14 year old with abnormal PFT - Low DLCO, etc.

[LisaSD]

I noticed my 14 year old daughter getting short of breath about 6 months ago and thought she just needed more exercise although her BMI is 18% and she does go out and play a lot.  However, the breathing got worse so I took her to the doctor.  They ordered a PFT to be done earlier in January.  I got the results about a week ago.  They are:

1.  There is a proportionate decrease in FVC and FEV1 with a preserved FEV1/FVC ratio consistent with a Restrictive Lung Defect.      
2.  Reduced total lung capacity measured by Plethysmography suggesting a mild restrictive pattern.    
3.  Elevated residual volume suggests Air Trapping.    
4.  DLCO is Moderately Reduced.

Her adjusted DLCO value was 54% of the predicted value, her TLC was 76%, the FVC was 79%,  and the FEV1 was 81%.  I took her to the ER the other night because she couldn't breath and her chest xray looked okay and the EKG appeared normal.  Can anyone offer any ideas as to what might be wrong?

[National Jewish Health]

The pulmonary function tests (PFTs) values are consistent with the diagnosis of a restrictive lung disorder, an infrequent occurrence in adolescence.  That her chest x-ray was interpreted as normal is very good news, but given the PFTs values and her worsened shortness of breath, it is imperative that she be seen by a lung specialist, also known as a pulmonologist, to have further evaluation, including a CT scan of her lungs.  You should request this consultation without delay.

There are a number of restrictive lung diseases that fall within the category of interstitial lung disease and a normal chest x-ray in the early stages of the disease is common.  In addition, while >99% of people with asthma have PFTs that show an obstructive pattern to air flow, a small percentage of people with asthma will show a restrictive pattern.  It is very important to make the correct diagnosis so that therapy may be initiated and prevent permanent lung damage.

One final note:  There is a medical condition called sarcoidosis that usually, but not always, shows up in the lymph nodes within one’s chest.  There is an uncommon form of this disease, called endobronchial sarcoidosis that occurs only within the airways, and can cause obstruction and/or restriction.  In addition this condition frequently has a feature called bronchial hyperreactivity, a feature most commonly associated with asthma, a disease with which endobronchial sarcoidosis can be confused.

These terms will probably be unfamiliar to you but your daughter’s doctor will understand and be willing to arrange for the lung specialist consultation.

Please let us know what transpires.

Good luck.

[LisaSD]

The CT came back with normal findings.  The only thing noted was her 23 degree Thoracic Scoliosis. Any ideas on what could cause the PFT results if the lungs are normal?