We are always looking for contributions to include in the newsletter!!!
This is my first experience putting together a newsletter ( my husband John put the professional look to it ). As you will read about in the "our story" section, our son Thomas was diagnosed with bilateral RB at 15 months and had his right eye enucleated and chemotherapy for the left. He had his last dose of chemo, almost one year ago, so we're not a new family, but not old either! What I found most helpful about previous newsletters was reading other families' stories, so I have included 4 stories in this edition. If you are a family with a newly diagnosed child, I hope these stories help you fell less "alone" and give you some support.
The other main focus of this newsletter is to inform everyone of the fundraising efforts that have taken place and which are still needed to purchase a retinal camera for the New Children's Hospital, Westmead. As you will find out, this piece of equipment is extremely important in the treatment of RB and other diseases that affect the retina, and we don't have one in Sydney!
There are also some updates on hydroxyapatite implants, and general information about artificial eyes as well as a good summary about RB genetics that I'm sure we've all been told about, but forget over time.
Regards,
Karen Avgenicos
A picnic will be held at Bicentennial Park, Homebush, on Saturday 17th April at 11.30am for RB families to either catch up with old friends or meet new families. There is a play area for the children, a bike track and electric barbecues if you are really keen. Please phone Murray Chapman on (02) 9544 8065 if you are able to attend. A map is attached to the newsletter to guide you to the spot!
Editors Note: Thank you to Murray and Samantha Chapman for hosting the newsletter on their homepage.
I took him to our optometrist on the 11th October 1994 (my birthday of all days). He said there were adhesions (scars) on his eyes and some kind of growth but he couldn't specify what it was. He said Dean needed to see an Opthamologist and went to make the appointment for us. When I was told I had to be in Canberra in just 3 days time I began to feel a little more concerned.
It was a warm spring day in Canberra. We had a long wait to see the doctor. I read brochures on cataracts while we waited so I built my hopes up to maybe 'growth means cataract'.
Dean had drops put into his eyes and an examination. It soon became clear to us that he had next to no vision in his left eye. The doctor then had a look at his eyes and bluntly told us "It's like this:- he could die and he'll more than likely have to lose his eye". At this point we became absolute wrecks and collapsed in a heap. All this time our 4 year old Dean sat between us totally bewildered. When we finally somehow pulled ourselves together we were told we'd have to go to Sydney and that our appointment was in 3 days time at the Eye Hospital.
I remember staggering out of the office in a daze. It was hard not to let the tears flow and to stop them when they did. The kids knew something was wrong but it was too hard to explain as we weren't too sure ourselves what it was.
We spent the night in Canberra with friends and somehow managed to phone and tell my mother. It wasn't the best birthday present I could have given her either. That same afternoon Dean said to me "Mum, why did that man say I'm going to die?" I felt as though I'd had a dagger driven through my heart. All I could do was explain that his eye was sick and he could get very sick because of it so he had to go to hospital in Sydney.
The next day (at home) we were surrounded by family as we prepared for the trip I wandered around like a zombie trying to get organised. It was best that Brent (our older son) stayed home. It was hard to leave him behind.
We drove back to Canberra the following day getting a broken windscreen on the way (our luck was definately out!!). A friend was driving us to Sydney that afternoon. We had accommodation arranged at a ritzy (expensive) motel close to the eye hospital. We arrived in Sydney late that night. Neither of us could sleep as we both felt quite ill with worry as to what may lay ahead.
The next morning at the eye hospital we were told on arrival that Professor Bilson was no there that day even though we had an appointment. We saw a few other doctors who began to shed light on the matter telling us it could be one of two conditions but Retinoblastoma was the most likely cause (although at 4 they thought he was a 'little old'). Prof. Bilson finally did arrive and explained the facts to us and made arrangements for Dean to be admitted to the Children's Hospital.
We had to take a cab to the hospital (what a hairy trip that was!) where we arrived with feelings of confusion and fear. Dean settled in well though and soon became popular with the nursing staff. We couldn't both stay at the hospital so Mick was able to go to Ronald McDonald House which is a place for kids with cancer and their families to stay during treatment. 'The House' proved to be an invaluable place of escape for us over the next few months/years.
The following day tests began. The C.T. scan showed the tumour to be a large mass in Dean's left eye and unfortunately the right eye was affected as well but the tumour was pin head sized.
Dean's eye was to be enucleated (removed) the next week. I counted down the days as all I wanted was for it to be gone once I was aware of how it was threatening his life.
