The age of yes

 

Last week Asa had a spurt of language development. 

Since about 11 months old, he's gotten by with only a couple of words, mostly Mama and Dada (though he understands far more).

This past week he more than doubled his repertoire, adding Yes (and its Amharic equivalent, Awo), No, and Oh dear.

Of these words, the one he uses most is Yes.

Ask him anything -- say anything with rising intonation at the end of the phrase -- and "Yeh" is what he'll most likely say in response (often followed by laughter).

Folk knowledge and parenting books have prepared us for the Terrible Twos, an age when "No" becomes children's favourite word, and they refuse to cooperate in any plans they haven't hatched themselves. 

Asa won't be two for another 4 or 5 months, and hopefully he'll skip the terrible part. In any case we're enjoying his willingness to go along with our plans for the time being.

His tolerance was clear last Friday, on our night walk across London.

I was struck by it as we were crossing Lambeth Bridge.

Wind and rain were buffeting us. But Asa sat contentedly in his buggy -- looking out through a visor studded with raindrops at a world of blurry, refracted streetlights and darkness.

As long as there was food in his belly; he wasn't too hot or cold; and we were there, I figured, the world would be all right by him.

A long walk

The walk turned out to be more of an adventure than we'd expected.

We'd taken the wrong bus from Peckham, and reached Waterloo after the other walkers had left. Hurrying to catch up with them, we were handed a map, oriented briefly, and set off.

En route, opposite Westminster

It wasn't until we got to the first checkpoint, where shelter and refreshments were meant to be available, that we realised something was awry.

Finding the place closed and locked, we called back to base. 

We were in the wrong place. 

It turned out that we'd been given the 15-mile map rather than the 6-mile one.

There were two walks happening that night, the longer one looping around South Kensington, and the shorter one confined to Trafalgar Square, the City, and the South Bank.

We'd gotten most of the way to South Ken before we realized what had happened.

By the time we got back to Waterloo, most of the other 6-milers had already finished and gone home. 

And the 15-milers were just setting off; we caught sight of their backs as they headed off towards South Ken with a whoop.

Some wore orange wigs, and one or two were dressed as carrots.

A word for our sponsors

It would have been nice to have gone with the rest of the crowd, but the three of us enjoyed the walk anyway. 

We got outside of our usual tramping grounds, saw some new parts of the city, and had some quiet time to ourselves.

And in the process we raised over a thousand pounds for retinoblastoma research.

Many thanks to everyone who sponsored us!

More soon on Asa's IAM procedure, which went smoothly.

The website for donations for eye cancer research remains open: http://www.justgiving.com/walk-with-asa

A new strategy

Last Wednesday’s visit to the hospital was the first time Asa has shown distress when he’s seen doctors and nurses. 

As soon as a nurse began to escort us towards the place where his vision was going to be assessed, he started to cry -- anticipating what was coming (stinging eye drops, anaesthetic gas, soreness…).

An exam under anaesthetic (EUA) later that day showed that although his left eye is stable, there is continued tumour activity in his right eye. 

This is pretty much the same story as for each of our EUAs since the end of systemic chemotherapy.

Up until now, the new tumours seemed to be treatable with cryotherapy. But this time the previous session’s cryo didn’t seem to have worked so well. “It  isn't controlling things enough," the ophthalmic surgeon Mr Sagoo told us. 

“We have to think of another strategy.”

I.A.M.

The possibility that Asa might receive IAM was mooted back in July, and since then the doctors have debated whether or not it was warranted.

IAM -- intra-arterial melphalan -- involves infusing a chemotherapy drug (melphalan) directly into the eye. It avoids some of the nasty side-effects that accompany whole-body chemo. It also means a higher dosage of the drug can be aimed at the eye.

Possible side-effects are squint, drooping of the eyelid, and dilation of the pupil. Usually these are transitory, but in some cases melphalan can damage the retina and lead to long-term loss of vision.

The long-term effects are difficult to know because the procedure is so new. First developed in New York in 2006, it has been performed in the UK since 2008.

Despite all this, the balance of risks and benefits is better for IAM than for the alternative, which is radiotherapy.

It will be done on Wednesday, September 19th, at Great Ormond Street Hospital.

Walk with Asa

Two days after the IAM, Selam, Asa, and I will walk across London at night to raise money for retinoblastoma research.

We’ll be walking together with other families affected by eye disease.

The money we raise will go directly to the Childhood Eye Cancer Trust (CHECT) -- an organization that supports research on more effective treatments for Rb.

