Sarcoma Awareness Week Part One

Sarcoma is a type of cancer that I know all too well. This week is all about raising awareness of this cancer type and the inspirational people that have fought it/are fighting it. I hope you find this weeks blog posts interesting and informative.

What is Sarcoma?

Sarcoma’s are rare cancers that develop in the supporting tissues of the body, for example muscle, bone and nerves. They are some of the most common childhood cancers. About 55% of sarcoma’s affect the limbs and about 15% affect the head and neck, the remainder will be found in the abdominal area inside your body.

There are three main categories of Sarcoma: Soft tissue cancer, Primary bone cancer and Gastro-Intestinal Stromal tumours. There are also about seventy different sub types of sarcoma which fall into these three main categories. Osteosarcoma falls under the primary bone cancer category.

Sarcoma UK

Sarcoma UK is the main UK charity dealing specifically with all types of sarcomas. In March 2011, The Sarcoma Trust and Sarcoma UK officially joined together to become known collectively as Sarcoma UK.

Sarcoma UK has one main aim: to get the best possible level of treatment and care for patients suffering from sarcoma. They achieve this through three different means:

·         They fund scientific and medical research into discovering what causes sarcomas and how to treat them

·         They deliver various types of support and information services which cover all aspects of sarcoma

·         They raise awareness of sarcoma with members of the public, healthcare professionals and policy makers.

The fact that sarcoma affects a lot of children is horrifying, especially when it could mean having limbs amputated. Georgie had to have his jaw replaced; such a horrific and painful experience for anyone, let alone a sixteen year old boy. However not many people are aware of this type of cancer, I wasn’t until it affected someone close to me.

Sarcoma UK has a small team of hard working staff who work alongside doctors, nurses, researchers and other cancer charities. It has a board of trustees, many of whom have had personal experience with sarcoma. It is a fantastic charity working hard to bring awareness to such a terrible form of cancer. It is so important to bring awareness to such a worthy cause and to get this charity as much publicity as possible. It provides much needed support and information to sarcoma patients and their families as well as much needed research into ways of preventing and curing the disease.

Sarcoma UK relies on voluntary donations as its sole means of funding their hugely important research projects into sarcoma. If you know of a way of fundraising for a charity then I really do urge you to bear Sarcoma UK in mind. Every little really does help in the battle against sarcoma cancers.

Osteosarcoma and Georgie

Osteosarcoma was the type of cancer that killed Georgie. He was fifteen when his jaw swelled up. At first he had a numb lip and weird tingling sensation. It took a while to get to the bottom of what was wrong but we eventually found out it was a tumour in the right mandible. After years of treatment and numerous surgeries, Georgie passed away just months before his eighteenth birthday.

On average thirty children per year in the UK develop osteosarcoma. This kind of cancer is more common in teenagers and is usually found in boys. It is rare to see it in a child under the age of five.

Osteosarcoma starts in the bone, usually at the end where new bone tissue forms as you grow. Any bone in the body can be affected, with Georgie it was the jaw, but it is commonly found in the arms and legs. The knee joint is a particularly common site for osteosarcoma.

The cause of osteosarcoma is unknown at the moment but there are some risk factors to consider: people with Li Fraumeni Syndrome are at more at risk of developing osteosarcoma, as are children who have hereditary retinoblastoma. If a child has had radiotherapy or chemotherapy in the past then they also have an increased risk.

INJURIES THAT DAMAGE BONES DO NOT CAUSE OSTEOSARCOMA. THEY CAN, HOWEVER, DRAW ATTENTION TO ANY TUMOURS THAT ARE ALREADY THERE.

Pain in the bone is the most common symptom of osteosarcoma. This may be on and off for a while at first and then become more severe and constant over time. Swelling may also occur. The bone can weaken and break, which is how many osteosarcoma cases are discovered. Most symptoms can be caused by something else so it is hard to know when to go to the doctor but if your child has persistent pain and swelling in the bone – I would recommend taking them for a check up.

To diagnose osteosarcoma, the doctor will do a series of tests including x-rays. If a bone tumour is suspected then a specialist will be needed to perform further tests such as biopsies. A physical examination of the affected area and a blood test may also be required. Bone scans, MRI’s and CT’s may also be required.

Treatment for osteosarcoma would depend on the size, position, grading and stage of the tumour. Surgery is usually a very important part of the treatment as is chemotherapy. Chemotherapy is usually used to destroy the cancer cells and shrink the main tumour before surgery is performed. It is then used again when surgery has been performed to blast away anything microscopic that may have been left behind. This is to try and reduce the chance of the cancer returning.

