FOUR THINGS YOU MUST READ ON SICKLE CELL DISEASE PATIENT

FOUR THINGS YOU MUST READ ON SICKLE CELL DISEASE PATIENT

This information for all ages has helped many families.

  1. Konotey-Ahulu FID. The inheritance of Sickle Cell Disease. New African January 2000, pp 40-43
    http://www.konotey-ahulu.com/pdfs/sicklecell_jan2001.pdf
  2. Konotey-Ahulu FID. The Person with Sickle Cell Disease. New African March 2001, pp 38-39.
    http://www.konotey-ahulu.com/pdfs/sicklecell_mar2001.pdf
  3. Konotey-Ahulu FID. The Teenager with Sickle Cell Disease. New African. June 2001, pp 40-42
    http://www.konotey-ahulu.com/pdfs/sicklecell_jun2001.pdf
  4. Konotey-Ahulu FID. The Adult with Sickle Cell Disease. New African Sep. 2001, pp 40-43.
    http://www.konotey-ahulu.com/pdfs/sicklecell_sep2001.pdf
    Also http://www.questia.com

Remember that these sickle cell disease children, teenagers, and adults have inherited from their parents other genes to make them brilliant, beautiful, and much else. They must be looked after properly to make them use their brilliant genes to become ACHIEVERS in life.

See www.sicklecell.md and learn.

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Good evening Prof: Should I marry this person?

Question: Good Evening Prof, A lady friend of mine is with SC since birth and she loves this guy who is AS. Should she go on with the marriage even though there is a 50% chance of having sickly children?

Kanad
ANSWER:
Dear C.M., It is not my normal habit to advise who should marry whom, but as you can see from the kanad pictured above with male phenotypes on one side, and female on the other your friend is “SC” (abnormal Haemoglobin ACHE ‘S’ gene from one of her parents, and abnormal Haemoglobin ACHE ‘C’ gene from the other parent, making her ache with sickle cell crisis at certain times.

As you observed, when the dice ACHEACHE on one side is thrown against the dice NORMACHE on the other the probability for each throw of the dice is 1 in 2 (50%) for ACHEACHE to show because the man will show NORM or ACHE with each throw. The sequence is unpredictable because the man may show NORM (‘A’) several times or ACHE (‘S’) several times. Moreover, depending on whether the lady’s ACHE is an egg carrying ACHE ‘S’ or egg with ACHE ‘C’ the children of this union may be ‘AC’ NORMACHE, (‘A’ from the man, ‘C’ from the lady, ‘AS’ NORMACHE like your lady friend’s man, ‘SS’ ACHEACHE, or ‘SC’ ACHEACHE like your lady friend. Please read this statement again until you can explain it to your lady friend. Now, my book “The Sickle Cell Disease Patient” describes exactly such a situation where a Staff Nurse “SC” asked me whether she should go ahead and marry her lover “AS”. After explaining to her just as I have done here, she said to me: “Doctor, I am a nurse and I can care for him when he is unwell. Moreover you have told your patients how to keep out of sickle cell crisis so even if we have “SS” or “SC” children we can cope.” Remember that my kanad shown above (Konotey-Ahulu Norm Ache Dice) has two main functions:

They show you (i) What Could Happen ie PROBABILITY, and what is more important (ii) PREDICTABILITY ie What Will Happen.

If someone tells me: “Doc, I have suffered too much with this hereditary ailment. I do not want any child of mine to suffer like I am doing. Show me the phenotype that I can marry so that even though I have ACHEACHE my children will never have ACHEACHE”. Well, simple: Pick the dice marked NORMNORM and it is impossible to have an ACHEACHE child. But remember that some ACHEACHE people are brighter, more beautiful, and more focussed than their siblings who do not ache. The first option is Genetic Gambling. The second option is Predicting Genetic Certainty.

