Whipping out the wiggly

Last month, Asa celebrated his third birthday. On the same day, he started to write. He didn’t write much -- the words were dog and van. But if you saw him write them, you’d notice the pleasure he took in it -- in the alchemy of turning letters into living, moving things.

A little over a year ago, when his second birthday was approaching, he’d had a massive relapse. A week later he had a Hickman line inserted -- a sort of artificial umbilicus that provides easy access to the bloodstream -- in preparation for chemo. And although the chemo treatment lasted only four months, the line was left in, just in case more chemo, or radiation therapy, were called for.

Three weeks ago he had his Hickman line removed. The operation was carried out at Great Ormond Street Hospital, and it was fast. In a matter of hours we were on our way home again, and our little boy was no longer trailing rubber piping.

Naming the numbers on the hospital gown. 15 minutes later the wiggly was whipped out.

The absence of the Hickman line (or ‘wiggly’ as it’s often called) means Asa can start to do some things that children often enjoy, that have been off-limits to him for the last year. Like swimming. Or just splashing around in the bathtub. (The wiggly is vulnerable to infection, and has to be kept dry.)

And his dad doesn’t have to worry so much any more when he’s rough-housing.

More significantly, the doctors’ decision to remove the wiggly reflects some confidence that he may remain stable for a while.

A play therapist models breathing gas through a face mask before Asa’s most recent exam under anaesthetic.

The treatments Asa’s receiving now --  cryo and laser therapy -- are largely succeeding in keeping the tumours under control. Every three weeks, when he goes under anaesthetic, the doctors see new areas of activity; but for the past few months they have been small, and in parts of the eye that are easily accessible to treatment.

In comparison with the diagnosis that preceded his first birthday, and the relapse that overshadowed his second, this is good news indeed.

Little big man

 Raising a child with visual impairment makes you see the world differently.

At dinner with friends the other evening, I was astonished to see Asa’s best friend Angèle, who’s just a couple of months older than he is, watching TV from across the room.

What’s so strange about that?

Well, for Asa to see what was on the screen, he would have to stand within arm’s reach of the television set.

That had come to seem normal to me.

That may sound weird. But consider some of the other characteristics that we accept as natural for toddlers: short stature, primitive grammar, a predilection for tantrums. And their special, compensating features -- a mania for play; an exuberance that’s almost never found in adults.

The fact is, I’d gotten used to Asa not seeing as we do -- just like I’ve gotten used to him being smaller and livelier and having chubbier cheeks. And sometimes I forget that other children don’t necessarily share all of these traits.

The normalization of abnormality is part of a process of psychological adaptation.

Indeed, something like this must be going on for Asa himself.

If the scales were to fall away from his eyes tomorrow, he’d probably be very disoriented. The world would look strange and dazzling.

Like the people in Plato’scave, he’d probably prefer to keep the shades on.

For now, leaving the cave isn’t an option anyway.

What we’re trying to do instead is make little steps towards the light.

Bifocals

How are we going about that?

The latest thing we’re trying is bifocals.

Aren't those for old people? you may ask.

Usually, yes. But then again, so are cataracts.

In Asa's case, a cataract (a side-effect either of his cancer or the chemotherapy he’s received) effectively blinded him in his left eye until it was operated on in September.

In the cataract operation, the surgeon removed the natural lens of the left eye, which had become opaque.

This all seems to have worked out pretty well. But as a consequence he's left -- at least for the time being -- with quite poor vision in this lens-less left eye.

For the past few months, we've been patching his good eye periodically, to give the left eye practice -- or more precisely, to give his brain practice at dealing with input from it.

And to help the brain along, we’ve been fitting him out with spectacles with a very strong lens on the left side, to compensate for the short-sightedness.

The new bifocals will provide some close-up magnification as well, which may help him with some of his hobbies: writing, painting, and, um, watching The Tellytubbies on his mum’s phone.

Perhaps we should get him a pipe and tweed jacket to go with those bifocals.

He'll reach the ripe old age of three this week.

Discovering poetry

The child does not exist who, between the ages of 2 and 5, does not display a predilection for poetry.

I stumbled across this quotation yesterday in a notebook I'd kept a few years ago -- copied from a text by the Russian writer, Kornei Chukovsky.

It seems apt, because just this week Asa's started rhyming.

We were sitting on a sofa in our hotel in Birmingham on the night before his last medical exam when, à propos nothing, he came out with two words that rhymed.

"What about, head and bed?" I asked in response.

"What about, light and tight?" he rejoined.

We kept the game going for a while. And since then it's become a regular way of passing time.

The fact that children should invent (or discover) poetry as a matter of course, as they learn to speak, is amazing to me.

Of course, Asa's been exposed to rhymes -- in books like You Are My I Love You, and in songs.

