Social taboos: a formidable challenge in cancer care

Abstract

Taboo surrounding cancer has continued to be a stubborn and refractory public health issue especially in South Asian countries. Disparities in cancer care remain ubiquitous. Differences in the manner in which cancer is perceived, addressed, and treated might partly be a result of varying cultural influences. This case report highlights the clinical course of a female patient with neurofibromatosis who later developed a large facial malignant peripheral nerve sheath tumour. The case particularly addresses the catastrophic impact of the ‘cancer-related social taboos’ on various dimensions of cancer care ranging from primary and secondary prevention to definitive management. The financial issues in low-income to medium-income groups as potential deterrents to optimum treatment have also been highlighted. Approach to the common challenges faced by an oncologist practising in a society plagued by misconceptions about health and disease and potential remedial measures to debunk these myths have also been discussed.

Background

Women’s health needs are largely ignored especially in patriarchal societies in developing countries worldwide even in the 21st century. The situation becomes arduous and grim when they suffer from cancer. Various social taboos and cultural myths surrounding the word ‘cancer’ along with gender discrimination delay the diagnosis of cancer in women and adversely affects the outcome. In this context, we discuss a case of women who presented to us with a huge malignant peripheral nerve sheath tumour (MPNST) over her face. We reviewed what caused the delay and how these barriers to effective cancer care for women can be addressed in developing countries.

Case presentation

A woman in her 40s, resident of a rural area, presented to a tertiary care centre in north India with a large swelling located at the right temporal and cheek region distorting the facial contour. There was a gradual increase in size of the swelling over a period of one and a half year, which was initially ignored by the patient and she used a dupatta (a long traditional scarf used by women to cover the head) to mask the increasing size of the swelling. There was a history of recent onset pain and paraaesthesia in the swelling. She had been taking some alternative medicines prescribed by an unregistered medical practitioner prior to visiting us. Notwithstanding the fact that the same proved futile culminating into a progressive disease, the inherent social taboos and unavailability of a public cancer treatment in the vicinity were tantamount to a primary delay of 1 year in seeking medical attention before she finally visited us.

Clinical examination revealed a 20×18 cm broad-based, multi-lobulated swelling present over the right temporal, periorbital and cheek region (figure 1). There were multiple firm and discrete subcutaneous neurofibromas present over the face and scattered all over the body. The patient had never sought any medical consultation or genetic testing for the same in the past. A detailed skin survey was conducted to look for any other stigmata of neurofibromatosis. However, apart from multiple neurofibromas, no other tell-tale sign was observed. An ophthalmological consultation ruled out the presence of Lisch nodules. Vision and the ocular movements in the affected eye were normal. There was no history of similar lesions or other clinical findings suggestive of neurofibromatosis type 1 (NF-1) among first-degree relatives.

Figure 1.

(A) Preoperative photograph of the patient displays a large swelling located at the right temporal and cheek region distorting the facial contour and multiple neurofibromas. (B) Postoperative photograph.

A contrast-enhanced CT scan of the face showed a large soft-tissue mass with the erosion of the lateral wall of the orbit and the zygomatic arch with medial displacement of the right orbital contents (figure 2). However, no intraconal and intracranial extension was noted. CT imaging of the chest and abdomen did not reveal any metastasis. The patient and her relatives were offered genetic testing which they refused in view of financial constraints.

Figure 2.

CT scan of the face shows large soft-tissue mass with the erosion of the lateral wall of the orbit and the zygomatic arch with medial displacement of right orbital contents.

The rapid increase in the size of swelling raised the possibility of a malignant transformation. The case was discussed in the multidisciplinary tumour board and a plan to proceed with surgery followed by adjuvant therapy based on the final histopathology report was made. She underwent wide local excision of the tumour followed by reconstruction of the surgical defect via a local flap. Histopathological examination showed dense cellular fascicles with alternate myxoid regions which stained positive for CD99 and S100, favouring the diagnosis of MPNST. She recovered well in the postoperative period and was satisfied with the aesthetic outcome. She received adjuvant radiotherapy as a part of multimodality treatment protocol. Thereafter, she was lost to follow-up.

Around a year later, she presented to our outpatient clinic with dyspnoea. She was evaluated and was diagnosed with multiple lung metastases. The status of disease and its prognosis were explained to the patient and caregivers in the vernacular language. She was referred to a medical oncologist for further management but the family refused further treatment after knowing grim prognosis and opted for best supportive care. The patient succumbed to the disease one and a half year after the surgery.

Global health problem list

• Cancer is the second leading cause of mortality in women worldwide, accounting for 14% of all deaths.

• Stark inequalities in cancer prevention, screening, treatment, and outcomes exist not only between high-income and low-income nations but also between multi-ethnic communities within countries resulting in healthcare gaps.

• A striking cancer fact in India is the associated social stigma, particularly among women.

• Primary delay in seeking medical attention is a major determinant of poor survival outcomes in cancer.

