!

!

Current Patient Weight: ___________________! ! ! ! ! "#$%&'(!)%*+,!------------------------!!!!!!!!./012%3+,!--------------------!!!! !

!

CHRONIC GVHD ACTIVITY ASSESSMENT- CLINICIAN Component Findings Scoring (see skin score worksheet)

45&*6+3%*#7(!5%(6!#8!%9&!(#5*! !!!!!!!!!!!!!!!!!!!!!!!!:!;<=!>3%?!@AA:B!

.#C+%DE+!(FE+5#(G(!! !!!!!!!!!!!!!!!!!!!!!!!!:!;<=!>3%?!@AA:B!

2#9H3#C+%DE+!(FE+5#(G(!>6G$+D#79$19#9HIG9F6%DE+B!#5!(7DF7*%9+#7(!(FE+5#(G(18%(FGG*G(! !!!!!!!!!!!!!!!!!!!!!!!!:!;<=!>3%?!@AA:B!

Skin

JEF+5>(B,!(+E+F*!*6+!E%5K+(*!7EF+5%*GC+!E+(G#9L!%9$!3+%(75+!G*(!E%5K+(*!$G3+9(G#9!G9!F3!%9$!3%5M!

E#F%*G#9!#8!7EF+5!

!

N#F%*G#9,!!!-------------------!

!

N%5K+(*!$G3+9(G#9,!----------F3!

Eyes ;GE%*+5%E!<F6G53+5'(!"+%5!"+(*!

>OG*6#7*!%9+(*6+(G%B!!

G9!I+5(#9(!P!&+%5(!#5!#E$+5!

/GK6*!4&+,!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!33!#8!O+**G9K! N+8*!4&+,!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!33!#8!O+**G9K

Mouth !

Mucosal change

2#!+CG$+9F+!

#8!FQRS)!

.GE$! ! !.#$+5%*+ <+C+5+

Erythema 2#9+!A!

.GE$!+5&*6+3%!#5!

3#$+5%*+!+5&*6+3%!

>TUV:B!

@!.#$+5%*+!> UV:B!#5!

<+C+5+!+5&*6+3%!

>TUV:B!

U!<+C+5+!+5&*6+3%!

> UV:B!!W!

Lichenoid 2#9+!A!

S&I+5M+5%*#*GF!

F6%9K+(>TUV:B!@!

S&I+5M+5%*#*GF!

F6%9K+(>UVHVA:B!U!

S&I+5M+5%*#*GF!F6%9K+(!

>XVA:B!W!

Ulcers 2#9+!A!

2#9+!A!

JEF+5(!G9C#ECG9K!> UA:B!W!

<+C+5+!7EF+5%*G#9(!

>XUA:B!Y!

Mucoceles* 2#9+!A!

@HV!37F#F+E+(!@!

YH@A!(F%**+5+$!

37F#F+E+(!U!

ZC+5!@A!37F#F+E+(!W!

!!!!!!!!!!! ! ! ! ! ! !

! [.7F#F+E+(!(F#5+$!8#5!E#O+5!

E%DG%E!%9$!(#8*!I%E%*+!#9E&!

! Total score for all mucosal changes

!

Blood Counts \E%*+E+*!]#79*!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

!!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!^17N!

JN2!!!!!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!^17N!

"#*%E!_;]!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!^17N!

JN2!

!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!^17N!

:!4#(G9#I6GE(!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!:!

Liver Function Tests "#*%E!(+573!DGEG57DG9!

!

!!!!!!!!!!!!!!!!!!!!!!!!!!!3K1$N!

JN2!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!

!!!!!!!!!!!!!!!!!!!!!!!!3K1$N!

=N"!

!

!!!!!!!!!!!!!!!!!!!!!!!!!!!J1N!

JN2!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!J1N!

=EM%EG9+!\6#(I6%*%(+ !!!!!!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!J1N!

JN2!

!!!!!!!!!!!!!!!!!!!!!!!!!!!J1N!

FORM A

Gastrointestinal-Upper GI

! "#$%&!'#()*(&!!+,!

! -./$*0)#!!!!!+,!

! 1#2'*#!3!4/5)().6!

