INPRACTICE-D-13-00054; Online Repository Figure E1 Jennings, et al. 1
THE MASTOCYTOSIS SOCIETY
2010 TMS PATIENT SURVEYFOR MEMBERSAND OTHERS
Understanding and Agreement
Concerning Responses to Questionnaire
If you would like to participate in this research survey,
1. Please READ and CHECK EACH of these statements:
□ I understand that my answers are for the purpose of research into the experiences and opinions of people with mastocytosis or other mast cell disorders.
□ I understand that I will not be receiving any financial compensation; my only compensation will be helping to further the understanding of this disease so that future research, treatments and care may be improved.
□ I understand that my answers will be completely anonymous (identification numbers on questionnaires are for quality control in processing).
2. Please check ONLY ONEof these statements:
I am an adult (18 years of age or older) who will answer the questions for and about:
□ myself
□ one of my children
□ another adult
□ I am younger than 18, but my parent or guardian will consider these questions with me.
DEMOGRAPHICS & DIAGNOSIS
1. Please tell us a little about yourself (check one):
□I have mastocytosis, mast cell activation disorder, or another mast cell disorder
□I am answering this survey for someone else who has one of these disorders
NOTE: “YOU” & “YOUR” REFER TO THE PATIENT IN ALL REMAINING QUESTIONS
2. What is your birthdate? Month______Year______
3. Are you male □ or female □?
4. How would you describe your ethnic group?
(You may check more than one box.)
□ Black
□ White
□ Hispanic, Puerto Rico or other Caribbean
□ Hispanic, Other Latin American
□ Native American
□ Middle Eastern
□ Asian, Chinese
□ Asian, Japanese
□ Asian, Other
□ PacificIsland
□ Other ______
5. What is the mast cell disease diagnosis that you have been given or what mast cell disease does your physician consider you to have?Please check all that apply.
□Cutaneous (skin) Mastocytosis:
□ Pediatric Mastocytosis
□Urticaria Pigmentosa, (or “UP”) relating to hives and skin lesions
□Telangiectasia Macularis Eruptiva Perstans, (“TMEP”)
□Solitary Mastocytoma, (single "clump" of mast cells or mast cell tumor)
□Diffuse Cutaneous Mastocytosis
□Systemic Mastocytosis (more than one organ with or without skin signs)
□Indolent (slowly developing) Mastocytosis
□Smoldering Mastocytosis
□Isolated Bone Marrow Mastocytosis
□Aggressive Mastocytosis
□Mastocytosis with associated hematologic disorder
□Mast Cell Leukemia
□Mast Cell Activation Disorder or Syndrome (MCAD, MCAS)
□ Idiopathic Anaphylaxis
□ Other______
□ Has not been determined
□ Not sure
6. Is your disease classification based on the CURRENT (2001) WHO (World Health Organization) criteria for mast cell disorders?
Yes □ No □ Unknown/Not sure □
7. Is your disease classification confirmed by test results or is it only suspected?
□ Confirmed by test results □ Suspected, but not confirmed □ Not sure
8. When did you first have symptoms? Month ______Year______
9. When did a physician first tell you that you might have mastocytosis,mast cell activation disorder, or other mast cell disorder?
Month______Year______
□ I have not been officially diagnosed with a mast cell disorder
(Optional) Describe or comment if needed ______
______
10. Who diagnosed your mast cell disorder? If more than one physician participated in your diagnosis, please check all that apply.
□ My primary care physician
□ My allergist
□ My haematologist/oncologist
□ My dermatologist
□ My gastroenterologist
□ Other, please specify______
□ Self diagnosed
□ I have not received a mast cell disorder diagnosis
SYMPTOMS, GENERAL PROBLEMS AND MEDICATIONS
11. Please check each symptom below that you have experienced in relation to your mast cell disorder. Please indicate (circle the number)HOW OFTEN each symptom affects you (1=never; 2=rarely; 3=occasionally; 4=daily) and HOW SEVERE each symptom is(1=not at all; 2= a little bit; 3= moderately; 4= extremely).
