Monday, October 24, 2016

Restrictive Lung Disease? At Eleven Years?

Max met with his pulmonary doctor for the first time last Friday. It was also Mimi's 6th birthday.
I thought all would be fine so I didn't mind it was her birthday morning. Max's myopathy has a mild to severe lung problem at eleven, we would think he would be fine now.

We headed in, Max playing on his DS. Hanging out in the waiting room. I'm drinking a coffee. We've gone to over 100 appts and therapies in the last year, so we are very used to all this.

The therapist takes Max back and begins the pulmonary testing. Max tries to do what she says but there are a lot of directions, and that's hard for him. (part of his condition). So she tries again and again. At first Max can only breathe out 44% of what of it expected. She tries a few more times...better. With Max trying his absolute best...he scores in the 60's and 70's. As you may imagine, I have no idea what this means. Do you? So we head off into the patient room to meet with the doctor.

The doctor comes in and starts getting a medical history. For the first time really, since the genetic appt over 14 months ago, I go into detail about the odd recurring bouts of pneumonia and bronchitis that Max had in First Grade. He responds saying that Max isn't able to expel all his breath and so he is sure that 4 years ago, Max would get a virus at school. It would then never totally clear, and he would keep getting bronchitis type illnesses. Back then. Four years ago. Because of his muscular dystrophy. (I think back to Max being exhausted and trying to keep up with school work. About the meeting when they thought he may have ADHD, which didn't make sense to me.)  So, in addition to him fighting through his day with weakening muscles...he was fighting ongoing infections in his lungs because his lungs weren't strong AND NO ONE HAD A CLUE!

He said that he wants to quantify the results he had with the therapist, by sending him to Hershey Pulmonary testing and putting him in a booth type room that tests what is happening with the muscles in his chest. He said he could tell from listening to his chest as well, that he was not getting rid of all of his air. 

He said that he is at risk for infections in his lungs. He said he thinks he has Restrictive Lung Disease. He put him on Flovent 2x in the morning and 2x at night. He ordered us a new Nebulizer and more Albuterol. He said that Max may need the cough assist machine and the VEST. (the Vest is what the Cystic Fibrosis patients use.) It vibrates their chest to expel any mucous. 

Max was tired just from the pulmonary testing. My mind started to go into a blank sort of shock. I had interned in my senior year of college at the Cystic Fibrosis Foundation in Harrisburg after two of my friends has died of it. On the doctor's jacket it says, "Cystic Fibrosis." So he's the CF dr... I know about that world. I don't want my son to need anything like that." I think.

I asked him, "So...what do we do if he has a cold...or a bronchitis like cough?" I said, "Should we call his primary doctor because I would think he would be worried for Max." and he replied with a laugh, "You need to call me. You would need to see me." He then told me what days he is at the Lancaster office and what days he is at Hershey.

The therapist came in and brought in an inhaler sample with a spacer. She taught us how to use the spacer. The doctor ordered the nebulizer machine and scheduled his follow up for in 2 months or sooner if needed. He said that Max needs a chest x-ray also.

I came home and looked up the VEST and it's between $4,000 and $8,000. It comes in a duffle bag, so you can take it wherever you go.

We are so thankful for our insurance. I am shocked by all this. I thought this was years and years away.

We have started the Flovent with the spacer. The notes came back too from the doctor. Depending on the results of the next two or three tests, Max may need a BiPap, Vest, and Cough Assist.

We also need to understand that Max goes to Hershey for colds/bronchial symptoms now. 
It's life changing, for sure. And just in time for winter.

I am so glad that he is homeschooling and that we are used to it. I also am relieved that Mimi is homeschooling too. Can you imagine if she came home with every virus from school? I cannot even imagine.

UPDATE November 4, 2016: The pulmonary test is back and confirmed that Max has a mild Restrictive Lung Disease due to his muscle weakness. His test matched what he showed during his office visit.
They are glad that the sleep study is scheduled for early January. May need extra oxygen during sleep they suspect.
Still the same protocol for him right now. Flovent and use Albuterol every two hours with colds. - call the office if he has a cold in case he needs supplemental oxygen/more intensive treatments.

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