Radiotherapy may also be considered as it can destroy cancer cells with high energy rays but do little damage to the normal surrounding cells.

Surgery would depend on the size and position of the tumour. The whole limb may need to be amputated, especially if the cancer has spread to the surrounding nerves and blood vessel. A prosthetic limb will be fitted. Obviously this is a major thing and I don’t want to appear to not take this seriously. Your child’s doctor should discuss this in a great amount of detail with you and both you and your child should receive lots of support if amputation is the only option.

In other cases, limb sparing surgery can be used to preserve the limb. This may involve replacing the limb with a bone graft using part of another bone in the body or it may involve replacing the bone with prosthesis. Georgie has his jaw replaced with another bone part TWICE.

Even with amputation, it should be possible for your child to adjust and still be able to participate in “normal” activities and sports are still a possibility. Long term effects should be discussed with you before surgery.

Osteosarcoma can be a very aggressive type of cancer, and unfortunately, survival rates are not very good. I have a particular hatred for this cancer type and I am hopeful clinical trials and research will discover new ways of treating, curing and preventing osteosarcoma. Please do visit my uncle’s site www.anticancer.org.uk for more information as he is a fountain of knowledge on the subject, and as a parent of a child, who had cancer, he is well informed and able to explain things much better then I ever could.

This is just an introduction to the theme I will be exploring this week but please contact Sarcoma UK if you are worried about anything you have read here today.

Sending lots of love and positive thoughts to anyone currently battling osteosarcoma or anyother sarcoma cancer.

Georgie: Two Years On

Greetings from Cyprus!

My birthday is now hugely bittersweet because it also marks the day Georgie died. Tomorrow will be two years since his death and I struggle massively with the fact that I get another year older on the day he stopped living. Everyone does their best to make the day nice but I was very close to Georgie and I can't help but think of him, as I do everyday. 

I loved Georgie very much. I still talk about him lots and I think about him all the time. He is still very much a part of my everyday life with this blog and my voluntary work but there is definately a hole in my world without him. I don't think that will ever change, he is simply irreplaceable. It's hard to find the right words so I will keep this blog post nice and short. I just wanted to publicly acknowledge what will be a very bittersweet day for me.

Love you Georgie. Missing you more and more everyday xxx

Cancer Treatment: Photodynamic Therapy

One of my best memories of Georgie is a weird one. He was undergoing photodynamic therapy (PDT) at the UCH Teenager Cancer Unit and I went to visit him there after work. Whilst he was having the treatment he couldn’t be exposed to light so we had to sit in the dark. We ate dinner in relative darkness and spent the evening just me, him and my Uncle Pan. I like to talk to my Uncle about all things politics and that sort of thing whilst Georgie and I had a shared love of One Tree Hill and we all support Chelsea FC so there was no shortage of things to talk about. It was actually one of my favourite evenings and I always think of it when I think of Georgie.

I’m going to focus on PDT today and I hope this post provides you with some interesting information about a treatment option which is not yet as well known as others such as chemotherapy.

Photodynamic Therapy

PDT is a treatment mostly used for non melanoma skin cancer cases but it can also be used to treat other cancer types. It is also known as photo radiation therapy, phototherapy and photo chemotherapy. It combines a drug, known as a photosensitizer, which makes cells sensitive to light and exposure to a particular type of light. There are different types of photosensitizing agents and each is activated by a light on a specific wavelength. Different types are used to treat different body parts.

How PDT Works:

The sensitizing drugs produce a type of oxygen that is able to kill nearby cells when they are exposed to their particular light. This directly kills cancer cells and may also be able to shrink and destroy tumours in other ways, possibly by damaging the blood vessels in the tumour to prevent it receiving the nutrients it needs to survive. PDT may also possibly trigger the immune system so it can attack the cancer cells. It can be used as part of a combination of treatment with surgery, chemotherapy, radiotherapy or biological therapy.

Treating Skin Cancer with PDT

PDT is sometimes used to treat cases of non melanoma skin cancer. A cream containing the light sensitive chemical is applied to the skin cancer and surrounding area, although it can also be used in tablet or injection form. A strong light is shone on the area for up to forty five minutes once the chemical has been absorbed and the light kills any cell that has absorbed the drug.

Treating Other Cancer Types with PDT

Research indicates that PST may be able to treat some cancer types that are found inside the body. It is mainly used to shrink larger tumours that are blocking the airway or food pipe and it can also be used to treat cancers found in the head and neck area, the oesophagus, and on the lining of internal organs. Georgie has osteosarcoma of the mandible (jaw) and PDT was used for this. The light used for PDT can only pass through about 1cm of tissue and can be used to relive symptoms and help the patient breath or swallow better. It can be used to treat patients in the very early stages of lung cancer or oesophageal cancer if the patient is not well enough to have surgery or chemotherapy.