Next to each of the symptoms listed below, please list the medications that seem to relieve it(if no relief, please leave blank).To help you fill out this question, there is a list of medications that are commonly used by mast cell disorder patients at the end of this survey(you can use the code number printed in front of each medication). Please also list any other medications that are not on this list, but that relieve any given symptom.If you do not know which medications you take for any given symptom, you can leave the medication section blank.
Example:
Itching
How often? 1 2 4 How severe? 1 3 4
Relieved by ______55______
Please note that in this example, “55” refers to “Steroid cream, topical“, as marked in the medication list at the end of this survey. You can also write down the names of medications not provided on the list.
SKIN (CUTANEOUS)SYMPTOMS:
□ Urticaria pigmentosa(UP) rash or “TMEP” eruptions
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Itching
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Flushing from the mid chest up, including the neck and face; usually dry
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Dermatographism(hives appear on previously clear skin when scratched or pressed)
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Darier's sign[flat, tan or red spot (urticaria pigmentosa) that becomes large red hive if stroked or scratched]
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Skin rashes (such as hives) other than UP or TMEP
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
PAIN SYMPTOMS:
□Bone
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Joint
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Muscle, nerve and connective tissue
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Stomach
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Upper abdomen
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Lower abdomen
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Chest
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Other pain, please specify: ______
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
OTHER SYMPTOMS:
□ Cardiac symptoms/angina
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ High blood pressure episodes
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Light-headedness and/or syncope (loss of consciousness)
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Headaches (migraine or other type)
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Angioedema (swelling of the throat and face)
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Brain fog/cognitive difficulties
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Wheezing or asthma
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Gastroesophageal reflux (GERD)
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Nausea and vomiting
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Bloating, abdominal
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Diarrheawith or without malabsorption
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Anaphylactic shock
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Fatigue
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Anxiety
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Depression
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Overall weakness (either episodes or constant)
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□Other symptom, please specify: ______
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
□ Other symptom, please specify: ______
How often? 1 2 3 4 How severe? 1 2 3 4
Relieved by ______
12. What medications have you taken for your mast cell disorder in the past that have NOT worked?Please list any medicines that you now takeor have previously taken for your mast cell disorder symptomsthat do NOT appear to relieve these symptoms or do not appear to work. To help you fill out this question, there is a list of medications that are commonly used by mast cell disorder patients at the end of this survey (you can use the code number printed in front of each medication). If you have taken other medications for your mast cell disorder that did not work and are not on the list below, please also include those medications.
______
______
13. What SIGNIFICANTside effects do you experience with the drugs that you take orhave taken in the past for symptoms related to your mast cell disorder?
Medication List:To help you fill out this question, there is a list of medications that are commonly used by mast cell disorder patients at the end of this survey (you can use the code number printed in front of each medication). Please also list any other mast cell disorder-related medications that are not on the list, but have caused significant side-effects. Also, please list each medication separately.
Side Effect List:(You can circle the number of a given side effect type and/or list other side effects)1 =(allergic reaction, including skin rash);2 =(nausea,vomiting, diarrhea, abdominal pain);3 =(light-headedness, dizziness and fainting);4 = (headache or migraine);5 =(change in cardiac rhythm or rate);6 =change in blood pressure; 7 =change in liver enzymes;8 =shortness of breath;9 =muscle pain; other (please specify).