At about this time we met the Opthalmic Liaison sister, Joanna McCulloch who helped a lot be explaining what was happening in simpler terms. Joanna became a friendly face who I was always happy to see. Dean was happy to see her too unless she was about to put drops in his eyes! I think we 'cried' on Joanna's shoulders more than once so its fortunate she has 'broad shoulders'! A couple of years later she's still there for us and I'm forever grateful for that.
Mick, Dean and I played tourist until the day of the operation arrived. The operation took four hours. The left eye was enucleated and cryotherapy (the tumour is frozen with cold gas) done to the right eye.
Three days later, after we had just made the arrangements to go home, a nurse tracked us down saying Prof. needed to see us. We were told the laboratory results were back on Dean's diseased eye. The tumour had begun to spread to his optic nerve and it was found in the 1st mm of the 8mm they removed so it would be best if Dean had chemotherapy to 'mop up' any RB that may have 'escaped' into his bloodstream.
We met Luce Dalla Pozza (Oncologist) that same day and I agreed to chemo. Mick wasn't with me at the time so when he walked back into the ward Dean coolly announced "Guess what, Dad? My hair's going to fall out!" Mick didn't take it all that well and to cut a long story short he went home sporting a sore hand after a fight with a sign post (that obviously won!).
After 2 weeks at home we went back to hospital expecting to start chemo but we ran into a few more problems as Dean's eye socket wasn't healing so this delayed treatment for a couple of weeks as chemotherapy may have stopped the healing process altogether.
Chemo finally started December 4th 1994 - 6 weeks after he lost his eye. His hair began to fall out 10 days later which brought home the reality of it all. We were really lucky during chemo (that there were no more infections after initial problems with the central line which required antibiotics) and no need for transfusions until course 5 was due and then he got an infection in the central line. (This meant that whenever the line was used he had high temperatures which caused him to shake and shiver). We spent a week at Young hospital but the 'bug' stayed in the line. We transferred to Camperdown and after a lengthy wait in the garden because Dean couldn't go near other oncology kids as his brother Brent had chickenpox a couple of weeks before and he had been exposed to them (but didn't get them). The following day, acid treatment was used to kill the 'bug' in the line. Fortunately it worked and he had his 5th course of chemo just before Easter 1995.
The 6th and final course was given in May 1995. Two days before his 5th birthday, in June, the line was removed and on his birthday he received his 'Make A Wish' foundation wish which was for real metal gold clubs, a bag and buggy. It was a very special day and a wonderful end to a very trying time.
It is now over 18 months since Dean finished treatment.
He had 3 monthly checks for a while and then went to 6 monthly checks that are soon to be yearly.
Dean has been an inspiration to us all with his courage and will to battle it out. He rarely ever complained. Occasionally now he will say "Mum, I wish I never lost my eye" which breaks my heart but he knows it is better for him to have lost it.
He started school in 1996 and is going quite well. He also plays a lot of sport and has had a successful first soccer season scoring goals! Sometimes other kids notice his eye and kids his age have no problems with it. It tends to be older kids that pick on him. Deans shrugs it off most times as he knows its the was it has to be and he says if they don't like it he doesn't have to be there friend.
Dean became a big brother in April 1996 to Blake.
At present the opthamologist makes a drawing (using coloured pencils and crayons) of the findings whilst the patient is anaesthetised. This means that the children are under anaesthetic for a longer than necessary period. It also means that it relies on the doctor's drawing skills. The Retinal Camera will allow a photographic image to be taken in seconds and also allow this real image to be transmitted to other specialists anywhere in the world for a second opinion.
In addition, the cameral will aid in tracking th progress of treatment as well as being a useful tool to help parents understand what is going on.
Every year, hundreds of examinations are conducted on children with Retinoblastoma as well as many hundreds more on children with conditions such as retinopathy of prematurity and shaken baby syndrome. The Retinal Camera also had the potential to relieve some of the burden on our health system by reducing theatre time and, at the same time, improving patient care.
If you or someone you know wishes to make a donation to this worthy project please send a cheque to:
A lot, lot more fundraising is needed - at least another $90,000.00. However, it is possible that if half the funds can be raised ($60,000) the hospital will pay for the remainder.
With much joy, we welcomed Thomas into our lives. Where Michael was a sickly, fretful baby, Thomas was a placid, bonny baby. I was much more relaxed and was able to breastfeed Thomas without difficulty. Michael's troublesome temperament was becoming more manageable, his palsy was only evidencedby slight clumsiness, and we had a new baby who was a picture of health.
Then when Thomas was 12 months old, my mother noticed his right eye was turning in. It seemed a minor problem and so when it took 3 months to get an appointment with the specialist we weren't worried. Besides, we thought that if the doctor was worried, we'd have been given an earlier appointment.
