Occupational Therapy which was established in America according to Hussey, Sabonis-Chafee O’Brien (2007) on March 15, 1917; has become of international importance in the health science field. Occupational Therapy which has become international is important to France which lies between Italy and Spain in Western Europe. France has been an active member of the World Federation of Occupational Therapy since 1964 (WFOT, 2004). According to (COTEC 2009), there are 6,553 Occupational Therapists working to serve 63.8 million inhabitants of France (France-Diplomatie, 2008).

France has a bicameral parliamentary system for a government, and also has a President and an appointed prime minister (France-Diplomatie, 2008) which is similar to the American President and Vice President. France is a prosperous country that also has national healthcare system, that it uses to supply its’ citizens with medical care. “France is the fifth largest economy in the world in terms of GDP – € 1,792 billion in 2006” (France-Diplomatic, 2008).

Unfortunately France has a stressed National Healthcare plan, and as life expectancy grows in France, the burden is on France’s national government to find ways to find financial support, Sorum (2005). The democratic government has a universal healthcare plan that is primarily supported through health insurance funds and the social security system which pays for roughly 86% of the population according to Sorum (2005). Current legislation has changed the structure of the healthcare program. This legislation was a result of an upheaval in the medical field. Sorum (2005) p.237, “At the end of 2001, many generalists refused to do night or weekend call until their reimbursements were increased from 17.53 to 20 Euros for regular office visits, 30 Euros for home visits, and more for night office visits”. Legislation passed in 2003-2005 went into effect to answer many of the healthcare problems. According to Sorum (2005), a general increase in co-pays along with an increase in fees charged and an increase in taxes of the whole society served to please all parties and stabilize the struggling healthcare plan (p. 239). This action seemed to emulate some of the methods used by neighboring countries with similar plans and healthcare woes.

One would believe that with a universal healthcare program that healthcare would be client-oriented. Hutton Andral (2000) found though, that methods of treatment are prescribed according to reimbursement by society rather than cost to the patient.

The options for physicians on cost minimization strategies to reduce cost to the patient seem dependent on the one hand on the state of medical technologies and the pace of innovation in the therapeutic area and on the other hand on the type of de-listing or reimbursement policy followed by the government (Hutton Andral, 2000, pp.84)

This behavior results in a client-centered type environment that is guided by overall cost and reimbursement rather than what’s cost effective for the client.

Occupational Therapy in France would seem to benefit from a general increase in reimbursement for services; unfortunately the sheer disproportion of occupational therapists per capita also takes effect on the state of O.T. The low density amounts of O.T.’s per inhabitants causes more stress on occupational therapists as well as more focus on the clinical end of the practice. According to the Association Nationale Française des Ergothérapeutes (ANFE 2008) [1] “The density of Occupational Therapists in 2008 reached 11 per 100,000 inhabitants. The comparison with other professional always reveals a gross disproportion, hardly acceptable to the needs of the French population” (p. 11). Even though there is a large disparity among therapist to client ratio some contributions to OT are in the workings.

According to (ANFE 2008) [2] the top two recipients of O.T. resources in France are: rehabilitation of the adult establishment (25%) and geriatrics (20%). As “Baby Boomers” continue to retire and to live longer lives the geriatric portion looks to grow exponentially. ANFE 2008 (HID 1998-1999) [2] “76.9% of women over 60 report having a disability whereas 72.6% of men report the same. With this in mind the ANFE has enacted the 2008-2012 National Alzheimer’s Plan. For this purpose (WFOT 2004) “O.T. action is mostly attended in home-adaptations and cognitive rehabilitation” (Para 2). ANFE also plans to work with the French Association of OT in Geriatry (AFEG) to focus on interaction between different medical professionals (ANFE 2008) [3]. To continue contributing to the field of occupational therapy worldwide ANFE has other goals, and according to WFOT (2004) they include: developing the publication of OT books on a national scale, designing a method to collect final dissertations for OT students, support research in OT, support creation of a new OT school, and creating “Ergoscope” a communications tool for O.T.’s for the 2008 / 2009 period (Para 4).

