CYSTIC FIBROSIS

Who are we talking about?L.L is a 4 year old

Preschooler

Has a 6 year old brother, who does not have the disease

Lives with both parents, brother and 2 dogs

Enjoys swimming twice a week

Past HistoryBirth history:

-Born at 41 weeks,

-Had meconium ileus at 9 days of life

-Had genetic screen: CF diagnosis

Family and psychosocial History

L.L. lives with his parents, a 6 year old brother and 2 dogs.

Both parents work.

The family lives at Virginia Beach.

Mother is from South Africa and has not family in the country.

L.L. was diagnosed with CF at 2 months of age.

Grandmother helps parents during hospitalization.

Cultural considerationsL.L father is a paramedic who works

in Virginia Beach: he is constantly aware of L.L health changes.

L.L is a preschooler, who miss his friends at preschool.

L.L borne with this disease so he has been receiving treatment since he was 2 months old.

Reason for admission1 week of coughing, rhinorrhea and fever.

His PCP ordered Amoxicillin for otitis media on left ear.

2 days before hospitalization: tachypneic

1 day before hospitalization: retractions, belly breathing, cough, sputum, post-tussive emesis.

Day of admission: O2 sat 89%, he usually has 97-98% at home, increased WOB, fever 103 F.

Primary medical diagnosis

Cystic fibrosis exacerbation

CF is an autosomal recessive trait where both parents gave the child the CFTR gene in order to have CF.

Treatment PlanCPT: medicated aerosol

treatment. 2.5 mg, 3ml inhalant nebulizer, qid

Oxygen therapy: nasal cannula 2.5 liter/min.

CPT: vest pressure hz=14, qid (20min)

Antibiotic therapy: Trobamycin, Ticarcillin-Clavulanate

Relationship with any medical diagnosis?

He is suspected to have pneumonia

This is common in patient with cystic fibrosis

Hard to get rid off thick mucus

Perfect environment for bacteria

In what developmental age is L.L?

L.L is in the Preschooler developmental ageAdvance perception of shape and

development of fine muscle coordination (scribble and draw).

They use their fingers rather than the fits to hold writing instruments.

Between 4 and 5 child can trace and copy a square.

Psychosocial developmentMain task is acquiring sense of

initiative.Energetic to learnPlay, work, live to the fullestFeel accomplish and satisfied in in

their activitiesStart feeling guilty if they

misbehaved Superego or conscious start

developingLearning right form wrong or good

from bad> beginning of morality.

Preoperational phaseShift from total egocentric to social

awarenessCan consider other point of viewCan think in one idea at the timeAssumed that everyone thinks as

they doMagical thinkingBelieve in the power of words

(literally)

Did he meet the norms?Yes! He met the norms of this developmental

stage.

Because:He drew circles and painted them. He

knew the colors of his pens.He felt accomplished after pushing the

green button to make the medication pump work.

He loved to make bracelets from paper and tape.

Sometimes he considered other points of view.

Developmental theory to care and communication

Psychosocial theory: L.L. is likes to play , has initiative and start differentiating between right or wrong: L.L. was allowed to play and to “ help the nurse on his own care”.

Cognitive theory, he considers others point of view and can think one thing at the time: L.L was explained one thing at the time and language was simple.

Physical assessmentResp: cough, wheezing bilaterally,

mucus, change to portable oxygen tank to ambulate, decrease appetite

Skin: peripheral IV access clean (dry and intact) skin warm. No bruises or rush

Psy/soc: has company 24 hrs (mother, father or grandmother)

Fall: likes to play and ambulate on room, has a portable oxygen tank

1)Impaired gas exchange

Excessive mucus in lung

Possible pneumonia

Cough, wheezing/1 wk

Hazy areas of opacification in lung

Retractions

O2 sat 80-90% on day of admission

Post tussive emesis 

O2 sat: 94% 3 liter/min

X-ray: opacity in lung

Mucus culture: in progress

Htc: 34.0% low

Expected outcome: : Patient will maintain O2 sat> 90% before discharge

Assess respirations, adventitious sounds, and cough, and mucus production, use of accessory muscles q4r

Use pulse oximetry to monitor oxygen sat. q2r or prn

Position head of bead> 45 degrees, encourage or assist with ambulation

Use of oxygen 3 to maintain O2 sat>90%

Encourage fluid intake > 1365 ml/day

Monitor CPT treatments with vest and albuterol nebulizer asking the specialist if the tx was complete or not.

