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Cystic Fibrosis, Trikafta and Christian Hope

Gareth Jones —

As an academic, most of my time is spent writing and speaking on biomedical issues from the standpoint of an individual whose outlook is informed by his background as a biomedical scientist, a biomedical ethicist, and a Christian.

In practice, all three contribute to a comprehensive picture that is, hopefully, true to each of these inputs. Unfortunately, if the human context is downplayed or even ignored, the result can be dispassionate and unduly theoretical. It is, therefore, helpful when there are personal connections since these throw a different light onto the dilemmas, and for the Christian, the science-faith connotations are brought into the open. It is such a personal perspective that informs the present article.

This article has focused quite deliberately on one condition and one family. This is not meant to amplify unduly the importance of this one condition but to reflect on it from an insider’s perspective. It is also a relatively rare condition but far from an extremely rare one. I could have focused on a large number of rare conditions, such as achondroplasia, Marfan syndrome, one of the lysosomal storage diseases, or neurofibromatosis. I could also have raised the question of expensive life-saving treatments for common disorders that are not readily accessible using public funds. But that is not the point of this article. Rather, it is to understand how Christians can come to terms with the fragility of human life and the increasing range of medical and scientific approaches becoming available to alleviate the worst of the effects of childhood diseases. Where is God’s hand in all this, and how do we recognize what is beneficial and what may be ambiguous?

Personal Reflections

Twelve years ago, my wife and I had two new grandchildren, born one day apart; a girl in one case and a boy in the other. The girl was everything one would expect of a lovely new-born child – the epitome of a ‘perfect’ new human being. She was self-evidently a gift of God, delightful and everything one could wish for. And she has continued to develop in this way, bringing immense joy to her parents.

The birth the following day of a little boy initially elicited similar responses, and yet within a matter of hours, these were overtaken by worry and deep concern when he had to have major surgery for bowel obstruction. This immediately suggested the diagnosis of cystic fibrosis (CF), a diagnosis confirmed by genetic analysis a couple of weeks later. The treatment he received at the time and has subsequently received has been state-of-the-art in a research-intensive children’s hospital, and his progress has been encouraging even though there have been inevitable setbacks and numerous hospital visits. The demands on his parents who love him dearly have been immense, and they have continued unabated throughout the years.

The contrast between the two births was dramatic, as has been the ongoing trajectory of natural growth and development in the one case, over against intense therapy-dependent growth and development in the other. Healthy development in the one is what one would usually consider to be ‘normal’; in the other, it is dependent upon high-grade technology in the form of very sophisticated antibiotics, drugs, and inhalers. If he had been born 30–40 years before he was, it is very unlikely he would still be alive. For instance, when the genetic disorder was first discovered in the 1930s, 80 percent of children born with CF died before the age of one. By 1970, life expectancy was around 12 years. Today, the median life expectancy of people with CF is around 40, although this varies considerably from one country to another. In other words, if very sophisticated facilities had not been available, and if the substantial costs could not have been met, he would have died during early childhood. As it is, his future lifespan and the quality of his life depend upon the continuation of the constantly improving treatment available to him, stemming from ongoing ground-breaking genetic and pharmaceutical research.

The costs of research over the past 40 or so years have been extraordinarily high (in the billions, not millions, of dollars), but it has been the range and nature of this research that has been responsible for what have been truly transformative results.[1] In other words, the quality of life of most patients with CF is completely dependent upon sophisticated science and technology. This is not an option but a necessity. Science and technology are enabling individuals with CF to realize far more of their potential as human beings than would otherwise be possible. In a small way, CF is reminiscent of the plight of the whole creation, that has been subjected to frustration; groaning as in the pains of childbirth, and longing to be liberated from its bondage to decay (Romans 8:20–25). This longing is eloquently displayed in the lives and trials of young people with CF.

The context for viewing CF is that this is God’s world, and children are a gift from God, regardless of any biological ‘deficiencies’ or ‘imperfections.’ All are to be loved and cared for. This is the fundamental premise that lies beneath every other reflection. From this, it follows that everything possible should be done to assist them, even though the sad realization is that what is feasible varies hugely from one country to another and even from one area to another within the same society. Children with the same condition and theoretically with the same life chances will have the opportunity to prosper in one case but not in another. Unfortunately, there is a major degree of inequality and unfairness in life, and this applies as much in this domain as in all others.