The development of an Alzheimer’s Plan is a very instrumental program / idea that could serve as a template for other countries. The other accomplishments France is striving for would help France catch up to the needs of its people as well as to other developed O.T. programs in other countries. These moves serve to improve O.T. in France and contribute more to the international file of O.T.

To deal with the demands of a growing population an influx of qualified O.T.’s are needed. According to COTEC (2009) there are 1,050 student O.T.’s in France’s eight programs of occupational therapy (p. 4). The occupational therapy schools are under the jurisdiction of the Ministry of Health and Education. The current length of the O.T. program in France is 3 years according to COTEC (2009, p. 4). To graduate as an Occupational Therapist in France schooling is necessary where upon completion the graduate will receive a diploma for occupational therapy. This differs from other countries such as the United States where Occupational Therapist qualifications are reserved for Masters level graduates. International educational contributions are not fully established from France due to a language barrier problem and lack of an O.T. database to share research findings. In response to information asked about journal information about education and other contributing factors from France the following was recorded: S. Mehanneche (personal communication, February 18, 2010) “I’m sorry. All papers and documents are in French. There are very few French speaking English in France. Therefore there is no translation.” Attempts are being made to expound on the education portion of France’s O.T. program. Ergothérapie, the French O.T. database is receiving more journals and is aiming to become accessible worldwide. Along with Ergothérapie reaching for new boundaries, the ANFE also looks to become more accessible; if the Alzheimer’s Plan of 2008-2012 is deemed a success it could potentially provide countless methods and information on therapy for Alzheimer’s patients. From the information that has been discovered France is not at the forefront in education, France has several instrumental steps to make to be in a league as the American or Australian occupational therapy programs.

Research in France is also scarce due to the language barrier and lack of available information. Through what info that is available the French are working in three key areas. According to European Cooperation in Occupational Therapy Research and Occupational Science (ECOTROS 2010) there are three therapists from France that are interested in doing research cooperation projects. These researchers are Marie Chantal Morel, Hadj Kalfat, and Eric Sorita. The research topics according to (ECOTROS 2010) are: Marie Chantal Morel- O.T. / O.S. Theory philosophy focusing in professional education (p.20), Hadj Kalfat- Assessment methods and outcome measures / mental health for geriatrics (p. 16), and Eric Sorita- Community based treatment / O.T. interventions and neurological disorders (p.28). These therapists / researchers are important to France because they look to push forward and gain new knowledge or develop new techniques that will help push France forward in the field of O.T. Research and developments like these are important because they help eradicate obsolete or ineffective modes of treatment. According to the Lancet (2007) a treatment for autistic children with psychiatric problems in France is causing huge problems (p.645). According to the Lancet (2007):

The therapy, called packing, involves wrapping a child tightly in wet sheets that have been placed in the refrigerator for up to an hour. When children are encased in this damp cocoon-with only their head left free-psychiatrically trained staff talk to them about their feelings. (Spinney, 2007, pp.645)

This treatment is commonplace in France for autism despite a 1996 French National Consultative Ethics Committee for Health and Life Sciences report on the treatment. This report states Lancet (2007) “There was no evidence to substantiate psychoanalytic models of autism, nor that therapies based on this model were effective” (p. 645)

Pushing the issue on doing research will help erase non-effective therapies like this from the practice. Without substantial evidence to prove that a technique is insufficient mal-informed therapists will continue to do treatment like this that could potentially do more harm than good. For this reason, France needs to allocate more resources towards the research and evaluation of new methods and techniques, until then France will continually not be able to contribute much to the O.T. field internationally for research.

It has been concluded that through the information available and what was gained from practice, education, and research from France that France is not as advanced as could be. Programs such as the Alzheimer’s Plan of 2008-2012 are a step in the right direction towards advancement of clinical practice and could be very instrumental in the practice after studies are done and results calculated. A hindrance is the lack of communication when viable resources are available (i.e. internet various journals / databases), leads the reader to believe that the O.T. program in France has not had a big push to publish their information on international basis. This lack of effort in sharing information can only impede things such as research. In the (ECOTROS 2010) co-operational research this is demonstrated by the meager three representatives for the whole country of France compared to 27 from the United Kingdom, 24 from Germany, and 14 for the Netherlands. With a more conscientious effort to put forth and share quality work, France could become one of the leaders in the field of occupational therapy, when it comes to the realm of practice, education, and research.