On the day I took Thomas to the eye specialist (Dr Craig Donaldson, who was at this stage not specialising in RB, but by fluke had just returned from Canada studying it), I was confused as to why he was taking so long looking into Thomas's eyes. Thomas was screaming, surely the doctor could see how distressed he was! Nothing could prepare me for those words "We've got some problems". Dr Donaldson told me that Thomas had tumours in both of his eyes and that he was blind in the right eye (compared with Dean's story, we were so fortunate to have had a doctor who knew what he was talking about, and break the new gently). The tumours in the left eye were around the outside of the retina, so he could still see quite well out of that eye. There was still some chance of saving the sight in that eye.
I couldn't believe what the doctor was trying to tell me. Thomas was our healthy child, he walked at 11 months, he did everything perfectly, how could he be blind in one eye and we had never noticed? As soon as John arrived at the specialists rooms shortly after, we broke down and sobbed in each others arms. We pulled ourselves together and took our little boy hoome who was quite bewildered by all the fuss. Later that afternoon we found out that Thomas would probably have to have his right eye removed. The thought of this made me feel sick, buy my family rallied around us and just kept saying that all that mattered was to save his life, and he would still have the other eye.
Over the next few days I became obsessed with having to find out if the tumours had spread to Thomas's brain. We had to wait 5 days for him to be admitted for tests that would tell us if the tumours had spread. Those were the longest days of my life. I couldn't sleep or eat. When the doctor came out of the first operation, he told us the tumours had been there a long time. Even the "good" eye was worse than he thought (because Thomas was screaming and moving so much) at the inital consultation - I thought we were too late. When he said that Thomas' right eye would have to be removed, I said "I don't care, I just don't want to lose him".
When we eventually found out the tumours had not spread, we were estatic. However, it wasn't too long before my fears about Thomas being blind began to overwhelm me, and my old feelings of anxiety and depression returned. My family was beside themselves with worry over Thomas and about me. Then one day I realised that my need to have 100% reassurance that the treatment would work was not going to make it any more likely to happen. I had to accept that there were no guarantees, and get on with looking after Thomas and Michael. They needed me to be well, and I could only be well if I stopped worrying and started taking one day at a time. I was surprised at how well I was able to cope when I adoped this attitude.
We then entered the world of chemotherapy. As those of you have experienced this know, there are only three types of children on the oncology ward: those who are having difficulties with chemotherapy or their illness and their very worried families; and those for whom no more can be done and their very sad families.
Every 3 weeks Thomas had to have an examination under anaesthetic and cryotherapy, immediately followed by three days of chemotherapy. During the 6 months of treatment, Thomas was in and out of hospital because of ongoing fevers caused by the chemo. making him more susceptible to infections. These were the most trying days, Michael spent many days and weeks with his grandparents, and without their support and my sisteers I do not know how we would have coped.
As well as dealing with your own child's cancer, when you are in the oncology unit you have to deal with the sadness and pain that confront others. We would sit and chat to other families, getting to know them. Sometimes you would find out their child is gravely ill, and may not live. Part of me found this extremely upsetting, but in some ways it made me realise how lucky we were. I would think about how these parents would give anything if their child only had to lose an eye and have 6 months of chemo. to ensure they would live. I would also think about the anguish of the families whose children had to lose both eyes, despite all attempts at treatments.
Thomas completed chemotherapy in March 1998 and the left eye has normal vision, which I think is a miracle. I know he can see well because the first thing he always sees in doctor's surgeries are the lollies (usually hidden in a corner!). As yet, no tumours have returned. Thomas wears an artifical right eye, which looks great. However, he has had problems with repeated infections. We have been told that this is probably due to his grubby fingers poking the eye (boys will be boys!) and it should settle as he gets older. It's funny how I seem to get more upset these days over this aspect of his condition (which in the big picture is quite trivial) than when we were going through treatment. I guess that's human nature though- once the crisis is over it catches up with you, and you start to absorb what has happened and experience the long-term aspects of the problem. It may also be because we don't have much contact with the oncology unit anymore, so we are not constantly reminded of how lucky we are that Thomas is alive and can see, but instead, "why us?" creeps in a bit more. Sometimes I think about how we will tell him that he may get other tumours as an adult and that he has a 50% chance of passing on the gene. I think about the pain he and we will go through if and when he decides whether he should have children. While it seems so cruel that at such a young age children ( or babies) have to experience dreadful pain and fear during RB treatment, as a parent you don't have to deal with the depression, anxiety and difficult questions that older children with cancer often present with.
