Participant recruitment

The study was approved by the National Human Genome Research Institute Institutional Review Board NHGRI (Protocol Number 14-HG-0125). Participants were recruited from August 2015 to May 2016 and drawn from the larger, ongoing INSIGHTS Study (Insights into Microbiome and Environmental Contributions to Sickle Cell Disease and Leg Ulcers Study) [16]. The participants in the study were adults (>18 years of age) with a history of leg ulcers or active leg ulcer(s) at the time of the study. Written or verbal consent was obtained at the beginning of each interview. Individuals who participated in the interviews received $50.00 compensation.

Data collection

We conducted twenty semi-structured, one-on-one interviews with participants in the INSIGHTS Study (NCT02156102). A standardized qualitative interview guide was developed. The guide was collectively agreed upon by the research team. Questions formulated for the interview guide were taken from instruments and measures used in the INSIGHTS study but were constructed to be open-ended. We asked questions that qualitatively measured the individuals’ quality of life (S1 Table) and aligned with Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me) measures and the Patient-Reported Outcomes Measurement Information System (PROMIS) measures [17–19].

We also investigated history of ulcers, manifestation of pain, social support, and participants’ guidance for health care providers and researchers. The interview guide was piloted and modified to allow for a more comprehensive exploration of each theme. All study participants had completed the INSIGHTS main study including demographic information, standardized ASCQ-Me and PROMIS measures prior to the semi-structured interviews.

Interviews were conducted by a researcher (NU or AB) in-person or over the phone. Interviews conducted in-person were done at the National Institutes of Health (NIH). All interviews were recorded and averaged 30 minutes (ranging from 18 min. to 73 min.). Forty percent (8 participants) of interviews were conducted in-person and there was a non-significant difference in time with in-person versus phone interviews (p-value 0.97), with the former being shorter in duration: 00:35:46 (SD 00:15:09) vs. 00:35:50 (SD 00:15:30).

Analysis

The audiotapes were de-identified and assigned numeric codes before they were sent for transcription by an external transcription service. Recordings from each interview were transcribed verbatim and subsequently analyzed. Two researchers were involved in the data analysis. Coding was first done by a member of the research team (NU) using NVivo 10 software (QSR International). An initial code list was developed a priori and data was coded using template analysis. Hierarchical coding was also employed to systematically organize the material. Reliability and consistency of the coding structures and transcripts were ensured by an independent assessment of all source data by a second member of the research team (BA).

There was 98% agreement between coders for parent nodes that were made by NU and reexamined by BA. The study did not calculate the kappa score for inter-rater reliability at the sub or child node level. Major themes identified among both reviewers were examined by the research team closely and chosen for analysis.

Pain

Physical function

Physical function was mediated by pain intensity; the presence or absence of pain dictated most participants’ extent of mobility. Physical movement was sometimes avoided and/or restricted due to the pain that often ensued and the potential for injury, bleeding, or re-opening of the ulcer. Participants noted that they felt a significant improvement in being able to walk, run, and play sports when they did not have an active ulcer. One participant explained the ulcers as “a proverbial ball and chain on your ankle because that's how it feels when you're walking and feel like you're dragging something with you, holding you down and in pain.” (PT male 50005)

Some participants revealed that because the pain associated with leg ulcers can be irregular in nature, the capacity to walk also becomes erratic. One participant remarked:

“Sometimes I wake up and can’t even put any pressure on my foot or can’t even walk on my foot. And when that happens, just happens, I don’t know when it’s going to happen, so I could wake up tomorrow and not be able to walk on my foot” (PT female 50021). In addition, participants expressed suffering other physical limitations due to their ulcers. Many perceived physical exercise as an extremely difficult task; this was especially true for those who had difficulty walking and for individuals who required the use of a walking aid (e.g. cane, a walker).

Social relationships

Upon examination of social interactions, we discovered that many participants felt as if they were hiding from the world due to their ulcer. In order to avoid being alienated, often respondents did not disclose their ulcer status to anyone, including extended family and friends. One participant mentioned:

“So you add that all up and you end up wearing a mask. I mean, no one, in a million years would ever suspect, that was going on with me at that moment. But it takes a lot of effort to make sure that you don't let people know that's going on.” (PT male 50005)

Almost half of the participants had significant others; many of them delineated the strains and tensions placed on existing relationships, as well as creating new social relations, by this chronic condition. One participant described the difficult conversations he had with partners as: “every mate or girlfriend [I] had to sit down… and explain to them what was going on or what it was…You can't spring it on anybody. So, it affects your relationships in that way” (PT male 50005). For a chronic condition, such as SCD-associated leg ulcers, the physical and emotional toll on intimate relationships was a common theme, particularly when a significant other acts as a caretaker. One participant felt that his increased reliance on his girlfriend led to tension in their relationship because he had to:

“sit back and play more of a, like, I don’t want to say submissive role, but—basically, like, my girlfriend, she’s had to do more domestic work than she did have to before. Because, you know, I could easily be able to pick that slack up. Like cut the food, you know, from sweeping up the house, and cleaning up. Especially with the ulcer getting bigger.” (PT male 50070)