Please consider sponsoring us. To find out more, or donate, see:

www.justgiving.com/walk-with-asa

Asa looking at butterflies

Advice for surviving chemo

Have you ever had the experience of coming up for air after working hard on something for a long time, and being surprised to find that other people have been going about life as normal?

That’s what it’s been like finishing chemo.

During chemo, the thought of taking Asa to a nursery, or on a train (crowded environments where he could pick up an infection) would have made us giddy.

Now, we can go out like normal families do, and there's rarely a day when we don't take Asa on an excursion by car, bus, or train.

On an outing in Frinton

Before the memories fade, we'd like to share a few things that might be useful for other families with children going through chemo.

Some tips

In no particular order, these were things we found useful for keeping Asa clean or safe:

 

•  a play pen, to keep him in a dirt-free space
  
  
• grapeseed oil & cotton balls for wiping his bottom during nappy changes (much less likely to irritate skin than wet-wipes)
  
  
• an in-ear thermometer to check temperature (quicker and more convenient than oral or under-arm thermometers) 
  
  
• clorhexadine mouthwash, applied with a foam 'lollipop' (to stop mouth ulcers from turning ugly)
  
  
• hand sanitizer for your hands and baby’s – plus his toys, the floor, etc. … (a supplement to frequent handwashing) 
  
  
• maintaining a “no shoes” policy in the room/s where baby spends most time
  
  
• vitamin syrup (even if baby's not eating well, you can at least ensure he's getting vitamins and minerals this way)*

Some other things helped keep us sane during the months when we were more or less under quarantine:

• watching movies, especially ones that could be easily interrupted and returned to without losing the plot (nature documentaries were good for this)
  
  
• taking up new hobbies – crosswords, crochet, sudoku, jigsaw puzzles, guitar, etc. (to while away time indoors / in hospital).
  
  
• getting counseling.  We were connected to a counselor through the Retinoblastoma Service at the Royal London. This was a tremendous help in terms of emotional support, dealing with the depression and uncertainty that followed the diagnosis and the stress of chemo.

We also had to stay on top of a complicated and intense set of duties. At one point, we were giving 5 different medicines per day – anti-nausea, anti-pain, antibiotics, mineral supplements, and so on – each on a different schedule.  Keeping a notebook for recording doses of medicines we were giving was crucial – especially since the times when the drugs were most needed were the times when we were most likely to be sleep deprived and stressed out.

Asa's pharmacopoeia

These days Asa’s making great progress – he eats, romps around, laughs heartily when we laugh, cries when he's feeling neglected, and generally acts like an ordinary toddler.

But the job (or battle, or game – choose your own metaphor!) is not over yet.  

Today he had cryotherapy in London, and the news is that he will undergo IAM  (intra-arterial melphalan – a procedure that will deliver chemotherapy directly to the right eye)  next month. 

More on that later.

We're grateful, at least, to have made it through this stage.

 

____________________________

* certain vitamin / mineral supplements are contraindicated for children taking chemo drugs for leukaemia, so check with your doctor before using them if this applies to you.

Please let us know if you have suggestions for other things that could be useful for getting children through tough times like this!

How Asa sees

We got three new pieces of information this week – about Asa’s vision, the state of the tumours in his eyes, and the genetic basis of his condition.

1. How Asa sees

On Tuesday afternoon, an orthoptist tested Asa’s vision. She waved toys in front of him to assess how well he could track moving targets, and used a set of cards with images of varying clarity printed either on the left or right, or top or bottom, to see whether he could discriminate between image and blank space.

Taken together with what we know from other examinations, the results suggest that Asa’s vision is worse than we’d supposed based on his everyday behavior.

In the right eye he probably has good vision only on the periphery, and in the left eye his vision is probably blurry.

The reason is that the right eye has a centrally located tumour, and in the left eye – while the tumours are located lower down, affording a decent visual field – what he sees is probably blurred on account of retinal detachment.

Squint with your left eye and hold your fist about 6 inches in front of your right eye. That’s probably roughly what Asa can see.

But now imagine that this is all you’ve ever been able to see. That this is what the world has always looked like.

Then this way of seeing ceases to seem so poor.

By combining two deficient signals, and using them to the max, Asa seems to achieve something equivalent to what one good eye could do.

His ability to navigate – walking around, exploring – is good on account of the peripheral vision in his right eye, while his ability to manipulate things directly in front of him, make eye contact and so on, relies mainly on the central vision in his left eye.

He carries this off so well that we’d come to doubt that he was really visually impaired.

2. State of the tumours

The next morning Asa’s eyes were examined under general anaesthetic.

In the left eye, there was no change since last time. But two small tumours in the right eye have shown new growth over the past 6 weeks.

