Showing posts with label rare disease. Show all posts
Showing posts with label rare disease. Show all posts

Wednesday, April 17, 2019

Don't Deny Me The Right to Life


I have hypogammaglobulinemia and I need to have one treatment of intravenous immunoglobulins (IVIG) once a month to stop me from getting fungus, viruses, bacteria, hospital superbugs and every other germ.  Without this medication, I am constantly going from one infection to another, without many days between. And each infection further damages the organs until death is the result, or death results from one of the infections.   In the last four months I have had septicaemia twice and bronchio-pneumonia once, and numerous ear and throat infections which has led to three long hospitalisations in isolation.
My medical Aid is Discovery Classic Comprehensive.  Since 2015 Discovery has been paying for my IVIG treatments.  The first IVIG was being produced by the Western Province Blood Transfusion Service (a charity) and was called Stabilized Human Serum.  And in their infinite intelligence, the Medical Control Council closed their laboratory because it was not up to standard, but put nothing it it’s place, bearing in mind that the WPBTS is a charity, they did not have money to build a new laboratory.
The Medical Control Council registered two different IVIG; Polygam and Octagam.  After a lot of red tape and medical review team efforts including registering a dispute, Discovery paid for Octagam for me for the next three years.  
Octagam was being imported by Octapharma and ran out of stock in the country half way through 2018.  We chased up every bottle from every hospital in the country and managed another two treatments.  We then motivated for me to use Polygam, although I had had an allergic reaction to it once before during a hospital visit.  They agreed and we started on Polygam for a few months – each time the allergic reaction got worse until my physician stopped the Polygam infusion because he said that the next time it would end up with me in ICU and intubated, if he could save my life.  He said that continuing with Polygam would be a catastrophe.
Discovery has a Rare Disease Department and they suggested that I get the Octagam via a Section 21 company (we applied to Equity) to import it.   This necessitated a lot of red tape, many forms and a payment to the Medical Control Council of R300 for approval.  All the documents were then sent to Pre-authorisation and escalated to the Medical Review Teach and Touch Health for approval for payment.
I have received notice that Discovery will pay up to R15 000 per treatment and I am to use Southern Rx to obtain it.  They are now telling me which company to import it from.   I have also received notification from Southern Rx that my share of the payment will be R166 207.20 per month for my dosage.
I don’t know anyone who has that kind of money to pay for medication that is life-saving and life-enhancing ie. that is needed to stay alive.
Our Constitution specifies that we have the right to life, and Discovery and the Medical Control Council is denying me the right to life.


Saturday, September 24, 2016

My Life

Having CIVD, rare disease or cancer (incurable, chronic, debilitating downward spiral is like living inside a snakes and ladders game.  I know because I play the game.  You roll the dice and you go up some ladders.  You hit a lucky stroke and you find yourself a step or two ahead.  And when you least expect it, you land on the head of the snake and you slide down so fast your head spins. It is a series of mini avalanches.   No sooner has the person recovered from one disease when another takes its place.  It is as though the various diseases are competing to see which can do the most damage in the shortest possible time.  CVID is about slowly watching the pieces fall off, bits that stop working, energy levels deplete day by day.  The body slowly disintegrates; things that worked yesterday don’t work today.  There is little warning, diseases come in the night and are full blown by the morning.   There is a constant battle going on inside – always hovering just under the surface, even when it’s a good day.  And with velocity…rapidly one symptom looms much larger than others and now the ER, doctor, physician has something to notice.  Body temperature tells you nothing.  With no immune system, the body shows no temperature.  Temperature shows the body fighting back.  CIVD leaves a much lower than normal core body temperature.  The lower the temperature, the more ill the patient becomes.  Just one more challenge – not having a temperature does not fit into the protocol of admission to hospital so you are sent home, often feeling that the doctors think you are a shirker.
The mind can’t remember, the bowels sometimes don’t work, the bladder is a problem, lungs struggle, bones ache; nausea never stops.  Even hair falls out; it was where it should be last night but in the morning the pillow looks like a shaggy carpet.  Skin lesions appear without a bump, blood vessels burst for no reason, skin itches like a million ants crawling beneath it. 