0= no symptoms 1=mild, occasional symptoms, with little reduction in oral intake during the past week 2=moderate, intermittent symptoms, with some reduction in oral intake during the past week 3=more severe or persistent symptoms throughout the day, with marked reduction in oral intake, on almost every day of the past week!

Gastrointestinal-Esophageal ! 7&'89#6)#!!!!!+,!

! +:&./89#6)#!

0= no esophageal symptoms 1=Occasional dysphagia or odynophagia with solid food or pills during the past week 2=Intermittent dysphagia or odynophagia with solid foods or pills, but not for liquids or soft foods, during the past week 3=Dysphagia or odynophagia for almost all oral intake, on almost every day of the past week!

Gastrointestinal-Lower GI ! 7)#$$9*#!

0= no loose or liquid stools during the past week 1= occasional loose or liquid stools, on some days during the past week 2=intermittent loose or liquid stools throughout the day, on almost every day of the past week, without requiring intervention to prevent or correct volume depletion 3=voluminous diarrhea on almost every day of the past week, requiring intervention to prevent or correct volume depletion

Lungs ! ;$/.<9)/%)()'!+=%)(*$#.'!

>2%5/.#$&!?2.<()/.!@*'('!A)(9!7)BB2').6!

C#8#<)(&!!!D#((#<9!$*8/$(!B/$!8*$'/.E!F!&$'!/%:G!

?"4HI!

!

J!>$*:)<(*:!

K).6%*!;$*#(9!7LC+!D#:M2'(*:!B/$!9*5/6%/=).G!

!

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!J!>$*:)<(*:!

Health Care Provider Global Ratings: In your opinion, do you think that this patient’s chronic GVHD is mild, moderate or severe? NO./.*!

IO!5)%:!

PO5/:*$#(*!

QO'*R*$*!

Where would you rate the severity of this patient’s chronic GVHD symptoms on the following scale, where 0 is cGVHD symptoms that are not at all severe and 10 is the most severe cGVHD symptoms possible: !!

!!!!!!!!!!!!!N!!!!!!!I!!!!!!!!!P!!!!!!!!!Q!!!!!!!!!S!!!!!!!!!F!!!!!!!!!T!!!!!!!!!U!!!!!!!!!V!!!!!!!!!!W!!!!!!!!!IN!

cGvHD symptoms Most severe cGvHD not at all severe symptoms possible!

Over the past month would you say that this patient’s cGVHD is XQO!4*$&!52<9!=*((*$!!

XPO!Y/:*$#(*%&!=*((*$!

XIO!-!%)((%*!=*((*$!

!!NO!-=/2(!(9*!'#5*!

HIO-!%)((%*!A/$'*!

HPOY/:*$#(*%&!A/$'*!!

HQO4*$&!52<9!A/$'*!

Z$)8!K($*.6(9!D7/5).#.(![#.:G!Functional Performance!D).!8*$'/.'!ES!&*#$'!/%:G!

! \#%]!@)5*!

! Z$)8!K($*.6(9!

@/(#%!7)'(#.<*!\#%]*:!).!P!Y).2(*'^!!

!

125=*$!/B!%#8'^!_______!D0!FN!B**(G!X!B).#%!8#$()#%!

%#8^!_____!B**(!!O!______!B**(!A#%]*:!).!P!5).2(*' !!!!!

@$)#%!`I!

!

!!!!!!!!!!!!8')!

@$)#%!`P!

!

!!!!!!!!!!!8')!

@$)#%!`Q!

!

!!!!!!!!!!!!8')!

,#.6*!/B!Y/()/.^!!

o! 1/(!8*$B/$5*:!

!

o! >9&')<#%!@9*$#8&!,*8/$(!-((#<9*:!

Score Lansky Performance Status Scale Definitions (circle from 0-100) (persons < 16 years old)

Karnofsky Performance Status Scale Definitions (circle from 0-100) (persons 16 years or older)

100 ?2%%&!#<()R*a!./$5#%! 1/$5#%!./!</58%#).('b!./!*R):*.<*!/B!:)'*#'*!

