Ezra Powell

Ezra Powell was a character from the episode Informed Consent. He was a 71-year-old research scientist who loses consciousness while working in his laboratory. Powell is depicted as a famous and prolific scientist who helped develop the protocol for the stress test and the standard diagnostic language for chest pains. He is portrayed by Academy Award winning actor Joel Grey.
==Medical History==
Ezra has been in and out of hospitals for over a year describing difficulty breathing, fatigue and periods where he lost consciousness, but his previous doctors have been unable to diagnose or treat him. Earlier, he had a minor melanoma removed and surgery for cataracts.
==Case History==
Ezra was brought to Princeton-Plainsboro Teaching Hospital when he collapsed while performing experiments on rats in his laboratory to further his research on cancer. He suffered a minor facial laceration on his lip when one of the loose rats took the opportunity to bite him while he was unconscious. Dr. House took his case and advised his team that the patient was suffering from minor tremors and difficulty breathing. His blood oxygen level was very low and his lungs were full of fluid. Dr. Chase was sure it was a problem with the heart, such as amyloidosis. However, Dr. Foreman thought it might be lung difficulties from breathing toxins in laboratories. Dr. Chase pointed out the chest X-Ray was clean, but Dr. Foreman pointed out the patient's EKG was also normal. Dr. House suspected that the treatment they were giving him was masking the patient's symptoms. He ordered a stress test. However, Dr. Chase pointed out that for a patient of Ezra's age, overstressing the heart or lungs could be fatal. Dr. House realized the risks but ordered the test anyway.
Dr. Cameron prepared Ezra for the test as he described his role in creating the protocol. However, he could not raise his heart rate to the target of 130 bpm without running out of breath. They realized the patient could not complete the test.
However, when they brought the failure to Dr. House, he insisted that they try again. Dr. Cameron said the patient was in pain because of fluid in his lungs, so this time he directed her to suction the fluid before the test. When Dr. Cameron and Dr. Chase performed the suction, Ezra told her that he realized they were trying to find out if it was his heart or lungs and that he was betting on both. Given his numerous hospital stays, he was likely just old and getting sick. They tried the test again, but again he could not complete it. Dr. Cameron informed Dr. House again, who again insisted on the test, this time on a hand driven treadmill. This time Dr. House observed the test. When Ezra again failed to complete it, Dr. House injected him with epinephrine. Dr. Cameron objected, but Dr. Powell agreed with the course of action. His heart rate started to go up to the target of 130. However, the EKG was still normal, which seemed to rule out a heart problem. Dr. Cameron went to stabilize the patient, but instead he asked her to inject the rest of the epinephrine, asking her to kill him.
At that point, Dr. Powell refused to take any more tests, asking the team to let him die. However, Dr. House thought he was just depressed from his illness. Dr. Chase pointed out that Dr. Powell had seen all the test results too and realized that they were not even close to getting a diagnosis, and even if they did know what was wrong with his lungs the damage was probably irreversible. Dr. House pointed out that without a diagnosis, it was impossible to tell whether or not the lung damage could be treated. Dr. Chase pointed out that wasn't Dr. House's decision to make. The team discussed the ethics of giving Dr. Powell a large dose of morphine to hasten his death given that otherwise he would suffocate in his own fluids. Dr. House thought the lung problems were the result of microplasma or streptococcus based pneumonia. If that were the case, it might be too late to treat him, but they could try levofloxacin. However, Dr. Cameron noted that Dr. Powell would refuse the treatment unless they could convince him it was worth trying.
At that moment, the team was paged to come to Dr. Powell's room. He had tried to disconnect his life support apparatus. Dr. House asked him to let them treat him, but Dr. Powell refused and threatened assault charges if anyone tried. Dr. House sent Nurse Previn out of the room. Dr. House asked Dr. Powell why he came to him, and Dr. Powell told him that he thought Dr. House would have the guts for euthanasia if he were asked. Instead, Dr. House attempts to do a test of his lung capacity, but Dr. Powell refuses to cooperate. Dr. House reminds him of the consequences of not taking treatment - painful, slow suffocation. However, Dr. Powell is adamant. Dr. House asks Powell to give him 24 hours to find out what is wrong, after which he agrees to help him die.