I marvel at the capacity of the human body to take this relentless and remorseless onslaught.  Survival is a mystery.  And why I would want to continue to live is bewildering – because under all of this there is still that will and that hope that tomorrow will be better.  Is the need to live an instinct or is there a soul that refuses to surrender?   I am bewildered by the enormity of the question of the soul.  I cannot comprehend that I am just a material thing and that I will not have consciousness after death.  Is my thought that I have an eternal soul merely my way of lowering the internal distress I feel when I think of death as being the end to my consciousness?    At what point do I say enough is enough – at what point do I surrender because life no longer has meaning living as I am now – why do I constantly hope that I will be helped, that I will get better, that my circumstances will improve.  When will I accept these circumstances so that my fate is more acceptable to me?  What kind of life is it to trudge through from one infection to another, some more severe than others?  What life is it that if you do even the smallest thing outside of your bed, you will repay in suffering every ounce of energy it allows you on a tolerably well day.     

Wednesday, August 3, 2016

Orphan diseases - orphan explanations

Having a rare disease (called orphan diseases by the medical researches) is a unique and exhausting challenge for the patients, their friends and families (that is if the latter two stay around long enough).   It is an isolating condition to have, as many will question the existence of the disease.
This is what I would like you to know about my rare disease:  
Most doctors have never heard of, nor seen a patient with the disease.
Ask me about my disease, don’t question its existence.
Do not give advice.  I know more about my disease than my own doctors.
Smiling, laughing and working on the internet does not mean that I am cured.  It just means that I am still doing these things despite being ill.
These diseases are only “rare” until it happens to you or someone you love.
Just because your disease does not fit into an easy diagnosable box does not mean that you should be dismissed or overlooked.
Research is a big deal because there are not enough of us to warrant government funding.
I hate it when people say “Shame, I hope you get better soon”- like it is some kind of flu.
Nothing about rare diseases is easy – not the diagnosis, not the medical care and not living with it. 
Physicians need to listen and be compassionate and understanding instead of being dismissive of you because they don’t want their own ignorance to be seen.
It is a very isolating condition that is imposed on your life and one that makes you look at your own mortality and the terror that the thought of your non-existence brings you.  
Stop saying that you know someone who also has the disease.  You will invariably be completely wrong and have only picked up one or two words you recognise.  When I ask if the person is still alive, I get told “no”… so I wonder what the hell the person was getting at.  I do not need that extra information.  It is hard enough to cope with what I have.  One of the words in your diagnosis may be one that someone else has heard but he or she seldom knows the different variations of that diagnosis.
One in three million means that it is possible.  And those “one’s” need to count!
A rear disease makes one learn things you never thought possible and your use of medical jargon will make you sound like a professor of medicine.  
It can’t be fixed with a simple visit to the doctor. No question can be answered in a single sentence.  There isn’t a drug that can cure it.  It’s always going to be like that without research.   Hope is sometimes the only thing we have. 

Tuesday, May 5, 2015

Don’t Bury me until I’m Dead!