But here is a beautiful true story: One of my brilliant ACHEACHE “SS” ACHIEVERS fell in love with a NORMACHE “AS” (Sickle Cell Trait) lady. They decided to go ahead and get married hoping that the first child will be from the NORM egg of the lady, and his ACHE sperm, then they will stop, and adopt their second child. Well Mr H.S. engaged this lady, married her, and they had a son, lovely son with all the elegance of the father and the combined genius of both of them, NORMACHE “AS” Sickle Cell Trait. The couple went on to adopt a daughter.

So my duty is to show the difference between Genetic Gambling (Probability), and Genetic Prediction with 100 per cent certainty. If ACHEACHE marries ACHEACHE all the children will be ACHEACHE as shown on the cover of my blue book:

See my website www.sicklecell.md Those who choose Genetic Gambling because they are madly in love should know what could happen. They will limit their family size as Mr H. S. and his wife have done.

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Sickle Cell Trait Confusion: Is It Deliberate? Or Is This Ignorance?

Sickle Cell Trait Confusion: Is It Deliberate? Or Is This Ignorance?

I speak with authority as one who was born into a Sickle Cell Disease home within a Sickle Cell Trait country. One in every 5 of us in southern Ghana including nurses, doctors, business men and women, judges, liars, thieves, university professors, Parliamentarians, athletes, crooks, footballers, Olympic Medallists, and boxers has the Sickle Cell Trait.

In Northern Nigeria with a population of 90 million there are 30 Million Sickle Cell Traits. One in every three babies born there in Kano, Sokoto, Maedeguru is Sickle Cell Trait. And in Accra where I worked at the Korle Bu Teaching Hospital every 1 in 5 babies of the 13000 consecutive deliveries we tested in 12 months had Sickle Cell Trait.

What is more, 1 in every 3 of the white people in Greece where Lake Kopais used to be is Sickle Cell Trait! And now, lo and behold, “In Fontana August is Sickle Cell Trait Prevention Month”. Are they serious in suggesting Sickle Cell Trait needs preventing? Making 1 in 5 of us Ghanaians feel guilty for being born because we are Sickle Cell Trait? Even Sickle Cell Disease Patients need not feel guilty because they often have brilliant genes that their siblings do not possess.

Seriously, believe me, there are two kinds of readers of this Facebook post:

(1) Those who want to learn from me whom Nobel Laureate Professor Linus Pauling listened to when I delivered the Martin Luther King Award Lecture in Philadelphia on the Topic “The Vital Difference Between Sickle Cell Trait and Sickle Cell Disease”, and

(2) Those who prefer what Fontana teaches on Sickle Cell Trait.

For those who have time for me, please set time aside and study the following articles very, very, very carefully:

SICKLE CELL TRAIT

  1. Blaming sudden death on Sickle Cell Trait? http://bit.ly/1Eutn19 
  2. Sickle Cell Trait Misinformation and Disinformation http://bit.ly/1CqYHib
  3. Further Communication on Sickle Cell Trait Misinformation and Disinformation and Sickle Cell Terminology: Disease  or Disorder?          http://bit.ly/1Gm4gNP 
  4. World Sickle Cell Day 19h June 2014 http://bit.ly/1FuNXPi 
  5. Beware of symptomatic sickle cell traits. Lancet, February 29, 1992, page 555. http://bit.ly/2d18oOL
    http://www.thelancet.com/journals/lancet/article/PII0140-6736(92)90377-F/fulltext
  6. Dangerously flawed diagnosis of sickle cell trait in compartment syndrome rhabdomyolysis http://bit.ly/2d4t9Zd
    http://www.sicklecell.md/blog/index.php/2016/09/dangerously-flawed-diagnosis-of-sickle-cell-trait-in-compartment-syndrome-rhabdomyolysis-article/
  7. Sickle Cell Trait: As with statins when leading editors disagree please give principles same weight as details/
    http://www.sicklecell.md/blog/index.php/2016/09/statins-when-leading-editors-disagree-please-give-principles-same-weight-as-details/
    http://bit.ly/2dy5fUJ
  8.  http://bit.ly/2bRQ7B1    Tafracher BMJ 8th June 1975

This Ghanaian word Tafracher allows me to call a spade a spade, as it were. [It allows me to say articles describing Sickle Cell Trait as Sickle Cell Disease are (Tafracher) rubbish for how can a Sickle Cell Trait man run at 7000 ft at Olympic Games and beat the whole world with a disease?] 

