Patient and Provider Perspectives on Postsurgical Recovery of Cushing Syndrome

Rachel Acree; Caitlin M. Miller; Brent S. Abel; Nicola M. Neary; Karen Campbell; Lynnette K. Nieman

J Endo Soc. 2021;5(8)

Abstract and Introduction

Abstract

Context: Cushing syndrome (CS) is associated with impaired health-related quality of life (HRQOL) even after surgical cure.

Objective: To characterize patient and provider perspectives on recovery from CS, drivers of decreased HRQOL during recovery, and ways to improve HRQOL.

Design: Cross-sectional observational survey.

Participants: Patients (n = 341) had undergone surgery for CS and were members of the Cushing's Support and Research Foundation. Physicians (n = 54) were Pituitary Society physician members and academicians who treated patients with CS.

Results: Compared with patients, physicians underestimated the time to complete recovery after surgery (12 months vs 18 months, P = 0.0104). Time to recovery did not differ by CS etiology, but patients with adrenal etiologies of CS reported a longer duration of cortisol replacement medication compared with patients with Cushing disease (12 months vs 6 months, P = 0.0025). Physicians overestimated the benefits of work (26.9% vs 65.3%, P < 0.0001), exercise (40.9% vs 77.6%, P = 0.0001), and activities (44.8% vs 75.5%, P = 0.0016) as useful coping mechanisms in the postsurgical period. Most patients considered family/friends (83.4%) and rest (74.7%) to be helpful. All physicians endorsed educating patients on recovery, but 32.4% (95% CI, 27.3–38.0) of patients denied receiving sufficient information. Some patients did not feel prepared for the postsurgical experience (32.9%; 95% CI, 27.6–38.6) and considered physicians not familiar enough with CS (16.1%; 95% CI, 12.2–20.8).

Conclusion: Poor communication between physicians and CS patients may contribute to dissatisfaction with the postsurgical experience. Increased information on recovery, including helpful coping mechanisms, and improved provider-physician communication may improve HRQOL during recovery.

Introduction

First-line treatment of Cushing syndrome (CS) is surgical resection of the primary disease-causing lesion, which may be a pituitary tumor in the case of Cushing disease (CD), an ectopic ACTH-secreting tumor, or ACTH-independent adrenal lesion(s).^[1] However, the sequelae of chronic hypercortisolemia, including cardiovascular, musculoskeletal, metabolic, cognitive, and psychiatric disease, may persist long after surgical cure.^[2–7] Patients also require cortisol replacement medication (CRM) after surgery to avoid adrenal insufficiency and life-threatening adrenal crisis until the hypothalamic-pituitary-adrenal axis recovers.^[1,8]

Health-related quality of life (HRQOL) is decreased in CS, and it remains lower than the reference population after biochemical remission is achieved.^[9–14] A recent meta-analysis of 2643 patients with CS demonstrated that HRQOL and cognitive functioning improve but do not normalize to those of the general population after treatment.^[15] An Endocrine Society Clinical Practice Guideline on CS treatment recommends lifelong management of the comorbidities associated with chronic hypercortisolemia to improve mortality and HRQOL.^[1]

Drivers of reduced HRQOL have been explored.^[16–19] These include patient dissatisfaction with the medical profession regarding diagnosis, treatment, and aftercare for CS. Patients cite delays in diagnosis after the onset of symptoms,^[17,18] a lack of adequate information and education about treatment and recovery,^[16,17] and a dearth of health care professionals who are informed about CS^[16,18] as particularly detrimental to quality of life.

To design interventions aimed at improving HRQOL during remission from CS, a robust understanding of patients' perspectives on the postsurgical recovery process is needed. Given physicians' advisory role, and the dissatisfaction of patients with the quality of that relationship, it is also important to understand the physicians' perspectives on recovery. To our knowledge, no data exist on providers' opinions of or approaches to CS recovery.

Our survey approach was designed to further characterize patients' experience of recovery from CS, identify modifiable factors that may contribute to reduced HRQOL after surgical cure, and to compare patients' and physicians' perspectives on the recovery from this disease. Importantly, we attempted to incorporate both questions with "objective," quantifiable answers, and others that could be interpreted using a hermeneutic phenomenology approach.

