Starting chemo

On Sunday we took Asa back to London to begin chemotherapy.  The first doses of drugs were given on Monday, and we returned home late the following day.  During the stay in hospital we had an opportunity to reflect on the pros and cons of Asa's condition versus other kinds of cancers, and the treatment regimen he's on.

One thing was vividly impressed on us: Asa isn't the first or the last child to have cancer; his isn't the worst or the best case that's been seen; and the treatment regimen he's on isn't the most or the least radical that's been used.  In short, we gained a sense of the ordinariness of what, a week ago, seemed like an exotic diagnosis.  And the normality of a course of treatment that, after the doctors announced it a few days ago, seemed to make removal of his eyes look like an attractive option. 

"Everyone here has a Hickman"

A Hickman line is a port used for delivering drugs directly into veins, and for taking blood out of them.  It’s like a permanent IV – one that can be kept open for months or years, with a plastic stopper to cap it.  In Asa’s case the plastic tube enters his body on the left side of his torso, and snakes under his skin to the jugular vein in his neck, where it enters his bloodstream.

Asa's Hickman line, exposed during a change of dressings.

On our first evening in the hospital, Malachi, an 8-year-old boy whom we met outside the ward kitchen, pulled his shirt up to show us his Hickman – his 'wiggly', as the nurses call it – as if it was nothing at all. He looked very much like a normal 8-year-old boy, cautious around strangers, but with a brightness in his eyes that showed he was observing you carefully.  He had been on the ward for almost a month, receiving treatment for cancer.

Angela, a nurse who has seen dozens of children with cancer, noted that compared to leukaemia and other childhood cancers, retinoblatoma has a very high cure rate, and requires less radical treatment. 

Angela's take on retinoblastoma: 

"If you've got to have cancer as a child, this is the one to have."

A cure worse than the disease?

When we first thought about Asa's treatment options, systemic chemotherapy seemed like the least attractive choice.  The drugs are toxic – the technical term for them is cytotoxins, cell-killers – and the common side effects, including hair loss, impaired intestinal function, and erosion of the mucosa of the mouth, are directly related to their function of short-circuiting the growth of rapidly-developing cells throughout the body, including but not limited to cancers.

Samples of Asa's blood, and a syringe of Vincristine,
one of the three chemotherapy drugs he's taking.

Giving repeated doses of these drugs to children who are undergoing rapid development seems like madness, and a recipe for long-term health problems. 

Perhaps, in the future, it will be seen as madness.  But at the moment there are no other good options.  In Asa's case, the tumours in his eyes are too big to be treated any other way. If his eyes were removed, he would still be advised to take a protective course of chemo to mop up cancers that might be hanging out elsewhere in the body – and the dosage and duration would be the same as for the chemo that would have been prescribed first off. 

Rates of bilateral enucleation (removal of both eyes) after chemo are low these days, our doctor tells us – around 2% – so preemptive enucleation has nothing to recommend it.  And lasers and other more focused therapies just aren't adequate to the job – unless, that is, the tumours are shrunk by chemo first.

That, then, is the course of therapy he's getting: Chemo to do the tumour-shrinking, followed by focused therapies to attack the tumours when they're down.

Frequent blood tests are going to be necessary to track progress over the coming months. And the progress we expect to see is of a strange sort. Low blood counts are desired, because they are direct indications of the drugs' action.  At the same time, they will serve to show how Asa's body as a whole is being assailed by these toxins, with results being anemia, vulnerability to infections, and liability to spontaneous bleeding. 

A long course of treatment

One of the unpleasant implications of all this for Asa is that he will be a patient for much of his childhood – under close medical surveillance until 5 years and beyond.  For the next 6 months he will be spending more time in hospital than in nursery school.

The unpleasantness of this prospect is tempered by the fact that the medical system here in the UK is a particularly humane one. Regular home visits by community nurses will minimize the amount of time he has to spend in hospital.  And technological and clinical innovations make therapy less painful than it used to be.

The Hickman line is a case in point: On Monday night, I witnessed a nurse pushing a dose of anti-nausea drugs down Asa’s Hickman line while he slept.  If he didn’t have the Hickman, this would have required a painful injection, or the equally painful installation of an IV catheter.  As it was, Asa barely stirred as the drugs entered his veins.  Changing his nappy is now a more invasive procedure than administering intravenous drugs.

None of this makes the immediate challenge of helping Asa through chemotherapy any smaller.  Two days after the drugs entered his bloodstream, he is already clearly suffering.  As I’m writing this, at 3 AM on Thursday, Asa has been crying most of the night and with a new intensity; and despite frequent attempts to get him to eat, he’s accepted very little food since we returned from hospital.

Amidst all this we try to bear in mind that his suffering is less than that of other children. 

But it's scant consolation, faced with a child who's suffering, to reflect that other children suffer even more.

_____

A postscript on developmental effects of chemo in infancy: Dr Judith Kingston, the consultant managing Asa's care, has seen hundreds of children with cancer go through chemo, and attests that long-term effects on development are very rare.  The oldest children who went through this as infants are now in their 20s, so there's still a possibility of later-life problems.  But there’s reason to be hopeful.  If anyone has good information on this, please let us know.

