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.

The first cycle / Ethiopia

I got back from Ethiopia two weeks ago, and went straight from the airport to the hospital.

Asa had been admitted two days before with a fever, and according to protocol he was put on IV antibiotics, to be kept under observation until 48 hours after the fever subsided.

I found him and Selam in good form, Asa chatting away (sometimes incomprehensibly) to anyone in range and -- something new, since I'd been away -- saying “Thank you” for the least courtesy anybody paid him.

At the Queen Elizabeth Hospital in Woolwich.
I’d sat the doll on the side of the bed; Asa quickly adopted the same position.

That was on Thursday.

By the weekend we were all back home together.

Second-line chemo, so far

On balance, the first cycle of this second course of chemo went better than we’d feared. There were no mouth sores, no need for an NG tube, and only one day of vomiting.

But it didn't go quite as smoothly as we’d hoped. 

There were 3 episodes of infection: a bout of conjunctivitis which gooed up his eyes, and 2 other infections that caused fevers. 

And he needed 3 blood transfusions: two for platelets and one for red blood cells.

We don't yet know what effect the drugs have had on his tumours.

For that we have to wait until the next EUA, in 3 or 4 weeks time.

A heart murmur

When Asa was admitted with the second fever, doctors detected a heart murmur.

Asa was anaemic at the time, and the murmur was put down to that.

But on reexamination this week -- with the infection gone and his blood counts back to normal -- the murmur was still there.

One of the chemo drugs, Doxorubicin, can affect heart function. To check whether he was fit to receive it, Asa received an echocardiogram exam a month ago.

At that point he was given the all clear.

Might the drug have caused some damage to his heart since then?

Another echo yesterday suggested that his heart is normal.

We hope the murmur is innocent.

 
Meetings in Ethiopia

During my visit to Ethiopia, I met with some families whose children are affected by Rb, and doctors who treat the condition.

“It's really devastating to think about,” said Dr Emebet Girma, a doctor who runs the Ophthalmology Department at the main referral hospital in southern Ethiopia.

“I send children for chemo [in Addis Ababa], but I don’t know if they get it. I do exenteration, but I don’t know [if they’re going to make it].”

Exenteration is a drastic operation that involves not only removing the eye but also cutting away surrounding tissues.

It’s done in cases where the cancer has spread beyond the eye, and where there aren’t better options.

Dr Emebet sees more than 100 cases of retinoblastoma per year in Awasa Hospital. 

That compares with about 50 per year in the U.K.

The majority who turn up in Awasa are children whose eyes bulge from tumours that are very advanced.

Success stories

More hopeful was the meeting with families in Addis Ababa whose children have received treatment for Rb.

These are success stories.

But that’s not to say that things have been easy for them.

The three families I met had to leave Ethiopia to get the treatment their children needed -- traveling to Nairobi, and in one case to the U.S.A.

The children -- Abel, Bisrat, and Edelawit -- are now apparently cancer-free, and full of life.

It was great to meet them and their families.

Meeting of people concerned about retinoblastoma, in Addis Ababa, February 2013: L-R: Jed, Emebet Mammo, Etseganet, Getahun, Abel (in Getahun’s arms), Netsanet, Ermias, Bisrat (in Ermias’ arms), Dr Abu, Edelawit, Mekonnen.

We’re all keen to do something to help improve the state of Rb surveillance and treatment in Ethiopia.
 
At the same time Selam and I are reminded of how lucky we are that Asa can get the treatment he’s receiving in London.

Thanks to my mum and dad, and to all our neighbors -- especially Luke & Sophie, and Fireweyne -- for keeping Selam and Asa company these past weeks.

Relapse. Birthday.

Wednesday's  Examination Under Anaesthetic yielded some unexpected news. 

In Asa's left eye, which had been stable since the end of primary chemo in June, there were 4 or 5 new tumours, and one previously treated tumour that was growing slowly. There were also some new seeds.

