Pheochromocytoma is a major cause of morbidity and mortality in the multiple endocrine neoplasia type 2 (MEN 2) syndrome. For the physician, surgical treatment seems well justified even though bilateral adrenalectomy will induce iatrogenically complete loss of adrenocortical function. For the patient this treatment may be a cause of medical problems as well as worry. We have evaluated quality of life after bilateral adrenalectomy in 27 MEN 2 patients through a combined oral and written approach. Mortality was low (one of 27), as was serious morbidity. Most patients had adapted well to the postadrenalectomy state. However, fatigue, worry, and noncompliance with daily medication often caused problems

Berg, B.

Ponder, B. A. J.

Ponder, M. A.

Telenius-Berg, M.

Werner, S.

English

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Henry Ford Hospital Medical Journal Volume 37 Number 3 Article 22 9-1989 Quality of Life After Bilateral Adrenalectomy in MEN 2 M. Telenius-Berg M. A. Ponder B. Berg B. A. J. Ponder S. Werner Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal  Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Telenius-Berg, M.; Ponder, M. A.; Berg, B.; Ponder, B. A. J.; and Werner, S. (1989) "Quality of Life After Bilateral Adrenalectomy in MEN 2," Henry Ford Hospital Medical Journal : Vol. 37 : No. 3 , 160-163. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol37/iss3/22 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Quality of Life After Bilateral Adrenalectomy in MEN 2 M. Telenius-Berg,* M.A. Ponder,* B. Berg,* B.A.J. Ponder,* and S. Werner§ Pheochromocytoma is a major cause of morbidity and monality in the multiple endocrine neoplasia type 2 (MEN 2) syndrome. For the physician, surgical treatment seems well justified even ihough bilateral adrenalectomy will induce iatrogenically complete loss of adrenocortical function. For the patient this treatment may be a cause of medical problems as weU as worry. We have evaluated quality of life afler bilateral adrenalectomy in 27 MEN 2 patients through a combined oral and written approach. M ortality was low (one of 27), as was serious morbidity. Most patients had adapted well lo theposladrenalectomy slale. However, fatigue, worry, and noncompliance with daily medication often caused problems. (Henry Ford Hosp Med J 1989;37:160-3) Comptete lack of endogenous production of adrenocortical steroids is considered a significant health handicap. Be-cause pheochromocytomas are potentially life-threatening tu-mors, the benefit of adrenalectomy outweighs the iatrogenic handicap of comptete adrenocortical failure. The clinical man-agement of adrenal disease in muttiple endocrine neoplasia type 2 (MEN 2) is controversial. Surgical treatment is safe in that the risk for recunence of pheochromocytoma is low. Bilateral adre-nalectomy is therefore recommended even when there is only unilateral involvement, van Heerden et al (I) found a low morbidity and mortality in their patients after bilateral adrenalectomy. We have advocated a more conservative approach, using one of two different techniques. The first atternative is unilateral adrenalectomy, after which the patient is closely followed. When evidence of tumor in the contralateral adrenal gland is found, the patient witl have the second, ie, final, adrenalectomy (2). Because pheochromocytomas in MEN 2 rarely are malig-nant, we have considered an even more conservative approach as justified in a pilot study for suitable patients. This second atter-native is subtotal adrenalectomy in which the pheochro-mocytoma is removed along with the adrenal medulla and most ofthe cortex. The remaining adrenocortical fissue is left in situ as a vascularized rim (3). Our choice of these strategies is based on tumor development in the adrenals being frequently asynchronous. Unilateral adrenalectomy may give the patient several years of freedom from replacement with corticosteroid hormones. Many patients have already had a total thyroidec-tomy for medullary carcinoma for which they receive replace-ment therapy with 1-thyroxin. However, the adrenal treatment is more complicated and demands more understanding and coop-eration from the patient. The international debate about the management of pheo-chromocytoma confirms that this is a difficult issue (1). We therefore feet that more information is needed to establish safe guidelines for the management of MEN 2 pafients. As availabil-ity of medical care may vary in different parts of the world, there is need for a choice of options in different countries. Moreover, not onty physicians but atso the pafients themselves have great concern about the loss of adrenal function which necessitates complete dependence on exogenous corticosteroids supplied daily in divided doses. Some of our patients have been so wor-ried about these prospects that they have delayed, and a few have even declined, bilateral adrenalectomy. The aim of this ongoing study is to assess quality of life after bilateral adrenalectomy from analysis of data on the medical (somafic and psychological) consequences as wett as the prac-tical