by Manuela Manuel
July 2005, A hospital in Kent, England.
Dreary, weather-worn brick buildings, parts of which date back to 1856 (the early years of Queen Victoria’s reign). Despite the antiquated surroundings, the apparent lack of modern facilities and up-to-date medical equipment, it is here that the diagnosis of MPN is made by a cardiologist. A ‘Good News, Bad News’ statement following various tests.
“Nothing to suggest heart disease, but hypercoagulable” Just one word at the end of a consultation - I remember it well, although at the time, I neither queried nor took more than a cursory interest in the diagnosis.
Would it have made a difference if I’d heeded the phrase ‘I’m sorry to have to tell you …….’? I doubt it. I try to remember what followed next but, without noticeable symptoms to cause concern at the time, the day’s journal is consigned to the mind‘s trash can.
Two weeks’ later, I heard it through the grapevine, much concern about raised platelet count. Another referral, this time to a hematologist. “The bone marrow biopsy reveals ‘clustering of megakarycotes, platelet and anisocytosis with some giant forms present, needs Jak2 mutation analysis.” What does this all mean?
Who can I turn to? I need a hero! Bonnie Tyler’s cry isn’t heard, Shrek doesn’t quite fit the bill and the pharmacist merely recites dosage instructions for hydroxycarbamide and aspirin.
March 2011, London’s Heathrow, ‘All my bags are packed, I’m ready to go’on yet another trip of a life time. Travelling with my favourite niece. We’re en route to Vietnam, Lao and Cambodia. I’ve been working through a list of 100 Places to see before you die and doing pretty well, not too many will be left when I have to unpack my bags for the last time. I’ve achieved the impossible dream and walked on all seven continents. So, Non, je ne regrette rien, no reason ever to say ‘I wish I had …..’
An amazing, unforgettable shared experience. An awe-inspiring journey, exciting and exhilarating. Except … a headache has persisted for days. Heat, humidity, dehydration. Any self-diagnosis, except perhaps attributing it to ET, PE or DVT.
Then, One night in Bangkok’s busy international airport while waiting for our connecting flight to London, the grey headache, assumes a different hue. Is it Just my imagination? I see red and there’s an incessant pounding in my skull. The dread of boarding another plane surfaces through the morass as I realise that dogged independence will not suffice this time. So aunt allows niece the dubious pleasure of taking control, cajoling, encouraging and persuading. Of course, I do not want to succumb to pain, make a fuss or miss the flight. We scour the terminal building for a pharmacy and eventually see the blue neon sign ‘Boots’, as familiar to everyone in the UK as Walgreens is to everyone in the USA.
Forget generic paracetamol, I’ll try something new – Tylenol and 2 litres of water. The headache recedes slightly and we board the plane. Ahead is a 15 hour flight home. At times like these, the windmills of your mind grind the seeds of fear as their sails tilt, driven by the relentless wind. I feel anxious. My companion, wise beyond her years, hides her concern well – we have lots in common.
Back in Devon, I regularly draw the attention of medical professionals to the persistent headache but more intriguing to them is the unexplained lower platelet counts which, over three months and without the daily dose of Interferon, are now within ‘normal’ range. Tongue in cheek I ask whether Tylenol could be responsible? If so, would it be hailed the newest treatment for ET? Not seriously, of course. By now, lung pain and shortness of breath are prevalent symptoms.What’ll I do?
August 2011, an emergency VQ scan (circulation of air and blood through the lung) shows bilateral pulmonary embolism. A slight variation of the phrase I dread. ‘….. very sorry to have to tell you that …. Please wait here for your hematology consultant’. Heparin is administered and I’m instructed to see my local doctor who will set anticoagulant dosages and arrange regular INR (International Normalized Ratio to check coagulability of blood) monitoring.
