The Role of Patient-Physician Communication on the Use of Hydroxyurea in Adult Patients with Sickle Cell Disease

Abstract

Objective:

This qualitative study analyzed the perspective of patients living with sickle cell disease (SCD) on their process of deciding whether to take hydroxyurea (HU), and the role of physician communication in patients’ decision-making process.

Methods:

From October 2015- July 2016, we conducted semi-structured interviews among patients with SCD (N=20) that were audio-recorded and transcribed. Participants were ≥ 18 years old, a patient of an urban adult sickle cell center, able to provide informed consent, and English-speaking. We iteratively developed codes and used thematic analysis to organize the key themes.

Results:

Most participants were female (65%), middle aged (M=44, SD=12.2), and 55% were prescribed HU for an average of 10.4 (SD=4.7) years. Participants described 3 key factors that influenced their decision regarding HU treatment: 1) Lifestyle, 2) Health status, and 3) HU characteristics. Four themes emerged about provider communication and HU treatment decisions: 1) Provider’s advisement, 2) Shared-decision making, 3) “Wrestled,” and 4) Not feeling heard.

Conclusion:

Providers who engaged in shared-decision making empowered participants to decide whether to start HU treatment. Participants who felt their providers were not listening to their concerns expressed disengaging from HU treatment. During discussions about HU with patients living with SCD, providers must understand the multi-faceted aspects that impact patients’ decision and empower patients to engage in such discussions. Further research is needed to understand the role of shared-decision making among patients with SCD to improve management of SCD.

1. Introduction

Sickle Cell Disease (SCD) is an inherited blood disorder due to a mutation of the beta globin gene, which leads to polymer formation and sickling of red blood cells [1]. There are an estimated 100,000 people living with SCD in the United States (U.S), affecting a predominantly African American patient population [2, 3]. The sickling of red blood cells obstructs blood vessels, leading to a myriad of complications including chronic and episodic pain (vaso-occulusive crises), hemolytic anemia, stroke, acute chest syndrome, deep vein thrombosis and pulmonary embolisms [4].

Hydroxyurea (HU) is an oral daily medication that is FDA-approved to manage SCD and is recommended as an effective treatment for patients living with SCD [5]. The landmark randomized control trial, the Multicenter Study of Hydroxyurea in Patients with Sickle Cell Anemia, found that HU was associated with a reduction in the frequency of pain crises, hospitalizations, episodes of acute chest syndrome and blood transfusions compared to patients not taking HU [6]. HU is also associated with improved health-related quality of life among the SCD patient population and has been associated with decreased fatigue as well as improved general health perceptions and social and physical functioning compared to patients living with SCD who did not take HU [7–9]. Given the existing evidence on HU for adults with SCD, HU is recommended in the recent NHLBI guidelines for patients with SCD who have ≥ 3 pain episodes in a year, recurrent or severe acute chest syndrome, severe symptomatic anemia or pain that affects quality of life [10, 11].

Despite the known benefits, HU is underused among people with SCD [5, 10–16]. Studies have shown that between 58 – 70% of patients who were eligible for HU were not taking HU [13, 18]. Not only are HU prescription rates low, but among patients prescribed HU, adherence remains suboptimal with poor adherence ranging from 64–74%, and poor adherence is associated with lower quality of life indicators [19–21]. Barriers to clinically optimal HU utilization are complex, involving the patient, parent/social support, provider and the healthcare delivery system [12].

Providers report multiple barriers to HU utilizations including insufficient knowledge about HU as a treatment option and concerns about the side effects of HU (e.g. concerns about cancer, reproductive health, and unknown long-term risks) [5, 14]. Providers have also cited unclear treatment guidance and feeling uncomfortable managing HU in the SCD patient population. Furthermore, providers report uncertainty about how to address perceptions of patient hesitancy towards HU [17].

There are also many patient-level factors that lead to HU underutilization. The existing literature suggests that patients’ beliefs and concerns about HU medication characteristics and about the necessity for HU are factors that contribute to their adherence and subsequent health outcomes [22, 23]. One study examined the attitudes towards HU among SCD patients who currently, never, and formerly were prescribed HU. Participants who stopped taking HU commonly cited stopping with their physician’s recommendation or not liking the way HU made them feel [23]. This study also found that patient perceptions about the benefits of HU, lack of knowledge about HU, and the impact of side effects were barriers to taking HU [23]. There is data to suggest that the interaction between the patient and physician is a significant factor in the decision to start, continue or discontinue HU. However, this communication process has not been well described.