The Christian’s duty is to advocate for as much equality as can be achieved in whatever ways are feasible. This will range from advocating for high-quality public health systems open to those with limited means, providing for a wide spectrum of disease conditions, including unglamorous ones like behavioural and mental health conditions, such as foetal alcohol spectrum disorder (FASD). Those with expertise in these areas should be supported by the church as they seek to bring attention to the need for improvements in relevant health and welfare services to provide for those all too readily ignored by society. Many of these conditions are long-term ones, meaning that support has to be sustained for decades in many cases.

In light of these considerations, gratitude is to be shown for what is made available, scientifically, socially, and communally; all of which are blessings from God. The work of research scientists, doctors, and other healthcare workers is to be received as an outcome of God’s general providence and grace. This is demonstrated by the manner in which God provided for the Israelites, through the manna and quail in the desert, as long as they followed his directions about when to eat it and to learn to trust God for his provisions (Exod 16:1–36). Human agency should always be seen alongside God’s providential action since we are to learn that he has provided the means by which human beings are to respond to vicissitudes such as a pandemic. Utilizing the scientific means at our disposal is a response to God’s overtures, whether recognized or not, rather than an exercise in rebellious autonomy. Gratitude is also to be shown for a society’s political systems and health policies that make therapies accessible as equitably as possible across a whole society, regardless of any ability to pay for life-transforming medications.[2]

The love and care shown by many parents to their children with chronic illnesses, especially when the ongoing demands on their time and resources are steep and very draining, are a source of considerable gratitude. They are to be encouraged and supported in as many ways as possible. Where church communities can assist, they are to regard this as an important outworking of the gospel in loving and serving those in need of assistance. The example provided by the early churches’ support for widows in need constitutes a germane blueprint here (1 Timothy 5:3–16). Of these, some were in real need, whereas others were not. This distinction may be helpful in responding to some diseases, and yet this is unlikely to assist in approaching the treatment that is truly transformative, where all who could benefit using medical (genetic) criteria fit the category of being in real need. In the unlikely event of some being funded and others not, regardless of their clinical condition, the real need distinction becomes relevant.

The Harsh Reality of Cystic Fibrosis

Approximately 70,000 individuals worldwide have CF, a condition that is more common in Caucasians of European descent.[3] Cystic fibrosis is the most common autosomal recessive cause of early mortality in Caucasians worldwide[4] and is caused by a recessive gene that manifests itself when both parents are carriers. Around one in twenty-five people in the population are carriers, although most are unaware of their carrier status. When both parents are carriers, the chance of having a child with CF is twenty-five percent.[5] This is due to a defective protein that results from mutations in the gene encoding the cystic fibrosis transmembrane regulator (CFTR). These mutations reduce the release of chloride ions in epithelial tissues, and hyperactivate the epithelial sodium channels that aid in the absorption of sodium ions.[6]

The result is that the mucus becomes dehydrated and thickened, making it suitable for bacterial growth. This occurs in a range of organs and body systems, including the sinuses and lungs in the respiratory system; the pancreas, liver, gallbladder, and intestines in the digestive system; the reproductive system; and the sweat glands. One result is that the sweat is very salty, meaning that people with CF lose large amounts of salt. In the lungs, the thick sticky mucus leads to shortness of breath, a chronic cough, and repeated chest infections; in the pancreas, it reduces or stops enzymes from being released into the gastrointestinal tract to digest food, causing problems with poor weight gain and malnutrition due to malabsorption.[7]

Affected individuals take numerous medications each day (including intensive antibiotic therapy and aerosols) from as soon after birth as possible, and they receive constant physiotherapy. They eat extra food (gorging on fatty foods, a challenge when siblings are discouraged from indulging in such diets) and are encouraged to exercise vigorously. As the individual with CF gets older, the disease advances, and hospitalizations increase in frequency. A major thrust of therapy is to delay for as long as possible damage to the lungs since, until very recently, the only avenue open to them at that point was a double lung transplant.[8]