Procedure:


In this method a split-thickness skin graft is harvested from the donor site, either thigh or buttocks. It is then meshed either manually or in an Ampligreffe or any other suitable meshing apparatus.[40,41] Meshing of the graft causes an expansion in its size to 4 or 6 times its original one. The meshed graft is then applied on the dermabraded recipient skin and bandaged as in any other form of tissue grafting. The main advantage of this technique is that the graft can take care of a vitiligo lesion that is 4-6 times that of its original size. Additionally meshing allows the graft to be applied on areas over joints and other areas with difficult contours.

This technique is increasingly being practiced in India and is a simple, cost- effective procedure with good cosmetic results.

Principle


: In this technique of vitiligo, grafting the split-thickness or ultra-thin skin graft is cut or smashed into very small pieces and applied to the dermabraded recipient skin.[42,43]

The donor: recipient ration is approximately 1:10.

Procedure:


A split-thickness or ultra-thin skin graft is first taken from the donor area preferably thigh or buttocks. It is then smashed/cut into thin pieces.The cutting process is continued till the graft is converted into a uniform mesh or paste. This mesh is then mixed with either hyaluronic acid or antibiotic ointments and is then spread evenly over the dermabraded recipient area as in any other form of tissue grafting.[42] The recipient area is then covered with a collagen dressing and this dressing is removed after 7-8 days. The advantage of this method is that a relatively larger area can be covered by a small sized graft. The results are almost similar to those achieved with non-culture epidermal cell suspension (NCES) technique. Additionally, no expensive reagents or laboratory support is required as in NCES procedure. Some difficult to treat areas like the hairy skin, the joints and bony prominences can also be treated with this technique. The disadvantage is that it is difficult to spread the grafted tissue evenly on to the recipient area.

Figures 34.7 and 34.8 shows good results with smash graft on joints.

Three main cellular grafting techniques are described in the world literature. These are non-culture epidermal cell suspension technique, cultured melanocyte transplant and non-culture follicular suspension technique

Synonyms: non culture melanocyte transplant, non-culture melanocyte-keratinocyte cell transplant (NCCT), basal cell suspension technique.

Principle:


The different cellular components of a STSG are separated and a suspension is prepared out of these cellular components. The suspension contains epidermal keratinocytes and melanocytes’ this is applied on to a dermabraded recipient area. The donor: recipient ration is 1:10.

In this cellular grafting procedure a split-thickness skin graft is harvested from a suitable donor area and this is treated with 5 ml of Trypsin-EDTA solution for about 45-60 minutes in an incubator at 37°C. This step separates the cells of the epidermis from the underlying dermis. The next step is the neutralization of Trypsin which is achieved either by using 2 ml of 0.5% trypsin inhibitor solution or washing the graft with DMEM or any other suitable medium repeatedly. The treated graft is then taken in a petridish with the epidermal side downwards and the dermal cells are teased out of the graft with forceps. The overlying dermal tissue is discarded and the solution with the cellular component is centrifuged for about 10 minutes at the end of which the cells pellet are seen suspended at the bottom of the centrifuge tube and the epidermal pieces are floating at the top, which is discarded. The cell pellet is then mixed with a about 0.8 ml of Dulbecco’s Modified Eagle’s Medium (DMEM) medium (also called M2 melanocyte medium) and the suspension thus obtained is transferred to a 1 ml tuberculin syringe. After the recipient bed is created, the cell suspension is spread thinly and evenly with a spatula on to the dermabraded recipient skin after removing needle. The area is then dressed with collagen dressingto hold the transplanted cells and the dressings are removed after 1 week. As an alternative to the DMEM medium, patient’s own serum or hyaluronic acid can be used as it improves the viscosity of the cellular suspension.[53]

This technique requires expensive laboratory equipment and is usually practiced only at research centres.

