AP401+Paediatrics

=AP401 Paediatrics =

Resources

 * **Subject** || **Lecture Notes** || **mp3** ||
 * Infant Feeding & Special Dietary Needs || [|2008] [|2007] || [|2008] ||
 * Immunisation || [|2008] [|2007] || [|2008a] [|2008b] ||
 * Paediatric Analgesia || [|2008] [|2007] || [|2008] ||
 * SMART - Handout || [|2007] ||  ||
 * SMART - Slides || [|2007] ||  ||
 * Cystic Fibrosis || [|2008] [|2007] || [|2008] ||

Outline
Date: Friday, October 19 Handouts: [|Infant Feeding & Special Dietary Needs] Audio: Infant Feeding 1 & Infant Feeding 2 Note: The lecture audio for this presentation is posted in mp3 format and provided for the purpose of revision support only
 * Infant Feeding & Special Dietary Needs: Jean-Pierre Calabretto**

• Understand the basis for artificial alternatives to breast feeding • Identify practical issues related to artificial feeding • Describe the basic concept of enteral and parenteral nutrition**
 * • Understand the principles of infant feeding and the benefits of breast feeding

Date: Monday, October 22 Handouts: [|Immunisation] Audio: Immunisation Note: The lecture audio for this presentation is posted in mp3 format and provided for the purpose of revision support only • Understand different definitions used in immunisation • Identify the aims and importance of immunisation programmes • Identify and demonstrate important aspects of the Cold Chain • Describe pharmacy practice issues related to immunisation**
 * Immunisation: Jean-Pierre Calabretto**
 * • Understand immunisation as a public health initiative

Nine routine immunisation encounters in life - 7 in first 4 years of life (as children do not have strong immune systems) - 9 for Aboriginal & Torres Strait Islander children (live in high-risk areas) NIPSA changes almost yearly Boosters drop in number after 4 years of age - leading to lower immunisation rates due to the large gap (91% at 12-15 months, 83% at 72-75 months) - use an immunisation calculator/calendar - Australian Childhood Immunisation Register developed in response to decline in rates (records details of vaccinations given)
 * National Immunisation Program Schedule (NIPS)**

   **Definitions** //Control// -  When there is reduced incidence, prevalence, morbidity or mortality of the disease to a locally acceptable level as a result of deliberate efforts. Continued intervention is required. //Elimination // - Permanent reduction to zero of incidence of infection from a specific disease in a defined geographic region. Continued intervention required. //Eradication// - Permanent reduction to zero of worldwide incidence of infection from a specific disease. No further intervention required. //Extinction // - Irreversible removal of an organism from the environment. //Vaccination // - Injection of a killed/altered microbe in order to stimulate the immune system against the microbe, thereby preventing disease. //Immunisation // - Immunisation means both receiving a vaccine and becoming immune to a disease, as a result of being vaccinated against that disease

quantity - coverage - % of target population fully-immunised for specific disease quality - positive outcomes re quality measures (cold chain, education, vaccine safety surveillance)
 * Immunisation Program Measurements**

//the system of transporting and storing vaccines within the safe temperature range of 2-8 degrees from the place of manufacture to the point of administration// "strive for 5!" - gives a 3 degree leeway for protection from temperature fluctuations - ensures people receive an effective product - professional responsibility and liability (prevent situations where child may require revaccination due to ineffective vaccine) - vaccines are expensive
 * Cold Chain**

some vaccines are unstable at room temperature, some damaged by freezing and other can be damaged by light exposure. - injecting a denatured vaccine (decreased or no antibody response) - injecting a frozen vaccine (sterile abscess at injection site, decreased or no antibody response)

purpose-built vaccine fridges - reduced risk of freezing (unlike domestic fridges) protect the power supply to the fridge - tape in the plug to prevent accidental disconnection - do not place in direct sunlight fridge maintenance - regularly check door seals, in-house audits, clean regularly, do not store food or drink - regular servicing by technician
 * Vaccine Storage**

//monitors/indicators// temperature monitor - min/max thermometer - recommend that fridge temperatures be documented twice daily - monitor mark (heat indicator) monitors exposure to heat over time (14 days to full scale colour change at 12 degrees) freeze indicator - activates at 0 degrees - background paper stains blue if activated

- check heat/freeze monitors if included - refrigerate vaccines immediately - do not remove vaccine packaging (acts as an extra barrier to cold)
 * Unpacking vaccines**

- remove ice bricks from freezer to sweat/condition for at least 60 mins prior to packing (reduce freeze risk) - place freeze, mix/max thermometer - ensure a barrier between ice bricks and vaccines (shredded paper, polystyrene chips) - secure the lid - check temperature before leaving the pharmacy, when vaccines arrive at destination and before vaccines are administered
 * Packing vaccines for transport**