The well-publicized story of an individual with CF has been that of Alexander Stobbs in the UK. He has been one of the most high-profile people with CF, as a result of a 2008 UK TV documentary on him entitled, A Boy Called Alex. This was followed by his 2009 book, A Passion for Living.[9] As a choral scholar at Kings College Cambridge, he sang in the choir a few times each week on top of his normal studies and then topped this off by conducting a performance of Bach’s St Matthew Passion in his first year. Previously, while still at school, he had conducted Bach’s Magnificat. Alex Stobbs is exceptional on two counts: his musical ability and his determination to live life to the full in the face of a life-threatening condition.

Now aged 31, CF has dominated his life. In his own words:

I’m a musician, and my ability to compose, practice for and participate in rehearsals and concerts as I used to had been rapidly decreasing. Seeing friends who I love involved a great deal of apprehension, simply because I knew I’d be out of action for the next few days due to the tiredness it would cause. Large parts of my day were spent doing physio, treatments - or just resting due to my energy levels being depleted. As well as using oxygen a great deal at home, I was also using a portable oxygen concentrator on the rare occasions I did leave my flat, which was, psychologically, a huge change that I never fully got used to.[10]

He has had to spend many weeks of the year in hospital for intravenous antibiotic treatment.

For four years, he was on the lung transplant list, during which time his life was constantly on hold and was marked by uncertainty. This, in part, was due to having his phone on constantly in case a call came through from a transplant coordinator. Recently, all this has changed on account of a major breakthrough in treatment (see below).

The Apotheosis of Scientific Discovery

From the initial description of CF in 1938, little happened until 1953, when it became evident that people with CF have excessively salty sweat. Over the next 30 years, there was consistent scientific progress, much of it in the US, driven by those with personal experience of having children with CF. This was supported by philanthropists and diverse groups of scientists with myriad complementary disciplinary skills.[11] The trajectory is a compelling one, as advocates for CF children and their families made conspicuous strides with both the scientific work and the philanthropy on which it was heavily dependent. Invariably, they were driven by sadness at the serious illness of these young children and all-too-often their early deaths. Tragically, in some instances, it was not just one child in a family afflicted by CF, but two and even three in the same family. Life expectancy during the 1950s–1970s was low, with children usually dying in their teens and earlier. What stands out from these years are the huge costs required for the scientific work, most of which was privately sourced, the ability of those behind the various CF foundations to repeatedly raise countless millions of dollars, and the creativity and commitment of scientists from a wide range of biomedical disciplines. Early on, it was realized that treatment had to begin early, ideally from birth. This was because the longer there was no treatment, the greater the damage to the lungs as well as other organs. Consequently, children who had reached the age of two without any treatment had a severely reduced life span.

Over these years, there were major breakthroughs in unravelling the cellular defect in CF, followed by the discovery of the CF gene in 1989. It is interesting to note that one of the main geneticists behind this discovery was Francis Collins, currently Director of the National Institutes of Health in the US, and a leading evangelical scientist and the founder of Biologos, the organization that emerged from his book: The Language of God.[12] Each of these discoveries involved vast swathes of work, inevitably encountering one blind alley after another. A therapeutics development program was initiated, with efforts directed towards developing drugs that would correct the non-functioning CFTR protein, the long-term aim being to work towards a cure for CF.

Increasing understanding of CF revealed its complexity, in particular, the approximately 2000 known mutations of the CFTR gene, the most common of which is the F508del mutation.[13] The challenges posed by this complexity have become evident over recent years as concerted attempts have been made to understand the disease and its mechanisms and sketch ways of subsequently correcting the disorders. Progress has been gradual but has also been substantial, as life expectancy has increased from one year to the next. Not only this, morbidity has been reduced, as one drug after another has come online.

The drugs were designed very precisely, the aim being to correct different mutations, with clinical trials following at each stage. In this way, one milestone after another was passed, with drugs emerging that would prove helpful for one group of CF patients after another. By 2008, it became apparent that it is possible to treat the root cause of CF, leading in 2012 to FDA approval of Kalydeco for a number of specific, but largely rare, CF mutations (G551D), that affect four to eight percent of CF sufferers.

