Aftercare
Why?
Aftercare is essential to ensure that late effects and relapses are recognized and treated as early as possible.
One of the KinderAugenKrebsStiftung’s main concerns is to ensure that our children remain healthy and well cared for in the long term. To this end, we regularly attend congresses and training courses – we pass this information on to you and keep you up to date.
The following information is particularly valuable for families whose children have been diagnosed with a gene mutation.
Aftercare bilateral RB
PRINCIPAL QUESTION
The question facing everyone who is confronted with a diagnosis of bilateral retinoblastoma is: What and how much do I want to know – for myself – for my child?
We are convinced that there is a minimum level of knowledge that both parents and those affected (depending on age) should have themselves.
This consists of knowing the risks in order to be able to develop mindfulness and attention and enable early diagnoses.
This includes open communication between parents and child as well as intelligent self-observation.
THE BIOLOGY OF RB
It is important to understand the biology of RB in order to be able to deal with the diagnosis of bilateral RB properly and responsibly:
In people with bilateral retinoblastoma, one copy of the RB gene is usually defective in all body cells.
This means that there is one healthy copy of the RB gene and one defective copy in each cell. In the cell, the RB protein is responsible for preventing the cell from dividing indefinitely (“stop signal”).
In people with the bilateral form of retinoblastoma, only one gene produces this protein – in healthy people, two genes produce this protein.
So if one gene fails, the protein continues to be produced.
However, because one copy is defective, the probability that no more RB protein will be produced is greater, because not two genes have to lose their function, but only one.
As I said, without the stop signal, this one cell can then divide unhindered, which can lead to cancer. BUT if this cell is intercepted and destroyed by the immune system before it can harm the body, the body can recognize these cancer cells and destroy them.
A strong immune system is therefore important!
STUDY SITUATION
Studies have shown that various secondary primary tumors occur more frequently in certain phases/time windows.
These studies are difficult for the layperson to interpret correctly and are difficult to transfer to individual cases because some of the data is old, cohorts are very small and questions are unspecific. We are happy to make these studies available and also offer regular Zoom meetings on this important topic.
Information on this and on the available studies can be requested by emailing info@kaks.de.
One thing is certain:
- People with bilateral retinoblastoma have an increased risk of skin cancer (see also “Skin cancer prevention”).
- Preventive care in the form of regular skin screening once a year is simple and extremely sensible!
- Osteosarcomas, i.e. bone cancer, occur more frequently during puberty because bone growth is particularly strong at this time.
The most important thing in this phase is attention and self-observation in cooperation with the child.
The child should be able to observe its body and report changes to parents and doctors.
Important: Bone pain is normal during puberty and is not uncommon as growing pains even in “healthy” adolescents. - Whole-body MRIs as screening: some doctors recommend whole-body MRIs for early detection and screening.
This is a very individual decision, as the stress for the children can be high and lead to worries that may be unnecessary.
The studies on MRIs for early detection only show a demonstrable benefit in people who have been irradiated.
ONE or TWO whole-body MRIs may be useful in order to have comparative images later. - X-rays should be avoided, for acute injuries and accidents as well as at the dentist and orthodontist.
Alternatives are MRI or ultrasound.
FUTURE OF CANCER TREATMENT
One thing is certain, and this should be a source of great encouragement to all those affected: We are in the age of precision medicine, which opens up completely new treatment options for cancer. Effective therapies and early detection will lead to far fewer people dying from cancer.
Prof. Dr. Michael Hallek from the University Hospital of Cologne, one of the most renowned oncologists in Germany
“The treatment of cancer is currently undergoing a radical change that is unprecedented in history. We will be able to control many cancers in the future with the drugs and procedures we are developing and researching today. This is brilliant and fascinating.”
Aftercare unilateral RB
In around a third of children, the disease affects both eyes (bilateral retinoblastoma).
If the latter is the case, this is almost always an indication of a hereditary retinoblastoma.
The tumors can either form at only one location in the eye (unifocal) or at several locations (multifocal).
The treatment of choice is initially local treatment.
Individual small retinoblastomas can be safely destroyed with local forms of therapy(laser coagulation, cryotherapyor brachytherapy).
However, repeated application is often necessary.
If the tumors are already too large for such treatment, in some cases chemotherapy can be carried out with the aim of reducing the size of the tumor (chemoreduction) in order to subsequently enable local treatment (i.e. laser, cryotherapy or brachytherapy).
However, the disease is often so advanced in one of the two eyes that it does not make sense to preserve the eyeball and enucleation is therefore performed.
In cases where chemotherapy is being considered for the treatment of the better eye, it is sometimes possible to wait until the more severely affected eye is enucleated.
This is because the therapy can lead to a strong regression of the tumor, so that treatment that preserves the eyeball is still possible.
