Evidence-­‐Based  Physical  Therapy  Practice  for  Patients  with  Scleroderma   - PDF Document

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  1. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   i       Evidence-­‐Based  Physical  Therapy  Practice  for  Patients  with  Scleroderma   _________________________________________________________________________________________________     A  Case  Report    Presented  to       The  Faculty  of  the  Department  of  Physical  Therapy  and  Human  Performance   College  of  Health  Professions  and  Social  Work   Florida  Gulf  Coast  University           In  Partial  Fulfillment     of  the  Requirement  for  the  Degree  of     Doctor  of  Physical  Therapy     _________________________________________________________________________________________________       by   Matthew  W.  Attia   2014  

  2. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   ii         APPROVAL  SHEET     This  case  report  is  submitted  in  partial  fulfillment  of     the  requirements  for  the  degree  of     Doctor  of  Physical  Therapy     ____________________________ Matthew Wade Attia   Approved:  May  2014 ____________________________ Sharon Irish Bevins, PhD, PT Committee Chair ____________________________ Jacqueline van Duijn, DPT, OCS This case report has been examined by the signatories, and we find that both the content and the form meet acceptable presentation standards of scholarly work in the above mentioned discipline.

  3. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   iii       Table  of  Contents   Title  Page……………………………………………………………………………………………………………...i   Approval  Sheet…………………………………………………………………………………...........................ii   Table  of  Contents………………………………………………………………………………………………...iii   List  of  Tables………………………………………………………………………………………………............iv     Abstract……………………………………………………………………………………………….......................v   Introduction.………………………………………………………………………………………………………..1     Purpose…..………………………………………………………………………………………………...4   Case  Description..………………………………………………………………………………………………...5   Patient  presentation……….………………………………………………………………………….5   Patient  history  &  system  review…………………………………………………………………5   Examination……………………...………………………………………………………………………5   Objective  measurements……………………………………………………………………………8   Strength…………………………………………………………………………………………8   Soft  tissue  mobility…………………………………………………………………………8   Posture..…………………………………………………………………………………………9   Range  of  motion  (ROM)…………………………………………………………………..9   Other  examination  recommendations……………………………………………10   Interventions……………………………………..…………………………………………...............10   Problems,  Goals,  and  Plan  of  care…….…………...…………………………………………..18   Outcomes…………………………………………..…………………………………………...............21   Discussion………………...……………………………………..……………………………………...............23   Conclusion………………..……………………………………..……………………………………...............27   References………………..……………………………………..……………………………………...............28      

  4. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   iv       List  of  Tables   Table  1.  ACR/EULAR  2013  Systemic  Sclerosis  Classification.  …………………………………3   Table  2.  Initial  evaluation  of  active  cervical  ROM.  ……………………………………………..…10   Table  3.  Interventions  Performed  Date  of  Initial  Evaluation  (October  29th,  2013).  ..12   Table  4.  Interventions  Performed  on  Last  Visit  (November  26th,  13).  …………………..13   Table  5.  Plan  of  Care  at  Initial  Evaluation  (October  29th,  2013).  …………………………...20   Table  6.  Problem  list,  Goals,  and  Assessment.  ……………………………………………………..22                            

  5. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   v       Abstract     Scleroderma  i.e.  systemic  sclerosis  is  a  rare  rheumatic  autoimmune  disease   characterized  by  fibroblast  dysfunction  with  excessive  collagen  deposition  leading   to  vasculopathy  and  subsequent  dysfunction  throughout  the  body.  This  case  report   addresses  the  care  provided  to  a  71  year-­‐old  Caucasian  female  who  was  treated  for   cervical  dysfunction  in  an  outpatient  orthopedic  clinic.  The  symptoms  experienced   by  patients  with  the  diffuse  progressive  form  of  the  disease  are  diverse  and   systemic.  Physical  therapists  should  ensure  that  patient  care  is  multidisciplinary   and  has  an  emphasis  on  prevention.  It  should  also  be  focused  on  delaying  functional   limitations,  treating  exacerbated  symptoms,  and  gathering  baseline  information  to   track  disease  progression  and  guide  further  treatment.  Current  literature  and   evidence-­‐based  research  is  very  limited  regarding  physical  therapy  and  our   potential  interventions  to  this  patient  population.  This  case  report  identifies  the   treatment  provided  to  this  patient,  as  well  as  recommendations  on  providing   current  and  research-­‐based  interventions  specific  to  a  patient  with  a  diagnosis  of   systemic  sclerosis.                

  6. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   1       Introduction     The  term  “scleroderma”  is  an  umbrella  term  for  multiple  sub  groups  of  the   connective  tissue  &  vascular  disease  systemic  sclerosis  (SSc).  Scleroderma  can  be   either  localized  or  systemic;  localized  scleroderma  has  a  more  favorable  prognosis   than  systemic  scleroderma  as  it  only  affects  the  integumentary  system  (“umm.edu”,   2012).  Systemic  scleroderma  can  present  as  either  limited  or  diffuse  cutaneous  SSc.   Limited  SSc  affects  the  skin,  esophagus,  and  distal  joints,  while  the  much  more   severe  diffuse  SSc  is  progressive  and  compromises  the  majority  of  the  internal   organs  and  the  integumentary  system.  Patient  presentation  and  disease   characteristics  of  diffuse  SSc,  also  known  as  progressive  systemic  sclerosis  (PSSc),     includes  skin  thickening,  cutaneous  ulcerations,  joint  pain,  contractures,   gastrointestinal  complications,  esophageal  dysmobility,  a  compromised  respiratory   system,  kidney  disease,  and  a  decrease  in  cardiac  function  (Goodman,  2009).  The   increased  risk  of  mortality  in  PSSc  has  a  magnitude  of  250%  in  comparison  with  an   age  and  sex-­‐matched  general  population  (Elhai,  2011).       Systemic  sclerosis  is  classified  as  a  rheumatic  autoimmune  disease  and  has   previously  been  subcategorized  as  a  disease  of  connective  tissue  metabolism.   Current  research  is  challenging  conventional  thinking  to  consider  SSc  as  a  vascular   disease  (Matucci-­‐Cerinic,  2013).  Progressive  SSc  is  different  from  other  rheumatic   diseases  in  that  patients  with  the  disease  display  systemic  vasculopathy  and   fibrosis.  Universally  throughout  the  body,  the  intima  of  blood  vessels,  the   pericapillary  space,  and  the  interstitium  of  the  skin  are  filled  with  extracellular  type   1  collagen  (Warrington,  Nair,  Carbone,  Kang,  Postlethwaite,  2006.;  Goodman,  2009).    

