NICU Fundraiser

Last week I started a fundraiser towards BC Women's NICU (Neonatal Intensive Care Unit), where my daughter Gemma has been for six months since birth.  As you may not know the NICU is a part of BC Women's Hospital and not BC Childrens, even though the building is connected they are a seperate foundation.  Most donations go towards the Childrens hospital so the Womens side (NICU) has less funding.

I would like to thank those of you helping us with our fundraiser.  I have been touched by so many people; friends, strangers, distant reletives, NICU parents... people that barely know us who are trying to support our cause. I would also like to thank my mom's work (Save-On-Foods) who has organized two 'Spin for Kids' events where they have donated 100% of the proceeds ($1100) towards our fundraiser.

If you are interested in helping please e-mail me at cabostik@hotmail.com with your address and I will mail you a stamped envelope.

Thank you everyone for all of your support! 

Love Amanda, Charlie & Gemma :)

August 21st, 2010

Hi everyone,

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

July 17th, 2010

 

Gemma is 5 months old and

weighs 10lbs 6oz.

In the last update Gemma had an accidental extubation and we felt that her new tube was causing her irritation and secretions. She had changed so much and this continued for a few weeks, where she was fussing and tiring out. Then a week ago she had another accidental extubation but right after Gemma seemed to be doing much better! so we think our suspicion of the tube was correct. None of the RT's agreed but it was too coincidental.

Gemma has had a good week of being a lot more awake, alert and content.

On July 14th we had Gemma baptised.  The service was performed in her room at the hospital with Charlie, my mom, dad, sister, grandma and our chaplain friend Philip. Gemma wore the dress that both my sister, my niece, nephew and myself wore, which was from my great grandma. It was a nice occasion and we were even presented with a baptism certificate and candle.

The next day on July 15th (also our wedding anniversary) there was an opening for Gemma to go in for a muscle biopsy, we were hoping to get more information before making a decision but the next available date wasn't until September. We went ahead with the procedure and Gemma went to the OR to have a section of her muscle removed for testing. It was a difficult decision but it is the only way that we are going to find out more information about her condition.

Gemma has recovered quite well and we are hoping and praying that something positive can come out of it so that we can have some hope for her future. The neurologist believes that the prognosis will remain the same (that her muscles will weaken over time) however if the muscle is different that they suspect then it could alter the prognosis. After the biopsy we were told that Gemma didn't have very much muscle but they should have enough and we should know some results within a few weeks.
It has been an exhausting rollercoaster for us not knowing what the future holds for Gemma.

Thank you for all of your thoughts and prayers,
Love Amanda, Charlie & Gemma

July 3rd, 2010

Hi everyone,

Gemma is now four and a half months old and weighs over ten pounds.

A few weeks ago we had Gemma on another ventilation weaning plan and she was able to be weaned to minimal settings on the ventilator so we extubated her and put her onto a new type of machine using a face mask. We were hoping she wold be successful but unfortunately she failed after a few hours and had to be reintubated. We are not sure why she failed but the new machine is built for adults and there isn't a version for infants that can support her properly.

Gemma had been having a good five weeks of being alert and active throughout the day and having more movement, having good oxygen saturaion of her blood, and not requiring much suctioning. She was moved into a private room are we were able to bring her out for baths and cuddles and be quite interactive she was very content. Then last Sunday Gemma accidentally extubated herself and required three attempts to be reintubated (with the use of drugs and a paralyzer). Throughout the week she has had a lot of secretions, been quite lethargic, and required frequent suctioning. This may all have been caused by the reintubation but nobody is sure why that would be the case, possible inflamation or another side effect. It is hard to see her go from doing so well and being so active to being like this.

We had a care conference on Tuesday regarding Gemma's future care plan and we were unexpectedly disappointed to hear that the neurologist believes that though Gemma appears to be getting stronger right now, she will outgrown her muscle strength at some point which will lead to a lot of difficulties in her future. We were not given this information before and the doctors aren't sure if providing her with a tracheostomy would be beneficial. We were given a few days to think things over and follow up with a smaller meeting on Friday in which we discussed what in order to move forward in making a decision we would need more information. We requested a second opinion from another pediatric neuroligist (hopefully one with congenital muscular disorders - as the specialist we have now has only seen these type of disorders in older children, they are saying cases like Gemma are extremely rare). We also discussed that we would want more information about the pros/cons of a muscle biopsy (what it can tell us) and to find out about tracheostomies (procedure, benefits, risks) and learning more about what the Canuck Place as to offer.

It has been extremely difficult because we went from feeling hopeful to be placed to having to make a decision about what is best for Gemma's future. Receiving new information has convinced some doctors that 'end of life' care might be the best. But it is very hard not having a diagnosis and to hear this about a baby who has come so far.

It is the most difficult position to be in to see your child grow and get stronger, but have doctors feel that her underlying conditions will be limiting to her later in life. We really don't know what to do but we are hoping that more information will help.

At this point we are focusing on how Gemma is doing, with her recent incident we are waiting to see if this is an indication of things to come or if this is just her recovering from the reintubation.

We really don't know what to do but we are asking for your continued prayers.