Principle:


It replenishes melanocytes selectively by creating a melanocyte rich suspension. The donor: recipient ration can be as high as 1:100

Procedure



:

The epidermis undergoes trypsinization and the melanocytes and keratinocytes are dissociated. The melanocytes are further seeded in a melanocyte medium containing growth factors and cultured over 15 to 30 days. The cultured melanocytes (free suspension or epidermal sheets) are then transplanted on to dermabraded recipient skin.

This is a novel cellular graft technique by using the hair follicle outer root sheath cell for transplant. Cosmetic results obtained with this procedure are almost similar to those seen with NCES technique.

Principle:


This is another cellular grafting technique wherein the melanocytes present in the hair follicles are utilized in repigmenting resistant vitiligo. The outer root sheath of the hair follicle is a rich source of inactive melanocyte. Theseinactive melanocytes function as stem cells and hence can be harvested and used in vitiligo.

Procedure:


The procedure is almost similar to NCES technique but here extracted hair follicles are used instead of a split-thickness skin graft. The hair follicles can be extracted by the follicular unit extraction (FUE) method. The hair follicle is decontaminated by washing with antibiotics. Enzymatic dissociation of ORS is done by addition of trypsin and incubated at 37 C.Mechanical disruption of the ORS is done by vortexing and the ORS cells are separated from the hair shaft by a cell strainer. The dissociated cells are examined microscopically for viability and the cell suspension can be transplanted onto the prepared recipient site. This technique is in a nascent stage, however it has shown good repigmentation comparable to NCES .

The surgical techniques discussed above have various advantages and disadvantages. (Table 34.3) [50, 58]

Table 34.3: Advantages and Disadvantages of grafting techniques

In addition to the grafting techniques, various other methods also have been used including tattooing, excision with primary closure and therapeutic wounding.

Tattooing:


In tattooing, artificial pigments are introduced into the depigmented lesions for permanent camouflage. This can be done with a hand held pin vise or an electrical device.

Excision with primary closure:


The depigmented areas are removed and the wound is sutured; this technique is useful for small vitiligo lesions.

Therapeutic wounding:


Wounding of the lesions stimulate the melanocytes from the periphery of the lesion as well as from the hair follicles which migrates and re-pigments the lesion. Various modalities which are used for therapeutic wounding include dermabrasion, laser ablation, cryosurgery, needling, and local application of phenol or trichloroacetic acid. [59]

Besides the technical aspects of various procedures (detailed in the earlier section), the other important practical aspects include:

• Choice of technique (Which procedure should be performed, where and why?)
• General pre and post-operative considerations,
• Role of phototherapy,
• Complications and their management.

The choice of technique depends on the dermatosurgeons’ skills, experience and the availability of facilities in the dermatosurgery set up. However, the factors which are considered while planning a vitiligo surgery also determine the choice of surgical technique employed. Based on these factors, an algorithmic approach to choosing an appropriate surgical technique in stable vitiligo can be evolved. (Illustration 34.3

In cases of pediatric segmental vitiligo, NCES is suitable. It can be followed by phototherapy for faster results. Tissue grafting techniques are usually not recommended due to constraints of immobility in this population subset. In adult, population both tissue grafting and cellular techniques can be employed based on the site and total area of depigmentation.

Segmental and focal vitiligo are most amenable to surgical treatment. Amongst the non- segmental type, lesions located on the glabrous skin are suitable for surgical intervention. The acromucosal types are usually not responsive.

The location of the lesion plays an important role in determining the choice of grafting technique. (Table 34.4)

Table 34.4: Anatomical location and choice of grafting technique

Small areas (1-4 cm) – All techniques work well in vitiligo involving small areas (1-4 cm) and technique should be chosen based on anatomical location and cost to the patient. In cases of large areas, NCES, smash or UTSG is preferred.

The general pre and post-operative aspects have been outlined in Box 34.6. Specific pre and post-operative procedural aspects have been dealt with in the description of procedures.

