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Sample Letter of Medical Necessity for the Katie Beckett Medicaid Waiver Application

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Letter of Medical Necessity for TEFRA/KATIE BECKETT
Eliana Rachael Fier

D.O.B.   PRIMARY CARE PHYSICIAN:   Xxxxx Xxxxx M.D. address address address address
Katie Beckett Review Nurse
Georgia Health Partnership
1455 Lincoln Parkway, East
Suite 750
Atlanta, Georgia  30346-2200

Re: Eliana Rachael Fier
D.O.B.:  xx/xx/xxxx S.S.N.:  xxx-xxx-xxxx
Medicaid I.D. #:  xxxxxxxxxxxx
Age:  8 years 

Eliana Fier is a 8-year-old girl with a diagnosis of Tay-Sachs disease (330.1), a fatal genetic disorder that causes progressive destruction of the central nervous system.  As a result, she has additional diagnoses of Seizure Disorder, Hypotonia, Intermittent Hypertonicity with Spasticity, Severe Developmental Regression/Paralysis, Blindness, Swallowing Dysfunction with Aspiration, Reactive Airway Disease, Gastroesophageal Reflux Disease, H/O GI Bleed, H/O Aspiration Pneumonia, PEG feeding dependent, Delayed Gastric Motility, Constipation, Ankle Contractures, and Hip Dislocation. 

Eliana’s prognosis is poor.  No cure exists for Tay-Sachs disease.  She was not a candidate for a stem cell transplant, as determined by Joanne Kurtzberg, M.D. of Duke University. Children with this disease typically have a life span of 2-5 years.

Pediatrician:   ,name, M.D. phone  
Neurologist: ,name M.D.  phone
Pulmonologist: ,name M.D.  phone
Gastroenterologist: ,name M.D.  phone
Endocrinologist: ,name M.D.  phone
Ophthalmologist: name, MD  phone
Orthotist: name, CPO  phone


Regression of developmental milestones began at approximately age 8-9 months.  She regressed dramatically from age one to two, and she currently functions at less than a one-month-old level in her gross and fine motor functioning.  She was below the first percentile in functioning for children her age when she was 12 months old, and has continued to regress since then.  Functionally, she is essentially paralyzed, and requires complete care.  She is unable to move or lift her extremities without support, except when in an episode of clonus.  She is no longer able to lift her head while in a prone position on the floor or over an exercise ball.  She can no longer prop sit even briefly.  She is able to stand while wearing a Bennik Trunk Vest and Hip-Knee-Ankle-Foot Orthotics, and with the support of an adult.  She has had progressive visual decline and has been blind since 19 months.  She responds to voices and social situations by opening her eyes wide and attempting to turn her head toward the loudest voices.  She responds to touch of her face, but tenses when her arms or legs are stroked. Hearing appears to remain intact.  She is non-communicative, other than intermittent deep breaths and grunts when she appears to feel comfort.  She listens to books on tape and to music for stimulation. She winces and squeezes her eyes tight when in pain.    She has frequent seizures.  She appears comfortable and she is easy to sooth.  

Eliana does not take any food by mouth.  She does not swallow her secretions.  Her gag response is no longer intact.  Oral and nasal suctioning has increased in frequency.  The use of the Scopolamine patch has decreased secretions and the need for postural drainage. She has a PEG tube and receives all of her feeds and oral medications via the G-tube. She receives 3 different medications via a nebulizer around the clock (every 3 hours), multiple medications via G-Tube throughout the day, tube feedings 4 times a day over 1 -2 hours each, chest physical therapy (CPT) with the “Vest” twice a day and as needed, use of the Cough Assist machine 4-times a day, manual CPT throughout the day, nasopharyngeal suctioning throughout the day as needed, and oral suctioning as needed throughout the day.  She has delayed gastric motility and severe constipation, and has had a partial ileus.  She requires frequent venting of her G-Tube throughout the day, and occasional use of rectal enemas and digital rectal stimulation.  She has periodic urinary retention requiring bladder massage for complete bladder emptying.  She has had 3 menstrual cycles this year with severe diaper rash during those times. This requires frequent checks for skin breakdown and application of ointments on a regular basis.  She has eczema on her hands and axilla and is at risk for skin breakdown and decubitus ulcers. Her skin care needs include frequent position changes to relieve pressure points, use of moisturizing lotions one to two times a day, topical steroids as needed, and application of medicated powders. Eliana has frequent seizures and must be monitored for apnea and choking during seizure activity. She has chronic dry eyes with abrasions that are slow to heal. This requires application of eye drops every hour and eye lubricant every 2-4 hours.  Eliana requires physical therapy every day, and receives 30 minutes of therapy twice a day, as tolerated, by her mother and nurses.  