However, if the worse eye is already blind or there is infiltration of the anterior segment of the eye or the optic nerve, there is no alternative to enucleation.
If the disease also progresses in the second, initially better eye (tumor infestation of the optic nerve or choroid; vitreous seeding), percutaneous irradiation is often the only treatment available to preserve the eyeball.
Again, this is only carried out on the condition that sufficient vision is preserved.
If this is not the case, the second eye must also be removed so as not to endanger the child’s life.
As the risk of modern chemotherapy appears to be lower than that of radiotherapy alone, efforts are now being made to avoid percutaneous radiotherapyif possible, particularly in the first year of life.
However, the effectiveness of percutaneous radiotherapy for retinoblastoma, which is very sensitive to radiation, remains undisputed.
RB-World App
Learning and benefiting from each other – for good provision throughout life. KAKS has formed a working group for you to improve care for RB survivors affected on both sides.
We want to develop a platform to collect experiences, medical information and important contacts and make them available to everyone.
We started with our adult RB survivors, who have already gained a great deal of experience in their lives.
They and we would like to share these with you and integrate your important experiences as well.
We use the RB-World app for this and are constantly developing our documents there as “living documents” with your support and participation in the project.
If you would like to take part, please contact Katja König in the app (kkh@kinderaugenkrebsstiftung.de).
RB register
The Retinoblastoma Registry, headed by PD Dr. Petra Ketteler from the Department of Pediatrics III Pediatric Hematology and Oncology at Essen University Hospital, has been in existence since November 1, 2013.
Unlike with all other types of cancer, this has not yet been done for retinoblastoma and is an important step towards systematically collecting information on retinoblastoma in the form of a database in order to record data and optimize treatment methods.
As retinoblastoma is a very rare disease, there is little data available for a risk-adapted evidence-based selection of different therapies, such as removal of the eye and various eye-preserving treatments such as icing, laser treatment, radiation or chemotherapy.
These treatment options vary in their success rates and range of long-term outcomes.
The data collected in the registry serves to better inform affected patients and parents and forms the basic prerequisite for the development of interventional clinical studies with the aim of improving the early detection, treatment and aftercare of affected patients.
The database and the existing biobank also support accompanying research projects in the field of retinoblastoma.
Important hotline: If there are any questions from the pediatric oncology side regarding the correct aftercare, there is a hotline available at Essen University Hospital: 0201-723-2003.
Parents and doctors who have questions about the aftercare of their patients or children can contact this hotline.
You can find all important information about the RB register under this link.
OPILATER
What happens after surviving cancer?
Survivors have to deal with various consequences of the disease, but are often inadequately informed about them.
The “Optilater” consortium, initiated by Prof. Uta Dirksen and Prof. Viktor Grünwald and coordinated by the WTZ Essen, aims to change this and improve the long-term care of people who have or have had cancer throughout Germany.
You can find all important information about the RB register under this link.
Interview with Prof. Dr. med. Uta Dirksen.
Everything for good aftercare after cancer.
What happens after surviving cancer?
What care needs are there depending on age after a cancer diagnosis? Survivors have to deal with various consequences of the disease, but are often inadequately informed about them.
RB & skin cancer
Dr. Benedikt Hofmann, specialist in dermatology, explains skin cancer prevention for RB patients.
Patients with the hereditary form of RB have an increased risk of developing melanoma compared to the normal population and should therefore take UV protection more seriously.
Here is a current one from 2021, a relatively large study with data from around 1800 RB patients in the follow-up: https://pubmed.ncbi.nlm.nih.gov/34153328/
Even if the mechanisms of increased tumor development are not yet fully understood, minimizing the carcinogenic effect of UV light on the skin of young patients is urgently recommended.
This applies in particular to patients with hereditary RB, especially after chemotherapy and radiotherapy and the presence of other risk factors (light skin type, familial risk of atypical navi or melanoma).
The patient’s parents are usually aware of this thanks to the exemplary information provided by KAKS.
But how do we best protect the skin of young patients?
Physical sun protection is absolutely correct, including good (tested!) UV-protective clothing and the usual sun rules.
If a stay outdoors with somewhat more intensive UV insolation is unavoidable or if UV-protective clothing is no longer worn, then the free skin should be protected with a sufficient amount (!) of SPF 30-50.
In most cases, the SPF is applied too thinly and therefore never reaches the values of the specified maximum light protection capability.
The SPF should also protect against UVB and UVA.
For an adult without an increased risk of skin cancer, a factor of 30 is usually sufficient.
For patients with an increased risk, and unfortunately this includes RB patients, an SPF 50 should be used.
Of course, according to the current UV index.
With an index of 3, an SPF 20-20 would normally be sufficient, with a UVI of 8-10, 50 would of course be advisable.
On the question of the health risks of the higher SPF products in relation to the ingredients. All SPFs approved in the EU show no indication of a health risk when used correctly.