Dr. House starts a new differential. When his team objects to his promise to Dr. Powell, he reminds them that it is his plan to figure out what is wrong, but they have to focus on that task. Dr. Chase agrees to do blood cultures for bacteria to test for pneumonia. He can do it in less than 24 hours if he spins down the blood samples first. Dr. House also orders Dr. Chase to do an amylase, D-dimer, C-reactive peptide, urine test, and take a bronchial sputum sample. He orders Dr. Cameron to check Dr. Powell's house and lab for radiation and toxins. He orders Dr. Foreman to do a bone marrow biopsy. When Dr. Foreman expresses incredulity that they can do all that in 24 hours, Dr. House remarks that if they don't finish something, they can do it at the autopsy.
The team works through the night. However, by the next morning, the blood cultures are negative and the biopsy results are not back. The lab was clean enough for surgery and there were no insects or viruses on the lab rats. Dr. House notes that Dr. Powell was dictating notes onto tape, and thinks he might be having troubles with his memory. When Dr. Cameron points out this is unlikely, Dr. House counters that if it is a symptom, it could help narrow down the diagnosis to a disease that attacks both the lungs and brain. He orders the team to continue the tests.
They give Dr. Powell a scan of his brain. He says he doesn't want them to fail, but he figures they will. Dr. Wilson checks out the possibility of leukemia, but there is no sign of it in Dr. Powell's bone marrow. There's some acellularity, but it can easily be explained by normal alcohol consumption. However, Dr. House points out that if Dr. Powell is telling the truth about not drinking, it could be a fungus. However, Dr. Wilson reminds Dr. House it's more likely Dr. Powell lied during his medical history. Dr. Cameron comes in with the news that the brain scan was clean and the tests on Dr. Powell's cognitive functions are normal. Dr. Wilson soon rules out a fungus as the cause of the symptoms.
Dr. House goes to Dr. Powell after the 24 hours and Dr. House lies to him and tells him he has a multiple myeloma. However, Dr. Powell is suspicious - that wouldn't cause lung problems. Dr. House counters that it's associated hyperviscosity syndrome, the blood in his body is too thick to flow through his lung tissues. However, Dr. Powell knows his calcium is normal and there was no m-protein in his urine. When Dr. House tries to say it's just an odd presentation, Dr. Powell looks at Dr. Cameron, who clearly doesn't believe what Dr. House is saying. Dr. House asks for 12 more hours, then 6. Dr. Powell refuses any further tests. Dr. House points out there is no evidence he has a terminal illness, and then refuses to follow through to help him die. At that point, Dr. Powell asks to be discharged, even though he knows he will slowly suffocate to death. He then asks Dr. House if he's really going to let him die like that.
Dr. Powell continues to get worse. Finally, Dr. House leaves his office and his team follow him. Dr. House goes to Dr. Powell's room and tells his team to leave. Dr. Cameron tells him he can't do it, but he says he has the duty to administer a prescription painkiller to a patient in pain. When Dr. Foreman tries to intervene, Dr. House threatens to inject him with the morphine instead of the patient. Only Dr. Chase stays behind to assist.
Dr. House injects the patient while Dr. Chase closes the blinds. Dr. Powell drifts into unconsciousness and his vital signs monitor gives off an alarm. Suddenly, Dr. House goes to get a laryngoscope and tells Dr. Chase he intends to revive Dr. Powell, but leave him unconscious so they can continue to test him. He intubates Dr. Powell and tells Dr. Chase to get a ventilator.
With Dr. Powell unconscious, at law they can perform any test a reasonable person would consent to. He orders his team to continue tests, then notices that Dr. Cameron's head scan caught the top of the lungs and showed scarring. Combined with the acellulatities in the bone marrow, this could mean an autoimmune disease. Dr. House orders intravenous immunoglobulin in the event it is lupus and a colonoscopy. However, Dr. Cameron leaves the case in disgust.
The colonoscopy appears to be clean, but during the test Dr. Powell's oxygen stats go to 89 and keep dropping. Dr. Cuddy finds out about the case and tells Dr. House that she is supportive as it is the hospital's job to treat the ill, not to end their life. However, Dr. Foreman comes to report the immunoglobulin has made Dr. Powell worse. Dr. Chase suggests it might be idiopathic pulmonary fibrosis, but they would need an open lung biopsy to confirm. Dr. House decides to deal with Dr. Cameron's insubordination by telling her to read one of Dr. Powell's articles.
