I was born on 16 October 1961 at the Krugersdorp Hospital. My Mother, Gail, gave birth to me after a short labour. Not expecting anything untoward, my parents (my dad is Len) were very shocked as I was born with no ears and no ear canals. I was born with the genetic disorder called Treacher-Collins Syndrome. The symptoms of this disorder vary greatly, ranging from almost unnoticeable to severe. Most affected patients have underdeveloped facial bones, which result in a sunken appearance in the middle of the face, a prominent nose, and a very small jaw and chin (micrognathia). Most have varying degrees of hearing loss or deafness. Some people with this condition are also born with a cleft palate. One must appreciate that in 1961 there was very little reading or research or understanding of TCS especially here in South Africa where TCS is less prominent.
I consider myself a mid-range affected TCS person and am hard of hearing.
The first challenge for my parents was that I could not suck due to the underdeveloped jaw. This resulted in my having rapid weight loss. At two months, I was below my birth weight. After trying to feed me using eye droppers and teaspoons, my parents took me to see a paediatrician, Dr Phillip Kushlick, who recommended that they feed me sweetened condensed milk as it was very rich substitute for milk. To this day, I can’t stand the sight or smell of condensed milk.
When I was about 12 months old, I had a series of plastic surgery operations to reconstruct my ears (my cute little one of a kind cabbages) which lasted about 6 months. I got my first hearing aid (a bone conductor hearing aid with a head band) just after my 2nd birthday. The biggest issue that my parents faced was the overall lack of knowledge about TCS at the time as well as a general prejudice among the medical fraternity, many thinking that my mother was mad and a raving lunatic. Many wanted to see me institutionalised. My plastic surgery was done by the legendary Dr. Jack Penn, founder of the Brenthurst Clinic in Johannesburg. Dr Arthur Amoils was the ENT who insisted that I get a hearing aid where others advised that I be simply institutionalised as there was no hope. While in hospital, I was confined and my parents and family had no access to me. They could only see me through a small round window. I actually bonded with the nurse so much that I refused to go to my mother when she was at long last able to touch and hold me. I had my arms put into splints (using wooden tongue depressor sticks) so that I would not put the bandages off. This did not necessarily help as I still managed to rub the bandages off causing one operation to have to be redone. I was also not allowed to fall.
Soon after my series of operations, I started attending speech therapy (from when I was about 2 years old). My first speech classes were done at the Speech and Audiology Department of the University of the Witwatersrand (just behind the Planetarium). One of the first exercises given to me by the speech therapist was to blow a ping pong ball through a straw across the carpet. I continued with speech therapy until I was about 14 years old.
One day, aged nearly 3 years old, we were traveling along a country road and as usual they were pointing out animals and the sounds they make. I blurted out the words “gobble, gobble” upon seeing a turkey. My mother and grandmother nearly drove off the road in tears as they then realised I could hear and had the prospect of more words, sentences and even a normal life.
My folks had a second child in 1964 when Bronwyn was born. She sadly died and left us after suffering and dying of pneumococcalperitonitis when she was 6 and I was 10 years old.
I have worn a “traditional” single bone conductor hearing aid nearly all my life (conductor on the left side of the head), the one with a headband. I had various versions of hearing aids over the years from Danovox, to a locally made aid from “HAC” developed by Mr Smith from the Hearing Aid Centre in Johannesburg, to the various Phillips models. The biggest nightmare for my folks would be when I would not take off the aid in the rain or when I would go canoeing and then fall into the lagoon or for a soccer ball hitting the aid and it getting damaged – all this meant were frequent visits to the hearing aid centre for repairs. In the initial years I had to contend with just having a “skin-coloured” bone-conductor. It was only later that I was able to get a brown-hair-coloured bone-conductor. The band also initially only came in grey and I had to replace the covering with a brown shoe lace.
I attended main stream schooling throughout my school years. This was a non negotiable for my mother. She was once again accused of being mad and in need of help when she tried to enroll me at a convent school in Klerksdorp and the Mother Superior told her I need to go to special school and that she needed help.
