An emotional journey but sometimes light hearted look at living with Aspergers and Teenage Cancer from Ryan's point of view, aged 15 and that of his mum, a little bit older. Join us on our winding and bumpy road trip down Lymphoma Road, stopping at Autism Central.
Yesterday 5th August 2017 we hit a new milestone. Three months post chemo and cancer free. I can't believe the progress Ryan has made, it has astounded even me. The doctors are impressed and I'm impressed. His hair is growing back beautifully. It just looks like a really short crew cut now. He has put on a wonderful amount of weight that puts him now absolutely on the typical average scale for his height and age. He also no longer has Adrenal Insufficiency either which surprised even the doctors that he recovered so quickly.
We now need to go and buy him new clothes because he's grown so well and improved so much and I’ll need to buy new school uniform ready for September. Ryan’s sleep patterns are still a mess though, to the point he is almost nocturnal and that's not for want of trying to form some sort of routine by waking him in the mornings and letting the dogs into his room, but he does seem to tire still quite easily and he did manage to pick up a cold a couple of weeks ago because he went to school for the last day of term.
Ryan spent the final day in school with his friends because they weren't doing any work particularly and it was just nice to him to touch base before going back in September. He seemed to enjoy the experience when he came home and he was he was quite good, considering. He was less tired than I expected him to be, but as the night went on and in the early hours of the Friday morning he developed a stinking cold and quite a temperature so I rang his oncology nurse on Friday morning just to check things because I knew he was going to get ill at some point, I just didn't expect him to get ill after just one day in school.
I just wanted to check the protocol now that he is post chemo, is it just treat as a normal cold with rest fluids and meds, which it was, but because he had such a high temperature the nurse did want the consultant to see Ryan at Clinic but Ryan steadfastly refused as he says he's had enough of hospitals to last him a lifetime and he didn't want to go because every time we’ve been they have kept him in. The nurse wasn't particularly happy with me but I did promise that I would monitor him closely and if I was at all concerned I would take him back. I am happy he has just a genuine cold and nothing more serious which unfortunately all 4 of us ended up getting, but although three of us have now recovered Ryan is still got it lingering on, so it shows his immune system is still quite weak.
Ryan is happiest at home with our dogs.
We've been asked several times if we're going away for the summer what our plans are and as I've tried to explain to everyone that asked our home is Ryan’s safe place. He doesn't like going away anyway and as we've spent so much of the last year in the hospital in Cardiff away from home to have a staycation is more appealing to us than the additional stress of organising and going away somewhere different outside of his comfort zone. Ryan wouldn't cope with a change of scenery anyway because of his autism not to mention the three dogs.
We've been offered the use of a caravan by one of the Kids Cancer Charity but the unknown and upheaval that it would have meant for Ryan put him into distress so it was decided that it would be better not to go away at this stage, Instead to let him recuperate at home where he feels the most relaxed.
I genuinely didn't think we would get this point. The last two years and eight months have been a blur of stress. Yet coming to the end of treatment coincides perfectly with Cancer Survivors Day on June 4th so what better way to mark this day than with our own survivor story.
As a result of the steroid chemotherapy that Ryan has been on for his Lymphoma treatment, he has developed Adrenal Insufficiency. This is hopefully a temporary condition that will improve once chemotherapy treatment has finished, however, only time will tell.
There are two types of Adrenal Insufficiency. Primary and Secondary.
Ryan’s type is secondary and can be traced to a lack of ACTH, a hormone which causes a drop in the adrenal glands production of cortisol but not aldosterone. This form of secondary adrenal insufficiency has occurred after receiving a glucocorticoid hormone, in Ryan's case, Dexamethasone steroid as part of his Cancer treatment.
Cortisol is normally produced by the adrenal glands, which are located just above the kidneys. Cortisol has many functions, but its most important job is to help the body respond to stress. The high dosage use of Dexamethasone to treat Ryan's lymphoma has caused a hormonal imbalance which hopefully will rectify itself as Ryan’s body begins the healing process once treatment finishes.