90 Y)./$!$*'($)<()/.'!).!89&')<#%%&!'($*.2/2'!#<()R)(&! -=%*!(/!<#$$&!/.!./$5#%!#<()R)(&b!5)./$!')6.'!/$!'&58(/5'!/B!:)'*#'*!

80 -<()R*a!=2(!()$*'!5/$*!c2)<]%&! 1/$5#%!#<()R)(&!A)(9!*BB/$(b!'/5*!')6.'!/$!'&58(/5'!/B!:)'*#'*!

70 ;/(9!6$*#(*$!$*'($)<()/.!/B!#.:!%*''!()5*!'8*.(!).!8%#&!#<()R)(&! C#$*'!B/$!'*%Bb!2.#=%*!(/!<#$$&!/.!./$5#%!#<()R)(&!/$!(/!:/!#<()R*!A/$]!

60 d8!#.:!#$/2.:a!=2(!5).)5#%!#<()R*!8%#&b!]**8'!=2'&!A)(9!c2)*(*$!#<()R)()*'! ,*c2)$*'!/<<#')/.#%!#'')'(#.<*!=2(!)'!#=%*!(/!<#$*!B/$!5/'(!8*$'/.#%!.**:'!

50 Z*('!:$*''*:!=2(!%)*'!#$/2.:!52<9!/B!(9*!:#&a!./!#<()R*!8%#&!=2(!#=%*!(/!8#$()<)8#(*!).!#%%!c2)*(!8%#&!#.:!#<()R)()*'! ,*c2)$*'!</.'):*$#=%*!#'')'(#.<*!#.:!B$*c2*.(!5*:)<#%!<#$*!

40 Y/'(%&!).!=*:b!8#$()<)8#(*'!).!c2)*(!#<()R)()*'! 7)'#=%*:b!$*c2)$*'!'8*<)#%!<#$*!#.:!#'')'(#.<*!

30 e.!=*:b!.**:'!#'')'(#.<*!*R*.!B/$!c2)*(!8%#&! K*R*$*%&!:)'#=%*:b!9/'8)(#%!#:5)'')/.!)'!).:)<#(*:!#%(9/269!:*#(9!./(!

)55).*.(!

20 +B(*.!'%**8).6b!8%#&!*.()$*%&!%)5)(*:!(/!R*$&!8#'')R*!#<()R)()*'! 4*$&!')<]b!9/'8)(#%!#:5)'')/.!.*<*''#$&b!#<()R*!'288/$()R*!($*#(5*.(!

.*<*''#$&!

10 1/!8%#&b!:/*'!./(!6*(!/2(!/B!=*:! Y/$)=2.:b!B#(#%!8$/<*''*'!8$/6$*'').6!$#8):%&!

0 d.$*'8/.')R*! 7*#:!

Today’s Date: ________________________ MR#/Name: ________________________

FORM B

CHRONIC GVHD ACTIVITY ASSESSMENT-PATIENT SELF REPORT

Symptoms Please rate how severe the following symptoms have been in the last seven days. Please fill in the circle below from 0 (symptom has not been present) to 10 (the symptom was as bad as you can imagine it could be) for each item.

Not As Bad As You Present Can Imagine 0 1 2 3 4 5 6 7 8 9 10

Your skin itching at its WORST? O O O O O O O O O O O

Your mouth dryness at its WORST? O O O O O O O O O O O

Your mouth pain at its WORST? O O O O O O O O O O O

Your mouth sensitivity at its WORST? O O O O O O O O O O O

What is your main complaint with regard to your eyes?

Eyes

Please rate how severe is this eye symptom, between 0 (not at all severe) and 10 (most severe):

0 1 2 3 4 5 6 7 8 9 10

Vulvovaginal Symptoms (females only)

Do you have any burning, pain or discomfort in the area of your vagina, vulva or labia? OR Do you have any discomfort or pain with sexual intercourse?

