Respite

We had an important meeting with Asa's clinical team today in London.

The conclusion:

Both radiotherapy and enucleation are off the agenda -- at least for the short term.

We're to travel to Birmingham for a second opinion within the next 2 weeks, but it looks like more chemo and laser/cryo are on the cards.

We'll soon provide a fuller update. 

In the meantime, Hallelujah.

A hard decision

This week's EUA didn't turn out the way we'd hoped. 

"The right eye has relapsed," the doctors told us after they examined Asa.

"Areas we've treated many times with cryo and with chemo, … last time they looked stable and avascular. 

Today they're growing.

More cryo is not the answer. We've done a lot of it, and it's not controlling things.

Second-line chemo held things at bay, but also in the longer term is not working.

I'm afraid we are recommending radiotherapy.

We don't have another option."

Radiotherapy

In the past months we've thought about radiotherapy -- it was clear that it would be on the cards if another relapse occurred -- and we tried to prepare ourselves psychologically.

Last week I spoke with a friend who received radiotherapy for Rb as a child, and asked her how she felt about the treatment -- its effectiveness in attacking her tumours, versus the side effects.

When she'd been treated, radiotherapy was the only treatment for Rb -- that or enucleation.

"Increased risk of second cancers is the main concern," she'd said immediately.

Radiotherapy is very effective at killing active tumours but it also damages other tissues, raising the risk that other cancers will develop, later in life.

The key questions are, By how much is this risk raised?

And how much sight is the treatment likely to preserve?

There's no point in giving radiotherapy -- and incurring that extra risk of later cancer -- to eyes that won't provide useful vision.

 

Asa's vision

As anyone who's spent much time with Asa can attest, he has very useful vision.

Just the other weekend, when we visited the London Aquarium, he was running around confidently in its dimly lit rooms and tunnels.

At the London Aquarium, July 6, 2013

But we realise now more clearly than ever before that he's in danger of losing his sight completely.

On Wednesday, the doctors for the first time raised enucleation as a possibility for the left eye, because they can't tell what's going on behind the cataract.

Ultrasound provides some information on what's going on there, but only on a gross scale -- "we can't see subtle changes," they said.

Let's consider in more detail, then, what radiotherapy involves.

 

What we know about radiotherapy

What we know about the treatment that's been recommended is this:

• It's external beam radiotherapy (a beam of radiation directed at the eyes from outside).
  
  Since the tumours in Asa's eyes are diffuse, they can't use the gentler forms of radiotherapy; instead they would have to treat the whole of both eyes.
  
  
• It's given under general anaesthetic, with a mask to immobilise the face.
  
  This stops the patient moving around, which would decrease precision.
   

  

  A mesh template for a mask used in radiotherapy.
  Image from the Macmillan website

   

   

   

   

  
  




• The course of treatment is usually one month.
  
  Treatments would be given from Monday to Friday, with a break on weekends -- i.e. 20 sessions in total.
  
  
• And, unlike with chemo, it's very rare to carry out further courses of radiotherapy after the  initial course.
  
  It either works or it doesn't.

What we don't know

There is of course a lot we don't know about radiotherapy.

We’ve heard that whole-eye radiotherapy sometimes affects the lacrimal gland (the tear duct), producing a debilitating "dry eye" syndrome that may be permanent.

How likely is it that Asa would suffer that?

And how high is the risk of second cancers?

The answer to this question depends in part on 

• how much long-term follow-up has been done
  
• and how much of that long-term follow-up involves children who've received equivalent doses of radiation, with equivalent precision, to what Asa would be getting. 

The doctors at the Royal London deflected some of our questions on Wednesday, encouraging us to ask the radiotherapists at Barts who carry out the treatments.

If anyone has relevant information on any of these questions, we'd be grateful if you would contact us.

 

A hard decision

What the choice seems to boil down to is this:

Certain blindness (double enucleation), with a lower risk of later-life suffering, or possible sight (radiotherapy), with a higher risk of later-life suffering.

