RETINITIS PIGMENTOSA

Retinitis Pigmentosa is the most common hereditary, degenerative eye disease. The disease is typically slow in its progress. Most people affected by the disease do not become blind until they are between the ages of 40-50. However, there are cases that show more rapid progression.

In Retinitis Pigmentosa, abnormalities in the retinal pigment epithelium and the rod and cone photoreceptors of the eyes lead to progressive blindness. Symptoms begin with night blindness which is followed over time by increasing tunnel-vision and, sometimes, by loss of vision in the central field.

There is currently no cure for Retinitis Pigmentosa. However, blindness can be postponed by up to 10 years through a daily regiment of Vitamin A.

Retinitis Pigmentosa became familiar to 13 year old girls and gay men everywhere during Cycle 3 of the Tyra Banks vehicle, America's Next Top Model, when contestent Amanda revealed that she suffered from the disease. The sad irony was that it was Amanda's pale blue eyes which were her most beautiful feature.

This sick gets a:

1 on my "lethality scale" (1-10)
and a 1 on my "distrubing scale" (1-10)

KURU

Kuru (Mad Human Disease) is a PrP (prion) related disease (see Fatal Familial Insomnia) that causes neurological effects such as truncal ataxia, headaches and uncontrolled tremors ultimately leading to death. Kuru is transmissible between humans via the consumption of an infected person's flesh. The brain meat, in particular, is highly infectious because the prions exist there in a higher concentration.

Kuru was endemic to the Fore tribe of Papua New Guinea. Fore tribe members practiced ritual canibalism as part of burial ceremonies. The men of the tribes ate the best cuts of meat while the women and children were left to consume the organs and brain. It is thought that this difference in consumption practices caused the increased prevalance of Kuru seen in the women and children of the Fore tribe.

Scientists conjecture that the outbreak of Kuru which plagued the Fore tribe began when they consumed someone who had been suffering from Creutzfeldt-Jakob disease (a similar prion-based illness.)

In Kuru, death occurs within 18 months of the onset of symptoms. However, like its cousins Mad Cow Disease and FFI, the disease can be latent for up to 40 years from the time of contraction.

Because this sick is fatal and is passed through canibalism, I give this sick a
7 on my "lethality scale" (1-10)
and a 7 on my "disturbing scale" (1-10)

PROTEUS SYNDROME

Named after the sea-god Proteus, Proteus syndrome is an extremely rare (200 cases since the disease was isolated in 1979) hereditary disorder that causes uncontrolled growth of bones, muscles, tissues, skin and lymphatic vessels leading to severe disfigurement as the disease progresses.
Victims of Proteus Syndrome are born with a completely normal appearance but as they grow older growths of skin and bone begin to alter their appearance and cause health complications.
Affected individuals may die early due to complications from extraneous tissue and bone growth. Sometimes, the very mass of the tumors can end their lives. For instance, the most famous Proteus Syndrome case, Joseph Merrick (The Elephant Man), died when the weight of his head broke his neck while he was sleeping.
Scientists are still trying to figure out what causes Proteus Syndrome, but there is some evidence that it is linked to the PTEN gene on either chromosome 10 or chromosome 16.
There is no prescribed treatment, but there has been some success treating the illness with Rapamycin.

Due to the horrible disfigurement this disease causes and the fact that it often leads to early death, I give this sick a
4 on my lethality scale (1-10)
and a 10 on my disturbing scale (1-10)

PROGERIA

Progeria is an extremely rare, genetic condition (1 in 4,000,000) which causes exceptionally rapid aging. Children who are born with this ailment usually die by the time they are 13 years old.

Though the disease is genetic, it is not usually inherited. It is a mutation which causes a change to LMNA, a gene on chromosome 1 that is crucial in producing the membrane which surrounds cell walls. It is this change to the cellular membrane that causes the rapid aging seen in progeria patients.

There is no treatment for Progeria. Instead, doctors focus on solving the problems that the disease causes, such as heart disease, through surgery and profilactic measures.

90% of progeria cases die from cardiovascular causes such as stroke or heart attack.