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Abstract and Introduction
Methods
Results
Discussion
References

Table 1. Phase 1 patient survey questions
1. What was the cause of your Cushing syndrome?
2. Are you currently taking cortisol (Cortef, hydrocortisone, prednisone, dexamethasone) replacement medication?
3. If you are no longer taking cortisol replacement medication, how long after your surgery were you able to discontinue replacement medication?
4. If you are no longer taking cortisol replacement medication, please describe the tapering procedure you used and what tests you had done before discontinuing replacement.
5. How would you describe the recovery process?
6. What did you find helpful in coping with the recovery process?
7. If you consider yourself recovered, how long did your recovery take?

Table 2. Phase 2 patient survey questions
1. What is your sex? Male, female
2. What is your age in years?
3. In what country do you live?
4. What was the cause of your Cushing syndrome? Pituitary, adrenal, other, unknown, other
5. Have you had surgery to treat your Cushing syndrome? Yes, no
6. How many surgeries have you had to treat your Cushing syndrome?
7. What was the date of your most recent surgery for treatment of your Cushing syndrome?
8. Check one option below that describes your surgery. Pituitary, adrenal, other
9. When you were discharged from the hospital after surgery, which cortisol replacement medication did you take? Hydrocortisone, prednisone, dexamethasone, prednisolone, none, unknown, other
10. What was the cortisol replacement medication dose that you were discharged on from the hospital after surgery?
11. Are you currently taking any type of cortisol replacement medication? Yes, no
12. If yes, what medication are you taking now? Hydrocortisone, prednisone, dexamethasone, prednisolone, none, unknown, other
13. What is the total daily dose?
14. If no, what medication was the last cortisol replacement medication you took after surgery? Hydrocortisone, prednisone, dexamethasone, prednisolone, none, unknown, other
15. How long after your surgery did you stop taking cortisol replacement medication? Please answer in months.
16. How did you take your cortisol replacement medication over time? None, constant, decreased, unknown
17. If you selected "decreased" for question 16, please select the option that best describe your tapering process. Begin rapid, begin slow, end rapid, end slow, equal, unknown
18. Who initiated the decision to stop or reduce your cortisol replacement medication? Doctor, myself, both, unknown
19. Which tests if any were performed after you were discharged from the hospital? ACTH blood test, cortisol blood test, ACTH stimulation test, ITT, cortisol urine test, dexamethasone suppression test, no tests, unknown
20. Except for sick days, did you ever increase your dose of cortisol replacement therapy? Yes, no, unknown
21. Were you ever changed to a different cortisol replacement medication? Yes, no, unknown
22. Have you taken any of the following medical therapies since your surgery? Ketoconazole, metyrapone, mitotane, no medical therapies
23. How would you describe your overall recovery experience? Positive, negative, mixed, neutral
24. After your surgery, did you lose weight? Yes, no, unsure
25. Are you now satisfied with your weight? Yes, no, unsure
26. After your surgery, did you suffer from lethargy (lack of energy)? Yes, no, unsure
27. After your surgery, did you suffer from pain in your joints? Yes, no, unsure
28. After your surgery, did you suffer from mental exhaustion? Yes, no, unsure
29. After your surgery, did you suffer from depression? Yes, no, unsure
30. Did you receive sufficient information about the postsurvey recovery experience? Yes, no, unsure
31. Who gave you information about the post-survey recovery experience? Endocrinologist, neurosurgeon, both, neither, other
32. Do you think those close to you were adequately prepared for your postoperative recovery? Yes, no, unsure
33. Would a standardized information sheet about the postsurgical experience from a professional organization of endocrinologists be helpful? Yes, no, unsure
34. Have you returned to work yet? Yes, no, unemployed
35. If yes to question 34, how many months after surgery did you return to work?
36. When you returned to work, did you work full time or part time? Full time, part time, other
37. What did you find helpful in coping with the recovery process? Friends and family, physicians, support groups, physical therapy, exercise, analgesia, antidepressants, massage, entertainment, activities, work, rest, religion, none, other
38. If you consider yourself recovered, how long did your recovery take? Please answer in months.
39. Was or is your recovery time longer than you expected? Yes, no, unknown
40. Do you have any other thoughts that you would like to tell us about your recovery experience after surgical treatment for Cushing syndrome? What could have improved your recovery experience?