  7. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   2       The  hallmark  signs  of  systemic  sclerosis  include  three  physiological  characteristics:   small  vessel  vasculopathy,  production  of  autoantibodies,  and  a  hyperactive   fibroblast  dysfunction  (Hoogen  et  al.  ,2013).    Type  I  collagen  is  the  major  collagen  of   tendons,  skin,  ligaments,  cornea,  and  many  interstitial  connective  tissues,  with  the   exception  of  very  few  tissues  such  as  hyaline  cartilage,  brain,  and  vitreous  body.  The   hallmark  fibroblast  dysfunction  leads  to  unregulated  type  I  collagen  deposition.  In   healthy  individuals,  type  I  collagen  provides  appropriate  tensile  stiffness  (Gelse,   Poschl,  &  Aigner,  2003).  Due  to  the  deposition  of  collagen  within  the  extracellular   space,  the  intima  of  blood  vessels  systemically,  and  the  interstitium  of  skin;  fibrosis   occurs  resulting  in  a  decline  in  function  throughout  the  body.   The  classification  of  SSc  has  been  recently  updated  by  the  American  College   of  Rheumatology/European  League  Against  Rheumatism  (ACR/EULAR)  from  the   original  classification  by  the  American  College  of  Rheumatology  of  1980.  The  new   classification  criterion  is  more  sensitive  to  patients  in  the  early  phase  of  the  disease   process  and  allows  for  more  patients  to  be  classified  correctly  (Hoogan  et  al,  2013).                  

  8. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   3       Table  1   ACR/EULAR  2013  Systemic  Sclerosis  Classification   ITEM   SUB-­‐ITEM   SCORE   Skin  thickening  of  the   fingers  of  both  hands   extending  past  the  level  of   the  metacarpophalangeal   joint   Skin  thickening  of  the   fingers     9   Puffy  fingers   Sclerodactyly  of  the   fingers   2   4   Fingertip  lesions   Digital  tip  ulcers   Fingertip  pitting  scars   2   3   Talengiectasia     2   Abnormal  nailfold   capillaries   Pulmonary  arterial   hypertension/  Interstitial   lung  disease   Reynaud’s  phenomenon     2     2     3   SSc-­‐related  autoantibodies     3   A  score  of  9  is  required  to  be  diagnosed  with  SSc.  More  details  available  (Hoogan  et   al,  2013)                

  9. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   4       Scleroderma  has  the  highest  mortality  rate  of  any  of  the  rheumatic  diseases.   There  is  currently  no  cure  for  PSSc.  The  prognosis  of  PSSc  is  dependent  on  the  level   of  systemic  involvement.  The  leading  causes  of  mortality  found  in  patients  with  PSSc   include  pulmonary  hypertension,  cardiac  dysfunction,  and  renal  disease.  The   prevalence  of  PSSc  within  the  United  States  ranges  from  300,000-­‐400,000  or  2  to  10   per  1,000,000.  It  affects  women  more  than  men  with  a  seven  to  one  ratio,  and  has  a   slightly  higher  prevalence  in  the  African-­‐American  population  (Goodman,  2009;   Brandenstein,  1999).     Purpose.  Systemic  sclerosis  is  a  rare  disease  that  presents  differently  in   affected  patients.  Based  on  these  factors,  available  evidence  based  research   literature  for  physical  therapy  intervention  for  patients  with  PSSc    is  very  limited.   Current  treatment  recommendations  and  guidelines  exist  for  the  medical   management  of  scleroderma  (Kowal-­‐Bielecka,  2009);  however,  they  do  not  exist  for   physical  therapy  interventions.    The  lack  of  available  information  makes  it  difficult   for  a  physical  therapist  to  optimally  treat  a  patient  with  a  diagnosis  of  systemic   sclerosis.  The  purpose  of  this  case  report  is  to  discuss  the  effects  of  the  disease   process  relevant  to  physical  therapy,  evidence-­‐based  physical  therapy  interventions   relative  to  SSc,  and  SSc-­‐appropriate  patient  care.   Due  to  the  variable  presentation  of  the  disease,  physical  therapy  intervention   will  vary  on  a  case-­‐by-­‐case  basis.  Physical  therapists  need  to  administer  validated   and  evidence-­‐based  evaluations  and  interventions  based  on  professional  guidelines   to  deliver  quality  patient  care.  The  current  available  literature  specific  to  educating   physical  therapists  concerning  scleroderma  is  limited.  Therefore,  it  is  important  for  

  10. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   5       the  physical  therapist  to  keep  in  mind  the  pathophysiology  and  disease  process  of   scleroderma  when  treating  the  functional  mobility  of  patients  diagnosed  with  PSSc.     Case  Description     Patient  presentation.  The  case  patient  is  a  71  year-­‐old  Caucasian  female   with  a  medical  diagnosis  of  Scleroderma  for  a  duration  of  5  years.  The  patient  was   referred  to  physical  therapy  by  her  rheumatologist  for  neck  pain.  The  patient  was   past  the  average  age  of  disease  onset,  which  is  typically  the  early  to  middle  forties.     Patient  history  &  system  review.  The  patient  was  seen  in  an  outpatient   physical  therapy  setting  in  which  the  patient’s  subjective  medical  history  was   recorded  via  a  medical  history  questionnaire.  The  questionnaire  was  positive  for   arthritis,  vascular  disease,  previous  deep  vein  thrombosis  (DVT),  recent  weight  loss   or  gain,  headaches,  and  anxiety.    The  patient  stated  that  her  reason  for  obtaining  her   referral  was  neck  dysfunction,  and  chief  complaints  secondary  to  her  SSc  included   GI  disturbances,  and  difficulty  using  her  hands.  The  patient  had  degenerative  joint   disease  and  osteoarthritis  of  the  bilateral  cervical  spine  confirmed  by  X  ray.       Examination.  Based  on  the  patient  presentation,  objective  and  functional   measures  were  taken  pertaining  to  the  patient’s  cervical  dysfunction.  Neck   symptoms  are  noted  to  be  present  in  nearly  90%  of  patients  diagnosed  with  SSc   (Barnett,  2005).    Symptoms  typically  include  tightening  of  the  skin  of  the  neck  but   may  include  limitations  of  ROM  and  decreased  strength.  The  Neck  Disability  Index   (NDI)  was  chosen  by  the  physical  therapist  to  evaluate  the  patient’s  functional   status.    This  test  is  considered  to  have  moderately  high  validity  (0.69-­‐0.70)  and  test-­‐ retest  reliability  (0.89)  (Vernon  &  Moir,  1992).  The  raw  score  on  the  NDI  was  13  

