In 1883, Mother Marianne, a member of the Sisters of the Third Order Regular of Saint Francis (based in Syracuse, New York) received a plea for help in caring for leprosy sufferers from King of Hawaii. More than 50 religious institutes had already declined his request for sisters to do this. However, she enthusiastically:
"I am hungry for the work and I wish with all my heart to be one of the chosen Ones, whose privilege it will be, to sacrifice themselves for the salvation of the souls of the poor Islanders... I am not afraid of any disease, hence it would be my greatest delight even to minister to the abandoned ‘lepers.’"Along with six other Sisters of St. Francis, 45-year-old Mother Marianne arrived in Honolulu in November 1883. She became the manager of the Kakaʻako Branch Hospital which served as a receiving station for patients with leprosy gathered from all over the islands.
In November 1888 she moved to Kalaupapa both to care for Belgian priest (right) and to assume his burden. Father Damien was already known internationally for his heroic care of the leper colony there. He had contracted the disease himself in 1884, and was now dying from it. He was canonized for his work by Pope Benedict XVI in 2009.
Mother Marianne and two assistants ran the Bishop Home for leprous women and girls, as well as the Home for Boys at Kalawao, founded by Father Damien for boys and young men.
Despite her close contact with the patients, Mother Marianne never came down with the disease. She lived the remainder of her life in Hawaii, and died of natural causes at the age of 80.
What is Hansen's Disease (Leprosy)?
Hansen's Disease is a is a chronic disease caused by two bacilli: and . It causes skin sores, nerve damage, and muscle weakness that gets worse over time.Leprosy was recognized in the ancient civilizations of China, Egypt and India. References to the disease were found on Egyptian papyrus in about 1550 B.C.
Throughout history, leprosy has been feared and misunderstood, and has resulted in significant stigma and isolation of those who are afflicted. It was thought to be a hereditary disease, a curse, or punishment from the gods. During the Middle Ages, those with leprosy were forced to wear special clothing and ring bells to warn others as they walked by.
M. leprae is a slow-growing, intracellular pathogen that cannot live outside its host. It can only be grown in animals, not in a laboratory, and is therefore more difficult to study than other bacteria. Armadillos and immunocompromised mice are used in M. leprae research.
M. leprae multiplies slowly, and symptoms can take as long as 20 years to appear. Armadillos are the only animals other than humans that have been found to become naturally infected with M. leprae.
The mode of transmission of leprosy is still unclear. Though widely assumed to be spread via the respiratory system through nasal droplets, broken skin is also a possibility. An showed that armadillos may be a transmission vector for leprosy in the US.
The clinical manifestations of Hansen's Disease form a spectrum based on the cellular immune response to M. leprae. Patients with good T-cell immunity (Th1 type) towards M. leprae exhibit tuberculoid (TT) leprosy which is also known as pauci-bacillary leprosy, a milder form of the disease. It is typically characterized by skin discoloration.
Those with poor T-cell immunity towards M. leprae typically exhibit lepromatous (LL) leprosy or multi-bacillary leprosy. This form is associated with symmetric skin lesions, nodules, plaques, thickened dermis, and frequent involvement of the nasal mucosa resulting in congestion and nose bleeds.
Patients with LL leprosy have been documented to have large numbers of organisms in the skin. They exhibit many skin lesions with slight hypopigmentation, and less sensory loss in the lesions.
While people with LL have high titer antibodies to M. leprae, they also have an impaired cellular immune response to the bacillus. Changes in immunity of the host as well as treatment can result in worsening of the clinical course of the disease.
All forms of leprosy may cause some degree of peripheral neurological damage in the arms and legs that causes sensory loss in the skin as well as muscle weakness. People with long-term leprosy may lose the use of their hands or feet due to repeated traumatic injury resulting from lack of sensation. If left untreated, it can cause progressive and permanent damage to the skin, nerves, eyes, and limbs.
How is Hansen's Disease Treated?
In 1941 the course of leprosy radically changed with the introduction of the first medication to treat it. Promin, a sulfone drug, seemed to work, but required many painful injections. Dapsone pills were found to be effective in the 1950s, but in the 60's and 70's, M. leprae developed resistance to dapsone. At the same time, a three-drug combination of dapsone, rifampicin (Rifadin), and clofazimine (Lamprene) was determined to be very effective in killing M. leprae. This multi-drug treatment (MDT) was recommended by the WHO in 1981 and remains, with minor changes, the therapy of choice. MDT takes from 6 months to a year or even more, depending on clinical manifestations of the leprosy infection.How common is Hansen's Disease Today?
Official figures from the reveal that almost 182 000 people, mainly in Asia and Africa, were affected by Hansen's Disease at the beginning of 2012. Approximately 219 000 new cases were reported during 2011. Hansen's disease is rare in the U.S. although there are currently about 6,500 cases; with 3,300 requiring active medical management. Most (97 or 65%) of these cases were reported in California, Florida, Hawaii, Louisiana, Massachusetts, New York, and Texas.The Key Facts About Hansen's Disease Today
Leprosy remains the most misunderstood human infectious disease. The stigma long associated with the disease still exists in most of the world and the psychological and social effects may be more difficult to deal with than the actual physical illness. The key facts of Hansen's Disease today include:- Most (95%) of the human population is not susceptible to infection with M. leprae, the bacteria that causes Hansen's disease.
- Treatment with standard antibiotic drugs is very effective.
- Patients become noninfectious after taking only a few doses of medication and need not be isolated from family and friends.
- Diagnosis in the U.S. is often delayed because health care providers are unaware of Hansen's disease and its symptoms.
- Early diagnosis and treatment prevents nerve involvement and the disability it causes.
- Without nerve involvement, Hansen's disease is a minor skin disease.
Have any of you seen a patient with Hansen's Disease?