For questions with associated drop-down menu options, those options are listed next to the question. All questions had an open-ended option.
ITT, insulin tolerance test.

Table 3. Phase 2 physician survey questions
1. How did you hear about this survey? Email, letter, both
2. What is your sex? Male, female
3. What is your age range? 20–29, 30–39, 40–49, 50–59, 60–69, 70–79, 80+
4. In what country do you live?
5. How would you describe your practice setting? Hospital, office, other
6. How many patients do you see with surgically-treated Cushing syndrome each year?
7. Approximately how many patients have you seen with surgically treated Cushing syndrome in total throughout your medical career?
8. What type(s) of cortisol replacement medication do you usually use after surgical treatment for Cushing's syndrome? Hydrocortisone, prednisone, dexamethasone, prednisolone, other
9. If you use more than one type of cortisol replacement medication, how do you choose which medication to use?
10. What is the range of time that your Cushing patients take to discontinue cortisol replacement medication? What is the approximate median?
11. What recommendations do you tend to make to patients taking cortisol replacement medication after surgery for Cushing syndrome? Nontaper, taper
12. What is the lowest dose of hydrocortisone would you use for a Cushing patient before completely stopping therapy?
13. What is the lowest dose of prednisone would you use for a Cushing patient before completely stopping therapy?
14. What is the lowest dose of dexamethasone would you use for a Cushing patient before completely stopping therapy?
15. What is the lowest dose of prednisolone would you use for a Cushing patient before completely stopping therapy?
16. What is the lowest dose of other cortisol replacement medication would you use for a Cushing patient before completely stopping therapy?
17. Regarding routine (non-sick day) therapy, who initiates changes in cortisol replacement medication? Myself, patient, both
18. Which tests, if any, do you perform to assess whether patients should stop replacement therapy?
19. Except for sick days, have any of your patients increased their dose of cortisol replacement therapy? Yes, no
20. How would you describe your patients' overall recovery experience on average? Positive, negative, mixed
21. What information do you routinely provide to your patients regarding the recovery experience after their surgery? Length of recovery, medication use, symptom recovery, nothing in particular, other
22. Does the neurosurgeon operating on your patient routinely provide information to your patients regarding the recovery experience? Yes, no, unknown
23. How do you give patients information regarding the recovery experience after their surgery? Discussion, written, other
24. Do you routinely include family and/or significant others in this education process? Yes, no
25. Would you welcome a standardized information sheet from a professional organization (such as the Endocrine Society or Pituitary Society) to help educate your patients and their relatives about their postsurgical recovery? Yes, no, use my own
26. Which of the following do you suggest to patients as helpful during the recovery process? Friends and family, support groups, physical therapy, exercise, analgesia, antidepressants, massage, entertainment, activities, work, rest, religion, education, none, other
27. What is the range of time you think it takes for your patients to fully recover after their surgery? What is the approximate median number?

For questions with associated drop-down menu options, those options are listed next to the question. All questions had an open-ended option.