But how readily he makes the idea his own!

And this is really part of a whole bundle of discoveries that children make around his age -- including music, dance, symbols and patterns.

For Selam and me, interest in these aspects of Asa's development is heightened by the fact that his vision remains problematic.

Perhaps we shouldn't be so concerned. Deaf children start to sign at around the same age as hearing children start to babble: Even with a sensory disability, there are certain important developmental processes that seem just to unfold anyway.

But so much of our experience of the world depends on sight, that we wonder what aspects of Asa's development might be set back, and we try to think of things we might do to make up for it.

Latest ups and downs

The past few months we've been through some ups and downs. (When hasn't that been the case?)

Two months ago, at one of his regular exams under anaesthetic in Birmingham, the doctors noted some tumour growth very close to the ciliary body -- a part of the eye where there's a lot of blood flow in and out. If tumours are active in this region of the eye, it's dangerous, because it means that cancer could spread through the bloodstream and take root elsewhere in the body.

During the following weeks I corresponded with doctors in the US, Canada, and Switzerland -- those we'd called upon at the last fork in the road, when radiotherapy had been recommended -- to get their opinions on what to do. The doctors in Birmingham had agreed that in a case like this (Asa is now among the perhaps 1% of retinoblastoma patients who do not respond to chemotherapy) it made sense to get a range of opinions.

It was a great relief when, at the following exam, the tumours near the ciliary body turned out to have responded well to local treatment. For the time being at least, radiotherapy is off the table again.

But we can't rest too easy. Last Friday Asa had his 22nd exam under anaesthetic, and although things are relatively stable, he's still never had a single exam at which new tumour growth of some kind or another hasn't been visible.

These are phenomenally active tumours, and if they weren't kept in check they'd doubtless be fatal.

Counting time

Asa’s disease, a cancer of the retina, affects children almost exclusively, and is most active during the part of the lifecourse when the eye does most of its developing -- from birth to five years old.

After five, the chances of new tumour growth declines dramatically. And the closer you get to five, the lower those chances get too.

In two weeks time, Asa will turn three.

So each month without a major relapse is a victory.

Each month that he sees is something to celebrate.

And in each day, there’s poetry.

5 pieces of advice on how (not) to talk about cancer

How we talk about illness can profoundly influence our experience of it.

Last week we picked up in a hospital waiting room a pamphlet called “Lost for words.”

It’s a practical and clearly written guide on “how to talk to someone with cancer.”

We wish we’d come across it earlier.

As parents of a child who has cancer, Selam and I often talk for him (Mercifully, Asa doesn’t yet understand his condition) and others talk to us as his supporters and carers.

Some misunderstandings that recur in our conversations are well addressed in the pamphlet.

http://be.macmillan.org.uk/be/p-251-lost-for-words-how-to-talk-to-someone-with-cancer.aspx

In lieu of sending it to everyone we know, we’ve taken from the pamphlet five lessons that seem important -- expressed here in our own words.

(Following the format of the guide, we write about how to talk to “a friend with cancer”; but the advice applies equally for those caring for someone with cancer.)

1. Don’t worry too much about exactly what to say.

When a friend has cancer, trust that voicing your concern / sympathy / desire to help -- and being there for them -- will be appreciated.

Too often, people obsess over getting the message just right.

This can lead to a self-imposed gag, and end up making the cancer patient feel all the more isolated.

There are, however, some things one can do to minimize the risk of clangers:

2. Before you put your own or someone else’s cancer story on the table, reflect on the similarities and differences. 

Some stories can be very valuable -- providing insights into new treatments to pursue, or ways to make sense of experience.

Other stories can raise false hopes, or create unnecessary fears.

3. Avoid over-confident or over-optimistic declarations.

Don’t say, “As long as you stay positive, it will all work out fine”!

Cancer doesn’t work that way.

It’s true that people who survive often attribute their survival to psychological or spiritual fortitude -- In the words of a charming young woman who had retinoblastoma as a child: “With a bit of Aunty Faith and Uncle Percy (perseverance), there’s nothing you can’t do.”  

But if a sufferer adopts this belief and then doesn’t get better, it can feel like a personal failure.

In such cases, the physical illness is compounded by feelings of guilt.

Sympathy and hopes or prayers for the best will almost always be better received than optimistic declarations.

4. When using humour, follow the lead of the person who’s suffering.
   

In the right moment, laughing about one’s predicament (or any facet of it) can be a most uplifting thing.

But don’t reel off your top 10 cancer jokes impromptu!

            Instead, take cues from the person who’s living with the condition.

           

5. Meditate on what it means to live with uncertainty.

In Birmingham last week we met a woman whose son, at nearly 4 years old, was having his 39^th examination under anaesthetic.

In the course of these many exams, they had seen progress and reversals.

