Amyotrophic Lateral Sclerosis (Lou Gehrig ’ s Disease)

1. Amyotrophic Lateral Sclerosis (Lou Gehrig’s Disease) Rod Sorensen, D.O. Marshfield Neurosciences April 26, 2007

2. Synonyms • Amyotrophic Lateral Sclerosis (ALS) • Motor Neuron Disease (MND) • Lou Gehrig’s Disease

3. Lou Gehrig • NY Yankees baseball star beginning in 1925 • Once in the starting line-up he never missed a game until 1939 • Lifetime BA prior to 1938 was about .350

4. Lou Gehrig • 1938 season was disappointing, especially late, not Mr. October • Ed Barrow (1938 counterpart of Brian Cashman) took the opportunity to decrease Gehrig’s pay from $39,000 TO $36,000 • 1939 - terrible spring training and early season

5. Lou Gehrig • Lou was beginning to have falls and significant balance problems • His baseball skills were visibly impaired • Lou’s first AB in 1939 resulted in a weak liner to right field, caught by a 21year-old rookie Red Sox outfielder, Ted Williams • Late in that same game, the pitcher walked Joe DiMaggio, preferring to pitch to Gehrig • 8 games into the 1939 season, Lou went to manager Joe McCarthy and benched himself

7. Lou Gehrig • The numbers • 14* years (retired at age 35) • 2130 consecutive games • 2721 hits • 493 HR • Lifetime BA - .340 (.295 in 1938, .143 in 1939)

8. Lou Gehrig • Continued to travel with the team in 1939 and, as captain, gave the line-up to the umpires at the beginning of the game • Often had to be accompanied by someone, to avoid the possibility of a fall

9. Lou Gehrig - Diagnosis • ALS was diagnosed after Gehrig was evaluated at the Mayo Clinic from June 13-19, 1939 • Wife Eleanor was phoned on June 19 (Lou’s 36th birthday) and was given the diagnosis and told that Lou would be dead by 1942 • Eleanor chose not to tell Lou that he had an incurable, fatal disease

10. Lou Gehrig - Diagnosis • Lou Gehrig Day at Yankee stadium was planned for July 4, 1939 • While preparation for the celebration occurred GM Barrow informed Eleanor Gehrig that Lou would need to find a new job - both Lou and Eleanor were surprised and infuriated by this

11. Lou Gehrig - Farewell Address “Fans, for the past two weeks you have been reading about a bad break I got. Yet today I consider myself the luckiest man on the face of the earth. I have been in ballparks for seventeen years and have never received anything but kindness and encouragement from you fans. Look at these grand men. Which of you wouldn’t consider it the highlight of his career just to associate with them for even one day?”

12. Lou Gehrig - Farewell Address “Sure I’m lucky. Who wouldn’t consider it an honor to have known Jacob Ruppert. Also, the builder of baseball’s greatest empire, Ed Barrow? To have spent six years with that wonderful little fellow, Miller Huggins? Then to have spent the next nine years with that outstanding leader, that smart student of psychology, the best manager in baseball today, Joe McCarthy?”

13. Lou Gehrig - Farewell Address “Sure I’m lucky. When the New York Giants, a team you would give your right arm to beat, and vice versa, sends you a gift - that’s something. When everybody down to the groundskeepers and those boys in white coats remember you with trophies -- that’s something. When you have a wonderful mother-in-law who takes sides with you in squabbles with her own daughter -- that’s something.”

14. Lou Gehrig - Farewell Address “When you have a father and a mother who work all their lives so you can have an education and build your body -- it’s a blessing. When you have a wife who has been a tower of strength and shown more courage than you dreamed existed -- that’s the finest I know. So, I close in saying that I might have been given a bad break, but I've got an awful lot to live for.”

