idea was bandied about in leading scientific journals during the mid- to late '80s by AIDS researchers, who also claimed that AIDS was spread more efficiently in Africa due to extreme sexual promiscuity, blood-drinking rituals, and children playing with dead monkeys." (5)

Farber noted that HIV had also been discovered in a Western blood sample from the 1950s, and that eminent scholars have remained skeptical about AIDS originating in Africa.

Three Nagging Questions About AIDS in Africa

I pored through a batch of papers on African AIDS for this column. The papers were academic to the bone, generally dry--lacking the bite and immediacy of Farber's articles, which never fuzz the individual impact of AIDS.

Searching for published studies that support the medical orthodoxy on HIV and antiretroviral drug therapy, I quickly grew discouraged; among the few I dipped into, the assertions and claims too often seemed faith-rather than evidence-based. The writings of AIDS "heretics," on the other hand, at least supplied acceptable references, often profusely; and their citations were predominantly articles and reports by medical establishment authors in peer reviewed journals.

After acquainting myself with this reading material, I still couldn't make sense of the variation in definitions of AIDS in sub-Saharan Africa, which differed slightly regionally, and differed sharply with the relatively uniform definition in the US and Europe. How did this situation evolve?

I still couldn't comprehend why AIDS was said to be spreading heterosexually in Africa, while in the U.S. and Europe the largest groups at risk were mainly those at risk in the early 1980s--some gay men who abused recreational drugs, intravenous drug addicts, and hemophiliacs. Assuming for a moment that HIV is the cause worldwide: how does a virus, the minutest, simplest form of life, know to infect the heterosexual sub-Saharan African population but only particular subpopulations in North America and Europe?

And I still couldn't piece out why the establishment had fixed on the hypothesis that AIDS was responsible for the apparent upturn in mortality among Africans. Why not the plethora of diseases in Africa historically linked to extreme malnutrition, chronic poverty, and primitive sanitation--under worsening economic and political conditions over the past two decades?

Africa's Varying Definitions of AIDS

WHO clinically defined AIDS in Africa at an international conference in Bangui, Republic of Niger, in October 1985. The following year, WHO published its "Bangui definition," as it quickly was labeled, and very soon the definition was applied in clinics and hospitals throughout Africa. Eventually, the WHO definition of African AIDS was also applied in developing nations on other continents. (6)

Under this definition, Africans could be diagnosed with AIDS if, for instance, they were coughing for a month, suffered from diarrhea more than a month, or manifested a 10% weight loss. Abdominal pain, fever, and vomiting could be qualifying symptoms as well. Only two major symptoms and one minor one were necessary. No HIV antibody tests and CD4 cell counts were required; these AIDS tests were too costly anyway for general use in most areas of sub-Saharan Africa. The whole lot of these symptoms, it must be emphasized, resembles those of many African illnesses recognized since the 19th century.

By comparison, in the US, Europe, and highly industrialized nations elsewhere, there was a specific list of opportunistic diseases for diagnosis during the early AIDS period; among them, dementia, diarrhea, Kaposi's sarcoma, and Pneumocystis carinii (an uncommon form of pneumonia striking gay men who inhaled nitrite drugs recreationally).

Again, in comparison, it can be said that African AIDS has been linked with diseases abundantly found on that continent (particularly in impoverished areas), while AIDS on the North American and European continents was associated at first with a substantial percentage of unusual opportunistic infections.

In the US, the Centers for Disease Control & Prevention (CDC) redefined its AIDS definition in 1993; thereafter, patients could qualify if they tested positive for HIV antibodies and had a low helper T cell count. One noteworthy consequence of the CDC's 1993 redefinition: 60% of Americans subsequently put on antiretroviral cocktails (including protease inhibitors, phased into orthodox treatment in 1996), were healthy (symptom free!) when they went on this therapy.

Back in Africa, shortly after WHO issued its Bangui definition, two new definitions entered the stage, independent of each other, and in practice more in competition than in cooperation with each other. Concluding that the WHO definition was clinically inadequate because of its potential inapplicability, the CDC and the Pan-American Health Organization (PAHO) created these new definitions; ever since, developing nations in Africa and other parts of the world may select the WHO, CDC, or PAHO definition, whichever suits each country. (They can also pick from the tighter AIDS definitions in the industrialized nations.)

Charles E. Gilks, a visiting scientist at the Kenya Medical Research Institute, Nairobi, published a paper in the British Medical Journal in 1991, in which he argued that the clinical definition for African AIDS "is an unworkable concept because patients with underlying immunosuppression disorders such as AIDS can not be easily distinguished from chronic disease patients; i.e., pulmonary tuberculosis, renal failure, uncontrollable diabetes, or diarrhea with weight loss." (7) Celia Farber referred to Gilks' paper in her first report, observing that diseases endemic in parts of Africa such as TB, malaria, and parasitic infections can themselves, "independent of HIV ... lead to severe immune depression." (8) She then quoted a warning by Gilks about a pernicious effect of the several AIDS definitions loose in Africa: "Substantial numbers of people who are reported as having AIDS may in fact not have AIDS." (9)

I'll end with another brief excerpt from Farber connected with the African AIDS definitions. She interviewed a French nurse, a man she identified as "Krynen," working with orphans in the Tanzanian region of Kagera, not far from the Ugandan border.

"'If people die of malaria, it is called AIDS,' Krynen said. 'If they die of herpes, it is called AIDS. I've even seen people die in accidents, and it's been attributed to AIDS. The AIDS figures out of Africa are pure lies, pure estimate.'" (10)

My next column and the last in this series on AIDS (December Townsend), will examine the questions I asked earlier here about the extent of heterosexual transmission in Africa and the AIDS epidemic believed to be ravaging sub-Saharan Africa. It will feature comments by Michael Ellner and Dr. Roberto Giraldo.

References

1. Two of Shenton's documentaries on HIV-AIDS won the Royal Television Society Award (UK), and the British Medical Association Award. NewAfrican, Dec. 1998, is the source for this information. Shenton has also published a book on AIDS, Positively False (St. Martin's Press, NY, 1998).

2. Farber C., Out of Africa, part one, Spin, March, 1993.

3. Ibid.

4. Peter Barry Chowka, in Natural Healthline, 1 May 2000, points to John Moore, a researcher at the Aaron Diamond AIDS Research Center, NYC, as the ultra-orthodox member saying publicly that it would not be inappropriate to charge HIV skeptics with genocide, and to Canadian virologist Mark Wainberg, president of the International AIDS Society, as the member suggesting that these skeptics warrant criminal prosecution.

5. Farber, op. cit.

6. This whole section on the definitions of AIDS in Africa was synthesized from the following articles: Craven BN, et al, Time consistency and the development of vaccines to treat HIV-AIDS in Africa, Economic Issues, Vol. 8, Part 1, 2003. Rasnick, David and Fiala, Christian, But-what about Africa? (Unpublished paper, 2004; obtainable through GOOGLE, through title.) Dirty tricks over AIDS figures, NewAfrican, April 1998. (This "cover story," except for the first paragraph, is an article by Dr. Christian Fiala about AIDS in Africa.) Farber C., Out of Africa, part one, Spin, March 1993. Many more articles on African AIDS are available at www.virusmyth.com

7. Gilks CF, What use is a clinical case definition for AIDS in Africa? BMJ, 1991 Nov 9,303(6811):1189-90.

8. Farber, op. cit.

9. Ibid.

10. Ibid.

by Marcus A. Cohen

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