Some sources advise that the body needs approximately one month of healing for every month of treatment it received for cancer. Ryan had two months of Radiotherapy and 6 months of Chemotherapy so we are looking at 8-12 months of post treatment healing, but the doctors have already forewarned us that Ryan faces 2-3 years of maintenance ahead.
The symptoms of adrenal insufficiency usually begin gradually. Chronic, worsening fatigue and muscle weakness, loss of appetite, and weight loss are characteristic of the disease. Nausea, vomiting, and diarrhoea occur in about 50 percent of cases. However many if not all of these are also common in Cancer and we had put Ryan's symptoms down to chemotherapy side effects.
A random blood monitoring test done in hospital, because Ryan was vomiting despite anti sickness medication showed severe Hypoglycemia, (or low blood sugar) which warranted further tests that discovered the adrenal diagnosis.
Because the symptoms progress slowly, they are usually ignored until a stressful event like an illness or an accident causes them to become worse. This is called an adrenal crisis, or acute adrenal insufficiency. In most patients, symptoms are severe enough to seek medical treatment before a crisis occurs. However, in about 25 percent of patients, symptoms first appear during an adrenal crisis which is what happened in Ryan’s case.
Symptoms of an adrenal crisis include sudden penetrating pain in the lower back, abdomen, or legs; severe vomiting and diarrhoea, followed by dehydration; low blood pressure; and loss of consciousness. Left untreated, an adrenal crisis can be fatal. Ryan was diagnosed by a ACTH Stimulation Test. In this test, blood and/or urine cortisol levels are measured before and after a synthetic form of ACTH is given by injection. In the so called short, or rapid, ACTH test, cortisol measurement in blood is repeated 30 to 60 minutes after an intravenous ACTH injection. The normal response after an injection of ACTH is a rise in blood and urine cortisol levels. Patients with either form of adrenal insufficiency respond poorly or do not respond at all. In Ryan’s case, he responded poorly.
Ryan is being treated now with hydrocortisone tablets three times a day and we have been trained in using an injection crisis kit in the case of any future adrenal crisis. He has to carry a steroid alert card at all times and during any time of illness or infection he is required to double his normal daily dose of tablets called a stress dose, for the duration of the illness or infection.
We, fortunately, have not yet had to use the crisis injection, but we have been required to up his dose during a recent 10 day hospital stay between chemotherapy cycles, for a staphylococcus infection.
I can never thank the doctor on the Teenage Cancer Trust Unit in Cardiff Hospital for trusting her gut that day and requesting a random blood monitoring test be done. Up until this point, I had assumed his constant vomiting was down to the effects of chemo, but the doctor disagreed as he was on 2 different types of anti-sickness medication. She had a hunch. She saved Ryan’s life.
With this being our penultimate cycle I have had several family and friends comment to me “oh I bet you can't wait to get back to normal”. This has really thrown and upset me because it seems to come across that they have failed to comprehend the fact that we have 2-3 years of maintenance ahead, at least 2 more general anesthetic surgeries. Not to mention physiotherapy as it is expected Ryan will take 12+ months to heal from the effects of being poisoned from the inside out. Normal is long way off yet.
While everyone else appears to all be getting excited that Ryan is coming to an end of his chemotherapy protocol treatment, Ryan himself is becoming more and more tired with the cumulative effect of the onslaught of the chemotherapy drugs, and is spending longer periods of time in hospital after each cycle with infections due to his impaired immune system now as a result. Ryan is far from excited. Ryan is exhausted.
We still have one more cycle to get through, but he is already worrying about the cancer coming back. He says it will be ‘just his luck’ after all the other problems he has faced in his short life. He is so worried in fact that he asked me in the car the other day if he would be allowed to refuse treatment if it does come back. I can not even describe to you the heartbreak that gave me.
Homeschooling was finally agreed to start on March 20th, but Ryan was ambulanced to Cardiff that day with his Adrenal Crisis, so has yet to start with the tutor. Missing an entire year of school is a issue and dilemma that we will also have to face before life can ‘get back to normal’. Do we put Ryan back a year, into an unknown cohort of peers that have already settled and made their friendship groups or do we keep Ryan in the same school year with his friendship group, but not enter him for any GCSE’s as he has missed to much work now to be able to catch up. I will be discussing this topic in a separate post at a later date.