Reglan 2mg 4 times a day
Prevacid 15 mg 2X/day
Klonopin 1.0 mg 4X/day and prn
Lamictal 100 mg at 12pm and 75 mg at 12am
Baclofen 2.5 mg twice a day and 5.0mg once a day
Singulair 4 mg each night
Miralax 17 mg (one capful) a day 
Benadryl 7.5mg 4 times a day
Xopenex Nebs 0.63 mg every 3 hours and PRN
Atrovent Nebs 0.25 mg 2X/day and PRN
Pulmicort Nebs 0.25 mg twice a day
Maalox 5cc once a day and prn
Polyvisol one dropperful once a day
Scopolamine Transdermal 1.5 mg patch every 48 hours
Zavesca 100 mg 3 times a day
Ambrotose glyconutrients one scoop once a day
Peptamen Junior enteral formula 150-200 cc 4 times a day
Yogurt 2 tsp in each feeding 4 times a day
Other PRN medications:  Diastat, Tylenol, Motrin
Completed a course of Azithromycin and Prelone one week ago.

Eliana requires daily physical and occupational therapy exercises. In-home PT and OT were discontinued 1-2 years ago, as was recommended in her last evaluation.  Her last physical therapist created a personalized therapy routine for Eliana’s parents and nurses; it is a 30-60 minute session, which is should be done a few times a day.  The primary goal of therapy is to maintain good respiratory functioning to keep her alive.  This requires strengthening of oral musculature, prevention of reflux and aspiration, prevention of constipation and abdominal distention, strengthening muscles of the neck and trunk, and adequate positioning and positioning changes.  Eliana has a kid kart for seating.  She has Hip-Knee-Ankle-Foot orthotics which are used for standing.  Eliana’s caregivers provide OT with oral stimulation and hand positioning and stretching.   She wears hand splints much of the day and AFO’s at two-hour intervals. 

Eliana’s family has acquired equipment to help with her care and comfort.  She has ankle-foot orthotics (AFO’s) to prevent ankle contractures, which she wears for 2-3 hour intervals during the day and night.  She has wrist splints which she wears for 2-4 hour intervals.  She received hip-knee-ankle-foot orthotics to assist in the standing position and she uses a Bennik trunk vest to help with sitting during her exercises. She currently uses a wheelchair/stroller for seating, support, and change in positioning. She uses a reclinable jogging stroller or a wagon for transportation. She has a bath chair. She has an exercise ball and a Tumbleform roll.  

Eliana has and uses the following medical equipment:  the Vest; a Cough Assist machine; a suction machine; standard and portable nebulizers; a kangaroo tube feeding pump; standard and portable pulsoximeters; an oxygen concentrator; and oxygen canisters.

Eliana lives at home with her parents and 3 brothers (ages 15, 13, & 10).  Her father works in xxxxx.  Her mother stopped working in April 2005 due to Eliana’s increasing medical needs at home.  Eliana’s parents’ medical goals are to maximize her comfort, maintain her current motor skills and strength, and to aggressively maximize her respiratory care.  They would like to provide care at home, not in an institution.  They hope to maintain a relatively normal life for their three sons.

Eliana was born at 40 weeks gestation to a 36-year-old mother and 35-year-old father after an uneventful pregnancy.  She weighed 7lbs, 8 oz.  Hypotonia was diagnosed relatively early in infancy, with gross motor delays.  A pediatric neurologist evaluated her at 9 ? months, with a decision to follow her progress and reassess at one year of age.  A developmental assessment at Baby’s Can’t Wait at 10 months diagnosed a severe gross motor delay, and in-home physical therapy twice a week was started.  Her fine motor skills and speech started to regress.  An occupational therapy evaluation at 12 months found her to be at < 1%ile for her age for gross and fine motor development.  A speech evaluation at the same time was equally grim.  In-home OT twice a week and Speech Therapy once a week were added to her therapy schedule.  