Scientific developments took place one step at a time. In 2015 FDA approved the drug, Orkambi, for people with two copies of the common F508del genetic mutation. This was followed in 2019, by the next major step with the drug, Symdeko(tezacaftor and ivacaftor tablets). And finally the triple drug at the heart of this article, Trikafta (elexacaftor, tezacaftor, and ivacaftor tablets). This was the first triple-combination therapy for those with the most common and most severe type of CF, and in doing so marked what could be the start of a new era for many CF sufferers. Each new drug represented a distinct advance in the efficacy of treatment, with the appearance of Trikafta signalling a move to a new plane of effectiveness. Over the years, numerous combinations of molecules were tested through all three phases of clinical trials, with each molecule working on a different part of the CFTR protein. The aim in all cases was to restore the balance of salt and water in the lung, pancreas, gut, and sweat glands.

Trikafta, in particular, marks the beginning of a new era of genetic medicine. It is approved for patients 12 years and older who have at least one F508del mutation in the CFTR gene. This is estimated to represent ninety percent of the CF population. Its three component drugs each target different areas in the defective CFTR protein; the goal being to enable the protein affected by the CFTR gene mutation to function more effectively.[14] This represents a major advance even though some patients have mutations that are ineligible for treatment. When successful, the broken channel in the CFTR protein is fixed with a synthetic molecule that in turn changes a patient’s entire physiology, from the saltiness of their skin to the volume of air in their lungs.[15]

However, as with any drug regime, there may be adverse reactions, that have to be taken into account by those involved in administering and taking the drug.[16] These are not the subject of this article and will not be traced any further, but they serve as a reminder that no matter how effective a treatment may be, there will always be limitations and restrictions.

The manner in which Trikafta has transformed the life of Alex Stobbs is illuminating, an experience that is far from unique. He has commented:

I take two tablets in the morning and one tablet in the evening - both with a good dollop of fat. That’s it. It boggles the mind how such a simple routine (3 tablets!) can bring about such dramatic changes in my life. Kaftrio (the name of the drug in the UK; also known as Trikafta) started having an effect within an hour or so after taking my first dose. Two weeks after starting it I no longer woke up with 10-minute coughing fits – in fact I barely cough at all. My lung function is the best it’s been in at least 12 years (risen by 10–12% so far, from an average of 32–34%.)[17]

He has more energy than he can ever remember; he has gained weight, he can walk up and down hills for kilometres at a time (without oxygen), and he has commenced jogging. He can even contemplate playing sport. This transformation is well known, but unfortunately, it is extremely expensive (currently $469,000 per year in New Zealand) and requires the country’s health services (or occasionally a benefactor) to cover the costs.[18] Remarkable as it is, it is not a cure and is best suited for those patients with one or two copies of the F508del mutation.

There is also a small percentage of patients with CF who have what are described as ‘nonsense mutations’ and who lack any CFTR protein. There are no therapeutic avenues open to them at present, leading to thoughts once more of gene therapy, an approach that was tried a number of years ago without success.[19]

This brief account of the progression through the stages of scientific discovery fails to do justice to the excitement, creativity, and commitment of the numerous individuals and groups responsible for what can only be described as a remarkable unfolding of scientific comprehension. The progression was based on gradually increasing knowledge of the pathology underlying CF and how it might be rectified. Each new drug has been designed to perform a particular function and overcome a specific error in the mechanisms controlling the movement of chloride ions into and out of cells. It has amounted to a very determined series of efforts to understand the basic cellular errors giving rise to CF. This is exceedingly methodical science at all levels, from the cellular to the clinical, made possible by remarkable human ingenuity and driven by the needs of patients and their families.

Reflections on Science and Faith

The developments outlined above can be viewed simply as a case study in some of the remarkable achievements of medical science in providing hope for a relatively small number of patients at enormous expense. One interpretation is that this is science and medical treatment for the elite. A countervailing message is that the lives of people with a debilitating and life-shortening condition will be transformed and enhanced, and they should be offered healing if available. Beyond this, the knowledge gained by this research will eventually permeate through to other conditions, including far more common ones.

