Patient from Boston

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The patient is from Boston and she pronounces the word head with two syllables. She wears her grey hair short around her face and the frames of her glasses are stylish and complement her eyes. Her spouse is small and Italian. He wears a hearing aid in his left ear, a turquoise polo shirt and he speaks loudly with the accent of a hitman. She scans the three women observing and tells them not to get old. She is seventy- five, but has the energy and demeanor of someone younger.
She says she has trouble eating, and adds perhaps that’s not a bad thing; referring to her rounded shape. The doctor asks exactly what seems to be the problem, because the typical parkinsonian tremble occurs at rest, not when engaged in activity. That sort of tremor is an action tremor, or an essential tremor, what Katherine Hepburn suffered from. He asks her to perform several dexterity measures, and her tremor appears slight, hardly incapacitating. The physician points at her and tells her she is not being honest with him and she concedes, that she feels the tremor is larger than it perhaps appears.
Though she currently takes two and a half pills of Sinemet three times a day, she feels little effect from the medication. The doctor asks what she eats for breakfast, and she tells him she had a bowl of cereal with milk. His eye brows shoot up and he exclaims the amino acid building blocks of proteins in the milk compete with the levodopa for access from blood to brain. That may be why she has noted no benefit from the medication. If she is uncertain whether her medications are working, she should attempt a purely vegetarian diet for a week or two, with as little protein as possible to see if the levodopa is working to alleviate her symptoms. A second solution is to increase the dosage of Sinemet (levodopa/carbidopa). He tests her arms and feels some cogwheel rigidity in the muscles. She writes a sentence for him to demonstrate the state of her penmanship, and her handwriting is miniscule. Yes, the physician nods, her writing is typical of someone with Parkinson’s.
The doctor asks the spouse whether he sees any improvement in his wife’s symptoms and he shrugs. He comments later she has told him she feels unable to move from her chair, and the physician nods and states initiation of movement is hard for those with PD, though when they get moving it becomes hard to stop. She mentions she exercises every morning and the other day performed thirty-one repetitions of sitting to standing. The physician praises her for exercising and encourages her to continue, telling her that is the best thing she can to for herself. He writes a new dosage schedule to increase her medication and arranges for a followup visit.

My Dad and PD

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For brief seconds, it appears he wears makeup to convince the world he is almost eighty. Odd patches of increased pigmentation dots his cheeks, brow and hairline. What is this disguise and why is my father wearing this old face?

In December he will turn eighty and my sisters would like to visit and celebrate. He sees no reason for a party, whether we would travel a hundred miles, or thousands. We discuss this on the walk home from the Indian restaurant; one of the few places open on a Sunday evening. His right knee is new, he calls it his DuPont knee. His swing is somewhat shorter on this side than the left, his shoe hits the ground at mid-foot under him, rather than hitting with his heel, in front. For the past few days, the left knee has been bothering him. He can’t recall how he might have strained it. The pace is slow, half as slow as the normal walking tempo. His chin tends to drift downward, and his gaze turns towards the ground, and his wife reminds him to put his chin up, and for a bit, his eyes settle on the scenery in front of him.

I see the tremor in his left hand when he is due for medication. He performs most movements slowly; bringing individual salads to the dinner table one at a time, rather than carrying a salad bowl in either hand. When he holds a mug or cup, the liquid inside lists precariously to the side.

In the middle of the night I hear him talking in his sleep. He’s having a conversation with someone. He talks loudly and articulately, then pauses as though listening for the person’s reply. He’s speaking again, and I wonder whether he’s really on the phone. I don’t understand the words, only the cadence of sentences and I drift back to sleep wondering about all the times I heard my father on the phone, while I slept in the adjoining room. Later he tells me the house we lived in had only eight hundred square feet.

When he was younger he cut his hair short, so short he looked almost balding. Now his straight white hair stands up. It covers his entire head and I muss my hand through it and tell him his hair is wonderful longer. His eyebrows retain some darker shades of grey and grow like wild bushes high and curling towards his brow. He seems content. When I ask him what he is doing when I catch him in his chair, he tells me he ‘s sitting. I take the chair in the other corner and sit with him.

