Medical History Edit
Hannah suffers from CIPA, a very rare genetic disease that prevents her nerves from feeling the sensation of pain, heat and cold. She is unable to sweat and as such has difficulty both regulating and judging her body temperature.
Hannah has many injuries that are typical for someone suffering from CIPA, such as small scars on her lips, tongue and mouth from biting them with her teeth and not feeling the sensation. In addition, she suffered a severe burn as an infant from sitting on a hot stove element.
Hannah is frequently in hospital emergency rooms. Because of her condition, she is unusually clumsy as she feels no pain from falling and doesn't take precautions to brace against falls. As she doen't feel pain, she usually requires a full work up to ensure she has not suffered a serious injury.
Hannah's heritage is Ashkenazi Jew. CIPA and other genetic diseases are more common in this and other close knit ethnic groups due to the low genetic variability between individuals.
Case History Edit
Hannah was involved in an automobile accident with her mother. Her mother was badly injured but Hannah did not lose consciousness and was able to call 911. Her most serious issue appeared to be that a piece of metal from the car had broken off and become embedded in her left leg just above the knee, and she had minor facial lacerations causing manageable bleeding. She was taken to the emergency room of Princeton-Plainsboro Teaching Hospital.
Hannah was being treated by Dr. Foreman, who had been asked to help out in the ER because of the snowstorm that evening. He was treating the leg wound when Dr. House found him there. Dr. Foreman started to use antiseptic on the wound and Dr. House noted that instead of jerking her leg away, she actually moved it towards Dr. Foreman. Dr. House asked the patient for her name and told her she had CIPA. Hannah denied it. Dr. House ordered a full set of x-rays, blood tests for infection, an EEG and a spinal nerve biopsy. Dr. Foreman was incredulous as there are only about 60 documented cases of CIPA in the entire United States. However, Dr. House was insistent. Dr. Foreman said the patient had denied having CIPA, but Dr. House asked why she would deny having a disease she shouldn't even know about - most laypersons would ask what CIPA is. She obviously knew what CIPA was. He also pointed out that although she was wet and covered in snow, she wasn't shivering. Hannah said it was warm in the ambulance. He pointed out the scarring of the lips and tongue typical of what happens to infants with CIPA. She claimed she smashed into a window. He noted that Hannah flexed her muscle into the pain stimulus of the antiseptic rather than away from it - she was faking the response, but had never felt it. He also noted that her last name was Jewish and Ashkenazi, a group with higher risk of CIPA. Dr. Foreman still pointed out that Hannah denied having it and that if it had been undiagnosed by this time, she would have died from the disease's complications. Dr. House pointed out that if she admitted having CIPA, she would know that they wouldn't let her see her mother until they had completed their tests, which would take hours. Dr. Foreman was still unconvinced, so Dr. House whacked Hannah in her right shin with his cane. She didn't flinch. Dr. Foreman was convinced.
Dr. House went to Dr. Cuddy to ask that Hannah be assigned to him. Dr. Cuddy was afraid that Dr. House had a personal interest in the case due to his chronic pain. She approved all the tests except the nerve biopsy, which could result in paralysis. She agreed to hear him out again if there was a problem with the EEG.
The EEG went poorly because Hannah refused to cooperate and removed the EEG leads. Dr. House ordered sedation, but Hannah wouldn't agree to it, and they couldn't get consent because her mother was unconscious and she had no father. In any event, they did try to sedate her, but she had enough strength to fight back and because of her insensitivity to pain they couldn't do anything to discourage her from struggling. Dr. House said to break a bone if that was what it takes and that Hannah wouldn't feel it anyway. Dr. House went to sedate her himself. When he arrived, Hannah was still demanding to see her mother. Dr. House argued with her and they started comparing who had it worse - someone who didn't feel pain at all or someone in constant pain. When Hannah turned around to show where she burned herself on the buttocks, Dr. House took the opportunity to inject her with a sedative. He ordered nitrous oxide if she woke up again and wouldn't cooperate.
The EEG was normal. Hannah recovered from the sedative and told Dr. Cameron they didn't have to have sedated her, but Dr. Cameron disagreed. Dr. Cameron was taking Hannah's body temperature with an oral thermometer and telling her about the normal test results when Hannah suddenly lost consciousness and went into cardiac arrest. Dr. Cameron called a code blue. She noted Hannah's temperature was 105F and she called for ice packs and cooling blankets. The nurse noted that Hannah wasn't flushed or sweating, but Dr. Cameron told the nurse her temperature reading. They doused her with ice and applied cooling packs.
They managed to lower Hannah's temperature. Dr. Cameron thought it might be an infection but a lumbar puncture showed normal proteins and no white blood cells. Dr. Chase thought it might be a problem with her liver, but her transminases were normal. Dr. Foreman thought it might be drugs, but the tox screen was clean. Dr. Cameron thought that their tests may have made her sick, so they should re-run them to see which one had an effect. Dr. House wanted to biopsy a spinal nerve, but Dr. Foreman pointed out that risking paralysis when the only symptom was fever appeared reckless. Dr. House pointed out that obviously the nerves of a CIPA patient were different, but Dr. Chase pointed out that her nerves problem was congenital and the symptom was new. Dr. House realized none of his team would do it, so he went to get Dr. Cuddy's approval.