For internal cancers, the light sensitizing drug is injected into your blood stream, normally via a cannula. Cells all over the body absorb the drug but it usually lasts longer in cancer cells so about two or three days after receiving the chemical, a light will be shone to activate the drug usually via an endoscope. You may need local or general anesthetic as the laser light is switched on when the tube reaches the cancer.

PDT Side Effects

Your skin and eyes may be sensitive to light for up to six weeks after the treatment due to the light sensitizing drugs used. Direct sunlight and bright indoor light will need to be avoided for around six weeks after the therapy and skin may become very sensitive if it is exposed to light around this time period. PDT can cause some burns, swelling or scarring to nearby healthy tissue. Other side effects can include coughing, difficultly swallowing, stomach pains, and breathlessness but this is usually temporary.

I hope you have found this informative. I know it sounds scary but it did seem to help Georgie whilst he was having it. To find out more, please visit www.cancerresearchuk.org

xxx

Blog Post 200: International Rare Disease Day 2013

Today is International Rare Disease day. I am going to use this day to raise more awareness for  rare conditions that have affected people I know and care about. Some of this information will have been covered in this blog before but it’s always good to go over things and refresh memories as well as raising awareness for newer followers of this blog!

On a personal note, this is my 200th post on this blog!!! Thank you so much to everyone who reads it and to everyone that provides me with encouragement, support and great post ideas. I would also like to use this post to publicly thank David Collins for his unwavering support and guidance. David is a very humble man but he is someone I admire greatly and look up to. Thank you David x

George Pantziarka and Li Fraumeni Syndrome

Of course I have to mention my beautiful Georgie. He was a Li Fraumeni sufferer and I want to raise awareness of this condition in the hope that it saves others from a similar fate.

The TP53 Gene:

The TP53 gene acts as a tumour suppressor. It creates the p53 protein which kills cancerous cells. When the TP53 gene fails, the body loses one of its key ways of stopping damaged cells developing into cancer. Some people with cancer find their TP53 gene is damaged within the tumours but is functioning normally throughout the rest of their body. However, some people are born with a damaged TP53 gene. This is usually an inherited problem and it passed down from parent to child. These people have a very very very high risk of developing cancer.

Li Fraumeni Syndrome:

LFS is one of the most serious kinds of inherited TP53 disorders. It is a rare disorder that greatly increases the sufferers risk of developing several kinds of cancer especially children and young adults. The most common cancers associated with LFS are: breast cancer, osteosarcoma (a type of bone cancer) and soft tissue sarcomas.

Diagnosing Li Fraumeni Syndome:

LFS is rare so not much is known about it. Genetic testing and genetic counselling are used to confirm whether or not someone is suffering from LFS. If it is confirmed then this person will need to be regularly screened to check for cancer. The sad fact is, people with LFS are very likely to develop cancer so it is hugely important for them to be screened as cancer is easier to treat the earlier it is diagnosed.

Please look into genetic testing if you meet the following criteria:

An individual with:

·         Adrenocortical cancer at any age

·         Choroid plexus cancer at any age

·         Rhabdomyosarcoma below 5 years

·         Breast cancer below 30 years old (if eligible for BRCA1/2 testing, this should be performed first)

·         Two or more primary cancers of the following: sarcoma, breast, brain, adrenocortical or any childhood cancer; at least one below 46 years old

Two relatives affected with:

·         Sarcoma, breast, brain, adrenocortical or any childhood cancer; one relative below 36 yrs and one relative below 46 years old

Three relatives affected with:

·         Sarcoma below 45 years old and

·         any cancer below 45 years old and

·         any cancer below 45 years old or sarcoma at any age

Georgie and Li Fraumeni Syndrome:

Georgie’s mum died when Georgie was one years old. She was 29 years old and had ovarian cancer. Georgie was then diagnosed with cancer a few months later on his second birthday. At the time it was written off as a horrific coincidence by doctors. It wasn’t until Georgie was diagnosed with cancer for the third time at the age of fifteen that we discovered he had Li Fraumeni Syndrome which had probably been inherited from his mother.

It took a very long time for Georgie to be diagnosed with the osteosarcoma that eventually killed him. At first he was told it was a dental problem. Had we have known he had LFS; he could have been diagnosed much quicker as alarm bells would have been ringing. We will never know if this could have saved his life. We live with the “what if?”