To improve HU utilization, more research is needed to understand patients’ perspective on the process of deciding whether to take HU and how patient-provider communication can impact that process. Our study aims to fill an existing gap in the literature by analyzing patient perspectives on their decision to initiate HU and the role of patient-provider communication in the decision-making process.

2. Methods

2.1. Study Population

Patients were invited to participate via an email from providers at the Johns Hopkins Sickle Cell Center for Adults that introduced the study. Contact information was included in this email, and patients were instructed to opt-out of being contacted by the study team if they did not wish to participate. Participants were eligible if they were ≥ 18 years old, a patient of the Johns Hopkins Sickle Cell Center for Adults, able to provide informed consent, and English-speaking. We used purposive sampling to recruit patients across the spectrum of HU utilization including: 1) currently on HU, 2) never started HU despite being recommended, and 3) taken HU in the past but no longer. All participants provided oral informed consent, either over the telephone or in person. Participants did not receive any compensation for their participation in this study. The Johns Hopkins Institutional Review Board approved this study’s protocol.

2.2. Study Development and Design

We conducted semi-structured qualitative interviews with patients living with SCD to understand the factors that influenced their decision to initiate HU treatment and the role of provider communication in their decision making process. Interviews were conducted by a masters level counselor who had no previous interaction with the patients (SB). The interview guide was developed with expert guidance from study authors, including an adult hematologist and clinical psychologists. Questions were modified throughout the interview process using an iterative process. Interviews were conducted until thematic saturation was achieved. Open-ended questions included topics such as participant’s opinion on HU discussions with their physicians, factors influencing their decision to decide not to take, start, or stop HU therapy, and how participants felt their decision impacted their relationship with their physician (Appendix A).

2.3. Data Analysis

Analysis was conducted by co-investigators who did not conduct the interviews. Two investigators (SC, SMJ) independently identified themes using open, inductive coding to iteratively develop the codebook. Subsequently, one investigator (SMJ) applied the codes to the transcripts, and 10% of the transcripts were reviewed by another investigator (SC). Any issues throughout the coding process were discussed with the PI (MNE) to ensure proper application of the codes. Thus, instead of calculating inter-coder reliability, we used a discussion and consensus process to resolve any issues that arose during the coding process [24–26]. We did not have an a priori hypothesis; rather this qualitative study was focused on generating hypotheses [26, 27]. Thematic analysis was used to develop and organize key themes. We used QSR NVivo Version 11.0 to code the transcripts and assist in consolidation of quotations summarized in our analysis.

3. Results

From 2015–2016, qualitative interviews were conducted either in person or over the telephone by a trained research assistant (SB). We identified 53 potentially eligible participants, of which 39 were reached, 5 were no longer eligible (moved away from clinic or had not been introduced to HU), 10 were not interested in participating, 3 consented but did not complete the interview, and 21 completed an interview. All but one interview were audio-recorded and transcribed for a final sample of 20 participants. Demographic and clinical characteristics are described in Table 1. The sample was mostly in their mid-forties (M= 44.0, SD= 12.2) and female (65.0 %). About half (55.0 %) of participants were prescribed HU and were prescribed for an average of 10.4 (SD: 4.7) years.

Table 1:

Demographics and Clinical Characteristics of Study Population

	N= 20	%
Age, mean (SD)	44.0	12.2
African American/Black	20	100.0
Female	13	65.0
Ever Prescribed HU	11	55.0
Years Taking HU (If Prescribed), mean (SD)	10.4	4.7
Self-Reported HU Treatment Status at Time of Interview*
Current	3	15.0
Never	4	20.0
Current, Resumed	6	30.0
Stopped	7	35.0

^*Participants self-reported their HU treatment status during the qualitative interview.

Key themes were organized into two overarching constructs: (1) Factors Influencing Whether to Initiate HU Treatment and (2) Role of Provider Communication in HU Decision Making Process. Quotations from interviews are followed by the age, gender, and the self-reported HU status of the participant.

3.1. Factors Influencing Whether to Initiate HU Treatment

Lifestyle Considerations

Participants expressed how HU did not fit with their routines, and many participants specifically described difficulty taking the medication. For some participants, adherence was a barrier to taking HU, while others described feeling burdened by a large number of pills. Participants also felt that taking a daily medication did not make them feel healthy, which decreased their interest in HU treatment.

