Abbie's Story

Abbie Elizabeth Shaw
1st  March 2001 – 11th  September 2006

December, 2002

On the 9th December, 2002 following a short illness we discovered that Abbie our 21 month old daughter had a tumour in her abdomen which was thought to be a Wilms tumour. On 17th December 2002 she went to St Georges hospital as an outpatient for a biopsy of the tumour. During the procedure she was found to be bleeding into the tumour and a decision was made to remove the tumour along with her left kidney and adrenal gland. Abbie spent several days in intensive care at St Georges hospital and was released just a few days before Christmas and on Christmas Eve she was given her first dose of chemotherapy drugs.

The results of the biopsy of her tumour were very disappointing. The tumour was much worse than we could have ever expected as it was actually an adrenal neuroblastoma which is a more life threatening cancer than Wilms. Also Abbie had NMYC amplification (meaning the cancer is aggressive with the cells dividing up to 200 times more quickly than normal). We were very hopeful that we had caught it early and that any residual following the operation would disappear on its own accord.

July 16th 2003

Not good news we're afraid.

Abbie has had a brilliant few months - nursery, holidays, swimming, playgrounds etc. She has been regularly monitored and in June her blood pressure went up and various tests began to indicate that the cancer had returned. The Marsden booked her in for an MIBG scan, (which uses a radioactive substance which attaches itself to these particular cancer cells), which she had a week ago. Lying still for almost 60 minutes with a large scanner plate just a cm or so above your body whilst 5, 10 minute pictures are taken isn't easy for anyone at the best of times but she was an absolute star and with the assistance of a very long Postman Pat video, she did it.

At the weekend Abbie started limping and developed a droopy eyelid. Her appetite also rapidly diminished - all symptoms of the return of her condition. Today we received the results of the MIBG scan.  This  shows the cancer is back and has spread to her bones and bone marrow. This spread means that she has been put on a very intensive course of chemotherapy for seventy days, followed by an operation to remove a secondary abdominal tumour, stem cell removal, high dose chemotherapy, stem cell rescue, radiotherapy and the 6 months of follow up drugs.

Looking at the positives our consultant told us she has treated much worse cases than Abbie’s that have survived. She is fit and healthy, (which makes this whole thing even more unbelievable) and only on Saturday morning was running around the garden at a rate of knots being Abbie! She has an amazing immune system, (her cancer is the only illness she has had in the last year) and a strong personality. Treatment of high risk neuroblastomas such as Abbie's has progressed a lot in the last few years and whilst the survival rate isn't as good as for some other cancers the new procedures are starting to impact the results. We are lucky to be just half an hour from the Marsden hospital in Sutton where she will be treated which is one of the World's leading cancer hospitals.

So it's not been a good week!!!

August 10th 2003

In the last couple of weeks Abbie has had a Hickman line inserted into her chest; bone and bone marrow samples taken, a CT scan and has had three doses of chemo with the fourth due to start next Sunday.

Abbie is surprisingly well, the chemo to date has not had particularly adverse effects. The first dose did cause sickness and as a result of the chemo her blood platelet count dropped. She began to bleed from her Hickman line and as the blood would not clot she required a platelet and blood transfusion at the Royal Surrey. She then developed a mild allergic reaction to the platelets and as her white blood cell count is very low, (in medical speak she is neutropenic and cannot fight infection) she developed a temperature which had to be treated with a 6 hourly dose of intravenous antibiotics. This meant we had to sleep at the Royal Surrey for 4 nights but fortunately were allowed out between doses. As soon as she was discharged we drove to the Marsden for two nights of chemo.

Abbie has also had a CT scan under a general anaesthetic. This shows a tumour of approx 5cm x 7cm has grown back in the site of the original tumour - an ultrasound at the end of May had shown nothing at all so it shows how quickly this stuff attacks. This news is disappointing as she may require more surgery to remove it, but is fairly inconsequential in the scheme of things. She is still Adrenal Neuroblastoma Stage 4, (the most severely spread) and N-MYC positive, (the most aggressive type) so things can't get any worse.