It would be unhelpful to expect parallels in Scripture, but the account of the healing of the blind man is instructive (John 9). Here was a condition that had been present from birth, just as CF is present from birth. Jesus was adamant; neither this man nor his parents had sinned, but it provided Jesus with an opportunity to heal him. And this is exactly what he did, using materials at his disposal. For Jesus, this was an opportunity for the works of God to be displayed in and through this man. Of the many elements within this episode, one that stands out is the simple retort that: “I was blind but now I see” (John 9: 24). While any similarities between this healing and that in CF should not be overstated, a number of helpful lessons emerge: there is a place for the healing of congenital conditions, there is a role for physical (scientific/medical) intervention, and God can be glorified when interpreted appropriately. Christians can find hope through healing and can rejoice when illness is successfully combatted. The multiple attempts at finding ways of healing CF as outlined previously do not signify a lack of faith on the part of Christians; they illustrate the use of God-given abilities to rectify pathologies that destroy all that is best in God’s world.

Once the fundamental premise that this is God’s world is accepted, there is no place for either a sacred-secular divide, or alternatively, a science-faith dichotomy. This is because the world we know is God’s handiwork and incorporates the actions of the humans he has brought into being. True, there is much we do not understand, and there are many aspects of the world that seem to be out of control and broken. But it is also a world in which humans have been given responsibility for themselves and their environment, including the task of being co-creators with God in improving conditions and rectifying what has gone wrong (Psalm 8:5–8; Heb 2:6–8).

In these terms, the overwhelming response should be one of gratitude at the power of science and the fruitful way in which it has been applied to improve the health and prospects of one particular group of patients. This is consonant with the well-known trajectory of Christian health care through the ages. It is putting science to good use in showing love for one’s neighbour and in seeking to rectify that which is out of control and destructive (Mark 12:30–31; James 1:27).

God’s purposes in helping broken people depend in part on scientific input that is integral to his providential purposes. Not all scientific developments are valuable, nor can all be justified, because scientists are broken, as are the remainder of humankind.[20] However, the course of the discoveries, in this case, do not stem from malevolent intentions on the part of any of the major players; they have been driven by concern for the sick and for uncovering means of remedying the sickness. Consequently, each scientific project is to be assessed on its merits, using the biblical adage that “you will know them by their fruits” (Matt 7:16–20). They illustrate very convincingly that God’s care and human care are, in this instance, directed towards the same ends. God’s care reminds us that what is important is the way in which we care for others, not the way in which we attempt to control others and lord it over them.[21] The scientific work behind CF has had as its goal the prospect of adding value to the life experiences of these children and young adults, thereby enhancing all they are as those who image God and providing them with a better way.

This is the positive side of science that is to be applauded in the case of CF. But even here there is a necessary counterpoint, namely, science must never be elevated to the status of an idol. Even the very best of human endeavours, including science, can become idolatrous.[22] Nevertheless, this fear, genuine as it may be, should not be used as an excuse to downplay the contribution of science within God’s purposes. It is not to be feared but to be utilized in the service of God’s creation. But what about the cost of the drugs? The enormous developmental costs cannot be downplayed, but there is little value in devising therapies, especially dramatically transformational ones, if they are to prove too expensive to be used by those for whom they were designed.[23] This is an ethical conundrum, that undoubtedly has theological overtones.

The costs of Trikafta are extraordinarily high, raising the question of whether those suffering from CF and their parents can legitimately expect such costs to be covered by the public purse. In Christian terms, can one expect what might seem like elite treatment? This is not a question confined to CF; hyper-expensive treatments are a feature of potentially effective and highly innovative treatments for a range of conditions, from heart transplants to gene-targeting therapies. The competing arguments over the cost of drugs have been traversed repeatedly over recent years, and the problems are likely to increase as more scientific breakthroughs come onto the market. The costs of drug development, clinical trials, and production often run into billions of dollars. Debate centres on what costs have been borne not by the pharmaceutical companies but by universities and hence public money, and also by private money. When patents expire, costs can be reduced when generic, and biosimilar drugs become available, but these will be future prospects.

