“I have retinoblastoma”.  Medical professionals would correct me – “you had retinoblastoma – now you are cancer-free”.  But am I really free from cancer?

I was born with aggressive tumours in both eyes, but my cancer was eventually destroyed after several long years of intensive treatments, relapses and more treatments. Thanks to expert care, my left eye was even saved with some useful vision.

So I am cured, right?  Yes and no.  The cancer is gone, but I live with the far-reaching consequences of it and its treatment every day (a radiation-damaged eye socket that can barely hold an artificial eye, deteriorating sight in my remaining eye, stunted bone growth around my temples, radiation-induced chronic cluster headaches…).  The genetic mutation that caused my cancer is a dormant volcano in every cell of my body, and its rarity means that seeking effective health care is a constant challenge.

About 50% of children with retinoblastoma have a genetic mutation not just in the tumour, but in cells throughout the body.  This includes all children with cancer in both eyes, and about 15% of children with cancer in one eye.  This “constitutional” mutation elevates the carrier’s lifelong risk of other cancers, particularly soft tissue sarcomas, osteosarcoma (bone cancer), brain tumours and malignant melanoma (skin cancer).  Just one tiny mutation of the RB1 gene could light the touchpaper to another firestorm.

Radiotherapy treatment for retinoblastoma further increases the risk of second cancers, and often these radiotherapy induced cancers behave differently from similar cancers that occur without any prior treatment.  Though copious published data describes our increased cancer risk, little evidence exists to guide follow up care or treatment when cancer strikes again.  The patient and their medical team must trust one another and find their way forward together, using related knowledge to form their best guess care plan.   This is a long, lonely, arduous path to walk, as a patient and a caregiver.

Second cancers have touched my own life too many times already, and took my beloved father in 1997 within three months of his diagnosis.  In just three years, I watched 12 beautiful people die from cancers subsequent to retinoblastoma.  Most were diagnosed late because they did not have effective follow up care and their primary doctors dismissed their concerns of early warning signs.  9 of my friends were diagnosed with leiomyosarcoma, a cancer that is very rare generally but is the most common cancer in adults following retinoblastoma.

I am blessed to have a wonderful primary doctor who listens to me, respects my own knowledge and follows through on my concerns.  Her understanding, humility and willingness to learn may save my life.  Sadly, many adults who had Rb are not so lucky, stonewalled by doctors who think they know more than the patient, who are not aware of the lifelong risks.

Ruling out common ailments before considering rarer conditions may be a good approach for the general population, but for someone with an RB1 mutation, the delay in diagnosis can kill.  This is especially frustrating when the patient is educated about the potential implications of symptoms, yet the doctor resolutely thinks only of horses, not zebras, as the galloping herd approaches.

These challenges following retinoblastoma are rarely discussed.  Mindful of the impact on parents going through the trauma of their child’s eye cancer diagnosis, we adults tend to say little about second cancers.  So awareness, understanding and support is very limited, among the public and among medical professionals responsible for our ongoing care.

You may notice my abstinence from using the word “survivor” in this writing.  “Survivor” implies the experience is in the past, completed, overcome.  I am still living through the consequences of my cancer every day, and I will do for the rest of my life.  I am still surviving it.

I understand why people say “I have retinoblastoma” or “my child is stable” years after the cancer is cured.  Even after tumours in the eye are flat and calcified, with exceptionally remote probability of regrowing, the lifelong risk of second cancers can hang like a smoke shroud, suffocating the new life that wants to bud and beautify the world.

Those of us who carry the RB1 gene mutation can never really say we are cured.  Every persistent ache, unusual lump, headache or other lingering symptom could be significant.  The goal for us and for those who love us is to find a balance between having enough knowledge to be strong self-advocates, and enough self-discipline and peace to store that knowledge in the back of our mind where it can be quickly referenced but where fear of it does not dominate our lives.

I am so thankful for the global community entwined by the shared experience of retinoblastoma.  We understand one another, share knowledge, console, encourage and build up.  Our close-knit, caring “family” reminds each of us daily that while we are dealing with the challenges of having a rare cancer syndrome, we are not alone on the journey.

There are days when I want to forget cancer completely, to live beyond the reach of all its complexities.  This is rarely possible though, especially as I volunteer full time running World Eye Cancer Hope, investing everything I can give into building up effective care for all with Rb around the world.  This is often a lonely, physically and emotionally challenging journey, but one that has led me to many wonderful people across the world who have blessed me with friendship and shared understanding.

In two days, I will fly to California to serve as a bride’smaid to one of my dearest friends.  People have been very surprised to learn that we only met in person for the first time in October 2013, when we shared a holiday in Spain, but we were communicating for years before that wonderful wintersun escape.  We were brought together by retinoblastoma and we share the long hike through unknown territory of life-beyond, but our friendship is so much more than that.  Just as this rare cancer syndrome seeks to destroy life, so those of us living with its ever-present challenges enrich one another’s living.

A rare and beautiful friendship brightens our world like phoenix flowers after a devastating wildfire.  In turn, as larkspur, poppies and lilies create shade in which the new generation of flora can rise up from a tortured land, so we hope the story of our lives nourishes those who come after us, so that they may grow strong and tall and full of hope for the future.