The potassium levels in Abbie's blood are now very low, so she requires medicine to boost them. She has been prescribed huge doses of the most foul tasting stuff which she refuses to take. Forcing medicine on a two year old is very distressing in such large quantities. We just can't understand why all children's medicine is not chocolate flavoured. This has meant two trips to the Royal Surrey just to collect prescriptions and many inventive ways of trying to disguise it in her food.

On a very positive note Abbie's limp has gone and her eye has improved. She looks very fit and healthy - you really wouldn't think anything was wrong. She is eating reasonably well, drinking lots of milk and has minimal weight loss. Most of her hair disappeared very rapidly last week but neither she, Jamie nor her friends have batted an eyelid. She appears to need less sleep and no longer has a daytime nap. Last week I took her to a friends where she had a good old bounce on a trampoline, went for a picnic at Painshill Park and had tea at a neighbours.

As Abbie's prognosis is relatively poor we are contemplating having her treated in New York. There is one hospital which has had better results in treating Neuroblastoma patients using antibodies from mice with the aim of teaching the body to fight Neuroblastoma cells itself. In fact the treatment was featured on Trevor McDonald's programme in June. We have asked her consultant to look into this for us and will keep you posted.

October 15th 2003

Abbie completed day 70 or the 8th course of her intensive chemo at the end of September. She's coped pretty well with the intensive chemo with minimal side effects apart from getting a number of temperatures due to her low blood count which has led to many more nights in hospital than we would have liked. (43 nights out of 73) including 2am visits to the Royal Surrey.

Over the last week or so she has been undergoing a number of tests to see how well she has responded. Thankfully the news is pretty good. The tumour has completely disappeared and was not detectable on the CT scan, her bone marrow appears to be clear but there was a small amount of disease detected at the site of the tumour in her abdomen by the MIBG scan, (radioactive iodine that attaches to Neuroblastoma cells) and in the bone sample taken from her left hip. We are hopeful that this should all be taken care of by the treatment still to come.

Abbie is in fact unique at the Marsden as the only Stage 4 Neuroblastoma case where the tumour has completely disappeared at this stage. This probably has a lot to do with the fact that her primary tumour was removed in December and she was being closely monitored following the initial occurrence but is still an indication of how well she has done.

Abbie has just spent another few days at the Marsden having her stem cells 'harvested' from her blood. Next week she goes into St Georges, Tooting for a few days to be 'opened up' to check for any tumour remnants - despite the tumour having gone they want to double check visually that there is nothing left behind.

Once she has recovered from the surgery she will start the high dose chemotherapy. Although this only takes a few days to administer it is very strong and destroys at lot of the function of her bone marrow so following this they re-introduce her stem cells to help her recover and kick her system back into life. This means she will be in hospital for at least 6-8 weeks to allow for the treatment, all the side effects, (which are much nastier this time due to the higher doses) and for normality to return to her blood. There then follows 2 weeks of radiotherapy.

Following this we have decided to proceed with an antibody therapy that is only currently available in one hospital in the States - the idea is that your body is taught to fight neuroblastoma cells. Our consultant is just initiating contact with the hospital but it sounds as if they will be happy to accept Abbie. Will let you know when we have more news.

It's a long old process but it's been nice to see some of the old Abbie return in the last few days.

November 4th 2003

Abbie started high dose chemotherapy at midday on Friday. She now has a gastro feeding tube inserted through her nose which is used mainly to administer her drugs but is also used for having overnight feeds to bulk her up. Despite the fact that the tubes can stay in for 1 month, hers have already come out three times. Putting them in is a very unpleasant experience.

The first high dose drug she received is Busulphan and she has had sixteen doses of this through her tube.  It's so toxic that the nurses have to gown up to crush the tablet and everyone has to leave the room during preparation in case any is inhaled. So far Abbie has not been too badly affected apart from sickness. Tomorrow she begins the second drug Melpahalan which is an hours infusion through her Hickman line or wiggly as she calls it. Then on Friday her stem cells will be reintroduced to assist her recovery.  She is expected to get mucositus within 7-10 days which is basically inflammation of the entire digestive tract.