The only surgery I had was the initial plastic surgery to develop my cute little ears, removal of back teeth to allow space for the others, the straightening of my nose which was broken during an accident during a wild game capture exercise (during my years training to be a nature conservation officer) and a few sinusitis operations to fix my sinuses. I have had no reconstructive surgery on my face.
I was offered the opportunity at the age of about 10 to attempt surgery to open my ear canals but due to the 50/50 chance of facial paralysis, I decided that I was happy with who I was and did not need surgery. If no one liked what I looked like, bully for them, they need not be my friend.
I attended a Jewish pre-school in Klerksdorp before going on to Milner Primary for grade 1. My Dad was then transferred to Pretoria where I attended the next 2 years at Hatfield Primary. By the time my Dad was again transferred, this time to Vereeniging, I had completed my primary education at Milton Primary and I then went off to General Smuts High School, where I finished my schooling. I never failed a year in all my 12 years of schooling. This is not to say that in the last 2 years, when the then new Principal and the English teacher at GSHS contrived to have the authorities force me to do English at the lower level, a development which meant I could not go onto University as one’s home language had to be at the higher level to get university exemption.
This did not stop me from furthering my education. I had been medically exempted from compulsory military service (a requirement by law during the Apartheid regime that all white males had to report for military service or face 6 years imprisonment). This is the only time I have used my loss of hearing and my ears to my benefit (else I would have sat for 6 years in jail as a conscientious objector). I am a pacifist and support a gun free South Africa and did not believe that military conscription would solve the problems of our country. I used this time to go to Technikon instead of military service.
On leaving school at the end of 1979, I went on to do a diploma in Nature Conservation at the Pretoria Technikon. I was in Pretoria for 2 years and then in 1982 I did my practical year in Stellenbosch with Cape Nature Conservation.
I did not complete the course and I had realised that this was for me a hobby or nice to do thing and not a career I wished to pursue. I have never regretted the decision.
While at school, I played soccer until “under 13” age group. The game was by then getting too physical and my hearing aid was getting damaged too often, I then started competitive swimming and excelled to being able to swim at provincial level for Vaal Triangle. My main stroke was breaststroke, but I forever felt defeated as I had to swim against my cousin Douglas Eagar, who was the National record holder for both 100 and 200 metres at the time. I also did athletics excelling at 800, 1500 and 3000m and also going on to do the 3000m steeplechase while at Technikon. I was the cross-country team captain in my final year at school. This then lead me to do road running and I focused on the 10, 16 and 21 km races. I only did one marathon before getting serious Achilles tendon injuries and also after having to decide to study after hours or continue to do running. My marathon time was a 2hr 47.11.
While at school, I also attended Cub Scouts from the age of 7 and then Scouts from age 11. I went on to attain the highest award in Scouting by achieving my Springbok Scout Award (same as Eagle/Queen Scout). I was also awarded the Chief Scout Merit Commendation Award. I later became am Adult Scout Leader for a few years. I was also involved at the Methodist Youth Club at our local church.
While at school and at Technikon, I did not have any girl-friends. I had a number of friends who were girls. I never had any relationships as such. Not to say I did have crushes on a few girls. But I was different and as such I suppose I was not the ideal guy to date. But I did have some very dear and close friends; not many, but those that were sincere and genuine with their friendship like Deon and Briony are still my best friends to this day. They saw behind the face and the hearing aid etc. They saw me, Mark, and for that I am forever grateful.
Having made the decision at beginning of 1983 to not carry on with my Nature Conservation career, I however did not know what I was to do and was unemployed for about 8 months. I was then employed in September 1983 by Anglo American Life Assurance Company as a new business sort clerk, a very repetitive job. I however moved on quickly into the systems development department doing testing of the IT systems being used by the company. This led to my being transferred to Cape Town in January 1985. In 1986, I enrolled to study through UNISA to do a law degree, after qualifying for an “old age” university exemption. Part-time distance learning did not work for me. I then looked at what other options were opened to me.