Ryan was anemic when his was discharged from hospital at the end of cycle 5 and we only got to spend 2 nights at home before he was then admitted to our local hospital on the 3rd night with vomiting, temperature of 39.1C and rigors. Not quite as impressive as he Adrenal Crisis of 40.1C, but still up there in his top 3. His anemia had worsened as expected so he was given his first ever blood transfusion. His blood group is the same as mine A+, he said that is the highest mark he will ever get on any test in his life. Nice to see his sense of humor hasn’t diminished.
The timings of his admission however and need to 10days IV antibiotics meant that we got to spend Easter in hospital which transpired to have it’s benefits as Ryan was inundated with chocolate eggs and treats and his mother treated him to a McDonald's double sausage and egg muffin every morning to help with the calories. She is such a sucker…
The timings of cycle 6 are as such that Ryan’s final chemotherapy will be given on older brother Owen’s 18th birthday. Owen is therefore traveling to Cardiff that day with his girlfriend to have a party on the Teenage Cancer Trust ward and then we will treat him and the lovely Ffion to a cheeky Nandos in Cardiff Bay which is something to look forward to.
Since being discharged from Carmarthen I have got myself somewhat more organised and now make Ryan homemade double sausage and egg muffins every morning to aid his recovery and weight gain.
Ryan completed chemotherapy cycle 4 of 6 in March. We were discharged and managed a week at home before he was unfortunately admitted to Carmarthen hospital with febrile neutropenia. This is a whole body reaction to an infection when there is a low immunity due to chemotherapy. His temperature reached 40.1C and his lymphocyte white blood cell count was 0.2. When the count falls below 1, this is called neutropenia and infections become a high risk.
Neutropenia is very common in cancer patients and we have done well to avoid an episode up to this stage. The standard oncology protocol is to spend a minimum of 48 hours on IV antibiotics and 7-10 days on growth hormone injections to support the recovery of the white blood cell count. It didn’t go quite as smoothly as this however for Ryan, but that is whole other story that will be told later.
Chemotherapy is likely to cause a low white cell count - particularly neutropenia. If you or a patient you care for presents with any form of infection while undergoing chemotherapy treatment it is urgent they receive a same day full blood count and that you know to look out for the following:
If neutrophils <1.0 and the patient is febrile (>38C on one occasion or >37.5C on two occasions taken half an hour apart), admission to hospital is required for IV antibiotics.
If neutrophils >1.0 and the patient is febrile but well, consider oral antibiotics after checking with GP/Local Hospital/Oncology team.
If neutrophils are >1.0 and the patient is febrile and clinically unwell please liaise with your emergency contact team.
If you are ever unsure. Get checked. It is the age old ‘better to be safe, than sorry’ scenario. Chemotherapy is well known to lower your resistance to infections. When you have lower than normal white blood cells your body is less able to fight infections. When you are neutropenic it is strongly recommend to not spend long periods of time in crowded places, such as cinemas, shopping centres, swimming pools etc. It is also sensible to avoid close contact with any family or friends who have obvious coughs, colds, flu etc.
You should take it very seriously if you do develop an infection whilst on chemotherapy and for up to four weeks after treatment. Signs of an infection are:
A temperature above 38C on one occasion or above 37.5C on two occasions during a half hour period.
A sore throat.
A chesty cough.
An upset stomach.
Needing to pee more often or pain when going.
Feeling generally unwell.
Redness, discharge or shivery episodes after use of hickman or other central line.
Don’t delay seeking advice and treatment. Infections are a SERIOUS risk and can become life threatening very quickly if ignored.
Fortunately Ryan recovered well from this latest blip and we put it down as just another bump on our badly paved trip, down Lymphoma Road.
Ryan's lymphoma is being driven by the Epstein-Barr Virus which causes glandular fever. The EBV invaded his T-cells whilst he had Glandular Fever and turned them cancerous causing dysplastic lymph cells to grow in his throat resulting in a soft tissue tumour forming.