I know my reaction was I didn’t want to be that person who has to take medication every day (23, F, Stopped).

Other participants described accessing medical care as a barrier to initiating HU treatment, especially since HU requires consistent monitoring. Life events, such as moving, changing or losing health insurance, impacted participants’ access to care and subsequently their decision to take HU.

I was out of town and I didn’t want my blood count to go down low … I wasn’t able to get it checked on a monthly basis so I felt that I would be safer just not taking it (42, M, Current Resumed).

Health Status

Participants’ current or potential future health status impacted their interest in taking HU. Participants highlighted that while they were not presently ready, when thinking about their long-term health, they felt HU would be important to their well-being. Other participants described deciding to take HU because it offered the potential of improving their health.

I didn’t want to get sick if there was any chance that I could prevent being sick then I would want to do that to the fullest capability (42, M, Current Resumed).

Among participants who started taking HU, many described initiating HU treatment when they were experiencing frequent crises or hospitalizations and felt they were out of options in terms of medical treatments. Feeling so sick, participants explained that they felt they were willing to try anything that would potentially help.

I was having crises every three months, to the point where we’d have to go to the emergency room…I was open to anything that would change that (48, M, Current Resumed).

HU Characteristics

Participants had conflicting viewpoints about HU and its side effects that impacted their decision to take HU. In particular, participants were divided as to whether HU would help alleviate their SCD symptoms. Some participants described improved symptoms whereas others described not feeling that HU improved the way they felt, which made them uninterested in taking the medication.

My main takeaway was it helps with the frequency of crises, the duration of crises, the severity of crises, and after the first couple of months, when all of that proved true, I really didn’t question it too much anymore (48, M, Current Resumed).

This experience contrasts with another participant who stopped taking HU because they did not find it helped their SCD symptoms.

I was still getting sick…. It seemed to me like there was no change…. that was frustrating to me (48, M, Stopped).

Participants also had differing opinions about how HU compared to other treatments, like chronic transfusions and pain medications. A minority of participants felt transfusions worked, and they could feel the effects immediately as compared to HU. Conversely, another sentiment expressed was that transfusions had long-term negative effects, and they did not like how long it took for chronic transfusions.

The thing that stood out to me the most was that they said this will help and it wasn’t a painkiller. It wasn’t a temporary solution for when I had a problem (48, M, Current Resumed).

Participants reported a range of concerns regarding side effects. In particular, trying to have children dissuaded participants from taking HU. Some participants also expressed hesitancy to take HU due to the drug’s potential toxicity.

I wasn’t interested in taking more drugs and a drug with possible toxic effects (57, M, Never).

Participants who stopped taking HU often explained how their physician decided to take them off HU due to falling neutrophil counts. Participants who had previously taken HU described how they did not like the way HU made them feel and how it did not help to eliminate their crises. Participants who stopped taking HU were often concerned that if they started HU again the same side effects would recur, and it would not reduce their pain crises. Another concern described was the potential impact of HU on their immune system and how this would impact their day-to-day life. One participant who stopped HU treatment described their experience that led them to stop taking HU:

I was just concerned about the whole cancer thing. I think he said the white blood cells kind of fights off viruses and stuff, and if mines were so low, then I would be more susceptible to more illnesses (39, F, Stopped).

Misinformation about HU was another reason a minority of participants did not want to take HU. One participant believed they could not consume alcohol or smoke while taking HU and described wanting to experience young adulthood as their friends did:

You cannot smoke or drink…. that’s my tug and pull … I just turned 21 and I want to experience all of that…. And I am an occasional smoke (23, F, Stopped).

3.2. Role of Provider Communication in HU Decision Making Process

Provider’s Advisement

Participants conveyed how their provider explained that HU could potentially improve their health situation and management of SCD. They described placing their trust, and often their family members’ trust, in the hands of their physician during the HU decision-making process.

I just did what [doctor] said. He said it was good for me so I just … followed his lead (39, F, Stopped).

Shared Decision Making

Participants conveyed having a discussion with their provider about HU that led participants to feel that the HU decision was theirs to make. They highlighted how their provider laid out the treatment options, compared the benefits and side effects of each option and left it up to the patient to decide whether to take HU. Some participants described how this empowered them to decide whether to take HU. Even when participants ultimately decided not to take HU, they expressed satisfaction and trust towards providers who utilized a shared decision making communication approach.