  11. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   6       with  a  26%  calculated  score,  which  indicates  mild  to  moderate  dysfunction.    A   functional  status  assessment  revealed  independence  without  difficulty  in  her   activities  of  daily  living  prior  to  her  chief  complaint  of  neck  symptoms.  At  the  time  of   the  initial  evaluation,  the  patient  reported  that  her  level  of  independence  was   modified  independent  with  moderate  restrictions  due  to  pain.  The  disease  process   of  SSc  includes  widespread  deposition  of  collagen,  and  involvement  of  small  vessel   vasculopathy.  Due  to  this,  all  structures  of  the  neck  need  consideration  in  addition   to  an  orthopedic  cause  for  the  patient’s  symptoms.  Based  on  the  known  disease   process  of  SSc,  considerations  need  to  include  possible  dysfunction  of  the  cervical   lymphatic  system,  the  thyroid  gland,  salivary  glands  and  ducts  etc.,  as  the   dysfunction  may  be  related  to  a  non-­‐orthopedic  origin  and  thus  require  appropriate   referral.         Although  not  completed  in  this  case,  a  more  thorough  evaluation  of  patients   with  SSc  should  be  administered  due  to  the  global  effects  of  the  disease  process.  The   list  of  available  standardized  tests  is  extensive,  and  choosing  a  test  is  highly  variable   and  based  on  symptom  presentation.  Scleroderma  population-­‐validated  tests   regarding  disability  assessment  include  the  Scleroderma  Health  Assessment   Questionnaire  (SHAQ),  Symptom  Burden  Index  (SBI),  and  Cochin  Hand  Function   Scale  (Pope,  2011;  Rannou,  2007).    Due  to  the  known  pathophysiology  of  systemic   sclerosis  and  the  scope    of  physical  therapy,  the  physical  therapy  outpatient  facility   is  an  appropriate  setting  for  these  objective  measures  of  function  to  be  taken  and   monitored.    

  12. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   7       It  is  recommended  that  pulmonary  function  testing  should  take  place   annually  for  patients  with  SSc  (Khanna,  Gladue,  Mclaughlin,  et  al.,  2013).  It  therefore   seems  appropriate  for  the  physical  therapist  to  be  involved  in  the  evaluation  and   management  of  cardio-­‐pulmonary  function.  The  Cambridge  Pulmonary   Hypertension  Outcome  Review  is  reliable  and  valid  for  patients  with  SSc.  It  contains   a  symptoms  scale,  and  measures  for  activity  limitations,  and  quality  of  life   (Gornberg-­‐Maitland,  2008).  Pulmonary  function  testing  should  take  place  to   establish  a  baseline,  monitor  the  progression  of  the  disease,  and  provide  grounds  for   early  intervention.     Hand  dysfunction,  including  tightening  of  the  skin,  flexion  contractures  of  the   joints,  digital  ulcers,  loss  of  strength,  and  pain  have  been  found  to  be  some  of  the   most  common  and  easily  recognizable  symptoms  of  scleroderma  (Goodman,  2009;   www.scleroderma.org,  2013).  Hand-­‐specific  functional  disability  assessments  that   are  validated  for  this  population  include  the  Cochin  Hand  Function  Scale,  the   Arthritis  Hand  Function  Test,  and  the  Hand  Mobility  In  Scleroderma  Test  (Pope,   2011;  Rannou,  2007).  Joint,  tendon,  muscle  health,  and  the  mobility  of  the  skin  are   compromised  in  this  population  secondary  to  the  deposition  of  collagen  around  and   within  the  soft  tissue  structures.  The  deposition  of  the  collagen  leads  to  a  cascading   effect  of  muscular  disuse  that  leads  to  atrophy.  Inflammatory  processes,  combined   with  disuse,  also  lead  to  abnormal  changes  in  joint  surfaces  and  joint  deformities.   Healthy  synovial  joints  require  movement  in  order  to  nourish  the  articular  cartilage   of  a  joint.  The  articular  cartilage  on  the  contacting  surfaces  of  the  bones  of  a  joint  is   necessary  to  reduce  the  coefficient  of  friction  between  the  articulating  surfaces.  

  13. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   8       When  the  synovium  of  a  joint  and  the  articular  cartilage  are  disrupted,  pain,   inflammation,  and  decreased  range  of  motion  will  occur  (Levangie  &  Norkin,  2005).   Scleroderma  affects  the  joint  in  multiple  ways.  The  end  result  is  limited  range  of   motion  that  leads  to  structural  changes  and  decreased  function.  It  would  appear   logical  that  structural  changes  of  the  joints  will  also  lead  to  alterations  in  muscle   length  tension  relationships  that  will  lead  to  further  strength  deficits.  Physical   therapists  need  to  be  aware  of  this  disease  process,  and  understand  that  aggressive   therapy  interventions  may  lead  to  an  exacerbated  inflammatory  response,  and  cause   detrimental  effects,  similar  to  what  is  seen  in  patients  with  rheumatoid  arthritis.     Physical  therapists  should  assess  joint  mobility  and  focus  on  preventing  or   maintaining  affected  joint  range  of  motion.     Objective  measurements.   Strength.  Manual  muscle  testing  demonstrated  decreased  strength  of  the   cervical  musculature  at  3+/5  in  flexion  and  side-­‐bending,  as  well  as  shoulder  muscle   weakness  of  the  shoulder  adductors  and  flexors  at  3/5  and  4-­‐/5  respectively.  Deep   neck  flexor  muscular  strength  and  endurance  was  not  assessed,  although  research   confirms  that  reduced  performance  of  this  muscle  group  is  associated  with   increased  risk  of  neck  pain  (Falla,  Gwendolen,  &  Hodges,  2004).    Manual  Muscle   testing  has  been  shown  to  be  a  valid  measure  of  strength  with  a  strong  correlation   to  EMG  and  dynamometer  testing,  and  has  test-­‐retest  and  inter-­‐rater  reliability   ranging  from  82%  to  97%  (Cuthbert  &  Goodheart,  2007).     Soft  tissue  mobility.  Soft  tissue  mobility  was  noted  to  be  moderately   restricted  in  the  erector  spinae,  suboccipital,  and  upper  trapezius  musculature.  A  

  14. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   9       meta-­‐analysis  revealed  that  the  inter-­‐rater  reliability  of  detecting  soft  tissue   changes  was  strong  with  a  Cohen’s  Kappa  (K  value)  of  0.03  (Stochkendhal,  M.,   Christensen,  H.,  Hartvigsen,  J.,  Vach,  W.,  Haas,  M.,  Hestbaek,  L.  et  al.,  2006).       Posture.  Moderate  forward  head  and  rounded  shoulder  posture  was  noted.   There  is  a  lack  of  agreement  regarding  the  validity,  reliability,  and  significance  of   forward  head  posture  in  the  literature.  Greater  forward  head  posture  was  found  to   be  correlated  to  a  greater  level  of  disability  (Yip,  Chiu,  &  Poon,  2008).    However,   Silva,  Punt,  Sharples,  Vilas-­‐Boas  &  Johnson  (2008),  state  that  the  reliability  and   validity  of  assessing  forward  head  posture  by  observation  is  unclear,  as  well  as  the   therapeutic  effect  of  improving  forward  head  posture  on  neck  pain.     Range  of  motion  (ROM).  ROM  was  measured  with  a  goniometer  for  rotation   and  an  inclinometer  for  lateral  side  bending  and  flexion.    Williams  et  al  (2009)   stated,  “The  single  inclinometer  and  Spin-­‐T  goniometer  have  both  good  reliability   and  concurrent  validity  but  require  further  evaluation.”  (p.  153).    A  thorough   literature  review  reported  that  “The  CROM  has  the  advantage  over  the  single   inclinometer  and  goniometer”;  however,  the  author  goes  on  to  say  there  can  be  no   strong  recommendation  for  any  tool…  and  the  goniometer  is  practical,  is  accepted   by  clinicians,  and  has  a  reliability  ICC  value  of  >  0.76  (Jordan,  2000).          