o Yes o No o Not applicable

Patient Global Ratings: 1. Overall, do you think that your chronic graft versus host disease is mild, moderate or severe? 0= none 1= mild 2=moderate 3=severe 2. Please circle the number indicating how severe your chronic graft versus host disease symptoms are, where 0 is cGVHD symptoms that are not at all severe and 10 is the most severe chronic GVHD symptoms possible. 0 1 2 3 4 5 6 7 8 9 10 cGVHD symptoms Most severe cGVHD not at all severe symptoms possible 3. Compared to a month ago, overall would you say that your cGVHD symptoms are: +3= Very much better +2= Moderately better +1=A little better 0= About the same -1=A little worse -2=Moderately worse -3=Very much worse

Attach copies of:

Adults (persons 18 years or older): Children/Adolescents (persons 17 years or younger): -Lee cGvHD Symptom Scale -Lee cGVHD Symptom Scale (persons 8-12 years old may -Human Activity Profile complete with help of the health care professional) -SF-36 v.2 -ASK - Activities Scale for Kids -FACT-BMT -CHRIs-Generic and Disease Specific Inventory

3

NI H C G V H D O R G A NS SC O R E [A D APT E D F R O M F I L IPO V I C H 2005]

SC O R E 0 SC O R E 1 SC O R E 2 SC O R E 3

PE R F O R M A N C E SC O R E:

K PS E C O G LPS

Asymptomatic and fully active (ECOG 0; KPS or LPS 100%)

Symptomatic, fully ambulatory, restricted only in physically strenuous activity (ECOG 1, KPS or LPS 80-90%)

Symptomatic, ambulatory, capable of self-care, >50% of waking hours out of bed (ECOG 2, KPS or LPS 60-70%)

Symptomatic, limited self-care, >50% of waking hours in bed (ECOG 3-4, KPS or LPS <60%)

SK IN

Clinical features:

Maculopapular rash Lichen planus-like features Papulosquamous lesions or

ichthyosis Hyperpigmentation Hypopigmentation Keratosis pilaris Erythema Erythroderma Poikiloderma Sclerotic features Pruritus Hair involvement Nail involvement

% BSA involved

No Symptoms <18% BSA with disease signs but N O sclerotic features

19-50% BSA O R involvement with superficial sclerotic features

(able to pinch)

>50% BSA O R deep sclerotic

(unable to pinch) O R impaired mobility, ulceration or severe pruritus

M O UT H No symptoms Mild symptoms

with disease signs but not limiting oral intake significantly

Moderate symptoms with disease signs with partial limitation of oral intake

Severe symptoms with disease signs on examination with major limitation of oral intake

E Y ES

Mean tear test (mm):

>10 6-10 <5 Not done

No symptoms Mild dry eye symptoms not affecting ADL (requiring eyedrops < 3 x per day) O R asymptomatic signs of keratoconjunctivitis sicca

Moderate dry eye symptoms partially affecting ADL (requiring drops > 3 x per day or punctal plugs), W I T H O U T vision impairment

Severe dry eye symptoms significantly affecting ADL (special eyeware to relieve pain) O R unable to work because of ocular symptoms O R loss of vision caused by keratoconjunctivitis sicca

4

SC O R E 0 SC O R E 1 SC O R E 2 SC O R E 3

G I T R A C T No symptoms Symptoms such as dysphagia, anorexia, nausea, vomiting, abdominal pain or diarrhea without significant weight loss (<5%)

Symptoms associated with mild to moderate weight loss (5-15%)

Symptoms associated with significant weight loss >15%, requires nutritional supplement for most calorie needs O R esophageal dilation

L I V E R

Normal LFT Elevated Bilirubin, AP*, AST or ALT <2 x ULN

Bilirubin >3 mg/dl or Bilirubin, enzymes 2-5 x ULN

Bilirubin or enzymes > 5 x ULN

L UN GS

F E V1 D L C O

No symptoms

FEV1 > 80% O R LFS=2

Mild symptoms (shortness of breath after climbing one flight of steps)

FEV1 60-79% O R LFS 3-5

Moderate symptoms (shortness of breath after walking on flat ground)

FEV1 40-59% O R LFS 6-9

Severe symptoms (shortness of breath at rest; requiring 02)

FEV1 <39% O R LFS 10-12

JO IN TS A ND F ASC I A No symptoms Mild tightness of

arms or legs, normal or mild decreased range of motion (ROM) A ND not affecting ADL

Tightness of arms or legs O R joint contractures, erythema due to fasciitis, moderate decrease ROM A ND mild to moderate limitation of ADL

Contractures W I T H significant decrease of ROM AND significant limitation of ADL (unable to tie shoes, button shirts, dress self etc.)