“People never understand,” she said, “that if things look good at one exam, it doesn’t mean you’re out of the woods -- that you’re in remission.”

“There’s no telling what you’ll find next time.” 

Good news one day can be undone the next.

What cancer teaches us is how to live with uncertainty.

Every month, every week, every day of life lived free of suffering is something to celebrate.

Update

Asa’s exam last week produced very positive results: For the first time in 18 months, there was no new tumour growth to report.

We are profoundly grateful for this, and remain hopeful that the treatment will continue to produce good results.

We go back to Birmingham for another exam in 3 weeks time.

Another lesson

Since the last blog post, I've been to Ethiopia, we walked across London to raise awareness of eye cancer, and we moved house.

Things have been kind of busy.

But really the biggest news is that the treatment Asa's been receiving -- a combination of chemotherapy (using a single drug, Carboplatin) and aggressive cryotherapy -- seems to be working.

He has had two exams under anaesthetic in Birmingham since the treatment began, and the results have been more positive than we felt beforehand we could hope for.

At the Carrots Night Walk, with Cathy and Edith

The tumour load in the right eye has decreased to less than 10% of what it was before the start of this treatment.

In the doctor's words: “We're not there yet, but we're definitely headed in the right direction.”

In addition, Asa had a cataract operation at the end of September.

That went smoothly, and with the cataract out of the way it’s possible to show that there was very little new growth of the tumours in his left eye during the time that they’d been hidden from view -- more than 3 months.

No more chemo

The qualification to this catalogue of good news is that Asa had a severe allergic reaction to his last dose of chemo, two weeks ago, at Great Ormond Street Hospital.

We had just finished our lunch and were settling down for an afternoon in the out-patients' ward (the full dose takes 3 hours to infuse) when Asa suddenly became grouchy and sleepy-looking, and we lay him down on the hospital bed.

Within five minutes he was running a high fever, had vomited, and his blood pressure was plummeting -- heading into anaphylactic shock.

Thankfully the response from the medical team was almost immediate.

Within half an hour he was stable, and though he and Selam stayed in hospital for observation overnight, he was all right from there on.

The upshot, in any case, is that he'll receive no more chemo: No more Carboplatin because of his allergic reaction to that drug; and no more of the other drugs because he's already had as much of them as the doctors think he can tolerate.

Talking and reading

After this traumatic experience, Asa regressed for a few days, and stopped using his potty. (Did we mention he toilet-trained himself a few months ago?)

But that was a passing thing, and within a short while he was back to his normal routines, and chatting away happily to anyone who'd listen.

A typical conversational gambit for him is:  

“[The] 345 [bus] to South Kensington [goes by] King's College Hospital.”

Delivering a speech

Admittedly it’s not the best conversation-starter. But the enthusiasm with which he conveys this kind of information!

One of the things that strikes me about his language development is how many words he takes on board without having any notion of their real meaning.

What South means, for example, or King, or College.

Without knowing these things, phrases like this one nonetheless bind together memories -- in this case of an outing on the bus, and visiting King’s College Hospital for a flu jab.

A passion for symbols

This aspect of Asa’s language learning -- the ready use of words whose accepted meaning is obscure -- is surely common to all children.

More unusual, it seems to us, is his passion for numbers and letters.

He sees them everywhere:

• Two manhole covers next to each other in the park are an 8
  
• A piece of toast he ate at lunch yesterday became, at various points in the meal, an E, an F, an r and a 1.

Homework

The joy he takes in seeing things makes our latest piece of homework difficult.

We've been advised to patch his eyes on alternate days -- the right one, one day and the left one, the next -- to give his brain a chance to readjust to input from the left eye. (For 3 months before the recent cataract surgery the left eye provided little input at all.)

We started this on Saturday, patching the left eye -- which was no problem at all -- and yesterday we patched the right eye, leaving him with only the very impoverished vision from his left eye.

Chilling with one eye patched

Wearing glasses with a +12 lens for the left eye to compensate for liquid removed from his lens along with the cataract (and with the right eye patched), he is still very far-sighted: able to see things from across the room, but almost blind to things that are right in front of him.

We were unsure how he'd take to this -- Would he pull off the patch, and insist on using his ‘good eye’?

Remarkably, he didn’t. 

Instead, he tolerated the impaired vision all day, groping his way from room to room in the new house; occasionally bumping into a wall or a door, whereupon he’d reorient and take another tack; and generally taking it all in his stride.

Selam reported, after a short time that she spent wearing an eye patch to keep him company on Friday, that even with her 20:20 vision in the seeing-eye, she felt handicapped.

It’s yet another lesson in adaptability from our little tutor.