Dr. Cameron wondered if they should tell Hannah how badly injured her mother was, but Dr. Chase recommended not letting Hannah know until it was absolutely necessary, and that it was the mother's attending who should break the news in any case. Dr. Foreman was hoping Dr. Cuddy would turn down Dr. House's request for the nerve biopsy. However, Dr. Chase noted that, in the end, no one ever turns down Dr. House. He thought the only way to prevent the biopsy was to find out some other way to diagnose Hannah. Dr. Foreman pointed out all the previous tests were normal and they had reviewed her extensive medical history. However, Dr. Chase realized something - the first thing you ask a patient is "Where does it hurt?". The location of pain is an important diagnostic tool. Hannah was insensitive to pain, but only because the nerves didn't transmit the pain to the brain. She might not be totally impervious to pain, so if they provided enough stimulus, they may be able to get her to feel it. Dr. Cameron likened it to torturing a patient, but Dr. Chase noted that with her insensitivity, it would be no more painful than pricking a finger to draw blood.
Dr. House tracked down Dr. Cuddy at a nearby restaurant to request the nerve biopsy. However, Dr. Cuddy knew that the most common complication of CIPA was infection and the fever fit perfectly with that. However, Dr. House noted her LP was clean and her urine and white blood cell count were normal. Dr. Cuddy suggested cancer, but all the scans had been clean. Dr. House told her he thought her nerves were interfering with the body's ability to thermoregulate. There were several possibilities including amyloidosis and sarcoidosis. Dr. Cuddy agreed to the biopsy.
Meanwhile, Dr. Chase had set up a test. He wanted Hannah to put her hand in both hot and lukewarm water while he performed an fMRI. A differing response to the heat could indicate a vasular problem. Hannah still wanted to know about her mother, but Dr. Chase assured her she was okay. Hannah put her hand in the hot water, but she didn't take her hand out when Dr. Chase told her to. Dr. Chase rushed in to remove it, but not before she suffered second-degree burns. Hannah was still more concerned about her mother.
Next, Dr. Foreman put Hannah into a head clamp and injected her brain with missing chemicals that should heighten her nerve sensitivity. He was looking for sarcoma. He told her bone pain was the worst there was and she needed to tell him if she felt anything. They started the procedure by drilling a hole into her skull. Hannah continued normally and talked to Dr. Foreman about all the fights she got into. She never knew when to stop. All of a sudden Hannah started screaming and tudding at the headgear. They removed the clamp. She was bleeding from the holes they drilled, and Dr. Foreman asked where it hurt. Suddenly, Hannah smiled and ran out of the room. Dr. Foreman realized she had faked the reaction to get the opportunity to leave.
Dr. Wilson came to Dr. House and admonished him for taking on the case. Although the patient was sick now, she wasn't when Dr. House agreed to take the case. Dr. Wilson figured Dr. House was curious about her lack of pain and wanted to apply that to his own chronic pain. However, Dr. Foreman rushed in to tell Dr. House that Hannah was threatening to jump from the 2nd floor balcony. Dr. House didn't see how he could help.
Dr. Cameron and Dr. Chase were trying to talk to Hannah, who was standing on the railing. They told her she was having a paranoid delusion and they weren't trying to hurt her. Hannah wanted to see her mother and they agreed to let Hannah see her if she would come down. All of a sudden, Hannah complained that she had lost feeling in her legs. She lost her balance on the railing and fell to the floor below.
Hannah regained consciousness and found herself in bed. Dr. Cameron asked her if she felt anything. Hannah had six broken bones, a concussion, fever, erratic heart rhythms, and a complete lack of sensation of any kind below the waist. However, Hannah said she felt fine and asked if her mother was out of surgery.
Dr. Cameron reported that Hannah's paranoia had disappered, but her vital signs kept getting worse and, if they continued to do so, she might only live for a few more hours. Dr. Chase was sure the paralysis caused the fall as Hannah had complained about the lack of sensation before she fell. They did an x-ray of her spine and found no damage to the spinal cord or compression from any of the vertebrae. Dr. House thought that the paralysis and paranoia indicated a neurological problem and insisted on the nerve biopsy. Dr. Chase said there were other tests they should run first, but she had tested negative for Syphilis and all other STDs, the ANA was negative for vasculitis, and there was no clot on the MRI. Dr. Cameron suggested a thyroid storm. Dr. Foreman agreed - it matched they symptoms, including her slow glucose reaction and her low potassium. Dr. House wanted to consult with an endocrinologist and was told that Dr. Bennett was on call.
