 Hi and welcome to Nursing School Explained. Today we'll be discussing Ray's syndrome. Ray's syndrome is a very uncommon but unfortunately very serious syndrome that can occur mostly in children. And Ray's syndrome, typically the etiology is unclear, so we don't exactly know where it comes from but it seems to be linked to occur after a viral illness such as chickenpox and upper respiratory infection, influenza and so forth. And children who during such a viral illness are treated with aspirin have about a 35 times higher risk of developing Ray's syndrome. Now generally aspirin is approved to be administered to children three years or older but when children are treated with aspirin during or immediately after a viral illness they can develop this very serious syndrome, Ray's syndrome. So in general we should stay away from recommending aspirin in the treatment of fever or pain for children in general and use other agents such as acetaminophen and ibuprofen but keep in mind ibuprofen only if the child is older than one year old. And another cause of Ray's syndrome can be a fatty acid oxidation disorder and this is also a very rare disorder that typically is only found out about after the patient goes through an episode of Ray's syndrome. So what happens? So in Ray's syndrome the virus when the addition to the exposure to the aspirin causes damage to the liver cells. And remember the liver is a detoxification organ and ammonia is a byproduct that is usually broken down by the liver but if the liver is damaged it will lead to increased levels of ammonia and this increased level of ammonia can cross the blood brain barrier causing cerebral dysfunction and encephalopathy in addition to cerebral swelling. Now fluid and electrolyte balances are also very common as well as acid-base imbalances and then coagulopathies so trouble with blood clotting, remember the liver is always in charge of coagulation as well. So signs and symptoms usually occur in five stages and so initially the patient might have this viral illness three, four days later they might start to vomit, they might present as being lethargic. This lethargy can lead to being combative and confused all the way to coma and decorticate posturing and remember that decorticate and deseribrate posturing are descriptions of what's happening with the body physically when there is swelling of the intracranial cavity. So decorticate position and remember everything is going to go to the core so the patient's arms will be flexed and there as well as their hands and then in deseribrate posturing the arms will be pointing away with the fist pointing away and that is usually a very late sign of posturing and pretty much a sign of imminent death and then in the last stage here patient will develop seizures eventually leading to respiratory failure. So you can see this very mild viral infection leading to the sliver damage and encephalopathy can very quickly escalate to death. So the most important thing here is that we educate our patients not to administer aspirin to children for any kind of viral illness and if early symptoms occur after a viral illness such as vomiting and lethargy so any sign of altered level of consciousness should prompt us to let them know that they should seek medical care right away to prevent these cascading events. Now diagnostic tests for Ray syndrome usually include a thorough history and physical to find out about the recent viral illness as well as the administration of the aspirin in addition diagnostic studies will show increase in liver function tests as well as the increase in ammonia levels blood sugar will a lot of times will be low as well and then coagulation studies such as PT INR and PTT will be altered because of the coagulation pathies that stem from the liver dysfunction. Now treatment is of course supportive care patients may require mechanical ventilation if they're unable to maintain their own airway and then it is treated with corticosteroids and barbiturates as well as mannitol to decrease cerebral edema these are very effective medications to do that and mannitol specifically is also an osmotic diuretic basically acting like a hyperatonic solution pulling fruits from the cerebral cells that are swollen and edematous and excreting it in the urine but of course we'll have to be careful monitoring kidney function as well as their electrolytes because imbalances certainly can occur here. The patient will also be treated prophylactically with anti-seizure medications because we know that seizures are a complication of Ray syndrome and vitamin K will be administered to help with the coagulation. Now nursing care in the treatment of a patient with Ray syndrome will be supportive as well as the administration of all these medications certainly will have to monitor vital signs as well as the patient neurologic status very frequently to determine any cascading down these signs and symptoms here. Frequent eyes and nose because of all these medications and the administration of hyperatonic solutions as well as monitoring their labs to check their electrolytes and then emotional support for the patient and of course the family because this is a child who just had the flu and now they are seriously ill at the hospital with this very rare disorder of Ray syndrome so we'll need to provide emotional support to the family, explain, educate to them as to why this happened and what the treatment is that's going to happen here for this patient. So thank you for watching this video on Ray syndrome here at Nursing School Explained. I will see you next time.