Box 34.6: General pre and post-operative aspects

The role of phototherapy (narrow band UVB) post vitiligo surgery has been well demonstrated with various tissue grafting and cellular techniques. Phototherapy can be started within 1-2 weeks following surgery. Concurrent use of narrow band UVB exerts a stimulatory and proliferative effect on the grafted melanocytes; thus post- surgery phototherapy enhances and accelerates the repigmentation.

In cases of UVB therapy which is initiated after split thickness skin grafting, repigmentation can occur within two weeks and a better colour match at the recipient site is seen. Repigmentation with cellular techniques has been observed within 3-4 weeks after surgery and can progress till 6 months and this can be enhanced with phototherapy. Use of excimer laser has also shown good results post punch grafting.

From these assessments, 3 problems Meera faced, in order of significance was developed:

This essay will explore the pathophysiology, management and psychosocial aspects of jaundice in the paediatric patient, specifically in neonatal babies. Information has been gathered through the use of history taking, examination, and analysing the patient’s medical records to form a case study in which the topics aforementioned will be considered in relation to the case study.

Section A – Case History

TH is a 2 day old male Caucasian baby who was admitted to the neonatal intensive care unit (NICU) presenting with neonatal jaundice. TH’s mother had a premature rupture of her membranes (PROM) consequently causing TH to be born prematurely at 33 weeks, and weighing in at 1779g. This was her first pregnancy (primigravida). He was born in good condition and was instantly crying on delivery. At birth, TH had a heart rate of 150bpm, a respiratory rate of 42 breaths per minute, temperature at 37.2°C, and SATs of 100% in air. He was slightly grunting so facial oxygen was given, however shortly on admission to AMU, he was self ventilating. The mother was fine, but was moved to the post natal ward to be put under surveillance in case she developed chorioamnionitis or sepsis. She would come down to the neonatal unit to bond with TH once to twice a day for a couple of hours.

TH was then nursed in an incubator where intravenous fluids were commenced (10% dextrose through a cannula running at 4.4ml/hour). He was given cefotaxime in the morning to prevent him from getting an infection, and then again at midnight. He was also routinely given intramuscular konakion (vitamin K) in his thigh. The mother wanted to breastfeed, and although skin to skin contact was made, feeding could not be established. He was therefore fed 5ml of SMA prem 1 2×12 hourly via a nasogastric tube (NG tube). A small amount of urine and meconium was passed, and there was no vomiting or colic.

On examination TH had a head circumference of 30.1cm, with his fontanelles being normal when palpated. His eyes were fine, palate intact and his respiratory system was clear bilaterally. Heart sounds that were heard were normal, femoral pulses were felt, the abdomen was soft when palpated and the spine was not deformed. His anus was patent, genitalia were normal and there was no deformity of any of his limbs with normal tone. When tested, the moro reflex was positive and normal and he demonstrated a good grasping reflex.

The next day (24 hours after birth), TH was now tolerating 2×12 hourly feeds of expressed breast milk (EBM) from his mother and SMA prem 1 at 90ml/kg/day via an NG tube. The cefotaxime was continued.

48 hours after TH’s birth, he was now on 120ml/kg/day of 2×12 hourly feeds of EBM and SMA prem 1via his NG tube. He had passed urine and opened his bowels twice that day. A yellowing of TH’s skin was observed so his serum bilirubin levels (SBR) were checked; a result of 234µmol/l was obtained, which is above the normal treatment line. As it was confirmed that TH was jaundiced he was immediately transferred to the neonatal special care unit, where double phototherapy was commenced. That same day in the evening, TH’s SBR had been reduced to 202µmol/L after receiving phototherapy.

Section B – Pathophysiology

Introduction

Jaundice, or icterus, is characterised by the yellowing discolouration of the skin, the deep tissues and the sclera of the eyes. 1 It is usually clinically detected when the serum bilirubin levels are greater than 40-50µmol/L (when the jaundice is observable). 2 3 In TH’s case, he is most likely suffering from neonatal physiological jaundice which is relatively common, particularly in premature infants, however a differential diagnosis of pathological and physiological causes must be made so that serious concerns may be noted and ruled out. 3 Although the jaundice does fade in time as the liver of the premature infant matures, it can be dangerous if the SBR are at high levels (hyperbilirubinaemia). 2 3

