Here is the latest with our beautiful 7 month old daughter Sage who has a rare lung disease: Idiopathic Pulmonary Arterial Proteinosis and idiopathic Pulmonary Hypertension with no known cause, two rare conditions in babies. We found this out a week after her 6 month birthday. When we brought her in to the hospital, the doctors were shocked that she had been living like a normal baby this long. Sage is so strong she seemed like a pretty normal baby for her first four months.
In hindsight, there were definitely signs. Sometime around 4 months she stopped gaining weight. I was staying home with her, breastfeeding on demand, as well as encouraging feeds. There were developmental regressions too. Tummy time was horrible, rolling over stopped, and being vocal and standing assisted stopped as well. My doctor sent me to physical therapy due to 'low muscle tone'. She was just too exhausted from her little heart and lungs working so hard.
The worst sign was her supposed 'breath-holding spells' which our doctor diagnosed. Sometime around this 4 month mark Sage had an incident where she got upset. Her eyes rolled back in her head and she slumped forward, and passed out, pale, blue lips. Apneic spells affect 5% of babies and are characterized as involuntary breath holding during crying or being upset causing them to lose oxygen and pass out. Over the course of the next couple months these spells had increased to 5 or 6 a day. We didn't know it was her heart failing.
I owe it all to my lactation consultant, Juli Walter. I had a visit with her to figure out why Sage wasn't gaining. She watched me nurse and noticed that Sage's breathing was unusually labored. She called my doctor and told me to see him and have him watch me nurse. That doctor visit resulted in him sending me to Lurie Children's hospital. I walked in expecting them to do some tests and possibly admit her. I had no idea it was such an emergency.
When I came in they checked her blood oxygen level. This is testing on hand and foot and should read 95-100 for a healthy individual. Sage was living in the 60s-80s and when even mildly upset desaturating as low as the 20s. Her lung disease is so incredibly rare, especially in a baby, that they really don't know much and can't really give us much information for now. Sage was born with this and because she's so strong, she made it this far.
Sage was admitted to Lurie Children's hospital Tuesday, June 6. She's had 6 blood transfusions and is on a host of medications. Her treatment for the condition includes a subcutaneous remodulin pump (similar to that of a diabetic), sildenafil (viagra), and bosentan all of which will be her long term treatment to manage the PH. She had an oxygen tube, 2 central lines and an arterial line to accommodate all of these medications and more. The first few weeks they took away all of the work for her and she was intubated, completely paralyzed and sedated.
On July 3rd, Sage decided she was over the tube and ready for extubation. During a daily re-taping of her tube, and at the right second, she jerked her head and the tube came out which resulted in major crisis, alarms and several doctors running in to remedy the situation. They decided to give her a chance and see if she could handle it before reintubating. She graduated to a BiPAP machine and eventually a nasal cannula.
Sage's throat has not closed from being intubated for so long, so she will go home with an ng tube and we hope that she will heal enough for her to be able to drink liquid again.
The next step is to wean her off sedatives, and eventually have her live on the PH medications alone to make sure she will stay stable. There is a very risky treatment for the pulmonary alveolar proteinosis for her age group so we will continue to watch that and possibly consider later if need be. Lastly, we as parents, will need a good amount of education on this new level of care for Sage.
Our doctors and nurses at Lurie Children's Hospital have been absolutely amazing. Knowing we can trust our care providers had made more bearable what sometimes feels like a nightmare. They have been so comforting and on our side very step of the way. We have learned to stay strong and live in the moment in a way that nothing else could teach us.
- Sue Gottschall
- Celia Leventhal
- Kirby Longbrake
- Pamela Hoffman
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