bronchail asthma & cystic fibrosis in pediatricpptx

Asthma is defined as a chronic inflammatory disorder associated with variable airflow obstruction and bronchial hyper responsiveness and characterized by recurrent episodes of wheeze, cough, shortness of breath, and chest tightness.
Asthma affected an estimated 262 million people in 2019 and caused 455 000 deaths.
(1) PHARMACOTHREAPY
(2) NON PHARMACOTHREAPY
India has estimated 15-20 million asthmatics with a prevalence 10-20% in 5-11 year children.

1. BRONCHIAL ASTHMA SUBMITTED TO SUBMITTED BY MRS. GEETA RANI NAYAK MS. PUNAM BISWAS ASSISTANT PROFESSOR MSC NURISNG 1 ST YEAR CON, AIIMS BBSR CON, AIIMS BBSR

2. Theme: “Asthma Education Empowers”

3. INTRODUCTION

4. DEFINITION Asthma is defined as a chronic inflammatory disorder associated with variable airflow obstruction and bronchial hyper responsiveness and characterized by recurrent episodes of wheeze , cough , shortness of breath , and  chest tightness .

5. EPIDEMIOLOGY The incidence of asthma has steadily increased in both developed and developing countries from  1970 to 2000 Asthma affected an estimated 262 million people in 2019 and caused 455 000 deaths. (WHO 2022) India has estimated 15-20 million asthmatics with a prevalence 10-20% in 5-11 year children.

6. GLOBAL BURDEN

7. CONT… A Hospital based study in Bengaluru showed that the prevalence of asthma steadily increased from 9% in 1979 to 29.5% in 1999 . It decreased by 3% in 2004 and further by 1% in 2009 . However, persistent asthma increased by 20-72% and persistent severe asthma by 4-11% from 1999 to 2009

8. 02 03 01 RISKS FACTORS Heredity Urbanization Allergy

9. ETIOLOGY

10. BIOLOGICAL IRRITANT Dust mites Tobacco smoke Cockroaches Cooking fuel Pollens Mosquito coil Fungi Sprays Pets Compressed wood furniture Viral infection Food

11. BASED ON TRIGGER FACTOR ETIOLOGICAL FACTOR (1) extrinsic (2) intrinsic (1) atopic (2) non atopic TYPES

12. P A T H O P H Y S I O L O G Y

13. Exposure to specific antigen Activated mast cells, T cells, eosinophils release histamine, leukotrienes, cytokinin and prostaglandins Bronchospasm and Increased bronchial secretion Increase vascular permeability particularly eosinophil causes endothelial damage Airway inflammation and obstruction Irreversible damage to lumen causing edema, scarring, fibrosis Thickened basement membrane

14. CLINICAL MENIFESTATION

15. RECURRENT COUGH

16. DYSPNEA

17. PROFUSE SWEATNG

18. NASAL FLARING

19. STAGES STAGES TIME CHARACTER Early phase reaction Within 15-30 minutes Bronchoconstriction Late phase reaction 4-12 hours later Tissue inflammation Airway resistance Decreased oxygen flow

20. DIAGNOSIS

21. CONT… (1) HISTORY (2) PHYSICAL EXAMINATION (3) BLOOD INVESTIGATION EOSINOPHIL COUNT TOTAL IgE LEVEL SPECIFIC IgE LEVEL (4) IMAGING CHEST X RAY (5) SKIN TEST WITH ALLERGEN (6) FUNCTIONAL EXHALED NITRIC OXIDE

22. https://youtu.be/7oTfvJff7go?si=HpeIST6k75TPGfxP

23. GRADES: SEVERITY CLINICAL FEATURE INTERMITTENT COUGH + MILD WHEEZING FOR SEVERAL TIMES A YEAR+ OCASSIONALLY DYSPNEA MILD PERSISTENT COUGH + MILD WHEEZING FOR ONCE A MONTH + DYSPNEA MODERATE PERSISTENT COUGH + MILD WHEEZING SYMPTOMS ONCE OR MORE A WEEK SEVERE PERSISTENT COUGH + MILD WHEEZING CONTINUE EVERYDAY

24. MANAGEMENT

25. CONT… (1) PHARMACOTHREAPY (2) NON PHARMACOTHREAPY

26. CLASSIFICATION OF DRUG USED FOR MANAGEMENT OF ASTHMA QUICK RELIEVER CONTROLLER LONG TERM SYMPTOMS RELIEVER ACUTE ATTACK LONG TERM RELIEVER BRONCHOSPASM SABA IPRATROPIUM LEUKOTRINE RECEPTOR ANTAGONIST ICS THEOPHYLLIN MAST CELL LABA

