This blog came about due to the outpouring of love, support, and affection from many people about our son Michael. Many people want to get updated on things, see photographs, see test scans, and just generally keep up with how he is doing. While the email updates (put up on here as well for those who didn’t get them) worked well for a small number of people–it didn’t give everyone who wanted information that access so hopefully this will fill that void.

Over time I’ll pull in relevent posts from my personal blog and a “history of” Michael’s journey, but in a small nutshell for now:

Michael was born March 26, 1990 six weeks early after holding off delivery for 2 months. We were told from ultrasounds to expect him to have Achondroplasia (a form of dwarfism) because his head looked large in comparision to his body. It turned out his chest was actually small in comparison to his head and at about 10 days old he was diagnosed with Jeune’s Syndrome. He was on oxygen 24 hours until around 2.5 years and then intermitently after that, a feeding tube was placed at 1 year of age, and the right side of his rib cage was expanded when he was 5 years old. He was hospitalized frequently for both failure to thrive (couldn’t gain/keep weight on) and pneumonia. At 8 years old he was diagnosed with Severe Chronic Neutropenia (body doesn’t make white cells correctly), and he was started on G-CSF shots to increase his production of white cells which greatly helped his general health.

In January of 2007 he came down with pneumonia and his health pretty quickly deterioted having cold after cold dropping to pneumonia that seemed unrelated to his white cells. In March/April of 2007 his pediatrician noticed in a chest x-ray that his spine seemed slightly curved. In September 2007 his spine was labeled at 43 degree curved to the right, in October it was verified as 48 degree curved. November 2007 he was hospitalized with pneumonia like symptoms and despite antibiotics and multiple runs of steroids he continued to have coughing and breathing issues through to March. In March 2008 they did a flexible bronchoscopy to see if there was bacteria/infection that just wasn’t getting cleared up and found that the airways to his right lung were collapsed. We were referred to a doctor at Cincinnati Children’s Hospital for a rigid scoping and from 9/8-9/11 went there for all kinds of testing (updates about that can be found in posts on those dates). In yet another nutshell, it was found that Michael’s does not, indeed, have Jeune’s but has Shwachman-Diamond Syndrome, and that his right airway is compressed tightly shut the whole length of the airway due to the spine twisting and basically kinking the airway around the spine as it turns.

We spent from September 2008 through July 2009 going to Cincinnati at least once a month trying to optimize Michael’s health to get him through a surgery in July to straighten his spine and *hope* it would take the pressure off his right airway which we found was not collapsed but pinched closed causing his left lung to do 80% of the work. Unfortunately the morning of the surgery Michael reacted to platelet transfusions and he crashed respiratory wise and they had a hard time stabilizing his oxygen level. The amount he received was miniscule compared with the blood products that they would have to pump during the surgery and anesthesia felt we had to make a choice of “do no harm” and the surgery was cancelled permanently.

We are trying to come to terms with making Michael comfortable and keeping him as healthy as possible without doing anything aggressive, he cannot be under anesthesia for any reason. Michael is currently on oxygen to help out his left lung, has obstructive and restrictive lung disease, has a PICC line (permanent IV) to receive TPN nutrition without which he just drops weight steadily. Through it all he has amazed me by being a beautiful and strong young man–he is very precious and the world is a better place with him in it.