Top critical review
A good read if you're going to do genuine research on SCID afterwards (CONTAINS MAJOR SPOILERS)
on 13 September 2012
I first read this book when I was about sixteen years old, in the school library one lunchtime. At the time, I had already heard of SCID, but it was so mindbending - the bubble concept, that I really couldn't deal with it. So this book was an interesting look in at the management of someone in chronic sterile isolation - and the things this entails. Recently, I have been taking more interest in SCID, so ordered the book for myself. I have since been reading and re-reading it.
Adam Simmonds suffers from SCID and has lived in sterile isolation all his life. Following the loss of a previous baby - Sarah - who had SCID and passed away at four months, Adam is delivered by Caesarian and placed in a sterile isolation bubble). He and his mother move to a new neighbourhood. There Anne (the main protagonist) and her class are carolled by their teacher into taking weekly lessons at Adam's house, to give him some peer company (much to the dismay and digruntlement of Adam's overprotective mother). Adam has already unintentionally terrified Anne on one previous chance encounter, but she can't help but be fascinated by him as well. She makes SCID her class project, and thus begins a friendship with Adam in return for being his 'life taster' This friendship blossoms into romance; however, Anne accidentally lands Adam with a storm paparazzi attention after arranging a trip to the cinema in his transport bubble for his sixteenth birthday. Anne feels very guilt afterwards and cannot face Adam, but Adam loves the attention. Since Adam can never leave his bubble, or even touch her, and Anne struggles with the normal feelings of sexual confusion, adolescence and teenage angst, their relationship, however true, does suffer. Then some doctors read about Adam in the papers and offer to try a ground breaking bone marrow transplant, which may allow Adam to grow an immune system and walk out of his bubble forever. Adam goes through this, but unfortunately becomes ill immediately afterwards, necessitating his premature removal from the bubble. Treatment doesn't work, and around Christmas time, after Anne sneaks into his room and they secretly make love, Adam passes away, leaving Anne with a host of confused feelings, and the task of letting him go, and tasting real life for herself.
This book is heavily based on the true story of a little Texan boy, David Vetter. David, like Adam, was born by C-section in 1971, after the loss of his older brother at seven months from SCID. He was placed in a sterile isolation bubble where he lived all but two weeks of his life. Like Adam, David lived off baby food for a long time, until tinned food was substituted, having been thoroughly tested for bacteria first. Like Adam, David had to have his food lukewarm. Like Adam, David had to use a plastic potty instead of a toilet. Like Adam, everything going in and out of the bubble had to be thoroughly sterilised by the hospital first, in a time consuming process. Like Adam, a film was screened specially for him at the cinema, which he attended in his transport bubble. Like Adam, he suffered a power outage which threatened to leave him with no breathable air, to be rescued by a generator. Like Adam, following a half-matched bone marrow transplant in 1984 (in David's case it was from his sister, Katherine) David, then aged twelve, became ill and had to be removed from the bubble for treatment. Like Adam he died shortly after. Unlike Adam, the cause of death was identified by postmortem as lymphoma - triggered by traces of the dormant Epstein-Barr virus in Katherine's blood (then undetectable by blood screening). Unchecked due to David's lack of immune system, the cancer ran wild in David's body, causing his illness and death. Since David lived in such controlled conditions, this was the first conclusive proof that the virus could trigger cancers.
There are many medical accuracies and observations about this book. However, there are also key inaccuracies that I think it is only fair to point out in the interests of correct information:
- Adam had an older sister with SCID. David's form of SCID was sex linked - as the mutation was carried on the X chromosome (and all females have an extra backup copy of this), only males could get his form of SCID. There are other forms of SCID that affect both males and females too, but these are much rarer. So Adam must have had a different, rarer type. This is not strictly speaking an inaccuracy, as it would require the same degree of isolation, and be treated the same way - with a bone marrow transplant - but it is worth pointing out as the book does not mention this or explain it.