By this time I had met Mary Gillett, who is a few years older than me. She had had her fair share of ups and downs and challenges in life (that is another story that needs to be written). We shared many of the same interests esp politics and social issues and have similar values and outlook in life and she was also involved in Scouting. We began dating in early 1986. We then got engaged in September 1986 by which time we were living together. Quite a few of the family started counting the months only for us to prove them wrong.
By then too I had found out that the University of the Western Cape offered under-graduate studies on an after-hours basis in the evenings. This meant I could continue to work and earn a living and study at the same time. In 1987, I enrolled to do a Bachelor of Economics degree and spent the next 6 years travelling from work to classes at about 4.30pm Mondays to Thursday. At that stage Mary and I only had one car, so she would pick me up, I would drop her off at home and then along Settlers Way and Modderdam Road to the UWC campus. 1986/87 was the height of Cape Town’s “Witdoek”/Cape Flats war, so the environment was always tense at campus with many students and lecturers often being arrested by the Apartheid authorities and many an evening lecture came with the whiff of teargas in the air. I graduated at the end of 1992.
Before getting married, Mary did her own research to better understand this thing called TCS. We had been going through a rocky stage of our relationship at that stage. She by nature, is an inquisitive type and will go to the ends to find out things and information etc. Some of the information she had found was not palatable to me or my parents. We had got on with our lives and did not feel the need to research any more than what had been done thus far. We had accepted that it was a genetic mutation as there was no known TCS cases in our family for at least 5 generations back. What Mary had found was that it was indeed hereditary and could be passed down. Once we had settled the dust on this matter, we started attending genetic courses and going to genetic counseling seeing the renown Professor Peter Beighton of the UCT Dept Human Genetics.
In October of 1987, I got married to Mary, at the St Cyprians School Chapel in Cape Town, a rather grand affair with me wearing top hat and tails, a very smart occasion, especially after I had planned to just wear a normal suit. The months after our wedding were not an easy time for us. There was lots of pressure for us not to have children due to my having TCS. After Mary and I had done a lot of additional research and consultations with the Genetic Department of the University of Cape Town, we decided to that we would indeed have a family. There were suggestions that if TCS was picked up during the scans and tests during the pregnancy, that we should abort. I said no to abortion as my view was that if our child was to have TCS, who better a mentor he/she would have in me given what I had gone through and how I had learnt and coped with the situation I found myself in? I also said that those advocating abortion were saying that I should not exist either. Our first child, Nicholas was born in August of 1989. Nicholas was born with very mild traces of TCS. He has normal hearing and normal ears however one can see from his jaw and eyes and nose that there are traces of TCS. When he was just a few months old, we went to the famous Carel du Toit Centre at Tygerberg Hospital for hearing tests as from his responses we were not sure if he could hear or not. It turned out that he was just stubborn! He has normal hearing but narrow ear canals that caused him lots of ear infections. There is nothing that grommets cannot remedy.
We were put in our places went we realised how blessed and lucky we were and how grateful we needed to be when a couple opposite us sat with their dearest child who was suffering from a condition where the skull has stopped growing but the brain was too big for the skull and was in immense pain.
We then decided after many discussions and self reflection to have another child. In December 1993, Christopher was born. He has no traces of TCS. We did however, as we had done for Nicholas, we did regular scans and tests. By this time, research and knowledge of TCS had developed where the problematic chromosome had now been identified. I can honestly say that pregnancies were tense times in our household as we were all like cats on a hot tin roof for 9 solid months.
In 1985 when my company transferred me to Cape Town, after it had merged with Southern Life. I remained at Southern Life until it was bought out by Momentum Life resulting in me being retrenched in May 1999.
While at Southern Life, I was moved into the Human Resource Department initially focusing on HR Systems, but then into HR Remuneration research, HR Management reporting, and onto doing all administrative labour law issues ensuring company policies and procedures were compliant. I was then asked to take over the Staff Pension Fund when a colleague died. That was in 1992. Since then I have grown and developed my career in the pension and retirement fund industry.
