Moulage: Pediatric Cystic Fibrosis Thick, Sticky Secretions

Skill Level:

Beginner

Ingredients:

1 Tbsp. waterbased lubricating gel

1 Tbsp. petroleum jelly

Equipment

Masonite board

Palette knife or utensil

Technique:

On a masonite board, combine gel and vaseline. Stir the gel mixture thoroughly with the back of the palette knife to blend, creating a thick-sticky mucus.

Using a cotton swab or small paint brush, lightly blot secretion mixture to the lower, bottom of the nares, drawing it downward toward the lips.

Tip or Trick:

To create the characteristic oily, bulky stools: Use an insulating expanding foam product (such as those typically used to seal joints around windows and doors) to create small, 1-2 inch circles on wax paper- allowing space for foam to fully expand. Once expanded, approx. 1-2 minutes, using a gloved hand that has been lightly coated in baby oil- press down lightly on top of foam to create an indentation. Allow foam to fully set until dry; approximately 1-2 hours.

Once set, apply a simulator safe colorant to the stool and allow to air dry. Place fatty stool in diaper that has been pre-treated with several drops of synthetic odor: foul. Baby oil or Lubricant jelly may be brushed over the stool to create an oily residue.

The addition of both odor and sensory cues to the training scenario increases realism while providing the participant with valuable clinical insight.

Cleanup and Storage:

Using a soft clean cloth or make-up remover towelette, remove the mucus and remaining product residue from the skin of the simulator according to manufacturer directions.

Training Scenario:

Simulation Suite Location:

Clinic: Follow up appointment

Set the Stage: Use a high-fidelity simulator to create realistic movement and crying in the newborn. Loosely wrap simulator in a newborn blanket and place in mothers arms with partially full baby bottle. Prompt mother to be concerned and attentive to newborns' cries, aware of her babies' overall sense of discomfort, abdominal distention and foul smelling flatus.

Confederate: When prompted, advise the "mom" to discuss newborn history including feeding times, diaper changes and sleeping schedule. Mother to appear exhausted, concerned about the baby being so colicky and uncomfortable in addition to being overwhelmed with the many duties of new parenting. Mom is G-1, P-1.

Support roles: Consider having other roles in the simulation such as a grandmother who explains all of her children (including mother of newborn) were colicky as babies.

Patient Chart:

Include chart documentation that supports date and time of birth, apgar scores, birthing weight and bi-weekly visits indicating a "failure to thrive."

When taking the newborns vitals, set scale to indicate an additional 4% weight loss from last weight and decreased breath sounds.

Use in Conjunction With:

Abdominal distention

Increased frequency of stools

Failure to thrive (despite adequate appetite)

Flatulence or foul-smelling flatus, steatorrhea

Jaundice

Simulated "Fatty or Oily" stool

Odor: Foul

What the experts have to say:

MEDICAL SIMULATION SCIENTIST

Cystic fibrosis is an inherited disease which leads to progressive damage to the respiratory system and chronic digestive system problems. The clinical presentation of cystic fibrosis varies widely in severity of symptoms, and rate of disease progression in the organs involved. Clinical manifestations also vary with the patient's age at presentation.

Cystic fibrosis is generally diagnosed during infancy (6-8 months), with 66% of patients diagnosed by age one. Neonates may present with meconium ileus, a bowel obstruction that occurs when the meconium in the infant’s intestine is thicker and stickier than normal meconium, creating a blockage in the ileum. More rarely cystic fibrosis may present with other features such as extreme generalized edema, with widespread swelling of the skin due to effusion of fluid into the extracellular space (anasarca). The inflamed skin will be cool to the touch, may appear red, dry and flaking. The skin will reveal depression, or “pitting”, when pressed which will last for a few seconds due to loss of elasticity. Increased susceptibility to sheer and painful skin is also a result of the anasarca. Patients younger than 1 year may present with wheezing, coughing, and/or recurring respiratory infections and pneumonia. GI tract presentation in early infancy may be in the form of steatorrhea, failure to thrive, or both.

Meconium ileus occurs in ~10% cystic fibrosis sufferers. Infants who experience meconium ileus may present at birth with severe abdominal distention, bilious vomit and sometimes abdominal wall erythema and edema. The abdominal distention may be contribute to respiratory distress. A less obvious indicator of cystic fibrosis is delayed passage of meconium (>24-48 hours after birth) and/or prolonged cholestatic jaundice. Undiagnosed infants and children may present with increased frequency of stools, which suggests malabsorption (ie, fat or oil drops in stools).

Patients with pancreatic insufficiency have fat-soluble vitamin deficiency and malabsorption of fats, proteins, and carbohydrates (however, malabsorption of carbohydrates is not as severe as that of fats and proteins). Patients sometimes present with generalized failure to thrive (despite an adequate appetite), flatulence, abdominal pain, and abdominal distention. Infants may develop a chronic or recurrent cough, which can be dry and hacking at the beginning and later become productive. Prolonged symptoms of bronchiolitis occur in infants. Paroxysmal coughing followed by vomiting may occur.