Rather, there is greater damage due to DNA damage to the skin that occurs and can be proven if UV protection is not adequate.
The risk assessment of the Federal Office for Risk Assessment and the Federal Office for Radiation Protection clearly positions itself on the basis of the scientific data. What we do not recommend are SPFs with a high proportion of nanoparticles, which whiten less but whose effects on human tissue have not yet been 100% clarified.
Hormonally effective SPFs are no longer approved in the EU.
Here is information on the LSF from the BFR: https://www.bfr.bund.de/cm/343/sonnencreme-und-co-gibt-es-gesundheitliche-risiken.pdf
Two other aspects are important: UV-induced DNA damage occurs even before the first signs of sunburn. Therefore, please apply prophylactic sunscreen regularly depending on the UV index.
Please do not forget to give vitamin D to young patients when applying UV protection consistently.
Without UV, no vitamin D…
Skin cancer – the dark side of sunlight
The feeling of light and warmth on the skin and the brightness evokes a sense of well-being in most people that we find very difficult to escape. However, sunlight does have a downside: the ultraviolet radiation it contains.
When consumed in excess, this causes damage to skin cells, which in the worst case can be the cause of skin cancer. Of course, our body can repair some of this damage itself.
However, this wonderful property unfortunately works much less efficiently in children and young people than in adults.
This is the reason why sunburns in adolescence are a major factor in the rising rate of skin cancer in adulthood. We should protect ourselves.
UV protection is important.
Unfortunately, UV damage occurs even before a sunburn “warns” us.
Proper UV protection starts earlier, in the mind first!
We need to be aware of the damaging effects of UV radiation and not underestimate them. Observing the UV protection rules is the best precaution and has been proven to help prevent the development of skin cancer.
The most important sun protection rules
- Avoid strong sun, especially at midday.
- Dress – the best and simplest protection is UV protective clothing and headgear.
- Apply sunscreen generously, at least sun protection factor 30. Apply plenty of sunscreen half an hour before going out in the sun and repeat several times a day.
- Protection when bathing, preferably UV swimwear.
- No sunbeds, is prohibited by law for children and young people in Germany!
- Babies should never be exposed to the blazing sun!
Sun cream should be avoided in the first year of life. - Be a role model!
And set a good example.
The most important precaution.
We don’t even need a doctor for the simplest precaution: our skin is right in front of our eyes and we can look at it at any time as a family or partner.
If moles change significantly from the overall picture of existing moles or show abnormalities according to the simple ABCDE rule you should specifically show them to a dermatologist. This simple form of mindfulness can save lives.
ABCDE rule
The ABCDE rule helps with the self-examination of pigment spots for the early detection of skin cancer:
A = Asymmetry: asymmetrical growth, not round or oval
B = Boundary: uneven, blurred, smooth change to normal tissue
C = Color: unevenly strong pigmentation or multicolor
D = Diameter or dynamic: rapid growth, often enlarged diameter (5 mm or larger)
E = Elevation: growth nodular in height or newly formed on otherwise flat ground
Of course, an examination by a dermatologist is safer.
All people with statutory health insurance are currently only entitled to skin cancer screening by a dermatologist from the age of 35.
Some insurance companies offer this from the age of 20.
The reason for this is that the rate of skin cancer increases significantly from this age.
Mind you, the rate of skin cancer, not the rate of precancerous lesions.
These can already develop in adolescents.
And this is particularly the case with vulnerable skin types.
This is where the preventive care provided by statutory health insurance companies falls short and unfortunately does not differentiate between risk groups.
However, every dermatologist will also carry out skin cancer screening for non-eligible patients at a reasonable cost as a self-pay service.
RB & X-ray
8-year-old Julia is at the orthodontist to get her first braces.
12-year-old Paul twisted his ankle playing soccer today and has severe pain in his ankle.
Four-year-old Emma has fallen on her shoulder on her bike and can’t move it.
These are stories of everyday parenting that don’t always prevent a visit to the doctor and the associated X-ray.
But is that really the case?
From bone fractures and joint inflammation to suspected cancer: radiological methods have become indispensable for doctors. However, there are differences that we need to be aware of and that are particularly important for children with a hereditary form of retinoblastoma.
Because the following applies to them: AVOID RADIATION!
The most important imaging techniques are the classic X-ray, computer tomography (CT) and magnetic resonance imaging (MRI).
X-ray and CT are based on the same principle: in both, the body is illuminated with X-rays, so to speak.
The MR tomograph, on the other hand, produces an image of the inside of the body with the help of a strong magnetic field and radio waves.
X-rays are most commonly used to examine broken bones and joint problems.