After being retrenched, I worked on my own in 1999 and 2000 trying to establish my own business, but this did not succeed for a number of reasons but mainly due to the lack of funding. After many job applications and few interviews, I was fortunate to be offered a position at Anglo Platinum (now known as Anglo American Platinum) as the Retirement Funds Manager and Principal Executive Officer in January 2001.
I first became aware of BAHA in its infant years in the late 1990’s but declined to pursue it due to the early reports of high infection and rejection rates.
During 2007, after complaining about the persistent pain the headband was inflicting on me, my wife was seriously worried about how these headaches were affecting my life especially at work. We had traveled overseas as a family and my family saw me 24/7 and realised how often I took off my hearing aid to rest my head. One of the consequences of having a head-band all these years, was that a permanent indent over my head had developed and a deep indent where the bone-conductor was positioned. This caused immense discomfort without even realising it. I had become almost immune to the discomfort.
Upon returning from our travels, I started enquiring in earnest about the BAHA after further encouragement from a work colleague and research done by 2 other colleagues. [I am indebted to Dan le Roux, Anjeanette Tait and the late Dr Rob Harrison]. At an appointment my wife and I had with Anjeanette Tait (audiologist with my company) we met up with Tamara Turton (now Tamara van Zyl) from SouthernENT (the suppliers of Cochlear BAHA in South Africa) who told us all about the BAHA and allowed me to test the “kiddie’s version” with the soft band for a period of time.
When I first put on the kiddies version I was horrified at the noise around me. My wife and Tamara were puzzled as the noise I heard was from the air conditioner which they had screened out.
After doing more research/investigation – a family effort, I decided at the end of 2007 to proceed with having the BAHA implants on both sides – i.e. bilateral implant operations. We commenced with the first operation in February 2008 performed by Dr Martin Gill at the Olivedale Hospital in north-west of Johannesburg (right side – 2 stage implant process). The implant went well, however, due to difficulty with the anesthesia – due having a small mouth and narrow throat, it was decided that the next operation would be a combined one on both sides – the completion of the right side i.e. the second of the 2 stage process as well as to do a single phase operation on the left side. After the mandatory 4 month recovery period for the first implant to allow the titanium implant to fuse with the bone which regrows after the drilling , we commenced with the second operation in May 2008 in what I can only describe as a testing experience for Dr. Gill and his team. He did the anesthesia using a pediatric tube through my nose.
In June 2008, I had my first switch on of the BAHA (the right side).
WOW, what an experience!! The first thing I asked was “what’s that noise?”, only to be again told it was also the aircon. My eyes were so wide open as to the new sounds and noises that I had never heard – what a truly life changing experience.
My family then surprised me. They had bought me an adapter for my new BAHA to connect to my cell phone and an iPod. Tamara had her iPod with her, so we connected and she played some music – WOW. I had never heard music like that ever – it was truly MAGIC and my face showed it – my smile was huge. I got home and Christopher, my youngest son, then connected his iPod for me and put on Bohemian Rhapsody by Queen – which I have always considered the greatest song and piece of music ever. Well well, my mind and head were blown away – I had never heard such pure joy ever – six and a half minutes of true wonder. That weekend I went to the theatre to see “We will rock you – a tribute to Queen”. Music is such a pleasure to now listen to.
The next 3 months was quite testing on my head. New sounds, new noises, new experiences. In addition I now had a higher degree of directional hearing and that took some getting used to.
In August 2008, I got the second BAHA. Another WOW experience.
The 3 months after the second BAHA was switched on, were again quite exhausting on my head. I was tired each day due to the brain having to work so hard. Both inner ears were now working full steam. The thing that was taking its toll on me was the amount of input the brain had to endure. Sounds coming from all directions, like hearing glasses tinkling on the other side of the restaurant, to hearing conversations taking place far away, things that I had never experienced before. What I had also now realised was, how much I had not heard in work place meetings and how much I had relied on lip reading all my life. It was like information overload. This started getting better by late October.