Dr. Cameron speaks to Dr. Wilson, who confronts Dr. House about the case. Dr. House tells him that he would not object to helping Dr. Powell end his own life if he were terminal, but Dr. Wilson feels that Dr. House is only keeping Dr. Powell alive to try to solve the mystery of what disease he has.
They perform the open lung biopsy. Dr. Cameron comes to discuss the article with Dr. House. In the 1960s, Dr. Powell had injected radiation into infants without the parent's consent to see if they would suffer a reflux of the urethra. They got into an argument about patient's rights, and Dr. House reminded Dr. Cameron that if Dr. Powell had his way, he would be dead now. When Dr. Cameron said that they could withhold treatment and not kill him, Dr. House argued that the only choices were to help him live or help him die - they couldn't do both. He looked down to the surgical suite, and noted that it probably wasn't pulmonary fibrosis. However, Dr. Powell started to crash with a high heart rate and low blood pressure. Dr. House came down to assist, borrowed a stethoscope and found no breath signs on the right side. This indicated his lung had dropped, his chest was filling with air and it was pressing against the heart. Dr. House performed an incision to let the air out of his chest, and this stabilized the patient. However, at that point he noted by accident that the patient was still responding to pain stimuli on the left side, but not the right side. Dr. Foreman thought it was just the loss of reflexes under anesthesia, but Dr. House thought he may be losing sensation in some parts of his body. However, Dr. Chase thought it was merely a CNS caused by the hypoxia when he started to crash. Dr. House wanted to perform anti-sensory evoked potentials, but those could only be performed on a conscious patient. Dr. House thought they might also check his deep tendon reflexes instead, but Dr. Chase pointed out that wouldn't work - to stay on the ventilator, he also needed to stay on muscle relaxants. He finally suggested testing pupilary reflexes, but that only tests the brain stem. Dr. House realized they would have to bring him out of the induced coma to do the evoke potentials.
Dr. House brings Dr. Powell out of the coma to his surprise and consternation, but he brings him up to date on the progress of their diagnosis. However, Dr. Powell refuses the evoke potential test. Dr. House tells him they have assigned a nurse to keep an eye on him, so he might as well cooperate, but he still refuses and asks to be discharged. When Dr. Chase points out that he has two chest tubes, Dr. Powell tells him to remove them, and when they don't he yanks them out himself. Dr. House sprinkles some water on his face and tickles his feet, noticing only his left foot responds. This confirms that something is attacking both his lungs and nerves. However, it isn't affecting his brain, so it is only affecting his peripheral nerves and bone marrow. This could indicate Kawasaki disease, lymphoma, or sarcoidosis. He realizes he needs a skin biopsy, but Dr. Chase and Dr. Foreman realize the patient will never agree to it. Dr. House approaches Dr. Cameron to do it, but although the three diseases he has in mind are treatable, Dr. Cameron points out it could be one of a hundred other conditions that aren't. Dr. House loses his temper and tells her to do her job. She says she won't lie to him, and Dr. House agrees.
Dr. Cameron goes to see Dr. Powell and confronts him about his radiation experiments. He justifies them by the fact that the research led to breakthroughs that saved hundreds of thousands of other patients with kidney disease. He tells her that informed consent and patients' rights hold back research. At that point, Dr. Cameron uses a scalpel to painfully cut off a piece of Dr. Powell's skin.
Surprisingly, Dr. Powell is supportive of Dr. Cameron's actions, praising her for taking a stand on an issue for once, and acting based on what she believes.
The tests on the biopsy sample quickly rule out the three diagnoses. However, Dr. House has been inspired by a young woman and tells Dr. Chase to test for amyloidosis. They had rejected it when they thought his heart was fine. However, Dr. House thought that they simply hadn't stressed his heart enough to show it was damaged. Dr. Chase did the test, which confirmed amyloidosis, which is usually treatable. However, the protein type was AA - an untreatable and terminal form of the disease.
Dr. House goes to the patient to tell him he has amyloidosis. When Dr. Powell suggests skepticism over the diagnosis, Dr. House tells him there would be no reason to lie about him having a terminal form of the disease. Dr. Powell congratulates Dr. House for the diagnosis.
Dr. Powell dies overnight, apparently because Dr. Cameron gave him the fatal dose of morphine he wanted.