Love Amanda, Charlie & Gemma

June 17th, 2010

Hi everyone,

Gemma is 4 months old (2 months corrected) and weighs 9lbs 7oz.

Gemma is growing bigger and stronger by the day. It is hard to believe how far she has come and all of the difficulties she faced. We know that she will have many challenges ahead but we are taking things day by day.

Gemma remains on a ventilator but we have been weaning her settings and she has been doing quite well. We have been told that with her brain and muscular conditions that she shouldn't be as far as she is but she is proving to them that she is a fighter. On Monday they are going to attempt another extubation, we are extremely nervous about it but hope it goes well so please say a little prayer for her.

Here is a list of Gemma's progress:
-Longer alert periods.
-More range of movement (she can tilt her torso and head, pull her legs in, lift her legs against gravity, spread her toes and even do little kicks!). Physio is considering casting her legs but we might have them hold off.
-She has moved down to just 'pressure support' on the ventilator, which means that she is initiating all of the breaths herself (she needs the ventilator for her pressures - to keep her lungs expanded - but there is a machine that she might be able to graduate to that does this without being intubated).
-She has been free from all conditions (edema, pneumothorax, chylorthorax...etc) and all IV's and lines for over a month and a half.
Gemma is the third oldest baby in the NICU of over 50 babies, the oldest above her are 8 & 12 months! we are hoping and wishing for the day that it will be our turn to go home.

Thank you for all of your continued thoughts and prayers.
Love Amanda, Charlie & little miss Gemma Violet

June 7th, 2010

Gemma is almost 4 months old and 9lbs.

Things have been going better than last month.

Gemma has been doing well and we were able to get her down to minimal ventilation settings and did better than expected so we trialed an extubation but unfortunately she failed.

Gemma is able to initiate all of the breaths but she is very pressure dependant so we have her on 'pressure support' and have a new plan to wean her rate. The doctors don't know if she will ever be able to breath on her own but I am pushing them to make every effort to see what she will be able to do before making any decisions.

Things can be extremely frustrating at times when you don't feel like you have a say over your own child.  I feel that this next wean is pushing her too quick but only Gemma will be able to tell us what she is ready for.

Charlie and I went in for our EMG test today to see if we had any underlying muscular/nerve conditions to help figure out Gemma's condition but everything was 'normal'.

We are waiting on her DNA results from Germany to see if they can figure out which genes are affected, which might give us possible direction towards her diagnosis. Gemma's condition remains undiagnosed and we may never find out what she. Genetics think she has a rare/dormant/hereditary disorder where an abnormal chromosome on each of our sides was passed down through generations until we (two people that were carriers) came together to produce a baby that (out of 1 in 4) could get it.

Gemma has been doing quite well, she has no IV lines, no medications (except for her suspected reflux), she is moving more, having long active and alert periods, and having more range of motion in her legs.

Thank you for your thoughts,
Love Amanda, Charlie & Gemma

May 9th, 2010

In the last update I was happy to be able to write about Gemma's progress, it seemed like we were getting so far and doing well. I am disappointed and sad to have to write that things haven't been going very well.

In the last week and a half Gemma has been having a lot of 'de-sats', where her vitals have been fluctuating and she has been requiring oxygen. She is normally on 'room-air', which meant that her lungs were healthy and she was able to do more work on the ventilator. At first we thought it was a bit of a food intolerance or reflux but it continued and her CO2 increased so we ended up having to take a step back with her ventilation and put her on the old mode that does most of the breathing for her. It helped give her a break from whatever she was struggling with and she was able to go back to room air intermittently but lately she has been requiring oxygen again.

Gemma appears to have a bit of pneumonia but she seems to have had it a while and it isn't affecting her white blood cell count or haemoglobin. The last few days she has been looking more comfortable so perhaps it was a bit of exhaustion from doing a lot of work on the other mode of ventilation, combined with a few other changes.

This past Thursday someone from Genetics came by to have me sign consent for Gemma's DNA to be sent to Germany for testing to see if they can help give her a diagnosis. The DNA was already in storage so they didn't have to perform any tests on Gemma. The reason it took a while for the DNA to be sent off is that they had to get funding, almost $6,000 that has been approved by MSP. We are grateful that Genetics followed up and are trying to help us work towards a diagnosis.

Just as we were feeling hopeful we were blindsided on Friday with devastating news about Gemma's MRI scan. It shows that some original brain trauma that they weren't concerned about has actually gotten worse and that her brain isn't growing at a normal rate. We were previously told about things she *might* not be able to do with a Neurological Muscular/Nerve disorder, without a diagnosis they weren't sure, but with the MRI they have a lot more 'concrete' evidence of what they believe she won't be able to do, which is quite extensive. They expressed serious concern as she already has other underlying conditions and this makes things a lot more complicated. Her care seems to be put on 'hold' and their recent pro-active attitude has drifted towards an overall feeling of uncertainty about her future and talk of palliative care.

We have asked to speak further with Neurologists next week to see if we can find out more information to give us a clearer image of what her future would be like and set up another care conference to discuss what will be the next step.

We appreciate your support and ask you to keep us in your prayers.
Love Amanda, Charlie & Gemma