Giving thanks

 

Yesterday Asa had an exam under anaesthetic at the Royal London Hospital.

tearing up the waiting room

There was a long wait while he was in the operating theatre. And when Selam picked him up from the recovery room, his right eye was red and swollen.

           

Had the IAM not worked again? 

Perhaps they'd used cryo instead -- which causes swelling…?

A turn of events

When the doctors came around to debrief, they were upbeat:

"We're happy with the way he responded to the IAM," they said. 

The tumours they’d seen last time had "flattened out" since the second dose of IAM, they explained.

They had used cyrotherapy -- but that was to treat a small area on the roof of the eye.

Asa in regulation kimono

This is a huge relief.

As soon as a space is available on the list, they'll proceed with a third dose of IAM -- probably either next Wednesday or the following week.

Counting our blessings

Today Americans are celebrating Thanksgiving.

I’m in the US right now -- to attend an anthropology conference in San Francisco and an African studies conference in Philadelphia.

But today I’m spending time with relatives in Alabama.

I just skyped with Selam and Asa, and both of them looked well.

We have much to be thankful for!

One Rb World

 

Earlier this month, we took part in a conference on retinoblastoma in global perspective.

The One Rb World conference brought together people from Europe, North America, the Middle East, and several countries in Africa -- all working with Rb, or themselves touched by it.

The participants were split roughly half and half between medical professionals and parents of children with Rb or Rb survivors (people who themselves had Rb as children).

Most of the presentations during the 2 days of the conference were by doctors, but about half of the time was allotted for discussion, which gave space for testimonials and suggestions for improvements in the quality of care from parents and survivors.

More than once, mothers wept telling their stories -- of the scepticism they'd encountered when first seeking help; of delays in diagnosis, and doctors' egos getting in the way of efficient care.

One of the most memorable presentations was on the psychological effects of Rb treatment -- how the repeated exams and interventions, and the uncertainty about outcomes, might place children and parents at risk of syndromes like Post Traumatic Stress Disorder.

One family that participated, the Lloyds, gave powerful testimony about the psychological effects of Rb exams and treatment on their daughter Daisy.

The world seems a hostile place when you grow up being subjected to invasive medical exams on a monthly basis; and when your family are consumed by worry.

And yet the Lloyds haven’t just suffered in silence.

In 2004 they founded Daisy's Eye Cancer Fund, an NGO that now has chapters in the US, Canada, and Kenya, as well as the UK.

It was Daisy’s Fund that organized the conference.

Rb in Africa

There was a lot of attention to Africa at the conference, for a few reasons: 

 

• Africa is the area of the world with the greatest unmet need for Rb treatment. More than 70% of children with Rb in Africa die, compared with less than 5% in Europe and North America.
• There was a strong showing by Africans at the conference, and by people from elsewhere who work in Africa.
• Abby White, one of the founders of Daisy Fund, has family connections in Africa: her father was in the 4^th generation of a British family in Nairobi. (Diagnosed with Rb by a Kenyan doctor, he was very lucky to survive.)
• The Kenyan chapter of Daisy Fund is doing great work -- recently spearheading the development of a national strategy for Rb that could serve as a model for the region.

An Ethiopian connection

Ethiopia is immediately to the north of Kenya, and at the moment there is a small trickle of Rb patients from Ethiopia who make their way to Nairobi for treatment.

Through Abby we’ve made contact with several people in Ethiopia who are working on Rb, including families whose children have the disease.

Selam has been corresponding with some of the parents by email. Recently she wrote this to them:

[When Asa was first diagnosed] we had a chance to talk about cases in Ethiopia, and we were sad to hear that there is not much done for those children except enucleation (removal of the eye) to save their lives. Since we heard that, we think a lot about those children and families. We really are happy to be in contact with you so that we can discuss how we can contribute to the care of these children together. Personally I feel that there is a huge gap in awareness of Rb among health professionals and also the public, which contributes to late presentation and death of children. I would love it if you would share your thoughts with me and also on how we can work together to bring a change.

We're hopeful that our conversation with parents and doctors in Ethiopia will lead to improvement in the way Rb is dealt with there.

Asa's latest treatment

In her message to the parents in Ethiopia, Selam also shared our most recent news about Asa’s treatment:

Since [the end of systemic chemo in May] Asa has been on a monthly, scheduled eye examination under anaesthetic. Each time the doctors have examined him, they have seen new tumours, which they have been treating with cryotherapy (i.e with extreme cold).

About a month ago the doctors decided to use a different treatment because the small tumours in the right eye keep coming back. The treatment is called IAM (Intra Arterial Melphalan) and it is delivering the chemo drug directly into the eyeball through an artery in the eye (thankfully his left eye has been stable since May, i.e the end of chemo).

Last Wednesday [October 17] he had an eye examination and there were 5 new tumours in the right eye. The doctors want to give him a second round of IAM, at a higher dosage, and see the change in a month's time.

The second dose of IAM will be given this coming week, on Hallowe'en.

 

Asa on the day of his first dose of IAM. A doctor marked his forehead with an arrow -- to make sure the drugs were delivered to the right (i.e. correct) eye.

The One Rb World conference was audio-recorded, and the material should be available in 2013 through the Cure4Kids website.

Thanks again to everyone who has contributed to our fundraising for the Childhood Eye CancerTrust. Gifts have continued to come in after the event, including from some anonymous donors.

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.