We discovered on Thursday that Abbie's hearing has been badly affected by one of the previous chemo drugs and she has permanently lost her high pitch hearing. It was very shocking as her hearing was perfect in July and we had no idea that her hearing had suffered. She has been fitted for hearing aids at the Royal Surrey, but since then we have been told that it is unusual for children who lose their high pitch sounds to be fitted with aids.  

December 9th 2003

It is exactly a year today that Abbie was diagnosed with a tumour. We are pleased to say that she has virtually made a full recovery from her high dose chemo and stem cell transplant and has been allowed home for the last few afternoons. I am hoping that she will be fully discharged on Thursday morning which marks 6 weeks since she was admitted for the stem cell transplant and her 93rd night in hospital over the year. She has also had 13 blood transfusions and 16 platelet transfusions and by the end of treatment in Feb will have had over 20 general anaesthetics.

Abbie tolerated the high dose chemo surprisingly well. The first side effect to kick in about a week after the chemo was mucositis - her gut had been stripped by the chemo and she had a few weeks of stuff regularly coming out of both ends in an array of colours. Amazingly her mouth did not ulcerate, (which normally happens and causes a lot of pain and discomfort), but she did get the most appalling nappy rash. Then three weeks after being admitted just as she was beginning to eat and get back to normal, her liver function began to show abnormal results and her weight and girth measurement increased showing signs of one of the nastiest side effects of the chemo - veno occlusive disease, (VOD). This occurs when the liver gets bunged up and therefore can't operate properly. In most cases restricted fluids help the liver get better of its own accord but in extreme cases it is life threatening. Over a 5 day period Abbie's girth measurement increased by 4 inches and her weight by over a kilo - she turned into a little barrel and was very uncomfortable due to the enlarged liver and the fluid that was building up inside her. Thankfully over the last week her body has returned to normality.

After a great deal of research and consultation with the UK's neuroblastoma experts we have decided to hold off taking Abbie to New York for antibody treatment for the time being. Abbie has responded incredibly well to her treatment to date and, assuming the high dose chemotherapy and the radiotherapy work as expected, the antibody therapy would appear to offer little in the way of increasing her chances. We have been told that her response has been one of the best out of all the patients in the last couple of years and that her chances are now 50/50. Obviously there are no guarantees. It is heart breaking to meet the parents whose children are not responding well to treatment when the inevitable lays ahead for them. I just don't know how they begin to cope.

In herself Abbie has regained her sense of humour and is amazingly cheerful, she is beginning to eat small amounts of solid food and spends a couple of hours each day running riot around the ward. On the 20th January Abbie starts 14 doses of radiotherapy, with each one under a general anaesthetic. The radiotherapy will lose her an inch in her total height as some of her vertebrae will be hit. On the 6th February she will have completed the bulk of her treatment and will take '13-cis retinoic acid' orally for the last 6 months of her treatment.

In the next few weeks we are getting Abbie's left eye checked as we suspected it may have been diseased although it seems ok now, and a second opinion on Abbie’s hearing to see whether she actually need hearing aids. We are all really looking forward to having a normal Christmas.

February 5th 2004

Abbie was finally discharged from hospital on 11th December. Thankfully, she recovered from the mild VOD, (liver problems) but unfortunately required a daily injection into her thigh to ensure her blood clotted correctly, (a consequence of the VOD). The last injection was on Boxing day and we were amazed that the community nurses volunteered to visit her on Christmas day and Boxing day so we didn’t have to go to hospital. Despite having 2 Hickman lines, a Portacath and a feeding tube to access her little body she still had to have this particular drug via an injection - unbelievable.

In early January we received a call from the paediatric radiotherapy consultant at the Marsden to say that Abbie's radiotherapy had been brought forward to 12th January. This was great news but we then sadly discovered that a space had been freed up, as Naomi, a four year old Neuroblastoma patient that we knew very well had passed away on New Years Day.

For 14 days, Abbie received a quick dose of radiotherapy under a general anaesthetic. We thought that the gradual build up of anaesthetic combined with the radiotherapy would make her tired and sick but she tolerated the radiotherapy very well, although her appetite has been knocked again. Interspersed with the radiotherapy have also been a number of tests and scans to re-stage her. Thankfully the results, (some of which can take an agonising 2 weeks to come through), are the best we could possibly hope for - a CT scan showed no sign of the tumour, an MIBG scan showed no presence of neuroblastoma anywhere in her body, her urine which can reveal the presence of this particular cancer was normal and both her bone marrow and bone samples were clear. She is therefore now in complete remission.