However, Dr. House instead went to Dr. Cuddy's home for the consult, and she reminded Dr. House that Dr. Bennett was on call. He said he wasn't answering his phone. Dr. Cuddy pointed out that this could have been done over the phone too, but Dr. House said he had to show her the file. She thought it might be thyroid storm before she looked at the chart. Dr. House reported Hannah's hormone panel was normal, although her TSH was low. Her CPK enzymes were elevated - 275. However, Dr. Cuddy soon ruled out thyroid storm - the CPK would be higher and the low potassium could be explained by the bronchodilators they were using. She then told him that she had just spoken to Bennett and accused him of interrupting her for personal reasons and sent him away.
When Dr. House returned, Hannah was worse and Dr. House had ruled out thyroid storm. He ordered the nerve biopsy. His team still objected, but he insisted. He made his way to Dr. Wilson's office who asked if Dr. House had ordered a spinal nerve biopsy. When Dr. Cameron confirmed this, he asked to speak to Dr. House privately.
Dr. Wilson had found papers in Dr. House's office about growing nerve cells in culture. Dr. House admitted that if you grew nerves from a CIPA patient you could graft them into another patient and this might prevent them from feeling pain. Dr. Wilson reminded him he would have to be on immunosuppresants for the rest of his life. He also reminded Dr. House he was risking Hannah's life. When Dr. House pointed out that risk is part of medicine, Dr. Wilson shot back that the risk is to improve the patient's health, not the doctor's. Dr. Wilson asked if he was being objective about his decision.
Dr. House went back to his team and told them to biopsy a nerve where it would be unlikely it would result in paralysis. Dr. Cameron and Dr. Foreman did the biopsy and when Dr. Cameron viewed it showed that the insulation around the nerve bundles was almost completely missing although the individual nerves still had insulation. This indicated the nerves were being attacked from the outside - secondary demylination. There must be something outside the nerves attacking them, a systemic disease instead of a nerve disease.
Dr. Cameron left the differential to tell Hannah her mother was out of surgery. Dr. House went after her to tell her the appropriate time to do that was after the differential. He suggested it might be a metabolic disease. Dr. Cameron still went off even after Dr. House suggested getting a nurse to do it. She told Dr. House to work with the other doctors about the possibilities.
Hannah went to see her mother in intensive care. They were planning more surgery for her mother, but her mother was more concerned about Hannah. When her mother apologized for the accident, Hannah apologized for having to get her to rush her to the ER again. Her mother lost consciousness at that point. Dr. Cameron noted that Hannah's blood pressure was rising and they had to get her back to bed. All of a sudden, Hannah noticed that her hand was wet and Dr. Cameron noted that she was crying. All of sudden, Hannah started to feel pain in her head.
Dr. House added the pain to the differential and asked what it meant. Dr. Chase noted it wasn't accompanied by tingling or itching, but Dr. Foreman thought the pain was irrelevant because it was clearly emotional. However, Dr. House still wanted to know what brought on the pain. Dr. Cameron said they were arguing about whose fault it was. He focussed on the fever, peripheral neuropathy and paranoia. A lot of metabolic conditions cause those, but if the guilt about the accident was a symptom, there was only one remaining diagosis - Vitamin B-12 deficiency. Metabolic conditions often cause such symptoms, such as Alzheimer's disease causing euphoria and pain causing clinical depression. They argued about when the guilt started. Two years before, she had been a model student, now she was always fighting. Dr. Cameron thought that Hannah may be trying to show her mother she can take care of herself, but Dr. Foreman pointed out then her guilt would be rational, not a symptom. Dr. House noted that giving her Vitamin B-12 would be an easy way to check, and safe as well. However, Dr. Foreman noted that Hannah was given a routine B-12 and multivitamin shot on admission.
Dr. House went to talk to Dr. Wilson because he thought that if it wasn't a problem with B-12, it might be leukemia. Her white blood cell count was low and she had a lot of eosinophils. Dr. Wilson noted her immunoglobulin e-level was borderline and a bone marrow biopsy was recommended to confirm. However, Dr. House had already ordered one. Dr. House grabbed a bite of Dr. Wilson's sandwich and when Dr. Wilson admonished him, he thought of something.
The team was about to give Hannah nitrous oxide so she wouldn't move during the biopsy when Dr. House rushed in and told them to stop. Dr. House had realized that her symptoms started the last time they gave her nitrous oxide, during the EEG. When Dr. House ordered an MRI, Hannah once again started having paranoid delusions. Dr. House changed his plans and rushed her to an operating room. With no need to give her an anesthetic, Dr. House ordered two scalpels, forceps, iodine antiseptic and a large steel bowl. He sterilized her abdomen and started cutting. Hannah screamed but Dr. House reassured everyone she was faking and she soon stopped. Dr. House ordered a retractor and the forceps and removed a large tapeworm. Most likely, Hannah had eaten undercooked fresh water fish. The tapeworm absorbed all the B-12 she ate, leaving her with the deficiency. A normal patient with a tapeworm would be in great pain. The tapeworm was over 25 feet long, but was less than half as long as the longest ever recorded.
Hannah's mother was taken to recovery and Hannah was taken to see her. She was warned about moving too quickly, which would risk tearing her stitches.
Dr. Wilson suggested to Dr. House that he ask Hannah for a spinal nerve, but he figured that she had no reason to give it to him.