Update on Zakary...Post #57

[Cajuncowboy]

It is Spinal Muscular Atrophy, Type 1, and it will be fatal. They are trying to determine how much time he has left.

Rich asked me to thank all of you for the thoughts and prayers. I will keep this updated as I learn more.

I'm not going to stop praying for him.

 

[the kid 05]

It is Spinal Muscular Atrophy, Type 1, and it will be fatal. They are trying to determine how much time he has left.

Rich asked me to thank all of you for the thoughts and prayers. I will keep this updated as I learn more.

is it one of those 90% chance of being fatal things? i sure hope so even though the odds will be stacked their is hope

poor little guy,

 

[silverbear]

It is Spinal Muscular Atrophy, Type 1, and it will be fatal. They are trying to determine how much time he has left.

Rich asked me to thank all of you for the thoughts and prayers. I will keep this updated as I learn more.

Aw, crap... I don't know what else to say...

In the last 24 hours, I learned my Uncle Jim passed away out in Oregon, some 2 months back... I also learned that my Uncle Del down in Florida had a stroke in early March, and is in a rehab center right now, relearning how to speak... yeah, my family's kinda kept me out of the loop on a few things...

And y'know, none of that bothers as much as this does... Uncle Jim was in his mid-80s, and had been suffering from Alzheimer's these last 3 years, in a nursing home... in a younger day, he was a wild man, as funny a guy as you'd want to meet, and I hated the thought of him in a home, his faculties fading away... and Uncle Del will be OK (unless he has further setbacks)...

But a little kid, going through all this... it doesn't seem right...

I hurt for your buddy, Hos, as I'm sure you do...

 

[zrinkill]

All my Prayers for that poor child.

 

[needforspeed]

is it one of those 90% chance of being fatal things? i sure hope so even though the odds will be stacked their is hope

poor little guy,

According to the FMSA website, Type I patients generally have a lifespan of less than 2 years. During that time, they have to be on equipment to help them with breathing because their lungs don't develop properly.

The following is taken directly from the FMSA website:

Children with SMA who have difficulty swallowing are at risk for aspirating when eating. Sometimes the child may aspirate his/her own secretions. The child may choke while eating and may also experience weight loss as swallowing becomes more difficult. Assistive feeding may be necessary. Two possible options are:

1. Nasogatric Tube (NG-Tube): a surgically placed tube through the nose that goes directly into the stomach.
2. Gastrostomy Tube (G-Tube): a surgically placed tube through the skin that goes directly into the stomach.

Because a child with SMA Type I has difficulty coughing, contacting a respiratory therapist is very important so you can be instructed in chest physiotherapy (CPT). CPT is a method of clearing the lungs of accumulated mucus by using positioning and clapping on the chest to assist in loosening secretions. Saliva can settle in the nasopharynx causing a faint gurgling sound. Often the secretions or mucus need to be removed by the use of a suction machine. Blowing raspberries and bubbles encourages respiratory strength.

Individuals with SMA can also benefit from the use of a cough assist machine. The CoughAssist™ achieves this by applying a positive pressure to the airways to inflate the lungs, and then rapidly shifts to negative pressure to pull the air out of the lungs. The rapid shift in pressure produces a high expiratory flow from the lungs, simulating a cough. This technique, referred to as "mechanical insufflation-exsufflation," avoids airway damage while clearing the lungs of secretions. The device offers patients greater comfort and quality of life without the use of invasive procedures and equipment. Patients as young as 4 months have been able to use the CoughAssist™ successfully.

Respiratory distress can be monitored by measuring the level of oxygen saturation in the blood using a tool called a pulse oximeter. A small clip or tape with a red light and a sensor is placed on the patient’s finger or toe to determine the oxygen saturation. Children with SMA Type I usually require breathing support while sleeping. Some children require more breathing support, especially with colds. There are several options to consider.