Last Friday Abbie went up to the Marsden to have her Hickman lines removed so that marks the end of the heavyweight treatment. For the next 6 months she will take an oral drug but that is a joy compared to everything else she's had to take. Remarkably she is the same weight now as she was when she relapsed but her appetite has taken a knock so she is still on an overnight feed, (which goes down her nasal gastro tube), for the next few weeks. This does however make administering her medicines a piece of cake - every child should have one!

Finally on Monday she had her hearing re-checked - it is now thought that Abbie does not require hearing aids, but she has lost a lot of her high frequency hearing, (above 3kHz for the technically minded), so we need to keep a watch on any effect this has on her development.

She spent a few afternoon sessions back at nursery during the Radiotherapy and is now attending 4 mornings a week as life thankfully starts returning to normal. There has been a noticeable change in her personality in the last few days as she begins to enjoy life again. We are now planning some holidays and hope to be able to have a social life again!

April 27th 2004

Abbie has done really well since our last update. She has just finished her 3rd round, (of a total of 6), of Retinoic Acid, a vitamin A derivative, the purpose of which is to kill off any remaining or newly growing Neuroblastoma cells. This treatment, whilst so simple and straightforward compared to everything to date, has improved the survival statistics by 10-15% in the last 10 years.

The capsules, (which we pierce open and mix the contents with fromage frais), are actually the most powerful acne treatment around and the dose prescribed for Abbie is extremely high. As you can imagine as we reach the of each 14 day course her skin is extremely dry and blistered around her mouth but it recovers quickly and it doesn't seem to bother her.  

Abbie's high pitch hearing loss does appear to be permanent but does not seem to be affecting her too much. At the moment we have decided against having digital hearing aids fitted but will ensure that her progress is regularly monitored.

Abbie has now returned to nursery for four mornings a week and is thriving. She had her celebrated her 3rd birthday on 1st March and had a great time at her party with lots of her friends in attendance. Her hair is growing back well, (slightly darker than before) and her fitness and appetite returning. We have also managed to squeeze in a couple of holidays which both Jamie & Abbie thoroughly enjoyed.

November 2004

As Christmas approaches Abbie is on wonderful form. She is an active and very keen member of Glenesk Kindergarten. She has been selected to be a cow in the school nativity play although she is a little disappointed that she’s not a donkey. She is now learning to read and can already write her name which was completely self taught - not bad for a 3 year old. Abbie now attends swimming, dancing and gymnastics which she is really enthusiastic about. Her hair has now completely grown back and is a mass of brown curls rather than the blonde wavy hair she had before. December marks two years since Abbie was first diagnosed. Although that time has gone enormously quickly it seems like another life time ago. We are still attending the Marsden regularly but at the moment everything seems to be OK.

July 2005

Some of you will know that for the past three months, Abbie has had her leg in plaster. On the 21st March following a routine check up at the Marsden where she was given a completely clean bill of health Abbie attended a party at a soft play area. By the evening she was limping. I telephoned her consultant the next morning who said she was in fantastic health and it was probably nothing to worry about but as we were due to go to on holiday to South Africa I decided to take Abbie to casualty. Despite being referred up to the children’s ward and several doctors examining her she was given a clean bill of health but due to her previous medical history she was given a urine test which came back a few weeks later as completely clear. The limping however continued and when we arrived back from holiday she was x rayed and it was found that she had a tibia stress fracture.  Abbie was put in plaster for 2 weeks but after the plaster was removed it was obviously not right and was put back into plaster a few weeks later. After another few weeks her leg x rays looked worse which we felt was because the initial plaster was removed too quickly, and her treatment had been mismanaged, however she was also referred to a pediatrician at the Royal Surrey. On examination he discovered a small lump in her bowel which he believed was constipation so Abbie was put on Lactulose for a week or so. Another urine test was done and we were also referred back to the Marsden. At the Marsden they x rayed her leg and gave her an ultrasound but saw no significant signs of relapse but initiated another urine test. Unfortunately the results from this test indicated her cancer was back and we were told on 22nd June that it was extremely likely that she had relapsed. Mike and myself have been in total disbelief because Abbie has been so incredibly well and had displayed none of the relapse signs that we have encountered before. Abbie spent last week having more tests - a bone and bone marrow biopsy, a CT scan and a MIBG scan. A blood test last week revealed she had low platelets which was a poor sign and at lunchtime today after two incredibly tearful and stressful weeks, Mike and myself received final confirmation that Abbie’s cancer has extensively returned spreading to her leg and bone marrow. There is also Neuroblastoma disease within an enlarged lymph node (4cms) near her left groin.