This leaves unanswered the moral questions of having drugs available, but being priced out of reach of ordinary people. The question then becomes how much health benefit will they provide? Is this a few weeks or months of a better quality of life for adults, or is it many years of good health for young people who otherwise would die at an early age? The moral imperative for the latter is far more pressing than for the former, no matter how much one would always like to be able to improve an individual’s wellbeing. The tensions are palpable in high-income countries but are heart-rending in low-income ones, where there is insufficient support for routine, let alone cutting-edge, treatment. They bring into the open the financial resources available, or not available, for public health care in respective societies and the fairness of government policies. In considering the matter for Christians, it is pertinent to ask whether it will enable those receiving treatment to more clearly mirror their design as one of God’s creations? Will it, in other words, bring glory to God?

Trikafta comes out positively on a cost-benefit analysis, although huge efforts are needed to ensure that the drug company is not being exploitative in seeking to maximise its profits by charging whatever prices it thinks the market will bear, especially when much of the research was funded by private interests. The case for providing hyper-expensive treatment such as Trikafta for many (not all) CF patients stems from the large number of life years it promises and the transformation of a sickly young person into a flourishing adult.

The challenge of the biomedical realm is that biomedical endeavours are inseparable from specifics demanding precise and, on some occasions, contentious answers.[24] Seeing God in medical treatment is ambiguous, since we are let down when healing does not come and when research leads nowhere. Even the most sophisticated biomedical science will let us down, and some young people with CF will die at a tragically young age. Our expectations will be dashed. Where then is God in this journey of what may be interpreted as premature decline? Technology per se has no answer to decline and death, no matter how much life expectancy is increased. These constraints apply to the most exciting advances in the treatment in CF, as in any other area. Trikafta, with all its stupendous results, will not lead to an illness-free existence, but neither is this to denigrate its potential benefits and blessings.

A Christian paradigm faces up to the inevitability of suffering and mortality, not in a fatalistic way, but by seeking to be faithful to Christ in the midst of suffering.[25] When confronted by suffering and uncertainty, the Christian is to examine the technology available and the manner in which it might be used to assist in this situation. How best can I respond as a follower of Christ, whether or not success eventuates?

Trikafta shows genetic medicine at its best. This is far removed from theorizing that it will usher in a new Jerusalem or alternatively lead to an Armageddon. Such scenarios lack an informed scientific base and are unhelpful and misleading. The example of Trikafta and CF brings the discussion back to the reality of real human suffering and of stunning medical improvement. A basis in science and medicine is frequently a good place to start for theological analysis by bringing to the fore an evidence-based approach.

Trikafta is a basis for Christian hope, not as an end in itself, but as a pointer to the works of God and the manner in which he uses the gifts he has bestowed upon his created beings. It is one of the pieces of a jigsaw, the completion of which represents one means of restoring a broken world. It is a sign of hope when approached through the looking glass of the redemption made possible by Christ and the eternal hope that stems from what he did on the cross and in being raised from the dead.

Gareth Jones is Emeritus Professor of Anatomy, University of Otago. After being Head of the Department for many years, he served as Deputy Vice-Chancellor (Academic) and then as Director of the Bioethics Centre. He has written extensively on biomedical ethical issues in the academic literature and also at the science-faith boundary within a Christian context.

[1] Bijal P. Trivedi, Breath from Salt: A Deadly Genetic Disease, a New Era in Science, and the Patients and Families Who Changed Medicine Forever (Dallas, TX: BenBella Books, 2020). This book is a mine of information about every aspect of CF and the philanthropic and research endeavours that lay behind the impressive strides made over 50 or so years.

[2] D. Gareth Jones, “A Pandemic, Science and Faith,” Stimulus 27.1 (2020): https://hail.to/laidlaw-college/article/AxfHv3n/print.

[3] For details see, Don B. Sanders and Aliz K Fink, “Background and Epidemiology,” Pediatric Clinics of North America 63/4 (2016): 567–84. See also, Virginie Scotet, Carine L’Hostis and Claude Férec, “The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery,” Genes (Basel) 11.6 (2020): 589.

[4] Pamela B. Davis, “Cystic Fibrosis since 1938,” Am J Respir Crit Med, 173/5 (2006): 475–82.