The good news at the start of cycle 4 was that his EBV plasma readings had come back as zero for the 2nd month, which tells us that no more cancerous cell are being made in his bone marrow. There are still EBV cells in his blood so hopefully this cycle and next 2 cycles will finish killing off the cancer cells that are still in his blood. Ry will be given remission status when the EBV plasma has been zero for 6 months and still faces at least two years of maintenance ahead after the six chemo cycles have finished.
The week of SMILE chemotherapy went well although he had some impressive fluid retention on day 4. This can be a sign that that kidneys are not working as well as they should be, so he has had some extra tests to make sure everything is working as it should be.
We had only been home for 2 days when Ryan went routinely to the toilet and I heard him swear from downstairs. I ran up to him and he asked me if it was ok that his pee was dark green. Please don't judge me when my first response was to giggle before I then reached for my phone to text his oncology nurse. It is another sign of kidneys being a bit tired and we were advised to ensure he drinks plenty to wake them up a bit.
Things took a bit of a turn though a week after getting home, when Ry developed an infection which unfortunately hospitalised him as he didn't have enough immunity to fight it without a bit of help. What we expected to be the usual standard 48 hours of IV antibiotics turned into an ambulance transfer to Teenage Cancer Trust unit at Cardiff hospital for a 10 day course of IV antibiotics and a unfunny time with non diabetic hypoglycemia that no one was initially able to work out why.
After numerous test and several days on a glucose drip Ryan was diagnosed with steroid induced Adrenal Insufficiency, I was given training on how to treat the new addition to Ryan's health list and we were finally allowed to go home after a 13 day stay, for a weekend break before chemotherapy Cycle 5 and the onslaught begins all over again.
I will cover more detail about the adrenal problems in a separate post.
The whole of April is given over to Autism Awareness Month with hundreds of activities and events taking place to raise awareness and much needed funds for charity. It begins with World Autism Awareness Week taking place every year from 27th March and cumulates with World Autism Day celebrated on 2nd April each year. The week aims to raise awareness, with educational events, exhibitions, social media posts and discussions, bringing people from all walks of life together.
Autism is a lifelong developmental condition that affects how a person communicates with and relates to other people. It is also a spectrum condition, which means that, while all people with autism share certain difficulties, their condition will affect them in different ways. Asperger syndrome is a form of autism. Many people with autism are of average or above average intelligence. Some people with autism experience over (hyper) or under (hypo) sensitivity to sounds, touch, taste, smells, light or colours. People with autism often become extremely anxious in unexpected and unfamiliar situations. You can read more about what autism spectrum condition (ASC) is here.
The so called campaign group ‘Autism Speaks’ uses the tagline ‘Light it up blue’ for Autism. However the organisation has come under increasing fire and scrutiny recently regarding their approach to wanting to cure autism as a disease. This stance has angered many families and individuals calling for boycotts of their campaigning. I personally do not agree with this mind set or manifesto, but regardless of your opinion of this particular group, they have certain got the world talking about this emotive topic.
There are many ways in which you can get involved and raise awareness whether you choose to ‘Wear Red Instead’ for the group Actually Autistic or you could choose to be ‘Positively Purple’ in support of the National Autistic Society (NAS). I am a member of the NAS and am extremely grateful for the support and guidance I have received from them since Ryan’s diagnosis in 2008. I personally choose however to not wear any particular colour, but instead talk, talk and talk some more to anyone and everyone who will lend me half an ear.
As far as I am concerned you can wear any colour you like, so long as you open up conversations, increase exposure and move towards global acceptance. Take part in, organise or sponsor; walks, bakes, fancy dress, auctions, raffles, quizzes, fun runs. The list is endless and you are only limited by your imagination in whatever you fancy to spread the word for this unique way of life. The more unique and different the better. Celebrate the differbility as opposed to a disability.
It matters not how you get involved, just get involved. Comment below to let me know how you advocate for autism awareness, or join the chat on social media with the hashtags #autismawareness and #waaw
Education is the most
powerful weapon which you can use to change the world. ~ Nelson Mandela.
