Pulmonary Lymphangiectasia

Today Grant saw the pulmonologist because he's still wheezing. The good news is that the wheezing is not due to fluid in his lungs, but left over mucus from his ear infection.

The pulmonolgist also told us that in about a week Grant can stop his breathing treatment. (The bummer part about this is that he is not sure it ever helped Grant in the first place, even though we've been doing it for a year and a half.) But, we are so happy to not have to torture Grant by holding him down every morning while we force a mask on him. This will mean that Grant is off ALL medications! Yeah God!

The doctor said that we are allowed to start having play dates, going to music class etc! He reiterated to me that he would recommend Grant mostly play in open air environments and stay away from sick kids, but he said this summer is a good time to push Grant. We started today with his first play date of the season. Grant had a blast.

When I asked the doctor about the long term prognosis he said we'd still have to hole up next winter and get shots for RSV. He said that at this point it appears that Grant still has pulmonary lymphangiectasia. (See description below.) He said that we can do tests on Grant's lungs when he's 5 to see if he's outgrown it. I told him that we're praying he'll be 100% healthy by the time his little sister arrives. :)

Pulmonary Lymphangiectasia: (as described on the website littleleakers.com, dedicated to those who suffer from this disease)

Pulmonary Lymphangiectasia is a condition in which there are widespread dilation of the lymphatic channels in the lungs. The cause of Pulmonary Lymphangiectasia is unknown. The signs of PL can sometimes be seen prenatally, but most commonly postnatal. Many infants are stillborn. The newborn infants show respiratory distress.

The typical presenting symptoms include rapid breathing, and recurrent cough or wheeze. An examination of the patient usually reveals the presence of fine crackles throughout the lungs.

Diagnosis of pulmonary lymphangiectasia can be difficult. Although chest x-rays are usually abnormal, the x-rays can reflect fluid in the chest cavity or lung tissue. Chest CT scans can show patchy areas of fluid and over inflation in PL patients.

Most patients who survive through early infancy continue to have respiratory problems (wheeze, recurrent cough, and pneumonia) in the first several years of life. Evidence has shown that symptoms improved in most patients after infancy and were notably better by the age of 6 years old. The only treatment is medical support until patient is no longer in need of support. The evidence suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as in most cases and clinical findings improve after the first year of life.