Long-Term Survivorship — When The Cancer Battle Lasts a Lifetime

Diagnosed with Embryonal Rhabdomyosarcoma in my neck as an infant in 1973, I am one of the first survivors of my type of childhood cancer. Here I am, 48 years post-diagnosis, which most people hear and think “Oh great, you won your cancer battle. It’s in the past, way behind you.”

My doctors too believed that even as I sought care over the years when strange things started to happen – sudden hearing loss, facial palsies, trouble chewing food, frequent choking, difficulty speaking, neck weakness, an arm that was atrophying away, inability to use my hand, trouble breathing, heart changes, trouble sleeping – the list went on and on, and more than once I was told it was “all in my head.”

I was told that I was "cured".

Once I outgrew my care at my children's hospital oncology ward, I was discharged into the world of primary care. I was told that I was “cured” and given a 4x6 inch index card listing my total radiation dose, the type of chemotherapy I had been given, and instructions for my follow-up care as an adult to be an annual CBC and thyroid level testing (thanks to needing my thyroid removed from treatment late effects in my teens). No other special care was advised, and no imaging instructions except “no x-rays unless an emergency” due to exceeding my lifetime dose for radiation – and a hefty dose it was, 40 Gy of Cobalt-60 radiation. This is way more than is used today and the treatment methods were far less precise. This is the story of many childhood cancer survivors treated in the 70s–90s,

Most survivors today are told of possible late effects from chemotherapy – an increased risk of certain types of cancer, heart damage, hearing loss, and peripheral neuropathy to name a few. I wasn’t even told of those, mostly because doctors didn’t even know the late effects at the time – it was only through observing people like myself that they were able to collect that data.

One thing that most survivors of all ages are not told, however, is of the late effects of radiation. While the dosing of today’s radiotherapy is much lower and typically divided into much smaller increments given on a frequent basis, my dosing was huge and given in only 8 fractions, meaning each dose carried a whopping radioactive punch. However, even with today’s lower dosing and photon and proton delivery methods, patients are still encountering late effects from radiation, and those radiation impacts can extend far away from the original target zone for treatment.

Radiation Fibrosis Syndrome – ever hear of it? I sure didn’t, not until I was 47 and a physical therapist friend of mine started doing some research on my huge assortment of medical problems. After much digging, she came across some articles about radiation-induced fibrosis in breast cancer survivors and speculated I might be experiencing something similar. It turns out that radiation is “the gift that keeps on giving”. Radiation continues to impact our body well after the initial treatment ends. While most cancer survivors are familiar with the intended cell damage (and hopefully tumor death) that occur with radiation, as well as the subsequent burns and scarring that radiation causes immediately afterward, most of us (and most MDs) are clueless about what comes next when radiotherapy ends.

What truly happens after radiation?

Radiation fibrosis is very insidious and most of us don’t realize that it is occurring until it is quite advanced. There are several stages. First, there is a phase of scar tissue development from active fibrosis (that is the part we start to see right after radiotherapy when the skin darkens and becomes stiff), followed by a slow, hidden phase of microvascular damage where the blood supply to tissues is compromised, causing scarring not only in blood vessels (which can lead to loss of blood supply) but also atrophy and cell death in nerve and muscle cells and even necrosis (death and decay) of bone tissue. Subcutaneous fat and muscles can start to waste away. Your skin can further atrophy and can continue to scar and fibrosis, contracting and seemingly supergluing the skin to the structures underneath, restricting movement, and even pulling body parts inward into contracted positions. This process varies in length, with its resultant symptoms taking years, if not decades, to become apparent.

Any nerves that run through the path of the radiation site can be impacted, and their damage can extend to areas that seem well outside the treatment zone by virtue of their axons running long distances throughout the body. My neck took the brunt of my radiation treatment, leaving everything downstream to the rest of my body as well as some cranial nerves that ascend back up to the head in the path of destruction.

It took well over a decade from the onset of my radiation fibrosis syndrome symptoms to find a diagnosis and get appropriate care – and that care I had to seek myself upon my own referral (thank goodness I have my doctorate in optometry and was able to do the medical journal research to find my diagnosis and refer myself to the appropriate specialist for care). I got no help from a large number of physicians of varying specialties I visited along the way.

Some of you younger survivors are probably already piped into survivorship clinics, places to “graduate to” once you are in remission for usually 5 years or longer, where the doctors can monitor for long-term and late effects from treatment. However, if you are a young adult survivor there is also a fair chance that you aren’t even going to those clinics even if referred, being glad to just put the whole cancer experience behind you and try to live a normal life free from the reminders of your cancer past. For some, the clinics are inconvenient to visit, and then of course there is the cost – all those extra visits have copays and deductibles, and very few young adults have the financial resources to cover those kinds of annual expenses.

And then there are the older long-term survivors like myself, who were left to fend for ourselves, long ago discharged to the care of PCPs woefully uneducated about the late effects of cancer treatment. We were the original guinea pigs for cancer therapies and are the first to experience the late effects – and the last to be brought back into the medical system for appropriate care. We, as a group, have been extraordinarily let down by the medical system and the failure of our medical doctors to be referred for appropriate care. We have been mocked by medical doctors for “inventing” symptoms, and have been told we need mental health meds when in fact our autonomic nervous systems have taken a huge hit from radiation damage. Some have even had heart surgery done by surgeons unaware of the fact that the damage was caused by radiation and that different procedures were actually advised for survivors. Our nervous systems are shot from radiation damage and peripheral neuropathy from chemotherapy meds is our norm.

So if you are a survivor experiencing wonky symptoms and aren’t getting answers, keep radiation damage and late effects from your cancer treatment on your radar, and fight for getting appropriate diagnoses and care, seeking out survivorship and radiation fibrosis specialists. You will have to advocate for yourself medically for the rest of your life – it is a lifelong battle for care, rehabilitation, and having the mental fortitude to face what lies ahead.

Photo courtesy of author.