Progeria was first discovered in 1886 by Jonathan Hutchinson. It was also independently documented in 1897 by Hastingls Gilford. The disease's full name is: Hutchinson-Gilford Progeria syndrome (HGPS).

This is a truly devestating illness with 100% mortality over a relatively long period of time.
Its physical manifestations are disturbing and painful to the victim.

So, this sick gets a:
9 on my lethality scale (1-10)
and a 9 on my disturbing scale (1-10)

RICKETS

Rickets is a nutritional deficiency characterized by softening of the bones caused by lack of vitamin D and calcium. At particular risk are breast fed babies whose mothers don't get enough sunlight, a major source of vitamin D.

The reason that lack of vitamin D has such a profound effect on the bones, is the vitamin D is necessary in order for the body to absorb calcium from food in the stomach and intestines. Without vitamin D, the calcium is not absorbed but, rather, passes through the body and is excreted.

Children suffering from Rickets can sometimes present to doctors as abuse victims as many of the symptoms resemble damages caused by child abuse.

Those suffering from rickets are treated with Ultraviolet Light Therapy and dietary changes to increase the intake of vitamin D, calcium and phosphates.

Because this disease disables and disfigured children, but is not typically lethal, I rate it a

1 on my "lethality scale" (1-10)

and a 6 on my "disturbing scale" (1-10)

BELLS PALSY

Bells palsy is a paralysis of cranial nerve VII resulting in inability to control the facial muscles on the affected side. Many conditions result in facial paralysis. Doctors use the term "Bells Palsy" to describe facial paralysis for which no cause is apparent.

The onset of the facial paralysis is very sudden, typically causing complete or paralysis of the affected side of the face within 24 hours.

No cause for Bell's palsy has been isolated. However some evidence suggests herpes simplex type 1 infection might play a role in triggering the symptoms.

Bells Palsy is often treated with anti-inflamatory and anti-viral medications, but there is some argument about the efficacy of treatment.

Even without treatment most (85%) Bells Palsy sufferers will get better within 3 weeks of onset. With the other 15% recovering within 6 months.

One unpleasant complication that can occur with this ailment is a chronic loss of the ability to taste.

This is a relatively harmless though strange sick.

So, this ailment rates a

1 on my "lethality scale" (1-10) and a

4 on my "disturbing scale" (1-10

MARBURG VIRUS

Marburg Virus is a haemorrhagic fever of African origin which causes high fever, bleeding and organ failure. Mortality after infection is said, by some, to be over 90%.

Marburg is extremely similar to its more famous cousin the Ebola Virus. In fact, both viruses are structurally identical. It is because they produce different antibodies that they are classified as seperate diseases.

The Marburg virus is named after Marburg Germany where the disease was first documented in 1967. The virus was traced back to lab monkeys.

The disease is indigenous to Africa where, it is believed, a natural reservoir of fruit bats stores the virus during times of dormancy.

There is no treatment for Marburg virus. No antivirals have been proved to slow its progress. Victims are treated with supportive medications and fluids.

This disease is passed via body fluids including blood, excrement, saliva and vomit.

Symptoms are flu-like at onset, but quickly proceed to rash, haemorrhaging, hypovolemic shock and multiple organ failure.

This is a very harsh sick.

So, this ailment rates a
10 on my "lethality scale" (1-10)
and a 10 on my "disturbing scale" (1-10)

FETIFORM TERATOMA

A Fetiform Teratoma is a tumor with tissue and organ components resembling those of a fetus; including limbs and organ systems.

Teratomas are part of a group of tumors called nonseminomatous germ cell tumor (NSGCT). All tumors of this class are caused by abnormal growth of germ cells and embryonal cells.

Teratomas of embryonal origin are hereditary; teratomas of germ cell origin are not necessarily congenital. The kind of cell from which the tumor develops does not have any affect on the tumor, except for determining the location of the tumor in the body.

Fetiform Teratomas are not necessarily malignant and can often be removed with excellent results.

So, this ailment rates a 3 on my "lethality scale" (1-10)

and a 10 on my "disturbing scale" (1-10)