Children Health Pws,Romania,09,Blichfeldt,24.4
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Children Health Pws,Romania,09,Blichfeldt,24.4 Children Health Pws,Romania,09,Blichfeldt,24.4 Presentation Transcript

  • CHILDREN WITH PWS MEDICAL CARE Susanne Blichfeldt MD Denmark April, 2009
  • Prader-Willi Syndrome
    • a very special condition
    • so many different aspects and questions
    • symptoms change during the years
    • not all children have all ``PWS-problems``
    • but they have a lot in common
  • PWS daily care
    • Medical care
    • Psychological care
    • Social care : understanding and treatment
    • So many aspects to take care of
    • So many persons involved
    • And : all need to know about PWS
  • PWS : what is our goal
    • A happy - healthy - child
    • no pain, happy days, friends
    • strengths of the child to be cultivated
    • no misunderstandings and no unrealistic expectations but: respect for the child
  • PWS is a genetic condition The genes ``missing´´ seems to cause DYSFUNCTION OF THE HYPOTHALAMUS
    • Hunger - satiety
    • Temperatur : instability & changed sensation
    • Pain : high threshold or changed
    • Endocrine : growth and puberty hormones
    • Salt balance in blood
    • Sleep : sleep cycles affected, day- sleeping
    • Behaviour is different from normal
  • FLOPPY INFANT
  • The newborn with PWS
    • A very floppy infant. Slight dysmorphology
    • Very characteristic face expression
    • No cry, sleeps a lot
    • Shows no interest in food:
    • Can´t suck or swallow:>> tube feeding
    • Often luxation of joints:: hips, feet
    • Hypogonadism: boys: testicles ``down`` ?
  • THE FLOPPY BABY WITH PWS
    • A VERY QUIET BABY
  • HOW to treat the child with PWS the first weeks
    • Tube feeding and try bottle feeding every 3-4 hours
    • Stimulation: wake up the child
    • Movement of the body
    • Physiotherapist can give advices, early!
    • Regular physiotherapy from now on!
  • First year of life with PWS
    • Gradually the child ``wakes up´´
    • The child becomes a better ``eater``
    • But still often ``a very long meal`` falls asleep
    • Still a very easy child, few demands!
    • At one year : sit up, babble, more alert
  • FOOD? FOR SMALL CHILDREN WITH PWS
    • Food: breast milk or formula the first months
    • then gradually introduce ``baby food`` at relevant age.
    • The small child often eats slowly and falls asleep when eating: many breaks and more meals than usual are needed.
    • The dietician is important for the child
    • calculate : calcium, iron and vitamins !
  • Medical questions..small children
    • Temperature instability : high or low temperature CAN occur without illness !
    • but ask the doctor for a check !
    • Sleep : The small child sleeps a lot, often.
    • falling asleep at meals is not a seizure
    • Seizures are rare, most often with fever
  • PWS: growth and weight the first years
    • Some are short at birth but not all
    • Some have low weight at birth but not all
    • Often poor weight gain the first months
    • Growth can be slow but not always
    • (look at the child!)
    • Regular clinic-visits to check weight & length
    • Growth hormone treatment ? When ?
    • An often asked question
  • GROWTH HORMONE TREATMENT recommended for most children with PWS
    • Normal sleep study before is needed
    • Normal examination of throat needed
    • Blood tests for all hormones and ``blood sugar``
    • When to start? No rules! After sleep study!
    • Side effects ?: fluid retention first days, diabetes ? intracranial edema (rare)
    • Positive effects : growth, muscles, breathing and motor function. No effect on appetite !
  • What more to check
    • Eyes : squint often seen – glasses ??
    • Teeth : enamel defects often seen in first teeth. Second teeth seems normal. sticky saliva .
    • Teeth brushing often, regularly!
    • Joints and feet: luxation? Right shoos
    • Spine : scoliose, rare early in life, seen later
    • Skin : sensitive, take care of the sun!
  • The child with PWS after 2-3 years
    • More interest in food ..gradually
    • Better motor function, great variation
    • Better understanding
    • Some have severe speech problems
    • More awake
    • Special interests? Not very demanding
    • Still a rather mild and quiet child
  • The child after 2-3 years with PWS
    • Regular weight control : 4-5 times per year
    • Height, muscle function : Growth hormone?
    • Spine and joints : check 2-3 times per year
    • Eyes and teeth : regular checks
    • Behaviour : advices are strongly needed!
    • Ideal : paediatrician 3-4 times per year
    • dietician: minimum 4 times p.year
    • physiotherapist: regularly
  • THE CHILD AND THE WEIGHT
    • Weight (normal) at birth is around 2,5-4 kg
    • At 12 moths : around 9-11 kg
    • At 24 moths ( 2 years) 11-14 kg
    • At 3 years: 12-15 kg… and then 2 kg pr year!
    • IN PWS: the same is recommended!
  • HOW to help the child ?
    • At 3-4-5 years of age most children with PWS are VERY interested in food.
    • Without help a severe overweight is seen
    • Parents and teachers must help the child: to stop ..and leave the table. not to discuss
    • We can`t ask the child to be responsible for the meals, this is to ask for the impossible. like asking the child not to have PWS
  • What is most important?
    • To create good habits: about
    • 1. food, meals and eating
    • 1. motor activity
    • Overweight and motor inactivity :
    • the biggest problems in PWS world-wide
  • A HAPPY CHILD WITH PWS. Walks with support
    • He enjoys to move
  • Early intervention is needed to prevent obesity for that reason! have a daily program
    • And then
    • something special
    • in PWS
  • Bed Wetting and Water Intoxication
    • Many are not dry during night after age 3-4
    • Treatment with anti diuretics :
    • Take care that the child does not drink a lot before sleeping when taking this medication:
    • The water will stay in the body and dilute the blood causing low salt concentrations:
    • This can cause severe seizures ( cramps)
  • WARNING!! Abnormal pain threshold
    • WHAT DOES THAT MEAN ?
    • ABDOMINAL CRISES CAN BE OVERLOOKED
    • If a child with PWS vomits or does not eat
    • the child can be very ill !!!
    • FEW COMPLAINTS WHEN BROKEN BONES
    • When a child suddenly cannot walk :X-ray !!
  • Most asked questions
    • Is there a cure for PWS?
    • Any medication to prevent obesity?
    • Gastric banding ?
    • Any food better?
    • A special diet?
    • Autism disorder, psychiatric disorder ?
  • The answers
    • To most of these questions are:
    • no
  • Behavioural management
    • Look at developmental age :
    • where is he or she ???
    • Social and cognitive ages can be different :
    • Always ask yourself:
    • what does he/she really understand
    • And then set the expectations after that
    • Clear messages: as for small children !
  • THE CHILD WITH PWS IN THE SCHOOL
    • Normal weight is possible if the teachers are educated and actively involved in the treatment of the child (PWS)