Hyperbilirubinaemia

Hyperbilirubinaemia is when the levels of bilirubin in the blood are higher than the normal values (TH’s being 234µol/L); bilirubin is a product of the lysis of red blood cells in the body. 5 Breakdown from red blood cells produces unconjugated (or indirect) bilirubin, which is insoluble in water, so it must be metabolised in the liver so that it becomes conjugated and can pass through the gut and be excreted in the stool and urine. 2 5 In neonates, high levels of unconjugated bilirubin can cross the blood-brain barrier; this is potentially harmful to the neural tissue of the brain and may result in causing bilirubin encephalopathy or kernicterus. 2 5 TH’s hyperbilirubinaemia and jaundice only become present 2 days post partum. Although not serious at the moment, one must come up with a differential diagnosis for TH to make sure that potential pathological, life threatening causes such as kernicterus, are dealt with immediately.

Kernicterus

The term kernicterus literally means, “jaundice of the nucleus”. 3 This is due to deposition of high levels of unconjugated bilirubin, for example in maternal-foetal Rhesus disease. 3 Acute features of the latter would include abnormal muscle tone and posture, apnoea and convulsions, none of which were present in TH on examination. 5 In serious cases which would be fatal, the bilirubin would be deposited on the basal ganglia, brainstem and thalamus nuclei, which would show up as bright yellow in colour. 3

Pre-Hepatic Causes

Haemolysis of an infant’s red blood cells is one of the most common causes of “pre-hepatic jaundice”. 2 Pathological haemolytic jaundice can be subcategorised into two types: intrinsic, where the defect is in the cell itself, or extrinsic where the problem is outside of the cell. 6 An example of intrinsic haemolytic jaundice could be spherocytosis, where the red blood cells are sphere shaped rather than biconcave and can burst easily due to their less flexible configuration. 3 An extrinsic example could be Rhesus disease; this is where the antibodies from a mother can destroy her baby’s blood cells due to an incompatibility of blood types between the two. 7 When a pregnant woman who is Rh negative whose husband is Rh positive, carries a foetus who is also Rh positive, the blood of the foetus would cross over to the placenta and cause anti-Rh agglutinins to be created in the mother’s blood. 8 These would then pass back to the foetus and eliminate the Rh positive blood of the foetus, consequently causing haemolytic jaundice and anaemia. 8 It would usually take more than one pregnancy for the mother’s blood to build up enough anti-Rh antibodies to damage the foetus, so this would be unlikely in TH as this was his mother’s first pregnancy. 8 In TH, the most likely pre-hepatic cause would be physiological. This would probably be due to the immaturity of the baby’s liver not processing the bilirubin when foetal red blood cells are haemolysing to make way for the adult red blood cells. 1 As the bilirubin would be unconjugated, it cannot be excreted and so the baby would become jaundiced.

Hepatic Causes

Although some causes of hepatic jaundice are idiopathic, it is most commonly seen due to an acute infection, such as hepatitis B, or the immaturity of the baby’s liver being unable to cope with the conjugation of the bilirubin. 1 3 In Th’s case, it seems to be due to the immaturity of his hepatocytes failing to take up or conjugate the bilirubin. A possible reason for the lack of conjugation could be due to decreased levels of UDPG transferase activity. 2 This enzyme is responsible for the conjugation of bilirubin to glucaronic acid; reduced hepatic glucurodination results in an increased proportion of bilirubin mono-glucuronide in bile.2 As most of the bilirubin would predominantly be unconjugated, there would be minimal excretion of bilirubin and jaundice would be seen.

Post Hepatic Causes

Jaundice can also occur in infants due to structural congenital abnormalities. 3 Malformations of the biliary system and congenital obstructions, such as biliary atresia which is characterised by the failure of bile duct development during embryogenesis, can cause obstructive post hepatic jaundice with conjugated hyperbilirubinaemia. 3 5 There is also a genetic component to structural abnormalities such as Alagilles’s syndrome (or biliary hyperplasia), which is an autosomal dominant where the lack of bile ducts are also accompanied by mental retardation and skeletal abnormalities. 3 Problems like congenital obstruction would need immediate specialist investigation and early surgical treatment. 5 Infants with an obstruction or congenital abnormality would usually become jaundiced within the first 24 hours, so it is unlikely that TH would have this.