inconveniences that patients encounter. We present our first results on these patients' initiat concem about future practical problems and their psychological adaptation (Table). Patients, Materials, and Methods We examined 27 MEN 2 patients after bilateral adrenalec-tomy (13 Swedish, 13 British, and one Syrian resident in Kuwait). At the time of the investigation most patients were of middle age (median age 52 years, range 20 to 73 years). Two patients were deceased at the time of this study; both had been followed by one of us (MTB), and their problems were well known. Since most patients had had surgery several years earlier (median follow-up 6 years, range 4 months to 28 years), they had had ample time to adapt to their situafion. Eighteen or two-thirds of the patients had their adrenal tumors diagnosed as a result of family screening, whereas nine or one-Submitted for publication: October 20, 1989. Accepted for publication: November 16,1989. ^Department ol Medicine. Faculty of Medicine, Kuwait. tDepartment of Pathology, Faculty of Medicine, Kuwait. tCancer Research Campaign, Human Cancer Genetics Research Group, Cambridge. England. SDepartment of Endocrinology, Karolinska Hospital, Stockholm, Sweden. Address correspondence to Dr Telenius-Berg, Departmentof Medicine. Faculty of Med-icine. PO Box 24923. 13110 Safat. Kuwait. 160 Henry Ford Hosp Med J—Vol 37, Nos 3 & 4, 1989 Quality of Life after Adrenalectomy—Telenius-Berg et al Table Important Issues for Patient Information Before Bilateral Adrenalectomy Degree of physical handicap Problems wilh dmg intake Qualily of medical care Problems coping with job Psychological distress and worry third were diagnosed because of clinical symptoms only. How-ever, 21 (76%) of the 27 patients had classical symptoms of pheochromocytoma before surgery. They were therefore well motivated for surgery and probably had a positive attitude to-ward enduring the hardships of being adrenalectomized. Information was compiled from the patients themselves, as well as from first-degree relatives, and in some cases from the physicians involved. The patients were interviewed twice, first via a written questionnaire and then orally. The same protocol was followed for the oral interview with the possibility to add information not covered by the questionnaire. If the patients were not followed by one of us, their physicians were also inter-viewed. First-degree relatives of the deceased patients were in-terviewed and asked to answer as they thought the patients would have done. The questions were chosen so as to give an-swers to specific objects of concem for the treating physicians as well as for the patients themselves. The answers were graded ei-ther dichotomously (yes/no) or according to a scate (0-2). Results The written and oral examinations gave similar results, ie, consistency and reproducibility were satisfactory. The preva-lences for difficulties within the problem areas are presented in the Figure (compare with the Table). Degree of physical handicap Medical problems related to the adrenalectomy or the lack of endogenous producrion of corticosteroids and leading to ex-traordinary hospital admissions were encountered in nine (33%) of the 27 patients. This percentage includes one postoperafive death. This 74-year-oId man had an uneventful immediate postoperative course, but ten days after bilateral adrenalectomy he suffered a massive hematemesis and ultimately died in multi-organ failure. His pheochromocytoma had been detected 14 years earlier, but he had declined operation for both the adrenal and thyroid tumors since he had had no subjective symptoms for the first 12 years. This frequency of hospital admissions does not include office visits for routine care or for adjustment of corticosteroid re-placement, nor routine shift to parenteral therapy during gas-trointesfinal upsets or other intercunent disease. Other somatic medical problems encountered were long-lasting fafigue, back-ache, impaired tolerance (to infections, stress, and tow carbohy-drate intake), hypoglycemia, and hyperpigmentafion. Further analysis ofthe medical problems will be reported separately. Per cent Physical Med.care Drugs a b c Profession Worry Figure—Problems met by MEN 2 patients after bilateral adre-nalectomy. Y axis; Fraction of patients with problems. X axis; Physical and medical problems, medical care, problems with medication in emergency, problems with medication in gas-trointestinal upsets, problems in remembering to take medica-tion, problems in daily work, and worry about medical complications. Problems with drug intake Medical complications may be related to difficulties or non-compliance in taking medicines as prescribed. Eleven (42%) of 26 patients had difficulty remembering to take their medica-tions. Two patients indicated that this occurred "often," whereas the others answered "sometimes" or "occasionally." One patient had made it a routine to take the complete 24-hour dose once daily because of her forgetfulness. One patient stopped taking her medicines when she became depressed. This type of problem was considered