Gabriel spent the night with his dad and Lucie. It was a stable night, although he wakes up regularly and prefers to stay in his arms rather than in bed.
This morning, the long-awaited pulmonologist finally came by!
He manages 6 patients with the same pathology in the region. In reality, this patalogy is very rare and is mostly found in southern hemisphere countries (we should have played the lottery, we’re pretty lucky…).
Summary of my notes:
1. Severity of pathology & Phase
- Severity: too early to say, but not among the most serious.
- Disease stage: May still be inflamed, but probably already fibrotic.
2. Examinations
- CO₂: A gasometry of the day demonstrate that Gabriel is making a respiratory effort and is unable to evacuate CO2.
- Scanner: not immediately indicated, but probably soon.
- Future: consider immune therapy.
3. Treatments
- Maintain azithromycin.
- Nutrition: promote weight gain (Infatrin).
- Corticosteroids: consider new pulsotherapy (dose to be confirmed).
- Infection prevention: to be discussed with allergist (possible vaccines).
- Ventilation: introducing NIV
- Reflux / aspiration: Continue anti-reflux.
4. Respiratory physiotherapy
- Continue techniques.
- Frequency: 2x/day min.
- NIV: to be introduced
- Not necessarily a vacuum cleaner
5. Swallowing / ENT-Fono
- Risk of reflux / broncho-aspiration → speech therapy to avoid the risk
- Probe withdrawal in the future.
6. Homecare and hospital discharge
Exit criteria
- Complete drug withdrawal (remaining lorazepam, hydroxyzine, risperidone, neuleptil, neozine)
- Apartment equipped with various machines
- Home team: physio, fono, pneumo…
- Objective: avoid re-hospitalization.
- Written report from the expected Dr. (plan + equipment + procedure).
7. Outings & travel
- Chat, outings, trips: We forget
- Plane: to be re-evaluated according to progress and if necessary to be taken.
Arterial gasometry and excess CO2 explain Gabriel’s recent fatigue.
So we weren’t crazy when we told the team and he told us “It’s normal”.
A treatment adjustment is planned and the use of NIV (non-invasive ventilation) has been recommended to help him open his lungs better.
At the same time, we had an online consultation with a pulmonologist and a physiotherapist from São Paulo. Their analysis confirms obliterative bronchiolitis, but according to them, the hospital is not administering exactly the right treatment in the right doses, and this is more tiring than anything else (particularly with regard to corticoids)
They will send us a report with precise recommendations for the hospital.
Summary of my notes:
- Aspiration: don’t aspirate too often, as this also removes air and weakens the lungs. The aim is to rehabilitate and keep the airways open, not just to remove secretions.
- Aerolin (salbutamol): ineffective in obliterative bronchiolitis, as this disease does not respond to beta-2 as in asthma.
- Pulmicort (inhaled budesonide): not very useful, as the particles do not penetrate deep enough into the bronchioles, remaining mainly in the throat and causing discomfort and coughing.
- Oral corticoids (prednisolone): dangerous long-term (weakens muscles, immunity, bones, increases risk of infection). Not suitable for continuous treatment.
- Pulsotherapy (high-dose corticosteroids over a short period): this is the indicated treatment, but the dose must be carefully adjusted in mg/kg and efficacy monitored.
- Endocrinology: after so many corticoids, Gabriel’s hormonal axis is probably blocked → need to assess and replace with hydrocortisone + plan “stress doses” in case of exams or surgery.
- Recommended inhalation treatment: use Seretide-type combinations (combining corticosteroid + long-lasting bronchodilator), which are better suited to keeping bronchioles open.
- Respiratory physiotherapy: essential, focusing on muscle re-education and keeping the airways open.
- The overall aim is to reduce inflammation, avoid after-effects, train the respiratory muscles and enable the lung to compensate with the areas that are still healthy.
Tomorrow we’re going to try and tune everyone up.
Another day in emotional yo-yo mode, but despite everything, the specialists confirm that there’s still hope that in a few years’ time he’ll be able to lead an almost normal life, even with a fragile lung.
So we’re holding on to that hope. ❤️