Table 4. Useful coping mechanisms reported by patients and physicians
Coping mechanism	Phase 1 patient survey (N = 91)		Phase 2 patient survey (N = 337)		Phase 2 physician survey (N = 49)		P value^a
Coping mechanism	% (n)	95% CI	% (n)	95% CI	% (n)	95% CI	P value^a
Work	2.2 (2)	0.6–7.7	28.4 (96)	23.9–33.5	65.3 (32)	51.3–77.1	<0.0001
Religion	8.8 (8)	4.5–16.4	41.5 (140)	36.4–46.9	8.2 (4)	3.2–19.2	<0.0001
Exercise	16.5 (15)	10.2–25.4	42.7 (144)	37.6–48.1	77.6 (38)	64.1–87.0	<0.0001
Massage	3.3 (3)	1.1–9.3	23.1 (78)	19.0–27.9	2.0 (1)	0.4–10.7	0.0172
Family/friends	38.5 (35)	29.1–48.7	82.8 (279)	78.4–86.4	87.8 (43)	75.8–94.3	NS
Rest	16.5 (15)	10.2–25.4	73.9 (249)	69.0–78.3	42.9 (21)	30.0–56.7	0.0003
Physicians	15.4 (14)	9.4–24.2	49.0 (165)	43.7–54.3	–	–	–
Support groups	23.1 (21)	15.6–32.7	45.4 (153)	40.1–50.7	57.1 (28)	43.3–70.0	NS
Activities	6.6 (6)	3.1–13.6	44.8 (151)	39.6–50.2	75.5 (37)	61.9–85.4	0.0016
Entertainment	4.4 (4)	1.7–10.8	37.7 (127)	32.7–43.0	12.2 (6)	5.7–24.2	0.0117
Analgesia	8.8 (8)	4.5–16.4	30.3 (102)	25.6–35.4	18.4 (9)	10.0–31.4	NS
Antidepressants	3.3 (3)	1.1–9.3	30.3 (102)	25.6–35.4	36.7 (18)	24.7–50.7	NS
Physical therapy	2.2 (2)	0.6–7.7	13.9 (47)	10.7–18.1	28.6 (14)	17.9–42.4	NS
Other	44.0 (40)	34.2–54.2	11.6 (39)	8.6–15.4	4.1 (2)	1.1–13.7	NS
Nothing helpful	0.0 (0)	0.0–5.1	2.4 (8)	1.2–4.6	4.1 (2)	1.1–13.7	NS
Education	–	–	–	–	30.6 (15)	19.5–44.5	–

^aStatistical comparison between phase 2 survey patients and physicians is shown, and was obtained using 2 proportion z tests with subsequent Bonferroni correction.
Abbreviation: NS, not significant.

Table 5. Patient concerns mentioned in the final open-ended question of the phase 2 patient survey
Patient concern (N = 280)	% (n)	95% CI	Sample quotations
Felt unprepared for recovery and/or wanted more information on recovery	32.9 (92)	27.6– 38.6	"[I needed] better aftercare."
			"I wish someone would have told me … what I would be experiencing after surgery."
			"Information on how to recognize adrenal insufficiency/crisis would have been helpful."
			"I am 'in the dark' as to what's going on … at this point."
			"Recovery from having Cushing's has been really rough for me and my family. We had no idea what to expect, or how long it would take."
			"A list of more critical symptoms in which we should call the doctor would have been great to avoid all of the nagging unsure phone calls in which we have to hear, 'oh don't worry, that's normal.'"
Providers were not familiar enough with CS or provided inaccurate information	16.1 (45)	12.2–20.8	"I needed one single doctor who knew Cushing's but could also act like a primary care [provider] while my body resolved multiple problems. My Cushing's doctor wasn't interested in helping me through the little stuff, and my primary care [provider] didn't know anything about Cushing's recovery."
			"I am shocked that most medical professionals are NOT FAMILIAR with Cushing's syndrome or its recovery."
			"I just wish physicians were more familiar with Cushing's. It is so frustrating … [and] adds to the mental exhaustion of trying to fight off this debilitating disease."
			"I wish my endocrinologist had prepared me for what to expect in recovery … She said I would be able to return to work 2–3 weeks after surgery. It took me 3 months before I could return to work."
Difficulty with weight loss/persistent body image issues	14.3 (40)	10.7–18.9	"I'm upset that I've put weight on and wish I could have liposuction … "
			"I lost some weight after the surgery, but never ever came close to coming near what I weighed before the surgery."
			"The weight gain makes me feel horrible about how I look. My husband left after the surgery as he said he could not take the weight gain."
			"I had a big problem with my body image after I lost weight after recovering. I still don't feel very comfortable with my body."
Found/believed they would have found a support group beneficial to their recovery	12.1 (34)	8.8–16.5	"Peer support regarding the range of recovery experiences … was something that I should have sought out earlier."
			"I would have greatly benefited from a Cushing's support group."
			"Talking/reading up on people with the actual syndrome and how they handled [recovery] would have helped me out a lot more than going into the recovery process 'blind.'"
			"I would encourage endocrinologists [to provide patients] with information on local or virtual support groups that deal with Cushing's recovery."
			"I started a blog and a support group for patients in the early diagnosis and recovery of Cushing's. This allowed me to connect with others who were going through the same things I was and saved my sanity, knowing that I wasn't alone."
Recovery was longer than expected	7.5 (21)	5.0–11.2	"I think knowing how long the process could take … would have better prepared me."
			"I thought I would undergo surgery and walk out of the hospital well. Two years later I'm still not completely back."
			"A more realistic time frame for recovery [would have helped] … I expected a quick recovery."
			"It really needs to be stressed to patients that it will take a really long time to get better."
Wanted more information on recovery for family members/felt misunderstood by family	3.9 (11)	2.2–6.9	"Family members and patients need a more thorough education about the disease and symptoms as many of us spent years thinking it was our fault and something was wrong."
			"Formal information about recovery from the doctor to patient and his/her family member probably [would] help a lot."
			"I was not warned of all the physical things I would be going through (for example, joint pain, depression, physical exhaustion) so my family just thought I was crazy."
Experienced musculoskeletal pain	18.6 (52)	14.5–23.5	"I had so much joint and muscle pain I could hardly move."
			"I am very sore all of the time."
			"My shoulders froze, my knees gave out."
			"I never, never imagined I would go through such excruciating pain in the joints and muscles!"
Experienced fatigue	16.1 (45)	12.2–20.8	"The worst part of recovery is the physical fatigue."
			"Not having energy to do things I expected to be able to do was very disappointing."
			"[One of the] hardest parts of recovery for me was the lack of energy."
			"I am still very tired."
Experienced depression	11.1 (31)	7.9–15.3	"I felt ill-prepared for the severe depression I experienced."
			"I did go to talk therapy on my own, but that should have been required by the endocrine team [as I had] many issues with depressive thoughts."
			"I think an antidepressant should have been prescribed upon diagnosis of Cushing's and sustained through recovery. My depression was debilitating, but avoidable."

