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* 1. What is your specialty?

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* 2. What is your sex?

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* 3. How long has it been since you completed fellowship in hematology / oncology?

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* 4. Are you Latino or Hispanic?

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* 5. What is your race?

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* 6. In what country do you practice hematology?

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* 7. How many SCD patients are followed in your program?

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* 8. Are you the primary hematologist for SCD patients?

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* 9. In a typical week how many patients with SCD do you see?

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* 10. Do you routinely evaluate your patients' understanding of their sickle cell disease including genotype, complications, treatment and prognosis?

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* 11. Does discussing prognosis for patients with sickle cell disease include discussing...

  Yes No
Current patient health status including sickle cell disease activity
The complications patients have had from sickle cell disease (stroke, VOC, transfusions, acute chest)
Bone marrow transplant or other treatments that may modify sickle cell disease course
Expectations for the future health of a patient with sickle cell disease
How sickle cell disease affects activities of daily living, including work absenteeism
Life expectancy

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* 12. In your clinical practice do you have a protocol or clinical pathway for...

  Yes No I don't know
Initiating hydroxyurea therapy for your patients?
Offering bone marrow transplant to your patients?
Discussing prognosis with your patients?

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* 13. In the routine care of your patients with sickle cell disease, how often do you discuss...

  Very Often (1) (2) Sometimes (3) (4) Infrequently / Never (5)
Current patient health status including sickle cell disease activity or other medical problems
The complications patients have had from sickle cell disease (acute chest, stroke, pain, transfusions)
The complications patients may, but have not yet had, from sickle cell disease
Expectations for the future health of a patient with sickle cell disease
How sickle cell disease affects activities of daily living, including work absenteeism
How sickle cell disease affects peer relationships
Life expectancy

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* 14. What prompts you to discuss prognosis? Please select all that apply.

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* 15. In the routine care of your patients with sickle cell disease, how often do you discuss...

  At each non-acute visit Annually Every 2 - 3 years Every 5 or more years I do not routinely discuss this
Disease progression with your sickle cell disease patients?
Life expectancy with your sickle cell disease patients?

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* 16. Do you have a genetics counselor regularly available at your clinic?

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* 17. What is a reasonable interval to re-visit patient prognosis?

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* 18. Is it important to develop clinical pathways or protocols for communicating diagnosis to your patients with sickle cell disease and their families?

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* 19. Why are such pathways or protocols important or unimportant to you?

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* 20. What, if any, barriers are there to discussing prognosis? Please select all that apply.

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* 21. Do you think improving patients' understanding of sickle cell disease through routine re-visiting of prognosis would change your patients'...

  Strongly agree (1) (2) Neutral (3) (4) Strongly disagree (5)
Willingness to start hydroxyurea
Willingness to start chronic transfusions
Willingness to have a bone marrow transplant
Medication adherence
Pregnancy planning practices
Willingness to participate in research
Advocacy for resources for sickle cell disease clinical care and research

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* 22. What is the median life expectancy for African Americans in the United States (please make your best estimate)?

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* 23. What is the median life expectancy for people with Hgb SS disease? Please give your best estimate.

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* 24. Do you discuss statistics about life expectancy or risk of death to motivate your patients to adhere with treatment plans?

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* 25. In general, do your patients have the same understanding of life expectancy for people with sickle cell disease as you do?

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* 26. In general, how fearful are

  Very (1) (2) Somewhat (3) (4) Not Very (5)
Your patients for their future because of sickle cell disease

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* 27. How prepared are your patients at transition to adult care with respect to:

  Very Prepared (1) 2 Somewhat prepared (3) 4 Not at all prepared (5)
Making appointments
Filling prescriptions
Knowing potential complications of sickle cell disease not yet experienced
Knowing their past medical history
Understanding how sickle cell disease can affect the ability to work
Navigating the emergency department

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* 28. What percent of your patients come to the adult hematology clinic with a medical history from the pediatric hematologist?

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* 29. What percent of your patients do you believe have an effective transition?

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* 30. How often do your patients with sickle cell disease ask you about life expectancy?

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* 31. Given the limitations of this instrument, please provide any additional comments about your views on communicating prognosis to patients with sickle cell disease and their families that you think are important.

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