27. FREQUENCY OF SYMPTOMS STEP OF THERAPY LUNG FUNCTION SYMPTOMS <2 EPISODES/ MONTH I FEV1 >80% PEF <20% SYMPTOMS >2 EPISODES/ MONTH II FEV1 >80% PEF 20-30% SYMPTOMS ALMOST DAILY NIGHT SYMPTOMS >1 WEEKS III FEV1 >60% PEF >30% SYMPTOMS ALMOST DAILY NIGHT SYMPTOMS >1 WEEKS LOW LUNG FUNCTION IV FEV1 <60% PEF >30% SEVERLY UNCONTROLLED SYMPTOMS EVEN ON STEP IV THERAPY V …….. GUIDE TO CHOOSE THERAPY

28. STEPWISE MANAGEMENT OF ASTHMA IN CHILDREN

29. CONTINUUM CYCLE OF ASTHMA MANAGEMENT

30. ROUTE OF ADMINISTRATION OF MEDICATION ORAL ROUTE INHALED ROUTE MDI SPACER DRY POWDER INHALER NEBULISER

31. INHALERS MDI

32. CONT… SPACER

33. https://youtu.be/cjyf7lWnEBI?si=dDqqJ52ORXt_zc7B

34. MDI WITH SPACER

35. MDI WITH SPACER WITH MASK

36. DISKUS

37. https://youtu.be/mfiShjE9P-Q?feature=shared

38. TURBUHALER

39. Status asthmaticus

40. DEFINITION Severe exacerbation that does not respond to repeated or continuous administration of SABA in an emergency setting

41. SEVERITY OF ASTHMA SCORING Score Respiratory rate (<6 years) Respiratory rate (>6 years) Wheezing Accessory muscle use <30 <20 None None 1 31-45 21-35 End expiratory Questionable 2 46-60 36-50 Full expiratory Apparent 3 >60 >50 Both ex and ins Maximal

42. M ETABOLIC CORRECTION M USCLE SPASM M UCOSAL EDEMA M UCUS SECREATION M ONITORING INFECTION M V 6M MANAGEMENT

43. Approach to asthma exacerbation in children

44. NON PHARMACOTHRAPY

45. PREVENTION The primary prevention is to prevent sensitization to allergens. Avoid unnecessary use of high spectrum antibiotics . The secondary prevention is to prevent occurrence of symptoms in sensitized child is to avoid environmental triggers predominantly the tobacco smoke and indoor pollutants such as dust mites, cockroach, fungi, pollens and other smokes . The tertiary prevention is to control the symptoms by environment control and long-term use of anti-inflammatory drugs and quick relievers as rescue medicines in any exacerbations.

46. NUTRITIONAL MANAGEMENT Suggest smaller frequent meals for better diaphragm work and also to help to decrease the chances of gastro esophageal reflux Always prefer home-made traditional food Encourage fresh fruit, vegetables, butter, curd, picks fish and food with omega-3 fatty acids.

47. PATIENT EDUCATION Family support Misconception elimination Community education Nutritional education Environmental control Support to issue Self esteem enhancement

48. NURSING DIAGNOSIS (1) Ineffective airway clearance R/T bronchoconstriction, creased mucus production, and respiratory infection AEB wheezing, dyspnea, and cough. (2) Ineffective breathing pattern R/T decreased lung expansion AEB dyspnea and wheezing. (3) Impaired gas exchange R/T ventilation perfusion Imbalance AEB dyspnea, tachypnea, and tachycardia (4) Activity intolerance R/T imbalance between 02 sup- ply and demand AEB reduced daily activity and exercise tolerance. (5) Fatigue R/T physical exertion to maintain adequate ventiation AEB use of accessory muscles to breathe (6) Parental fear and anxiety R/T child's hospitalization and breathlessness. (7) Deficient knowledge due to insufficient information on child's condition. (8) Interrupted family process R/T having a child with a chronic illness. (9) Risk for deficient fluid volume R/T difficulty in taking fluids, insensible fluid losses from tachypnoea and diaphoresis (10) Risk for suffocation R/T interaction between individual and allergy

49. COMPLICATION EMPHYSEMA HYPOXEMIA CARDIAC ARRHYTHMIAS ATELECTASIS COR PULMONALE PSYCHOLOGICAL

50. Children with viral infection triggered asthma tend to outgrow the disease by 5 years of age when the immune system attains adult levels. Some more outgrow the disease by 8 years when the airway caliber reaches adult levels, more so in boys . During adolescent period majority tend to outgrow the attacks. However, those with atopic asthma (high IgE level) and children with low lung capacities and girls have the tendency for asthma remaining for an indefinite period PROGNOSIS