Asa's status, and the way forward

Yesterday Selam, my mother, and I took Asa to London for assessment by retinoblastoma specialists.  In due course I’ll describe the health workers we met there.  Our first impressions are that they’re brilliant people who wear their knowledge and expertise lightly.  Based on the examinations they carried out and on what they told us, we’re now in a much better position to understand the stage of the disease and the treatment that’s necessary.

The stage of the disease

The International Classification for Intraocular Retinoblatoma grades severity on a scale from A to E.  A represents the best outcome, where chances of preserving vision are very good, and E represents the worst, where the eye must be removed to prevent metastasis. 

Asa is a D in both eyes.  There are multiple tumours in each eye, and they are large.  One indication of their size is that, while ocular tumours are conventionally described in millimeters, those in Asa’s eyes were described to us in centimeters: The largest in the left eye is 1.9 x 1.3 cm, and that in the right eye is 1.3 x 0.5 cm. 

The tumour in his right eye, while smaller, is more disadvantageously positioned, already impinging on the crucial area between the fovea and the optic nerve, and interfering with Asa’s sight.  The long-term chances of saving vision are therefore greater for the left eye than the right.

The left eye, however, is far from well: there are seeds – tiny pieces of tumour – that have already split off into the vitreous area of the eye, and should these grow, they would likely cause blindness in the left eye too.

Retcam image of left eye.  The mass at the bottom is the largest tumour; the spots and clouds above are subsidiary tumours.

Had the disease in each eye been fractionally worse, the doctors would have recommended removal of Asa’s eyes.  We’re grateful that they did not.  And we’re grateful too that there’s no evidence of the cancer having spread outside the eye.

What treatment do they have for this?

For children with bilateral retinoblastoma (cancer in both eyes) the standard treatment is systemic chemotherapy to shrink the tumours, accompanied by more focused therapies to attack them once they have shrunk. 

The first round of treatments will begin next week, and last about 5 months.  This includes 6 cycles of chemo, each lasting 3 weeks.  After every 2 cycles, he’ll be evaluated, and lasers or cryo may be used to attack the tumours. 

During the chemo, Asa is going to be delicate: We’re going to have to take special care to protect him from infection and to preserve his appetite.  His blood counts will need close monitoring, and if they plunge very low he may need blood transfusion.

A team effort

To pull all this off, we’ve been offered the help of a large team, including 

-- community nurses who will visit Asa at home each week to check blood counts and change the dressings on the shunt that will be used to funnel drugs into him;

-- paediatricians at our local hospital, whom we can call upon to manage any infections that Asa picks up along the way;

-- oncologists and ophthalmologists in London. 

We’ll also be relying on Asa’s own resilience and adaptability.  On love and support from friends and family.  And on God.

With help from these sources we pray Asa will overcome the cancer and retain his sight. 

There’s a long road ahead.

Asa's eyes

When Asa was 5 or 6 months old, Selam noticed something about his eyes.  Under certain light conditions, they would reflect light back, the way cat’s eyes do. 

“This child has animal’s eyes,” she said.

Neither Kuri nor I thought much of it.  It was amusing, we thought; a trick of the light; not a cause for alarm.

When I arrived in Ethiopia 3 days ago, Selam had just come across some information online that suggested this could be a sign of disease.  Whiteness at the back of the pupils is a characteristic of retinoblastoma, a cancer of the eye.  It’s rare, but more common in infants than adults.

Another sign of the disease is that in flash photographs with “red eye”, the eyes of affected children appear yellow or white rather than red. 

Going through old photos, Selam noted that this “yellow eye” phenomenon showed up for Asa in photos from 3 or 4 months ago.

The earliest photo of Asa showing "yellow eye". Note the contrast with Asa's companion Mekdas, who has red eye.

Yesterday morning we took Asa to an ophthalmologist in Addis Ababa who examined his eyes and corroborated the possibility of retinoblastoma.  In the afternoon he got an ultrasound, which confirmed the diagnosis.  Present in both eyes, it’s more advanced in the left eye than the right.

Left untreated, retinoblastoma can kill within 2 years.  Each year, 40 or 50 children are diagnosed in the UK, and 1 or 2 die.  As with other cancers, the prognosis depends largely on the stage of the disease.

In Asa’s case, we don’t yet know the stage of the disease.  A CT scan today should tell us more.  At the least, his vision is likely to be impaired.

But Asa shows no other signs of illness.  He looks and sounds like a normal, healthy child: inquisitive, playful, alert to everything around him. 

Within the last two days he’s made great progress in getting around on two feet, yesterday walking across the living room holding his mother’s hand. 

He’s begun to say, “Mama,” using the word to call Selam to him, or as a response when she says his name.

It’s remarkable to be told by a doctor that your child losing his sight is a good outcome.  It will take some time for us to see things from this perspective. Right now, scenarios worse than blindness are difficult to contemplate.

On Monday we’ll fly to England to seek treatment there.

We’re hoping and praying that Asa’s sight will be preserved.  And when we reflect on it, we recognize that we would love him just as much had he been born blind.