Cystic Fibrosis: Across the Age Span
What nurses need to know
Linda Thrift
CF Nurse Specialist
May 2005
What is CF?
• Autosomal recessive condition
• Commonest lethal genetic condition among Caucasians
• 1:25 people are carriers
• Carriers are asymptomatic
• 1:4 chance per pregnancy of a child with CF
• More than one child with CF in the family
Genetic Defect
• >1000 CF genes identified
• CF gene first identified 1989
• Dysfunction of cystic fibrosis transmembrane regulator (CFTR) gene
• Defect in the protein that codes the transport of sodium and chloride across the cell membrane
• All exocrine secretions are thicker than usual
What is CF?
• Multi organ disease - lungs, pancreas, liver, GI tract, skin, sinuses, and reproductive organs
• Complex therapies involving medications, physiotherapy and maintenance of nutrition
• Cared for by a multi-disciplinary team including a respiratory physician
Northern European Folklore
Woe to that child which, when kissed on the forehead tastes salty. He is bewitched and soon must die.
Diagnosis
• Guthrie test
• Sweat test
• Genotyping - Delta F508
• Adult diagnosis - an increasing occurrence
The Course of CF Lung Disease
• Progressive infection and inflammation in the lower airways
• Staph Aureus
• Pseudomonas Aeruginosa
• Mucoid Pseudomonas
• Burkholderia Cepacia
• Oral antibiotics
• IV antibiotics - in patient or at home
Clinic Visits
• Monthly/3 monthly
• Height & weight, sputum test, spirometry
• FEV1 and FVC
• Multidisciplinary team
Goals of Nutrition Management
• Evidence shows correlation between weight and lung function and can be a predictor of outcome
• Methods to increase energy intake
-enzyme supplementation
-high energy diet
-supplements
-enteral feeding
Pancreatic Enzyme Replacement Therapy (PERT)
• Taken to prevent malabsorption of fat and fat soluble vitamins
• Dosage calculated by the individual depending on the amount of fat in the meal/snack
• Drugs used -Creon 10 000, Creon Forte, Panzytrat, Pancrease,Cotazym
Achieving the Nutrition goals on the ward
• Twice weekly weight
• Checking that the supplements get to the patient
• Overnight feeding
• Food vouchers - Subway, McDonalds, Muffin Break & staff canteen
Fat Soluble Vitamins
• Vitamins A, D, E and K
• Blood levels checked annually (Prothrombin Ratio for vitamin K)
• Drugs used - Multivite 6, Rocaltrol, Calcitriol, Micelle E, Vitamin E
• Vitamin K (not fat soluble)
Goals of Physiotherapy Management
• Airway clearance
• Independent with nebulisers
• Encouragement to exercise
• Maintenance of good posture
Achieving Physio goals on the ward
• Liaising with the Physiotherapist
• Ensuring that physio has happened out of hours or at weekends
• Encourage getting dressed and going out for a walk
• Empty sputum pots frequently
Nebulised Medications
• Mucolytics - Hypertonic Saline,Pulmozyme
• Antibiotics - Tobramycin, Colymycin (Colistin)
• Nebuliser - Pari LC Plus and filter for antibiotics
Sidestream or Pari LC Plus for Pulmozyme
Psychological Impact of Hospital Admission
• Frequent hospital admissions
• Disruption of regular routine
• Are we meeting their needs?
• Being cared for by people who know nothing or little about CF
• Segregation / Isolation
• Normal people who have a chronic illness
Increasing Survival
• Babies born now are expected to live >30 years
• Earlier diagnosis
• Physiotherapy
• Early introduction of pancreatic enzymes
• Antibiotics
• Research +++
Transition from Paediatric to Adult care
• 16 - 18 years of age
• Preparation for transition - sees doctor alone, encouragement with self care
• Formalised process - paed. & adult CF team
meeting, CF NS attends last paed. clinic
appt, “walkabout”.
• Communication between adult and paed. Teams
Increasing survival - new issues
• CF Related Diabetes (CFRD)
• CF bone disease
• Liver disease
• Pregnancy
• Infertility - male
• Lung transplantation
Glucose Intolerance
• Raised blood glucose when unwell or on Prednisone.
• Precursor to CF Related Diabetes
• Blood glucose checked fasting (pre breakfast) & 2 hours after meals
• Individual perception of this diagnosis
CF Related Diabetes
• Different clinical entity to Type 1 & Type 2
• High fat and high carbohydrate diet
• Limited restriction on sugars
• Blood glucose checked before meals prior to insulin
• Followed by Diabetes team as an out pt.
• In patient referral for assessment
CF Bone Disease
• Low bone mineral density
• Causes - vitamin D deficiency, prolonged steroid exposure, delayed puberty
• Diagnosed by Dual Energy Xray Absorptiometry (DEXA)
• Treatment - vitamin D, calcium supplements, exercise
• IV Biphosphonates
Liver Disease
• Thickening of bile causes the formation of plugs which block the bile ducts
• Portal hypertension
• Cirrhosis
• Liver transplant
• LFTs, palpation of liver and spleen gastroscopy
Pregnancy
• Optimal health pre pregnancy
• Genetic testing of partner
• Pregnancy can be well tolerated with good medical care
• Good communication between obstetric and medical team
• Breast feeding is not recommended
Male Infertility
• Congenital bilateral absence of vas deferens (CBAVD).
• CF diagnosis made at the time of fertility investigations
• Sperm aspiration and assisted reproductive techniques available
• Awareness of infertility - age and stage
Lung Transplantation and CF
• Time of referral
• Transplantation assessment process
• Both lungs transplanted
• CF does not recur in the new lungs
• 16 CF transplants to date in NZ
• Currently 5 on the waiting list in NZ
•
End of life issues
• Terminal stages are not easily defined
• FEV1 <30% predicts survival rate of 50% at 2 years
• Use of oxygen - overnight +/- portable
• Non invasive ventilation (BiPAP or VPAP)
• Hospital Vs Home Vs Hospice
• Supporting the individual and their family
Take Home Message
• No longer a disease of childhood
• Multi organ disease managed with complex therapies
• Respect for the individual - they manage their CF out of hospital
• The CF Nurse Specialist is here to help!
Useful CF websites
• www.cfnz.org.nz
• www.cftrust.org.uk
• Journal of Cystic Fibrosis
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