At the moment it’s very difficult to evaluate those risks.

As the doctors are wont to say, "We don't have a crystal ball" to see the future.

 

Walk with Asa

In September we will again walk across London at night, to raise money for retinoblastoma research.

We do this in hope:

• that research may identify better treatment options than what’s available right now;
  
• that other families may be spared the hard decisions we have to make;
  
• and that other children with retinoblastoma may be spared Asa's suffering.

We invite you to support us here:

http://www.justgiving.com/walkwithasa2 

Reassurance

Yesterday I wrote to our doctor to ask what the neuro specialists had said at the Multi-Disciplinary Meeting, on viewing Asa's MRI scans.

Did the meeting throw up any new information on what might be behind the head pain? 

And what was the implication of the gliosis that had showed up in the MRI?

The doctor's reply:

No - the scan was not thought to show any cause for his head pain – the area of gliosis is very small and non-specific and is probably related to minor trauma ?during delivery and is definitely not the cause of his pain.

One of the nurse practitioners wondered if the pain could be a manifestation of migraine which is sometime seen in 2-3 year olds.  However, we will check the IO [intra-ocular] pressure in the left eye at his EUA to rule out glaucoma first.

We're greatly reassured by this.

The question of what's causing Asa's head pain remains, but we're steadily eliminating some of the scarier possibilities. 

The next EUA is on Wednesday, 17 July.

For now, we're making the most of our freedom -- and a recent spell of good weather.

On the Millennium Bridge, July 10, 2013

Relief

We don't like to add unnecessarily to the sum of anxiety in the world.

So it's with both humility and gratitude that we confess our fears about brain tumours were unfounded.

I quote here from the email we received from our doctor on Friday night, summarising the scan results:

From the report and my untutored eye there is nothing on the scan to account for Isaac’s symptoms [i.e. head pain] – the report mentions a small area of scarring (gliosis) on the right side  but I am sure that this is not causing any pain - I will discuss the scans at the neuro-oncology MDT [multi-disciplinary team meeting] on Tuesday and get back to you if anyone has any further thoughts on the problem.

It's worth noting that although the doctor describes herself as "untutored" in interpreting MRI's, she does in fact have 30 years of experience in treating retinoblastoma. (She is a self-effacing but extremely competent consultant.)

Although not a specialist in neurooncology, she would surely recognise the kind of brain tumours that are most commonly associated with Rb if she saw them.

Further questions

Obviously there are questions that remain.

• What might have caused the scarring -- the gliosis -- that was mentioned in the MRI report? 

• If the scarring doesn't account for the head pain Asa's had, could it have other implications?

• And if there's nothing in the MRI to account for the head pain, what is then behind it?

These things we need more information on.

Focusing on the positive

The main thing to focus on for now is that the scan provides no evidence of brain tumours.

That, our greatest fear, is ruled out.

For now everything is cool

Asa's third summer

We ended with a cliffhanger last time, describing 3 possible outcomes of Asa's last EUA.

As it was, the results of the EUA were much better than we'd feared, but not quite as good as we'd hoped.

The tumours in Asa's eyes appear to be stable.

They didn't shrink much as a result of the second-line chemo, but nor did they grow.

That puts the results somewhere between options 1 and 2 in terms of the scenarios we'd anticipated -- No dramatic response to chemo, but no new treatment required, at least for the moment.

Looking for signs

One encouraging point is that the ophthalmologist who examined Asa under anaesthetic didn't see any blood vessels inside the tumours -- blood supply being one of the things that would keep them growing.

So it's possible that despite the lack of shrinkage, they may have had the life taken out of them.

The cataract

The cataract in Asa's left eye, however, has definitely gotten worse.

This may be a result of retinal detachment, which could be due to the tumours (the retina was already detached at diagnosis, 18 months ago) or to the treatments he's received.

Because the cataract obscures much of Asa's left eye from the doctors' view even when he's under anaesthetic, we had to visit Moorfields -- London's specialist eye hospital -- for a high-resolution ultrasound, a way of "seeing through" the cataract.