- Unsurprisingly, given the fictions and media coverage, one might assume that sterile for life is, or used to be, the norm for treatment of SCID. In fact, this is even stated in a medical definition of SCID that Anne reads at one point. In fact this is not the case, and never was. In David's case, his older brother had been a tissue match with his sister Katherine and a bone marrow transplant was carried out, but the baby died from pneumonia at seven months. Assuming David would also be a match with Katherine, the parents coneived and carried him to term, delivering into isolation with the view to doing the bone marrow transplant as soon as possible. It was only after it was discovered that Katherine and the parents were not matches for David that the isolation bubble became, inadvertently, his permenant home while they waited for medical science to catch up. It almost did. David is the only boy in medical records, who spent his life in an isolation bubble as a long term management. After David, such systems were considered unethical and untennable due to the developmental differences and deprivations he faced, and the reductions on his quality of life. It is never explored in this book, what the doctors' ethical justifications are for electively putting Adam into long term isolation, when no soon hope of a cure for his type of SCID existed at the time of his birth.
- Apart from obvious things like never having felt wind, felt grass under his feet, worn shoes or felt the touch of human skin, the book does not really go into depth about the psychological differences in perception experienced by someone who has spent their whole life in a bubble. Adam seems surprisingly well adjusted and educated about the world considering his lack of personal experience, lack of a peer group and his overprotective mother. On the other hand David had a rocking and thumb-sucking habit (seen in other children who spent time in isolation). This stopped as soon as he came out of the bubble. He also never understood the phenomenon of visual perspective - to him buildings on the horizon really WERE small, and really WERE rectangular and nothing would sway him in this view. When he went outside in a mobile germ-free suit that NASA made for him, it never occured to him to walk round the house to find the back yard. He went straight out forwards, because he didn't understand that houses were 3D and had backs as well as fronts. He didn't understand what was happening if, watching TV, he saw a person riding over a hill - someone had to explain what was going on and for that reason, he preferred shows set in one room, such as chat shows, which he could relate to from experience. None of these are the case with Adam - he seems to have gained understanding. Perhaps it comes with age, as Adam spends considerably longer in the isolator than David, and is quite a bit older than David was when the story begins. On the plus side the painstaking politeness combined with subtle egocentricity, gently frustrated imperiousness and need for control, that David cultivated out of a skewed and often unstructured world over which he had little control; an extensive reading and categorising of people and motives; and a need for subservience to those he was dependent upon, was portrayed very well, I thought, in Adam in this book.
- In David's case, it took a dedicated team of technicians, electricians, researchers, consultants, psychiatrists, psychologists, hospital staff trained in sterilisation, and a dietician to keep him alive and continually maintain/repair the slowly wearing and tearing isolator bubble system. There seems to be surprisingly little contact with these people in this book. Over time discussions between specialists and people working with David, about the justifiability of keeping him isolated as he grew older, with no cure in sight and a reduced quality of life, intensified exponentially. Again, we see none of this - although given the book is told from Anne's point of view, perhaps that is unsurprising as she would not be party to them.
- I was curious to know what the particulars of the strain placed on Adam's body were that finally killed him. Was it lymphoma, like David, or something else? What would cause such a strain in an otherwise healthy - moreso than most, arguably, given he is infection free - person? He hadn't had chemotherapy to put his body under strain, as most people do (chemotherapy is only needed to prevent the patient's immune system rejecting the bone marrow, but those with SCID have no immune system to prevent, so they don't need it). Without a justifiable explanation, the logic here, for me, fell apart and I was left asking why it had to end sadly with no good reason? Although at the same time, I understand that in the book they are dealing with the unknown, so perhaps it is not a totally unjustified outcome, that they are left wondering what happened, rather than finding definite answers.
As to the main story - Anne's story - I honestly felt there was nothing very special about it, which is why I decided to be such a stickler for accuracy concerning Adam in this review. She talks about herself and her physical imperfections, her friend who's sluttiness disguises actual sexual confusion and insecurity (ending happily, though someone abruptly, with her acquiring a steady boyfriend and a dramatic shift in her interests), how she feels about boys, how she feels about her classmate and family. Anne's voice itself though, and her way of expressing herself, is VERY well done and believable - although it does get a bit wearing when she KEEPS on putting herself down, whingeing to anyone who listens about the trials of her life, and narrating in an ironic tone. But at the same time, that's part of the brilliance of the voice, as it rings so true of many teenagers. Despite all that she's a courageous and sympathetic character, simply because she always tries to do the right thing in the end, and tries to reach people for their own sake rather than for some gain for herself.
All in all, definitely worth the read, and very emotional first time round too. But for best results and more insight, combine with real life research on SCID as well.