Acquiring the BAHA has been a real life changing experience which I am glad I did. I have even been complemented for looking good with my hair being free and no longer having the band pushing it down. I still have some way to go to get fully used to all the new sounds, noises and experiences. The mental filtering of noises and sounds is taking longer than I anticipated.
One of the positive outcomes already has been the comments from friends, family and colleagues about the improvement in my speech. This is great feedback and shows that improved hearing definitely has an impact on the quality of one’s speech. Apparently, I am told, my singing has also improved from the monotonous drone it used to be, although this is up for debate.
I have since worked out a system to use Bluetooth to connect my Cochlear adapter to my BAHA so that I can have a hands free cellular connection while driving.
Due to my life-changing experience, I decided it was time for me to give back to the community and to help others who have TCS or are about to acquire or have a BAHA. It is my wish to help those in doubt or those without the support structures and to encourage them to reach their full potential and that they too can make a success of life. I have established a Facebook support group for those who had or were acquiring a BAHA.
My career at Anglo American Platinum continues as I am their Senior Manager responsible for Retirement Funds and Principal Executive Officer for most of their Funds (among the largest in South Africa iro membership size and assets under management). In addition, I serve as an Independent Trustee on 2 small Funds and am Vice Chairperson for the Principal Officers Association. I have also been invited to speak at numerous retirement fund conferences (both locally and internationally). Nicholas and Christopher have finished their schooling at St Stithians College. Nick is about to complete his 4th and final year towards an Actuarial Science Degree at UCT and Chris is writing his final Matric exams and hopes to be accepted at UCT next year to do a Degree in Architecture.
I still sleep like the dead, never hear my snoring and of course never woke up when the children were babies. I need to always wear the healing caps when I sleep otherwise my abutments get overgrown, infected and sore. I still experience sinus problems resulting in mucous build-up while I sleep. But these are not major issues but more of a nuisance factor
This is, in short, my life story. I hope that it will help others that have TCS or have or are considering acquiring the BAHA, that life is what you make it. With the right support and determination of a “mad and lunatic” mother, having caring, loving and supporting parents who showed me the way forward in love, a truly understanding and supportive wife and family; but also with the right get up and pick yourself up attitude and learn from the mistakes we have made (and there are many I have made), life is there for the taking. You can make it all you want. It is not without its ups and downs, many hours of loneliness, lots of tears and hurt and not without ridicule and teasing. It is not without narrow minded prejudice from a few, but don’t ever let them get in your way. Life is what you choose it to be and to believe in yourself. It is in your hands and to take all the positives with the love and support and care of those around who love you unconditionally. Anyone is welcome to contact me at firstname.lastname@example.org.
Mark de Klerk
If by, Rudyard Kipling
IF you can keep your head when all about you
Are losing theirs and blaming it on you,
If you can trust yourself when all men doubt you,
But make allowance for their doubting too;
If you can wait and not be tired by waiting,
Or being lied about, don’t deal in lies,
Or being hated, don’t give way to hating,
And yet don’t look too good, nor talk too wise:
If you can dream – and not make dreams your master;
If you can think – and not make thoughts your aim;
If you can meet with Triumph and Disaster
And treat those two impostors just the same;
If you can bear to hear the truth you’ve spoken
Twisted by knaves to make a trap for fools,
Or watch the things you gave your life to, broken,
And stoop and build ’em up with worn-out tools:
If you can make one heap of all your winnings
And risk it on one turn of pitch-and-toss,
And lose, and start again at your beginnings
And never breathe a word about your loss;
If you can force your heart and nerve and sinew
To serve your turn long after they are gone,
And so hold on when there is nothing in you
Except the Will which says to them: ‘Hold on!’
If you can talk with crowds and keep your virtue,
‘ Or walk with Kings – nor lose the common touch,
if neither foes nor loving friends can hurt you,
If all men count with you, but none too much;
If you can fill the unforgiving minute
With sixty seconds’ worth of distance run,
Yours is the Earth and everything that’s in it,
And – which is more – you’ll be a Man, my son!