Within a week, I’m trapped in a spiralling sequence of appointments at various diagnostic clinics. PE isn’t being linked to MPN nor is account being taken of the two other secondary conditions, Erythromelalgia and Microcytic Anemia, the symptoms of which plague me. Why not? This is all I ask. Another consultation, another variation of the dreaded phrase “I have to be brutally frank, but I think you might have a malignancy.” A CT colonoscopy scan is arranged much against my wishes ‘in order to eliminate the possibility from the equation’. In one of my weaker moments, I give in and agree. The results show no malignancy. I breathe a sigh of relief.
November 2011 Chest pain and shortness of breath persist. I have a fever. An x-ray reveals nothing untoward. At the MPD Voice symposium in London, Professor Tiziano Barbui answers the question ‘Why do MPNs cause blood clots? I could Cry me a river but there’d be no point.
February 2012 It never entered my mind when I telephoned the doctor seeking advice about dropping INR levels that, within the hour, I’d find myself admitted to the emergency unit of the local hospital. It’s too late now to ignore the possibility of another PE incident. The existing patient records are set aside. A fresh file is being created to fulfil admission formalities. Yet again, I hear myself reciting a slimmed down version of my ‘complicated medical history.’ At the mention of MPN, blank looks cross the faces of the two clinicians questioning me.
Life takes on a surreal aspect. Have I dreamed a dream, perhaps been recalling a scene from House or ER? Am I actually hearing the conversations taking place at the nurses’ station? “Can’t do a thing for this patient; she’s obviously in pain but doesn’t want pain killers; hasn’t provided next-of-kin contact details.“
Did I imagine being wheeled down endless corridors at speed, did I see flashing red lights above doors and hear the hum of scanners? Am I actually in a holding bay with three other patients awaiting transfer to appropriate wards?
Yet another version of the dreaded phrase. “…Sorry, but the scan result is positive’. Tell me its not true, but it is “pulmonary embolism in the right lower lobe, some of which may be chronic.” The blood pressure monitor reads 217/83. Who wants to live forever?
Because I live alone, there is a reluctance to discharge me with high blood pressure and an INR level of 1.2. An assessment takes into account self-management of MPN/interferon and PE/phenindione. I protest. I’m more than capable of taking care of myself and I’m anything but lonely. There is a need to demonstrate that I can administer the required emergency daily dose of Heparin but I’m so weary and frustrated that even removing the cap from the syringe is embarrassingly beyond me at that moment. The nurse breathes a sigh of irritation and mutters: “So you do need help!” She removes the cap from the needle, hands the syringe back to me and observes with unblinking eyes.
The patient information leaflet states “You will remain under the care of the consultant in the hospital” and “You will be discharged from the service by one of the nurses caring for you when appropriate.” I’m discarding the hospital gown and I’m leaving here.
The sun set hours ago, there is a cold wind blowing. The hospital parking lot is dimly lit. I click the key fob and my car’s headlights wink at me. I’m reclaiming my independence, albeit under supervision. The sun will come out tomorrow.
Oh, what a beautiful morning. I’m On the road again ……. only I’m not on Route 66 and it’s not the unique, lilting voice of Willie Nelson emanating from the radio but the beautiful strains of Copland’s Appalachian Spring which fill my car with optimistic bliss as I wait for the rush hour traffic to start moving again.
What a difference a day makes. Back in my own bed, tired as I am, sleep eludes me. I’ve had two PE incidents in six months. So far I’ve received scant information and no reassurance about this latest diagnosis. I’m back home and I should be Feeling good but ET has returned home too, accompanied by three unruly offspring: nagging Bone Pain, harassing Headache and irritating Itching.
I try to remember the words of an old song, a distraction technique which sometimes drives me to distraction. You’ve got to Accentuate the positive, eliminate the negative, latch on to the affirmative, Don’t mess with Mister in-between
The next time I’ll be prepared. The signs and symptoms will be familiar. I’ll know what to do. I will survive.
© Manuela Manuel and MPNforum.com, 2012. Unauthorized use and/or duplication of this material without express and written permission from this blog’s author and/or owner is strictly prohibited. Excerpts and links may be used, provided that full and clear credit is given to Manuela Manuel and MPNforum.com with appropriate and specific direction to the original content.