The ultrasound seemed to be consistent with the initial EUA results: The tumours looked no bigger or smaller than they were before chemo, and there's some evidence that they're calcified.

Summer holidays

In the 2 weeks since the exams, we've enjoyed ourselves.

We spent a weekend with the grandparents in Essex, and last week we took a trip to Liverpool, where I was attending a conference (which I blogged about here).

Fulfilling a wish of Selam and grandma Kuri, Asa visited Anfield Stadium, home of Liverpool FC.

Our warrior in Liverpool

An audio recording of 30,000 people singing "You'll Never Walk Alone," played over the stadium tannoy -- a sound rather like a rising and falling wind -- made an impression on Asa, and he's since been imitating it, singing to himself.

Another exam

Tomorrow Asa has an MR (magnetic resonance) scan at Great Ormond Street, to check out some recurring head pain.

For several weeks now he'll be playing happily, or just hanging out with us, and all of a sudden he'll grab his head and cry.

This began during the latter part of chemo, and at first we assumed it was a side-effect of the drugs, which can cause discomfort and pain.

But it's persisted long since the chemo drugs left his system.

The scan might shed some light on this.

Our worst fear is that a brain tumour could be to blame. We've hardly spoken of this, because it's such a hard thing to contemplate.

The results, which should be available by Thursday, will, we pray, put those fears to rest.

Speaking and seeing

This week I got back to London after two months in Congo.

During the first weeks away I was able to talk with Selam by phone; after that we communicated mostly by text message. 

Asa didn't do all that well on the phone (when we'd talk, he'd often just laugh), but on my return I've discovered that he's made amazing progress in speaking.

His vocabulary reflects common preoccupations of children with animals (dog, cat, bunny, bear, and ait [Amharic for rat]) and vehicles (car, truck, plane, tractor, bus, and train). These things recur in his picture books and in toys; some of them he can also see through our front-room window, or on walks around the neighbourhood.

A lion driving a car... Asa digs this book.

Other words reflect experiences that are less common in childhood.

"Chemo", for instance, Asa uses to refer to a drip set. (He used the word when he last had a blood transfusion.)

And “ambulance” means something different to him than to many kids, because he’s used to riding to hospital in them.

A conversation

Yesterday, Selam and Asa were still in bed when I was about to head out the door, and I crawled over to them to say goodbye.

Asa opened his eyes and looked at my clothes.

"Yellow. Jacket."

"Yes, Daddy's wearing a yellow jacket," I said. "I'm going to ride my bike."

"Bike," he said. "Motorbike."

"Not a motorbike, just a bike," Selam corrected.

"Out?" Asa said.

"Yes, I'm going to work."

"Not yet."

This caught me by surprise.

I couldn't bear to say I was going right away.

"Not right away. Soon," I said.

"Back?" Asa asked.

"Yes, I’ll come back later."

That was by far the most complete conversation we've had so far.

The cataract

While Asa's language abilities have improved, his vision has deteriorated during the time I've been away.

As we learned in April, there's a cataract developing in his left eye. It's now visible in the form of a milky whiteness in the pupil.

The left eye was formerly his stronger eye. Now, apparently on account of the cataract, he's switched his preference to the right eye. When he's looking at books, or at images on his mum's smartphone (a favourite new toy), he'll cock his head and orient towards the right eye.

This right eye, however, has a centrally located tumour. And so the window he's peering through must be very small indeed.

Unexpected by the doctors, it’s unclear whether the cataract is a side-effect of the cancer, or the chemo, or independent of them.

Getting through chemo

Another wild card that came up in the past two months was a bacterial infection in the Hickman line, during the second cycle of chemo. This had us scared. Asa was in hospital for 8 days.

The Hickman line was eventually removed, and then reinserted a week later.

Thankfully the third and fourth cycles of chemo have gone relatively smoothly, without infections or long spells in hospital.