  15. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   10       Table  2   Initial  evaluation  of  active  cervical  ROM   Active   Motion   Tested     Flexion     Left   Rotation     Right   Rotation     Left  Side   Bending     Right  Side   Bending     Range  of  Motion  in  degrees     52     26     41     25     20   Other  examination  recommendations.  Considering  the  disease   progression  and  common  manifestations,  objective  measures  should  have  been   taken  regarding  hand  and  cardio-­‐pulmonary  function.  Practical  tests  and  measures   include  grip  strength,  finger  range  of  motion  assessment,  pulmonary  function  tests,   and  cardiopulmonary  endurance  tests  such  as  the  six-­‐minute  walk  test  or   submaximal  exercise  testing.  Vitals,  including  heart  rate,  respiratory  rate,  and  blood   pressure,  should  be  monitored  throughout  patient  care.  Each  of  the  above  would   establish  baseline  information  to  monitor  progress,  indicate  need  for  services,  and   help  guide  physical  therapy  interventions.     Interventions.  Physical  therapy  has  successfully  helped  patients  with  PSSc   manage  the  following  symptoms:  regression  of  joint  range  of  motion,  respiratory   pathology,  decreased  cardiac  function,  decreasing  skin  elasticity,  and  pain.  The   above  list  of  manageable  symptoms  of  PSSc  are  all  either  directly  related  to  life  

  16. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   11       expectancy,  or  directly  correlated  to  functional  independence  and  quality  of  life   (Casale,  Buonocore,  &  Matucci-­‐Cerinic,  1997;  Joven,  Almodovar,  Carmona,  Carreira,   2009;  Ngian,  Stevens,  Prior,  Gabbay,  Roddy,  Tran,  …  Nikpour,  2012;  Pinto,  2011;   Tyndall,  Bannert,  Vonk,  Airo,  Cozzi,  Carreira,…Ulrich,  2010;  Yuen,  2012).  Skilled   physical  therapy  is  essential  to  ensure  patients  with  scleroderma  do  not  aggravate   the  inflammation  of  joints,  and  that  exercise  is  performed  at  a  safe  level  considering   compromised  cardiopulmonary  health.     Interventions  that  were  administered  to  the  patient  included  gentle  cervical   strengthening  and  ROM  exercise  activities,  manual  interventions  of  cervical  traction   and  soft  tissue  mobilization  techniques,  and  modalities  that  included  moist  heat  and   cryotherapy.  Interventions  performed  to  the  case  patient  on  the  day  of  the  initial   evaluation,  and  at  the  last  therapy  session  administered  are  provided  in  Table  3  and   Table  4  below.  Evidence  for  the  applied  interventions  to  the  cervical  spine  in   patients  with  SSc  is  limited.  However,  there  is  evidence-­‐based  literature  that   supports  positive  results  from  relevant  interventions  to  other  parts  of  the  body  in   patients  with  SSc.                                

  17. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   12       Table  3     Interventions  Performed  Date  of  Initial  Evaluation  (October  29th,  2013)     Interventions   Dosage  and  Details   Range  of  Motion:   Chest  Lift-­‐Chin  Tuck     Isotonics:   Shoulder  Shrugs     Manual  Interventions  To:   Time  Elapsed:  4  Minutes,  Repetitions:   10,  Sets:  2.   Time  Elapsed:  4  Minutes,  Weight  –   Pounds:  0,  Repetitions:  10,  Sets:  2.   Time  Elapsed:  24  Minutes,  Tx  Depth:   Moderate,  Side:  Bilateral,  Technique:   Myofascial  Release.   •Erector  Spinae   •Suboccipitals   •Upper  Trapezius   Manual  Traction  To:   Cervical  Spine   Time  Elapsed:  8  Minutes,  Clinical  Use:   Post  Activity   Technique  1:  Distraction   Technique  2:  Distraction/R   Rotation/Flexion   Technique  3:  Distraction/  L   Rotation/Flexion     Time  Elapsed:  10  Minutes,  Location:   Cervical  Region,  Clinical  Use:  Pre   Activity,  Skin  Integrity  Post:  Intact.   Modalities:   Moist  Hot  Pack                    

  18. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   13       Table  4     Interventions  Performed  on  Last  Visit  (November  26th,  13).     Interventions   Active  Range  of  Motion  and   Strengthening:   Dosage  and  Details         Time  Elapsed:  4  Minutes,  Repetitions:   10,  Sets:  2.   •Chest  Lift-­‐Chin  Tuck       Time  Elapsed:  3  Minutes,  Weight  –   Pounds:  0,  Repetitions:  10,  Sets:  2,   Position:  supine,  Additional  Detail:  With   Stick.     Time  Elapsed:  4  Minutes,  Weight  –   Pounds:  0,  Repetitions:  10,  Sets:  2.     Time  Elapsed:  4  Minutes,  Repetitions:  3,   Side:  Bilateral,  Technique:  Passive,  Body   Position,  Seated,  Hold:  15  Seconds         Time  Elapsed:  32  Minutes,  Tx  Depth:   Moderate,  Side:  Bilateral,  Technique:   Myofascial  Release.   •Shoulder  Flexion       •Shoulder  Shrugs     Flexibility:   Levator  Scapulae  Musculature   Manual  Interventions  To:   •Erector  Spinae   •Scaleni   •Suboccipitals   •Upper  Trapezius     Manual  Traction  To:   Cervical  Spine     Time  Elapsed:  8  Minutes,  Clinical  Use:   Post  Activity   Technique  1:  Distraction   Technique  2:  Distraction/R   Rotation/Flexion   Technique  3:  Distraction/  L   Rotation/Flexion     Time  Elapsed:  10  Minutes,  Location:   Cervical  Region,  Clinical  Use:  Pre   Activity,  Skin  Integrity  Post:  Intact.     Modalities:   Moist  Hot  Pack      

