Around 9 o’clock on a Saturday night in January 2017, I heard weird noises in the bathroom. I opened the door to find my son curled over the toilet.

“It’s only cranberry juice. It’s only cranberry juice,” he muttered into the bowl. I took a glance- that wasn’t cranberry juice.

I can hardly believe it now, but we debated if vomiting blood was serious enough to worry a doctor with on a weekend. A phone call later, we knew it was. We carried Josh to the car. I sat with him (and the bucket) in the back seat trying to keep him awake.

“Stay with me, Josh,” I repeated over and over. It would have been funny to notice I’d stolen my comments from Grey’s Anatomy. Except it wasn’t.

Josh lost consciousness as we arrived in the ward. Nurses moved decisively into action. They may have trained for a scenario like this, but their hesitations of unfamiliarity made it clear it was more foreign than common. They scrambled to summon the on-call doctor, instructing us to “sit over there” while a panicking trainee managed to revive my son.

In the next few minutes the team had taken blood samples, made a series of flustered calls to find a bed in the ICU and a paediatric intensivist.

The ICU only allowed Shannon to accompany Josh into the unit. Even then, it was only long enough for him to sign parental consent forms for an immediate surgical procedure.  Like a scene from a movie, nurses had already hung the room with sterile green curtains. The intensivist stood, arms stretched wide, as nurses pulled a sterile gown and gloves onto him.

“You should go now. Right now.”

During the next forty-eight hours, Josh underwent a whole series of tests and treatments to try and keep him alive. By then we knew he had cancer, but we didn’t know what kind. The ICU team weren’t treating his cancer though – they spent the next few weeks treating the problems that the cancer had caused.

On that first night, Josh had an emergency procedure to insert a central line. That’s a drip, but instead of the drip site being in the hand or arm, a surgeon inserts it into a major artery (for Josh it was at the top of his inner thigh). A central line has multiple “ends” which allows the team to run multiple drips with different medications at the same time.

In Josh’s case, the central line was used to run three concurrent transfusions of FFP. Instead of using the central line to use multiple medicines they were using all three to get fresh frozen plasma (FFP) into Josh’s body. Fresh frozen plasma is the liquid part of blood. It helps clotting which is the system the body uses to makes scabs and stop nose bleeds. Whole blood is just normal blood. In Josh’s case, he needed plasma to try and stop the bleeding fast and then had a transfusion of another nine units of whole blood to replace the red blood cells which he’d lost during the bleed.

Before Josh was even stable, he went into the operating theatre for a liver biopsy where the surgeon removed a chunk of liver. The tissue was immediately sent to a pathology lab to find out what sort of cancer Josh had. At the same time, the surgeon used a gastro-endoscope that allowed him to see inside Josh’s throat and stomach.

The first result we received was that Josh had inoperable haemorrhaging gastric varices. The surgeon explained that varices are swollen blood vessels. They are caused by variety of illnesses. Left untreated, the blood vessels swell and eventually begin to bleed. They require emergency attention. Joshua’s were positioned in his stomach which made a surgical solution impossible.

In this instance the varices were caused by portal hypertension as a result of a portal vein thrombosis. The portal vein connects the liver to everything else. A thrombosis means a blood clot in a vein. A commonly used term is DVT- a deep vein thrombosis. A portal vein thrombosis isn’t a big deal on its own, but the underlying causes are usually serious. It is an indicator that something is wrong, usually cancer or liver disease.

The blocked portal vein was the source of portal vein hypertension. Josh had high blood pressure, but only in that one part of his body. Blood had backed-up behind the blockage. Bodies are clever, so the blood was being rerouted to other veins to get around his body. These veins aren’t designed for such heavy traffic though, the veins in Joshua’s stomach became swollen and developed the varices that caused such a serious bleed.

The surgeon wouldn’t speculate on the biopsy results except to say that Joshua’s liver was “full of tumour.” The results, he told us, should take about two days to arrive.