In his right eye, moreover, the tumours that had earlier responded well to Melphalan had started to relapse.

These areas are at the front of the eye -- as the doctor put it, "almost where the retina finishes."

And the seeds that were there last time had not responded to the cryotherapy.

Asa looks out the window of the Royal London Hospital, before his EUA.

Treatment options

When Selam picked Asa up from the recovery room, both of his eyes were red and swollen from cryotherapy.

Cryo is a stop-gap measure: Since too much of it can cause retinal detachment, this approach doesn't hold much promise for controlling the tumour growth in the long term.

The area of tumour activity is also too wide for the more gentle kinds of radiotherapy -- such as radiation delivered through a "plaque" inserted into the eye, or so-called lens-sparing radiotherapy.

The treatment options therefore boil down to second-line systemic chemo, or whole-eye radiotherapy.

The decision is difficult.

Second-line chemo involves drugs that are more toxic than the ones Asa received during the first half of 2012.

The drugs are doxorubicin and topotecan.

We've not been able to find out much about their effects in retinoblastoma.

This is because it's rare that they're needed for Rb. Usually the first-line regimen -- in combination with other treatments such as laser and cryotherapy -- is enough to control this cancer.

In the US, our doctors tell us, topotecan has been used intra-arterially (directly to the eye) with some success. This hasn't yet been done in the UK.

More commonly -- on both sides of the Atlantic -- the topotecan and doxorubicin combination is used for neuroblastoma, a cancer that affects the nervous system.

Because the prognosis for neuroblastoma itself is worse than for retinoblastoma, it's difficult to gauge precisely the chances of success in Asa's case.

But the doctors tell us that these drugs are very effective against neuroblastoma, and in the few cases they've seen that merited it, against Rb too.

Side effects

We do know that the side-effects of these drugs can be severe.

Doxorubicin can lead to mouth sores when blood counts are low, and -- if dosage is too high -- cardiac fibrosis (stiffening of the heart muscles), which can be fatal if it's not checked.

Topotecan's side effects include diarrhoea.

And this is on top of the immunosuppression, nausea, malaise, and hair-loss that chemo drugs generally cause.

Going with chemo

Despite all this we've come down on the side of chemo.

Whole-eye radiotherapy, while less traumatic in the short term, would lead to scarring and long-term damage to the tear glands, which can be debilitating in later life.

We can't commit Asa to that, when there's a chance that chemo might produce equivalent results without lasting damage.

The new schedule

Asa will be given the drugs every 3 weeks, over the course of a week each time, with EUAs every 2 cycles.

There may be between 4 and 6 cycles in all.

We'll be spending a lot of time in Great Ormond Street over the next few months. 

And probably a fair amount of time in our local primary care centre -- King's College Hospital -- when Asa picks up opportunistic infections.

A change of lifestyle

On Friday Selam took Asa to a singalong session at the local library, and Soft Play at the Peckham Leisure Centre -- a sort of padded playground, with slides and ladders and a sea of plastic balls.

These are some of the things he's taken pleasure in over the past months.

These activities take on new value as we realise he'll likely have to withdraw from them when the chemo begins.

Only within the past month has Asa started to establish friendships with other children -- notably Angel, the daughter of our neighbours Luke and Sophie, who's just a couple of months older than he is.

Asa and Angel

Hopefully Asa and Angel will continue to play together. But it will be a while before Asa returns to the daycare centre he's been attending.

Asa's birthday

Today Asa is 2 years old. Yesterday (Sunday), friends gathered with us to celebrate his birthday.

Selam coaches Asa in blowing out his candle.

Asa paid little mind to the toys he was given, but enjoyed the attention and the sweet things to eat.

"Happy Birthday, dear Asa." Vanita and Deia, and Millie and Nathan applaud.

Selam and I enjoyed it all. And it gave us a chance to explain to some of the friends we're closest to here in London what we're expecting as Asa begins the new course of chemo.

By the time he gets his first dose of the new drugs, I will be in Ethiopia on a work trip. So we're relying on friends and relatives to help out in the meantime.