- advise client to give the vaccine to the nurse as soon as arrive at practice - OR collect vaccine from pharmacist and store in GP's monitored fridge until required //vaccines should never be taken home to unmonitored domestic fridge (strict guidelines for use in these fridges)//
 * Dispensing vaccines**

-** order what you need (eg quantity used in last 2 week period minus the amount left over from last period, plus a 10% buffer) - do not stockpile - one trained person responsible for cold chain management (with backup staff during leave/holiday) - do not discard funded vaccines unless directed by SA Immunisation CU
 * Wastage reduction

Immunisation side effects - most-side effects are short lived and do not lead to any long-term problems (mild fever, unsettled pain/soreness/redness at the injection site, mild 'flu-like' symptoms - benefits of immunisation (prevention of disease) greatly outweigh the side effects experienced
 * Consumer education**

Medication - concurrent medication is not usually an issue - corticosteroids and immunosuppressive agents can interfere with the development of active immunity - oral cholera and typhoid vaccines may be rendered ineffective by the simultaneous use of antibiotics and anti-malarial medicines - paracetamol recommended for mild pain and fever post-vaccination (or as prophylaxis) <span style="color: rgb(227, 38, 38)"><span style="font-size: 12pt; font-family: 'Times New Roman','serif'">(The routine use of paracetamol at the time of immunisation is no longer recommended, due to the use of better vaccines with fewer side effects)

Date: Monday, October 22 Handouts: [|Paediatric Analgesia] Audio: Paediatric Analgesia Note: The lecture audio for this presentation is posted in mp3 format and provided for the purpose of revision support only • Discuss the various advantages and disadvantages of the routes of administration for paediatric analgesia**
 * Paediatric Analgesia: Sean Turner**
 * • Understand the therapeutic options available for paediatric analgesia

Non-verbal clues - blood pressure, heart rate, oxygen saturation, respiratory rate, palmer sweating - facial expression, posture, sounds Wong-Baker faces - child may end up choosing a happy face when in pain Oucher scale
 * Pain assessment in children**

We want to look for improvements in pain score

-** dummies - distraction (cartoons) - relaxation - hot/cold packs - massage - TENS (transcutaneous electrical nerve stimulation) - guided imagery (hypnosis, 'imagine being somewhere else')
 * Non-pharmacological treatment

//first line// paracetamol, NSAIDs, paracetamol/codeine, tramadol (increasing use), narcotics //adjuncts// ketamine (local anaesthetic), clonidine (analgesic and sedative), diazepam, local anaesthetics (creams, epidurals), gabapentin (neuropathic pain, spinal surgery, burns, nerve damage)
 * Options - drug**

15-20mg/kg/dose oral (or rectal - need to increase dose) 6 hourly (max 90mg/kg/day for 2-3 days max, then scaling down) __caution__ in liver damage (bike accidents etc) obesity (take height into consideration) oncology?
 * Paracetamol**

be aware of different preparations with different concentrations, and other preparations containing paracetamol

ibuprofen (also as suspension), diclofenac (tablets and suppositories), naproxen, piroxicam __safety__ - asthma - renal (make sure child is hydrated) - bleeding - bone growth safe and effective when used at appropriate doses in appropriate patients
 * NSAIDs**

Painstop Daytime and Panadeine Forte (Panadeine not recommended) Painstop Nighttime contains promethazine (sedation) which restricts the amount of paracetamol that can be used
 * Paracetamol / Codeine**

mixed action (opioid, serotonin, noradrenaline) //role// - decreased risk of respiratory depression and constipation - not recommended but is used in children __adverse effects__ - nausea, vomiting, constipation, dizziness - genetic polymorphism (CYP2D6) - care with 100mg/mL drops (1mg/kg dose -> if 20kg, 0.2mL not 2mL)
 * Tramadol**

- Oxycodone / Oxycontin (SR) - Morphine / MS Contin (SR) - Codeine (use is decreasing, better off using oxycodone as an opioid) - Methadone (burns patients) - others - Hydromorphone?
 * Oral narcotics**

effective in neonatal procedures (prior to injection, heel stick sampling) due to release of endorphins when tasted (does not work if given parenterally) - 2mL of 12-50% sucrose 2 mins prior to procedure - same effect from breastmilk?
 * Sucrose**

- diazepam (muscle spasm) - oxybutynin (anticholinergic, reduce bladder spasm) - ketamine - clonidine
 * Other oral adjuncts**