Global health problem analysis

Health-seeking pattern of women in developing countries has always been a challenge for policymakers because of a myriad of factors—lack of knowledge and empowerment, poverty and unavailability of healthcare facilities in the vicinity.^1–3 Women empowerment remains a major challenge to fulfil even in the 21st century. A significant number of families in developing countries, including India, are by and large patriarchal in structure. Women depend on the men—father, husband, brother or son—for the decisions that concern their bodies and lives. This patriarchal mindset initially fails to recognise the ailments of the women, and later on delays medical attention for the same.⁴ Women herself play a number of roles in the family—of mother, wife, daughter and sister; unfortunately, they ignore their own problems leading to primary delay in the treatment. Moreover, there is a social reluctance to accept the diagnosis of cancer, widely considered to be a bad omen especially among women. This further results in primary delay and bears a significant impact on the overall survival from an oncological perspective. Furthermore, poverty proves to be a major barrier to accessing quality cancer care.⁵ Not only cancer treatment is a financial catastrophe for the poor, but even its anticipation also prevents them from seeking medical care. Unavailability of healthcare facilities in the vicinity, over-crowded hospitals and long waiting time for treatment of cancer in public hospitals further add to the long list of causes of delay in cancer care.

MPNST is an aggressive soft tissue sarcoma associated with dismal clinical outcomes and its risk increases dramatically in individuals with NF-1. Von Recklinghausen’s neurofibromatosis is an autosomal dominant hereditary disease with total penetrance and variable expression with the highest rate of spontaneous mutation among all human genetic diseases.⁶ NF-1 is associated with an approximately 10%–15% lifetime risk of developing MPNST. Due to this association, classic genetic studies of this malignancy have largely focused on the loss of the NF1 gene as the primary recurrent event.⁷ It is, therefore, advisable that patients with this disease, especially potential parents seek genetic counselling and be well apprised of the risks of passing the disease to subsequent generations. However, in most Asian countries, including India, genetic counselling still remains at a nascent stage equipped only with a handful of trained geneticists. A classic example is that of the pressing need for early screening of BRCA gene mutations. Though genetic counselling for BRCA mutation testing is common in most developed countries, India still faces several challenges in mainstreaming the same.⁸ Apart from the cultural bias and psychological trauma from the disease per se, the economic burden from genetic counselling and testing is high, especially for females from underprivileged backgrounds.⁹

Another critical issue is that these tumours can attain massive dimensions if patients are uneducated, oblivious and under the influence of social stigmas about the disease, thereby leading to primary delay in seeking medical attention. Resection of such giant tumours results in significant functional and aesthetic morbidity. Proximity to vital structures in the head and neck region poses another challenge to both oncologists and the reconstructive surgeon. Reconstruction of these complex defects is a technically demanding procedure and postoperative outcomes may not always be excellent in terms of function and aesthetics. Most of the patients with head and neck tumours are elderly patients with additional medical comorbidities. An optimum reconstructive procedure should thus be selected while counting personal, social and financial aspects into consideration.¹⁰

Various remedial measures have been proposed to overcome these barriers and socio-cultural beliefs that preclude an optimum cancer treatment in developing nations. Less than 20% of Indians are covered under health insurance schemes. Hence, various centrally sponsored initiatives like Health Minister’s Cancer Patient Fund and the Ayushman Bharat Scheme have been launched aimed to cater to patients living below the poverty line.¹ In addition, healthcare providers need to be flexible and culturally sensitive to the needs of different ethnic and racial groups in order to ensure the provision of equitable cancer care in increasingly diverse communities. Gupta et al¹¹ conducted a qualitative study with in-depth interviews and focused group discussions to evaluate cancer awareness and stigma from multiple stakeholder perspectives in north India. The authors suggested various actions to combat cancer-associated stigma. These include spreading awareness through posters, media, health talks in schools, colleges and social events; free screening programmes; small-scale loans for rehabilitation of cancer patients; simultaneous counselling of family members and addressing their fears pertaining to cancer and demystifying misconceptions. Cancer survivors are the best ambassadors to spread the cancer awareness and may prove instrumental in debunking various cancer myths and misconceptions. Another significant concern for the cancer survivors is the stigma and workplace discrimination. Stergiou-Kita et al¹² highlighted the prevailing misperceptions regarding the work abilities, productivity, reliability and future impact of cancer recurrence on the workplace leading to a discriminatory behaviour towards cancer survivors. The authors emphasise on promoting various anti-stigma programmes and anti-discrimination legislation targeting the ongoing myths regarding cancer and survivors right to work.

Undoubtedly, health should be a guaranteed right for all people. Hence, in developing countries, medical care for the less favoured classes is a responsibility that should be borne not only by their governments, but also an aid and collaboration from various health and political international institutions.

Low awareness levels regarding cancer and associated social taboos are considered to be a serious public health issue because they prevent early recognition of the disease leading to delay in holistic diagnosis and treatment and eventually poor outcomes. Hence, these taboos pose a great challenge to oncologists, especially in the developing world and warrant multidimensional redressal. The sheer amount of fear and myths associated with the disease truly confirm it to be ‘The Emperor of all Maladies’.¹³

Patient’s perspective.

Patient’s husband informed us: ‘When I visited the hospital and observed so many patients – irrespective of their age, gender, and cast – suffering from cancer, I have believed that cancer is like any other disease and can affect anybody. I have also seen patients with cancer doing well after the treatment and enjoying their life. I regret why I delayed the treatment for my wife and allowed her to suffer. Perhaps, had there been a public cancer hospital near to my village, I could have taken her there early and she could have lived with me longer’.

Learning points.

• Stigma with cancer is deep-rooted in the minds of certain ethnic populations since time immemorial and continues to haunt oncologists and healthcare workers even in the 21st century.

• There is an urgent need to sensitise the society to address cancer stigma and discrimination with regards to gender inequity as barriers to effective cancer care.

• Government must ensure the availability of quality cancer care in all parts of a country and must support the financial cost of the treatment for poor patients.

• Participation of patient advocates, including community leaders and religious priests in various awareness programmes, can be a useful initiative to demystify these myths.