  19. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   14       It  has  been  shown  that  a  combination  of  connective  tissue  massage,  joint   manipulation,  and  home  exercise  significantly  improves  fist  closure,  measurements   of  finger  range  of  motion,  and  Cochin  hand  functional  disability  scale  scores  of  the   hands  in  patients  with  SSc  (Bongi  et  al.  2009).  It  is  reasonable  to  assume  that  similar   interventions  applied  to  the  cervical  spine  and  neck  of  a  patient  with  SSc  would   produce  similar  results.  This  study  demonstrated  improved  efficacy  of  the   aforementioned  interventions  compared  to  home  exercise  alone.  Oral  aperture   tightness  and  decreased  size,  known  as  microstomia,  is  a  common  side  effect  of   facial  soft  tissue  sclerosis.    Collagen  deposits  in  the  perioral  tissues  prevent  normal   opening.  A  study  conducted  by  Yuen  et  al.  showed  how  stretching  and  exercise  could   increase  lost  oral  aperture  size  in  patients  with  SSc.  A  non-­‐exercise  group  displayed   a  slight  loss  of  aperture  size  at  a  3  month  follow-­‐up,  and  the  exercise  group  showed   a  significant  amount  of  improvement  (Yuen,  2012).     Muscular  strength  improved  after  a  12-­‐week  exercise  program  containing   resistance  and  aerobic  training;  specifically  improving  1RM  leg  press  and  bench   press,  back  pull  and  hand  grip  strength,  and  overall  muscle  function  (Pinto,  2011).   This  indicated  that  strength  gains  can  be  made  by  patients  suffering  from  systemic   sclerosis  with  the  diagnosis  of  ILD,  which  will  improve  their  ability  to  perform   functional  daily  activities.  The  parameters  of  the  interventions  were  poorly  defined;   however,  the  important  take-­‐away  presented  was  that  this  population  achieved  the   benefits  of  strengthening  exercises  and  it  carried  over  to  improved  function.     The  cardio-­‐respiratory  system  can  be  greatly  affected  in  patients  with  diffuse   SSc.  In  multiple  studies,  interstitial  lung  disease  (ILD),  pulmonary  hypertension,  and  

  20. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   15       cardiac  pathology  have  been  identified  as  leading  causes  of  death  (Ngain,  et  al.,   Joven,  et  al.,  Tyndall,  et  al.,  2010).  The  involvement  of  the  lungs  has  a  direct  effect  on   both  quality  of  life  and  life  expectancy.  A  Cochrane  review  conducted  by  Holland  and   Hill  (2008)  confirmed  that  physical  exercise  significantly  improved  pulmonary   function  related  to  ILD.  This  review  demonstrated  that  physical  therapy   interventions  have  directly  improved  one  of  the  highest  causes  of  mortality  and   disability  in  patients  with  systemic  sclerosis.  Additionally,  exercise  training,   specifically  aerobic  and  resistance  training,  has  been  shown  to  improve  pulmonary   hypertension  (Arena,  2011),  resting  heart  rate,  aerobic  threshold,  and  time-­‐to-­‐ exhaustion  (Pinto,  2011).  Dyspnea,  decreased  forced  vital  capacity,  decreased  lung   volume,  and  fatigue  are  signs  and  symptoms  experienced  by  patients  with  SSc.    Also   pertaining  to  patients  with  SSc  and  a  diagnosis  of  interstitial  lung  disease,  a  case   report  of  a  54-­‐year  old  female  demonstrated  that  a  three-­‐time-­‐per-­‐week  cycle-­‐ ergometer  exercise  training  program,  lasting  6  weeks  and  progressed  from  50%  to   80%  of  peak  workload,  increased  oxygen  consumption  and  workload  at  anerobic   threshold  by  4.2  mL∙kg-­‐1∙min,  and  six-­‐meter  walk  test  by  101m  (Shoemaker,  2009).   As  mentioned  previously  in  this  report,  functional  ability,  pulmonary  function,  and   exercise  tolerance,  assessed  by  measures  such  as  the  SHAQ  and  6-­‐minute  walk  test,   SF-­‐36,  and  manual  muscle  testing  have  all  shown  to  be  positively  influenced  with   physical  exercise  (Chernev  et  al.  2009;  Antolioli  et  al.,  2009;  Oliveira,  2009).     It  is  important  for  the  physical  therapist  to  consider  the  increased  risk  of   osteoporosis  and  thus  risk  of  fracture  when  implementing  an  exercise  program  for   patients  with  scleroderma  (Avouac,  2012).  A  two  to  six-­‐time  increase  in  

  21. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   16       osteoporosis  was  identified  in  this  population  with  several  factors  and  influences   including  decreased  activity  secondary  to  symptoms,  and  medications.  Physical   therapists  should  be  aware  of  this  increased  risk  for  fracture,  and  educate  patients   on  safety  and  recommended  activities  to  increase  bone  density.  No  studies  were   found  that  confirm  this  population  is  capable  of  offsetting  their  risk  of  osteoporosis   with  exercise.     Although  not  present  in  the  case  patient,  myositis,  dermatomyositis  and   polymyositis  are  inflammatory  processes  of  the  musculature  that  are  commonly   seen  in  autoimmune  diseases  such  as  SSc.  An  acute  case  of  myositis  in  a  patient   diagnosed  with  diffuse  systemic  sclerosis  who  was  admitted  to  a  hospital  for  acute   weakness  received  treatment  of  physical  therapy.  Interventions  included  resistive   strengthening  exercise,  range  of  motion  exercise,  and  gross  and  fine-­‐motor  training.   Interventions  were  performed  in  conjunction  with  steroid  treatment.  The   assessment  demonstrated  that  the  patient’s  strength  improved  from  1-­‐2/5  to  4-­‐5/5   in  the  upper  and  lower  extremities.  The  authors  reported  that  more  research  was   needed  to  examine  and  describe  the  appropriate  frequency,  intensity,  type,  and   timing  of  the  exercise  (Chernev,  Gustafson,  &  Medina-­‐Bravo,  2009).     Thermal  modalities  such  as:  superficial  heat,  paraffin  wax,  and  ultrasound   can  be  used  in  conjunction  with  low  force  prolonged  stretching  to  increase  the   length  of  the  high  collagen  content  fibrotic  tissues  (Casale,  1997).  Collagen  displays   plastic  deformation  to  these  types  of  loads,  which  would  promote  increased  joint   range  of  motion  and  soft  tissue  length  (Casale,  1997).  Paraffin  bath  treatments  in   conjunction  with  hand  exercises  have  been  shown  to  improve  joint  mobility,  

