December 2023 - CHILD AND CHEST HEALTH CARE

Treatment of cystic fibrosis,now available for children,Dr. Deo Kumar Jha, M.D.,Pediatrician and Pulmonologist,Ghaziabad,Delhi and NCR

Pediatrician and Pediatric Pulmonologist Dr.Dev Kumar Jha,Formerly at AIIMS,New Delhi - 29 Dec, 2023 - Professionals Guide, - 0 Comment

Cystic fibrosis is a genetic disease with Autosomal recessive pattern.

It is more commonly seen in children born out of consanguineously married couples

It was considered a diagnosis of children from western countries,till Dr Sushil Kumar Kabra,from AIIMS,New Delhi made the diagnosis,approachable and affordable,by developing an indegenous instrument to measure the sweat chloride level ,essential for the diagnosis of cystic fibrosis in children.

It primarily affects respiratory systems ,gastrointestinal system and genitourinary system.

The primary pathology is mutation of CFTR gene ,responsible for transport of electrolytes which carry water with it across the cell membrane.

The mutation,causes the secretions in respiratory tract ,gastrointestinal tract and genitourinary tract,very thick.

The sufferer,gets difficulties in clearing secretions from the respiratory airways,bacteria get stuck in the thick secretions and the child develops repeated episodes of cough,visits many clinicians with very little benefits,till the final diagnosis of cystic fibrosis is made at the centre with expertise in CF (Cystic fibrosis). Chest X-Ray at initial stage shows only bilateral hyperinflation which is easily confused with asthma.

As per the gastrointestinal system,initially there is frequent watery diarrhoea,and later on constipation and intestinal obstruction popularly known as meconium ileus equivalent occurs in older children. Some children may be born with intestinal obstruction ,called meconium ileus.

Adults may suffer from infertility due to absence of vas deference in male and tube blockage in female .

The diagnosis is based on sweat chloride analysis,and mutaion analysis.

The most common mutation is seen on delta 508 F location of gene,all over the world.

The only option for the treatment till recent years was supportive therapy, in the form of enzyme LIPASE with meals,hypertonic saline inhalation to make the respiratory secretions thin,and chest physiotherapy to induce cough and taking out the pulled secretions to make airways clean. Inhalational steroids to subside the airways inflammation and keep it in control and antibiotics to treat and prevent respiratory tract infections,known in this case as pulmonary exacerbation due to infections..

In the past few years,there have been many multicentric research which gave the medicines that regulate CFTR gene.

In a study on 506 children aged 12 years or more,ELEXACAFTOR 200mg,IVACAFTOR 150mg and TEZACAFTOR 100 mg were given 12 hourly for 144 weeks.

At the end of 144 weeks, there was significant improvement in lung functions in the form of mean rise of 14.1% in FEV1 and marked decrease in pulmonary exacerbation,0.2 per year only.

The BMI went up with mean increase of 1.61 kg/metre square.

Cystic fibrosis questionaire was also also improved.

There was marked improvement in symptoms.

Adverse effects were moderate and the main adverse effect was infective Pulmonary exacerbation,followed by cough ,headache,oropharyngeal pain and nasopharyngitis

This therapy is popularly known as triple therapy or ETI therapy.

This is very costly therapy but safe and effective with its limitation of availability.

Dr Deo Kumar Jha,M.D., Pediatrician and Pulmonologist,Ghaziabad,Delhi and NCR,www.childandchestdoctor.com,You Tube channel-Dr Deo Kumar Jha

REFERENCE: Daines CL, et al. Eur Respir J. 2023;doi:10.1183/13993003.02029-2022:

DUPILUMAB, EFFECTIVE TREATMENT FOR ASTHMA IN CHILDREN,Dr Deo Kumar Jha ,M.D,Pediatrician and Pulmonologist,Delhi-NCR

Pediatrician and Pediatric Pulmonologist Dr.Dev Kumar Jha,Formerly at AIIMS,New Delhi - 15 Dec, 2023 - Professionals Guide, - 0 Comment

When asthma is not controlled even on high dose inhalational corticosteroids with other controller with or without oral corticosteroids,it is called severe therapy resistant asthma(STRA).

If the child is diagnosed with type 2 airway inflammaton, biologics are the choice in these scenario.

There are so many biologics in the market but few are approved for children.

According to a study on 408 children with the mean age of 8.9 years ,Dupilumab was found to be consistently effective and safe.

Dupilumab was used as an injection by subcutaneous route.

It was given in the dose of 100mg,twice in a week for children equal to or less than 30 kg of weight.

For children more than 30 kg of weight ,the dose was 200 mg biweekly.

According to the study protocol,the dose was increased to 300 biweekly in children equal to or less than 30 kg of weight in mid study period.

The treatment was given for a period of 52 weeks.