It’s not a matter of … telling me this way or the highway. She does listen and. accept my concerns. She tries to work with me as far as my concerns. Not only … things like hydroxyurea, but like pain medication and that type of thing. So it’s been a good back and forth relationship (57, M, Never).

Many participants shared how their provider answered all their questions. By taking the time to explain, these participants described having their concerns surrounding HU treatment alleviated, which helped them to decide to start HU treatment.

From a doctor’s point of view that is trying to help patients with chronic pain… hydroxyurea honestly is our only hope as for medication. There’s nothing else out there… if you’re not getting the bone marrow or anything else, it’s basically just hydroxyurea or chronic blood transfusions…. I am a very well informed patient and I do have a great relationship with both doctors…. I do have a great relationship that I can talk to them about the medication or, you know, anything that is not even sickle cell related (44, F, Current Resumed).

“Wrestled”

A couple of participants explained that their provider was very opinionated about them taking HU. One participant described how even though he knew how strongly his provider felt about him taking HU; he still had not decided to take HU. Some felt that the provider was insistent but respected their decisions even if their provider did not agree.

I’m pretty much like, “Why is she still talking about this? I tell her no every time, will she give up?” But she doesn’t. She asks me every time…I’m respectful and I listen to her and then I tell her my opinion about it (34, F, Never).

Another participant highlighted his provider’s insistence on the benefits of HU for his medical situation:

We wrestled. There was a lot of times I would get sick and she would see me in the hospital because I got admitted for sickle cell anemia and she would come through and she would talk to me and she would be like, I really suggest, and I’m like no. I don’t think so… She talked to me a lot about it, but I had plenty of time to go to her with any concerns and stuff like that. She was very open (37, F, Stopped).

Not Feeling Heard

Among some participants who stopped taking HU, they described not feeling listened to or heard by their physicians such as feeling dismissed about concerns about side effects

I felt like I told him and he wasn’t hearing me, so I would just deal with it on my own (56, F, Stopped).

4. Discussion

Participants living with SCD expressed factors that shaped their HU treatment decision and the impact of conversations with their providers on their decision-making process. While previous analyses have investigated patients’ attitudes towards HU and barriers to HU adherence, few studies have qualitatively assessed patients’ perspective on their HU treatment decision-making process, especially the role of patient-provider communication. Key themes that emerged regarding factors influencing participants’ decision to start HU were: 1) Lifestyle, 2) Health Status, and 3) HU Characteristics. Participants expressed how their routines and life factors created barriers to starting and adhering to HU. Participants’ perceived future, current, or a sudden decline in health status as a reason to consider HU as a treatment option, and many participants described how their social network also led them to consider taking HU. Overall, there were mixed views on HU side effects, HU efficacy, and HU compared to other treatment options.

In terms of the role of provider communication, four key themes emerged: 1) Provider’s advisement, 2) Shared-decision making, 3) “Wrestled”, and 4) Not feeling heard. Some participants described trusting their provider and trying HU at their provider’s suggestion. Others shared that their provider described the benefits and side effects of HU, answered the patients’ questions, and left the decision up to the patient. Many participants who described provider communication styles based in shared-decision making expressed feeling empowered to decide whether to take HU. Even if participants ultimately decided not to take HU, they expressed trusting and feeling satisfied with their patient-physician relationship when their provider utilized shared decision-making. On the other hand, some participants described providers who strongly advocated for HU and were persistent in advising HU treatment. Some participants described how they stopped taking HU, because they felt the concerns they raised were not acknowledged by their provider.

In our study, a commonly described reason for deciding not to take HU or stopping HU treatment was that participants did not perceive the benefits of HU because they were still getting sick or experiencing pain crises, which is consistent with previous studies [23, 28]. This demonstrates the continued need for more research on effective counseling and communication about short-term HU toxicities that must be overcome to reach the therapeutic benefits [23], as this remains a persistent barrier to consistent HU treatment. Additionally, adherence and concerns about pill burden and lab tests were reasons participants were hesitant to take HU or decided to stop taking HU. Higher levels of adherence have been associated with increased perceived benefits of HU [19]. One previous analysis found that forgetting to take HU, issues with accessing HU (e.g. costs and remembering to refill their prescriptions) were some common reasons adolescent/young adult participants did not adhere to HU [20]. Combined with our findings, this suggests a need for innovative models of care that increase patients’ ability to engage in the necessary lab monitoring and HU refills as well as interventions to improve adherence counseling.

