Gemma is 6 months old (4 months corrected) and weighs over 13lbs.
After the last update Gemma had a series of lung collapses so we treated her with IV antibiotics in case she was developing pneumonia. One of the doctors suspected that it was her 'underlying condition getting worse' and that she was outgrowing her strength. The collapses went back and forth within a two week period, clearing up too quickly to be an infection. We braced for the worst but it all of a sudden went away and she has done extremely well since then.
We got the results back of Gemma's muscle biopsy. The results showed very little muscle, it was described as fatty tissue with strands of muscle fiber and this would be in keeping with an end-of-stage muscle deterioration process. They were unable to do any testing with the sample due to the lack of muscle and a diagnosis wasn't found. It is unknown how Gemma appears to be getting stronger and having more movement with such little muscle! (they said perhaps she has more muscle in other locations of her body).
After the scare with her collapses I was able to convince the hospital to provide us with a bed to bring Gemma out on so that we could interact with her better, cuddle her and help with her physio. We also got a tumbleform chair for her to sit up in for her to have more head movement and look around. After a lot of convincing we were able to organize with the NICU, PICU and management an unprecedented trip outside with Gemma on a portable ventilator to the adjacent courtyard! It was such an enjoyable experience for Gemma to feel the sun and breeze, and to listen to birds chirping.
Gemma has been doing really well, very active and alert the whole day, having more movement and interaction. She has been smiling, scrinching her nose, turning her head, rubbing her eyes when she is tired, gripping and swatting at her toys and developing more and more of a personality every day. We have been having a lot of wonderful quality time together.
In the past couple of meetings we have been discussing what our options are and what the next step should be in Gemma's care. We are limited with options as Gemma can't remain in the NICU with an ET tube (tube connected to the ventilator that goes down her mouth and throat). It is becoming a bit dangerous as Gemma likes to hold on to her tubes and as she grows there is more risk of accidental extubations.
Some of the specialists worry that Gemma will lead a short and difficult life, and have therefore encouraged 'end of life' care. We have stressed that it is something we can't do at this time because we see her progress everyday and know that she isn't at that stage. The neonatologists and nurses support whatever path we want to pursue as they see the value in bringing Gemma home and doing what is best for our family. We are faced with very challenging decisions as no matter what path we take it requires a decision and intervention.
If we move forward then it could mean giving Gemma a tracheostomy (a permanent airway that requires surgery), and g-tube, and either remain in hospital or to take her home. We have been in contact with other families who have been in similar situations to learn more about what life is like for their child and family at home with a trach and all of the care that is involved. All situations are different so it is hard figuring out what it would mean for Gemma with all of her conditions but we are in the process of learning as much as possible before making a decision.
In the middle of trying to sort things out Gemma has of course presented herself with another condition that there is concern about. The ventricles in her brain have been increasing in size and a neurosurgeon came to evaluate her. She appears to have ex-vacuo hydrocephalus, the CSF (cerebral spinal fluid) is enlarging the ventricles as the brain is not growing at a normal rate, this normally regulates but sometimes there is a bit of a blockage (caused by birth trauma). If the fluid doesn't drain normally then it enlarges the ventricles, which enlarge the head. Babies heads are not fully formed so there is room for expansion but it can still be dangerous. If the ventricles continue to enlarge then they want to consider giving Gemma a 'shunt' that would be inserted semi-permanently inside the head and drain to her stomach.
We are keeping an eye on things over the next while to decide how it is best to proceed.
Thank you for keeping us in your thoughts and prayers,
Love Amanda, Charlie & Gemma
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