Alagille Syndrome, Medical Trauma and Mental Health: In Conversation with Shambhavi Ravishankar

Having grown up in India with a rare disease known as Alagille Syndrome, Shambhavi’s commitment to fighting for the rights of persons living with rare diseases led her to study law.

Shambhavi Ravishankar is a Felix Scholar and lawyer, qualified with a Masters in Human Rights Law from the University of Reading. Her areas of practice include public policy, legislative research, human rights, health laws and environmental laws. She works as Legal Advisor to the Organization for Rare Diseases India (ORDI) for advocacy, outreach and awareness and legal and policy support. She also serves as a director on the Board of the Alagille Syndrome Alliance, an international not-for-profit organisation that is pushing for a cure for Alagille Syndrome as well as rendering patient support services.

Maitreya Shah: You have been living with Alagille Syndrome, a rare genetic disorder. Can you tell us a bit about coping with its challenges since your early childhood?

My disorder was detected at the age of six, by which time I genuinely feel a lot of damage — and strangely enough, the body’s ability to cope — had already happened or begun. For me, my childhood was about constantly listening to my body (it is even now, come to think of it). From the age of 1 to 12, I was a joyful kid trying to live the so-called ‘normal’ life. However, there were several crises throughout, persistently affecting both my mental health and life at large. There were obvious issues with the bile ducts in the liver, and with the heart too. The constant juggling of hospital visits, school and everyday life were the other painful additions. I never went to a therapist until a month back; had I consulted one before, I think I would have been diagnosed with a psychological illness for sure — because living under the constant haze of a cloud is not normal.

Maitreya Shah: Having briefly worked with you before, I have some idea how the disorder has shaped your experiences when you were at the university, and at the place where you are working now. Can you elaborate on some of the barriers? 

Given the disorder, it is challenging to work continuously on a nine to five job that we lawyers are usually expected to do (though not impossible), with chronic pain and chronic fatigue as hurdles. Even at the university, I had issues coping with the stress because of the lack of inclusion. I remember completing my projects under strict deadlines with a heaving chest. My cardiologist, after much elimination, stated that the impact was more psychological than physical in nature, and helped me with ideas to deal with the stress. I have always loved journaling and sketching, so I returned to them as coping tools.

Maitreya Shah: Although the role of medics in providing healthcare to rare disease patients is precious, I have come across literature that talks about diagnostic gaslighting, medical post-traumatic stress disorder and medical trauma by and large.  Have you had any exposure to these? 

I definitely have experience with the trauma of having frequent blood draws and of constantly undressing; of people poking and pinching me with various devices; standing or lying in front of devices, or just being the guinea pig for new diagnostics that come into the country.

I must say, it is very hard, especially when you are a child. I was only doing as I was told; which usually meant hours and hours of waiting with anxiety and stress. Typically, before every blood draw, I had to fast for 12-15 hours, which was followed by long queues in laboratories, which added another two-three hours to the waiting period. When the actual blood draw happens, it is usually very matter-of-fact, without any effort to calm the child who has been hungry for hours, waiting for someone to draw her blood. With this disorder, hunger and stress are already absolute triggers for me, on top of which I would also be itching.

As a child, I would be so drained by this process of waking up super early, going to the lab, waiting for hours hungry and stressed out, and finally getting jabbed with a needle without even being asked if I was okay. Having done this for almost a decade and a half, I carry that trauma. Even today, seeing a child waiting in a hospital lab wing gets my heart racing. Such childhood trauma actually impacts a person’s personality even after many years. In developed countries, you can choose to have counsellors/therapists at the actual blood draw who would help the child through the process.

With respect to gaslighting, I haven’t experienced my disorder being downplayed; there is already a very limited understanding of it, so much so that I have seen many senior doctors actually Googling my disorder in front of me. My parents have also faced the apathy of a few of them, especially when they were asked questions like: how did you let your child get this? or, which one of you gave her the disorder? With newer generations, the awareness is gradually improving. Although, there is still a long way to go. I always joke, you know, if one had to map physically the number of hospitals or doctors a rare disease patient has to visit, the map would explode! (laughs)

I, however, have a lot of hope with the newer generations studying medicine, and I must give due credit to all the affected people and their families who are more vocal about their rights.

Maitreya Shah: Apart from the medical trauma, what have been some of the toughest moments of your life, and how did they affect your mental health?

The adverse impact on my mental health has been a continuous process with several specific moments and triggers. I mean, I could write a book to answer this question! (laughs) Nevertheless, I can remember two specific instances — one from seven months back and the other back in 2013-14. And when I look back, I see how linked these two breakdowns were.

Around November 2019, I had a serious breakdown about where my work and life were going. It was a total crisis of confidence about whether I had held myself back. To finally deal with it, I started some serious soul-searching and healing meditations, as well as in-person therapy sessions in March this year.