Having said all that, we are now living essentially a normal life. We try not to think too much about the future, because none of us knows what is around the corner. Today is the only thing we have guaranteed, so we try to make the most of it. Our children certainly reinforce this philosophy - they're only worried about when the next iceblock is coming. Several people have asked me whether having children was worth it after all the pain we've experienced, and I don't hesitate in saying "yes". Our children bring us indescribable joy, their smiling faces being the best therapy of all.
In January 1999, Sam had corrective surgery to the left eye to treat his severe head tilt. It has worked remarkably well. Instead of looking at people and things from his left shoulder, he now looks from the centre, and looks perfectly normal in photos.
Sam still has 3 monthly check ups with Prof. Bilson and still attends the oncology clinic every 2 - 3 months. Sam is on his 6th CAT Scan, but if it appears normal it will be his last (due in March). Sam is a normal 3 year old boy into everything, making up for lost time.
At the age of 18 months Emily was diagnosed on 15th Dec 97 with possible bilateral retinoblastoma. This was confirmed 2 days later by Dr Craig Donaldson at The New Children's Hospital - Westmead. Emily had 2 tumours in her left eye and 5 in her right, covering 75% of her vision. Dr Donaldson was not sure if he could save the right eye but said he would try. Chemotherapy was recommended to us as the best possible treatment method and Emily received her first round of treatment on 22nd Dec 97 and she was discharged on Christmas eve.
Like everyone else who's child is diagnosed with cancer the initial shock and trauma associated with the news is difficult to deal with. we still found it hard to come to grips with even though as the father I had bilateral RB and we knew there was some chance that it might be passed on. Little did we know that in fact the chance was 50-50. There was also the guilt factor which had to be overcome' only if I had... Well the botton line is you have to forget about the past.
We began our search for more information about chemotherapy, it's effects on the patient, how to deal with them and anything else that might be useful. Whilst the hospital provided some information we still felt inadequate and wanted to know more.
A good source of information was available from our local library. There we found many books and one or two even dealt with the negative side, questioning the effectiveness of chemotherapy. Even though very negative about the usefulness of chemotherapy on adult cancer patients it was very positive about it's use on childhood cancers such as retinoblastoma.
The Internet had a few good sites which offered either personal accounts, medical facts or general information.
Reading all this material encouraged us to believe there was hope. The more we read, the more we were convinced that a natural approach in Emily's diet was the way to go. It would assist in her tolerance to, and effectiveness of, chemotherapy. Ideas put forward suggested that diet affects the body's immune system and can feed the growth of cancer cells.
Emily's diet for the first 2 months of chemotherapy consisted of all the things that we thought ok. Milk, eggs, some red meat, fish, chicken, yoghurt, bread, cereals, vegies and fruit. After switching to a controlled diet we found Emily had more energy and looked healthier within a few weeks.
Red meat and chicken were eliminated from Emily's diet altogether. Red meat because the animals feed contains chemicals that passed on and while rich in protein it feeds cancer cells. Chicken is pumped full of hormones to make them grow and their protein feeds cancer cells. Orange and orange juice has been found to make a chemotherapy patient feel sick.
Organic foods are more difficult to find and more expensive, but they are free of chemicals. When digested chemicals make the immune system work harder weakening it's fight against cancer cells. We receive home delivery, go to a weekend organic market at Frenchs Forest and specialist health food stores selling organic products. You can also find references to organic in the white and yellow pages phone books.
It is our firm belief that Emily is a healthier person and has reponded to treatment because of her diet. Adopting such a change in diet is not easy, parents have to make sacrifices. We are also fortunate that at this time Emily is our only child, as we imagine that any such changes are much more difficult with other children around.
Six months after finishing chemotherapy, Emily had good sight in both eyes. There has been minor tumour re-growth, which has been treated locally (cryotherapy) and so far so good. Emily is still on monthly examinations under anaesthetic due to the recurrence of her tumours. No one will ever know if Emily and the tumours in both eyes would have responded any differently if we had not changed her diet. Nor can we say that the tumour re-growth after chemo would have been any worse.
In changing Emily's diet we believe we made the right decision and the sacrifice was worth it.
What is an artificial eye made of ?
The prosthesis is made of a very fine plastic called acrylic ( like dentures ).
It can be cleaned with dental cleansers, but ensure it is properly rinsed. It won't break if dropped, but will scratch. If scratched, infections can occur. The eye can be repolished by your eye-maker to remove the scratch.
Does the standard eye implant ( the ball inserted behind the socket when the enucleation took place ) ever need to be replaced ?