Couple from Rotan

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The couple comes from Rotan, the islands off the coast on Honduras, to see the movement disorder specialist; there are no practicing neurologists in the country. The patient is rugged, tall and has a receding hairline and wide shoulders. His wife is his equal in beauty, slender with dark long hair, pale fair skin and a thick very English accent. She makes eye contact easily with the physician behind the desk, while her spouse’s eyes travel about the small room with fluorescent lighting and insufficient ventilation.
As the doctor sits, the wife explains it has been about six months since her husband first complained about the the fourth child in their home. She adds they have three children; the youngest is an eight year-old boy. It was dinnertime and the family sat down together. The fourth child was a boy named Hector, and he was making a horrible mess with the spaghetti on his plate. Her spouse became enraged at the illusory boy, for not responding when he insisted on him using a napkin. Later on in the evening she tried to sooth her spouse by telling him he must have been having a hallucination, because they have only three children. When her spouse had calmed he recognized they did truly have only three children, but he remained confused about the boy at the table.
His eyes drift to the doctor and he explains there is something wrong, and it began perhaps nine months previous, when he noticed himself unsteady on his feet. He had been playing golf, had drunk a beer and fell in a sand pit. The unsteadiness remained and never completely went away, though sometimes he is less stable than others.
The physician asks whether he feels stiff, and he nods and adds he feels slow, like a man older than he is. He feels slow in the head, sometimes forgetting his children’s names. In her seat the wife states her spouse is thirty- eight and used to be a professional rugby player, and remains active, completing his seventh Iron Man competition six months ago.
The doctor inquires whether he trained for the event through the symptoms, and he nods agreeing, stating his times were proof there was something wrong- he finished more than an two hours later than his previous race. The physician approaches the patient asking him to sit on the examination table. He looks at his eyes, asking the patient to follow the pen as he moves it within the limits of his gaze. He brings the pen up high above the patient’s eyebrows and the brown eyes travel some before they move no higher. And he has difficulty gazing downwards without flexing his neck forward. The physician comments that he appears unable to gaze in vertical directions. The symptom has a name, supranuclear palsy, which occurs in a variant of Parkinson’s disease known as Progressive Supranuclear Palsy (PSP).
In the hallway the patient walks with narrow based small steps. The physician explains after watching the patient, the syndrome derives its name for the characteristic traits of PD, along with several other notable problems- rigidity, especially of the axial muscles (neck and back), postural instability, and impaired voluntary movement of the eyes. In PSP, there may be a mild dementia, but in his case, the hallucinations and dementia make the Doctor suspicious of two other parkinson plus syndromes: cortical-basal-ganglionic degeneration and dementia with Lewy bodies. In dementia with Lewy bodies, the hallucinations can occur even before medication is started. In cortical-basal-ganglionic degeneration the parkinsonism is complicated by problems with voluntary gaze and dementia. In all of these parkinson plus cases, some patients respond to Sinemet, feeling decreased rigidity and slowness, though there is no cure for the progressive illness. The wife looks at her hands in her lap, as her husband asks whether anything he can do would have an effect on the course of the disease. The doctor looks at him directly and says the majority of patients who receive the diagnosis require some type of walking aid after three to five years, and are either wheelchair or bed bound in eight years. The doctor looks down at his own hands momentarily and comments all the physical activity he gets has probably forestalled the illness to some degree, and he should remain as active as possible and the medication may promote movement, by making him feel less rigid. The patient nods and the physician explains how to gradually increase the dose to achieve a therapeutic effect.