At the end of the study period, common adverse effects were nasopharyngitis in 9%,pharyngitis in 6%, upper respiratory infection in 8% and eosinophilia in 3 %,

Serious adverse effects were seen only in 7 children

This study concluded that ,Dupilumab decreased the level of type 2 inflammatory biomarkers like level of serum IgE and blood eosinophils significantly.

It also concluded that ,this is the only biologic agent which reduced the number of asthma exacerbations as well as consistently and significantly improved lung function as measured by spirometry in the form of significant improvement in FEV1.

REFERENCES:

Bacharier LB, Maspero JF, Katelaris CH, et al. Assessment of long-term safety and efficacy of dupilumab in children with asthma (LIBERTY ASTHMA EXCURSION): an open extension study,Lancet Respir Med. Published online November 10, 2023. doi:10.1016/S2213-2600(23)00303-X

Tonsil removal for improvement in behaviour of children,Dr Deo Kumar Jha,MD.Pediatrician and Pulmonologist,Delhi-NCR

Pediatrician and Pediatric Pulmonologist Dr.Dev Kumar Jha,Formerly at AIIMS,New Delhi - 08 Dec, 2023 - Child Health Guides,Common Childhood Disease,Parents’ Guide,Professionals Guide, - 0 Comment

Sleep disordered breathing(SDB) is a common problem in children.

It has been ignored for years till there has been a facility to diagnose it.

Now,there is a facility for early detection of this common problem by a test called polysomnography.

Enlargement of tonsils and adenoid are the main cause of this disorder in children

The main cause of enlargement of tonsil and adenoids for a prolonged period of time is ALLERGY.

Parents are usually not aware of this problem in their children.

The common symptom is snoring in child with frequent disturbances of sleep.

It is graded by hypoapnea/apnea index(HPI) on polysomnography.

The child gradually develops behavioral changes,attention deficit,cognitive problems,day time sleepiness and later on hypertension.

There are two modes of treatment ,one is watchful observation and second is removal of tonsil.

Study was done on 458 children of the mean age of 6 years.

At the end of 12 months of follow up 394(86%),children were available.

It was observed that there was no significant difference between the two groups for the cognitive function.

There was a significant improvement in day time sleepiness and behavioral problems in the group who underwent adenotonsillectomy(surgical removal of adenoid and tonsil) ,moreover,the quality of life were better in the group who opted for the surgical removal of adenoid and tonsil.

CONCLUSION: In case of mild sleep disorderd breathing ,adenotonsillectomy is not advisable for improvement in attention deficit or cognitive problem.

REFERENCES:

Tonsil, adenoid removal improved sleep quality, some behavioral problems in children with mild sleep apnea. https://www.newswise.com/articles/tonsil-adenoid-removal-improved-sleep-quality-some-behavioral-problems-in-children-with-mild-sleep-apnea. Published Dec. 5, 2023. Accessed Dec. 5, 2023.

Is my child suffering from Asthma,Dr Deo Kumar Jha,MD. Pediatrician and Pulmonologist,Ghaziabad,Noida,Delhi-NCR

Pediatrician and Pediatric Pulmonologist Dr.Dev Kumar Jha,Formerly at AIIMS,New Delhi - 02 Dec, 2023 - Child Health Guides,Common Childhood Disease,Parents’ Guide, - 0 Comment

Know the fact, directly from expert-Dr.Dev,Child and child chest specialist

Is your child facing repeated episodes of cough-Yes/No

Is the cough more problematic during night-Yes/No

Is the cough more problematic while child is playing-Yes/No

Is the cough more problematic when the child eats or drinks cold items-Yes/No

Is the child facing shortness of breath/difficulty in breathing repeatedly-Yes/No

Is your doctor,preferring to nebulise the child more frequently-Yes/No

Is your child facing more cough and or shortness of breath while season is changing-Yes/No

Is your child having itchy skin rashes any time,in addition to cough and shortness of breath-Yes/No

Is your child having itchy nose,frequent running nose,stuffy nose,nose block more frequently in addition to cough and shortness of breath-Yes/No

Is your child takes more time for the recovery of cough and cold than other children-Yes/No

Is your child having cough and shortness of breath when there is no running nose or sneezing-Yes/No

Is any family member whether he or she lives with the child or does not live with the child ,facing symptoms of any kind of allergy-Yes/No

If the answer of most of the questions are YES, there is maximum probability that, your child is suffering from ASTHMA

Asthma is not a disease to be scared of but should be accepted and then appropriate treatment should be given to the needy child.

Asthma is one of the most common chronic disease of respiratory tract in children.

It affects 10%-30% children worldwide.

It is the most common cause of frequent school absenteeism for children.

It affects mental and physical health of children.

It gives financial burden to the family

After appropriate treatment there is negligible school absenteeism,no challenge to the physical and mental health of child and financial burden to the family is significantly reduced.

Dr Deo Kumar Jha,

MBBS(Gold Medalist)

MD(Pediatrics)

Fellowship in Pediatric Pulmonology,AIIMS,New Delhi