Only if the person decides to have a hydroxyapatite implant ( see further down ), which is usually only considered if the child's facial muscles and bones have stopped growing ( around age 8 as a minimum ), or if there are problems with the existing ball ( e.g :- ball expulsion ).
Are eye infections more common in young children ?
Yes, most definately. In children and babies, discharge is usually caused by the child touching the eye with fingers that may not be clean. Conjuctivitis results, which is pretty contagious ( i.e. spreads to the other eye or other people ). Discharge is also more common when your child has a cold, and conjuctivitis is more likely to occur during these times. If the prosthesis is not fitting correctly, or if it has been scratched, eye infections are also more likely. The good news it that infections found in young children invariably improve with time.
How do you treat eye infections ?
You can use mild medications as recommended by your opthamologist or if discharge persists and/or becomes thick your doctor ( GP or specialist ) may prescribe a broader spectrum antibiotic such as Chloromycetin.
You must use the whole course, even if the discharge eases or disappears - if you only use it for 2 or 3 days, the discharge may return and you will end up with continually medicating what becomes a low grade, chronic discharge problem.
If you have no trouble removing and inserting your child's prosthesis, an ointment is more efficient - place a worm of it in the back of the artificial eye, and repeat administration throughout the day with the prosthesis in as prescribed by your doctor. If you have trouble removing the prosthesis, use drops - administer when the child is lying down, so that you can gently pull the lids away from the eye to allow the liquid to penetrate behind the eye.
If your child has continual problems, the prosthesis should be checked by your eye maker to make sure there are no sharp edges and that it is not ill fitting.
Joanna McCullouch also said that an artificial eye that is poorly lubricated can also cause irritation, inflammation and eventually infection. The use of a lubricant such as Lacrilube, Refresh or Viscose can prevent this occurring.
How often should the eye be cleaned ?
If severe discharge is evident, daily cleaning is required. Monthly cleaning is sufficient if no discharge exists.
An eye that is too large or too small can cause discomfort and discharge. Insufficient cleaning can lead to discharge problems by causing giant papillary conjunctivitis ( GPC ) under the upper lid.
How do I make the process of removing or inserting my child's eye, or administering drops less traumatic ?
Give lollies ASAP!! ( That is the editors advice, not professionals ).
What is a Hydroxyapatite ( hydroxy. ) orbital implant ?
This implant, made of hydroxyapatite ( a substance similar to human bone both structurally and chemically ) would replace the existing standard ball implant behind the socket. They hydroxy. ball is similar in apprearance to coral as it is porous. It is wrapped in sclera and the porous nature of the ball allows blood vessels and tissues to grow in and become a part of the implant.
A peg is eventually attached to the implant to allow for natural movement of the prosthesis. The peg conveys socket movement directly to the artificial eye and helps support the weight of the artificial eye, thus reducing lower lid sag.
What are the benefits of having a hydroxy. implant ?
Because the implant allows the artificial eye to move naturally in conjunction with the normal eye, it is difficult to detect that the eye is a prosthesis. Only a pegged implant can focus the unnatural weight of the artificial eye on the peg, and not the lower eyelid.
How do I have a Hydroxy. implant inserted ?
Two operations are required. The patient's existing standard ball implant would be removed from the orbit and replaced with the hydroxy. implant (first operation). Once the socket has healed, the patient can wear the artificial eye. However, it is usually six months before the tissues have grown into the implant, then allowing a peg to be drilled into the implant through the lining of the socket (second operation). This operation can be done under a local anaesthetic. However, a general would usually be given to a child. A mild pressure bandage is placed over the socket for 24 hours. About four weeks later the peg can be inserted.
What problems/risks are associated with hydroxy. implants ?
( Thanks to Joanna McCulloch for this information )
1. It requires two operations, one at least requiring a general anaesthetic.
2. Most hydroxy implants have been successful. However, a few cases have been reported where the ball has been expelled out through the conjunctiva.
3. A few cases have also experienced chronic infection of the hydroxy. ball necessitating removal of the implant ( which is more difficult to remove than a standard implant because the tissues have grown into it ), cleaning and reinsertion.
4. It is a time-consuming process, taking 6 months for the implant to vascularise ( i.e. blood vessels and tissues to become properly attached ). Then the peg hole has to be drilled, then 4 weeks later the peg inserted.
5. It costs about $1000 for the implant, which will be covered somewhat by your health fund, but if you are a Medicare patient, you will have to pay for it.
We are hoping that Tzu-Chi will help again, they have been so generous in the past, but we are also hoping that you can spread the word. Mentioning the appeal and the usefulness of the camera to others may generate some success.
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