Sabine - Crohn Disease

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Background

Concise Summary

Sabene is a 33 year old afroamericoaboriginocaucasian single female with no children. She currently works at a flower shop and lives with Crohn's disease. Sabene first experienced minor symptoms of Crohn's disease when she was 15, but largely ignored them until the age of 19. At this time, Sabene began to feel uncommon stomach aches, and reported to her family physician. Over the next few years, Sabene was treated with a high-dairy and high-fibre diet, though this did not alleviate her symptoms. Sabene lost 60 pounds before finally deteriorating to the point of having to be admitted to emergency. Here, she was diagnosed with Crohn's disease.

Since her diagnosis in Windsor, Sabene has experienced significant complications, including peripheral neuropathy secondary to malnutrition. She went on and off Prednisone, and was hospitalized several times before finally being transfered to London, Ontario. In London, she lost 40% of her intestines and 60% of her small bowel to the surgeon's knife. Since, Sabene has undergone physiotherapy and has recovered quite nicely. She is on several medications to manage her bowel movements, and seems largely peachy and satisfied.

Person

  • Single 33 year old female
  • no kids
  • Father Haitian African-American; Mother native indian / white caucasian
  • Had a boyfriend at the time who was very stressful
  • Former smoker
  • Now works at a flower shop

History of Illness

  • Started having minor stomach problems at 15y.o. (largely ignored)
  • (more) Major problems started when she was 19y.o., which Sabene recognised to be uncommon stomach aches
    • After eating, there would be a rush of pain that was not normal
    • The pain was unpredictable
    • Lots of noises in her stomach while pain was "going" and after the pain
  • The pain affected her personal and professional lives
  • She had two jobs at the time:
    1. Waitressing
    2. Preparing food at a retirement home
  • Eating exaccerbated the problem, so she wouldn't eat for days
  • Within a year, she lost 60 lbs
  • Doctors tried Metamucil and a high dairy, high fibre diet, but were unable to diagnose the problem
  • Eventually, Sabene deteriorated and was hospitalized
  • First saw a specialist when she was admitted to the ER
  • She was biopsied, which led to a diagnosis of Crohn's disease
  • Had a fistula, which helped the specialist diagnose
  • She was prescribed Prednisone, subsequently felt great, and thought that she was "cured", despite the side-effects of Prednisone
  • Also had to cut out dairy and high fibre
  • Next few years were a rollercoaster of feeling well and feeling super-shitty
  • Severe pain while she was eating
  • With time, she deteriorated
  • Noises got louder as the years went on
  • Had diahrrea; had to go to the bathroom 10 times per day
  • During the day and in the middle of the night
  • Had lots of urgency, and sometimes didn't make it to the bathroom
  • Vomited at the beginning. Sometimes vomited blood
  • Felt weak, and couldn't function normally
  • Periods stopped, and she reported no periods for 7 years
  • Reported that friends didn't understand, and that some thought she had anorexia
  • Her hair's texture changed completely
  • At 22 y.o., she didn't have to straighten her hair. Grew in "silky and smooth". This is an indication of how weak it was
  • She was suffering sufficently that she requested surgery
  • Told that the Crohn's was in an inoperable area

History of peripheral neuropathy

  • Began around November, 1996
  • Had tingling in toes, then tingling in fingers
  • Feeling spread up feet and into legs
  • Suddenly felt like weights tied onto legs
  • Leg coordination went
  • Couldn't feel feet
  • Different numb areas in leg
  • Family doctor tried to have her walk straight line
  • She couldn't walk the straight line
  • Saw a neurologist 2 days later
  • He felt it was MS. MRI found it was not
  • Everybody was stumped
  • Sent her to London to see a neurologist here
  • A year and a half of MRIs and crazy tests (including loads of bloodwork) came next
  • Had to stop working because she couldn't drive because she couldn't feel her feet
  • Stayed this way for five years
  • Balance was bad, so she wasn't as active
  • Currently as an ataxic gait, and has done lots of physiotherapy

Most recent ill-health episode

  • 2002
  • had obstructions
  • Pain didn't move; hard spots didn't move
  • Was < 110 lbs until finally hospitalized and put on feeding tube
  • Kept in for 2 weeks and followed with CT scans
  • On Demerol for pain
  • Sent home
  • Got through holidays with the Demerol, but went back to the hospital when the Demerol ran out
  • Asked about Dr. Howard. The other doctor didn't enjoy that, but eventually referred her to Dr. Howard
  • Said that as soon as a bed would open up in London, she would go there
  • Weighed ~85 lbs (dry weight) when Dr. Howard saw her
  • She looked emaciated
  • Dr. Howard said he would take care of her

Happy Ending

  • Started with pain management
  • Wanted nutrition up; put on TPN
  • After that, she would be ready for surgery
  • Removed 60% of her small bowel during surgery
  • On TPN for another 6 weeks after
  • Since then, she has been "fine" and "great", and feels much better now than pre-surgically
  • Blood levels now are normal
  • Has since maintained her weight (which she gained within a couple of months)
  • Danger is that if 60% is gone, she might have short gut syndrome in the future
  • 1-2 bowel movements a days while on Cholestyromine (absorbes bile salts?)
    • Otherwise, she would have 4-5 movements per day
  • Can't easily eat salad or raw vegetables, but digests meat well

