Congenital Lymphangioma - Regina’s Story

An article by Jennifer Raw

In the autumn of 2010 I had the pleasure of meeting a remarkable young lady. Her name is Regina Addae; she is 13 years old and from Ghana. She is a cheeky, lively young woman, who suffers from a congenital lymphangioma that has been growing on her face since she was 2 weeks old.

Regina’s remarkable story beings in 2004, when nurse Kirstie Randall was working for the charity Mercy Ships, at that time the hospital ship was based just off the coast of Ghana. The operation was too complicated and too risky to perform aboard the ship, but Kirstie vowed to find her the help that she needed.

Over the past 6 years, with the help of Oral and Maxillo-Facial surgeon Abi Boys, and through the charity Abi founded; Willing and Abel, £30,000 was raised for Regina. This enabled her to spend 3 months in the UK last year, where her surgery took place at Barts and The Royal London NHS Trust. She is now living happily in Ghana where her incredible story has gained her wide acclaim.

Her tumour was unlike anything the surgeon, Professor Hutchison, had seen before and - the complexity of the malformation was such that it is potentially fatal in a number of ways.

Without intervention Regina would have risked eventual death from suffocation as the aggressive tumour continued to grow into her nose and mouth eventually compressing her airway. It was also expanding back into the cranium towards the brain and ominously encircled the left internal carotid artery. This would have lead to fatal consequences had the tumour grown further into the blood vessel She was also extremely anaemic (Hb 6g/dl - normal levels are between 11-13g/dl for children) and loosing approximately 50ml of blood every day from her eye.

Regina’s father, Stephen, came over with her for the duration of her treatment. Since, Regina was three years old, her parents Stephen and Kate had been searching for treatment. Yet they were unable to find help, consistently being turned down due to the complexity of the surgery and the seemingly insurmountable cost. It is common, as in Regina’s case, that families are ostracised from their communities when they have a child with a deformity such as Regina’s. The locals believed that the tumour showed that Regina was possessed by the Devil, and a curse was upon her family. Traditions such as witchcraft are still deeply entrenched in the cultures of West Africa, playing a role in many areas and levels of society; it is not uncommon for people to consult witchdoctors for help with illness or advice on decisions.

Regina’s hope came from a charity founded by the Oral and Maxillo-Facial surgeon Abi Boys. Willing and Abel is a pioneering charity that exists to meet the needs of children requiring highly specialised surgery. ‘Such cases are beyond the facilities of most government and mission hospitals in developing nations, and need to be dealt with on an individual basis’ remarks Dr Boys. The charity provides connections between these children and specialist centres, assisting with visas, passports, transportation and finances. The charity ethos is that by transforming the lives of individuals; entire families, communities and nations can be transformed.

Dr Boys contacted one of her seniors, Prof. Iain Hutchison, who agreed to take on Regina’s case and undertake the procedure forfeiting his fees. The operation promised to be one with the potential for huge complications. The tumour lay extremely close to the facial nerve, a structure that controls movement to the muscles of the face, therefore playing a crucial part in facial expressions. The facial bones were already greatly distorted by the growth which wrapped around the optic nerve, making it questionable as to whether the left eye could be saved; it was already protruding three inches out of the socket.

Before the operation all those involved were concerned about a number of things:

• The extent of blood loss that could occur, they had 6 units of blood ready and a perfusionist on hand to collect any blood she lost and recycle it back in.
• Whether the left eye could be saved.
• Whether the skin on the left side of her face could be saved or whether she would need, a ‘flap’, a transfer of skin from her back or arm, to her face.
• Whether she would need a tracheostomy (making an incision in the neck, into the windpipe to form a direct airway) to help her breathe.

Whilst everyone else worried over the enormity of the task, including the medical team, Regina remained confident that everything would be fine. Her confidence was founded on a dream she’d had previously where the operation had a successful outcome. A woman back in her home village of Prestea, Western Ghana, had informed Regina that she had also had a dream, in which her left eye was saved.

Regina’s faith in her dreams was rightfully placed and she triumphed against the odds. In a miraculously successful operation the one and a half pound tumour was removed in six lumps over the nine and a half hours. The perfusionist was sent home halfway through because there wasn’t even enough blood loss to cycle through the machine. The left eye was saved, all skin was conserved, and no flap or tracheostomy was needed.

This remarkable surgery shows the incredible difference modern medicine can make to the life of an individual. Not only did this operation save Regina’s life, it gave her hope of a life that she could only previously dream of. Since traveling back to Ghana she has gone from being ostracized to enjoying new found fame. At the airport, reporters were waiting for her and this has been followed by local television appearances. In the UK, she has made two BBC appearances and had several articles, including this one, written about her. She can now go to school, and make friends.