These were treated with cryotherapy – a freezing probe directed precisely at these spots. The cryo needs repeating to be effective, so Asa will be back for more in 3 weeks time.

3. Genetics

Results of the genetic test confirm that Asa has a change in the gene that regulates retina development. It's a strong change – a “fully penetrant mutation” – of the sort that almost always results in bilateral retinoblastoma.

This is what we expected.

The good news is that neighbouring genes are OK.  Asa’s not at risk of any other developmental abnormality.

Blood was also taken from Selam and me for testing, to check whether we have changes in our RB genes that could lead to a risk of retinoblastoma in other children we might have in future. These results will take a couple of months.

Round up

What we’ve learned will take a bit of digesting. But we have a much clearer understanding now of the state of Asa’s eyes. What comes with this is a heightened concern that he should retain what he has.

If there’s deterioration in either the peripheral vision in his right eye or the central vision in his left, he will likely end up much more compromised.

That said, some things we knew already bear repeating:

With the monitoring and treatment that Asa will receive over the coming years, this disease is very unlikely to endanger his life. The worst case scenario is blindness.

The period of the greatest danger in terms of tumour activity is from birth to 3 years. From 4-5 years, there’s a continued risk. After 5 years the risk decreases greatly.

Until that window closes, we won’t know what the long-term prognosis is, in terms of his sight. From month to month there may be changes, and it’s unpredictable. So we have to keep watching, and respond to problems as they arise.

The end of chemo

Asa's last dose of chemo was given 6 weeks ago, and he's now largely recovered from the side effects -- he's comfortable, his appetite has returned, and little hairs are sprouting all over his scalp.

I’ve been remiss in sharing this news, in part because as soon as the chemo finished, Asa developed chicken-pox, and we were thrown back into emergency mode.

He broke out in spots on May 28, and at that point, his immune system was still compromised, and we were afraid the chicken-pox was going to be unusually severe.

On doctors' advice, Asa was kept in hospital for 10 days on IV Aciclovir, a drug that slows the reproduction of the virus. 

In the event, the chicken-pox was mild, and the spots didn't seem to cause him much pain.

There was still some systemic imbalance that needed to be sorted out afterwards: Until last week, his potassium levels were still low, and we continued giving him supplements through his NG tube.

But by last Saturday, he had normalized, and we pulled out the NG tube.

Asa, a few days before his NG tube was removed.

Perhaps that would have been the appropriate time to have thrown a party.

It's difficult to describe what a relief it is not to be giving Asa medicine, after 5 months when he rarely went without at least one or two doses of some drug or another each day.

What next?

Many people would like to know whether the chemo worked. 

We may have partial answers within the next couple of days.  

This afternoon we travel to London, where Asa will have two eye exams -- one a vision test at which he's presented with various stimuli and the doctor tries to evaluate how well he sees; and another, tomorrow, when his eyes are examined while he's under anaesthetic.

We have to think about these tests not as the final exams after chemo, but as the first after chemo. 

The reason is that, even if the tumours appear quiescent, there's still a chance they could reactivate in future.

Asa will need exams under anaesthetic roughly on a monthly basis from now until he's about 5 years old (when the risk of new tumour activity decreases).

The prognosis we'll get tomorrow, then, probably won't be for the long term, but "for now".

Thank you!

This is a good time to acknowledge our gratitude for the people and institutions that have helped us get through the last 5 months.  A short and certainly not exhaustive list must include:

1.    Selam – who first noticed the signs of retinoblastoma, and sounded the alarm, and who has done more than anyone to bring Asa this far

2.     The National Health Service – which has given us access to excellent medical care, from specialists to community nurses who visit our home every week

3.    The grandparents, especially Kay and Clive, who have provided support to us all throughout

4.    Other friends and family who have kept our spirits up through the difficult times.

Thank you all!

A few words from Asa

It’s partly a testament to the contributions of all these people, and partly due to his own resilience, that Asa's been developing normally though all of this.

Right now he has a receptive vocabulary larger than we probably realise. He knows the words -- in Amharic or English or babytalk -- for breast ("tuut"), blender ("Nnnnngggg!"), hand-washing ("water-play"), piggy-back ("che-che"), "up", and "down". 

The comprehensible utterances he produces are limited to daddy ("Adada"), mummy ("Mama", which for some reason he says much less commonly), and "There!"

Beyond this, he is a great mimic, imitating sounds -- the noises of the washing machine, cars, coughs and sneezes, and the melodies of familiar songs ("This old man…" and "Twinkle, twinkle, little star") -- and actions like eating, scrubbing, and sweeping: things he’s seen us do a lot.