This afternoon, Mike and myself have been to the Marsden to discuss treatment options with her consultant. As Abbie has relapsed her prognosis is very poor. There are very few children who relapse from Neuroblastoma having had the treatment that Abbie has already received and have survived. We visited the Marsden not knowing if they could offer us any further hope or treatment. Fortunately as she has been off chemotherapy for 21 months there is still a chemotherapy option available to us which gives us a slim hope that we may still be able to save Abbie’s life. It means Abbie will need another Hickman line inserted and entails seven days of treatment every month for 4 months, with 2 nights out of the 7 in hospital. She will lose her hair again and will have to endure the same kind of side effects that she has suffered from in the past. If the chemotherapy works we may also be eligible to start a trial for an Antibody which may teach Abbie to fight the cancer herself. We feel that this is her best option for fighting this awful disease. The antibody is a similar treatment to the one we considered taking Abbie to the States to receive last year. It is so new to the UK that the trial will only be available from the Autumn. We are lucky that treatment options available have already vastly improved in the years since she was first diagnosed with this cancer. On the downside if Abbie does not respond to the treatment initially, she will be taken off chemotherapy and given lower dose care to give her the best quality of life but it will only prolong her life for as long as possible. Abbie will also receive radiotherapy to her leg as soon as possible so that the fracture begins to heal and hopefully she will be able to come out of plaster before the initial chemotherapy commences.

Despite the news today being disappointing it was expected and has put us out of misery. We are now looking forward to the treatment beginning and despite there being a slim chance that it will work has at least offered us a glimmer of hope in a very dark tunnel.  We are also thankful that we have had 18 fantastic months to enjoy Abbie. Other parents we know were not so lucky and never managed to achieve remission first time round. This will be our third attempt.

In light of Abbie’s relapse Mike and myself have decided to have both Jamie and Abbie christened at St Martin‘s church in East Horsley. This has been provisionally booked for 17th July at 12.30. This will go ahead if it does not interfere with her treatment plan. We are hoping that will be a really happy event - a celebration of both their lives. To avoid it becoming to overwhelming for them we will be inviting close family and Jamie and Abbie’s friends and their parents.

24th July 2005

A lot has been happening in the last couple of weeks - so here's as brief a summary as possible of her treatment.

Following our meeting with Abbie's consultant on 4th July we had to wait a whole week before treatment started.

Saturday 9th July a canular was inserted into her arm and bloods taken from it.

Sunday 10th July - Due to a lab error Abbie had to have more blood taken for a blood cross match. The canular would not work so she had to be pricked again. Later in the day she had a blood transfusion through the canular at the Royal Surrey which took seven hours as she had a simmering temperature.

Monday 11th July - Echogram at the Marsden to check her heart can cope with more chemo. Another prick and another blood test because the Royal Surrey forgot to check her blood clotting on Sunday. Blood had to be squeezed out of her arm because they could not get an adequate sample. How upset and angry was Abbie and how furious was I at this point! She then received a platelet transfusion at the Marsden followed by an operation to insert a Hickman line at St Georges. Abbie was so distressed that she went into shock and was sick on the way to St Georges. She then developed a temperature and it was touch and go as to whether she could have the op. Also St Georges tried to cancel the operation as they were running late but luckily the surgeon insisted that they go ahead when her temperature came down.