On 11/16/03, at 12 ? months, she was admitted to the Egleston Intensive Care Unit until 11/20/2003, with a diagnosis of Status Asthmaticus.  She required oxygen, intravenous steroids, and racemic epinephrine to stabilize her.  She was readmitted to Egelston Hospital 9 days later (ER 11/29/03, inpatient 11/30/03-12/10/03) with similar symptoms.  At that time, a work-up of her hypotonia was initiated; her primary work-up was genetic.  On 12/3/03, she had a swallow study in which she aspirated thin and thick liquids; an NG tube was placed and tube feedings were started that day.  Also on 12/3/03, an abdominal ultrasound confirmed she had a slightly enlarged liver.  On 12/4/03, an ophthalmologic exam revealed a cherry red spot, which is diagnostic of a lysosomal storage disease.  On 12/5/03, enzyme studies confirmed a diagnosis of Tay-Sachs disease.  On 12/5/03, a brain MRI with SPECT showed mild cerebellar and cortical atrophy.  On 12/8/03, a barium swallow showed normal GI anatomy.  On 12/9/03, a gastrostomy tube was placed.  On 12/10/03, she was discharged home with a new feeding pump and suctioning equipment.

Her constipation was treated with a change to an elemental formula (Peptamen Jr) and the start of Miralax laxative.  Reglan was started to enhance motility.  Her family is considered a fundoplication surgery to prevent reflux, but opted against it.  

Eliana had reactive airway episodes (possibly aspiration pneumonia) every 4-6 weeks from 12-30 months of age, typically requiring a short course of prednisone, an increase in frequency of nebulizer treatments up to every 1-2 hours, frequent nasopharyngeal and oral suctioning throughout the day and night, and frequent chest physical therapy.  She also required antibiotics (Amoxicillin) a number of times.  Reglan and Miralax doses were increased to help improve gastric motility and decrease reflux.  Her tube feeds were slowed down from 180 cc/hour to 100 cc/hour.  Nebulized Pulmicort was increased to twice a day. Because of her aggressive medical care at home, she did not require inpatient hospitalization for 11 months, despite her frequent episodes of respiratory decline.  

Eliana started having myoclonic seizures in March 2004.  She also had choreoathetosis, hypertonicity and muscle rigidity.  Her seizures started as an extreme form of her exaggerated startle reflex, and progressed into generalized myoclonic seizures occurring 5 to 20 times a day. Diastat per rectum was prescribed for prolonged seizure activity. Benadryl was started to try to decrease muscle rigidity, spasticity, and choreoform movements. Klonopin was started for its combined effects of muscle relaxation and seizure control. Klonopin was well tolerated.  Its muscle relaxation effects were dramatic, and allowed Eliana to prop sit and do other PT/OT exercises with less startling, spasms, or seizures

Eliana’s vision regressed.  By April or May 2004, she could no longer recognize faces or turn toward visual stimuli, except for light.

In July 2004, Eliana had a consultation at the Duke University Pediatric Transplant Center with Dr. Joanne Kurtzberg to consider a stem cell transplant.  Because of her age and late stage of the illness, it was felt that she would not survive the chemotherapy and immunosuppression required to do this transplant.  

On November 17, 2004, Eliana was admitted to Egleston Hospital for the first time in 11 months.  She had a GI Bleed, possibly related to an extended course of prednisone used to treat severe reactive airway symptoms earlier that month.  She was also found to have dilated loops of bowel, a partial ileus, and a high fever.  She was admitted for observation, continued to have temperature spikes, and vomited and aspirated her stomach contents the next day.  

She was admitted to the ICU in respiratory distress.  She was given CPAP with her nebulizer treatments, a cooling blanket, IV fluids and other supportive care.  Temperature spikes continued even after IV antibiotics were started.  Blood and urine cultures were negative.  Blood work was normal.  Chest X-rays showed a new pneumonia, likely due to aspirating bloody emesis.  After 4-5 days, a second IV antibiotic (Cleocin) was started, with a resultant normalizing of temperature and improvement of respiratory status.  