Physiological Causes of Jaundice

Neonatal physiological jaundice is when excess unconjugated bilirubin, which is not water soluble, is deposited in the skin instead of being delivered to the liver where it can be processed and converted into water soluble, conjugated bilirubin which can be excreted out of the body into the urine or faeces. 9 Physiological jaundice usually presents itself 48-72 hours after birth and disappears within a week; if it is physiological it is never present before 24 hours. 9 10 This is perfectly normal and affects approximately 50% of term babies and 80% of premature babies, just like TH who presented with jaundice after 48 hours. 10 The factors that are associated with physiological jaundice are multifactorial: firstly the levels of haemoglobin may be higher than required when foetal haemoglobin is being replaced with adult haemoglobin. Red blood cells may also have a shorter life span, meaning faster lysis and a quicker build up of unconjugated bilirubin. It could also be due to hepatic immaturity, especially in pre-term babies like TH. 10 This could be due to reduced glucuronyl transferase activity (which catalyses the deconjugation of bilirubin), reduced active uptake of unconjugated bilirubin, a reduced intracellular transport system, or a reduced active secretion of conjugated bilirubin into the bile ducts. 9 10

It is argued that breastfeeding is the best choice in terms of infant nutrition; 11 however breastfeed babies’ SBR concentrations tend to be higher than formula fed infants within the first week of life. 5 12 Breast milk is plentiful in the enzyme β-glucoronidase, whereas standard infant formula feeds have negligible amounts of β-glucoronidase; consequently infants who are formula fed are less likely to be jaundiced compared to breast fed infants. 13-15 β-glucoronidase plays a big role in neonatal jaundice because it increases the effect of the enterohepatic circulation of bilirubin, possibly due to sluggish gut movements, 5 by deconjugating intestinal bilirubin conjugates, producing unconjugated bilirubin that can be absorbed better by the baby’s intestines. 13 This is normal in utero as the placenta can facilitate the clearance of bilirubin, however after birth the enterohepatic circulation of bilirubin delays clearance, thus causing the baby to look jaundiced. 16 Casein hydrosylate in some formula feeds can inhibit β-glucoronidase, therefore infants consuming these formulas are less likely to develop jaundice compared to infants on routine formula feeds. 14 15 A study showed that the main inhibitor to β-glucoronidase in casein hydrosylate feeds is L-aspartic acid. 17 Furthermore, another study showed that if prophylaxis (β-glucoronidase inhibitors) was given to newborn babies a week after birth, there was a significant reduction in transcutaneous bilirubin levels compared to control subjects. 18 The fact that TH was started on expressed breast milk (EBM) 24 hours after birth, and then presented with jaundice 24 hours after his first feed of EBM, makes it highly probable that his jaundice stems from physiological origins. This is also coupled with the fact that he did not present with jaundice before 24 hours, strongly pointing towards physiological jaundice.

Section C – Treatment and Management

As neonatal jaundice has the potential to cause both acute and chronic impairments in brain function due to kernicterus, it is this that promotes therapeutic intervention. 19 The most effective and commonly used form of treatment for neonatal jaundice is phototherapy. 19 20 This is done by placing the baby under a lamp in which light is emitted from it in the blue spectrum.5 Phototherapy is used to convert the structure of the bilirubin molecule into an isomer that is water soluble so that it can be excreted in the urine. 20 The stereoisomers that are left are more polar than the predominant IXα (4Z,15Z) isomer which needs to be conjugated in the liver to be excreted. 20 The isomer that is most rapidly formed is one in which one of the double bonds in the bilirubin molecule has undergone a cis-trans isomerisation from a Z (zusammen) configuration to an E (entgegen) configuration. 20 This therefore converts the normal 4Z,15Z isomeric form of bilirubin to the water soluble 4E,15E isomer, which occurs in femtoseconds (10-15 of a second) due to the photochemical reaction. 20 TH underwent double phototherapy. This is slightly different than to conventional single phototherapy (described above). Not only is TH exposed to lamps emitting blue light, he is also wrapped in a fibre optic Biliblanket. 21 This involves a light generator, which produces blue or white light of varying intensities and is connected to a light-permeable fabric via a fibre optic cable. The fabric is then placed close to, or is directly in contact with the baby’s skin so that the treatment can occur. 21