clinically significant by the treating physicians in only two cases. Most pafients said they would increase or double the dose if they had forgotten to take the tablets. Four (16%) of 25 patients reported difficulty taking medicines during an emergency, and seven (28%) of the 25 patients had such problems during gastrointestinal upsets. However, only three (12%) of these pafients viewed such problems as having a negafive impact on their quality of life. Quality of medical care All but one patient were satisfied as having good and continu-ous health care and being seen regularly by competent medical specialists (many of whom have a special interest in MEN 2). Most patients lived only a short distance from the nearest hospi-tal. Five patients lived more than 50 km away, including one British missionary in Africa who had to travel at least 320 km to reach a hospital. Problems coping with work Seven of 24 patients reported problems of excessive long-last-ing fatigue after the adrenalectomy which created difficulty in coping with the same workload as before adrenalectomy. A few patients felt that one to two years of partial sick leave woutd have Henry Ford Hosp Med J—Vol 37. Nos 3 & 4. 1989 Quality of Life after Adrenalectomy—Telenius-Berg et al 161 been adequate for full adaptation. However, two-thirds of the pa-tients reported no problems in coping with work. Psychological distress and worry A totat of 48% of the patients did not regard their condition as a reat handicap, 8% had no decided opinion, while 44% consid-ered themselves more or less handicapped. A total of 18% re-ported intense worry about their condition, 50% reported slight to moderate wony, and 42% said they did not worry at all. How-ever, when asked how often they felt worried, 18% said they felt very worried often or daily, white 68% admitted they felt dis-tressed only at times, and 18% said they never wonted at all. A total of 25% were afraid they might not reach the hospital fast enough in an emergency, and one-third of the patients were concemed that they might ran out of the necessary medicines. Several patients chose not to travel abroad since they did not trust foreign medical care in case of emergency. Some said spontaneously that they were concemed what would happen in case of war. Discussion To advise a candidate for adrenalectomy on which option to choose, all aspects of quality of life after surgery must be con-sidered. Besides possible medical hazards, patients may face so-ciat and emotional problems of adaptation. The concept of qual-ity of life must consider physical, social, and emotional facets (4). Measurement of quality of life is becoming increasingly important in medicine (5). In some countries, such as in Swe-den, there is a strong trend to make the patient the ultimate deci-sion-maker instead of the physician as has been the tradition in the past. Regardless of whether the physician has the responsi-bility of making the final decision in the choice of therapy or is more of an adviser for the patient, that physician needs a factual basis of informafion to batance different altematives. Earlier quatity of life studies have mostiy emphasized objec-tive data (eg, frequency of hospital admissions and dependency on daily drag intake). Cunent interest is focused more on the patient's subjective experience—clinical symptoms as welt as physical, intellectual, and emotional funcfion (inctuding capac-ity to work, wony, and need for support from medical care per-sonnel and from famity members). In this study the combined oral and written interview had a good reproducibility, ie, the answers did not change when the same question was asked tater. The evaluating instrament should be able to detect small changes in how the patient values his sit-uation in life. Unfortunately, there is no gold standard as to the validity of this instrament, ie, how close the relationship is be-tween outcome of the investigation (the answers to the quesfion-naire) and the patient's quatity of life. Pheochromocytomas if left untreated or if treated too tate cany a high mortality. To delay or even decline surgery certainly carries a high risk. In a review of MEN 2 patients in Sweden, eight of 46 patients with pheochromocytoma died from their adrenal tumor (6). Most if not atl of these deaths probably could have been prevented. Today we have methods for earty diag-nosis, as well as improved management with an arsenal of alpha-and beta-receptor blocking drags in combination with modem anesthesiotogic techniques. We can safety advise surgical treat-ment for our patients knowing that the benefit in mortality and morbidity will outweigh the disadvantages of the operation. However, such arguments do not convince atl patients to pro-ceed with surgery. Some may feel that other aspects such as quality of life have the greatest impact on this decision rather than morbidity and mortality. One of our patients has declined surgery for her pheochromocytoma, which was diagnosed over ten years ago, because of the hardships her mother suffered after bilateral adrenalectomy. Her mother had had problems fotlow-ing