The literature on shared decision making communication practices among adults with SCD in the healthcare setting is limited. Our study provides preliminary support for the use of this method for SCD. Participants reported that providers who employed shared-decision making (e.g., laying out the benefits and costs of each treatment options and addressing patients’ questions and concerns) empowered participants to decide to take or not take HU as well as increased trust in their providers. However, a subset of participants who reported that even when their provider utilized shared decision making, they decided not to take HU treatment. For this subset of patients, providers should further assess and address patient concerns about HU as well as provide more concrete recommendations about HU if appropriate.

Our finding aligns with Sinha and colleagues’ analysis indicating that participants who took HU were more likely to have trust that their physician shared all the risks and benefits about HU with them [28]. However, Sinha and colleagues also found that participants not taking HU were skeptical about HU’s effectiveness and were unsure that all benefits and risks were known and discussed with them [28]. This contrasts with our findings, because even when participants described their providers as strongly advocating for HU during conversations that included shared-decision making, this did not necessarily lead participants to decide to take HU. Our findings highlight how building trust and open communication with patients living with SCD may lead participants to reconsider or be more open to discussing HU as a treatment option in the future.

More research is needed on the use of shared decision-making in SCD clinical practices to understand its role in HU treatment uptake, adherence, patient satisfaction, and long-term health outcomes. The majority of research on shared decision making and HU treatment decisions has focused on the pediatric population and their caregivers, use of clinical decision aids, and more recent analyses investigating gene-modifying treatments and shared decision making in HU care [28–31]. Future research should focus on understanding the impact of communication rooted in shared decision making on the SCD patient-provider relationship as well as treatment outcomes.

Trust in the patient-provider relationship is an important factor guiding patients’ decision to take HU. Discrimination and lack of respect towards patients with SCD interacting with the healthcare system has been extensively documented [32–35]. Patients with SCD often distrust healthcare professionals, which is associated with lower adherence to treatment plans [36–39]. Thus, our analysis points towards the need for increased efforts to improve communication skills that build trust among patients with SCD and their providers to improve HU uptake and adherence.

Our findings build on previous literature indicating the importance of patient knowledge and perceptions about HU as a treatment option in patients’ decision-making process [22, 23]. Future research should investigate how different modes of communication impact patients’ HU decision process. For example, further research is needed to understand how technology-based interventions may influence HU treatment uptake and health outcomes among adults with SCD. Recent evidence found that communicating with providers via a mHealth or technology-based platforms would be both accessible and of interest to patients with SCD for health information and self-management support, including for medication adherence [40–45]. One study found patients did not have a lot of information about SCD care guidelines but were interested in learning about these guidelines and found that technology programs could help them communicate and receive information with their providers about SCD healthcare decisions [45]. In our study, many participants expressed how their perceptions about the long-term benefits and side effects of HU influenced their HU treatment decision, and a minority expressed incorrect information about HU that factored into their decision. These results suggest a need for increased provider emphasis on education about HU treatment and investigation of innovative ways to support patient-provider communication outside traditional medical appointments.

The findings should be interpreted in the context of the study’s limitations. While the sample size is small, interviews were conducted until thematic saturation was achieved, which is consistent with qualitative methodology [26]. This study interviewed participants from an urban academic medical center with a comprehensive adult SCD center, and findings may not be representative of people living with SCD in rural areas or community healthcare settings. However, the detailed perspectives shared from participants living with SCD with a diverse experience with HU treatment advance our understanding on the SCD treatment decision and communication processes among adults with SCD.

4.1. Conclusions

These results highlight the critical role that providers have in a patient’s decision to initiate or continue HU use. Patients who reported that their providers gave detailed information on the risks and benefits and supported the patient’s autonomy to make the decisions to initiate HU was helpful to many patients. However, there were still some patients who were reluctant to initiate HU despite their provider engaging in these communication practices, often due to personal factors such as disease status, living situation or perceived lack of need for medication. Therefore patient-level individual counseling interventions may be needed to help support patients. These findings point to the need for more research on the impact of shared-decision making on health outcomes among patients with SCD.