  22. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   17       decrease  perceived  hand  stiffness,  and  improve  skin  elasticity  (Sandqvist,  2004).  It   is  important  to  remember  that  there  is  a  highly  compromised  vascular  system  in   this  population.  For  this  reason,  burns  have  a  higher  potential  for  occurrence  and   superficial  heating  should  be  monitored  accordingly.  The  available  evidence-­‐based   literature  regarding  modalities  is  almost  exclusively  concerning  paraffin  wax  bath   treatments  for  the  hands.  A  series  of  case  studies,  and  a  randomized  controlled  trial   demonstrated  that  paraffin  wax  hand  baths  of  20  minutes  duration  prior  to  and  in   conjunction  with  hand  exercises  demonstrated  statistically  significant   improvements  in  measures  such  as  joint  range  of  motion,  grip  and  pinch  strength,   perceived  stiffness,  and  function  items  on  the  Arthritis  Hand  Function  Scale   (Sandqvist  et  al.,  2004;  Manuso  &  Poole,  2009).  It  is  reasonable  to  assume  that   superficial  moist  heating  applied  to  the  case  patient’s  neck  in  a  fashion  similar  to   that  of  the  above  mentioned  case  study  may  improve  the  results  of  the  range  of   motion  and  strengthening  interventions.  All  interventions  administered  to  the  case   patient  were  centered  on  the  patient’s  cervical  dysfunction.  However,  the  case   patient  reported  dysfunction  of  her  hands  during  the  initial  evaluation  that   interfered  with  activities  of  daily  living,  making  it  pertinent  for  the  attending   physical  therapist  to  include  appropriate  evaluation  and  management  of  hand   dysfunction  to  the  plan  of  care.     Myofascial  release  techniques  are  another  intervention  utilized  by  physical   therapists  to  increase  or  maintain  ROM  (Hironobu,  2013).  However,  there  is  limited   evidence  supporting  that  it  is  beneficial  in  decreasing  pain  and  increasing  joint  ROM   (Masi,  2012).  There  needs  to  be  further  research  to  demonstrate  the  efficacy  of  this  

  23. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   18       intervention  in  patients  with  altered  inflammatory  responses,  such  as  patients  with   systemic  sclerosis.  This  intervention  also  could  prove  to  be  effective  in  the   treatment  of  connective  tissue  adhesions  and  palpable  tendon  friction  rubs.  Tendon   friction  rubs  are  described  as  coarse  cracking  and  crepitus  felt  on  palpation.  The   pathology  of  tendon  friction  rubs  in  SSc  is  attributed  to  non-­‐inflammatory  fibrous   deposits  on  the  surface  of  tendon  sheaths  and  the  overlying  fascia  (Rodnan  &   Medsgar,  1968).  Tendon  friction  rubs  have  been  attributed  to  a  more  severe  disease   progression  including:  increased  risk  for  developing  renal,  cardiac,  and  GI   involvement  as  well  as  a  reduced  survival  rate  (Dore,  2013).  Understanding  that  the   presentation  of  this  symptom  correlates  to  more  severe  organ  involvement  should   aid  the  physical  therapist  in  determining  the  need  for  referral  or  additional  organ   system  monitoring.     Problems,  Goals,  and  Plan  of  Care.  The  treating  physical  therapist   indicated  that  per  her  clinical  judgment,  a  combination  of  the  patient’s  age  and   comorbidities  i.e.  the  diagnosis  of  scleroderma,  the  interventions  selected  would  be   of  low  intensity  and  consist  of  what  was  considered  to  be  conservative  treatment.   The  patient’s  identified  problems  include  difficulty  with  ADL’s,  decreased  flexibility,   pain,  range  of  motion,  decreased  soft  tissue  mobility,  and  decreased  strength.  The   treating  physical  therapist  indicated  that  slower  than  typical  healing  times  were   anticipated,  which  was  reflected  in  the  documented  plan  of  care.     Based  on  available  evidence-­‐based  literature  and  the  known  disease  process   of  SSc,  goals  addressing  hand  and  cardio-­‐pulmonary  function  should  be  included,  as  

  24. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   19       those  systems  are  within  the  physical  therapist’s  scope  of  practice  and  directly   effect  functional  independence  and  quality  of  life  (Casale,  1997;  Antonioli,  2009).       The  physical  therapist  responsible  for  the  plan  of  care  determined  that  the   patient  would  be  seen  3  times  per  week  for  one  month.  These  recommendations   were  based  on  the  physical  therapist’s  clinical  judgment    without  availability  of  clear   recommendations  regarding  this  patient  population  in  the  literature.   Aforementioned  studies  identified  treatment  plans  that  ranged  from  single   treatment  interventions  to  plans  consisting  of  sixty-­‐minute  sessions  three  times  per   week.  There  were,  however,  no  comparisons  or  conclusions  that  indicated  a   frequency  or  treatment  duration  recommendation.       It  is  unclear  from  the  available  documentation  if  the  physical  therapist   modified  the  plan  of  care  based  on  the  patient’s  diagnosis  of  SSc.  Administered  care   led  to  decreased  functional  limitations  and  progressed  towards  developed  goals   regarding  the  patient’s  cervical  dysfunction.  However,  the  systemic  nature  of  the   patients  disease,  and  the  known  presentation  of  SSc  would  make  alterations  to   include  addressing  problems  such  as  increased  risk  for  osteoporosis,  decreased   functional  endurance,  and  declining  hand  function  appropriate.     A  copy  of  the  plan  of  care  created  at  the  initial  evaluation  is  provided  below   in  Table  5.  Goals  established  as  part  of  the  plan  of  care  are  detailed  in  Table  6.  The   original  language  and  level  of  detail  are  preserved  in  the  tables.          

  25. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   20       Table  5     Plan  of  Care  at  Initial  Evaluation  (October  29th,  2013).       Assessment:   The  client  tolerated  today’s  treatment/therapeutic   activity  with  minimal  complaints  of  pain  and   difficulty.  In  my  professional  opinion,  this  client   requires  skilled  physical  therapy  in  conjunction   with  home  exercise  program  to  address  the   problems  and  achieve  the  goals  outlined  below   (Table  5).  Overall  rehabilitation  potential  is  good.   The  patient  and/or  family  were  educated  regarding   their  diagnosis,  prognosis,  and  related  pathology.   The  client  exhibits  good  understanding  and   performance  of  the  therapeutic  activity  and   instructions  outlines  in  this  skilled  rehabilitation   session.       ADL’s.  Flexibility.  Pain.  Range  of  Motion.  Soft  Tissue   Mobility.  Strength.     Decrease  Pain.  Improve  Function.  Increase  Range   of  Motion.  Increase  Strength.     Range  of  Motion/Mobility  Improvements.  Muscle   Function  Improvements.  Pain  Relief.       It  is  recommended  that  the  client  attend   rehabilitative  therapy  for  3  visits  a  week  with  and   expected  duration  of  1  month.  Interventions  during   the  course  of  treatment  will  be  directed  toward   addressing  the  problems  and  achieving  the  goals   previously  outlined  (Table  5)   Active  Range  of  Motion  Activities.  Aerobic   Conditioning:  Upper  Body  Ergometer.  Client   Education.  Home  Exercise  Program.  Joint   Mobilization  Techniques.  Manual  Therapy   Techniques.  Modalities:  As  Needed.  Neuromuscular   Re-­‐education.  Self  Care/Home  Management.  Soft   Tissue  Mobilization  Techniques.   Stretching/Flexibility  Activities.  Therapeutic   Exercise.     Impairments  Identified:   Recommendations:  Skilled   Intervention  To:   Treatment  Emphasis  to  Focus   on:     Plan:     Amount,  Frequency  and   Duration:   Therapeutic  Contents:  