Tuesday 12th July - Radiotherapy planning ( On arrival I am told they need to tattoo her leg with 4 pricks). Fortunately because Abbie is so upset they decide to use pen marks instead. A last minute radiotherapy slot becomes available so Mike dashes down to the Marsden to help calm Abbie down.

Wednesday 13th to Friday 15th July - daily radiotherapy on her leg - Abbie has to lie still on her own in the radiotherapy suite for 2 x 2 minute periods whilst her leg is irradiated. We are able to see her on video camera and talk to her via microphone.

Monday 18th July - final radiotherapy session to leg, a platelet transfusion, her first chemotherapy of Topotecan and a ultra sound of her leg to check for blood clots.

Tuesday 19th July - Visit to Royal Surrey to get her leg put back in plaster then straight to the Marsden for her second dose of Topotecan plus a blood transfusion.

Wednesday 20th, Thursday 21st July - third & fourth chemotherapy

Friday 22nd - Admitted to the Marsden for 2 nights for her fifth Topotecan plus a 48 hour infusion of two other chemo drugs. We arrive at 9.15am and are told there are no beds available. Also her Topotecan has not been dispensed so we have to wait until 2.30pm before anything happens. The doctor who saw Abbie on Friday neglected to take bloods and so on Saturday when a blood test is done everyone is shocked to realise that her HB has fallen to 5.8 and her platelets are only 5. I don't ever remember her blood count being this low before. Abbie is also now Neutropenic which means she can no longer fight infection. Her low blood count means that Abbie had a blood and platelet transfusion on Saturday and a further blood transfusion on Sunday. On the positive side Saturday night was the first time in about 2 weeks that she did not get a temperature at night. Given that the Neuroblastoma has probably been causing the temperatures this is a good sign. Also we had been told that we would have to take her straight form the Marsden to Royal Surrey to start antibiotics as she is neutropenic and febrile but luckily as she didn't spike last night we are now at home.

Abbie has been very traumatised by the treatment and last week we felt she was beginning to go downhill. She had been excessively tired and withdrew into herself with her sparkle well and truly gone. Last weekend some large bruises appeared on her left leg and thigh - these were partly due to her low platelet levels, the cancer has also been progressively kicking in with her left eye drooping slightly, night sweats, temperatures and constant nagging pain, particularly at night which has prevented her and us sleeping well. On Sunday she seemed to hit rock bottom and hardly moved all day and completely stopped eating. Over the last couple of weeks her left thigh has been swelling up and it was decided on Monday she should have an ultrasound to check there wasn't a blood clot. It has turned out to be another enlarged lymph node in her leg so yet another sign of the cancer.

The last four days have been much more positive - she is eating small amounts has been more cheerful and now needs very little in the way of pain relief. Her leg is back in plaster following the radiotherapy and she is delighted with the luminous pink plaster the Royal Surrey ordered especially for her! She spent Wednesday afternoon at a party on very good form and on Thursday afternoon spent 2 hours at Holiday Club at Glenesk. Mr. Bishop tells me that she was the perkiest he has seen her for some time so hopefully that means the chemo is working.

We have now completed this cycle of chemo and need to wait until her blood counts recover before repeating the chemo cycle in about 3 weeks time. We are hoping that the chemo will begin to work and reduce the enlarged lymph nodes. If her leg improves we know that the chemo is probably working. We have arranged a meeting with Andy Pearson on Wednesday morning. He is now head of the children's dept at the Marsden in Sutton and is also the UK's leading neuroblastoma expert and probably the leading expert in Europe. I briefly met him on Friday without Mike and he tells me that Abbie is the first relapsed neuroblastoma patient at the Marsden to be offered these phase 2 trial drugs and probably the first child in the UK.

We did not know until last Tuesday that the radiotherapy and chemo could not be given together so we cancelled Abbie & Jamie's christening last week thinking that we would be on the ward. We have now postponed it until 14th August. In actual fact it was just as well as Abbie was so poorly last Sunday. I am really hoping that by 14th August her leg might even be out of plaster.

Finally Claire Attard and other friends are organising a big Fund Raising Event for Neuroblastoma Research on 21st September, so if you live locally please keep it free. Details to follow soon.

Thanks for all the letters, cards, texts, e mails etc etc. We really appreciate having such fantastic support from our friends.