***

Thanks to everyone who has contributed to our fund-raising for eye cancer research.

 
http://www.justgiving.com/walkwithasa2

Blind for a day

My mum likes to say that we learn about our bodies the way we learn about cars -- each time something goes wrong, you get acquainted with a new branch of mechanics.

As various treatments have been tried out on Asa, we've learned more and more about cancer and the eye.

The graph below summarises the treatments Asa's received these past 18 months.

Notes: IAM = intra-arterial melphalan. Primary chemo = vincristine, etoposide, carboplatin.
Secondary chemo = topotecan, vincristine, doxorubicin. On diagnosis, both eyes were stage D
in the International Classification System.

Situations where retinoblastoma fails to respond to both primary and secondary chemo are rare, and even at one of the world's specialist treatment centres, a doctor might see such a case only once every few years.

 Support research on eye cancer here.

Right now we're in a place, therefore, where epidemiology and large trials have ceased to help much, and clinical judgment becomes very important.

As Dr Jenkinson -- the oncologist we met with in Birmingham -- said, "We're beyond the situation where there's a firm evidence base."

What's required then is very close attention to the details of the disease as it's manifested in Asa.


Tumour topography

"The retina is like the inside of an egg-cup," the ophthalmologist, Mr Parulekar, told us.

In Asa's case most of the tumours in the right eye are around the rim of the egg cup. Not all the way around, but covering approximately half of the circumference. In places, they extend down towards the bottom of the cup.

But the fact that the majority of the tumours are on the periphery of the retina -- and not way at the back or floating in the vitreous jelly of the eye -- means they may be treatable with cryotherapy.

Sometimes referred to as TTT or "triple freeze-thaw", this technique involves inserting a probe into the eye and freezing the tumours.

Support the development of improved treatments for eye cancer. 

Blind for a day

Asa had received cryo before, but never as much as he did on Friday.

When we picked him up from the recovery room, his eyes were shut tight and his right eye, which had been treated, was puffy.

On the way back to the train station, we passed a helicopter, which he would have been delighted to see.

Thankfully the air-ambulance was not there on our account.

But he kept his eyes closed for about 24 hours -- all through the journey home, and for most of the following day.

It was sad to see how isolated he was during this period of blindness.

But marvellous to see his excitement, the next day, after he opened his eyes.

In the playground, the day after cryo. Asa opened his right eye (the only one that's currently useful)
less than half an hour before Selam took this photo.

The new plan is to use more cryo, possibly accompanied by chemo, to try to shut down the tumours in the right eye.

So Friday's experience will likely be repeated in coming weeks.

 
Where will this lead?


Will the cryo will succeed in controlling the tumours? 

And, if chemo's required, which specific drugs might be used?

We don't know.

But at least it now seems firmly established that radiotherapy or enucleation are not the only options.

Asa has a fighting chance of maintaining his sight without recourse to a treatment which would significantly raise his risk of later cancers.

The treatments that are open to us are not, however, without their own risks.

• Aggressive cryo carries a risk of retinal detachment, which can impair vision.
   
  
• More chemo would mean increased risk of longterm side effects from the drugs (including hearing loss for carboplatin, and cardiac damage for doxorubicin).
  
  
• Operating on the cataract in Asa's left eye (about which more soon) would involve a small -- but real -- risk of the cancer disseminating beyond the eye.
   

More generally, after a year and a half of living from month to month, never knowing what might be uncovered at the next exam, we're entering a period of even greater uncertainty -- when the prognosis may change from week to week. 

Or, as it did this past week, from day to day.

 Please support our fundraising for eye cancer research. 

http://www.justgiving.com/walkwithasa2

New hope

In the past week, Selam and I sought advice from every channel open to us on what might be done to save Asa's sight and minimise collateral damage from treatment.

We made phone-calls and wrote emails; we read scientific papers about the long-term impacts of radiotherapy; and on Sunday we went to our local Quaker meeting and asked for guidance on how to proceed.

To recap, Asa's predicament is as follows: He has had a relapse in his right eye, and a cataract has deprived him of sight in his left eye.

The choice we'd been offered was between radiation therapy, with the corollary of increased risk of other cancers in adulthood, or removing both eyes.

But the emailing and phone-calling paid off.

The experts we'd consulted -- in the USA, Canada, and Switzerland -- didn't feel that radiation was necessary, and on that basis the London team recommended a full review in Birmingham.

Asa was examined at Birmingham Children's Hospital -- the UK's other dedicated Rb centre -- on Friday.

And yesterday morning we were able to go to the Quaker meeting again, and share our joy: That a new set of possibilities had opened up in terms of treatment.

While much uncertainty remains, the choice before us is no longer so stark.

In short, there's new reason for hope.

Thank you to everyone who offered us advice, encouragement, or prayers.

On September 20th, Selam, Jed, and Asa will walk across London at night in support of eye cancer research. Please sponsor them here.