Regina’s success story is encouraging but can we really promote this kind of opportunity to everybody in less developed countries? Is it really economically viable to spend so much money saving the life of just one person? Why aren’t we helping the countries themselves to develop health systems so that they are able to treat their own people?

I spoke to the founder of Willing and Abel, Abi Boys, to find out more about her charities’ work and her thoughts on the ethical and social dilemmas that arise when her charity intervenes.

JR: How do you find the Kids that come to you?

AB: ‘Well several of the kids come from a hospital in Cameroon.... I worked there for 6 months just after I graduated from Medical School. The hospital has a massive catchment area - covering Chad, Nigeria and Libya as well as covering the whole of Cameroon. So I have developed a good relationship with the people that work there and they refer cases to us. Also the charity mercy ships refer patients to us, although Mercy Ship runs a full working hospital, in the form of a converted ferry along the coast of West Africa, they do have some limitations, and in more complex cases like Regina, they refer them to us. They needed somewhere with a big enough blood bank, and more specialist equipment for the surgery. There are also random cases that I find on my travels... such as a boy called Cedric. I met him on a plane in southern Cameroon... I could see he was jaundiced, and then he filled his nappy, I offered to help his mother as she changed him and noticed his poo was white... I suspected Biliary Atresia so took her e-mail address and things moved on from there. But we are open to receiving kids from any developing nations.

JR: And how do you decide which Kids you can help

AB: Each child presents their own very complex case, and so we treat each child on a case by case basis... taking into account not only the country they are from, their condition... but also their family and social situation and whether it is logistically possible, it gets quite complicated sometimes. We just transferred Aboubakar from Cameroon to South Africa via Chad and Ethiopia - but if it’s possible - even if its complex we figure that child is worth investing in.

JR: What are the main difficulties in organising for them to have treatment over here?

AB: The difficulties range from child to child and most are from rural villages; firstly we have to get the ID cards... in order to get Passports, before we can get visas. So you can see all the hoops we have to jump through to get things rolling. Then we often have arrange considerable transport just to get them to an airport, and brief them on very practical things such as using aeroplane toilets, using a knife and fork etc. and find suitable escorts.

JR: What are your thoughts on the economics of so much money being spent on one person?

AB: This is something that I am constantly thinking and being challenged about. I think there are two ways to look at it. Firstly, if you take a look at all the problems in the world... it can be overwhelming, you can see that you can’t solve every problem therefore you have a choice... something or nothing. And I decided it’s still worth changing something over nothing. Surgery is what I love best, it’s where I can help most practically and it’s close to my heart so I decided that I will do what I can to make some difference, doing what I do best.

Secondly, with the first case we had... a boy called Abel, whom the charity is named after, I had this patient in front of me and I had to find a solution to help him. I remembered something one of my heroes said... a woman called Heidi Baker. She works with orphans in Mozambique, she says ‘Change the world by seeing the one in front of you and loving that one’ the ethos of Willing and Abel is ‘See the one Love the one’ and as a Christian I look at human beings as a creations of God. He loves each one of us unfailingly and to him we are worth everything, so I should save the life of that one child if I can... no matter what the price.

JR: What about bringing them from their environments over to countries such as the UK for treatment?

AB: So it’s obviously not ideal bringing them out of their own environment and we try to send them to places that are of a similar culture to their own. But, for example, with Regina there was no other option apart from the USA or UK. We tried to find some home similarities for her here in London. A Ghanaian church was involved with her case, holding fundraising events for her here in the UK. When she was over here, Regina attended their church, where many of them spoke her language and were able to cook Ghanaian food for her and her father. We aimed to help them to not feel too overwhelmed and over exposed to our fast food, western culture, ultimately making sure that they don’t have any difficulties going back to their homeland after a sometimes lengthy stay in the western world.

After meeting Regina, I was so glad that the opportunity had been given to her in order to save her life, but I was aware of the enormity of cost and potential dangers of exposing her to apparent contradictory cultures. Although the worthiness of saving a life can never be questioned, it is important to consider if money would be better spent on long term projects and building local health systems. I think we would all agree that in the long term, building infrastructure, training locals in necessary skills and promoting health is one of the most beneficial ways to help countries ‘help themselves’ as it were, but this charity and case reveals a need, where the countries aren’t yet in a position to provide specialist treatment or help, and in this situation shouldn’t we do all that we can to fill this need? I think that the somewhat countercultural ethos behind Willing and Abel; an immense love for the individual, is an an attitude that, whilst keeping in mind the bigger picture, maybe we should be embracing more in our society today.

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Jennifer Raw is a second year Medical Student at University College London.

Further Reading

Find out more about the charity 'Willing and Abel', and Regina's story, by following this link.