What is OB?

Obliterative Bronchiolitis (OB) also known as Bronchiolitis Obliterans is a rare and life limiting form of non-reversible obstructive lung disease that occurs in children and adults after injury to the lower respiratory tract.

The small airways (bronchioles) are compressed and narrowed by fibrous (scar) tissue and inflammation and this can often lead to obstructive lung disease.s

Obliterative Bronchiolitis can be misdiagnosed as Asthma, Bronchiolitis, Pneumonia and Emphysema.

Symptoms can include

  • Wheeze/dry/chronic cough
  • Shortness of breath
  • Inability to maintain prolonged exercise
  • Recurrent chest infections

Causes

  • Post transplant
  • Post infection (pneumonia, adenovirus, RSV, HIV)
  • Exposure to toxic fumes
  • Aspiration
  • Rheumatoid Arthritis
  • Collagen or Vascular disease
  • Stevens Johnson Syndrome

Treatment

Mainly supportive, using anti-biotic, physiotherapy and sometimes oxygen therapy. Steroid and other inhalers have variable results. Surgery may be an option for a small number of patients. Lung transplants maybe an option in end-stage disease. Clinical course is very variable and prognosis for any individual is often difficult to predict.

Tests for OB include

  • CT scan (shows mosaic pattern in OB)
  • Chest x-ray
  • Blood tests
  • Lung Volume tests (show hyper ventilation due to air trapping)
  • Spirometry tests (show FEV1 less than 75%) Lung biopsies

What is Bronchiectasis?

Children with Obliterative Bronchiolitis can often have Bronchiectasis too. Bronchiectasis is an irreversible dilation or widening of the bronchiole tree (the branching tubes that carry air to the lungs). The bronchi are enlarged, inflamed and collapse easily. This means that the mucus that is normally carried away by tiny hairs (cilia) gets blocked. Mucus builds up and spills over into other tubes and these are more likely to be infected by bacteria. This then causes inflammation and damage called Bronchiectasis.

Symptoms can include

  • Coughing up phlegm in large amounts everyday. This can be exhausting and embarrassing.
  • Wheezy shortness of breath
  • Sinusitis
  • Coughing up blood- haemoptysis
  • Joint pain

Treatments may include

  • Exercise – to help loosen mucus
  • Daily Physiotherapy- coughing exercises
  • Anti-biotic to treat infections. Anti-biotic given prophylatically (you may hear this term used) help to protect against and slow the course of an illness or disease.
  • Nasal sprays for nose or sinus infection

Tests include

  • CT Scan
  • Lung function tests
  • Blood tests
  • Sweat tests
  • Bronchoscopy

What is Interstitial Lung Disease?

Although Breathtakers is intended primarily for suffers of OB much of the information on the support pages is generic and therefore may prove useful for sufferers of other rare lung disorders. As with OB there is often little information available and so hopefully this site may prove useful.

What are some of the forms of chILD?

  • Surfactant Protein Deficiency (several subforms of this exist as well)
  • Chronic Bronchiolitis
  • NeuroEndocrine Hyperplasia of Infancy (NEHI) also known as Persistent Tachypnea of Infancy
  • Hypersensitivity Pneumonitis (HP)
  • Connective Tissue Lung Disease
  • Bronchiolitis Obliterans (OB)
  • Bronchiolitis Obliterans Organizing Pneumonia (BOOP)
  • Pulmonary Interstitial Glycogenosis (PIG)
  • Growth Disorders
  • Development disorders such as Alveolar Capillary Dysplasia (ACD)

Does ILD affect adults?

Adults can also suffer from ILD. The most common and severe form is interstitial pneumonia/ idiopathic pulmonary fibrosis, which has not been found in infants and young children. The term “Idiopathic Pulmonary Fibrosis” however means scarring of the lungs for unknown reasons, which can occur in children.

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