Abbreviation: CS, Cushing syndrome.

Table 6. Patient quotations from an open-ended question about their experience of recovery
"I seriously thought about asking my doctors to put me in a medically induced coma, as sometimes is done with burn victims. I still wonder if that may be the best route for some CS patients after surgery."
"If it had been left to the medical profession, I know I would have committed suicide by now. They removed my gland and sent me on my merry way."
"There is a constant fear of recurrence that isn't addressed."
"I thought I was quite prepared for the physical problems associated with the recovery, [but] I was not expecting the mental torture! I had no idea that I would be suicidal at times … No one seemed to mention it. These things were much worse than when I actually had the disease!"
"I feel like my recovery would have been much easier if there had been more awareness of this disease. Friends were not there for me during my illness as they thought I 'looked healthy' and I really think some of them thought I was just crazy. (I even wondered that myself at times.)"
"Because Cushing's is an invisible illness, many people think that because I'm smiling, or because I have a good day, or that because the tumor is gone, I'm healed … and that's not the case at all. Once the tumor is gone, THEN everything starts to get bad."
"I was told I was 'cured' of Cushing's, however … I am sicker without the tumor than I was with it … If given the choice again, I would have left the tumor even if the result would have been death."
"This is a scary and confusing disease. The recovery is long and tiresome, some days feeling like you will never be normal again. It would have been good to have known beforehand that it was going to be like this."
"Cushing's recovery was a horror. If I hadn't had support groups on the Internet to know what to expect I never would have made it."
"Recovery was more difficult than living with Cushing's … Having Cushing's makes you feel isolated and antisocial. Your body changes and your mind changes and sometimes those symptoms, along with the pain, are invisible to others."
"Doctors that treat CS have never had CS and therefore cannot understand it in the way that CS patients understand it."
"The whole experience from being diagnosed to recovery was very detrimental to my family and marriage. If more information had been made available to me, my recovery would have been viewed differently by my family and husband."
"My family member still does not believe that I'm having a difficult time … They think that I'm just lazy."