==A reflection of House==
Powell is a brilliant introvert who prefers not to work with other people. Throughout his stay in the hospital, no-one visits him, so it can be assumed that he has no spouse or other such relationship, as well as no children or close colleagues (even Wilson and Cuddy visit House when he's in the hospital!). Although he is famous and well respected in his field, he also takes ethical shortcuts in order to make the breakthroughs he has made. He suffers from a debilitating illness that affects his ability to live and work. He is difficult to deal with and, when sick, generally thinks he knows better than anyone else and refuses help. He compares his former colleague, who died parasailing at the age of 84, as always having been an idiot.
==Reaching the diagnosis==
As we've seen, amyloidosis is not hard to test for - a sample of diseased skin, a microscope, a bottle of stain, and polarized light are all you need. This episode should have lasted five minutes after Chase suggested it!
However, it's not that simple. This wasn't a typical presentation of amyloidosis. The most common symptom of amyloidosis is kidney failure as the amyloid proteins tend to congregate there. In this type of amyloidosis, over 90% of patients suffer some kind of kidney problems. The heart is the second most likely organ to be affected. Lung problems are atypical. Moreover, in the United States, this type of amyloidosis usually follows a history of some type of chronic inflammatory disease, typically rheumatoid arthritis. More rarely, it's caused by a chronic infection, but this is more typical of undeveloped countries. Powell didn't have a history of either. Another typical symptom is purpura around the eyes, and that wasn't present here either.
In addition, Powell's asymmetrical neurological problems are also atypical. In most patients, sensory problems are symmetrical, start at the extremities, and work inwards. In addition, these neurological problems often appear as digestive problems.
However, when amyloidosis is suspected, a biopsy is the correct procedure. Although any affected area can be biopsied, in most cases, fat around the abdomen is the best choice as it's not too invasive and is often positive. However, if it's negative, it doesn't rule out the condition and usually a more invasive biopsy is necessary to rule out the condition.
==Explaining the medicine==
*Amyloidosis was on the table right away, but was also rejected right away because the patient has no problems with his heart. In most cases of amyloidosis, the amyloids affect the ability of the heart muscles to contract and this shows up on an EKG.
*Giving Powell a drug in order to increase his heart rate is acceptable when the patient has a very low tolerance for exercise, even on a hand treadmill. However, epinephrine would not be the right choice. Any vasodilator, Regadenoson, or Dobutamine would all be acceptable choices.
*To speed up the process with the blood cultures, Chase suggests running them through a centrifuge first. This separates the blood products with the plasma on top and the red blood cells on the bottom. In the middle is the so called "buffy coat" which would contain any bacteria, as well as the white blood cells and platelets. This part of the sample could be stained for the presence of bacteria.
*High amylase levels can indicate a number of conditions, most of which relate to the digestive tract.
*D-dimer levels show any issues with coagulopathy.
*C-reactive peptides are an indication of inflammation, but can also indicate liver failure.
*Bronchial sputum could be put in culture to show any indication of lung infection.
*In a bone marrow biopsy, one of the things the pathologist looks for is what percentage of the marrow is actually made up of cells. However, except in newborns, a result of 30% of the material being non-cellular (for example, fat) is normal.
*"KOH 10%" is a test for fungal infections. In this particular test, a 10% solution of potassium hydroxide is placed on scrapings from the skin where a fungal infection is suspected. Parts of the fungus can be seen under microscopic examination if present.
*House, of course, lies to Powell after his 24 hours are up, but Powell is also a trained physician and is ready for him. Multiple myeloma can cause a lot of symptoms, but generally doesn't affect breathing. House runs to hyperviscosity syndrome, which can be caused by multiple myeloma and can affect breathing, but also causes calcium levels in the blood to drop, and Powell knows that his levels are normal. House tries to say Chase screwed up the results, but multiple myeloma would also result in an increased production of myeloma protein (M-protein) which would show up in urine tests. Check and mate.
*Morphine reduces the heart rate and House gave him enough to slow his heart rate and cause unconsciousness and coma. However, House also knows that if he puts Powell on pure oxygen and keeps him breathing, he can maintain him indefinitely on a ventilator. Usually, however, barbiturates are used to induce a medical coma.
*It appears IVIG has an unfortunate side effect - it can make amyloidosis worse.
*Congo red was developed as an artificial dye, but is no longer used for that purpose as it's toxic and changes color when exposed to sweat. However, when exposed to amyloid proteins, the dye attaches to them and looks more orange under normal light, but turns apple green in polarized light.
*AA Amyloidosis is usually secondary to another condition, often rheumatoid arthritis. However, if the secondary condition is untreatable, so is the amyloidosis.
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