  26. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   21       Outcomes.  One  month  after  the  patient  initiated  physical  therapy,  all  initial   objective  measures  were  re-­‐assessed.  Improvements  were  found  in  all  areas  tested   with  progress  made  on  all  goals.  The  patient  was  able  to  increase  cervical  ROM  in   left  rotation  to  match  that  of  the  ipsilateral  side,  strength  improved  in  all  tested   cervical  and  upper  extremity  planes,  and  soft  tissue  mobility  improved.  Table  6   documents  the  initial  problem  list,  goals,  progress,  and  the  physical  therapists   assessment.  The  language  and  level  of  detail  used  by  the  treating  physical  therapist   is  maintained.  The  patient  subjectively  reported  a  decrease  in  pain  although  a   formalized  scale  was  not  utilized,  in  addition  to  reporting  decreased  limitations  in   activities  such  as  driving  and  carrying  light  objects.  Standardized  assessment  tools   such  as  the  SHAQ,  and  visual  pain  analog  scale  are  beneficial  to  document  progress   more  accurately.  Based  on  the  progress  made,  patient  compliance  and  stated   understanding  of  HEP,  as  well  the  patient  scheduling  to  go  out  of  town,  the  patient   was  discharged  from  physical  therapy.                    

  27. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   22       Table  6   Problem  list,  Goals,  and  Assessment.     Problem   Goal   Level  at  initial   visit10/29/13   Level  at  discharge   11/26/13   Physical   Therapist’s   Assessment   11/26/13     Questionnaire:   Neck  Disability   Index   Improve  by   10%:  To  drive   without   increased  neck   pain.   Decrease   forward  head   posture  to   minimum   26.00%   25%  improvement   documented   Posture:   Forward   head/rounded   shoulders   Moderate   “Some  progress”   documented   25%  of  Goal   Met       Range  of   motion:  cervical   spine   Increase  active   rotation  left  &   right  to  45   degrees:  To  turn   head  while   driving.   75%  of  Goal   Met   Active   Motion   Tested   Flexion   Range  of   Motion  in   degrees   52   Active   Motion   Tested   Flexion   Range  of   Motion  in   degrees   52   Left   Rotation   Right   Rotation   Left  Side   Bending   Right   Side   Bending   26   Left   Rotation   Right   Rotation   Left  Side   Bending   Right   Side   Bending   40   41   41   25   25   20   20       Muscle  Testing:   Cervical  planes   MMT   Muscle  Testing:   Upper  extremity   MMT   Improve  to  +4/5   Flexion  L&R  +3/5   Flexion  L&R    -­‐4/5   “good  progress”  noted   Bilaterally:  Adduction  -­‐ 4/5  Flexion  4/5   “good  progress”  noted   50%  of  Goal   Met   To  lift/carry   light  objects   pain  free   Improve   Bilaterally:   Adduction  to   +4/5  Flexion  to  -­‐ 5/5     Improve  to   slight   restriction:  To   sleep  through   the  night   Bilaterally:  Adduction  3/5   Flexion  -­‐4/5   50%  of  Goal   Met   Palpation:   Decreased   mobility     Erector  Spinae,   Suboccipitals,  Upper   trapezius:  Moderate   restriction  bilaterally     Erector  Spinae,   Suboccipitals,  Upper   trapezius:  Mild   restriction  bilaterally   “Excellent  progress”   noted   75%  of   Tissue   Mobility  Goal   Met        

  28. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   23       Discussion   The  primary  goal  of  the  physical  therapist  regarding  patients  with  systemic   sclerosis  is  symptom  management  in  order  to  improve  function,  longevity,  and   quality  of  life.  Improvements  in  early  diagnosis  and  early  intensive  multidisciplinary   treatment  have  led  to  increased  survival  length.  This  places  more  importance  on   maintaining  and  promoting  functional  ability  (Nihtyanova,  2009;  Casale,  1997).    In  a   study  conducted  by  Vincent  and  Wilson  (2006),  some  patients  placed  more   importance  on  maintaining  their  functional  mobility  and  what  they  considered  a   normal  life,  than  they  did  on  anticipation  of  a  shortened  life  expectancy.  A   systematic  review  with  a  meta-­‐analysis  has  shown  that  the  10-­‐year  survival  rate   among  this  population  has  risen  from  near  60%  to  slightly  above  75%  from  1985  to   2002  (Ferri,  2002).  Many  of  the  treatments  that  patients  with  PSSc  undergo  are   pharmacological  and  surgically  based,  including  systemic  medication  trials  and   immunosuppressive  therapy  (“umm.edu”,  2012).  Regarding  the  importance  of   maintaining  function,  the  physical  therapist’s  role  specific  to  patients  with  PSSc   includes  maintaining  joint  ROM,  limiting  connective  tissue  pain  and  stiffness,  and   promoting  cardio-­‐respiratory  fitness.    In  order  to  do  this,  valid  and  appropriate   objective  measurements  need  to  be  taken.  High-­‐level  evidence-­‐based  practice   literature  is  limited  in  regards  to  physical  therapy  examination  and  treatments,   however  the  literature  that  is  available  supports  physical  therapy  interventions.     Passive  and  active  skin  mobilization,  strengthening  exercises,  and  joint  range   of  motion  helps  to  prevent  contractures,  muscular  atrophy,  degradation  of  joints   and  subsequent  pain.  Joint  movement  circulates  synovial  fluid  that  is  compromised  

  29. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   24       due  to  decreased  vascularity  and  limited  motion.  This  supports  joint  nutrition  and   decreases  joint  articular  surface  breakdown  (Kisner  &  Colby,  2007).  Additionally,   joint  movement  stimulates  mechanoreceptors  that  are  influential  in  modulating   pain  (Kisner  &  Colby,  2007).    It  is  important  to  remember  that  each  exercise   program  requires  individual  customization.  A  patient  with  SSc  who  has  significant   joint  pathology  should  be  placed  on  an  exercise  program  that  does  not  exacerbate   joint  pain  or  swelling.  Not  doing  so  may  cause  degradation  of  articular  cartilage  that   will  in  turn  increase  the  inflammatory  process  and  dysfunction  associated  with  the   disease.  In  general,  joint  ROM  and  muscular  strength  should  be  the  focus  of   interventions  while  limiting  joint  inflammation  (Casale,  1997).  Furthermore,   exercise  prescription  should  be  based  on  objective  findings  and  progressed  based   on  patient  tolerance  and  results  (ACSM,  2013).  The  documentation  available  for  this   case  patient  over  the  one-­‐month  plan  of  care  provides  few  details  in  regards  to  the   rationale  for  exercise  progression,  and  only  partially  addressed  objective  findings  of   weakness.  It  is  important  for  the  physical  therapist  to  closely  monitor  patients  with   this  diagnosis  to  determine  the  efficacy  of  treatment,  and  adhere  to  evidence-­‐based   exercise  prescription  parameters.   Physical  exercise  directly  improves  lung  function,  and  is  considered  the  standard   of  care.    (Arena,  2011;  Baptista,  2012;  Pinto,  2011;  Shoemaker,  2009).  However,   according  to  one  study,  only  46%  of  patients  diagnosed  with  systemic  sclerosis   participated  in  physical  therapy  (Johnson,  2006).    It  is  very  important  to  note  that   exercise-­‐induced  pulmonary  hypertension  has  been  regarded  as  a  common  finding   in  patients  with  SSc  (Baptista,  2012).    This  speaks  to  the  importance  of  