For a long time Asa was a slacker in terms of chewing his food, but he's making progress on that front now, and in the last few days he's chewed and swallowed bread and raisins.  Biltong next.

Sick and visually impaired?

Recently I caught sight of Asa, and the way the light was falling on him made him look to me, for the first time, like the sort of children we sometimes see in hospital waiting rooms – Completely bald from chemotherapy.  And unwell.

This struck me, because for the most part, Asa doesn’t seem sick to us these days.  He’s so lively, so playful and happy that we often forget about his illness.

Of course, his hair has almost disappeared.  He sometimes looks pale. And he still sometimes needs an NG tube to get his medicine in.  But that’s about where the resemblance to a sick child ends.

Asa & granddad Clive

Sometimes friends ask how Asa is doing, saying “We heard he’s very sick.”  

It can produce an odd look from us, because it’s both true and untrue.

The end of chemo

During the 5th cycle of chemo, Asa was on a lower dosage (50% Vincristine), and the side effects were milder.  His appetite declined early on, but soon he was eating more or less normally again, and his energy levels were high.  He had none of the discomfort we’d seen during the 1st and 4th cycles. He needed a transfusion of platelets in the second week, but his haemoglobin level remained in the normal range throughout.

As the cycles of chemo have progressed, we’ve gotten more relaxed.  Whereas a couple of months ago we were keeping him confined to a few designated safe areas in the house, these days he roams widely (most of the time, on two feet), and he’s made the place his own.

Tomorrow, if all goes well, Asa will receive his last dose of chemo. 

It will take about 3 weeks to work its way out of his system.  After that, we hope we may be able to relax even more.

The latest on Asa’s eyes

The latest news we have on the cancer, from an examination 3 weeks ago, is this:

The large tumours in both eyes are inactive, and in the process of becoming calcified. 

But there are 50 to 100 seeds (small, beginnings of tumours) in the retinas of each eye; and in the left eye there are 2 vitreous seeds – seeds outside the retina.

Whereas before chemo both retinas were detached, the retina of the right eye has now become attached again.

Sporting shades after an eye exam, April 2012

The prospects of the retina re-attaching in the left eye are lower – As a doctor told us, if the retina hasn’t attached by the 4^th cycle of chemo, it’s unlikely to do so afterwards. 

This is important for a couple of reasons.  For one, a detached retina impairs vision.  For another, a class of therapies including laser and cryo aren’t possible if the retina’s detached (they can detach it even further).

Because the retina in the right eye has re-attached, laser therapy could be performed on the subretinal seeds in the right eye at Asa’s last session.  But nothing was done to the left eye.

So what are the options for the left eye? 

If either the seeds or the large tumours in the left eye become active again, then the first recourse might be intra-arterial chemotherapy (intra-arterial melphalan, or IAM), delivered directly to the eyeball rather than to the whole body.

And although the doctors haven’t mentioned it recently, there remains a possibility that if the therapies don’t work, the eye might need to be removed.

We hope and pray that won’t be necessary. 

Implications for vision

What does all this mean for Asa’s vision at present? 

With the retina detached in the left eye, the doctors infer that Asa can only see “light and dark” out of that eye.  And because of the size and position of the tumours in the right eye, they assume he has only limited vision there. 

But there’s a weird disconnect between the doctors’ judgments, based on examining Asa’s eyes under anaesthetic, and our observations of his behaviour from day to day.

First of all, he gets around, makes eye contact, snatches things from us, and generally behaves just like any other child.  There’s no obvious visual impairment at all.

Second, when Selam breastfeeds him, Asa often has one eye at least partially covered, as he lies sideways on her lap.  In this position, she’s able to test his vision in each eye by proffering things (her necklace, a mobile phone, and so on) and seeing whether he reaches for them. 

Selam’s impression, based on many such tests, is that Asa sees better with the left eye than his right.  He’s more likely to reach for stuff when his right eye is covered than when his left eye is. 

Which is precisely the opposite of what we’d expect from what the doctors tell us.

One interpretation of this is that the “light and dark” he gets from the left eye is better even than the partial vision he gets through the right eye (where the tumours are more centrally located). 

Since he doesn’t cooperate when people try to patch one eye (he protests and tries to rip the patch off), it’s difficult to test him formally.

But he’s clearly using what vision he has to the max.

Next month, after the last cycle of chemo is finished, we’ll have a follow-up vision test at the Royal London Hospital.  Perhaps then we’ll get a better sense of how well he’s seeing, with each eye. 

For the moment, it remains a daily wonder to see him roaming and playing, and defying anyone to call him sick or visually impaired.