instmctions, especially when she had recunent mental de-pression, and had stopped taking medicine which resulted in critical situations and threatening collapse. This case was the background of the present study. Our conclusion as to prognosis agrees with that of van Heerden et al (1) in that the overall prognosis after bilateral adre-nalectomy is good with low morbidity and mortality. In our se-ries the operative and postoperative mortality was low (one of 27 patients). This man delayed operation for many years since he was afraid of the disadvantages of a life without adrenal glands. He is thus an example of those MEN 2 famity members who are difficult to convince to have adrenalectomy and whose prog-nosis probably would have been better with early treatment. We also found serious morbidity to be low with the greatest hazards experienced by patients who through mental illness or general carelessness were noncompliant and stopped treatment. However, morbidity of low or medium severity was more common, necessitating repeated hospital admissions for invesfi-gations and metabolic control. Chronic fatigue as well as con-stant psychologic pressure were described by about one-third of the patients. A similar number reported that their condition was such a handicap that they had problems coping with their jobs. It is possibte and even probable that the cause of fafigue in some of these patients may be related to other disorders, such as me-tastasizing medullary thyroid carcinoma. Apart from being infonned of potential medical hazards, bi-laterally adrenalectomized patients must be welt educated about their condition and the various treatments necessary for different situafions. Patients must atso be responsible and compliant re-garding medication. No doubt the condition is a social and psy-chological burden with which these patients must cope, but thus far we have had little knowledge of these social and emofionat aspects. Quality of life atso includes psychological well-being, ie, the patients should feet reasonabty secure in their tives. Un-fortunately, the extent of medical advice necessary for these pa-tients may have the disadvantage of creating anxiety. Atl patients in our series had been wett informed that hormone replacement is essential and that stopping the regular medication would have serious and life-threatening consequences. This in-formation process is necessary in securing patient compliance and may be the reason we have seen so few medical complica-tions. However, for some patients this information can create anxiety, such as wonying that for some reason or another the suppty of medicines cannot be guaranteed. The intense wony experienced almost daily by 18% of the patients may in part be a side effect of the medical education provided by the physician with the best of intentions. This group may be helped through 162 Henry Ford Hosp Med J—Vol 37, Nos 3 & 4, 1989 Quality of Life after Adrenalectomy—Telenius-Berg et al further support and reassurance about the low risks and low inci-dence of complications. In general the psychological adaptation was good or very good in most patients. The patients' adaptation to different prac-tical inconveniences also seemed to be good. Chronic fafigue interfering with professional work seemed to be the most impor-tant side effect. Medical care for adrenalectomized patients in the countries studied (Sweden, England, and Kuwait) seems to funcfion welt, which may be partly due to MEN 2 research and pafient care attracting many physicians with this special interest. Acknowledgments We thank J.P. Monson, P. Rosswixk, N. Finer, P. Baylis, S. Shalet, J.J. Brown, N. Dudley, J.A. Thompson, J. Farndon, P.H. Sonksen, H. Gray, and the Cancer Research Campaign for their assistance. References 1. van Heerden JA, Sizemore GW, Carney JA, Grant CS, ReMine WH, Sheps SG. Surgical manageraent of the adrenal glands in the multiple endocrine neo-plasia type II syndrome. World J Surg 1984;8:612-21. 2. Tibblin S, Dyraling JF, Ingeraansson S, Telenius-Berg M. Unilateral ver-sus bilateral adrenalectomy in multiple endocrine neoplasia IIA. World J Surg 1983;7:201-8. 3. Hamberger B, Telenius-Berg M, Cederraark B, Grondal S, Hansson BG, Werner S. Subtotal adrenalectomy in multiple endocrine neoplasia lype 2. Henry Ford Hosp Med J 1987:35:127-8. 4. BergnerM. Measurement of health status. Med Care 1985;23:696-704. 5. Guyatt GH, Veldhuyzen Van Zanten SJ, Feeny DH, Patrick DL. Measuring qualily of life in clinical trials: A taxonomy and review. Can Med Assoc J 1989;140:1441-8. 6. Telenius-Berg M, Berg B, Hamberger B, et al. Impact of screening on prognosis in the multiple endocrine neoplasia type 2 syndromes: Nalural history and treatraent results in 105 patients. Henry Ford Hosp Med J 1984;32:225-32. Henry Ford Hosp Med J—Vol 37. Nos 3 & 4, 1989 Quality of Life after Adrenalectomy—Telenius-Berg et al 163 

Quality of Life After Bilateral Adrenalectomy in MEN 2

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Quality of Life After Bilateral Adrenalectomy in MEN 2

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