During her stay, General Surgery followed her for her partial ileus and decompressed her bowels twice using a rectal tube.  She was transferred to a general floor for 4 more days where she continued to stabilize.  Work-up for her GI Bleed was never done due the risks of anesthesia required for an endoscopy, and because she stopped bleeding.  She required a PIC line for TPN after her 5th day with only peripheral nutrition; this was taken out before discharge.  She resumed Peptamen feeds without difficulty.  Prilosec was changed to Prevacid.  All other medications stayed the same. 

She was discharged on November 27, 2004.  Her IV Cleocin was changed to oral Cleocin, which she took for 5 more days.  Approximately 6 weeks later, she developed bloody stools. This was thought to be a colitis caused by Cleocin.  This was watched closely.  Yogurt was added to her feeds 4 times per day as a probiotic.

Eliana had not been hospitalized for the following 5 years and 2 months.  On January 13, 2010, she was admitted for less than 24 hours because her G-tube Genie button had to be removed in the operating room.  It was replaced with a Mickey button with no complications.  

Although she was not admitted to the hospital over that 5-year period of time, Eliana had serious respiratory episodes requiring oxygen, hourly nebulizer treatments, steroids, antibiotics, and around the clock chest physical therapy.  She was treated at home and was given an ICU level of care.  Her medical course, by system, is given below.

Neurological Status
* Increases in seizure medication, Klonopin.  Currently takes 4.0 mg/day in divided doses.  
* During the 2006-07 winter, she started biting her tongue and required a mouth guard to prevent bleeding and aspiration of blood into the lungs. Baclofen was started to decrease muscle tone, which it did, but it also increased seizure activity.  The subsequent increases in Klonopin helped. 
* Lamictal was started in June 2007 and was gradually increased to 30mg 2X/day.  This medication helped relax her jaw muscles and decreased tongue biting.  It also helped relax her at the hip and ankles.  In July 2008, the dose was increased slowly to 45mg 2X/day. Lamictal was titrated up due to apneic episodes related to seizure activity.  She currently takes a total of 175 mg/day.
* Eliana has seizures throughout the day.  She must be monitored constantly for seizure activity, episodes of apnea that may accompany seizures, and choking on secretions during seizures.  She cannot be left unattended.  

Infectious Disease Status
* Eliana has had many infections including bacterial pneumonia, aspiration pneumonia, strep throat, Roseola, Otitis media, sinusitis, and GI infections. Infections with fever result in severe tachycardia, tachypnea, reactive airway symptoms, drops in oxygen saturations, and an increase in seizure activity.  She requires an ICU level of supportive care in the home to keep her out of the hospital.  She has been treated with the antibiotics Cleocin, Amoxicillin, Augmentin, Keflex and Azithromycin.  At the end of February through March 2011, she took a course of Azithromycin.
* Eliana is at risk for common childhood illnesses, however, her underlying disease places her at very high risk from a common infection.  During these episodes, she receives more frequent (Q1-2hours) nebulizer treatments, around-the-clock manual chest physical therapy, oral suctioning, and oxygen, in addition to her baseline Gastrointestinal, Neurological, Nutritional, and Respiratory care.

Gastrointestinal Status  
* Reflux. 
o She requires constant monitoring for reflux and gagging, which has resulted in choking and aspiration many times.  
o Since October 2005, she is rarely left in an upright position or on her back, due to her inability to forewarn the caregiver with sounds of discomfort.  She must be placed on her side or on her abdomen to allow her respiratory secretions or vomit to flow out, without the threat of aspiration into the lungs.  
o Eliana’s reflux is exacerbated by her constipation, gastric distention, delayed gastric motility and seizures.  She is taking Prevacid. 
* GI Bleeding.  
o Aggressive monitoring, prevention, and treatment of GI bleeding were required in 2005.  
o The bleeding has occurred sporadically, varies in intensity, and is often related to pressure or rubbing while lying on her stomach.  
o Gastric contents are examined every 6 hours.  
o If blood is noted, the stomach is rinsed and the contents reexamined for an active bleed. 
o  Maalox is used to protect the stomach if the bleed is mild.  She takes Prevacid twice a day. 
* Colitis with Bloody Stools.  November –December 2005.  
o Eliana has since taken Yogurt with her feeds as a probiotic agent.
* Constipation; Digital Rectal Stimulation. 
o Constipation continues.  Eliana takes Miralax every day.  Reglan helps to promote gastric motility, Yogurt helps as a probiotic agent, and Peptamen Junior is used because it is predigested and easier to tolerate.  Bowel functioning is monitored closely to avoid gastric distention and its dangerous sequellae.  She has required digital rectal stimulation 2-3 times a week when when her abdomen is distended.  
* Distended loops of bowel in upper abdomen as indicated by X-Ray in summer 2008.  This was thought to be exacerbated by the use of the Cough Assist machine.  Since then, gastric venting has been done after Cough Assist use with good results.  Since May of 2010, gastric distention has improved, her abdomen has been softer and flatter, and she has seemed more comfortable.