Tremor Predominant Parkinson’s Disease

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The patient who is a stocky wide- shouldered, healthy looking man, says he’s never slept through an entire night in his life. While making an extraordinary statement his face remains unexpressive, placid. His eyebrows and mouth convey no indication of emotion. The physician behind the desk shakes his head and comments the need for sleep varies considerably between people. Though few people respond to Seroquel so strangely. The first time the patient took the drug he slept for twenty-four hours straight. He describes trembling with cold for several hours on one occasion, on a separate night he blames the prescription for extreme tension, which made him pace through the house for hours. Clearly, he responds aberrantly to the medication.
The wife comments he thrashes during the night, sometimes calling out or shouting in his sleep; she reports this occurs three to four times a month. The doctor comments no one with PD has a normal sleep pattern and he describes how rapid eye movement sleep shifts to become out of phase with other sleep architecture- or wave patterns, allowing the person with PD to be able to move while dreaming. Essentially, the person is able to enact dream content, which is not possible in someone else whose sleep architecture is unaffected by illness.
The wife states they have been married forty- seven years. Her spouse leans towards her and comments they have been fighting for forty- six. An amiable banter flows between them. The wife offhandedly states her husband is an artist and describes the fine work he has been involved in, commenting he never has tremor when he’s working, it’s only later on when he’s resting. The physician responds,
“… that’s why it’s called a resting tremor…”
The patient asks whether he can increase the Mirapex he takes while experiencing stressful events, for example he is due to undergo eye surgery to for glaucoma- to release pressure on the orbits of his eyes. It requires the ophthalmologist create holes in the membrane at the back of the eye. The doctor with a head full of wavy and graying hair nods,
“Certainly, there is no contraindication to the drug.”
On physical examination, the doctor finds some rigidity on the man’s left side, and in the neck. When asked to raise his shoulders, he barely shrugs. His walk has little arm swing on either side, and when they retake their seats, the doctor asks how long ago was it when you first came in?
The wife thinks it was 2008, and the records on the computer confirm her guess. In 2008 the patient stated he had experienced symptoms of illness for five years prior to visiting a neurologist. He still gets sufficient symptomatic relief from Mirapex (pramipexole) a dopamine agonist, to avoid Sinemet. The doctor comments that most people require Sinemet after a year or two of treatment with an agonist, and he is an unusually fortunate case.

Teacher with Drug-Induced Parkinsonism

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The patient taught middle school for forty years, and she sits without leaning on the back of her chair. She comes for a consultation about whether she has Parkinson’s disease. Her husband has come with her, as a witness to the changes he has seen in her health. Her falls scared both of them. In the most recent, the patient carried groceries in each arm. She fell straight forward and broke her nose. When on the floor she was unable to rise without assistance.

The medical history of the patient has some red flags for the doctor; the patient doused her garden with spectricide and the toxin caused her thyroid to quit functioning. He mentions that there is a relationship, though not a causal one, between Parkinson’s disease and exposure to pesticides, heavy metals and other environmental toxins. She comments she has suffered from trigeminal neuralgia, inflammation of the fifth cranial nerve that produces intermittent bouts of shooting pain to the side of her face and jaw. Her husband notes he has seen tremor in her hands and a stiff walk, while the patient says she has experienced left- sided weakness, fatigue, forgetfulness, and problems with bladder control.

The patient has taken some medications that may have deleterious side effects. Pravastatin, being one of the cholesterol- lowering statins, recently made the news for its under- reported tendency to invoke muscle pain and weakness, especially in the legs. Her primary care doctor added Abilify, a novel tranquilizer to her medications when he thought Prozac was inadequate for her symptoms of depression. Abilify can block dopamine receptors and produce some signs and symptoms of parkinsonism and in addition can induce tardive dyskinesia in patients, uncontrollable movements of the face, tongue or other body parts and these may wane if discontinued, or become permanent with continued treatment.

On physical examination, the doctor finds no stiffness or rigidity in her muscles, and her gait is normal with a full arm swing. He comments that he can detect no signs of parkinsonism. When he places a tuning fork on the bones of her foot, and she is unable to feel the metal buzzing, though she can feel the vibration in the knuckles of her hands. She is able to discern whether her toes are up or down, but her perception of temperature is also impaired. The doctor tells her that she does not have the clinical features of Parkinson’s disease now. She may have had some symptoms of parkinsonism while taking Abilify, but those have gone away after stopping the medication. Based on her examination he diagnoses a peripheral neuropathy to explain some of her symptoms. The cause of her neuropathy will require more extensive evaluation. A B-12 deficiency, low thyroid function, medications or toxic insult are possible causes of neuropathy. He conjectures a toxic bath, like the kind she experienced, might result in a neuropathy, though the lower extremity problem resembles what a diabetic patient might incur. He urges her to see another physician whose specialty is the peripheral nervous system. He hands the patient and her husband a referral form. The other specialist will thoroughly explore the function of other nerves (nerve conduction studies), and order the appropriate blood and other tests that will aid in ruling out other disorders.

