AHN Founder Profile


Melissa Cull, AHN Founder

Melissa Cull

( AHN Founder )

 

How did I get into support work?  Well, to start from the beginning, I was born with a rare metabolic condition – Congenital Adrenal Hyperplasia (CAH).  I was a small baby and almost died before I was diagnosed as I was dehydrated and constantly sick.  I lost weight rapidly and had to be put in large doll clothes (it was the late 1960s, before premature baby clothes!).  I was so ill for first 3 months of life I was kept in hospital.  At birth it was difficult to tell if I was a boy or a girl.  Doctors took three weeks to discover what was wrong and confirm that I was female using blood tests, chromosome tests and x-rays.  Boys are more difficult to diagnose quickly and died in the past.

My parents were told I had the salt-losing type of CAH, a rare genetic life-threatening condition that affects the adrenal glands and requires lifelong treatment.  CAH is easier to identify in girls, as the external genitalia can look ambiguous, though inside everything is female.  Due to this pseudo-hermaphroditism, CAH is classed medically as an “intersex condition”.  It is caused by a missing enzyme in the adrenal gland which blocks production of the ‘stress’ hormone Cortisol.  This means that substances which would usually be converted occur in excess.  Some of these are weak androgens (male hormones) which can affect a baby in-utero. If a baby girl receives too much androgen, she becomes virilised.  The ambiguity may be corrected surgically.

Having an intersex condition is often shrouded in secrecy and shame, which it should not be. For a start, it is really more common than we are lead to believe.  At least 2% of the population or 1 in 50 families are affected by intersex.  CAH affects between 1 in 4500 to 1 in 10,000 people in the UK, with varying degrees of severity.  1 in 50 of the population carry the faulty gene.  It is an autosomal recessive condition, which means that both parents must be carriers.  If two carriers have children, then each pregnancy has a 1 in 4 chance of being a CAH child.  If that child then has offspring, they will all be carriers (unless their partner is a carrier when the chance of a CAH child is 1 in 2 or the partner also has CAH when all the offspring would have CAH). 

Society often shuns people with intersex conditions, as they don’t conform to the standard theory of only two sexes, male and female.  Many years ago in certain cultures intersex people where revered as “demi-gods” and considered special.  Today though it is sociologically unacceptable to be intersexed.  Babies have to be registered within 6 weeks of birth and the only official options are male or female.  This makes the correct speedy diagnosis of an intersex condition extremely important.  I am not ashamed to have CAH, it is genetic and there is nothing I, nor anyone else can do to prevent it.

Throughout childhood, the importance of not forgetting or refusing to take the steroid tablets I needed to stay alive was drummed into me.  I feel ill if I miss them so I’ve never stopped, but treatment is a constant balancing act.  Taking too much reduces childhood growth, increases your weight and suppresses the immune system.  Taking too little means you grow tall too quickly and your bones fuse early reducing final height.  At 5’ 1” (154cm), my height is reasonable, but I’ve always had weight problems.  Too little treatment also gives rise to excess androgens (the hormones which cause virilisation) hirsutisum (excess hair), deepening voice, high libido, aggression plus precocious (early) puberty in some cases.  More importantly, it increases the risk of a life-threatening “Adrenal Crisis”.

I’ve experienced quite a few  “Adrenal Crises” and even now, as an adult, flu or some other illness can cause a hormone imbalance and put me in hospital on a saline drip of intravenous steroids for a few days.  This happens when doubling or trebling my normal dose myself does not help me overcome the imbalance or the illness meant I couldn’t keep the tablets down/in my system.  I’ve had to learn to give myself injections of Hydrocortisone in case I am taken ill or even have an accident, as shock can also send you in crisis.  On a plus point, it’s much less painful to give yourself the injection than have someone else do it….

My childhood was full of blood tests, bone scans for growth and other tests but I also had to have repeated surgery.  I had my first operation for ‘normalising’ the external genitalia at the age of four.   It was quite scary having to stay in hospital not knowing what was happening.  This was when I started to realise CAH was serious. Later, I had further more surgery aged 12 and 14.   Doctors said I couldn’t have children as CAH reduced fertility, then they brushed the issue under the carpet.  