-** morphine (usually start with this) - fentanyl - not pethidine (accumulation of metabolites) //route// IV - SC is rarely used, IM is painful with low absorption //modality// - bolus injection - infusion //patient/nurse controlled analgesia// - 'lock out' time (constant button pressing may be due to lack of comprehension or lack of pain relief) - with or without background - monitor 'tries/good' //adverse effects// - nausea/vomiting (stops when morphine stops) - pruritis (facial) others - respiratory depression - constipation - urinary retention - sedation - vasodilatation
 * Parenteral narcotics

-** anaesthetic with analgesic properties (good for burns or if narcotics insufficient, but can be used with morphine) //adverse effects// - sedation - excessive secretions - nausea/vomiting - hallucinations/nightmares
 * Ketamine

-** EMLA (pirolicaine, lignocaine) - amethocaine gel (half hour before procedure/injection) - adrenaline/cocaine solution (tetracaine/adrenaline/cocaine) - Laceraine (adrenaline/lignocaine/amethocaine) - lignocaine gel
 * Topical

//buccal// - fentanyl lozenges (Actiq)

//intranasal// - fentanyl spray (in ambulances, ED, dressing changes)

//inhaled// - Entonox (50:50 nitrous oxide/oxygen) - Penthrane (methoxyflurane)

//intravesical// - oxybutynin - pethidine

//regional anaesthesia -// epidural/caudal - regional nerve blocks local anaesthetic - lignocaine, bupivacaine, ropivacaine adjuncts - clonidine - fentanyl

- motor block - sedation - nausea/vomiting others - infection - seizures - arrhythmias
 * Complications**

-** identify source of pain (anti-spasmodic vs analgesic) - anticipate predictable painful procedures (give sufficient analgesic in advance) - use multi-modal therapy - use appropriate doses - administer regularly (reduce relapse of pain) - recognise side effects and treat - administer for adequate duration - consider needs post-discharge (otherwise they'll return to hospital)
 * Summary

Date: Monday, October 22 Handouts: [|SMART - Handout] & [|Cystic Fibrosis] Slides: [|SMART - Slides] Audio: SMART & Cystic Fibrosis Note: The lecture audio for this presentation is posted in mp3 format and provided for the purpose of revision support only At the conclusion of this lecture series students should be able to: //WCH approach for children under 2 years of age:// - normal oral hydration (safe and beneficial for all ages) - saline nasal drops (suitable for all ages, used before feeds in young babies) - paracetamol (suitable for infants older than 1 month if irritable) - ibuprofen (>3 months) - demulcents (soothing syrups - safe/comforting - honey/lemon)
 * SMART & Cystic Fibrosis: Kingsley Coulthard**
 * • Understand the change in management of coughs and colds in children**

//nasal decongestants?// CNS toxicity in those under 2 years - <2 years: normal saline drops only - >2 years: Otrivin 0.05% ('junior') - >12 years: Otrivin 0.1%

//not recommended:// - nasal decongestants - combination cough and cold preps - antihistamines - steam inhalation - aspirin - chest rubs (not directly on skin) - zinc, echinacea
 * Common cold**

//acute cough is self-limiting// - no efffective meds for acute cough in paediatric patients any persistent cough requires medical review - foreign bodies - infection (pertussis, mycoplasma) - lung disease - cystic fibrosis - congenital abnormalities - psychogenic - smoking medications may be taken under medical supervision (serious side effects and accidental poisoning has occurred)
 * Cough**

- most common life shortening genetic disorder in Caucasians - multi-system disorder (affects exocrine glands in respiratory, GIT, hepatobiliary and reproductive systems) - lung disease - 90% disease morbidity and mortality
 * • Discuss the impact of cystic fibrosis on the individual and the community**

//complications// lung disease - chronic cough, large volume of thick sputum - shortness of breath - mucus plugs - chronic infection - inflammation -> bronchiectasis

//diagnosis of CF// - neonatal screening - sweat test - genotyping - clinical suspicion (meconium ileus in neonatal period - dalayed in CF, failure to thrive, bulky pale stools, recurrent chest infections)
 * • Understand the major negative prognostic factors for people with cystic fibrosis**

//prognosis with CF// - 90% mortality before 10 years in 1960s - median age survival now high 30s - pulmonary function major correlation with survival - early, aggressive therapy improves incomes - increased survival -> new challenges (liver disease, CF related diabetes, contraception, pregnancy)

//Treatment of PA - eradication// <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">first PA isolate – usually non-mucoid <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">mean age 6 years
 * • Understand the rationale (only, detailed regimens not required) for antimicrobial therapy in cystic fibrosis related lung infection**

<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">± IV therapy - 2/52 tobramycin PLUS one of <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">ticarcillin-clavulanate (Timentin), <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">ceftazidime, <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">meropenem, <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">aztreonam

<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">oral ciprofloxacin up to 3 months <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%"> - <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">caution sunlight and milk

<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">inhaled colistimethate / tobramycin 3 months <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">70-80% success <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Time to regrowth - mean 1 year