LFS and other TP53 disorders are rare and not much is known about them. There is little awareness about them amongst the general public and even the medical profession. There aren’t any dedicated support groups or charities, no information resources and no registry of the sufferers. There isn’t a community for the sufferers which can make it very isolating, lonely and terrifying for the sufferers and their families.

As I have mentioned before, my uncle has set up the George Pantziarka TP53 Trust to honour Georgie’s memory by providing support to families and individuals, to provide information on TP53 disorders including LFS and to get research funded to look into the condition.The forum on the website which is used to bring together people affected by the condition. Please do take a look and feel free to ask any questions – my uncle is always happy to answer!

For more information on TP53 and Li Fraumeni Syndrome, please visit www.tp53.co.uk

Dawn Green and Pesudomyxoma Peritonei

I have mentioned my beautiful fellow CRUK Ambassador and friend Dawn Green on this blog before. She is literally Wonderwoman and I am in awe of her.

In 2008 Dawn was diagnosed with Pseudomyxoma Peritonei. 

Pseudomyxoma Peritonei is a very rare type of cancer that usually begins in your appendix as a small growth, called a polyp. Or, more rarely, it can start in other parts of the bowel, the ovary or bladder. This polyp eventually spreads through the wall of your appendix and spreads cancerous cells to the lining of the abdominal cavity (the peritoneum). These cancerous cells produce mucus, which collects in the abdomen as a jelly like fluid called mucin.

Dawn was given just three months to live and the cancer was so rare that her doctors didn’t know much about it at all. Whilst fighting the disease Dawn noticed a distinct lack of emotional support for people fighting this form of cancer. 

Dawn decided to set up a charity to help others in her situation. Pseudomyxoma Survivor aims to provide emotional support for anyone dealing with this type of cancer and to prevent anyone feeling alone or isolated during their fight against the disease.

The charity fundraises and raises the profile of PMP whilst also funding research into the disease. Dawn is an incredible patient advocate for all survivors, and this results in increased awareness and higher survival rates worldwide.

If you would like to know more about this type of Cancer or Dawn’s charity then please check out her website, I will post the link below. If you are interested in getting involved then I’m sure she’d be happy to hear from you!

A cancer diagnosis is terrifying, especially when it’s a very rare cancer such as this one. More needs to be done to understand these rarer types of the disease so cure’s can be found and people can be saved. Dawn recently found out that her cancer has returned but thankfully it is slow growing. I wish her so much luck and I am sending so many positive thoughts and lots of love her way. I think Dawn is incredible and the World needs people like her. She is a tough cookie and she won’t let cancer beat her. We’re all with you every step of the way beautiful Dawn.

http://www.pseudomyxomasurvivor.co.uk/

xxx

My Beautiful Cousin Georgie xx

CLIC Sargent - The Great Mum's Get Together

Happy Wednesday one and all!

One of the charities I support wholeheartedly is CLIC Sargent. They helped Georgie so much when he was ill and since his death I have found support with them too. They kindly set up an Especially for You Fund in Georgie’s name so his memory can help other children and young people suffering from cancer. They are also very supportive of my fundraising and campaigning efforts as well as this blog. They are a a fantastic charity who do incredible things for young cancer sufferers and their families.

March is the month of Mother’s Day. A time when mum’s get to be centre stage and we take time to appreciate what they do for us. CLIC Sargent have a mum based even taking place in March and I wanted to highlight it here and encourage everyone to take part if they can!

This March see’s CLIC Sargent’s flagship event for mums takes place. The Great Mums Get Together asks mums across the UK to get together and have a cuppa for children and young people with cancer.

So whether you feel like hosting an indulgent afternoon tea, a pamper night or simply turning your usual coffee morning into a fundraiser, it all makes a difference. You can sign up to take part at www.clicsargent.org.uk/gettogether or for inspiration and ideas join the Great Mums Get Together Facebook page http://www.facebook.com/pages/Great-Mums-Get-Together/133197136737766.

To read more about CLIC Sargent please click http://pennysophia.blogspot.co.uk/2012/09/clic-sargent.html

To read more about the George Pantziarka CLIC Sargent Especially for You Fund please click here http://pennysophia.blogspot.co.uk/2012/10/the-george-pantziarka-especially-for.html

If anyone would like to host a Great Mums Get Together and raise money for the George Pantziarka Especially for You Fund to directly benefit CLIC Sargent, please  let me know!

CLIC Sargent is a hugely worthwhile charity to support and I know from personal experience that their fantastic work helps thousands of families through one of the hardest situations. No child should have to face cancer but I’m very glad a charity like CLIC Sargent exists to help those that do sadly have to fight this horrific disease.

xxx

Childhood Cancer Awareness Month – Cancer Types: Osteosarcoma

This is a very emotional blog post for me as osteosarcoma was the type of cancer that killed Georgie. He was fifteen when his jaw swelled up. At first he had a numb lip and weird tingling sensation. It took a while to get to the bottom of what was wrong but we eventually found out it was a tumour in the right mandible. After years of treatment and numerous surgeries, Georgie passed away just months before his eighteenth birthday.