I hadn’t seen a surgeon cry before.

In the course of that first week, I saw my son go from a healthy athletic teen to a glassy-eyed, stick-thin invalid. He received nine units of blood and still had three transfusions running concurrently. I couldn’t understand it. He’d been learning to ski just a month before. Sure, he’d lost weight over the previous few months, but he was training hard to get a place on the swim team. Josh had said he was tired after his exams, but who wouldn’t be when they were determined to bring home a full house of “A’s”?

How could this have happened? We asked the same question over and over. It’s the question I still ask and still get asked over and over by other cholangiocarcinoma patients and caregivers. Science hasn’t found the answer to that question yet. At the time though, we were hugely frustrated that no one would give us answers.

I’m pretty bright, and my husband is really clever, but it felt like we’d woken up in a dream world where everyone seemed to be speaking a different language to us.

“What’s this test for?” I asked.

“We’re checking his haemoglobin levels.”

“Why?”

“He can’t leave ICU until we see three consecutive normal results.”

“I meant why haemoglobin? Why is it so important?”

“There’s a liver transplant patient in surgery. We need to free up a bed.” I took a deep breath. The nursing staff had obviously been briefed to let the doctors do the talking.

“What is the normal range?” I tried.

“Around twelve, but we’d be happy with nine or ten for a cancer patient.”

Two days became three days and three days became a week. We still had no biopsy result. The medical staff still avoided answering our questions. Josh was still bringing up blood.

“You’ve got to stop vomiting,” a frustrated doctor told him. “Every time you vomit you damage the varices again, and they’ll never heal.” I was relieved that the doctor didn’t hear Joshua’s reply.

On day seven, Shannon and I sat numb and disbelieving on a bench under a sodden jacaranda tree as drops of rain permeated the canopy. That afternoon Shannon and I had been called to a bleak, windowless room. A woman we’d never met jumped right in.

“It isn’t great news. Your son has advanced cholangiocarcinoma. At least that’s what we are calling it for now. It’s definitely adenocarcinoma, but the pathologists are still fighting amongst themselves over where it started.”

“What does ‘not great’ mean?” Shannon asked.

“Let’s just say it’s like a ballerina losing her legs.”

“He has cancer in his legs?” I asked. Were they planning to amputate his legs? How did leg cancer make him vomit blood and what explained his weight loss?

“No,” she stifled a laugh. “He has cholangiocarcinoma.” She stood up and reached for the door. “I’ll leave you to tell…,” she looked down at her file, “Joshua. Remember, though, you can ask me anything you want.”

“Wait. Can you at least write down the name of the cancer?” Shannon asked.

“I can, but I wouldn’t spend any time with Doctor Google,” she said. She scribbled a word on a scrap of paper and walked away.

Over the coming days the entire paediatric oncology team seemed to time their rounds at the exact moment I’d gone to the bathroom or to the café downstairs to get coffee. Eventually, a neurologist friend of ours offered to step in and help us find out what was happening. She demanded that a member of the team sit down and explain the situation with her present so she could translate.

“Cholangiocarcinoma is so aggressive.” Our friend had done her homework. “Why haven’t you started treatment yet?” The oncologist avoided looking into her eyes.

“The multidisciplinary team has met. We’ve consulted with a paediatric hospital in the US and concluded that the distress of palliative treatments, will not be beneficial to Joshua’s quality of life.”

“You’re not going to do anything?” Our friend’s voice was incredulous.

“I am going to discharge you.” He turned to us. “This would be a good time to gather your family and friends to say goodbye.”

Cholangiocarcinoma is rare in adults, but it’s virtually unheard of in children or teens. No matter what the age though, a large portion of patients are diagnosed at stage 4 when organise (or blood vessels) begin to fail. The paediatric oncologists in our city had never had a bile duct patient before. This particular team had spectacularly low emotional intelligence, but like most bile duct cancer patients, we weren’t lucky enough to receive a diagnosis from an oncologist who had experience in this type of cancer.