But beware: “X-rays have biological effects and can cause cancer. But the emphasis is on can, because the increase in the normal risk of cancer is in the range of less than one per thousand,” explains Michael Wucherer, radiation physicist at Nuremberg Hospital: “The lower the radiation dose, the lower the risk. The dose depends on several factors. First of all, which parts of the body are to be imaged is decisive. The desired accuracy also plays a role, and depending on the question, an upwardly adjusted radiation dose must be used.” (Source: welt.de) A computer tomography examination is also associated with radiation exposure.
In contrast to normal X-ray examinations, CT can also be used to produce three-dimensional images that provide far more information than two-dimensional X-ray images.
The following also applies here: when using ionizing X-rays, the benefits and risks must always be weighed up!
And this is especially true for children with an RB mutation.
Parents should always discuss individually with the doctor concerned whether an X-ray examination is necessary or can be replaced by another examination method, such as MRI.
Or whether it can be dispensed with altogether under certain circumstances.
According to our research, this is often the case with dentists or orthodontists.
Magnetic resonance imaging MRI works without ionizing radiation.
It is based on the fact that all water molecules in the body behave like tiny magnets that react to an externally applied magnetic field.
If they are then excited with the help of radio waves, they emit energy when the radio source is switched off.
A computer then calculates the image from the large number of individual signals, each of which depends on the nature of the tissue in question and its surroundings.
MRI is considered a very safe and harmless method, as the body is not exposed to any radiation apart from the comparatively low-energy radio waves.
However, due to the strong magnetic field, the procedure is taboo for people who carry certain metals or, for example, pacemakers in their bodies.
The fact that the patient lies in a narrow tube for a long time makes the examination uncomfortable for many people.
The tomograph is a high-tech device that is extremely expensive to purchase and maintain.
Hospitals and doctors therefore only use MRI selectively, as each examination costs many times more than the other methods.
CONCLUSION: Minimize the use of X-rays!
This information should be known to anyone who might come into situations with their child where X-rays might be necessary – whether in a private, school or leisure environment.
The KAKS would like an individual X-ray pass with an additional note for RB patients.
In addition to any X-rays taken to date, this would indicate any measures taken due to the RB disease.
This way, in an emergency, the doctors providing care can act with foresight and, if necessary, dispense with a further X-ray of the RB survivor.
Pediatric oncology in Germany and Austria
The following list shows the locations of treatment facilities where you can find a pediatric oncologist for your children for RB aftercare – in addition to aftercare at the ophthalmologist.
Clicking on the links will take you to the respective websites of the treatment facilities.
If you would like to search more closely in your area, you can find help here:
CHILDREN’S ONCOLOGISTS NEAR ME
If there are any questions from the pediatric oncology side regarding the correct aftercare of RB patients, a hotline is available at Essen University Hospital: +49(0)201 7232003. Parents and doctors who have questions about the aftercare of their children or patients can contact this hotline.
Coordination office for psychosocial aftercare
The coordination center for psychosocial aftercare for families with children suffering from cancer is a very valuable facility, as KONA provides support for all questions and issues that arise after cancer.
The aim is to enable patients and all family members to enjoy a good quality of life.
The offer is varied:
Counseling: KONA has experience and knowledge in the field of childhood cancer and can therefore provide specific counseling and support in psychosocial aftercare and offer appropriate assistance.
Events: KONA offers experiential educational events for children and adolescents (patients and siblings), and group meetings and weekends for parents and families to exchange ideas and information.
Training and information: On topics relating to cancer in children, e.g. dealing with the disease for specialist agencies or other interested institutions (e.g. kindergarten, school).
Youth & Future project: advice and support on the subject of training and careers.
Coping with illness: How can I cope better with my fear of recurrence?
Is it normal that I still worry so much?
Who can I talk to about it?
How can I manage to lead a self-determined life despite the consequences of the disease?
Kindergarten, school: Which school is right for my child?
Is my child entitled to compensation for disadvantages at school and how can I obtain this?
Where do my child’s concentration problems come from and what can I do?
Exhaustion: What options are there for me to relieve stress in everyday life?
How can I recognize my own needs again?
Behavioral problems: Why is my child wetting again?
Why does my child react so aggressively?
Partnership, family, social environment: Where can I find support for problems in my partnership?
How do I deal with the lack of understanding from those around me regarding my child’s illness?
Do other parents feel the same as me?
Therapy options: Does my child need psychotherapy?
What is the most suitable therapy for my child, especially after a brain tumor?
How can I finance riding therapy?
Social law: Should I extend my severely disabled person’s pass?
Do I have the option of applying for a second inpatient rehabilitation program?
Financial problems: Where can I get help with my financial difficulties?
Grief: Where can I find help and understanding in my grief?
Information: Can there be late effects in my child?
What can I do as a precaution?
Training and career: How can I find a suitable training position even though I have health restrictions?
Should I state in my application that I have had cancer?
Elli’s Eyeland by KAKS
Our team will work hard for you with a great deal of personal commitment.
We are happy to answer your questions and are there for you.