The aim of our blog is to
educate and help just 1 person to support their child better. We hope to change perceptions of Autism and living with an invisible challenge; but
with so many myths and misconceptions out there it really is a 1 step
forward, 2 steps back process, although that is half the fun of constantly
challenging ourselves to find new ways of getting our message across.
Contrary to popular belief
many autistic children do make eye contact; it just may be less or different
from a non-autistic child. Many children with autism can develop good
functional language and others can develop some type of communication skills
such as sign language or use of pictures.
Children do not ‘outgrow’ autism but symptoms may lessen as the child
develops strategies to cope.
One of the most devastating
myths about autistic children is that they cannot show affection. While sensory
stimulation is processed differently in some children with Autism they can and
do give affection, but it may require patience on a parent’s part to accept and
give love on the child’s terms.
The above extract is taken
from an Autism Spectrum Disorders Fact Sheet distributed by the Centre for
Developmental Disability Health, and this is very much the case with Ryan. It is only in the last 2 years that he has started to give me hugs, but even now they have to be on his terms and initiated by him. He is fifteen now and I still live for the day I get a kiss. He has a number of sensory issues but with
the exceptional support he has received in secondary school and from my unending drive to
support and offer him extensive therapy he has made astounding progress in his
ability to cope and deal with being autistic to the point where his symptoms have
lessened so much some people who meet him for the first time are actually
surprised to discover his diagnosis. Don't get me wrong every now and then he has to throw us a curve ball just to keep us on our toes and remind us just how much effort he has to put in to appearing to function 'typically'.
So what have we done to get
to this point other than work on crushing each myth as we encounter it?
Wore wellies permanently for
a time (yes even in summer) while we worked on getting him to wear shoes.
Wellies are ‘roomy’ where as shoes were too constricting for him. He still to
this day does not like trainers, he finds them far too tight.
Wore bright orange
industrial strength ear defenders for a time or at least took them everywhere
with us so he had the option of putting them on if he became unable to cope (if they went on that was a visual clue to change the environment or risk a meltdown).
Took sunglasses out with us and also to school. They were very accommodating because he
doesn’t cope well with the fluorescent strip lights in school.
Kinaesthetic toys like stress
balls and stabilo writing pens.
Good choice / Bad choice
cards and major use of social stories by Carol Gray.
Egg timers that show visual
count-down to needing to do something with buzz at required time. Worked really well for Ryan.
Autism alert card that he
carries with him to show people.
Invested in a locator
devise as he had a major tendency for bolting. Not cheap, but who puts a value
on child safety. Well worth the money, we certainly got our monies worth on several occasions.
School have included him in
social skills classes and Circle of Friends support groups.
Intensive Speech and Language Therapy. I particularly like Black Sheep Press resources such as Speech Bubbles program.
SPELL framework: Structure;
Positive (approaches); Empathy; Low arousal; Links (between all components and aspects
of your child’s life).
Make use of & encourage participation
in clubs and groups both in school and out: 5x60, lunch time teacher led,
Engage in and encourage
child’s interests. Ryan loves 3 things in his life other than animals. Lego,
PC gaming and Photography.
On day while on our journey home from school Ryan said the following to me in the car,
once more crushing a few misconceptions:
I think I am
learning about emotions mum. We were playing truth or dare at lunch time and I
could see one of the boys getting upset. He looked like he was going to cry so
I told the others to stop the game and we went to do something else instead.
I told him how proud I was
and explained how he is learning social clues and non-verbal communication
skills. He added:
Yes I now know
what a happy face, angry face, sad face and funny face looks like now. I think
I am finally understanding them.
To coincide with national Nutrition and Hydration week, I thought it would good to share with you Ryan’s experience of malnutrition as a result of his lymphoma diagnosis.
Malnutrition is a common problem among patients with cancer, affecting up to 85% of patients with certain cancers. In severe cases, malnutrition can progress to cachexia, a specific form of malnutrition characterised by loss of lean body mass, muscle wasting, and impaired immune, physical and mental function.