On average thirty children per year in the UK develop osteosarcoma. This kind of cancer is more common in teenagers and is usually found in boys. It is rare to see it in a child under the age of five.

Osteosarcoma starts in the bone, usually at the end where new bone tissue forms as you grow. Any bone in the body can be affected, with Georgie it was the jaw, but it is commonly found in the arms and legs. The knee joint is a particularly common site for osteosarcoma.

The cause of osteosarcoma is unknown at the moment but there are some risk factors to consider: people with Li Fraumeni Syndrome are at more at risk of developing osteosarcoma, as are children who have hereditary retinoblastoma. If a child has had radiotherapy or chemotherapy in the past then they also have an increased risk.

INJURIES THAT DAMAGE BONES DO NOT CAUSE OSTEOSARCOMA. THEY CAN, HOWEVER, DRAW ATTENTION TO ANY TUMOURS THAT ARE ALREADY THERE.

Pain in the bone is the most common symptom of osteosarcoma. This may be on and off for a while at first and then become more severe and constant over time. Swelling may also occur. The bone can weaken and break, which is how many osteosarcoma cases are discovered. Most symptoms can be caused by something else so it is hard to know when to go to the doctor but if your child has persistent pain and swelling in the bone – I would recommend taking them for a check up.

To diagnose osteosarcoma, the doctor will do a series of tests including x-rays. If a bone tumour is suspected then a specialist will be needed to perform further tests such as biopsies. A physical examination of the affected area and a blood test may also be required. Bone scans, MRI’s and CT’s may also be required.

Treatment for osteosarcoma would depend on the size, position, grading and stage of the tumour. Surgery is usually a very important part of the treatment as is chemotherapy. Chemotherapy is usually used to destroy the cancer cells and shrink the main tumour before surgery is performed. It is then used again when surgery has been performed to blast away anything microscopic that may have been left behind. This is to try and reduce the chance of the cancer returning.

Radiotherapy may also be considered as it can destroy cancer cells with high energy rays but do little damage to the normal surrounding cells.

Surgery would depend on the size and position of the tumour. The whole limb may need to be amputated, especially if the cancer has spread to the surrounding nerves and blood vessel. A prosthetic limb will be fitted. Obviously this is a major thing and I don’t want to appear to not take this seriously. Your child’s doctor should discuss this in a great amount of detail with you and both you and your child should receive lots of support if amputation is the only option.

In other cases, limb sparing surgery can be used to preserve the limb. This may involve replacing the limb with a bone graft using part of another bone in the body or it may involve replacing the bone with prosthesis. Georgie has his jaw replaced with another bone part TWICE.

Even with amputation, it should be possible for your child to adjust and still be able to participate in “normal” activities and sports are still a possibility. Long term effects should be discussed with you before surgery.

Osteosarcoma can be a very aggressive type of cancer, and unfortunately, survival rates are not very good. I have a particular hatred for this cancer type and I am hopeful clinical trials and research will discover new ways of treating, curing and preventing osteosarcoma. Please do visit my uncle’s site www.anticancer.org.uk for more information as he is a fountain of knowledge on the subject, and as a parent of a child, who had cancer, he is well informed and able to explain things much better then I ever could.

My heart it with you if you or someone you love is battling osteosarcoma or if you have lost someone to it like I have.

As always, for my wonderful warrior Georgie

xxx

Childhood Cancer Awareness Month: The Long Term Effects of Childhood Cancer

So the good news is, more and more children are surviving childhood cancer. Survival rates beyond five years after diagnosis are improving all the time, and many more children will survive cancer then ever before.

Doctors try to make sure children with cancer don’t have long term problems following cancer treatment. However, some things can be affected and I’m going to summarise some of those possible effects in today’s blog post.

Possible Effects on Education and Intellectual Development.

Before I start, I would like to reassure people that Georgie suffered cancer three times and sat his GCSE’s whilst having intense treatment yet he still managed to get MUCH better GCSE’s results then I did (and mine were pretty good!).

The majority of children with cancer should be able to carry on with a normal education and should develop intellectually as normal.

Some children, usually those with brain cancer, can develop learning difficulties and may require special help at school but this would depend on their age and treatment type.

Your child’s doctor will be able to advise you on any special educational requirements and your child’s school should be able to help you find a way to carry on with your child’s education. I know Georgie’s school were incredibly understanding and helped him out in lots of different ways.