If you absorb all this information you can help your colleagues and even your doctors in saying exactly what Sickle Cell Trait is, and what it is not.

Felix Konotey-Ahulu FGA MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH FGCP FWACP FTWAS Kwegyir Aggrey Distinguished Professor of Human Genetics, University Cape Coast Ghana, & Former Consultant Physician Genetic Counsellor Sickle Cell & Other Haemoglobinopathies, Korle Bu Teaching Hospital, Accra Ghana, and 9 Harley Street London W1G 9AL [ www.sicklecell.md ] Twitter Felix@profkonoteyahul

Further BMJ Links especially for doctors, nurses & science graduates.

  1. Overseas Med. Graduates bmj.com/content/356/bmj.j574/rr-0
  2. Routine Tests not to be abandoned bmj.com/content/357/bmj.j2091/rr-15
  3. BMA AGM 2017 On Abortion bmj.com/content/357/bmj.j3116/rr

Finally, Sickle (S) is not the only aching gene we can be born with. The second commonest abnormal Haemoglobin aching gene is “C”. Test for “S” alone (Sickle Cell Test) is not enough. I always test for other genes, not just for Sickle Cell Trait. You can be Sickle Test Negative (that is No “S”) and yet be “C” Positive, enabling you and your Sickle-Positive-”S” spouse to have a child who has two aching genes “S” + “C” to produce Hereditary Rheumatism (Sickle Cell Disease), never ever to be called “SC Trait”, but only to be known as “SC Disease”. Sickle Cell Trait is “AS”, never “SC”. I was born surrounded by both. I know the difference.

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World Sicklecell Disease Patient Week – Videos

World Sickle Cell Disease Patient Week

After a successful week of videos in July I have put them all together in one post for you to view.

Introduction
There is such an event called “World Sickle Cell Day” which falls in mid-June every year.

For me who had two brothers and one sister (Victor Agbetey, Jerry Tei and Sussie Konotey-Ahulu) with hereditary cold-season rheumatism or hemikom as this has always been known in my Krobo Tribe in Ghana as the name for Sickle Cell Disease – one day in a year is not enough attention given to a very important problem.

Day 1
Professor Konotey-Ahulu explains the reasons behind the Sicklecell Disease Patient Week and a bit about his history.

Day 2
Professor Konotey-Ahulu interviews an achiever of over 50 years old.

Day 3
Professor Konotey-Ahulu talks about the various African tribes which have various names for the Sicklecell disease. He also explains the difference between trait and the disease.

Day 4
Professor Konotey-Ahulu gives a round up of the videos published and a bit more history on what he found during his career.

Day 5
Professor Konotey-Ahulu continues to talk to an achiever on how he stopped the disease from taking over his life and reduced crises periods.

Day 6
Professor Konotey-Ahulu explains his dice (KANAD) and how it can help explain how people get the disease.

Day 7
An achiever Akosua M Dankwa talks about the Sicklecell Disease and how it has affected her life.

Books
The Sickle Disease Patient book is now on sale at a 50% discount. The book can now be purchased here http://blog.sicklecell.md/shop/ FREE KANAD dice with each purchase whilst stock lasts.

Links
Facebook Event – https://www.facebook.com/events/305588243201034
Books – http://blog.sicklecell.md/shop/

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World Sickle Cell Disease Patient Week – 20% off our books

World Sickle Cell Patient week

Today is the start of World Sickle Cell Disease Patient Week and we are offering 20% off our books till 31st August 2017.

To view the event on Facebook look out for my videos during the week starting today. https://www.facebook.com/events/305588243201034 

World Sickle Cell Disease Patient Week

Use the following code at the checkout WSCDPW2017 to get you 20% discount  plus an added bonus a special free gift of a pack of kanad (Konotey-Ahulu Norm Ache Dice) when you purchase a book from our store during the World Sickle Cell Disease Patient Week.