  30. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   25       appropriately  trained  and  informed  clinicians  monitoring  vitals  and  patient   presentation  during  activity.  Physical  exercise  has  also  been  shown  to  enhance   overall  health.  It  improves  quality  of  life,  improves  functional  capacity,  and  has  been   shown  to  prevent  the  progression  and  improve  the  prognosis  of  multiple  diseases   (“CDC.gov”,  2012).  In  addition  to  the  health  benefits,  exercise  can  promote   psychological  health  and  provide  improved  quality  to  life  in  the  form  of  goal   accomplishment  and  giving  the  patient  a  feeling  of  control  (“CDC.gov”,  2012).     The  case  patient’s  physical  therapist  stated  that  she  was  mildly  familiar  with   the  diagnosis  of  systemic  sclerosis.  Based  on  the  clinical  judgment  of  the  physical   therapist,  the  case  patient  was  treated  for  cervical  neck  dysfunction  conservatively.   The  physical  therapist  anticipated  slower  than  typical  gains  and  increased  healing   times  due  to  the  vascular  compromise  secondary  to  the  disease  process.  The   administered  plan  of  care  and  one  month  of  treatment  interventions  showed  25%   efficacy  for  the  correction  of  posture,  50%  effective  regarding  improvements  in   strength,  and  75%  successful  in  improving  the  patients  cervical  neck  range  of   motion.     The  APTA  states    “Physical  therapists  provide  patient/client  management  in   primary  care  through  the  processes  of  screening,  examination,  evaluation,  diagnosis,   prognosis,  intervention,  education,  prevention,  coordination  of  care,  and  referral  to   other  providers  to  prevent,  remediate,  decrease,  or  slow  the  progression  of   impairments,  activity  limitations,  and  participation  restrictions,  and  lessen  the   impact  of  environmental  barriers,  and  optimize  cost-­‐effective  clinical  outcomes.  “   (APTA,  2012).  Based  on  this,  it  is  the  physical  therapist’s  responsibility  to  evaluate  

  31. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   26       the  body  systems  that  are  affected  by  the  disease  process  of  SSc  and  to  educate  the   patient  and  appropriate  health  care  practitioners.  Interventions  provided  in  regards   to  cervical  dysfunction  demonstrated  progress  towards  goals;  however,  assessment   of  deep  neck  flexor  strength  and  muscle  recruitment  during  respiration  were  not   mentioned  during  the  examination  or  course  of  treatment  and  could  have  an   influence  on  long  term  progress  (Falla,  2004).  Literature  concerning  evidence-­‐based   assessment  and  interventions  detail  the  need  to  address  the  known  systems  that  are   typically  affected  by  the  disease  process  of  SSc.  Although  available  literature  is   limited,  the  plan  of  care  provided  by  physical  therapists  should  address  morbidities   concerning  cardiopulmonary  health,  functional  strength  and  endurance,  and  hand   function.       The  extent  of  patient  education  was  not  clearly  stated  in  the  available   documentation.  Patients  with  SSc  should  be  educated  and  referred  to  resources   such  as  the  Johns  Hopkins  Scleroderma  Center,  or  the  Scleroderma  Foundation,   which  is  a  thorough  online  based  information  center  complete  with  everything  from   support  groups  and  forums  to  the  most  current  information  and  research.  Evidence   based  practice  would  have  also  included  a  baseline,  or  a  continuation  of  the   monitoring  of  the  major  systems  that  are  impacted  by  the  disease  process  that  are   within  our  professions  scope  of  practice.  These  would  include  pulmonary  tests  such   as  FEV1  and  lung  capacity,  blood  pressure  and  cardiac  response  to  graded  exercise,   and  functional  assessments  such  as  the  Scleroderma  Health  Assessment   Questionnaire  or  the  Cochin  Hand  Function  Scale.  The  inclusion  of  these  tests  would   help  form  a  baseline  with  which  to  measure  progression,  substantiate  appropriate  

  32. Evidence-­‐Based  Practice  for  Patients  with  Scleroderma   27       interventions,  and  provide  useful  information  to  clinicians  of  other  disciplines   (Follansbee,  2004;  Clements,  2009).       Considering  other  disciplines,  it  is  strongly  suggested  that  patients  who   either  display  signs  of  systemic  sclerosis  or  carry  the  diagnosis  of  SSc  are  under  the   care  of  a  rheumatologist  (www.hopkinsscleroderma.org).  The  global  effects  of  SSc   interfere  with  the  processes  of  nearly  every  body-­‐system.  Complications  and   symptoms  extend  beyond  the  content  of  this  case  report.    Notably,  SSc  has   potentially  profound  effects  on  the  renal,  GI,  and  sexual  processes,  which  require   professional  monitoring  and  consultation  for  appropriate  evidence-­‐based   management.   Conclusion   After  reviewing  the  literature,  it  is  clear  that  exercise  and  other  physical   therapy  interventions  are  beneficial  and  produce  significant  benefit  for  patients   with  SSc.  However,  there  is  not  enough  high  quality  evidence  regarding  exercise   protocols,  testing  measures,  or  treatment  guidelines.  The  disease  is  progressive  and   variable  from  patient  to  patient  so  interventions  need  to  reflect  patient  needs;   however,  there  is  a  lack  of  guidelines  that  could  help  physical  therapists  in   providing  evidence-­‐based  care.  Based  on  the  diagnosis  of  Systemic  Sclerosis,   physical  therapists  should  consider  evidence-­‐based  examination  and  intervention   for  all  symptoms  found  to  be  positively  affected  by  physical  therapy.  Additionally,   physical  therapists  need  to  be  aware  of  the  systemic  nature  of  the  disease,  and  the   recommendations  for  interdisciplinary  delivery  of  health-­‐care,  and  early   intervention  to  prolong  functional  independence  and  quality  of  life.  

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