2)Imbalanced nutrition: less than body requirements

Decrease absorption of nutrients en duodenum

Needs 150% amount of nutrients

Had meconium ilues/at 9 day

Gerd

Loss of appetite

Htc: 34.0% ( check for anemia)

NPO before attempt to put a PICC

Expected Outcome: Pt will maintain or gain healthy weight during

hospitalization

Measure weight daily. Same time, same clothing, same scale.

Assess the environment were eating occurs (pleasant environment)

Provide companionship while eating

Provide good oral hygiene

Small frequent meals high in calories and proteins: 3 meals + 3 snacks

Provide medications: Pancrelipase, zantac (for GERD), Miralax ( laxative)

3) Risk for dehydration:

O consistently >I and < maintenance fluid

NPO

Increase metabolic rate/ possible infection

24 before clinical: I&O= 602.43-801=-198.57

Day 1: I&O=159/250= - 91

IV fluid discontinue

Expected Outcome: will have an intake at least minimum to keep basal metabolic intake: 1365 ml/day before discharge

Monitor daily weight, with similar clothes

Evaluate fluid intake in relation to dietary intake

Monitor BP and HR

Assess for skin turgor, mucous membrane, LOCX3

Monitor I&O q4r

Monitor hydration input should be more than 1365 output should be 415 ml/day: q4r

4) Infection

Prior ototis

Mucous in the lungs

Low fluid intake

Opacity in the lungs

Possible pneumonia

WBC:11.5

Fever: 103 F

CF exacerbation

Expected Outcome: pt will continue to not show signs of infection such as temp: BP: <37.4, no skin break down, no thick mucous secretions before discharge.

Meets minimum output of 415 ml/day , good skin turgor.

Promote ambulation

Teach parent and child hand washing technique: minimum of 30 seconds

Assess sputum for specific bacteria

Assess for fever, fatigue, malaise, oral lesions. Q4r

Provide antibiotic therapy: Tobramycin,Ticarcillin-Clavulanate

5)Caregiver role strain

Father and mother are working and have to constantly to ask for day off or switch shifts at work.

A family member always stays wit the patient (mother, father, or grandmother)

Parents had another child to take care (6 year old).

Caregiver express satisfaction with his/her role before discharge

Encourage caregiver to identify family members or friends available who can assist with care giving.

Encourage caregiver to set aside time for self; identifying what can bring them peace and relaxation.

Acknowledge to the caregiver his/her role value.

Provide time to discuss feelings and concerns (Support group)

 

Research: Paternal experiences of their children’s diagnosis of

Cystic Fibrosis following newborn screening diagnosis

Research was conducted on fathers of children diagnosed with CF because few studies were conducted in comparison with mothers.

15 fathers were interviewed in Western Australia using semi-structured interviews.

Interviews were conducted at home or by phone.

Fathers felt as protectors and tried to hide their feelings.

It was easy to recall their wives reactions, but difficult to recall their own reactions.

Fathers tried to support the mother-child relationship by caring for the mother.

Parental level of emotional adjustment was unclear, but anxiety was more evident.

Study highlighted the importance of providing therapeutic services to voice their concerns without reservations.

Relationship between actual nursing problems

Teaching and discharge planning

Discharge with a PICC: antibiotic treatment for 3 to 4 weeks.

Continue with routine treatment of Cf (medication reconciliation):Albuterol 2.5 mg, nebulizer BIDVest 14 mega hz, 20 min BIDCreon 6 with 3 snacks, 3 mealsMiralax, once per day.

Teaching: How to take care of a PICC while at home.

References:Ignatavicius, D. D., & Workman, M.L.

(2013). Medical Surgical Nursing: Patient- Centered Collaborative care. St. Louis Missouri: Elsevier Saunders.

Priddis, L., Dunwoodie, J., Balding, E., Barrett, A., & Douglas, T. (2010). Paternal experiences of their children's diagnosis of Cystic Fibrosis following newborn screening diagnosis. Neonatal, Paediatric & Child Health Nursing, 13(2), 4-10.