Cardio caveat

  • Sleeps on lots of pillows to elevate upperbody
  • Heaviness on chest
  • Shortness of breath, especially when lying down or on exersion
  • Initially thought it was related to humidity
  • admitted to the ER for enlarged heart and heart failure (32 years old)
  • Spent 2 weeks in hospital
    • Received EKGs
    • Heart was not shrinking
    • hypertention
    • On medication to this day for her heart
  • No explanation for cardiomyopathy



Another version

  • During onset that led to diagnosis (1992-93 - age 19) 60 lb weight loss (165->105)
  • loss of periods
  • Diarrhea (10 times a day some at night) - disturbance of diurnal rhythm of the gut with urgency - blood teaspoons here and there
  • Vomiting - blood in vomit
  • Incontinence (hindgut issue)
  • Told to go on no dairy, high fiber
  • Midabdominal cholic pain - peristalsis
  • Pain almost makes you faint (parasympathetic pain) Pain during eating. Lots of sounds during digestion - less pain. Pain on drinking alcohol, No pain drinking water. Was drinking
  • Midgut and hindgut problems.
  • Perianal fistula - led to diagnosis of Crohn's
  • Prednazone - helped a lot, some sideeffects
  • Side effects - hairgrowth,  ??
  • Metethrexate discontinued after neuro symptoms set in
    • 1995 strange sensation in her fingers
    • 6 months later in her toes (Nov 96) sensations spread up into legs felt like weights tied to legs. Loss of balance and coordination, numbness in various areas in logs.
    • Thought is was MS. Wasn't (after MRI) another 1.5 years pass.
    • Now - high stepping gate. Usually walks with cane. Balance problems continue
    • Sensory neuropathy loss of proprioception of feet
    • This led to improper gate which causes MSK problems
    • Peripheral neuropathy related to nutritional myelopathy (spinal chord)
    • Long fibers affected
  • 2001 Weakness weight dropped 94lb. Hospitalized IV feeding.
  • [missed]
  • Dec 2002 - Visible peristalsis, bumps moving in abdomen, noises. Obstruction. Demoral. Sent home. went back in a couple of weeks
    • Hospitalized 6 weeks over 2 months, Tube feeding.
    • Thought she was dying ~100lb
  • Met Dr. Howard. February 2003. ~100 lb. (5'7" normal weight should be ~130)
  • All throughout - absorbtion issues (calcium, vitamins, proteins, etc.)
  • Had been on diarretics for 8 years
  • Edema caused bu low albumin (low protein) in blood. Incident of Knee swelling so badly that skin on the back of the knee split.
  • Dr. Howards plan
    • Control pain
    • Nutrition (TPN - Total parenteral nutrition - IV feeding)
    • Surgury May 2003 weight before ~120 from TPN
      • 60% of small bowel removed - Jejunum (except 10"), part of ilium


      • On TPN for 6 weeks
  • Noise and pain gone. Weight maintained.
  • Short Gut syndrome (so little gut left that nutrients can't be absorbed) was a possible risk given the area of involvement
  • While on TPN
    • Walking with walker before but on TPN felt stronger (before surgury)
  • After surgery
    • Hot flashes, eversince - maybe premenopause

Cardiomyopathy

  • Nutritional deficiencies � Deficiencies in thiamine, selenium, and L-carnitine have been reported to produce HF and replacement therapy results in improvement in cardiac function [43,44].
  • Thiamine plays an important role in normal oxidative phosphorylation and therefore myocardial energy production. Thiamine deficiency initially presents as a high output state secondary to vasodilation; this is followed by eventual depression of myocardial function and the development of a low output state [43].
  • Selenium deficiency decreases the activity of glutathione peroxide, resulting in increased free radicals that are toxic to cardiac myocytes. The development of Keshan disease, an endemic cardiomyopathy that affects children and women of childbearing age in areas of China, has been linked to Se deficiency [45]. The geographical distribution of Keshan disease is associated with local diets, which are nearly devoid of Se. An animal model of Keshan disease in Se deficient mice infected with Coxsackievirus has been described [46]. The mice have increased mutations in the viral genome, leading to a virus with greater cardiac virulence, causing a cardiomyopathy similar to that seen in Keshan disease. (See "Overview of dietary trace metals", section on Selenium).
  • Carnitine deficiency impairs the oxidation of fatty acids, resulting in lipid accumulation in the myocyte cytoplasm. This problem is reversed with L-carnitine replacement. (See "Carnitine metabolism in renal disease and dialysis").

Patient-doctor relationship


Possible Objectives

  • What happened to her heart?
  • What's the peripheral neuropathy thing about?
  • Did her pre-Howard doctors make a mistake? If so, how common are such mistakes, and how can they be prevented?
  • Dr. Howard described a close relationship with Sabene. Discuss.

From Dr. Howard:

For Thursday, I would be grateful if each group could prepare a 1 minute statement giving the PCL group's best explanation as to

  1. what was the cause of Sabene's myelopathy?
    • Kwashiorkor? [4]
    • Methotrexate [5] antagonizes folic acid absorption; B12 deficiency may ensue
    • Vitmain deficiencies [6]
  2. what was the cause of Sabene's dilated cardiomyopathy?


In my reading around the case, there is no one good explanation. However, I think we can come up with theories as to how they arose and how they are related to her nutritional state and/or her Crohn's Disease. These theories might also direct further care in her case. I would also like each group to discuss in your PCL time the issues of boundaries and how you would each determine what your individual boundaries are. Time permitting on Thursday, we might have some general discussion on this.

  • Sick identity, and the effects of getting sick when young vs. when old (having formed the personal identity before vs. forming it around the issue)

Resources