I did what they told me. I took my child home to die. My friend wasn’t going to let that happen- not without consulting cholangiocarcinoma experts first. After several false starts and a lot of time convincing, we eventually secured an appointment with oncology professor. The appointment was scheduled for 9pm on a Friday night.

“We don’t treat children,” he said without looking up. Despite his words, he was still re-reading parts of the thick file of results and scans we’d accumulated and sent before the appointment. We collectively held our breath. The professor nibbled on a biscuit. He looked at Josh. He looked at Joshua’s file. Then he looked towards a youthful looking doctor beside him. She nodded. He shrugged.

“If you were seventy, I’d tell you to go home,” he said. “But you’re fifteen. We have to try. We’ll present your case to the multi-disciplinary team on Monday.”

Josh palliative chemotherapy began immediately afterwards. And I was lucky enough to stumble on the cholangiocarcinoma foundation’s mentor program to get advice.

What was your experience with your diagnosing team?

What you should remember:

  • Learn the lingo
  • BDC is rare, don’t assume the diagnosing team knows anything about the disease.
  • Get second (and third) opinions until you find a team you trust to treat bile duct cancer. Then do what they say. If you don’t trust your team, find a team that you do trust and do what they say.
  • In countries with larger populations, there are specialist units for cholangiocarcinoma in certain hospitals. Get more information here.

These are the words I needed in that first month.

Oesophageal and gastric varices

Varices are swollen blood vessel. They are caused by variety of illnesses. Left untreated the blood vessels begin to bleed and require emergency attention. Bleeding varices sometimes cause people to vomit blood or patients might notice their stools are black and foul smelling.

Oesophageal varices (ones that lie in the pipes between the mouth and stomach) can be treated by placing a tiny band over the bleed. Gastric varices (varices in the stomach itself) are rarely treated surgically. In this case, the oncologist may decide to prescribe blood pressure medication.

Gastro-endoscope

A gastro-endoscope is a camera at the end of a surgical tube. The doctor can use this to see inside the throat and stomach. Joshua’s were performed under general anaesthetic.

Intensivist

Intensivists are doctors who specialise in patients requiring intensive care. Their job is to stabilise the patient. They don’t usually diagnose or treat the underlying illness. Depending on the hospital, the patient is “handed over” to the intensivist when they enter ICU. When the patient returns to the ward, the oncology team will resume care.

FFP

FFP is fresh frozen plasma. Plasma is blood where all the red blood cells have been removed. FFP is used in ICU situations because there are less constraints regarding matching blood types. FFP is used to help blood clot. In the case of bleeding varices, it would also help the varices heal more quickly.

Central Line

This is an über drip. Instead of the drip site being in the hand or arm, the surgeon inserts it into a major artery (for Josh it was his femoral). It has multiple “ends” which allows the team to run multiple drips at the same time. Often one of the “ends” is left unused to allow nurses to take blood for sampling.

Glassy Eyes

Josh’s eyes were glassy because one of the drips running was morphine. He wasn’t expected to live more than a few months, so the doctors used opiates which is unusual in South Africa. South African doctors will nearly always only use opiates as a pain relief of last choice.

Weight Loss

Rapid weight loss is one of the most noticeable symptoms of BDC. If the patient’s liver is compromised, it makes it difficult to digest fats. That, in turn, makes it difficult for the patience to sustain or regain weight.

Transfusion

One of my biggest surprises was that transfusions are nothing like seen on TV. It will be different in different countries, but here they are a slow business.

  • First the patient will be tested for blood type (always, even if he was tested the day before)
  • The blood is ordered from the national blood service and can take up to 24 hours to arrive
  • The blood arrives frozen. The medical staff must defrost it slowly (you can’t just bang it in the microwave because, although cooked blood (black pudding) is a much loved Scottish breakfast, it isn’t any good for replacing lost blood)
  • When the blood is defrosted, it is infused via a central line or drip on its slowest setting. Blood is tested extensively before being added to a blood bank’s supply, but everyone’s blood is slightly different. The oncology team uses a slow running IV to protect the patient from any adverse reactions to the blood.