My books are available here http://blog.sicklecell.md/shop/

kanad (Konotey-Ahulu Norm Ache Dice)
Free gift when purchasing our books till 22nd July 2017
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World Sickle Cell Disease Patient Week

World Sickle Cell Disease Patient Week

WSCDPW [World Sickle Cell Disease Patient Week]

There is such an event called “World Sickle Cell Day” which falls in mid-June every year.

For me who had two brothers and one sister (Victor Agbetey, Jerry Tei and Sussie Konotey-Ahulu) with hereditary cold-season rheumatism or hemikom as this has always been known in my Krobo Tribe in Ghana as the name for Sickle Cell Disease – one day in a year is not enough attention given to a very important problem.

Therefore, I am from July 12 2017, God willing, devoting a whole week to what I am calling WSCDPW ie World Sickle Cell Disease Person or Patient Week – the P is for Person or Patient for, as I hope to show you, some-one with sickle cell disease does not have to be going in and out of hospital regularly and frequently.

So there will be something for 12, 13 14, 16 17, 18 of July, with 15th July as a rest day. During the week matters concerning the Person with sickle cell disease will be discussed. My greatest credential is that from the day I was born several decades ago I was within my immediate family and the extended family surrounded by sickle cell disease relatives – this credential of mine is more important than the fact that as a doctor, I ran the largest Sickle Cell Disease Clinic in the world at the Korle Bu Teaching Hospital. And indeed more important than the fact that with Professor Linus Pauling (discoverer of the molecular pathology of sickle cell haemoglobin for which he got the Nobel Prize) on the platform I was chosen from among 24 Dr Martin Luther Jing Jr Foundation Award Winners for Sickle Cell Research world-wide, to deliver the Award Dinner Lecture in Philadelphia on Wednesday 31st May 1972, the title of my Award Lecture being “The Vital Difference Between Sickle Cell Trait and Sickle Cell Disease”. This does not compare with the fact that I knew about the sickle cell disease patient before I read Medicine.

So, the fact that I was born into a home where my sibllings, and cousins, and aunts, and uncles suffered from sickle cell disease is why I dare to introduce a WSCDPW or World Sickle Cell Disease Patient Week. My aim is not to indulge in controversy. My sole aim, and I mean this, my sole aim is to tell those like my brothers and one sister who inherited an abnormal haemoglobin from both father and mother to give them sickle cell disease – to tell them that they have inherited other genes from the same parents that can produce great achievement in their lives. I shall be greatly privileged to introduce some of these ACHIEVERS to the world, and to help those struggling at the moment with pain and other problems how to succeed. My 643-page book describes no less than 130 real patients and their problems and how they have succeeded or not succeeded in tackling them. Watch this space! My website www.sicklecell.md also has much information.

Professor Felix I D Konotey-Ahulu [whose parents were Traits for Abnormal Haemoglobin genes and whose 3 siblings had sickle cell disease].
MB BS MD(Lond) FRCP(Lond) FRCP(Glasg) DTMH (L’pool) DSc(Hon UCC) FGCP FWACP FTWAS ORDER OF THE VOLTA (OFFICER)
Kwegyir Aggrey Distinguished Professor of Human Genetics, University of Cape Coast Ghana, and Former Consultant Physician Genetic Counsellor Korle Bu Teaching Hospital Ghana and 9 Harley Street, London W1G 9AL.

https://www.facebook.com/events/305588243201034

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Lesson of the week – Dorothy S. Amanor-Boadu

How I, a sickle cell anaemia woman, hydrate myself intravenously at 36000 feet

Dorothy Amanor-Boadu SRN SCM
The Elite Nursing Agency & Health Promotion Centre P O Box AN 6519, Accra-North, Ghana. [Email: elitensg@yahoo.co.uk]

I was born with sickle cell anaemia [‘SS’]. My health, which had been pretty stable until a serious sickle crisis was precipitated by (I am embarrassed to say this) a dietary  indiscretion – overeating. This first severe crisis happened at the age of 21 when I was a nurse at the Nursing Training College, Korle Bu Hospital, Accra, and almost cost me my life. If it had not been for the expertise and dedication of my doctors Dr. F.I.D. Konotey-Ahulu and Dr. Alexander Bruce Tagoe who stayed on duty for 3 days with no rest, I would not be here to write this story.