Possible Effects on Growth and Development.

The pituitary gland is found at the base of the brain and produces hormones which regulate growth and development throughout childhood. Radiotherapy to the brain may affect this and if a child doesn’t produce enough of these hormones their growth will be affected. A man made hormone may be needed to help the child grow.

Radiotherapy can have an effect on growth and development, for example, if a child has radiotherapy on their leg, it may be shorter in length then the other.

It is important that your child’s growth and development is monitored regularly and any signs of their growth and development being affected will need to be investigated. Replacement growth hormone may be necessary.

Possible Effects on Heart and Lungs.

Certain types of cancer treatments, including chemotherapy drugs and radiotherapy, can affect the heart and lungs. Regular echocardiogram should be used to monitor the child’s heart and lung function tests may also be necessary. Sometimes the effects are not seen until long after the treatment has finished so regular monitoring is needed for a while after treatment.

Possible Effects on Kidneys.

Some chemotherapy drugs can lead to kidney problems but if your child has those particular drugs then their doctor will arrange tests to monitor and check their kidneys every so often. Any kidney problems are usually not severe and if your child hasn’t had any problems during treatment then it is unlikely they will develop any problems related to their treatment later in life.

Possible Effects on Puberty and Fertility.

I've mentioned cancer and fertility before. I work in a fertility clinic in Harley Street and I have learnt lots about the subject.

I can’t imagine that worrying about your child’s future fertility is going to be high up on your list of worries should they be diagnosed with cancer. It is important to try and find out if their fertility will be affected as it will need to be dealt with at a later date and could have an impact on them emotionally when they grow up. It can be very very distressing to consider your child’s fertility, especially at such a difficult time but being in the know about their treatment and its affects will help in the long run.

Your child will be checked for sighs of puberty at the time of diagnosis. If puberty is delayed or has not happened then hormone replacement will be needed so that puberty can occur when it’s time.

If you have any more questions regarding cancer and fertility please do read my blog post on the subject. There is a box on the side of the page with details of my workplace if you wish to consult a fertility specialist.

http://pennysophia.blogspot.co.uk/2012/05/cancer-and-fertility.html

Possible Second Cancer.

A very small number of children can be cured of cancer but then develop another type of cancer later on in life. It is important to know that this can be caused by a inherited gene mutation, whilst it is also sometimes caused by the cancer treatments themselves. Your child’s doctor can discuss this possibility with you but I urge you to remain vigilant, even when your child is given the all clear. Demand the doctor’s attention and through testing if you think a second cancer is a possibility.

Please read my blog post on gene mutations, especially Li Fraumeni Syndrome, as this is what led to Georgie being diagnosed with two further cancers after being cured of his first. If you feel you are at risk of a genetic mutation then please look into genetic counseling or visit www.tp53.org.uk for more help, support and advice.

http://pennysophia.blogspot.co.uk/2012/11/gene-mutations-and-cancer.html

http://pennysophia.blogspot.co.uk/2012/06/blog-post-100-tp53-and-li-fraumeni.html

As always, my thoughts are with anyone watching their child go through cancer. My love is sent to all the children suffering from cancer. This blog post is dedicated to my beautiful Georgie.

xxx

Childhood Cancer Awareness Month - Diagnosis and Treatment

Another blog post focusing on childhood cancer today! This time I’m going to be breaking down the diagnosis and treatment of childhood cancer.

As my family is well aware, there is a wide range of syndromes that run in families which can lead to an increased risk of a child developing cancer. Li Fraumeni Syndrome is just one of them and it was this syndrome that led to my cousin being diagnosed with cancer three times in his short life. When Georgie was a baby and was diagnosed with cancer for the first time, my uncle asked his doctors if it was linked to his mother, who had died a few months before. The doctors told my uncle it was just a horrific coincidence and nothing to do with genetics. It wasn't until Georgie was fifteen and diagnosed with the cancer that killed him that we discovered he has Li Fraumeni Syndrome and it had probably been inherited from his mother. It took a while for Georgie to be diagnosed this time and we have to live with the fact that had we have known he had LFS, he may have been diagnosed a lot quicker and this could have saved his life. Unfortunately for us, we will never know.

Luckily, huge advances have been made in recent years and more and more people are having genetic testing and genetic counseling to see if they have any inherited factors that could increase their risk of developing cancer. For example genetic testing for families with a history of retinoblastoma has become standard procedure which is a huge step forward.

Please do read my blog post about gene mutations for more information on this.