Table 2:

Lifestyle Considerations

Sub-Theme	Barrier
Adherence	I really don’t remember to take medicine every day…that’s another reason why I never committed to taking that medicine. (34, F, Never). I’m just bad with taking medicine. I try not to get put on medicine because I’m really bad with remembering (34, F, Stopped).
Daily Medication	I don’t want to take a pill every day of my life, I don’t. That doesn’t, that doesn’t sound healthy to me, that doesn’t sound like I’m getting healthy (P34, F, Never).
Patient without access to medical care	From September to March. That’s the longest I’ve been off of it [HU] since I was in college…Didn’t have health insurance. (35, M, Current Resumed).
Too many pills	I was taking…four pills a day. . that just got really difficult for me after a while. I was just like I can’t do that anymore. …. I tried to tell him that the four pills were too much and if I could lower the dose or anything and he wouldn’t let me. ….I just stopped taking it. (23, F, Stopped).

Table 3:

Health Status

Sub-Theme	Facilitator
Potential Benefits	To not be sick, I mean to avoid, less crises (34, F, Never).
Change in health status	After the miscarriage of my son…We really didn’t consider it until after we lost our sons (37, F, Stopped). We talked about it and she [hematologist] said well, you’re doing okay. And then after things started to go south he said we’re not going to talk about it anymore, we’re just going to do. (72, F, Current).
Sick enough/tipping point/willing to try anything that may help	I think we talked about it because I had just a slew of crises that year. I don’t know how many times I was hospitalized… (35, M, Current Resumed). When it came time for me to start hydroxyurea, I was at ground zero. I was so sick (43, F, Current). It was just something to help me not go to the hospital every day. Not something that’s going to completely stop it. So that’s probably… my little hope and joy is probably why I agreed to take it because I probably thought that’s what was going to happen. (23, F, Stopped).

Table 4:

Hydroxyurea Characteristics: Facilitators and Barriers

Sub-Theme	Facilitator	Barrier
Prefer to Alternatives	And I would much rather take a pill every day…than to have to sit in a room for eight hours once a month. (35, M, Current Resumed). I didn’t mind getting blood transfusions…but … prolonged blood transfusions have hazardous effects also (29, M, Current).	On the red cell exchange is when they pretty much said I don’t have to take it (34, F, Stopped) I actually preferred the transfusions over hydroxyurea (34, F, Stopped).
Ability to reduce severity of crises/Efficacy Issues and side effect concerns	If it’s going to help me stop having pain crises every other day, yeah let’s do it (34, F, Stopped). I was very much in favor of taking anything that would help me stave off the possibility of a crisis. (61, F, Current Resumed).	I didn’t believe that it was going to lessen my crises. (34, F, Never). I didn’t feel any different … so I was just like, why am I taking this medicine? (23, F, Stopped). Other than my hair. I don’t want to lose my hair. (39, F, Current Resumed). It can cause blindness, and that’s kind of what deterred me at first from it. (37, F, Stopped). I was concerned…whether it would increase the frequency of my crises and the severity (64, M, Stopped).
Ability to prevent future damage vs. cause future damage	I was told that any damage that was already done of course, it can’t repair that, but it can try to prevent any further damage. (42, M, Current Resumed).	All, the longtime usage of it, what can it do to your body (46, F, Never).
Pregnancy/After pregnancy		Having kids in the future and whether there would be any negative effects as far as having children (57, M, Never). I had already knew that hydroxyurea can cause birth defects before I got pregnant, so as soon as I felt like I was pregnant it was just like oh, no. That’s got to go. (39, F, Current Resumed).
Effect on neutrophil count		[Hematologist] took me off of it because my absolute neutrophil count was out of whack…It was too low because it was looking like I had a condition called neutropenia (48, M, Current Resumed).

Table 5:

Role of Provider Communication in HU Decision-Making Process

Sub-Theme	Decision about HU
Provider Advisement	He knew it would help, but at the end of the day it was,… my mother’s and my decision to make. But he was just very convincing….Not really pressured, but very convincing (23, F, Stopped). They strongly suggest that I start taking it, only because they said I would be a good candidate as far as the lesser crises (34, F, Never). He was definitive. It was like 2+2=4. It was that clear. You have respiratory issues and sickle cell anemia and you should be on hydroxyurea (43, F, Current).
Shared Decision Making	It’s not a matter of… telling me this way or the highway. She does listen and she does… accept my concerns. She tries to work with me as far as my concerns…it’s been a good back and forth relationship (57, M, Never). She always presents the information. She tells me it’s my decision. You can do this, this, or this, and sometimes that third option is nothing. And then she tells you what the risks are of doing nothing. She tells you what the risks are of all the options and then she basically lets you make the decision (56, F, Stopped). When it come to decision making…they pretty much let me make my decision because even though they know all of the medical side I know all about me (34, F, Stopped). I mean, my decision is my decision. I’ve always been a part of it and I’ve always said no. I’m of course a part of my decision making. (34, F, Never).
“Wrestled”	She has been adamantly chasing me with that for a while (37, F, Stopped). We have a very good relationship…I totally appreciate her experience and expertise and I think she appreciates…how well I keep myself informed and try to keep myself healthy so there’s a respectful relationship there; however, I do notice she has been a little bit forceful as far as convincing me as far as HU is something I may need to start considering… because of the frequency of episodes I’ve been having recently… It wasn’t a combative discussion, but it was certainly a discussion… where each of us had our own opinions and… I know at some point one of us has to relent and I have a feeling it’s going to be me (57, M, Never).
Not Feeling Heard	I didn’t feel right when I took it and I told him that and…made my mouth hurt… I told him that and he didn’t really believe me… He said that’s not a reported side effect and I said okay, but it does. So, I decided to stop taking it (56, F, Stopped).

Appendix A: Open-Ended Interview Guide: Adult Participant with SCD

Current Use

• What is your experience with hydroxyurea?

If not discussed- Have you ever taken it?

Are you currently taking it?

• What do you know about hydroxyurea?

• How did you first learn about hydroxyurea?

• What things did you think about when deciding whether or not to take hydroxyurea?

• What benefits do you know of or have you seen from taking hydroxyurea?

• What concerns did you/do you have about taking hydroxyurea?

Communication with Physician

• Tell me about the first time you talked about hydroxyurea with your physician. If they don’t remember the first time, ask about the ongoing discussions or previous discussion they have had with their physician.

• What was your reaction during the discussions with your physician about hydroxyurea?

If not discussed- Was there sufficient time to talk about your concerns or questions?

• Did you feel any pressure to take the medication? If yes- Tell me about this.

• How did you feel during the discussion about hydroxyurea?

• What kinds of questions did you have when you and your physician discussed hydroxyurea?

  If not discussed- What are the pros or benefits to taking hydroxyurea?

  What are the cons or risks/harm to taking hydroxyurea?

• Were all of your questions answered? If not- How did you handle that?

• When hydroxyurea was discussed between you and your physician was anyone else with you? If yes than find out what relation to participant.

• How would you change that?

• How would that have impacted the conversation and decision?

• Tell me how the decision making process went for you.

If not discussed- Did you feel like you were a part of the decision-making processes?

• What did you like most about the conversation and what the physician said?

• What if anything do you wish had been done differently?

• Who besides your physician did you talk to about taking hydroxyurea?

  If they talked to other people-How did they affect your decision to take or not to take hydroxyurea?

Choose one set of questions below based on current hydroxyurea status.

If the patient is currently taking hydroxyurea

• What things did you think about when deciding to take hydroxyurea? For example family, friends, medication concerns or lab visits or other things.

• How has your decision to take hydroxyurea impacted your relationship with your physician?

• What are your thoughts about ongoing communication about taking hydroxyurea?

  If not discussed- How comfortable do you feel bringing up hydroxyurea or concerns you have about taking hydroxyurea?

  How open to discussion do you feel your physician is about future options regarding hydroxyurea?

If the participant took hydroxyurea in the past but stopped

• What things did you think about when you decided to start taking hydroxyurea previously?

• What things did you think about when you decided to stop taking hydroxyurea?

• What role did the physician play in that decision?

• How has your decision to stop taking hydroxyurea impacted your relationship with your physician?

• What are your thoughts about ongoing communication about taking hydroxyurea?

  If not discussed- How do you feel about bringing up the topic or concerns you have about taking hydroxyurea in the future?

How does your physician make you feel about your decision?

If the participant has never taken hydroxyurea

• What influenced your decision to not start taking hydroxyurea?

• What would have to change for you to be interested in taking Hydroxyurea?

• How has your decision to not take hydroxyurea impacted your relationship with your physician?

• What are your thoughts about ongoing communication about taking hydroxyurea?

  If not discussed- How do you feel about bringing up the topic or concerns you have about taking hydroxyurea in the future?

How does your physician make you feel about your decision?