They did not go home during my critical stage complicated by thrombo-embolism brought on after a ‘cut down’ on my right ankle, performed due to inability to find any veins during the crisis. I was in hypovolaemic shock. Overeating is a known precipitating cause of sickle cell crisis

[1]. I went on to qualify as a State Registered Nurse in 1976, and worked as a Staff Nurse in charge of the Dermatology Department and Institute of Clinical Genetics Sickle Cell Clinic at Korle Bu Hospital for a little over a year. I was specially chosen to work with the sickle cell patients because my doctors thought I could empathise with them, which I did. When they were in pain, I identified with them, and wiped their brow.

I then left for the UK, a cold country, where I underwent Midwifery training at the Jessop Hospital for Women in Sheffield. I chose to do midwifery in addition to my SRN, because my mother was a well-known midwife in Accra. After this, I proceeded to the USA to specialise in Oncology Nursing, and even wrote a book

[2]. In the USA, I began to have about two hospital admissions per year for sickle cell crises with hospitalisations lasting up to 10 days at a time. All other crises occurring approximately every three months or so, lasting up to four days, were treated at home. I am now over 50 years of age, but from 1992 or thereabouts, my health gradually, but noticeably declined progressively resulting in more frequent and more severe episodes of crises. This could be dated from when I (foolishly) indulged in overeating again and ended up with an extremely severe crisis in Georgia. During this crisis, I developed a bedsore on the heel of my left heel in less than 18 hours of admission; in fact, it occurred while still in the emergency room! This crisis, occurring after I had left the restaurant with my husband and daughter where we had all enjoyed a Chinese dinner, was the second most severe in my life. [I had been taught to look for a husband who was ‘AA’, and I found one, and we have a daughter with ‘AS’ Haemoglobin Type, just like my parents]. Within 4 hours after the Chinese dinner, I presented myself at the emergency room in severe pain all over the body. Being a nurse, I often directed the doctors what to do. Quite a few times I asked them to ring Dr Konotey-Ahulu in London who would always ask the doctors to listen to me [Oh the wonder of Mobile Phones! Felix was always contactable].

I had never encountered any problems with flying on the ‘long haul flights’ (e.g.6- 9 hours from Ghana to UK/ USA and vice versa) until I reached the age of 40 years; then ‘all hell broke loose’.

Crises became even more frequent. I found it difficult to avoid the precipitating causes like late nights and (dare I say it?) too much good food.

Blood vessels for intravenous fluid (not blood usually) in crisis became more and more difficult to find, so a central venous access device was implanted into my chest which connects to a major blood vessel (superior vena cava) which, when in use, permits blood to be drawn from, and administration of intravenous drugs and fluids (drip). The device is also known as a ‘portocath/infusaport’ (depending on brand inserted). I have had this device over the past fifteen (15) years or so (each device being surgically implanted and removed/changed should infection, leakage or blockage be noted). Mine has been changed five times so far at an interval of every 2- 5 years. My port was initially inserted following an extremely bad crisis where I went into shock and no peripheral veins could be found anywhere on my body including my feet; any line obtained infiltrated within less than 12 hours. Finally, an intravenous (IV) line was inserted into my neck (central line) as a temporary measure until the port was inserted. (This is generally done in the operating room with a mild general anaesthetic or in the radiology procedures lab/room under fluoroscopic guidance with ‘twilight sleep’ induced (awake, but not fully aware)).

It was not until I went to live in the USA that I decided I had had enough of going to hospital for every crisis not resolved by oral hydration and Paracetamol, or other stronger analgesics. In the USA I found patients could come on a daily basis for intravenous drugs to be administered using the same IV site for up to 3 days or more by attaching a ‘heparin well’ to the butterfly or angiocath.

This gave me the idea of having this venous device inserted for me so I could have my intravenous fluids (drip) administered in the comfort of my own home, rather than on the hard emergency room stretcher and be charged $250.00 per hour for those uncomfortable facilities!