As I have said above, huge and significant advances have been made in relation to childhood cancer, especially when it comes to treatment and mortality rates. This is mainly down to the introduction of standardized protocols in clinical trials as well as the centralization of care.

There are many organisations and groups who co ordinate trials into childhood cancer and look into various issues surrounding it. These include the Children’s Cancer and Leukaemia Group (CCLG) which my friend and fellow ambassador Neil is part of. I’m going to ask him to do a guest blog post on this subject.

At the moment there are around thirty groups which have been set up to research specific tumour types and other disciplines surrounding childhood cancer.

There are twenty one specialist centres dedicated to looking after children with cancer in the UK and Ireland. These have been shown to significantly benefit these children by forming a close knit network. Each of these twenty one units is a centre of excellence and have multidisciplinary teams of specialists with experiences of treating the specific tumours seen in children. These centres are:

*Royal Aberdeen Children’s Hospital

*Royal Hospital for Sick Children Belfast

*Birmingham Children’s Hospital

*Royal Hospital for Sick Children Bristol

* Addenbrooke’s Hospital Cambridge

*Children’s Hospital for Wales Cardiff

*Royal Hospital for Sick Children Edinburgh

*Royal Hospital for Sick Children Glasgow

*St James’ University Hospital Leeds

*Leicester Royal Infirmary

*Alderhey Children’s Hospital Liverpool

*Royal Manchester Children’s Hospital

*Great Ormond Street Hospital

*University College London Hospital

*Royal Victoria Infirmary Newcastle

*Queens Medical Centre Newcastle

*Queens Medical Centre Nottingham

*John Radcliffe Hospital

*Sheffield Children’s Hospital

*Southampton General Hospital

*Royal Marsden Hospital Sutton

*Our Ladies Hospital for Sick Children Dublin

Treating Childhood Cancer:

The typical treatment for the majority of childhood cancers is surgery, chemotherapy and radiotherapy. Here is a summary of treatment options of some of the cancers seen in children:

*Acute lymphoblastic leukaemia and acute myeloid leukaemia– Usually Chemotherapy combined with steroids. Donor stem cells or bone marrow can be considered for patients with resistant ALL or those that have relapsed. Radiotherapy is sometimes used as well.

*Hodgkins lymphoma – Sometimes surgery is used to remove the tumour if it is at stage one but the main treatment is usually chemotherapy and radiotherapy.

*Non-hodgkins lymphoma – There are many different types of NHL and treatment will depend on what type the child has. Normally treatment is radiotherapy, chemotherapy and biological therapy although stem cell and bone marrow transplants can also be used.

*Brain and CNS tumours – If surgery is feasible then this is usually the preferred treatment option but chemotherapy can also be used. If the child is under three years of age then radiotherapy should be the last resort as it can damage the child’s still immature brain.

*Neuroblastoma – if the tumour is localized the surgery is usually the first option. Chemotherapy before and/or after surgery is also an option as is a stem cell transplant.

*Retinoblastoma – Smaller tumours are treated with laser treatment to the eye. Cryotherapy and thermotherapy are also options for smaller tumours. Larger tumours are usually treated with chemotherapy, radiotherapy or surgery to remove the eye. A combination of treatments is sometimes used.

*Nephroblastoma – Surgery to remove part or the entire kidney is an option as well as chemotherapy and radiotherapy before and/or after surgery

*Osteosarcoma – The main treatment option is surgery to remove the tumour and surrounding tissue. Sometimes amputation is necessary. Chemotherapy is usually used before and after surgery. Radiotherapy can also be used.

Osteosarcoma is the cancer that killed Georgie. He had it in his jaw. He had various treatments throughout his illness. He had his jaw removed and replaced twice but unfortunately microscopic cells were left behind and the tumour simply grew back. He also had chemotherapy and photo dynamic therapy amongst many other treatments.

*Rhabdomysarcoma – surgery is the main treatment option and chemotherapy or radiotherapy is usually used before and/or after surgery

Georgie was diagnosed embryonal rhabdomyosarcoma in the left temporalis muscle on his second birthday. He had a small dent in the side of his head after it was removed. He had chemotherapy through his “wiggly” (hickman line) and several other treatments and he managed to beat this cancer, although he was given a very low chance of surviving the disease.

New treatments such as immunology are being developed all the time and ways of treating childhood cancer is evolving.

Childhood cancer survival rates are improving all the time, and more children then ever are surviving. There is still a hell of a long way to go and unfortunately many children are still dying, my beautiful cousin included. It is important to know that there are many people out there dedicated to trying to find ways of preventing, treating and curing childhood cancer. I am very much looking forward to the day when childhood cancer becomes a very very rare occurrence.