It will be hard for all of us to be apart at this time. But we're determined to go on with our lives as close to normally as we can, while doing our best to keep Asa healthy.

World Cancer Day

By coincidence, February 4 is also World Cancer Day.

Check out the website, and consider the messages of the organisers on common myths about cancer.

 

Cancer is not a death sentence; it's not a matter of fate; it's not a disease of the wealthy, elderly, or developed countries; and it's not just a health issue.

Lessons from a strange year

It was one year ago today that we got Asa's diagnosis.

Looking back, we marvel at how quickly things have gone.

We left Ethiopia on the 9th of January, four days after the fateful visit to the ophthalmologist.

Asa saw our GP the day we arrived in the UK, and began treatment the following week.

 

He turned one during the first round of systemic chemotherapy.

A year into this journey, we'd like to share some lessons.

1.  Be thankful for what you've got.

That's lesson 1.

Although retinoblastoma has become a big part of Asa's story -- the eye exams and treatment regime setting much of the tempo of our lives these days -- to us he's still much more than an Rb case. 

In every other way -- curiosity, playfulness, sociability -- he's a normal kid. A wonderful kid. With as good a shot at life as any other.

trying on Daddy's shoes

And yet if it weren't for the support we receive from the medical system here in the UK we wouldn't be able to say that.

His life would be in danger.

So let us acknowledge again our gratitude to the NHS, and the many doctors, nurses, and support staff who make it such a great institution.

2. Be ready for curve balls.

Lesson 2 is "Expect the unexpected". 

For example, the low potassium levels that Asa suffered during systemic chemo -- requiring bottle after bottle of supplements -- were a surprise to everyone.

It's not a normal side-effect of chemo.

And the anatomy of the arteries supplying his eyes is abnormal (as it is for many of us -- we just don't have reason to check).

We only found out about that when he began intra-arterial chemo in September. 

More generally, we've learned this past year that things that you think would be tough may be easy. And things that you think would be easy may be tough.

Fear not the Hickman line, NG tube, neutropenia, or transfusion!

All of these things were scary at first. Very soon they became entirely innocuous to us.
 

Halloween party at Great Ormond Street, 2012

But when you've gotten used to crisis mode, a greater challenge may be dealing with normal life.

Worry, anxiety, and depression tended to descend on us during "peace time" more than during times of crisis.

Which makes lesson 3 particularly important….

 

3. Look for silver linings.

Chemo was hard, but also a blessed time in many ways.

Selam gave up her job in Addis Ababa to care for Asa full-time. And thanks to my employer, I was able to work from home.

So we were all together for 6 months -- locked down indoors at first, but then, after the chemo finished, with greater freedom. 

Frontispiece from "How We Work," a book Asa enjoys

On account of this, we witnessed more of Asa's development first-hand than we would have done had our lives continued as before.

That's a great gift, because he's not going to be one year old again.

Another blessing has been the relationships we've strengthened this past year.

Dozens of friends sent us photos bearing good wishes for Asa. 

We've received many warm messages.

And we've also forged new friendships -- with people we've met at hospitals, at the Childhood Eye Cancer Trust family weekend, and online.

Next month we hope to make further contact with families in Ethiopia whose children have Rb, and the doctors involved in their treatment.

 

4. Hope and pray for the best.

2013 is going to be a crucial year. The period of greatest risk for tumour growth is from birth to 3 years; after that the risk of relapse decreases.

It decreases even more dramatically after age 5. But we must take one year at a time.

If we can get Asa through to 2014 with the cancer in check, there's a good chance he'll keep his eyes.

Last Wednesday, an exam showed flattening of the tumours in his right eye as a result of the IAM.

But there were also three new seeds in that eye that needed cryo treatment.

The next EUA will be on January 30.

We remain grateful for the support, advice, and treatment we're getting, knowing it's among the best available in the world.

And we hope and pray for the best.

Happy new year!

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.