Having convinced my physician (who in America were haematologists) to agree to this, I treated most of my crises at home, administering the IV fluids and drugs knowing I could go on admission with a call to my doctor, if I thought things were not progressing well or I could no longer cope. With the use of the ‘hep well’, I would go to the emergency room to have it inserted (and labs drawn as ordered) and return a few days later when better to have it removed. After the hepwell other venous access devices were developed over
the years, including the ‘Hickman Catheter’ (catheter surgically inserted into a major vessel protruding through the skin with catheter hanging out on the surface), and the ‘Port O Cath’ which is a central venous access device surgically implanted under the skin in the areas of the chest, arm, or groin. I acquired my first one in 1990. When not in use, it cannot always be seen, but since I do not have too much adipose tissue to hide it, mine protrudes, and looks like a little round lump under the skin – I call it my 3rd breast!

dorothy_amanor-owusu
This device allowed me to avoid emergency room visits even more. I could now access (stick) myself with the ‘Huber needle’ and hook up my drip without assistance. All I had to do was call my doctor to let him know I was in trouble (i.e. in crisis) and he would call in my prescriptions to the hospital pharmacy. My husband would then pick them up and bring them to me at home, thus bypassing the Emergency Room altogether. Being a nurse, and working in the Haematology/Oncology areas, had really become useful to me in my personal life.

To think that if it had not been for Dr. FID Konotey-Ahulu and Dr. K.A Gbedemah my parents would not have allowed me to become a nurse as the latter thought the profession could prove physically taxing! I am glad I did nursing.

At this stage, I would like to acknowledge the following specialists: Dr. Jeffrey Giguere, in Greenville South Carolina, Dr. Laura de Castro, Durham North Carolina and Dr.Melanie Jacobs, Atlanta Georgia with gratitude for the trust and confidence they had in me to permit me to treat myself with minimal supervision, and without fear that I might become a drug addict and abuse the use of the port.

Well, back to our topic. After having flown on numerous occasions back and forth from the UK to USA and Ghana without incident (the planes by then were all using pressurised cabins and later prohibited smoking also which was helpful), I noticed after the age of 40 that on flights I was now tending to start ‘sickling’ approximately 3-4 hours into the flights. I was advised by Prof. F.I.D. Konotey-Ahulu to hydrate more before and after flights, to eat only a light meal prior to and during the flight, to move about more, and to start using Aerobic Oxygen 15-20 drops to a glass of water three times a day [3], which of course I drink on flights also. Following this advice and to make sure I got through without problems and inconvenience, I requested clearance from the medical department of the carrier I used most frequently (British Airways) for oxygen to be made available to me on the flights and permission for me to administer intravenous (IV) medications and fluids should it become necessary.

This procedure meant my physician and I had to complete a medical form which basically identified my condition and my requirements for the flight (oxygen for which an additional non-refundable charge of $150.00/£200.00 is required) and the physician’s opinion as to my fitness for the flight with confirmation that I did not need to be accompanied. With the assurance that I was a nurse and could take care of myself without assistance, the airline has to date always agreed to my requests.

After travelling yearly over a period of about 4 years, my physicians no longer have to fill forms for my medical clearance – a simple telephone call to the airlines medical division in London, U.K with confirmation of flight itinerary results in a printout attached to my ticket informing airline staff of my diagnosis and requirements always sufficed [Oh thank you British Airways! (and I hope I am not accused of advertising)]. I also request seating at the bulkhead/window (least inconveniencing to other passengers, should drip be required, also more legroom and space to manoeuvre in economy class).

During packing for my intended flight, included in my hand luggage is at least 1-2 litres of IV Fluids, Infusion sets, Syringes of different sizes 10/5/3ml with and without needles, Luer lock caps, adhesive tape, tegaderm dressing, normal saline and heparin flushes, alcohol swabs. Medications as prescribed including tablets and injection Phenergan, Pethidine, Paracetamol, Airtal or Percocet. One or two bottles of IV Ciprofloxacin and a small ‘sharps/biohazard’ container for disposal of needles and glass ampoules are also packed.