Pulmonary Status
* Eliana’s pulmonary status continues to weaken and she is always at risk for decompensation.  Her lungs are hyperinflated, she has clubbing of her fingers and toes, and she used to have tachypnea at baseline.  Over the past two years, she has had slower respiratory rates, drops in oxygen saturations, and periods of apnea.
* Increased demand for oxygen:  Eliana has portable oxygen tanks and an oxygen concentrator at home.  Oxygen is needed with even the mildest cold symptoms or a one-time fever spike.  Eliana was most recently on oxygen in February and March of 2011.  She needed 2 Liters per minute as a maximum.  Oxygen was weaned down and discontinued on March 5, 2011.
* Increased demands for Nebulizer treatments:
o Eliana’s nebulizer treatments have not been weaned from the every 3-hour schedule because of increased secretions and distress when given at longer intervals. 
* Positioning needs to address decline in pulmonary status:
o Eliana cannot be left on her back or seated in a chair without direct supervision. Trendelenberg positioning helps drain secretions.  Frequent position changes from side to side are needed to prevent consolidation of fluid in the lungs.
* Transdermal Scopolamine is used: one full patch (1.5mg) applied to the neck region every 48 hours to decrease oral secretions.  This has been very effective.
* Atrovent is now taken twice a day instead of once in order to dry secretions.
* Since January 2007, a Cough Assist machine has been used every 6 hours before feeds.
* Eliana uses the Vest Airway Clearance System twice a day and more as needed.

Skin/Mucus Membranes
* Eliana has had recurring sties on her eyelids, requiring close monitoring, keeping her eyes moist with manually assisted blinking and eye drops, and the applications of warm compresses.  
* In July, 2009, an abrasion was noted on her left eye.  She has been treated by Dr. Bordenca, an ophthalmologist. It was treated as an infection at first.  When it did not heal, aggressive treatment with artificial tears and Lacrilube were started.  In addition, she had her left eye glued partially shut due to her lack of blinking and need to keep her eye moist; this was done almost weekly for a while. On October 27, 2009, she had an outpatient procedure done at Scottish Rite Hospital in which her left eye was sewn partially shut.  The left eye remains half shut and the skin on the upper and lower lids have fused together nicely.  
* Since the fall of 2010, she has had a developing opacity on her right eye.  She is given artificial tears to both eyes every hour and Lacrilube ointment 3 times a day. Because it has not improved, she will be seeing Dr. Bordenca on March 17, 2011 to evaluate the need for surgery on her right eye. 
* Eliana requires aggressive oral care, due to dry mouth, lack of oral feeds, and inhaled steroid treatment.  She is at risk for oral thrush because of the use of Pulmicort twice a day.  Her teeth, tongue, and cheeks must be brushed and washed after each treatment and as needed. 
* In early 2007, as her ankle contractures were worsening, Eliana had skin breakdown on her feet, due to rubbing of her AFOs and possibly due to poor circulation.  Baclofen was started to decrease tone, but then seizures worsened.  The increases in Klonopin and the start of Lamictal both seemed to offset the seizures and decrease tone at the ankle, which improved the skin breakdown.  In 2008, she had skin breakdown on her ankles periodically requiring foot elevation, warm water soaks, and careful monitoring for skin infection.  She can develop skin breakdown on the heels of her feet if her AFO’s are kept on too long and when her range of motion exercises were done too aggressively. 
* Eliana gets serious skin irritation in the groin, buttocks, and axillary areas.  It worsens during periods prior to starting a menstrual cycle.  It is treated with zinc creams, topical steroids and medicated powder. 