The responses amongst participants regarding family social support ranged from a complete lack of support from families, particularly spouses, to high levels of caretaking involvement. In regards to the former, one participant noted:

“As far as family helping out, they think they understand, but they don’t understand. Honestly, all I can say is, unless you walk in my shoes, then you [don’t] know what I’m going through. But they don’t really help me out; I take care of myself.” (PT male 50002)

In contrast, even in cases where participants expressed strong familial and social support, a handful of participants opted out of accepting help. Self-management of ulcers seemed to be preferable due to the perception of greater competence:

“…[I] would just rather take care of it, rather than somebody else taking care of it. I don’t want no type of infection, and then it’s very sensitive, so I know how much pressure I can apply to it. I do with it myself. I don’t want my family members to take care of my ulcers.” (PT female 50021)

Social isolation

The experience of isolation manifested differently among female and male respondents. Female participants focused more on the inability to wear certain attire, such as skirts due to the location of the ulcer. “For me, too, being a woman is difficult. I don’t like to go to the beach or stuff like that because I always cover my ulcers. Normally, I always use socks, even in winter or summer.” (PT female 50011) While male participants focused on their physical activity.

“I’m a big fan of soccer; I call it football, where I come from…so not being able to play football [soccer] because I have a leg ulcer is terrible. Imagine sitting down in the house, and you can’t go out and play football [soccer] with your friends; it’s very depressing, very terrible.” (PT male 50002)

Irrespective of this, both sexes mentioned the impact of leg ulcers on many domains of their lives. Interestingly, especially in light of the previous finding, while levels of social isolation varied amongst the group, most participants tended to have positive outlooks towards self and life that developed over time. One participant noted:

“I view myself in a positive light generally. I think I have my moments of insecurity, just like everybody else, you know, but I am on a journey of trying to fake it ‘till I make it, so to speak, in the confidence department, specifically when it comes to my leg ulcers….” (PT female 50046)

Religious support

Regardless of family and social support, half of the participants mentioned religion as a means of mitigating the psychosocial burden of sickle cell disease and its associated leg ulcers. A prevalent theme included the belief that a supportive, higher power was incapable of making mistakes or placing individuals in situations without reason. Many of the participants who mentioned religion as a coping strategy reported taking comfort in knowing that:

“God doesn’t make mistakes, right. He sees everything, man; I’m very comfortable with my life, I’m very comfortable with the way I am. God doesn’t make mistakes; everything happens for a reason, you know. I mean, I know leg ulcers are never stuff for me, just a part of my life, that’s just the way it is.” (PT male 50002)

Religiosity and a trust in God served as a positive source of strength, particularly when family and social support was non-existent. One participant described an attempted suicide due to the emotional burden and implications of living with a leg ulcer, such as impeding her ability to physically play with her children. She stated: “And then I just looked up in the sky, and asked for God, for He told me not to do nothing like that, because I was just so frustrated with so much pain and not able to go out” (PT female 50063).

Individuals often surrendered their problems to a higher power. It is plausible to state that a sense of spirituality or religion helped attenuate negative emotions, buffer negative thoughts, positively influence their experience with chronic pain, and contribute to acceptance of their illness. Interestingly, most individuals expressing a strong sense of spirituality or faith also had positive responses when asked if they had been able to live life the way they want to.

Guidance for providers and researchers

Due to the chronicity of leg ulcers and the nature of SCD, many participants have had countless encounters with the healthcare system throughout their lives. Some respondents reported their belief that the healthcare system did not have a complete understanding of the extent of care needed to manage their ulcer. Some participants mentioned that future research should be done to “find out what causes it” (PT female 50003) and “what prevents it” (PT male 50002). One participant asserted that understanding the pathophysiology of leg ulcer pain is one task, but conducting research to find a cure for leg ulcers is needed.

“People need to understand that they're painful… They are life-limiting. They're embarrassing. We need to do a whole lot more work research-wise on how to make [leg ulcers] better; on how to cure them because I guarantee that most of the people that have them, even with the other stuff they go through with sickle cell, will say it's the worst thing they've dealt with.” (PT male 50005)

Participants also reported experiencing poor healthcare provider communication (S2 Table). One participant expressed the desire for physicians to understand their patients as individual people and not symptoms of disease. She also stressed that because everyone is different, treatment regimens that may work for some, may not work for others.

“He [the doctor] makes it sound like it's just so easy, like if you just do this, dress it, and just do it for a few weeks, you'll be fine. [The doctor said:] ‘I had this other patient that … they kept doing it, and they kept doing it, and they kept doing it, and now the ulcer's closed.’ And I look at him and I'm just like, you know, I have a life outside of coming to you and getting these ulcers treated.” (PT female 50060)

Limitations

The study is limited to a cohort of twenty individuals. While this may objectively seem like a small sample size, this study examines a population of individuals with a rare complication (leg ulcers) of an equally rare disease (SCD). Another limitation was the difference in procedure to conduct the interviews (phone vs. in-person). Given the inherent limitations associated with phone interviews steps were taken to assure the interviewee’s phone conversation were confidential and that adequate time was provided to each participant to feel at ease and to share their experiences with leg ulcers.