17. Lou Gehrig • Despite his failing health, Lou continued to travel with the Yankees throughout the 1939 season, including the World Series • NY mayor La Guardia asked Lou to become a parole commissioner for the city of New York - Lou finally accepted and was sworn in on Jan. 2, 1940 for a ten-year term at $5,700/yr. • Rocco Barbella, aka Rocky Graziano case

19. Lou Gehrig • Died in his sleep June 2, 1941at the age of 37

21. Amyotrophic Lateral Sclerosis • Epidemiology • Features • Diagnosis • Natural History • Treatment and symptom management

22. Epidemiology • Annual incidence of about 1-2 per 100,000 • More common with increased age, peak incidence at 65-70 years • More common in white persons • Male/female ratio about 2 • Mean survival is 3 years • NOT an inherited disease

23. Typical Features • Typical initial symptom is weakness • Hands are most common initial location, but can start anywhere • Often asymmetric • As weakness progresses muscle atrophy occurs

24. Typical Features • Fasciculations and cramps • Slurred speech and trouble swallowing - not usually at onset • Difficulty breathing - usually a later symptom • Drooling

25. Typical Features • Loss of dexterity • Clumsiness • Stiffness and spasticity • Exaggerated emotionality

27. Diagnosis • Exam • Definite - UMN & LMN signs in three regions • El Escorial WFN criteria – definite, probable, possible… • Electrodiagnostic studies • Imaging studies and lab testing when appropriate

28. Natural History • Up to 40% of motor nerve cells may already be lost by the time a patient presents with weakness • Fairly consistent pace of worsening, spread is typically to contiguous anatomic areas • Bulbar and respiratory involvement is usually later in course • Death in 3 years is typical

29. Treatment • No effective treatment for cure or improvement of disease • Riluzole • MAY slightly slow speed of progression • Reasonably safe • Expensive

30. Symptom Management • Drooling, depression, emotionality • Drying agents for drooling • Anticholinergic medications • Can result in thick secretions • Botox in parotid glands • Antidepressants for depression and emotionality • Tricyclic antidepressants often help with all 3 symptoms

31. Symptom management • Spasticity and cramps • Antispasticity medications • Can increase weakness • Muscle relaxants • Best medication for cramps is quinine - now unavailable due to FDA concerns

32. Symptom Management • Pain • Usually not a problem until later in disease when immobility occurs • Physical therapy stretching, heat therapy can be very helpful • Medications • Anti-inflammatories • Other meds including narcotics if needed

33. Symptom Management • Mobility • Home assessment by RN, OT, PT with appropriate devices recommended • Braces, collars and orthotics • Home ROM, stretching program • Wheelchairs, scooters

34. Symptom Management • Speech and swallowing issues • Communication devices • Nutrition consult • Optimize food consistency to assist swallowing ease • Supplements • Forward head position, chin-tuck • PEG tube when swallowing no longer safe and when patient decides they want it

35. Symptom Management • Respiratory problems • Secretion control • BiPAP • Non-invasive, intermittent • Improves survival • Tracheostomy and mechanical ventilation • Prolongs survival, but increases and prolongs dependency • Decision to do this requires careful consideration and family involvement

36. Palliative Care • In late stages of ALS, a number of different factors can produce physical and medical discomfort not unlike many other terminal illnesses • Palliative care physicians/nurses/other health providers are particularly skilled at recognizing and treating these problems • Contrary to what patients and families initially expect, death is usually not painful/stressful

37. Dependency • Lack of independence or self- sufficiency • That which is attached to something else as its consequence, subordinate, satellite, and the like.

38. Dependency • In ALS, the hardest part of the disease is probably the loss of physical capacity (despite normal cognitive abilities) and the associated loss of independence • Loss of limb use • Mobility issues • Eating, speech • Ultimately, often entirely dependent on someone else for virtually everything

39. Dependency • What happens with others around you - family, friends • Our reaction to people with physical disabilities - particularly those who were previously “whole” • Less interaction • Change in the interaction

40. Dependency • ALS patient’s responses • Withdrawal & silence • Depression • Anger & frustration

41. Dependency • How we can help • Don’t forget them • Be natural - maybe act like it’s a briefer, less serious illness (sure they are sick, but they are still the person we know) • Little things done a lot are better than big things done rarely • Get in their shoes • Remember their spiritual being