Kuru is a rare and incurable neurodegenerative disorder that affects the brain and nervous system. It is caused by an infectious protein called a prion and is most commonly found in isolated communities where certain funeral rituals were practiced. Contacting and treating patients with kuru requires great sensitivity and care. In this article, we will explore key questions around kuru:
What is kuru?
Kuru belongs to a class of progressive neurodegenerative disorders known as transmissible spongiform encephalopathies (TSEs) or prion diseases. Prions are misfolded proteins that can trigger normal proteins in the brain to also fold abnormally. This leads to brain damage and the development of small spongy holes in the brain, causing the spongiform appearance.
Kuru specifically affects the cerebellum, the part of the brain responsible for coordination and balance. As brain tissue is damaged, patients gradually lose control of their limbs and body movements. In the late stages, patients may be completely disabled and unable to eat or swallow on their own.
Kuru has an incubation period of 10-13 years before initial symptoms appear. Once symptoms manifest, the disease progresses rapidly and is always fatal. Patients typically die within 6-12 months of disease onset.
What causes kuru?
Kuru is transmitted between humans through ritualistic cannibalism. Historically, the Fore people of Papua New Guinea practiced funeral rituals where they would consume the bodies of deceased relatives as a sign of respect and mourning. This practice led to the spread of kuru within Fore communities.
The prion disease can be transmitted through exposure to infected nervous system tissue, often through consumption or injections. Women and children were most affected by kuru due to their roles in preparing the deceased bodies for consumption.
Once the practice of cannibalism ended in the late 1950s, the incidence of kuru rapidly declined. However, the disease may still be transmitted if infected tissue enters open wounds.
Who gets kuru?
Kuru primarily affected the Fore people and neighboring communities in the highland regions of Papua New Guinea where cannibalism was practiced. At the peak of the epidemic in the late 1950s, kuru accounted for 2% of all deaths among the Fore people.
Women and children were more likely to contract kuru due to their increased exposure during mortuary preparations. Children were also affected since their young age and lack of control over food preparation put them at higher risk of infection.
No one outside these indigenous populations has been diagnosed with kuru. The last sufferers today are elderly Fore people who were exposed decades ago before cannibalism ended.
What are the symptoms of kuru?
Kuru has a long incubation period before initial symptoms appear. The first signs are typically difficulty walking, instability, and slurred speech due to lack of muscle coordination. Over time, patients lose the ability to walk independently and require assistance eating and carrying out daily activities.
As the disease progresses, neurological symptoms emerge such as uncontrollable tremors or random bursts of laughing or crying. Patients may exhibit mental changes including memory loss, dementia, and withdrawal from family and friends.
In the final stages, patients can no longer sit up without support, swallow, or speak intelligibly. Since kuru targets the brain’s cerebellum, patients retain awareness and cognition even as their bodies deteriorate.
How is kuru diagnosed?
No definitive tests exist for kuru, so physicians rely on the patient’s medical history, a neurological exam, and ruling out other potential causes. Doctors assess the individual’s symptoms and disease progression to determine if it matches the clinical profile of kuru.
Brain scans like MRIs may show characteristic patterns of damage to the cerebellum. Analysis of cerebrospinal fluid via lumbar puncture can also provide clues by detecting proteins produced during nerve degeneration.
Finally, a brain biopsy after death can confirm the presence of kuru by revealing characteristic spongiform degeneration and prion protein deposits. However, biopsy results may remain inconclusive.
Is there a treatment for kuru?
Unfortunately, no effective treatment exists for kuru. Medical care focuses on alleviating symptoms and providing comfort care as the disease runs its fatal course.
Physical and occupational therapy can assist with mobility and coordination. Medications may help manage tremors, pain, and psychiatric symptoms like depression. Support with nutrition and hydration also helps patients maintain strength and quality of life.
However, no drugs can slow or stop the ongoing prion infection and neurodegeneration. Eventually, most patients will require around-the-clock palliative care.
How do you prevent kuru?
The most effective prevention of kuru transmission is completely avoiding consumption of human nervous system tissue. In places where cannibalism was practiced, education programs successfully ended these high-risk traditional rituals.
Controlling the use of infected surgical instruments or grafts can also prevent iatrogenic transmission in healthcare settings. Proper sterilization and single-use disposable instruments eliminate this risk.
Researchers continue seeking possible treatments that may combat prion infection. However, no vaccines or prophylactic medications currently exist that can reliably prevent kuru in exposed individuals.
How do you contact kuru patients?
Most remaining kuru patients live in Papua New Guinea’s remote highland regions with limited access to healthcare. Visiting and treating kuru patients requires significant travel and community engagement.
Traveling medical teams should first collaborate with regional government health departments to coordinate logistics like transportation, guides, and translators. Partnering with local village leaders builds trust and gets permission to enter their communities.
Doctors should be sensitive to cultural traditions and beliefs around illness when interacting with patients. Clear communication via a translator provides the best care experience. Any treatments or therapies offered must be fully explained beforehand and consent given.
Most importantly, healthcare workers need to approach patients and families with great compassion. A fatal, incurable disease like kuru warrants gentle counseling plus physical, emotional, and spiritual support.
What research is being done on kuru?
Kuru has provided unique insights for scientists studying transmissible spongiform encephalopathies. Ongoing research focuses on several key areas:
- Prion disease transmission – Understanding prion infection and spread between individuals.
- Disease progression – Mapping kuru’s pathogenesis over its long incubation period.
- Strain variation – Studying differences between regional kuru strains.
- Genetic resistance – Identifying genetic factors related to disease susceptibility and resilience.
- Species barriers – Examining how prions jump between human and other animal hosts.
- Treatment development – Searching for compounds that may inhibit prion replication.
Kuru field studies have been instrumental in these research initiatives. Continued work with at-risk populations provides unique insights not easily studied in a laboratory setting alone.
Conclusion
Kuru is a devastating and incurable neurodegenerative disease transmitted between humans via cannibalistic funeral rites. It continues to impact remote Fore communities in Papua New Guinea decades after this practice ceased. Providing compassionate patient contact and supportive care remains vital, as no treatments yet exist. Ongoing research to understand prion infection may someday yield new ways to diagnose, treat, and prevent kuru.