Finally, a letter from my physician giving reasons and the need for my possession of needles, lest some busybody security person suspect me of being a terrorist! Just a word here about my experience with pain killers on 3 continents. In Africa, neither Morphine nor Diamorphine are used for sickle crisis. In the USA Diamorphine is banned for clinical use. In the UK, both Morphine and Diamorphine are used for sickle cell crisis. In Ghana, we find Diclofenac, Airtal, and Ketorolac, with plenty of hydration, and occasionally Pethidine very briefly, more than adequate for the severest of crises.

Should I be flying with a carrier whose arrival and departure times are not always reliable a call to the airport to verify that flight is likely to depart on time is made. My goal here is to administer my intravenous fluids no more than an hour before checking in time to ensure adequate hydration for the flight. I have on occasion had to have my flight rebooked due to constant changes in flight departure time. Original departure of one airline that shall remain nameless was for 8pm, changed to 10pm, then 3am. Finally, the plane left without me at 5am. I took the flight 2 days later to get my hydration time right! The moral for other patients reading this article is this: ANTICIPATE PROBLEMS, AND PLAN HOW TO COPE WITH THEM! If necessary, cancel agreed plans (like a flight) in order to save your life.

My normal procedure now before any flight is to pack my bags about two weeks prior to the departure date (generally reduce stress – physical/emotional as much as possible several days/weeks before flight, and increase oral hydration three days before departure). On the date of departure I check in early where possible, return home to rest, take a bath prior to inserting my Huber needle into the centre of the port after cleaning it and an area of approximately 2” circumference with Betadine and alcohol swabs, using sterile technique secure/cover with appropriate dressing – Tegaderm/biocclusive (usually 3”x5”-this is a water repellent dressing applied to secure and protect port from invasion of pathogenic organisms etc) [Picture]. It is possible to shower/bathe without the insertion site becoming wet. Dressing change is required every third day and as necessary. Huber needle (90-degree angled needle) should be changed every 7 days, but should be removed as soon as no longer required for use to avoid risk of infection.

Once ‘hooked up’ I administer 500-1000ml of Dextrose/Saline or Normal Saline over 2-4 hours depending on total amount to be given and how I feel at the time. On completion, the Port is flushed with 10ml saline and 3ml U100 heparin flush, then capped and extension tubing tucked into my ‘bra’. IV infusion/tubing capped off and stored in a new clean self-sealing plastic bag, which is then added to my hand luggage.

Once on board the flight, with any signs of sickling (pain/discomfort) I first request for oxygen which, is either piped directly to my individual seat (from the cockpit) or via small portable tanks supplied by stewardesses. This is administered at 2-4 Litres/min depending on how acute the distress is. After 20 minutes or so, if no relief of symptoms are noted I inform the stewardess of my intent to administer IV Fluids and drugs, and request for a clothes hanger. (Usually at this point there is panic amongst the Air Crew because, despite the clearance printout from the medical department and the Ground Crew having all the details and specifics, the aircrew generally are left with only minimum information such as “they have a medical on board”).

At this point I usually get a visit from the Captain and Chief stewardess. Once everyone gets the fact that there was some miscommunication, I proceed to hang and prime the infusion tubing. The hanger is used to hang the infusion onto an appropriate fixture in front of or above me. Occasionally, depending on location of seat adhesive tape may be required to secure drip at a height to promote gravity drainage. Since the port is already accessed and ready for use all that is required is to flush the extension tubing with saline prior to administering IV medications and hooking up the infusion. Once the rate for infusion is regulated, a little nap with continuous oxygen usually averts any major crisis occurring even if not totally resolved. Most of the time, oxygen is not required for more than 2 hours and IV for 4 hours.

My IV line can be disconnected, flushed and capped off when I feel I no longer need it or at least thirty minutes before landing. To date there have been no complaints or concerns from other passengers. Everyone, crew and passengers alike have been considerate and helpful – willing to change seats where needed.