SYMPTOMS: Jaundice GI bleeding Meconium ileus Abdominal distention Intestinal obstruction Increased frequency of stools Failure to thrive (despite adequate appetite) Flatulence or foul-smelling flatus Recurrent abdominal pain Respiratory symptoms may include the following: Atypical asthma Dyspnea on exertion Chest pain Cough Recurrent wheezing Recurrent pneumonia Genitourinary symptoms may include the following: Undescended testicles or hydrocele Delayed secondary sexual development Amenorrhea

Physical signs depend on the degree of involvement of various organs and the progression of disease, as follows: Anasarca, red, dry, edemetous skin Nose – Rhinitis, nasal polyps Pulmonary system – Tachypnea, respiratory distress with retractions, wheeze or crackles, cough (dry or productive of mucoid or purulent sputum), increased anteroposterior chest diameter, clubbing, cyanosis, hyperresonant chest on percussion GI tract – Abdominal distention, hepatosplenomegaly, rectal prolapse, dry skin, cheilosis

Catriona D. Graham, PhD, Medical Simulation Scientist

Catriona Graham holds a Ph.D. In biomedical research with a concentration in neuroscience and is completing her M.S. in pharmacology and toxicology. She is an Advanced Clinical Simulation Fellow at the Dayton Veterans Administration Medical Center. Dr. Graham is a dedicated educator with 20 years of experience and is currently faculty instructor of Anatomy and Physiology

PRE-HOSPITAL SIMULATION:

Cystic fibrosis When previously teaching the science behind CF and encouraging my paramedic teaching team to develop their simulation I take my inspiration when making up my standardised patients or manikins from the attached article published in the UK daily mail. http://www.dailymail.co.uk/femail/article-3057794/Women-suffering-cystic-fibrosis-pose-powerful-picture-series-order-bring-hope-children-teens-diagnosed-life-threatening-condition.html These ladies are inspirational - sharing their photos which allow us to recreate their physical appearance. I have asked for support from CF patients who come in and teach my paramedic students but all are in awe of these ladies who ask us to understand the effects of CF on their bodies. Environmental - simulation - cf could be in any venue- just make sure that you have lots of medication / antibiotics or even physiotherapist card as your props. Get the history right! Smell and taste - CF is one of the few things that the literature says parents can taste when they kiss their children - CF patients have excessive salt- but is usually picked up in infants by routine screening these days Cystic fibrosis is caused by a genetic mutation; specifically a mutation in a gene called CFTR. A genetic mutation is when the instructions found in all living cells become scrambled in some way, meaning that one or more of the processes of the body do not work in the way they should. The CFTR gene normally creates a protein that regulates levels of sodium and chloride in cells. If the CFTR gene is defective, it results in a build-up of thick, sticky mucus in the body's tubes and passageways. These blockages damage the lungs, digestive system and other organs, resulting in the symptoms of cystic fibrosis. Most paramedics know the CF patients in our area as we can get called to support these patients regularly - and have learned to listen to them and their relatives - when these guys say they are ill - they are ill! Cystic fibrosis is most common in caucasian people of northern European descent. It is estimated that one in every 2,500 babies born in the UK will be born with cystic fibrosis and there are more than 9,000 people living with the condition in this country. The condition is much less common in other ethnic groups.

Dave Halliwell, PHD student

Dave Halliwell is a UK collaborator and has been studying simulation for his PhD which is nearing completion - after 7 years of PhD study he has some insightful takes on the best ways to enhance learning. David is a paramedic by trade, previous Olympic gold commander (sailing), and Head of Education for UK NHS ambulance service with 3000 staff. He is often found at international conferences talking about simulation!

NURSING SIMULATION:

Cystic fibrosis is a life-shortening genetic disease that has had significantly improved patient outcomes in the past decade with medical advances. As such, it is a great learning opportunity to incorporate key areas of GENETICS, EVIDENCE-BASED PRACTICE AND INFORMATICS!

For example, PREWORK could involve learners exploring the most evidence-based information on CF medications and/or Clinical Care Guidelines. One great resource is the Cystic Fibrosis Foundation website.

Here is a link from September of 2015: https://www.cff.org/For-Caregivers/CF-Clinical-Care-Guidelines/

COMMUNICATION:

As a pediatric topic, CF simulation has rich opportunity for learners to communicate in difficult situations with families experiencing getting a difficult diagnosis, dealing with life stressors, and working with family systems.

HEALTH EDUCATION METHODS:

Healthcare learners can develop teaching skills around the myriad of topics caregivers need to master to care for children with CF, including home nebulizers, medication routines, chest physiotherapy &/or use of vest airway clearance systems. Learners can practice assessing families’ emotional status. Education methods can be explored to consider what is truly effective and impactful for topics like sharing difficult facts about the disease, describing CF symptoms, and how to navigate the healthcare team. Learners will need to gain experience in providing hope while not giving false hope, a true communication art!

INSIGHT INTO LIVING WITH A CHRONIC CONDITION: Longitudinal Scenarios:

Patients and their families can have better experiences living with such chronic conditions as CF with healthcare team members who have insight into the caregiver strains and emotional impact of such illnesses. I have run a CF case as 3 shorter scenarios at different points in the patient and family’s lifespan.

Scenario 1: Infant and the mother receives the new diagnosis of CF. Once the mother hears this news, learners take turns educating her about immediate needs for care while she deals with a crying baby and is overwhelmed.

Scenario 2: Occurs when the child begins full day kindergarten. The mother meets with the school nurse to develop a plan of care. The learner’s challenge is to develop rapport and establish a foundation for a trusting relationship with a plan of care that includes clear communication between the mother at home and the school nurse.

Scenario 3: Occurs in the hospital after the 17 year old develops a respiratory infection that is not responding well to antibiotics. Learners can sense the struggles and emotions of the family and patient who may possibly progress to an end-of-life scenario.

PSYCHOLOGICAL SAFETY:

Simulation educators must also be sensitive to the lived experiences of learners as they may know someone with CF, may have had a recent personal loss of a loved one, and may have strong personal emotions as a result of such experiences. Psychological safety may mean telling students about the scenario outcome and inviting them to let you know they are not sure they can make it through this scenario. Most students make it through even though it may elicit strong feelings, but sometimes the personal situation is too sensitive and an optional learning activity may be necessary.

Sara Manning, MS, RN, CHSE

Coordinator of Clinical Simulation Education

NLN Simulation Leader

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