Neuromuscular Status
* Eliana has AFO devices to prevent painful contractures of the ankles.  She should wear these often, but must be monitored for redness and skin breakdown at pressure points.
* Eliana was prescribed wrist splints in early 2007 due to wrist contractures.  
* Eliana has HKAFO’s (hip-knee-ankle-foot orthotics) which allow her to stand upright.
* Eliana requires physical and occupational therapy exercises throughout the day as tolerated to maintain range of motion and existing muscle tone.  

Urologic Status
* Eliana sometimes retains urine for up to 8-12 hours, thought she typically urinates at regular intervals.  She has not had any known urinary track infections and has not required catheterization yet. This is monitored closely. Bladder massage is used as needed to help empty the bladder, and this has been effective.  During PMS, her abdomen becomes more bloated and distended, helping her to urinate on her own.

Endocrine Status
* Eliana has precocious puberty, which started with breast buds at the age of four.  She now has axillary and pubic hair.  She developed periodic vaginal discharge at age 6 or 7.  In May of 2010, at the age of 7 ?, she had symptoms of premenstrual syndrome. She had a small red dischage which was assumed to be her first menses.  She has had similar episodes in August 2010 and January 2011.
* Eliana was evaluated by an endocrinologist in December of 2007, at the age of 5.  Lab tests confirmed precocious puberty, which was felt to be related to her seizure disorder, with seizure activity near the pituitary gland.  Her family was offered treatment with monthly Lupron injections, but they opted against it.  
* Eliana has severe body temperature dysregulation, which is seen in end stage Tay-Sachs disease.  Body temperature and room temperature are monitored and adjusted carefully. Care must be taken when she is in the sun, as her temperature can shoot up to 103 degrees in less than 30 minutes.  She is also monitored closely when it is cold, as her body temperatures will go down to 92 degrees very rapidly.

Eliana Fier is under the age of 18 and is ineligible for SSI.  She is in need of nursing home care, but can receive equivalent care at home at a lower cost.  

Eliana requires 24-hour a day medical care.  She is chronically impaired and would be a suitable candidate for nursing home care if her family did not have the resources to care for her at home.  She is unable to move or speak, and lies limp in bed if she is not receiving PT/OT exercises, sitting in her wheelchair, or standing in her HKAFOs with assistance.  She is at high risk for skin break down and decubitus ulcers.  She is incontinent of urine and feces.  She has seizures daily, which vary in quality and intensity.  Slight movements, positioning changes, or touching her legs can trigger an episode of spasticity and clonus.  She frequently gags on thick secretions.  She has coughing jags when she cannot bring up secretions; this can stimulate her gag reflex and cause vomiting or reflux of stomach contents.  She requires repositioning to prevent accumulation of secretions in her mouth and throat.  She must have her suction machine at her side at all times, and she requires deep nasal and oral suctioning around the clock. She needs the Cough Assist machine (approximately 5-6 “sessions” with 4-5 rounds per session) at least every six hours to help bring up secretions.  Postural drainage in the Trendelenberg position is done when suctioning is ineffective in clearing secretions.  She receives nebulizer treatments every three hours around the clock, and frequent chest physical therapy.  She receives all oral medications by a feeding tube.  She receives 100% of her nutrition from enteral feedings by a feeding tube every 6 hours around the clock.  

Eliana is a severely medically fragile child who requires ongoing complex medical care.  She requires constant observation and assessment as even slight changes in her medical condition lead to instability and could result in death.  She is totally dependent in all activities of daily living.  Her condition is expected to worsen over the course of the year.  Her prognosis is poor.

Eliana’s parents are trained and capable of caring for her medical/nursing needs at home.  Her mother is a licensed physician who left her job as a psychiatrist to care for her.  Licensed Practical Nurses assist in Eliana’s daily care.  Her room has been set up to provide medical care for a terminally ill child as it has its own sink and refrigerator, and ample room for equipment.  Eliana has the VEST, a cough assist machine, a standard nebulizer, a portable, battery powered nebulizer, a bath chair, a wheelchair, orthotics, and exercise equipment.  The family rents a feeding pump, suction machine, pulsoximeter, and oxygen concentrator.  She is receiving appropriate medical care and nursing care in her home.

Thank you for your review of Eliana Fier.  Please call me with any questions at xxx-xxx-xxxx.


Xxxxxx Xxxxxx, M.D.
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