Respiratory Dyskinesia

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She calls frequently seeking help for irregular, fast chest wall and diaphragm movements that make her feel she can’t breathe and that death is imminent. The doctor explains that these irregular movements are leveodopa-induced dyskinesias, known by experts in Parkinson’s Disease as respiratory dyskinesias. She wasn’t due back for another four months. Her caregiver, a woman with dark blonde bushy hair, takes care of her medications, pulling bottles from her large bag to check how many refills remain.

The problem is the pharmacist. He cancels the previous levodopa medication when she gives him a fresh prescription for a new type of dopamine replacement. The doctor throws his hands up at this, exclaiming the job of the pharmacy is to fill the prescription correctly, not to judge if a medication should be replaced.

“They should have called me, if there was some discrepancy about the medication.” His jaw set, his hands express his agitation. The patient shakes her head conceding it is an awful situation.

She is prepared though, having brought a list of concerns with her. What can be done for constipation? The problem affects most people with PD. The remedies are not consistently reliable and the doctor urges the patient to incorporate all the methods; eat prunes, drink enough water, eat enough fiber, and get regular exercise. Miralax taken daily helps pass stool by increasing its fluid content. Other patients have tried senna leaf teas, though they can induce abdominal cramping and diarrhea.

The patient worries her blood pressure gets too high during the day- sometimes rising to a systolic level of 180. The doctor mentions she takes a medication to regulate her high pressure, and should not worry needlessly. The medication, Sinemet tends to reduce blood pressure further.

The physician leans back in his chair and urges the patient must find an outlet for her anxieties, and that yoga might be helpful in controlling the dyskinesias that obstruct her ability to breathe. He mentions the mind – body connection, asking the caregiver for the usual time dyskinesia’s are worse. She replies when they have a visitor coming, when the patient gets angry or frustrated, or any other stressful time.

Second Opinion

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The dark glasses he wears lie on the physician’s desk in front of him; he had cataracts removed from both eyes, but he still suffers from macular degeneration- a condition where center field of vision blurs and eventually leads to blindness. His visit is a result of a recent diagnosis of parkinsonism. He has come for another opinion.

Losing his hearing, the patient asks the doctor to speak up, interrupting him, as he speaks. The doctor repeats himself, with abbreviated thoughts. The patient holds his palm up towards the doctor, interrupting, telling him to let him speak. His hands are big, his finger long. Several of the fingernails on his left hand are cropped off, midway through the nail. He is 88 years old and expects to live to live to 120. An American chess champion in the over 75-year division, he visits Florida once a year to compete. He claims in his youth he could play ten games blindfolded, now he can play only one game this way; his short term memory is not what it was. But it is the tremor in his hands that bothers him, especially when eating soup. Three years ago, he noticed his handwriting became larger and shakier. He comments also he has lost the bounce in his step; he no longer rises up onto his forefoot when he walks. Balancing is tricky.

The doctor stands and takes the man’s hand, and folds it inward towards his shoulder and out. He tells the patient his upper body is supple, without rigidity. The patient concedes he was a magician, and takes a packet of cards from a small leather case in his trouser pocket. He describes a trick he was able to do with one hand, holding the deck of cards divided into two bundles, he was able to shuffle them with one hand. Standing, he positions the cards in his left hand, and then nothing happens. The doctor follows his actions, and nods, understanding dexterity is gone from his hands. The doctor assesses the patient’s sensation with a large tuning fork asking whether he can sense the vibration of the fork, when applied to the bony prominences of his feet and legs. Noteworthy, the patient fails to feel vibration applied to the right leg. The physician explains it is a cheap and easy way of assessing the integrity of the long sensory nerves in the body, and states the lack of sensation explains some of the change in his walking style, as he appears to have a mild sensory neuropathy. The cause, the physician guesses is from compression of the nerve roots in the spine. The doctor explains we rely on three mechanisms to keep us upright in space; position sense derived from the sensory nerves in our limbs that pick up vibration, fine touch and temperature; our vestibular system of the inner ear and our vision.