Drug#1______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects______
Drug#2______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#3______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#4______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#5______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#6______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#7______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#8______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#9______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
Drug#10______Side effects: 1 2 3 4 5 6 7 8 9
Other Side effects ______
14. Do you have aPRESCRIPTION for self-injectable epinephrine (examples: EpiPen or Twinject)? Yes □ No □ Not sure □
15. Do you CARRYself-injectable epinephrine with you?
Yes, always□ Yes, sometimes □ No □
LABORATORY INVESTIGATIONS AND OTHER TESTS
16. Please indicate all tests that you recall being done in relation to your mast cell disorderand how often the tests are currently routinely performed. Please circle the number(1= not done routinely; 2=four or more times per year; 3= two to three times per year; 4= once per year; for other timing between tests, please specify).NOTE: In database files, corresponding numbers are as follows: (5= not done routinely; 1= four or more times per year; 2= two to three times per year; 3= once per year;0 = no selection) due to a difference in coding that did not affect how respondents recorded their data since words (e.g., “not done routinely”) were selected from a drop-down box.
General clinical exam for cutaneous and/or systemic symptoms
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Serum Tryptase
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Complete Blood Count
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Serum Chemistries
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Serum Ferritin Level
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
24-hour Urine for Histamine or Histamine Metabolites
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
24-hour Urine for Prostaglandins
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Bone Marrow Biopsy
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Skin Biopsy
Test ever performed? Yes□ No□ Not Sure□
Visual Skin Assessment
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Photographic Documentation of Skin Involvement
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Bone Scan (a radioactive tracer is injected for this test)
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Bone Density (detects osteopenia and osteoporosis)
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
X-rays or CT Scan
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
c-kit (or other genetic testing)
Test ever performed? Yes□ No□ Not Sure□
Other test, please specify: ______
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Other test, please specify: ______
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Other test, please specify: ______
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
Other test, please specify: ______
Test ever performed? Yes□ No□ Not Sure□
How frequently (see key above)? 1 2 3 4 Other timing ______
17. Does your physician measure your serum tryptase levels at symptom-free intervals to establish a baseline? Yes□ No□ Not Sure□
18. Does your physicianmeasure your serum tryptase levels at the time of maximum symptoms (acute mast cell degranulation attack symptoms or anaphylaxis) to see if your level has risen? Yes, often□ Yes, sometimes□ No□ Not Sure□
19. What is your most recent baseline serum tryptase (if you do not know, please leave blank)? ______ng/ml
20. Have you tested positive for the c-kit D816V mutation?
Yes□ No□ Not Sure□ Test not performed□
21. Have you tested positive for any othergenetic mutations (e.g. JAK2)?
Yes□ No□ Not Sure□ Test not performed□
If yes, please specify ______
22. Do your mast cells express CD2 and/or CD25?
Yes□ No□ Not Sure□ Test not performed□
ALLERGIES
23. Have you been tested for allergies? Yes □ No □ Not sure □
24. What type of allergy testing was done?
SkintestYes □ No □ Not sure □
RASTor other blood IgE level testYes □ No □ Not sure □
Other Yes □ No □ Not sure □
25. What type(s) of allergy(ies) do you have?
Inhalant/Environmental Allergies (for example,dust mite, pollen, mold, animal dander):
Positive test: Yes □ No □ Not sure □
Causes problems: Yes □ No, but still avoiding it □ No □
If you have an inhalant/environmental allergy, please list to which substances you are allergic:______
______
______
Insect sting:
Positive test: Yes □ No □ Not sure □
Causes problems:Yes □ No, but still avoiding it □ No □
Latex:
Positive test: Yes □ No □ Not sure □
Causes problems:Yes □ No, but still avoiding it □ No □
Food:
Positive test: Yes □ No □ Not sure □
Causes problems: Yes □ No, but still avoiding it □ No □
If you have a food allergy, please list to which foods you are allergic: ______
Drug:
Positive test: Yes □ No □ Not sure □
Causes problems: Yes □ No, but still avoiding it □ No □
Please use the list below to select the drug types to which you are allergic:
□ NSAIDs (non-steroidal anti-inflammatory drugs)
□ Opioids (e.g., morphine, codeine)
□ General anesthetics
□ Local anesthetics
□ Antibiotics
□ Radiocontrast agents (used to enhance x-ray-based imaging techniques)
