[CIS PIDD] [cis-pidd] XLA, Bronchiectasis, Chronic sinusitis

Fri Apr 14 10:56:39 EDT 2017

Aggressive airway clearance therapy (ACTA) preceded by a mucolytic would be the anchor of his bronchiectasis therapy. In non-CF bronchiectasis dornase alpha does not help and may even make things worse. 7% hypertonic saline might be a better choice.
You might consider a bronch with BAL to determine if any unusual bugs are being missed. An eight year old’s sputum sample is some times more spit than sputum.
You might consider a longer course of oral antibiotics or even mimic the CF lung disease type protocol and bring him in for 14 days of IV antibiotics with a broader coverage and ACT.
More sinus surgery might not help especially as you have established bronchiectasis to explain his symptoms.
Hope it is of some help.

Javeed Akhter, M.D.
Clinical Professor of Pediatrics,
Rosalind Franklin University of Medicine and Science
Section Head
Pediatric Pulmonology
Allergy and Immunology
Director
Cystic Fibrosis Care Center
JMF Immunology Referral Center
Advocate Children’s Hospital, Oak Lawn, IL

From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Thursday, April 13, 2017 11:10 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] XLA, Bronchiectasis, Chronic sinsusitis

Santhosh,
1. You don't say what you are doing for chest PT or how bad the bronchiectasis is. I would have him use a flutter valve at least twice a day but a CPT vest would be better. You might consider a pulmonary lavage. I would push his IgG trough to at least 1000.
2. What does his sinus CT look like at this point.
3. The yellow mucus is probably coming from the nose/sinuses retrograde through the NL duct. How often does he use saline nasal irrigation? Have you ever added antibiotic to the nasal lavage fluid (e.g., Cipro 500mg/500ml)?
Richard Wasserman
Dallas

On Thu, Apr 13, 2017 at 10:48 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:
Hi Everyone,
Any thoughts regarding this case is greatly appreciated.
  I have a now 8-year-old male with XLA (BTK gene mutation confirmed) on IVIG replacement.  He has bronchiectasis in the lower lobes, chronic rhinosinusitis status post sinus surgery x 1. His trough IgG level have been >800mg/dl.

My questions are:
1. Pt has had chronic cough likely related to his bronchiectasis and his chronic rhinosinusitis and we have been unsuccessful in treating it in spite of doing chest physical therapy, asthma controller medication, sinus surgery, saline rinse, Tobramycin rinse. Pt has been treated with multiple courses of antibiotics in the past with no significant relief of symptoms. ENT is recommending one more sinus surgery but I am hesitant as the first one did not help. His direct sinus cultures have not yielded much except for occasional scant growth of H.Influence.  Anything else we could do to help this little guy.

2. He also has this weird history of yellow mucus coming from his eyes on and off with evidence of conjunctival injection. This discharge is more visible when he sneezes and coughs. He was checked by an ophthalmologist who ruled out lacrimal duct obstruction. ENT thinks this is related to his chronic sinusitis and the constant yellow drainage he has in his nose. Any thoughts?

Thank you,

Santhosh Kumar, MD, FAAAI
Assistant Professor
Children's Hospital of Richmond at VCU
VCU Heath
Richmond, VA
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