[5] Sanders and Fink, “Background and Epidemiology,” 567–84.

[6] Liam Drew, “Research Round-up: Cystic Fibrosis,” Nature 583 (2020): S18–19; Fahad A. Almughem et al., “Cystic Fibrosis: Overview of the Current Development Trends and Innovative Therapeutic Strategies,” Pharmaceutics 12.7 (2020): 616.

[7] James R. Yankaskas, Bruce C. Marshall, Beht Sufian, Richard H. Simon, David Rodman., “Cystic Fibrosis Adult Care: Consensus Conference Report,” Chest 125 (2004):1S–39S. Also Davis, “Cystic Fibrosis since 1938,” (2006).

[8] Cystic Fybrosis NZ, “Treatments & Care,” 2020, https://www.cfnz.org.nz/life-with-cf/cystic-fibrosis-care/.

[9] Alexander Stobbs, A Passion for Living: The Amazing Story of a Boy Who Makes Every Day Matter (London: Hodder and Stoughton, 2009).

[10] Royal Papworth Hospital, Kaftrio: Turning Around the Lives of Patients with Cystic Fibrosis, 2020. https://royalpapworth.nhs.uk/our-hospital/latest-news/kaftrio-patients-cystic-fibrosis-Alex-Stobbs.

[11] Trivedi, Breath from Salt, 506–509.

[12] Francis S. Collins, The Language of God: A Scientist Presents Evidence for Belief (New York: Free Press, 2007).

[13] Nathan Arnold, “FDA Approves New Breakthrough Therapy for Cystic Fibrosis,” 2019, https://www.fda.gov/news-events/press-announcements/fda-approves-new-breakthrough-therapy-cystic-fibrosis; Trivedi, Breath from Salt.

[14] Trivedi, Breath From Salt, 492–97.

[15] Trivedi, Breath From Salt, 397–410; 473–76.

[16] FDA. News Release: FDA Approves New Breakthrough Therapy for Cystic Fibrosis, 2019. https://www.fda.gov/news-events/press-announcements/fda-approves-new-breakthrough-therapy-cystic-fibrosis. See also Trivedi. Breath from Salt.

[17] Royal Papworth Hospital, Kaftrio: Turning Around the Lives of Patients with Cystic Fibrosis, 2020. https://royalpapworth.nhs.uk/our-hospital/latest-news/kaftrio-patients-cystic-fibrosis-Alex-Stobbs.

[18] Trikafta (known in the UK as Kaftrio) has been available in the UK on the NHS since June 2020 for CF patients 12 years and older with one or two copies of the F508del mutation. At the time of writing (April 2021) it has recently been approved in Australia to treat certain people with CF; it has yet to receive government funding. In New Zealand ivacaftor (Kalydeco) is publicly funded for those with the G551D mutation, but Trikafta is not; see Pharmac, “OIA response: public funding of Trikafta,” https://pharmac.govt.nz/about/what-we-do/accountability-information/official-information-act/2020-oia-responses/oia-response-public-funding-of-trikafta/.

[19] Trivedi, Breath from Salt, 478–86.

[20] Jones, “A Pandemic, Science and Faith.”

[21] D. Gareth Jones, The Peril and Promise of Medical Technology, ed. R. John Elford and Simon Robinson; New International Studies in Applied Ethics 8 (Oxford: Peter Lang, 2013), 231, 233.

[22] Christopher J. H. Wright, “Here Are Your Gods!”: Faithful Discipleship in Idolatrous Times (London: IVP, 2020), 52–65.

[23] David Hunter and James Wilson, Hyper-Expensive Treatments, Background paper (Nuffield Council on Bioethics, 2011); “How Should Therapeutic Decisions about Expensive Drugs Be Made in Imperfect Environments?,” AMA Journal of Ethics 19.2 (2017): 147–56.

[24] D. Gareth Jones, “The Changing Face of the Science-Faith Dialogue in a Biomedical Arena,” Perspectives on Science and Christian Faith 68.3 (2016): 165–75.

[25] Jones, “The Changing Face of the Science-Faith Dialogue in a Biomedical Arena.”