Later I found this first surgery consisted of removal of overgrown clitoris due to the excess androgens (Clitorectomy).  This extremely barbaric and painful form of surgery is no longer performed in most countries and since 1985, should never be done in the UK.  Other forms of reduction surgery maintaining the nerves for pleasurable sexual function are now performed. (Even so even these surgeries can cause damage to sensate function and unfortunately only now is any research being done into long term outcomes). The further surgery aged 12 and 14 was to reconstruct the vaginal opening to the correct place. (With CAH the vagina is often higher up inside the body and attached to the urethra).  This surgery (vaginoplasty) is often done around adolescence because of the problems menstruation would cause if it had nowhere to flow and the tissue is much easier for surgeons to work with when pubertal hormones kick in.

I grew up thinking that having a family was impossible and put marriage to the back of my mind.  At the time I wasn’t too interested and shut off, getting on with school as best I could with constant illnesses (your immune system isn’t too good and you are prone to getting any bug going).  Then after leaving school, I threw myself into my career.  Years later I found out that although fertility is considerably reduced in salt-losing CAH, pregnancy is possible, especially with new fertility treatments (though maintaining the correct hormone balance is more difficult and a caesarean is almost always required due to the scar tissue caused by previous surgery which makes normal delivery difficult).

I worked in engineering/electronics/computer industry for 14 years, moving my way up from assembly work to quality engineer (a difficult path for a woman).  However, I found it impossible to progress in such a male-dominated area and with a rare medical condition. In 1998, I changed career to work as an Editorial Assistant to a medical/scientific journal at a university.  After being made redundant from this I went on to work with computer databases in medical research. Employers always imply that my condition/need for medication mean I won’t perform.  CAH and high stress don’t mix, but I am relatively healthy – and have to work that much harder to prove my worth!

My hobbies have always been more ‘male’ than ‘female’ in this stereotypical world.  I particularly enjoy model making, DIY, electronics, computers, driving and car maintenance, but I also really enjoy cooking, listening to music, swimming, crafts and tapestry.  Research shows that women with CAH prefer male hobbies and sometimes professions (we are often so-called “Tomboys”).  It also shows they have good spatial abilities, maths and interest in sport.  Although I have excellent spatial abilities, I can’t abide sport and I’m hopeless at maths!

My friendships have been predominantly male since men seem on the same ‘wavelength’ given my hobbies and career.  However they see me more as ‘one of their mates’, or an amusement because I have similar interests.  This is extremely annoying when looking for a boyfriend – men complain that women are not interested in their hobbies, but when one is they consider it strange!   Men also have great difficulty in coping with the surgical aspects of CAH. (Having undergone a few gynaecological operations that left visible scarring and pain it can be difficult to get into intimate relationships).  Women can’t understand why I’m interested in so-called male pursuits.  I can’t understand so-called “normal” women very much either.

When I was younger, I asked questions about CAH, but found great difficulty getting answers.  My parents didn’t know much and doctors said take the tablets, have the surgery and everything will be OK!  Not knowing exactly why you are taking tablets or having surgery is extremely difficult to cope with.  Later, reasons for my tablets were given, but I still couldn’t get answers about my surgery and fertility because some of my medical records had been destroyed (due to NHS time limits and the hospital moving).

Eventually, aged 18, I spoke to Lesley Greene at RTMDC (now CLIMB).  I cannot really explain my emotions at this time but they were a mixture of relief at finding someone that finally understood what I was going through and nerves about starting on a path to get answers to my condition.  A large brown envelope arrived with lots of information on CAH.  My head was in turmoil: relief, anger and sadness for only just finding out.  I exchanged many letters and phone calls with Lesley as she helped me through understanding the information.

In 1991 a meeting on CAH was held in London by RTMDC.  I went, sat, listened and learned. For only the second time in my life I met others with CAH (the first being when I had the last surgery at 14 and was too frightened to ask questions as the doctors would not answer).  Meeting others with CAH for the first time at this conference helped, as I had felt so isolated. An experience that I will never forget!!  After that various meetings were held and eventually with so many families under their wing RTMDC decided to set up a sub-group for CAH. I volunteered immediately, not knowing what I could offer but wanting to help and yet not knowing what I was letting myself in for!!  I started as CAHG Newsletter Editor and continued this for approx 9 years.  I then went onto work as Adult Support Co-ordinator providing support to young adults, teenagers and also parents. Then until July 2004 as CAHG Secretary.