This is likely due at least in part to the presence of grandmother Kuri, who’s been living with us since April, and who provides such help around the house that Selam is able to concentrate better on keeping Asa happy.

Asa and grandmother Kuri, in the garden

Prospects

The prospects after the next EUA on June 19 are difficult to gauge.

There are at least three possibilities.

1. The most optimistic scenario -- the chemo has caused the tumours to recede dramatically, and no more treatment is needed.
   
   
2. Less optimistically, four cycles of chemo might have produced modest effects on the tumours, and two more cycles could be given before reevaluating progress.
   
   
3. The least optimistic scenario is that the chemo hasn’t had any substantial effect, and we need to consider other treatments.

It impossible to know which of these scenarios we’ll face – or whether, instead, we’ll receive another surprise.

Mixed results

Last Wednesday Asa was put to sleep and underwent an eye exam under anaesthetic. 

The first since the beginning of the new chemo, the exam showed that the drugs have had a "partial effect."

Asa and dad at the Royal London Hospital, before the EUA

In Asa's left eye, the tumours responded well to the chemo. 

But in the right eye, there's been a slight increase in tumour activity.

And in the left eye there's a cataract developing.

A mixed bag

This was not what we'd hoped to hear.

We had reason to expect that the TVD (topotecan-vincristine-doxorubicin) combination would lead to shrinkage of the tumours in both eyes. 

And the appearance of a cataract -- a clouding of the lens -- at this stage is unusual: puzzling to the doctors as well as us.

While cataracts can be removed through surgery, cutting into the eye when there are active tumours inside is not advisable. So treatment for the cataract itself will have to wait until the tumours are stable.

The main risk in the near future is that the cataract may make it difficult to monitor the activity of the tumours in that eye. 

Sight and development

An optometrists' exam the day after the EUA suggested that Asa's sight has not gotten appreciably worse over the last two months.

It was a relief to hear this.

Indeed to us -- his parents -- it seems that his sight might be improving. 

But how could this be, given the equivocal changes in the tumours, and the cataract in one eye?

The thing that suggests improvement is that Asa is increasingly naming the things he's seeing -- a "doggie" on the other side of the street, "Mama" in a family photograph... 

Evidence of the extent to which Asa's childhood is now medicalized:

1. The first time he uttered the word "arm," a couple of weeks ago, was when a nurse was asking where she should place his blood pressure cuff. 

2. For several days after his last admission to hospital, he walked around proclaiming his most recent temperature reading (proudly and unprompted): "[Thirty] six [point] nine!"  

It’s a form of words he's heard us use all too often, as we monitor him for signs of fever.

In the last week, he's made great progress in discriminating letters and numbers, pointing at and naming them in books, on toys, and anywhere else he finds them.

Sharper-sighted, or just smarter?

But there's a danger here of confusing the ability to name things with the ability to see them.

The gains Asa seems to be making in visual discrimination are probably due more to a spurt in language development than to improvement in his eyesight. 

In other words, they're the result of potential that had been untapped while he was unnable to put names to things.

While his progress is wonderful to witness, we're also seeing signs now of how he's adapting to compromises in his vision. 

Playing with an electronic toy that speaks and spells words to him, he'll occasionally hold the letters close to his face, or bend down from the waist to position his eyes close to them, with a twist of the head to get the best part of his visual field working for him.

Onwards

Despite the equivocal results of the chemo so far, the doctors have recommended proceeding with at least two more cycles (each lasting 3 or 4 weeks). 

The 3rd cycle was to begin on Monday, but Asa's had diarrhoea the last few days -- and he's lost a kilo in the last 2 months -- so he's been given a breather until later in the week.

Tomorrow he'll be reevaluated; we hope he'll prove to be stronger. 

With grandma Kuri in Whitechapel

With luck, the tumours in the right eye that haven't yet responded, simply need a more prolonged exposure to the drugs.

Jed is in Congo for the next several  weeks; Selam and grandmother Kuri are caring for Asa in London.