□ Other, please specify______
Other Allergies, please specify: ______
______
______
Positive test: Yes □ No □ Not sure □
Causes problems: Yes □ No, but still avoiding it □ No □
26. Do you take or have you ever taken allergy shots? Yes □ No □
Please specify for which allergens? ______
27. If yes, are these allergy shots helpful to you? Yes □ No □ Not sure □
28. In general, if you have taken allergy shots, were you able to tolerate them?
Yes □ No □ Not sure □
29. Do you take any other therapies for your allergies? Yes □ No □ Not sure □
30. What other therapies for allergies do you take? (Please list and indicate if helpful)
Medicine/TherapyHelpful?
______Yes□ No□ Not Sure□
______Yes□ No□ Not Sure□
______Yes□ No□ Not Sure□
______Yes□ No□ Not Sure□
OTHER POTENTIAL COMPLICATIONS
31. Have you been diagnosed with osteopenia or osteoporosis confirmed by a bone scan or bone density scan (dexascan)? Yes□ No□ Not Sure□
32. Do you take medication to treat your osteopenia/osteoporosis?
Yes□ No□ Not Sure□
33. Do you take calcium supplements?Yes□ No□ Not Sure□
34. Do you take vitamin D supplements?Yes□ No□ Not Sure□
35. Have your vitamin D levels been measured?Yes□ No□ Not Sure□
36. Have you ever been diagnosed with any form of cancer?Yes□ No□ Not Sure□
37. if you have ever been diagnosed with any form of cancer, please specify which types(s):
□Skin cancer, basal cell
□Skin cancer, other
□Hematologic/lymphatic (Hodgkins or other lymphoma, leukemia, myeloma or other), pleasespecify______
□Lung
□Breast
□ Prostate
□Colon
□Other, pleasespecify______
38. Have you been diagnosed with coronary artery disease?Yes□ No□ Not Sure□
39. Have you ever had a heart attack?Yes□ No□ Not Sure□
40. Do you have high blood pressure?Yes□ No□ Not Sure□
41. Do you have hypercholesterolemia (high blood cholesterol)?
Yes□ No□ Not Sure□
42. Please list any other conditions with which you have been diagnosed.
______
43. Please select any triggers of your mast cell disorder symptoms from the list below (check all that apply):
□ Heat
□ Stress
□ Exercise
□ Alcohol
□ Insect Stings
□ Medications
□ Odors
□ Other, please specify______
______
DIET/NUTRITION
44. Has a physician recommended a “low histamine” diet to you?
Yes□ No□ Not Sure□
45. Did the physician refer you to a dietitian/nutritionist?
Yes□ No□ Not Sure□
46. Has a dietitian/nutritionist recommended a “low histamine” diet to you?
Yes□ No□ Not Sure□
47. Have you ever followed a “low histamine” diet?
Yes□ No□ Not Sure□
48. If you have followed a “low histamine” diet, does this diet seem to improve your mast cell disorder-related symptoms?
Yes□ No□ Not Sure□
49. Have you tried an “elimination” diet?
Yes□ No□ Not Sure□
50. Do you feel that you are obtaining adequate nutrition?
Yes□ No□ Not Sure□
FAMILY TENDENCIES
51. Does anyone else in your family have a mast cell disorder?If more than one other person in your family has a mast cell disorder, please select all that apply.
Yes, confirmed □ Yes, suspected, but not medically confirmed□ No□ Not Sure□
52. If anyone else in your family has a mast cell disorder, please specify below. Also, please specify the general form of disease that your family member(s) has/have and the number of siblings, children or other relatives affected, if more than one. Please select from the following types of mast cell disorders (circle the number): 1 =cutaneous mastocytosis;2 =systemic mastocytosis (all forms); 3 = mast cell activation syndrome/disorder (MCAS/MCAD).