51. Journal

52. Preparation of Budesonide-Loaded Liposomal Nanoparticles for Pulmonary Delivery and Their Therapeutic Effect in OVA-Induced Asthma in Mice Xu Zuo   1 ,  Yinuo Gu   1 ,  Xiaoping Guo   1 ,  Wenxue Zheng   1 ,  Haoyu Zheng   1 ,  Yiming An   1 ,  Caina Xu   2   3 ,  Fang Wang Published on 2024 PMID: 38283200  PMCID:  PMC10811423  DOI:  10.2147/IJN.S441345

53. Abstract Purpose:  Inhaled corticosteroids, including budesonide (BUD), are widely employed for the treatment of asthma. However, the frequent use of corticosteroids is associated with numerous adverse effects and poses challenges to ongoing drug therapy and patient adherence . Budesonide liposomal nanoparticles (BUD-LNPs) were developed to improve the bioavailability of the drug and thereby improve the effectiveness of asthma treatment . Methods:  BUD-LNPs were prepared via thin-film hydration, and the characterizations, stability, and in vitro release of BUD-LNPs were studied. In vitro cellular uptake was observed by laser-scanning confocal microscope (LSCM) and flow cytometry . And the in vitro anti-inflammatory activity of BUD-LNPs was evaluated by measuring the expression of pro-inflammatory cytokines in activated macrophages. Besides, the accumulation time in the lung of drugs delivered via liposomal carriers and free drugs was compared in vivo . And the in vivo therapeutic efficacy of BUD-LNPs was assessed in OVA-induced asthmatic mice. Finally, in vivo biosafety assessment was performed.

54. Results:  The particle size, PDI, and zeta potential of BUD-LNPs were 127.63±1.33 nm, 0.27±0.02, and 3.33±0.13 mV , respectively. BUD-LNPs exhibited excellent biosafety and anti-inflammatory activity in vitro. Furthermore, compared with the free drugs, the utilization of liposomal nano-vehicles for drugs delivery could effectively extend the duration of drugs accumulation in the pulmonary system. Additionally, treatment with BUD-LNPs alleviated airway hyperresponsiveness, reduced airway mucus secretion, and mitigated pulmonary inflammation in OVA-induced asthmatic mice. And the BUD-LNPs demonstrated superior therapeutic efficacy compared to free BUD. Conclusion:  BUD-LNPs was successfully prepared with excellent stability and sustained release for 24 h in vitro . The data of anti-inflammatory activity, asthma therapeutic effects and safety studies indicated that drug delivery mediated by liposomal nano-vehicles was a feasible and desirable strategy for medical strategy and showed great promise in the clinical therapy of asthma.

55. Cystic fibrosis

56. INTRODUCTION Cystic fibrosis (CF) is the most common life-limiting recessive genetic disorder in Caucasians with an incidence of approximately 1 in 2500 children born in the United Kingdom . It is less common in African Americans, Asian Americans, native Americans and Indian By the end of the 1st yr of life, 85-90% of children with CF have pancreatic insufficiency , which, if untreated, will lead to malnutrition. Treatment of the associated pancreatic insufficiency leads to improvement in absorption, better growth, and more normal stools . DNA testing can be used prenatally and in newborns to identify the presence of the mutation. The American College of Obstetrics and Gynecology currently recommends screening for cystic fibrosis to any person seeking preconception or prenatal care. At present, all states include testing for cystic fibrosis as part of newborn screening

57. MUTATION The mutated gene responsible for CF is located on the long arm of chromosome 7 . This gene encodes a protein of 1480 amino acids called the cystic fibrosis transmembrane conductance regulator (CFTR). Most common mutation is F508del. The glycoproteins constitute a cAMP-activated chloride channel and regulate other chloride and sodium channels at the surfaces of the epithelial cells.

58. P A T H O P H Y S I O L O G Y Abnormally thick, sticky secretions Airway obstruction B ronchospasm , hyperinflation and chronic infection Impaired gas exchange Atelectasis, fibrosis, and destruction of pulmonary tissue Hypoxia, hypercapnea and acidosis Spontaneous pneumothorax, haemoptysis Pulmonary vasoconstriction Respiratory failure Pulmonary vascular resistance Death Enlargement of right ventricle Congestive heart failure

59. CLINICAL MENIFESTATION MECONIUM ILEUS ABDOMINAL DISTENSION VOMITING FAILURE TO PASS STOOL DEHYDRATION

60. CLINICAL MENIFESTATION CONT.. Respiratory and cardiovascular system: Wheezing Non productive cough Repeated pneumonia Bronchitis Purulent and copious sputum Chronic bacterial infection, Crackles, diminished breath sounds Retraction, hypoxia, cyanosis, tachypnea, Hemoptysis Sinusitis

61. DIGESTIVE SYSTEM Steatorrhea, Flatus, Growth failure, Xeropthalmia, Bleeding, Protuberant abdomen Intussusception Rectal prolapse, Biliary cirrhosis, Portal hypertension. Esophageal bleeding, and Pancreatic insufficiency

