Brief Answer:
Home oxygen therapy, preventive antibiotics, physiotherapy and care

Detailed Answer:
Dear Mr XXXXXXX

Allow me to commiserate with you. Indeed, it is unfortunate that your son is having to bear with this much trouble! May I ask how he landed up with CLD, and why he has a stoma? Please send me these two bits of information. Also, could you upload images of his chest X-rays and the results of his last blood counts and other tests? Did he receive any special vaccines? Does he have a normal heart function and are his heart ultrasound results normal? We will continue once you have provided me with the above information. Thank you for reaching out to me through this portal.
Regards,

Dr Taher Kagalwala

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Brief Answer:
Kindly go through the full answer!

Detailed Answer:
Dear Mr XXXXXXX

Thank you for your prompt revert. I believe that his getting infection repeatedly, and the collapse of a particular lung area each time suggests that that area may have to be looked at by respiratory specialists. An endoscopy of his bronchial tree may help to further understand the problem. In occasional cases, the defective lung segment may even have to be surgically removed; however, the child's delicate overall condition will preclude any such heroic surgery at the present time.

I am indeed an India-trained Paediatrician, but I have been working in the U. K. now for over 6 years.

I think your son's medical team is very guarded about his overall likelihood of complete cure, and hence are unwilling to look beyond each day. His perpetual need for steroids indicates that his condition is quite serious. Every time he is given steroids, his immunity actually goes down further, thus making him vulnerable to more infections. The fact that he also has pulmonary hypertension and a hole in the heart, plus his constant need for diuretics and sodium supplements suggest that he still has some way to go before he is out of the woods.

Surfactant helps only within the first few days, and not after that, so that treatment is out of the question.

I did ask you about any special vaccines. In the U. K., we give preemies 4-5 doses of a special injection that protects such babies against infection with the RSV - the Respiratory Syncytial Virus. This injection is a synthetic monoclonal antibody against the RSV, and it is given monthly during the winter months, starting in October or November. The RSV is a destructive virus that can wreak havoc with the already damaged lungs of a baby like your son. In countries like XXXXXXX we do not have the potential to diagnose RSV infection easily.

As you haven't told me which country you are in, I am not able to clearly see the problem in the right context - but it doesn't matter much as the baby already has received good treatment. I am assuming the other two siblings of this trio are okay.

Finally, we do consider a baseline antibiotic therapy for babies like your son. This can help prevent repeated LRTI's.

I hope this helps to some extent. Do try and retrieve his X-rays. I am happy to take a look at them.

Discuss referral to the respiratory team.

Thank you once again.

Dr Taher

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Brief Answer:
Pleasure to meet you, Dr XXXXXXX XXXXXXX

Detailed Answer:
I work as a middle-grade by choice in Blackpool. I have seen some patients with CLD who have needed home oxygen even in their second year of life. Weight gain in a patient who is on chronic steroid therapy - hmm. Look at that with some scepticism. I would therefore not be too happy to just see that weight gain. I know alternate-day steroids do cause the least suppression of the HPA axis, but the important word there is "least" - not "none".

I am happy he is under the respiratory team. I mentioned physio in the very first line of the first message I sent out to you but must have overlooked mentioning that in the full answer. Yes, there is no substitute for good physio - and I would explore this to the limit.

I was also happy to learn that he got Synagis therapy.

It is clear that adenovirus is a destructive virus that sometimes leads to lungs that become similar to those of the lungs of an adult COPD patient.

Azithromycin thrice a week is adequate, as most issues in such cases are caused by viruses and not bacteria.

I hope that answers all your questions.

Perhaps I would put forth the following questions:

1. Ask for their experience in managing children like your son and whether they anticipate his improvement.
2. Explore endoscopy and bronchial washings for culture and antibiotic sensitivity tests - he may well have a resistant bug inside his lung -something like C.burkholdaria or the ever-invasive Pseudomonas.
3. See where we are with physio,
4. Ask the question about surgery only when he is stable/older
5. Consider bronchodilators - combinations of salbutamol and ipratropium to begin with, but other drugs as they go along.
6 Minimising steroids - this should be done as a matter of importance.
7. Home-based ventilation would certainly be of help.
8. Remember, if there is a virus, antibiotics will not be of much help. Aggressive use of inflammatory markers, full blood counts and cultures is very important before rushing in blindly with ever-more powerful antibiotics.
"If it ain't broke, don't fix it" - would be my way of using antibiotics.

Thank you.

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Brief Answer:
Private vs NHS Public care - not a good discussion

Detailed Answer:
Dear Dr XXXXXXX

First of all, apologies for not answering sooner. I was a little busy with my own work.

CLD is a condition with a guarded prognosis for infants who are still needing oxygen after the age of 1 year. In addition, he has pulmonary hypertension, a need for electrolyte supplements, and has gone through a severe adenoviral infection. The good news is that he is putting on weight and that he HAS survived all the odds and is still fighting it out!

Having said that, I think the consultant looking after him has indeed given you a realistic outlook. Now, it is in your hands as to whether you want to take him private. I assume that would still have to be in a tertiary NHS setting, since he is a high dependency care patient.

I am not aware of a "particular" setting or doctor that may be able to offer him something better. As I have said, he is receiving most of the standard care that children like him receive.

A tracheostomy is a fairly serious procedure for someone of his age, and this will help him only if they can show that there is a blockage to the clearance of secretions, or prove that he is developing a laryngeal oedema or narrowing secondary to long-term ET placement - and as this is not the case with him, a trachy is unlikely to offer much benefit.

Finally, if you are looking at simply accepting the worst and taking him home to be looked after private nurses, you will need to discuss that with his consultant, the ethics committee of the hospital, the availability of high-flow oxygen therapy at home, the feasibility of doing this if you are living in a residential apartment setting or in a crowded locality, the presence of community nurses who can come out as often as needed to continue the high-level home-based high flow therapy, and so on.

On the other hand, I don't think we should be talking about palliation as yet. I think there is a chance - perhaps less than even - that he may still pull out of this in a year or so. However, his state is such that unless something heroic is done, such as a heart-lung transplant - he may require life-long medicines and care for his debilitated heart and lungs. Before that, though, other modalities such as a lobectomy etc. may have to be considered if there is only a single segment or lobe of the lung physically destroyed by the twin assaults of the CLD and the adenovirus.

I am sorry, but this is what it is. I respect your and your family's courage and resilience through all this. Perhaps, we will meet one day. And I will get to see your lovely son, all recovered.

Dr Taher

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