As against this, back in 2013-14, I had serious doubts about almost every friendship or relationship in my life, affecting me badly. It was also the time I was waiting for my postgrad offers from universities abroad. After a prolonged crisis, offers from a few UK universities and a scholarship interview helped me cope. It was then that I began to allow the idea that as long as my professional life was working in some form, how did it matter if my social life was non-existent? Back then, I was honestly afraid of going for therapy; because I thought it would unearth more problems with me. It was hard enough that my body was a problem and people were a problem. I was so sure my mind would be a problem too. Not viewing myself as a problem has taken years, and I am in a better space now.

And this doesn’t include the emotional lows of dealing with life-threatening spinal surgery, rehabilitation, bed rest and re-entry into school at the age of 12.

Maitreya Shah: From my own experience, I think there are not many therapists and professionals trained to deal with people with disabilities or rare disorders. How sensitised was your therapist about your unique challenges? 

Yes, I am aware of professionals who are not trained for this. They tend to give you the generic blood-boiler statements like, “Everyone has challenges. Even healthy/normal people.” However, thanks to my ORDI network, I found a psychologist who herself has cystic fibrosis. Her level of sensitivity towards disorders and associated challenges is amazing. Like, she can understand if I am having a good day and a bad day in the same 24-hour cycle. I can say I have been quite lucky that way. I have also recommended her services to several others I know.

Maitreya Shah: Given how immersed you are in the rare disorders community, do you find conversations on the intersection of psychology and physiological disorders? 

Yes, there is definitely a wide-ranging conversation about this intersection. Furthermore, there is so much awareness about managing developmental challenges as well, through occupational therapy and speech therapy, among others. Parents of patients are also well aware of these needs.

However, you see, the financial costs for physical healthcare often range in crores of rupees, which force people to prioritise their physical health, which also has costs associated with everything, from tests to equipment, devices, attendants/nurses, and so much more. Even blood and plasma transfusions are such a big challenge in India. So, there is an understandable prioritisation. Therapy of any kind isn’t cheap.

Maitreya Shah: Dating, sexuality and relationships are also important factors when we talk about mental health, especially as adults. How do you describe your experiences? 

I do not have great experiences because, you know, The Fault in Our Stars, A Walk to Remember — they’re only movies! To put it bluntly, my experience has been that people do not want to date someone with a chronic illness. In effect, I have been told that I am not an option because of my condition and because of its physical appearance-related manifestations. It has an effect for sure. When people around you are so much into dating and relationships, you do feel like you’ve been left behind or like you’re always on the outside looking in. But eventually, you do make peace with this fact, as you do with everything else that the ‘rare life’ throws at you; or at least I hope that is the case.

Maitreya Shah: How do you think society’s perception has contributed to your life with the disorder so far? What are some of the conspicuous challenges?

I recall how a school principal was scared of admitting me in their school, driven by the fear of taking the onus if something happened to me. There were no healthcare services or protocols at the school; not even at the university. Lack of accessibility is one of the major limiting factors for children with disorders, which affects them at an early age. Come on, it’s a child after all!

Although I have received all my education in a private school which is a different ball game altogether, I think a lot can be done at least in the public schools. A mechanism could be created for rare disease students in schools where a trained attendant equipped to handle their healthcare and accessibility needs would be allowed in the school. Again, if this arrangement adds to the financial burden on the student’s family, the arrangement is good for nothing. This is essentially the way disability offices or school counsellors work abroad, where they deal with specific needs.

There is also the issue of whether persons living with rare diseases come under the ambit of the RPwD Act, and whether or not they should at all. Ideally, rare disease patients and persons with disabilities face similar challenges with accessibility, sexuality and gender. But that is a whole other debate that shouldn’t take away from the creation and implementation of unique solutions that enable rare children to have a childhood.

Maitreya Shah: You have been working with the Organization for Rare Diseases India (ORDI) on several policy initiatives. Can you tell us more about the work you do? 

At ORDI, we have been working on several fronts simultaneously. We organise awareness runs called Racefor7 in over 20 cities across the country to spread awareness on rare diseases. We also work in policy advocacy, for instance, getting insurance cover for people affected by rare diseases. We are currently talking to different state governments to accommodate people affected by rare diseases in out-patient services of hospitals, which are currently closed amidst the lockdown.

Maitreya Shah: Do you have anything else you would like to share with our readers? 

For people at large, rare disorders might sound like sad stories that you use to gain perspective on your own life. Instead of showing sympathetic sad reactions, if you really care about the person, letting them show you what they need, and asking how you can be of help is most constructive. Affected people and their families can really show you the way — whether you are the general public, a policymaker, or an employer.

For affected people, do what works for you. You know what that is. Do not let people tell you otherwise, no matter how close they are to you. Advocate for yourself and for what you need in any given situation. It’s okay to do things at your own pace and in ways that no one else understands. Just create your own normal.

Maitreya Shah is a final year law student at Gujarat National Law University and a LAMP Fellow in the 2020-21 cohort. He is blind and has been working for disability rights in the areas of access to justice, inclusive education and digital accessibility. He is also a tech-policy enthusiast.

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