Cancer cachexia is also associated with poor response to therapy, increased susceptibility to treatment-related adverse events, as well as poor outcome and quality of life. Cancer cachexia is a complex, multifactorial syndrome, which is thought to result from the actions of both host and tumour derived factors, including cytokines involved in a systemic inflammatory response to the tumour.
Early intervention with nutritional supplementation has been shown to halt malnutrition, and may improve outcome in some patients. However, increasing nutritional intake is insufficient to prevent the development of cachexia, reflecting the complex pathogenesis of this condition. Nutritional supplements containing anti-inflammatory agents, for example the polyunsaturated fatty acid (PUFA) eicosapentanoic acid (EPA), have been shown to be more beneficial to patients than nutritional supplementation alone. EPA has been shown to interfere with multiple mechanisms implicated in the pathogenesis of cancer cachexia, and in clinical studies, has been associated with reversal of cachexia and improved survival.
When Ryan became ill in February 2016 he weighed 53kg. I chased the GP for the ENT referral in May 2016 by this point Ry's weight had dropped to 49kg. We didn't see ENT until July when they discovered the tumour by which point Ryan’s weight was now 46kg and by formal diagnosis at end of Aug he had reached his lowest weight of 42kg. So in total had lost over 10kg from Feb to diagnosis in Aug. The haematology consultant was quite concerned that Ryan's malnutrition by the time of diagnosis was going to make the fight even harder for us. Ry was not well enough, strong enough, or nutritionally able to begin Chemotherapy so the decision was made to fit him with a Nasal Gastric NG feeding tube, Ryan then went on to have a PEG, but this almost killed him with sepsis and peritonitis, undoing the small amount of success we had managed with the NG.
The surgeon that performed the emergency laparotomy was brutally honest with me in those early hours, he had never operated on a child with such low muscle mass due to malnutrition and he was very doubtful that the sutures from the operation would hold. The next 48 hours where critical. Either Ryan would make it or he wouldn't. He was doubtful.
Ryan defied the odds, made a full recovery and finally had a Mic-Key gastrostomy operation which has at last been a success and Ryan was weighed last week confirming he has gained weight to 50.4kg. Still a long way to go to get to ultimate goal of 60kg, but finally heading in the right direction.
Nutrition is critical for any growing child and for any adult to remain healthy. For Ryan it was a double edged sword. He was physically unable to eat due to the growing tumour in his throat, that we knew nothing about and the growing cancer was consuming inordinate amounts of calories and feeding off the little nutrients he had to grow. It really is a Catch 22.
Although Ryan is now able to eat a full normal diet again now, he is still being gastrostomy fed as well to ensure he has all the necessary nutrients he needs to fight the cancer.
Chemo round 3 should have started at beginning of February but had to be delayed as Ry's kidneys are struggling a little bit due to him being too dehydrated. The medics sorted that problem and re-hydrated him, but the following day when they took bloods his neutrophils had dropped to 0.6. They have to be above 1 to be able to have chemo. So we were delayed a 2nd day. On day three his bloods had dropped further to 0.5 so we were discharged and advised to go home and wait for them to improve.
Before we went home though, the doctor and dietitian came to chat about his nausea and lack of gastrostomy feeding. They have a few ideas, but they want to make only one change at a time so they know what works or what doesn't. A different anti-sickness, a different type of feed, they also want to start a diuretic and a laxative as his gut isn't working as well as it should.
We had a relaxing weekend at home and the community nurse came out the following Monday morning to check Ry's bloods again. They had raised to 1.1 so we headed straight back to Cardiff to commence cycle 3 exactly one week later than planned. That is one of the first things you learn with the old Cancer game. NOTHING goes to plan or the way you expect it to.
Finally cycle 3 commenced and he has done well again, with only a very small Ifosfamide reaction this time that only lasted for a few seconds. Then at discharge his neuts were 3.2, and we haven't even started the GCSF yet, so explain that one too me please; however his Hb had dropped to 86 and he was feeling a bit rough with sickness and chest pains. The doctors are not sure if that is due to low Hb or if he is coming down with a bug. If it was a bug it never took hold and he improved as the Hb went up.
We are over half way through treatment now with 3 more cycles to go. Cycle 4 starts Tuesday 7 March 2017.