62. Exocrine glands High concentration of sodium and block chloride in sweat dry mouth electrolyte imbalances Reproductive system Delay in development of secondary sex characteristics , difficulty in pregnancy,

63. DIAGNOSIS History Physical examination DNA testing Sweat chloride test Xray

64. https://youtu.be/qQy0zrgO0hg?si=V10fbWBZRSR40BAs

65. Management

66. Respiratory problem GI problem Endocrine problem

67. https://youtu.be/1Ufj3oU_M2w?si=XIb0vDWhzfUHhAKN

68. M E D I C I N E B ronchodilator P ulmonzyme H ypertonic solution A ntibiotics

69. GI problem Nutrition rectal prolapse H2 receptor blocker

70. Nursing diagnosis Ineffective airway clearance related to excessive pulmonary secretions. Impaired gas exchange related to airway obstruc-tion . Risk for infection related to altered body defences . Activity intolerance related to pulmonary conges- tion . Imbalanced nutrition less than body requirement related to poor intestinal absorption of nutrients. Ineffective coping (child and family) related to chronic illness.

71. PROGNOSIS Median age is 40 years. 50% of older than 18 years. Lung, heart, liver transplant increases the survival rate.

72. The changing epidemiology of pulmonary infection in children and adolescents with cystic fibrosis: an 18-year experience Jagdev Singh ,  Sharon Hunt ,  Sharon Simonds ,  Christie Boyton ,  Anna Middleton ,  Matthew Elias ,  Susan Towns ,  Chetan Pandit ,  Paul Robinson ,  Dominic A. Fitzgerald  &  Hiran Selvadurai   https://doi.org/10.1038/s41598-024-59658-4

73. The impact of evolving treatment regimens, airway clearance strategies, and antibiotic combinations on the incidence and prevalence of respiratory infection in cystic fibrosis (CF) in children and adolescents remains unclear. The incidence, prevalence, and prescription trends from 2002 to 2019 with 18,339 airway samples were analysed .  Staphylococcus aureus   [− 3.86% (95% CI − 5.28–2.43)] showed the largest annual decline in incidence, followed by  Haemophilus influenzae   [− 3.46% (95% CI − 4.95–1.96)] and  Pseudomonas aeruginosa  [− 2.80%95% CI (− 4.26–1.34)].  Non-tuberculous mycobacteria  and  Burkholderia cepacia   showed a non-significant increase in incidence. A similar pattern of change in prevalence was observed. No change in trend was observed in infants < 2 years of age. The mean age of the first isolation of  S. aureus  ( p  < 0.001),  P. aeruginosa  ( p  < 0.001),  H. influenza  ( p  < 0.001),  Serratia marcescens  ( p  = 0.006) and  Aspergillus fumigatus  ( p  = 0.02) have increased. Nebulised amikacin (+ 3.09 ± 2.24 prescription/year,  p  = 0.003) and colistin (+ 1.95 ± 0.3 prescriptions/year,  p  = 0.032) were increasingly prescribed, while tobramycin (− 8.46 ± 4.7 prescriptions/year,  p  < 0.001) showed a decrease in prescription. Dornase alfa and hypertonic saline nebulisation prescription increased by 16.74 ± 4.1 prescriptions/year and 24 ± 4.6 prescriptions/year ( p  < 0.001). There is a shift in CF among respiratory pathogens and prescriptions which reflects the evolution of cystic fibrosis treatment strategies over time.

74. BIBLIOGRAPHY BOOKS: Hockenberry j marilyn , wilson david ; Wong’s essential of pediatric nursing; 2 nd edition: 2019; Bronchial asthma and cystic fibrosis; Elsevier; 283-302 Kyle terri and charman susan ; Eessential of pediatric nursing; 2 nd edition ; 2017 ; Bronchial asthma and cystic fibrosis; Kluwer ; 128-140 Pal Panchali ; Textbook of pediatric nursing ; 3 rd edition ; 2018; Bronchial asthma and cystic fibrosis: paras medical publisher; 80-83 Martin J Richard; Neonatal perinatal medicine;11 th edition; 2022 Bronchial asthma and cystic fibrosis: Elseiver ; 789-792. Hockenberry , wilson ; Wong’s nursing care of infants and children ; Bronchial asthma and cystic fibrosis: 11 th edition ;2019 ; Elsevier ; 423-438 Nelson. Textbook of paediatrics, Bronchial asthma and cystic fibrosis: Elseiver ; 589-592. Ghai OP. Textbook of paediatrics, Bronchial asthma and cystic fibrosis: Elseiver ; 509-515.

75. ELECTRONIC SEARCH https://www.slideshare.net/slideshow/bronchial-asthma-251870376/251870376 https://www.slideshare.net/slideshow/cystic-fibrosis-250266717/250266717

76. Thank you