Liver Biopsy

In a biopsy, a surgeon removes some of the affected tissue. From there, it is sent to a pathologist lab where pathologists will diagnose the illness.

There are two kinds of biopsy commonly used for livers:

  • A needle biopsy is much less invasive for the patients as the surgeon uses a needle to collect a sample. It only provides a tiny sample of tissue, enough for diagnosis.
  • A surgical liver biopsy is a major surgery.  Livers can regrow (isn’t that cool?), so the medical team can afford to take quite a big “chunk.” Surgical biopsies are much more invasive and painful, but if the patient is healthy enough, they are preferable. It allows the surgeon to have a good look at the bile ducts and liver. Even more importantly, it allows them to collect enough tissue for second opinions (which you can read about here).

Joshua’s case was so rare that we ended up needing enough tissues for four sets of testing:

  • Two were done by the pathologists in Johannesburg. The first pathologist did not believe his own findings, so he sent them on to a second pathologist with no age information so that it could be confirmed.
  • A third test was performed by the Cleveland Clinic’s Second Opinion Service . They took all Joshua’s test information, re-diagnosed and sent a copy of its recommended treatment plan to compare with the one used by our own team.
  • A final test was done to identify whether Josh was a candidate for immunotherapy or targeted therapy
  • This is a further test that is strongly recommended for adult patients. The test identifies genetic mutations in the affected tissues. This is one key that can open the door to being a potential candidate for clinical trials.

Haemoglobin

This is the fancy term for red blood cells. Red blood cells are responsible for getting oxygen to every nook and cranny of the body. When there is an acute haemorrhage (bleed), there is simply not enough oxygen getting to where it should be in the body.

If bleeding varices are present, the medical team will use the haemoglobin level as proxy measure of whether the internal bleeding has slowed or stopped. Unfortunately, they will nearly always keep the patient in the ward until he/she has provided a stool sample that is not black, smelly and tar-like.

Adenocarcinoma

Cancers tend to be named by their point of origin- so “cholangio” means bile duct.
Cholangiocarcinoma falls into the adenocarcinoma family. This means that it is a cancer of mucus-secreting glands. There are so many different cancers in this family that one can’t say the survival rate is X.

Survival Rate

Survival rate in cancer is usually measured as the percentage of patients who live for five years or longer after diagnosis.

Portal vein

The vein that links the liver to the rest of the body’s organs.

Portal hypertension

This is high blood pressure caused by blood “backing up” behind a blocked portal vein. The patient experiences high blood pressure, but only in the portal region.

Portal vein thrombosis

A blood clot in the portal vein and usually the cause of portal hypertension. On its own, a portal vein thrombosis is not too serious – however, it is usually a sign of liver disease or cancer.

Palliative

Palliative treatments are treatments intended to reduce discomfort and extend the lives of patients. They are not dispensed as a cure.

Multidisciplinary meeting
(sometimes called tumour boards)

Most oncology teams have a periodical meeting (in our case weekly) where the medical oncologists, radiation oncologist, surgeons, radiologists and other specialists come together to discuss and agree treatment plans for new patients or changes to treatment plans for existing patients if they are required.

penny

Penny Castle ran a successful executive coaching business with a focus on using positive psychology techniques to help employees be happier for more than a decade. She suspended her practise in 2018 when her son was diagnosed with stage 4 cholangiocarcinoma.

 In January 2020, Penny re-opened her business to provide support for individuals suffering with complicated and anticipatory grief (including anxiety). Her clientele spans the globe which allows her to work online. She spends her free time with her husband, surviving son and a fleet of out-of-control pets. Most days you can find her working to help Reclaim Your Happiness here.