G E NI T A L T R A C T No symptoms Symptomatic with

mild signs on exam A ND no effect on coitus and minimal discomfort with gynecologic exam

Symptomatic with moderate signs on exam A ND with mild dyspareunia or discomfort with gynecologic exam

Symptomatic W I T H advanced signs (stricture, labial agglutination or severe ulceration) A ND severe pain with coitus or inability to insert vaginal speculum

* AP may be elevated in growing children, and not reflective of liver dysfunction

O ther indicators, clinical manifestations or complications related to c G V H D (check all that apply and assign a score to its sever ity (0-3) based on its functional impact (none 0,mild -1, moderate -2, severe 3) Esophageal stricture or web___ Pericardial Effusion___ Pleural Effusion(s)___ Ascites (serositis)___ Nephrotic syndrome___ Peripheral Neuropathy___ Myasthenia Gravis___ Cardiomyopathy___ Eosinophilia > 500 l___ Polymyositis___ Cardiac conduction defects___ Coronary artery involvement___ Progressive onset O T H E RS: _________

2

Lung function score (L FS) is computed from the extent of F E V1 and D L C O compromise, (>80% = 1, 70 79% = 2, 60 69% = 3, 50 59% = 4, 40 49% = 5, <40% = 6). The scores for FEV1 and DLCO are then added together, and the sum is reduced to an overall category according to the following table:

Category Lung function L FS

I Normal 2 II Mild decrease 3 5 III Moderate decrease 6 9 IV Severe decrease 10 12

LFS has not previously been utilized in chronic GVHD response assessments and its role in this setting needs to be determined.

1

Supplemental Information: Definitions for N I H Global Assessment[F ilipovich 2005], Intensity of Immunosuppression Scale (I IS) [G rkovic 2012], Therapeutic Intent (T I) [Mitchell 2010] and clinician 7-point global assessment of change (C G A)[Pavletic 2006], Lung Function Score[F ilipovich 2005].

N I H Global Assessment: 1. Mild cGVHD = One or two organ or sites involved with no clinically significant functional impairment (max individual organ score 1). 2. Moderate cGVHD = One organ or site with clinically significant but no major disability (max score 2), or three or more organs or sites with no clinically significant functional impairment (max score 1). A lung score of 1 is also moderate cGVHD. 3. Severe cGVHD = Major disability caused by cGVHD (score 3) of one or more organs or sites. A lung score of 2 or 3 is also classified as severe cGVHD. Intensity of Immunosuppression Scale (I IS): 1. None/mild = single-agent prednisone 0.5 mg/kg per day 2. Moderate = single-agent prednisone >0.5 mg/kg per day and/or any single agent/modality 3. High = 2 or more agents/modalities ± prednisone >0.5 mg/kg per day. Therapeutic Intent (T I): 1. Active: (1) increase systemic therapy because cGVHD is worse; (2) substitute systemic therapy due to lack of response; and (3) withdraw systemic therapy due to lack of response. 2. Non-active: (1) decrease systemic therapy because cGVHD is better; (2) not change current systemic therapy because cGVHD is stable; (3) alter systemic therapy owing to its toxicity. 3. Other: either did not receive any immunosuppressive therapy or did not meet any of the criteria.

Clinician 7-change over the past month (7-point scale):

1. Worsened (-3 = very much worse; - 2 = moderately worse; -1 = a little worse), 2. Unchanged (0 = about the same) 3. Improved (+1= a little better; + 2 = moderately better; + 3= very much better). This scale is change over the past

overall clinical history over the previous month.

!!Lee Score Please let us know whether yo have been bothered by any of the following problems in the past month:

!!!!!!!! !!!!!!

From Lee et al. BBMT, 8:444-452, 2002.

Short Form 36 Health Survey : SF-36 !!

!!!!!!!!!!

!!!!

!!!!!!!!!!!!!!!!!!

!!!!!!!!!

!!!!