//Treatment of PA colonisation// <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">not eradicate once colonised <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">usually mucoid PA (predictor of poor survival) <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">impacts on morbidity and mortality

<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">chronic inhaled colistimethate or tobramycin <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">acute exacerbations <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">2 weeks IV antibiotics <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Tobramycin PLUS one of <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">ticarcillin-clavulanate, ceftazidime, <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">meropenem, aztreonam, ciprofloxacin <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">repeat courses <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%"> - <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">tobramycin toxicity <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'"> TDM <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">renal function <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Audiograms <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%"> - <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">resistance

<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">other pathogens <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> //<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Burkholderia Cepacia //<span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> //<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Stenotrophomonas maltophilia //<span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'"> //<span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">MAC //<span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%"> - //MRSA//

//DNase (Pulmozyme)// <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Human recombinant deoxyribonuclease <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Breaks down extracellular DNA in mucus 2° to <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">chronic inflammation and tissue destruction <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">↓ <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">sputum viscosity - best taken at night <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">easier expectoration of sputum <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">↓ <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">chest congestion <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">↓ <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">dyspnoea <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">$1000 / month need to prove FEV1 increased by 10% for eligibility
 * Other treatments**

//Long-term inhaled hypertonic saline (6%)// - decreased acute exacerbations - 4-8mL bd inhaled (usually during physiotherapy) - pre-dose with salbutamol (as it can cause bronchoconstriction and irritation) - can start with 3% if needed

//Anti-inflammatory agents// Oral corticosteroids (often have asthma, so already on this) NSAIDs

//Macrolides// Azithromycin - anti-staph - interfere with neutrophil function - inhibits alginate production - reduces adherence of PA to epithelial cells

<span style="font-size: 7.5pt; color: rgb(0, 0, 124); font-family: 'Arial','sans-serif'">- <span style="font-size: 7.5pt; color: rgb(0, 0, 124); font-family: Wingdings-Regular"> ↑ <span style="font-size: 10pt; color: black; font-family: 'ArialMT','sans-serif'">QOL <span style="font-size: 7.5pt; color: rgb(0, 0, 124); font-family: 'Arial','sans-serif'">- <span style="font-size: 7.5pt; color: rgb(0, 0, 124); font-family: Wingdings-Regular"> <span style="font-size: 10pt; color: black; font-family: 'ArialMT','sans-serif'">↓ no. respiratory exacerbations <span style="font-size: 7.5pt; color: rgb(0, 0, 124); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 7.5pt; color: rgb(0, 0, 124); line-height: 115%; font-family: Wingdings-Regular"> <span style="font-size: 10pt; color: black; line-height: 115%; font-family: 'ArialMT','sans-serif'">↓ rate of decline in lung function

Dose <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: Wingdings-Regular"> - <span style="font-size: 10pt; line-height: 115%; font-family: 'ArialMT','sans-serif'">>40kg: 500mg Monday, Wednesday and Friday <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%">- <span style="font-size: 10pt; color: rgb(0, 0, 124); line-height: 115%; font-family: Wingdings-Regular"> <span style="font-size: 10pt; line-height: 115%; font-family: 'ArialMT','sans-serif'"><40kg: 250mg Monday, Wednesday and Friday

//Pancreatic insufficiency in CF// <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Good nutrition <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">→ <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">good lung function <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Pancreatic insufficiency 90% <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Lack lipase, amylase, protease <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Creon®, Cotazyme® <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Titrate dose to bowel actions <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">High calorie diet <span style="font-size: 10pt; color: rgb(155, 155, 205); line-height: 115%; font-family: 'Arial','sans-serif'">- <span style="font-size: 10pt; line-height: 115%; font-family: 'Arial','sans-serif'">Fat soluble vitamins – A D E K

__Liver disease__ decreased water and sodium movement into bile - bile and mucous dehydrated - bile stasis -> obstruction -> cirrhosis treat with ursodeoxycholic acid - high doses due to poor absorption
 * Other complications of CF**

__CF-related diabetes__ not autoimmune, median onset 18-20yrs - decreased insulin and insulin resistance - ketoacidosis is rare

onset usually symptomatic (screening important - not HbA1c) - annual OGTT in stable patients over 10yrs - usual clinical clues: polyuria, polydipsia, delayed puberty, poor weight gain, decreased lung function

management - early detection - education - nutrition (120-150% RDI energy, high calorie, not diabetic diet) - insulin

__Bone disease__ chronic malabsorption - increased risk of osteoporosis, osteopenia and arthropathy

__Reproduction__ men - almost always infertile (no vas deferens) - otherwise normal sexual function

women - anatomy normal - ?decreased fertility - thick cervical mucus - menstrual irregularities, annovulation