In terms of evidence based support showing that conventional phototherapy is effective in the treatment of jaundice, a randomised controlled trial (RCT) found that, “…in the 2000-2499g birth weight group (141 infants, serum bilirubin ≥ 171 µmol/L, average 212 µmol/L), phototherapy significantly reduced the proportion of infants with higher maximal serum bilirubin levels compared with no treatment (serum bilirubin ≥ 257 µmol/L: 18.6% with phototherapy v 42.3% with no treatment; P = 0.002)”. 22 Whether TH’s double phototherapy was necessary is another question as another RCT, “found no significant difference between double conventional and single conventional phototherapy”. 23

Although the risks of serious side effects are minimal whilst having phototherapy, babies usually can present with the following: frequent green loose bowels, skin rashes, dehydration if feeding is not regular (approximately every 2 hours, which TH was) and temperature fluctuations (hypothermia and hyperthermia). 24

Obviously double phototherapy will be more costly compared to single phototherapy due to the use of the fibre optic light cables. However, a study has shown that light intensity and the area of light-exposed skin can be increased due to white curtains being hung around the incubator during single phototherpay, via reflection. 20 These low costing curtains safely increased the efficacy of phototherapy, significantly more than single phototherapy and rivalling double phototherapy, and may prove to be useful to neonatal units when budgets are limited. 20

Section D – Psychological and Social Aspects

Epidemiology

A reason why newborn infants are subject to examination, testing and treatment could arguably be due to jaundice. Estimates of the incidence to jaundice are varied due to ethnicity and geography. 25 A study in Britain examined the trends in moderate neonatal hyperbilirubinaemia in Wirral hospital between 1991 and 2001. 26 They found that the incidence of moderate jaundice increased from 2.4/1000 births in 1991 to 5.5/1000 births in 2001 (p<0.0001). 26 It was also found that, “…readmissions for jaundice increased from seven in the first six years of study to 55 in the second five years (p < 0.0001)” showing that neonatal jaundice is on the rise, or that it is now more easily detected in infants. 26

Screening and Disease Prevention

The prevention of kernicterus requires a good screening test for hyperbilirubinaemia. Screening for this can include physical examination, or measuring the amount of bilirubin in the skin or in the serum. 27 A study in Denmark showed that one third of infants with extreme hyperbilirubinaemia were non-Caucasian. 26 Jaundice is not as easily recognised in infants with darker skin pigmentation by nurses or health care workers, so it is important that babies do not leave the hospital undetected to prevent any neurological damage from occurring. 26 Various transcutaneous instruments can be used to measure elevations in bilirubin levels if it is difficult to notice jaundice on examination. An example is the Minolta JM-103, which is easiest to use but has a tendency to be inaccurate when measuring in dark skinned infants as it overestimates bilirubin levels. 27

Psychosocial Aspects

Although common and usually harmless, jaundice in infants can cause stress for the whole family, particularly the mother in TH’s case. As bonding between TH and his mother was limited to approximately two hours a day, and the fact that breastfeeding could not be established after birth may cause TH’s mother some distress, especially with TH being her first child.

It was noted that children born between October and March (which TH was) were at a higher risk of infantile autism. 28 However this is contradicted as it also states that babies conceived by a primiparous woman were less likely to develop infantile autism. 28

Kernicterus must be avoided at all costs as studies have shown that the damage to the neural tissue can lead to an increased risk of disorders of psychological development. Although jaundice is relatively common in infants and the methods of treatment have a high efficacy and efficiency, kernicterus although rare, still presents itself in undetected children. A good screening programme and immediate treatment is a straightforward way to tackle neonatal jaundice and to make sure that a relatively harmless condition does not manifest itself in the baby.