Zeenat ChoudhrySection GSS The disorder faced by the Baxley family is known as kuru, which means “to be afraid” or “to shiver”, or GSS-Gerstmann-Straussler-Scheinker syndrome. The disease starts witha dizzy and confused feeling. It progresses to shaky and lurched movements of the body such as a hand that could not hold a pen. The shakiness of the body is constant and uncontrollable. This shakiness turns into a complete loss of muscle control leaving the patient unable to stand or sit without help. The victims are unable to talk or swallow without choking. The tongue becomes sounresponsive that the patient cannot pronounce words correctly. Facial muscles go into a phase of twitching and exaggerated expressions. Eventually, all movements of the body come to an end. The patient’s face looks frozen as the eyes stare widely without any sort of blinking or movement. Then, the result is typically death. In the book, victims of this disease unfold new symptoms related to the disease which they were unware of. Memory loss or dementia is anothersymptom of the disease by Bill Baxley, the father who died from GSS. Buddy, Bill’s son experi-enced insomnia as one the symptoms and had no idea whether it correlates to the disease. The family’s medical history showed no sign of insomnia being a symptom for GSS. The Baxley family’s sons thought that different mutations in the prion genes were linked with different dis-eases. While Bill, Billy and Faye had the GSS gene mutation, Buddy, who experienced insomnia,was thought to have inherited fatal familial insomnia. Several scientists worked together to get an insight into the disease. One of the first clue were the findings of various holes in the brains of those killed by the disease. The tangles orshapes inside the brain did not completely resemble of those with Alzheimer’s which is why sci-entists did not give up their research. Stanley Prusiner coined the term ‘Prion’ when he had fig-ures that there infectious proteins inside the brain associated with this disease. Pursiner insisted this type of disease cannot be caused by a virus as it doesn’t elicit any response from the immunesystem. He then concluded that the scrapie parts of the brain were proteins. Tom bird, a geneti-cist, was able to identify the mutated prion gene that causes GSS. He could test to see if this geneis present in others. A mutation in a prion, a misfolded protein transmitting its’ misshaped variants onto a normal protein, a gene is what causes someone to have GSS. If you had the gene, you would inherit it. There is a fifty-fifty chance that the child of someone with a GSS. The worst part is that there was no known cure for such a disease that ends up taking the lives of family members. At first, Bill Baxley, the father who had died from the same diseaswe, visited many doctors in hope to cure himself. However, the doctors were not successful in figuring out the dis-ease. They would describe his condition as a rare anomalous disease with a combination of Alzheimer’s and Parkinson’s. There was no cure for it even after the disease has been discov-ered. However, there was a way to find out which embryo cell contained the mutated GSS gene through PGD-preimplantation genetic diagnosis. Amanda, Buddy’s daughter, was pro-vided with the option of PGD as she also inherited the GSS gene. Embryos with the mutation canbe discarded while the normal embryos can be used to produce offspring without the risk of an inherited GSS gene mutation. The GSS mutation in Amanda’s family was 198 base pairs from thestart of the prion gene. It had one pair of DNA bases distinct from those in the nonmutated gene. Amanda had twin babies; a girl and a boy. The girl was named Ava and the boy as Cole. Despite all the hardship she faced in having them, she was proud that they were GSS free. It was surprising to see that Amanda, who once believed that it was wrong to kill the abnormal embryos, finally agreed to PGD. While reading the book, I thought she wouldn’t gothrough it, because it clashes with her religious beliefs. She believed that killing an embryo even after a few days of its conception is sinful. However, she was quite intelligent to have changed her mind. I would have done the same thing, because GSS certainly has no cure and by giving birth to a baby with GSS would mean killing it all over again. That is where the book title justi-fies itself as well. By giving birth to babies with the GSS mutation, they would only grow up too soon face the terrible circumstances and death associated with the disease. There is also a possi-ble chance of them passing it onto their own children which then leads to the same cycle. This way, more and more people would be dying. On the other hand, by giving birth to those free of the GSS gene, we are offering them a life free from guaranteed GSS suffering and death. If Bill had done PGD, his sons and his granddaughter would not have had to face the horrifying symp-toms and hopelessness marked by the disease.Work Cited1. Kolata, Gina Bari Mercies in Disguise: a Story of hope, a a Familys Genetic destiny, and the Science That Rescued Them. St Martins Griffin, 20182.