So now you know how Intravenous fluids can be safely and conveniently self- administered at 36000 feet!

Competing interests: None declared.

Acknowledgement: I thank Professor F I D Konotey-Ahulu for urging me to tell this story to encourage sickle cell disease patients never to give up.

1 Konotey-Ahulu FID. The sickle cell disease patient. Natural history from a
clinico-epidemiological study of the first 1550 patients of Korle Bu Hospital Sickle Cell Clinic. Watford, Tetteh-A’Domeno, 1996, page 122.

2 Amanor-Owusu D. Cancer Chemotherapy Manual. Legon, Accra. Ghana Universities Press, 2002

3 Konotey-Ahulu FID. Sickle-cell disease and the patient. Lancet 2005; 365: 382-83

Legend: Accessed Port covered with Tegaderm dressing. Note pallor of my nail: I have sickle cell anaemia with a cruising Haemoglobin level of 8 grams/dL.

Submitted by:Dorothy S. Amanor-Boadu

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Updated Publications now on Sicklecell.md

Below are listed my latest publications that I have updated on my website. You can view them here http://www.sicklecell.md/publications_articles.asp

2012
311 Konotey-Ahulu FID. Epistaxis from sickle cell disease must not be forgottenwww.bmj.com/content/344/bmj.e1097/rr/576087 BMJ Rapid Response 28 March 2012

312Konotey-Ahulu FID. World Sickle Cell Day 19th June 2012 www.sicklecell.md/blog/?p=132Featuring (i) The Inheritance of Sickle Cell Disease (ii) The Person with Sickle Cell Disease (iii) The Teenagwer with Sickle Cell Disease (iv) The Adult with Sickle Cell Disease.

313 Konotey-Ahulu FID. Further Communication on “Sickle Cell Trait Misinformation and Disinformation” and Sickle Cell Terminology: Disease or Disorder? www.sicklecell.md/blog/?p=127April 16 2012
314Konotey-Ahulu FID. Should clinicians edit Wikipedia to engage a wider world web? At least two examples of inaccuracy dictate caution www.bmj.com/content/345/bmj.e4275/rr/598116 BMJ 14 August 2012 Rapid Response

315Konotey-Ahulu FID. Management of an acute painful sickle cell episode in hospital: NICE guidance is frightening1 Sept 7 2012 www.bmj.com/content/344/bmj.e4063/rr/599158 [42 references]

316Konotey-Ahulu FID. Almost a quarter of Royal College Fellows say their hospitals cannot deliver continuity care. And they boast of something called National Institute of Clinical Excellence? www.bmj.com/content/345/bmj.e4942/rr/601191 September 7 2012 BMJ Rapid Response

317Konotey-Ahulu FID. There is no evidence that I was born on a Saturday. PRIVATE THOUGHTS – Postgraduate Medical Journal of Ghana 2012 (September); Volume 1, Number 1, pp 32-33 [Pointing out that the increasing use of “There is no evidence that ..” in scientific debate is itself unscientific]

318Konotey-Ahulu FID Bring back good quality paper in the print BMJwww.bmj.com/content/345/bmj.e6396/rr/610395 BMJ Rapid Response 23 October 2012

2013

319Konotey-Ahulu FID. Diagnosis and management of pulmonary embolism.www.bmj.com/content/346/bmj.f767/rr/633072 BMJ Rapid Response 26 Feb 2013

320Konotey-Ahulu FID. Liverpool care pathway BMJ and Channel Four News: Majority expert choice does not mean best choice. March 8 2013 www.bmj.com/content/346/bmj.f1303/rr/634971BMJ Rapid Response to “Nine out of 10 palliative care experts would choose Liverpool care pathway for themselves” Krishna Chinthapalli BMJ 2013; 346: 1103 (March 2, pages 2-3)

321 Konotey-Ahulu FID. Christianity and Africa. New African 2013 March, page 4http://bit.ly/Z0eb1K

322Konotey-Ahulu FID. Importance of history in the diagnosis of pulmonary embolismwww.bmj.com/content/346/bmj.f1692 March 19
BMJ 2013; 366: F1692

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