Results
A total of 1281 persons visited the webpage of the survey whereof 1245 responded to at least the first question (“In which country do you live?”). The respondents were from (by number of participants): US (801), Australia (90), France (81), UK (80), Canada (37), Sweden (35), Denmark (19), the Netherlands (8), Germany (7), Belgium (6), New Zealand (5), Mexico (4), Ecuador (3), Ireland (3), Spain (3), Chile (2), Dominican Republic (2), India (2), Norway (2), Philippines (2), Poland (2), South Africa (2), Switzerland (2), Argentina (1), Dubai (1), Greece (1), Hungary (1), Israel (1), Italy (1), Jamaica (1), Martinique (1), Portugal (1), Serbia (1) and Uruguay (1).
When asked “What type of cortisol deficiency are you suffering from?”, 939 participants (84%) defined their AI as primary (Addison’s disease, congenital adrenal hyperplasia, adrenal disease causing dysfunction of adrenal glands or removed glands) and 125 (11%) as secondary (pituitary or hypothalamic disease) while 51 (5%) were unsure.
Glucocorticoid Treatment Regimen
Overall, hydrocortisone was used by 75% of the participants, prednisone/prednisolone by 11%, cortisone acetate by 6% and dexamethasone by 4% of the participants, Table 1 . A high proportion of participants (40%) in Australia reported using cortisone acetate. Among the countries with at least 20 respondents, the use of prednisone/prednisolone was most common in Canada (27%), the US (14%) and Australia (11%) and dexamethasone was most commonly used in Australia (5%) and the US (4%). The distribution of therapies was similar between patients with primary and secondary AI. The majority of the patients were on BID (42%), or TID (32%), while 10% were on an OD regimen, Table 1 . Of the patients on a BID regimen, 58% took their medication in the morning and afternoon and 42% in the morning and evening. Patients with secondary AI took their BID dosing in the morning and afternoon (39%) rather than in the morning and evening (9%). 53% of the patients on prednisone/prednisolone were on BID and 5% on TID. For treatment regimen by therapy, please see Table 2 .
One quarter (23%) of the participants were dissatisfied or very dissatisfied with their current treatment, 18% were indifferent and 59% were satisfied or very satisfied. Patients with secondary AI reported less satisfaction with their current therapy than patients with primary AI. The ratings of satisfaction were similar among the different therapies.
Multiple daily dosing was reported as a problem by 38% of the participants whereof 15% were on OD, 35% on BID, 32% on TID and 17% on another regimen. Of those who did not find multiple daily dosing to be a problem 7% were on OD, 45% on BID, 32% on TID and 16% on another regimen. Among respondents answering that multiple daily dosing was a problem 94% reported one or more of the following: difficulties to remember/forgetting doses (particularly the midday and afternoon doses), difficulties in taking the medication at a specific time every day and/or difficulties to remember to bring the medication. Many reported that their days are planned according to their dose intake and for those who lead a busy life and are working, this was found to be challenging as some also reported that they do not want to be seen by their colleagues when taking their medication. Several reported that multiple daily dosing becomes very restrictive to an active life and that multiple daily dosing is a reminder several times per day that they have this disease. Many also reported health issues such as being fatigue and exhausted in the day, evening and the following day(s) if missing a dose. In addition, many reported that taking a missed dose too late disrupts their sleep and causes issues with insomnia. Some reported instability of physical and mental well-being with mood swings and ups and downs in energy levels. The patients who reported multiple daily dosing to be a problem also reported higher frequencies of impacted QoL, more fatigue and more activities altered due to their disease (data not shown).
Enduring efficacy over 24 hours was considered the most important feature of an optimal replacement medication (29%) followed by few side effects (25%) and low risk of adrenal crisis (22%). Similar responses were obtained from patients with primary and secondary AI.
Health-Related Questions and Hospitalizations
A majority of the patients (648 of 1026 [64%]) reported impacted quality of life (QoL) due to their illness, 87% (99 of 114) of patients with secondary AI and 60% (515 of 857) of the patients with primary AI. Approximately three quarters (73 of 99 [74%]) of the patients with secondary AI graded the impact on QoL as “quite a lot” or “very much”, Table 3 . The proportion of patients who reported impaired QoL varied with dosing regimen (OD > BID > TID). However, the level of impairment did not differ between the dosing regimens. A lower proportion of patients treated with hydrocortisone reported impaired QoL compared with patients receiving cortisone acetate or prednisone/prednisolone but the level of impairment did not differ between the therapies (data not country-adjusted).
All patients were asked “What activities do you need to alter due to your AI?”, regardless of whether they had answered that their QoL was impaired or not. A large percentage of the participants reported that they had had to alter their physical activity (56%), social life (40%), work life (39%) or family life (31%) due to their illness (the participants were allowed to choose more than one alternative). A higher proportion of patients with secondary AI (90%) than with primary AI (68%) reported that they had had to alter work life, social life, physical activity or family life, Figure 1.
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Figure 1.
Change in activities due to adrenal insufficiency. Responses to the question “What activities do you need to alter due to your adrenal insufficiency”? in an international patient survey. A total of 1001 subjects responded to this question.
A majority of the participants reported fatigue in the morning (57%) and during the day (65%) to be a problem. Fatigue was more pronounced in patients with secondary AI than in patients with primary AI, Table 4 . Of those reporting morning fatigue to be a problem, 75% also reported fatigue during the day to be a problem. Similarly, of those reporting fatigue during the day to be a problem, 85% also reported morning fatigue to be a problem.
In this survey, 61% of the respondents considered themselves fit to work while 17% did not. Additionally 5% of the respondents both considered themselves unfit to work and were on sick leave, 10% were retired and 7% were unemployed. Of those considering themselves fit to work, 72% considered themselves fit to work full-time, while 18% could work 75%, 9% could work 50% and 1% could work < 50%. Overall, 40% of the participants had been absent from work in the last 3 months and, again, this was more common among patients suffering from secondary AI (50%) than for patients with primary AI (38%). Almost one third (28%) of those being away from work or school reported more than 3 weeks’ absence in the 3 months preceding their participation in the survey, Table 5 . A higher percentage of patients treated with prednisone/prednisolone reported absenteeism compared with patients on hydrocortisone and they also reported more lengthy absenteeism (data not shown).
A majority of the participants were worried about long-term side effects. The participants were most worried about osteoporosis (79%), followed by obesity (64%), fatigue (52%) and cardiovascular problems (46%) (more than one alternative could be chosen), Table 6 . A higher proportion of those treated with prednisone/prednisolone were worried about long-term side effects than those treated with hydrocortisone (data not shown).
Overall, 32% of the participants reported that they increased their dose due to physical activity at least once per week (primary AI patients: 30%; secondary AI patients: 49%) and 66% increased their dose due to illness at least once per month (primary AI patients: 65%; secondary AI patients: 76%).
Overall, 38% of the participants responding to the question about hospitalizations (N=977) answered that they had been hospitalized at least once during the last 12 months (37% of patients with primary AI and 43% of patients with secondary AI). One third of the patients (32%) had been hospitalized more than once during the last 12 months. The reported reason for hospitalization was adrenal crisis, vomiting or an acute infection for 17% of the patients who had been hospitalized.