1. BiPAP (Bilevel Positive Airway Pressure) uses a nasal mask with a cap, which fits over the head to hold it in place over the nose. BiPAP provides a higher volume of air into the lungs during inhalation and inflates the lung greater than what the person can do on their own. During exhalation, the BiPAP pressure drops so that air can passively leave the lungs. The BiPAP machine can sense when the person is taking a breath and give the breath in synchrony with the individual. A respiratory rate is also set so that the BiPAP gives a minimum number of breaths per minute. The person can breathe above that rate and the BiPAP will deliver more breaths. CPAP (continuous positive air pressure) should never be used in patients with SMA.
   
2. Negative Pressure Ventilation refers to providing breaths into the lungs using a large chamber or tank that encircles the chest similar to the old Iron Lung. The chamber is connected to a vacuum pump that takes the air out of the chamber and, as a result, the chest wall expands to bring air into the lungs. A Port-A-Lung is an example of a negative pressure ventilator. It can be set to deliver a specific number of breaths per minute and a vacuum pressure.
   
3. Mechanical ventilators or respirators come in a variety of models. Mechanical ventilators are more complex, but also allow for control of more variables. The ventilator can be set to deliver a specific size breath at a set number of breaths per minute. Mechanical ventilation can be delivered with a nose mask, mouthpiece while awake, or through a tracheostomy tube. A tracheotomy is placement of a surgical hole in the neck to the large airway (trachea) that a tube can be inserted. A tracheostomy tube bypasses the mouth and vocal cords and goes directly from the skin to the trachea (wind-pipe). A respirator or ventilator is connected to the endotracheal tube or tracheostomy tube.

Source--http://www.fsma.org/booklet.shtml#whatis

My heart goes out to his parents. I'm not altogether sure that I could handle something like this.

 

[Zaxor]

It is Spinal Muscular Atrophy, Type 1, and it will be fatal. They are trying to determine how much time he has left.

Rich asked me to thank all of you for the thoughts and prayers. I will keep this updated as I learn more.

this just sucks I mean it just really sucks...sucky suck I don#t know any other way to say it

 

[Hostile]

I talked to Rich some more last night. Confirmed this is Type I Spinal Muscular Atrophy. The body produces a protein (that I think he called SMN) that is supposed to fight this stuff. Zakary shows no traces of this protein. I asked if they can't manufacture the protein then and give it to him. They are researching this. It will prolong his life, but not save it.

Basically I gather that this disease starts in the spinal column and slowly shuts down bodily functions. That is why he is being fed through a nasal tube and why he was on a ventilator.

While I was at the hospital with them the baby could only blink his eyes, smile at Rich, and wiggle on tiny foot. As the nerves die off (atrophy) they even quit telling the body to breathe, swallow, etc.

They are trying to get the baby into some test programs but those programs have to accept him as well. Again, they've been told this will prolong his life but not save it at this barring a miracle.

I'm holding out hope for that miracle. Thanks everyone for your thoughts and prayers.

 

[Danny White]

It is Spinal Muscular Atrophy, Type 1, and it will be fatal. They are trying to determine how much time he has left.

Rich asked me to thank all of you for the thoughts and prayers. I will keep this updated as I learn more.

That's horrible news Hos. We will keep him and all those who love him in our prayers.

 

[burmafrd]

There is nothing worse then this. A child. You really have to weigh quality vs quantity of life here. How much pain. A terrible thing in any event.

 

[Boom]

What a heartbreaking situation. I'm definitely going to hug my kids a bit longer tonight.

 

[phillycard]

Not much to say Hos. Keep your head up man. I'm still wishing all of you the best.

 

[Hostile]

My work raised $495 for Zakary. Rich is coming by sometime today to pick it up. They tried to take Zakary off the ventilator Friday and the results were nearly fatal. He won't be going home for whatever remains of his all too short life.

Thank you all for your prayers and thoughts once again.

 

[Hostile]

Rich just left. The donations overwhelmed him. They really need it.

A lot of decisions are ahead for them, including whether they want the hospitals to take extraordinary measures to save Zakary if it is needed.

He is holding out hope for a miracle. There are rare cases of a child surviving beyond 2 years with this disease, and one known case of 8 or 9 years according to their doctor. He thinks that maybe, just maybe a cure can be found in that time.

He wants me to come back to the hospital this week. I may try and get a picture if I can. let you all see this brave little guy you're pulling for but don't even know. This thread really moved my friend Rich.

Thank you.