When the physician summarizes his findings, he notes the patient has a mild action tremor, and a mild sensory abnormality in the right leg and foot. He would like an MRI to look at the blood vessels of the brain. However, the patient leaves on Thursday to his home state, he’d prefer to have the testing performed there. The doctor agrees to send notes to the physicians involved in his care and the conversation shifts to what sort of cutting edge therapies exist in the field of Parkinson’s and Alzheimer’s disease. Sitting behind the desk, the physician explains an experimental study in which people with Alzheimer’s are receiving GCSF (granulocyte- colony stimulating factor) to remove the amyloid plaques from the brain and improve cognition. The patient voices some interest in undergoing the same treatment, and the doctor wonders whether that would be ethical, or even practical, as the patient lacks the symptoms of those with the illness. He also notes when the amyloid is removed it can get stuck in small blood vessels, and result in micro-hemorrhages. He is unsure of the consequences of such trauma in the brain of a healthy, yet older individual. The man, wearing a woolen red sweater over a collared shirt, reaches into a file and withdraws the list of therapies he receives regularly from a physician whose specialty is aging. In the second or third line is a product called Neupogen, the same substance used in the research study for patients with Alzheimer’s.

Small and Golden-haired

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He’s small, golden haired and his voice is soft. Sitting in the chair opposite the movement disorders expert, his left hand jiggles as he rests it in his lap. After having a heart attack and triple by- pass surgery he noticed the tremor in his left hand. Sometimes now, he feels the toes of his left foot curl inward and his right hand seems shaky. An optician, he needs steady hands when he must retrieve an object from a patient’s eye. Sometimes his voice slurs, and he has to carefully enunciate his words, otherwise patients do not understand him. He and his wife have formed a plan; he plans to retire and give his practice to a younger man who has been working with him for several years.
The physician comments many people observe the first signs of illness after a trauma. The incident may be psychological, as in the death of a loved one, or a physical insult, as in a car accident. Both types of trauma exert an impact on the physical wellness of a person, allowing symptoms gone unnoticed, to suddenly become apparent. In Parkinson’s disease, physical manifestation of the disease presents when the majority of dopamine producing cells in the substantia nigra have died away.
Unlike other patients that come with a multitude of problems, typically… diabetes, high blood pressure, gastric reflux, degeneration of the disks in the back, diminished sensation in the feet, brought on by chronic high blood sugar, this patient appears healthy. He is slim, fit and well groomed. The pink button down shirt suits him. His voice is low, lacking volume, – comments the doctor, who asks whether this has always been the case. No, he admits, his voice has become much softer.
On physical evaluation the doctor finds minor rigidity in the left hand. A suggestion of rigidity is barely perceptible on the right side. In evaluating gait, the patient holds his left arm much more rigidly to his side, than the right that swings naturally with his stride. Fine motor movements decrease in amplitude and slow with repetition on the left side. Reflexes of both legs and arms are brisk, suggesting some other agent of illness at work. Parkinson’s disease patients who do not have other medical problems have normal reflexes. The physician asks whether he has had an MRI, and the patient states the most recent test reported areas of ischemia in the brain. The specialist shakes his head, agreeing the finding of brisk reflexes suggests he may have suffered a small lacunar stroke.
The doctor encourages the patient to exercise aerobically at least three times a week. He cites a research study conducted on parkinsonian monkey, in which they were trained to run on a treadmill. The running time gradually increased until they were jogging up to three hours per day. Animals who exercised were able to overcome the weakness and rigidity on one side of the body while the animals who were sedentary continued to drag one side of the body. When all the animals were sacrificed, and researchers found that those animals who had become athletes, had caused their remaining dopaminergic cells to re- sprout….Hence solid evidence, at least in monkeys, that regular aerobic exercise is good for the brain.