My quest for more information on CAH was fuelled, not only to help myself come to terms with CAH, but to help others avoid similar pain and anguish. I wanted to put something back for the support I had received.  After blindly looking for information alone I now met others in the same situation such as parents Kay  and Sue Elford who have become very good friends through working together on CAH support.  Together we looked for information, organised meetings and conferences to get parents, children and adults together.  Slowly over the years we have gained respect from the medical profession.

A few years later the opportunity arose to train as a Befriender for RTMDC (CLIMB), a role in which you learn to provide more support for people with rare disorders.  I readily volunteered and through this learnt more about my own feelings and what direction I wanted to go with support and life.  I enrolled in counselling courses, which I’m still taking, and one day I hope to do a degree in Psychology or IT.  I wanted to put back support I’d received but also be able to give better support to others because speaking and corresponding with Lesley was a turning point in my life – for which I want to say a “BIG THANK YOU”.

Another turning point was when, aged 31, I changed to a “Multidisciplinary Clinic” at UCL/Middlesex Hospitals for my CAH which I had learnt to cope with, but not well in all aspects.  I was experiencing problems from the surgery and could find no help.  Only now finally finding the missing answers to my jigsaw as my surgery notes had almost all been destroyed. (My new doctors later found some notes had been filed away in archives).  At the clinic, I was told that many of my problems were from years of over-treatment and lack of follow-up on the surgical aspects.  It was a great relief to understand this, but I also felt great sadness about my long search.  If I’d known this from my teens, I could have got on with life.  To Dr Gerard Conway, Miss Sarah Creighton, Dr Catherine Minto, Dr Naomi Crouch, Dr Lih-Mei Liao, Dr Carla Croft and Dr Emily Baker my most sincere and  “HEART FELT THANKS”.

What now?  I have always been very shy but in 1997 I took part in C4’s “Why Men Don’t Iron” documentary on hormones and brain differences (screened in 1998) and followed in 1999 by taking part in the BBC’s “Body Chemistry” series on the effects of hormones (screened in February 2000).  Why if I am so shy did I put myself in front of the public eye?   Well, because I want people to understand CAH and not be ashamed or have to hide it in secrecy, to educate ordinary people to be able to accept us.  If it gives even one person struggling to cope the opportunity to know that there is support and help available then it is worth it.

Recently the CAH Group and AHN  has been asked to present its views on treatment to the medical profession.  This recognition is a huge milestone.  Being shy I find public speaking difficult but again I decided that to improve treatment of others, I must.  I presented at the BAPU Conference 2000 in Cambridge on the surgical issues of CAH and jointly at the Atypical Gender Identity & Intersex Conference in London with my colleague and very good friend Sue Elford, chairperson of CAHG. In early 2004 I presented at the RCOG conference on Intersex.

As well as previously working with CLIMB as a Befriender and until July 2004 with CAHG of CLIMB, I’ve also set up an independent support network in 1999  (Adrenal Hyperplasia Network – AHN) for people with CAH – particularly adults and teenagers as well as to help clinicians and researchers.  A web site is under development for AHN.  With both CAHG of CLIMB and AHN, I’ve been working closely with the medical profession to help them with much needed research into improving treatment for CAH.  I’ve also been forging links with other support groups whether CAH overseas (Sweden, Australia, New Zealand, USA and France) or those with similar issues. 

My major project for CAH at the moment, is to write and compile a book with AHN and various medical professionals on the surgical issues of CAH for adults and teens, parents as well as GP’s and Nurses.  There is a definite lack of any information, let alone quality information, explaining what happens in non-medical jargon, pro’s and con’s of surgery and no surgery, possible complications, psychological issues, non surgical treatment, fertility etc.

I’ve devoted much of my spare time to support work since I was 21.  I’m now 36 and I hope to continue giving support in some way or another for many years to come. The rest of my future?  Hopefully to marry, providing I find someone who accepts my none-to-feminine hobbies and interests as well as my medical condition.  Possibly, to have children, if my fertility isn’t too reduced, though my career is important too.  To get on with life, and to travel.  To promote CAH research, education and support via AHN, as coping with CAH is very difficult without “Answers / Knowledge”.  It is so much easier to live with a condition such as this with all the pieces of the jigsaw!

© Melissa L. Cull 2001 - 2004

 


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