The struggle that happens inside of us when our abilities, skills, friends, family and dreams are stripped away due to a chronic and debilitating, often terminal illness, and our search for new foundations and meanings is often more stressful and difficult to bear than the pain and symptoms of the physical disease.  
It has been my biggest struggle and has caused me the most stress.  As everyone knows, stress makes every ache and pain worse than it really is.  There is mourning for all the things we have lost and a feeling of insecurity in every moment … what happens next?  I have been to psychiatrists and psychologists, I have spoken to others in the same predicament and I have read every self-help book that remotely has bearing on my problem.   I have spent hundreds of hours questioning even the very purpose of life, let alone the purpose of suffering.   What a terribly lonely road this is on which we travel.   I want to share with you a few of the insights I have learned on this journey.
This illness is not against you.  This is not punishment. You did nothing wrong. Illness is not an indication of weakness.  It is a message that you have worked hard enough, and it is now time to slow down to this moment. We need to leave every unnecessary thing behind. We have to stop rewinding the movie of our past lives and stop fast forwarding to a future of missed chances, unreachable goals and death.  We need to even let go of our search for answers.  We will know the answers soon enough.
Let us find a place of gratitude amongst the rubble of our old dreams…from a place that has no future ideas that can never become a reality, to a place where we can again discover the delight of uncertainty, never knowing the outcome.  This is a time when we need to let go of how we thought life would be and come to terms with how life actually is, in this moment. It is in this moment that we will find a sacred place for comfort, safety and peace.
Admitting that you are tired of trying to get better, tired of trying to pretend you are well when you are not, tired of explaining the unexplainable, tired of continually trying to fit into your old life, tired of accomplishing nothing in a day, even to yourself will bring a measure of relief.   Stop trying to please others, stop trying to be more than you are in this moment, because all that you are is good enough.  
Allow your body to guide you in what you can and cannot achieve in a day.  And then let it be.  If today you managed to get out of bed and get dressed, you have already achieved.  If you only managed to go to the toilet without help, you have already achieved.  There will always be days that you will wake up and be glad you never took your life yesterday.  There will also be days when you wake up and are sorry you did not take the departure ticket you were offered yesterday.  And this too is OK.
When doctors and nursing staff begin to withdraw their emotional input, know that this is only their way of dealing with their own emotions and it is no fault of yours.  Their withdrawal is a measure of how much emotion went into trying to get you well.  And it is OK.
When the pain becomes unbearable – stop, close your eyes and allow the sensations to move through your body.  Use your imagination and breathe in healing energy while breathing out the pain.  Do whatever works for you, but don’t beat yourself up about it.  Vomiting and pain cannot last forever, nor yet for very long.  As surely as the sun follows the rain, the pain will subside and you will be able to think once again.  Just try taking one minute at a time, and then the next minute, and eventually you will be able to do it for ten minutes at a time – when you get to an hour at a time, you are through your crisis.
This illness is an invitation to stop, slow down and do whatever it is that is most important to you.  Make a bucket list of things you are able to do and that you would like to do.   Then aim at just one of them.  If you are unable to do anything about it today, tomorrow holds the possibility of more energy that will take you one step closer to that goal.  Find something that is bigger than you – something you can do that will serve others, even if it is only using social networking.  It will present you with an issue that can give your life new meaning.  With today’s technology, you could join any number of groups with the same interests as you.  Just because you are ill, does not mean that you are unable to learn something new every day.  Learn a new language, learn to play a musical instrument, take a part of history that interests you and study it, read all the books you have always wanted to read but never had the time, start a journal that you can leave behind for your children or grandchildren so they can remember you or learn about what kind of a person you were.  Become your own best friend and do whatever it is you would tell your best friend to do under these circumstances. 
If you have a rare disease, become your own medical detective.  Read as much as you can about your disease.  Rare diseases are seldom seen by physicians and you are likely to learn more than your primary doctor and unless you are at a research station, you will also know more than your specialist or consultant.  Become an active participant in your own journey.  Become an exceptional patient.  Exceptional patients help the medical profession to see things they may have overlooked.  Often, it is the exceptional patient who will stumble on a cure for a previously incurable disease. 
Your life is not over.  You have only just started a new one.   Walk courageously (even if it is in a wheelchair or bed) forward.  A new day has dawned.  Every day you live is a miracle – you are not defeated.  Everything is OK and it is OK not to be OK.  

Monday, September 15, 2014

Snippet from the New Book: Anchor For My Soul

From the many years of counselling others, I have found that when you tell a story three times, that story…that event, loses its power over you.   I am going to tell my story so that I am freed from this negative trauma of my past.  I want to walk into the future without scabs, bruises or tears.

Something traumatic happened to me.  It was something far worse than being given a diagnosis of cancer.  It came as a terrible storm, a violent explosion of suppressed jealousy, hatred and anger.   A haematologist with a god-complex looked at something he did not understand, fought with a doctor who had to defend herself, irrespective of the truth or consequences of her lies, gave false information to my family that has had dire significance for me, has ruined my family, and made me ashamed, humiliated and embarrassed to be alive.  It swept away all that was civilised about a family.

If it were not for my mother, Professor Lynch of the University College Hospital in London, Professor M du Toit of the Bone Marrow Unit, Cape Town, Dr F Meyer and Dr Frieda Pienaar and her incredible team of specialists in Bloemfontein, I would not be here to tell you this story.  If you want to live and you have a serious life-threatening illness, it is important to find a team of medical professionals that want to save your life as badly as you want to live.


From my own experience, I know that it takes a lot more courage to live than to die.  My fervent prayer is that I will continue to have the courage to choose life over death.  My belief in a Higher Power and the aforementioned people, have been the anchor for my soul.