Dangly Earrings

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With reddish hair cut short and dangly earrings the patient sits as the doctor skims the pages of information she has filled in. Her right hand moves gently in her lap. He gazes up at her and asks what brings her to the movement disorders clinic. She looks down at her hands in her lap and raises her right hand to exhibit the fine tremble occurring between her two first fingers and thumb. As she does so the movement disappears. The physician asks when she first noticed the tremor and the woman replies it has been there awhile. At first she thought it was simply nerves, but when it kept reappearing she grew concerned and spoke with her father on the telephone. He was diagnosed with Parkinson’s disease when he was sixty five. She is forty-eight.

The physician explains to be diagnosed with PD one must have two of the three principle symptoms, and respond positively to the drug levodopa. The three classical symptoms are slowness, rigidity and tremor. Her emotions overtake her and she cries, taking a tissue from her bag, the doctor continues explaining there are far worse illnesses to be given, though there is still no cure for the progressive neurological disease. When she inquires whether her children are at risk, he comments the hereditary component is present in a minority of patients, though some studies have found a genetic component for the disease.

The doctor asks about other symptoms and the patient sighs and responds she has trouble sleeping, but has had the problem ever since she had children. Sometimes, she concedes she feels slow in the head. When she and her husband go to the movies she frequently doesn’t understand the humor. Across the desk the doctor smiles and asks whether she can recall the last show she went to. The patient dabs her eyes as her gaze drifts above the physician’s head, in thought, and she smiles ruefully unable to recall the title.

Switching seats to the examination table he asks her to raise her shoulders in a shrug. Her neck seemingly vanishes, her shoulders are so supple. Her arms and wrists however have some stiffness in the surrounding muscles. As he works to assess her rigidity, the doctor speaks about the importance of exercise. She smiles and remembers the title of the movie, Hangover Two. He commends her choice of cinema, affirming laughter is good. As he speaks her smile fades and he wonders aloud whether she is taking an antidepressant. She shakes her head no, telling him she prefers to take as little medication as possible. The physician concedes most of his PD patients eventually need an antidepressant to function at their highest quality of life. The patient looks at him without comment.

Time and a Scooter

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She is blond and well tanned. Her spouse wears green trousers a matching polo shirt. They have been married for forty nine years, which explains some of their dynamics. The husband scoffs at his wife and rolls himself to the side of his chair, away from her. She conveys he has lost some memory. The wife knows the facts and conveys them easily without stammering. She answers many of the doctor’s questions, when she sees her spouse stuck on a syllable. Like the small steps the feet take, the person with PD may have problems articulating thoughts- both repeat a motion, though little change occurs.

The doctor asks about the schedule of medications, and the wife replies that her spouse may forget a dose when occupied, so the schedule is constantly changing, though he strives to take his pills every three hours. The doctor rips a page from the pad of paper on his desk and begins constructing a table that would make up a medication diary. He states he can be of little use unless he is aware of how the patient responds to his medication; how long it takes for the pills to take effect, whether he experiences dyskinesias, and when they occur. He asks the wife to attempt the diary for a period of two weeks, so that he can see where patterns emerge. Email it to me, he says. With that information they can modify the daily course of drugs.

She is pleased that the physician has discouraged the two doses of night- time medication. The doctor insists the patient must have six hours of sleep nightly, at least. To make this a possibility he recommends the drug Seroquel, to be taken in gradually increasing quantities until the patient finds he is sleeping through the entire night. An enlarged prostate means the man must rise to urinate several times in the night; Depends may be needed when the quality of sleep improves.

Diagnosed with the illness fifteen years ago, the gentleman underwent deep brain stimulation surgery a year and a half ago. The physician asks whether they have seen an improvement in symptoms, and the wife shakes her head, doubtful. Then she notes her spouse no longer has tremor at all. While he demonstrates his gait in the hallway outside the office, the patient’s arms swing freely. The arm swing, the wife notes is also much better, he used to carry his right arm next to his torso. He takes the same amount of dopamine replacement.

After the physical exam the wife mentions her spouse fell in a field and she was unable to help him to his feet. They had to wait for some time, until another person appeared to assist. She worries he will fall again and wonders whether the physician can help them acquire a motorized scooter. The doctor writes the couple a prescription for physical therapy, with attention to gait and balance with the request to evaluate and fit the patient with motorized scooter. The patient comments to the physician he is unlike other doctors, he has given them some time.

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