If you have a child that is suffering from cancer, or you are a child with cancer, my heart is with you and I wish you nothing but the best.

For Georgie – I miss you more and more every single day xxxx

Childhood Cancer Awareness Month

December = Childhood Cancer Awareness Month in the UK. I don’t think I need to mention why this cause is so close to my heart but I do feel it is important to get the facts and figures out there for people to see. So here are some facts about childhood cancer for you:

The cancers seen in children are usually very different to those seen in adults and “childhood” refers to any child aged between birth and fourteen years old.

There are twelve main types of childhood cancer:

*Leukaemia

*Soft Tissue Sarcomas

*Kidney Tumours

*Brain and Central Nervous System (CNS)

*Bone Tumours (Like the one Georgie had)

*Carcinomas and Melanomas

*Retinoblastomas

*Gonadal and Germ Cell Tumours

*Liver Tumours

*Sympathetic Nervous System Tumours

*Other and Unspecified Tumours

Childhood cancer is quite rare and makes up 5% of all cancers. Around 1,600 children in the UK are diagnosed with cancer every year, which roughly works out at thirty one children per week. Around one in every five hundred children in the UK will be diagnosed with cancer.

The UK’s childhood cancer rates are amongst the lowest in Europe, with Northen Europe having the highest incidence rate.

Leukeamia is the most common childhood cancer. Two thirds of all childhood cancers are  leukeamia, brain and CNS tumours and lymphomas.

Surviving Childhood Cancer:

More children then ever are surviving cancer. The survival rate has doubled since the 1960’s and at least 5,600 MORE children now survive for more then five years after diagnosis.

Around 33,000 people in the UK have survived a type of childhood cancer and almost three quarters of children with cancer can now be cured of the disease.

For every ten childhood cancer sufferers – eight will now survive for more then five years after their diagnosis:

*Nearly all children diagnosed with retinoblastoma are cured.

*Survival rates for Hepatoblastoma have doubled since the 1960’s.

*Around six out of ten children diagnosed with neuroblastoma are cured.

*Eight out of ten children with kidney cancer survive the disease.

*Rhabdomyosarcoma survival rates have doubled since the 1970’s.

Childhood cancer deaths:

Cancer is the UK’s leading cause of death (from disease) in children aged up to fourteen. A fifth of all childhood deaths are down to cancer.

Brain and CNS tumours are the most common fatal type of childhood cancer.

Around two hundred and fifty children die from cancer every year in the UK.

Childhood cancer death rates have halved since the 1960’s.

Causes of Childhood Cancer:

We don’t know much about what causes childhood cancers but there are several things we do know:

*Rare genetic syndromes such as Li-Fraumeni Syndrome (what Georgie had) can greatly increase a child’s risk of developing cancer.

*Children with Down ’s syndrome have a greater risk of developing leukaemia.

*2/5 retinoblastomas are linked to a faulty gene that has been inherited.

*Children that have had radiotherapy or chemotherapy in the past are at greater risk of developing a second cancer (As Georgie did)

Blog Posts About Childhood Cancer:

Georgie Pantziarka – http://pennysophia.blogspot.co.uk/2011/10/georgie-pantziarka-xx.html

Mitchell Huth – http://pennysophia.blogspot.co.uk/2012/07/mitchell-huth.html

Harry Moseley - http://pennysophia.blogspot.co.uk/2011/12/childhood-cancer-awareness-month.html

Little Star Awards - http://pennysophia.blogspot.co.uk/2011/11/twinkle-twinkle-little-star.html

Retinoblastoma - http://pennysophia.blogspot.co.uk/2012/01/katy-bishop-petition-to-publish-signs.html

Joss Searchlight - http://pennysophia.blogspot.co.uk/2012/02/joss-searchlight.html

Ellie’s Fund - http://pennysophia.blogspot.co.uk/2012/02/ellies-fund.html

Li Fraumeni Syndrome - http://pennysophia.blogspot.co.uk/2012/06/blog-post-100-tp53-and-li-fraumeni.html

Georgie Hillman - http://pennysophia.blogspot.co.uk/2012/11/georgia-hillman-little-star.html

Neuroblastoma - http://pennysophia.blogspot.co.uk/2012/07/cancer-types-neuroblastoma-in-children.html

CLIC Sargent - http://pennysophia.blogspot.co.uk/2012/09/clic-sargent.html

The George Pantziarka Especially For You Fund - http://pennysophia.blogspot.co.uk/2012/10/the-george-pantziarka-especially-for.html

Having watched a loved one fight childhood cancer, my heart breaks for every family going through the same thing. Sending lots of love to all the incredible children out there fighting cancer. 

xxx