---

Montanna's Journal (Treatment)

Back

---

By Julie Cini

After Montanna's diagnosis at 3 1/2 months with SMA type 1, the most severe form of this tragic disease, we were told at the time there was nothing that we could do but go home and enjoy the time that we had left.

After overcoming the initial shock, Ross, Montanna's father, started his research into SMA - the disease.

When Ross started to tell me what he had found and what we could expect, I didn't want to know about it.

I now realize that at that stage I was in denial and was still hoping that my child would be different from the others.

As I now know, this disease can affect children in different ways yet the outcome is still usually the same.

Once I realized what Ross was trying to achieve, I got on the boat.

This was the most important turning point in what turned out to be the most beautiful 10 1/2 months with our precious angel Montanna.

What we found is Doctor's and people in the medical profession generally, in most instances, knew very little on what we can do as parents to offer the best possible care and quality of life to our children while they are alive.

We had to be one step ahead of the disease all the way, and up until the end, thankfully we were.

The following is an outline of what we did For Montanna's case, but feel there would be similar care for all true type 1 children (Werdnig-Hoffman disease)

Firstly, we were warned about the importance of infection control and we can't stress enough how important this is, as we knew something as simple as a common cold could take our precious angel away from us prematurely.

We always washed our hands before coming into contact with Montanna and avoided with Montanna large crowds like supermarkets, waiting rooms or any place really where we didn't have control of the people she may have had contact with.

We were fairly vigilant with this and soon people soon realized the importance of this and if they had cold or any bug, would keep their distance.

We by no means had Montanna wrapped up in a bubble but we were very cautious.

Even when Montanna was hospitalized, we would stress the importance of infection control with the relevant nurses and doctors. We even at one stage had a tag pinned to her clothes "please wash your hands before you touch me". This may seem over the top but it got the point across pretty well.

The next step we took after much research and discussion with Montanna's Neurologist was to put her on an experimental drug for SMA, but a well known one for treatment of epilepsy. (Valporic Acid- Epilum)

One of the most confronting times I had early on was when Ross, after one of his many in-depth research expeditions came and gently tried to break the news to me that my beautiful little girl would loose the capacity to swallow.

As a breastfeeding first time mother this was very hard to accept and it is really only now that I am beginning to appreciate Ross' perseverance.

So what Ross did was arrange an appointment with the local GP and we discussed putting Montanna onto solid foods.

He agreed and it helped me to accept what was likely soon to take place. The reasoning for putting Montanna on solids at this point was both due to reducing the risk of aspiration (inhaling liquid or milk into her lungs) which is higher with the more liquid type foods, and secondly was to give Montanna the opportunity to do something that most of us take for granted.

That is to enjoy the taste and feel of different foods, and of course maintain her nutritional requirements. It was also around this time that we discovered that Montanna would do better in certain positions and this would change from day to day.

It was only a short time after this that my efforts to breast feed her stopped, as the warning signs were there, she was finding it more difficult to deal with fluids.

Now knowing what was soon to follow we made an appointment with our Pediatrician requested the placement of a NG Feeding tube, which he agreed to.

Initially after the placement of the tube, Montanna was a little uncomfortable but within a day or two after settling down and receiving far better nutrition she took a new lease on life.

Another great advantage of the tube was the ability to now give Montanna her medication accurately.

We also around this time acquired the loan of a suction machine, which turned out to be the most important piece of equipment that we would later need. This machine was used both orally and nasally and helped to keep Montanna's upper airways clear.

It saved her life on numerous occasions especially in the last few months of her life.

Montanna, after a couple of con-current infections was placed on prophylactic antibiotics, firstly amoxicillin and then was later changed to augmentin.

Montanna was having trouble at around 5 months with constipation and with what seemed to be the over production of saliva.

We now started to look into her dietary requirements. After much research and information which was gratefully provided from other parents dealing with this disease we opted to change her formula. In the end we opted for a short chain peptide formula, which worked for Montanna.

The next piece of news that I discovered, and had trouble dealing with, was that Ross was now telling me Montanna would soon loose the ability to be supported in an upright position, and how soon this turned out to be true.

This was so painful to us, as one of the most beneficial and most enjoyable times for Montanna was in the bath. These kids just love water. I would sit and watch her for what seemed hours moving around freely and happily, as this was the only time she could move her legs.

We had used a bath chair up to this point and now with the prospect that she couldn't be upright to enjoy her bath time was one of the low points. Montanna with support loved to be upright as she loved to take in all that was going on around her. I now understand why they call this disease the robbing disease. It just seems to take away one thing after another.

It took us a while to work out a comfortable position for her, but in saying this we always had to be alert in case she needed a position change. I was really starting to hate Ross, because now he is telling me that the car seat was becoming a problem.

Bloody Bugger! - and he was right yet again. The dilemma of the car seat was not an easy one to solve. How could we safely carry her in the car? We did the best we could by putting in a bouncer seat that we roped in. Now Montanna's car journeys were limited to hospital visits only.

Supplemental oxygen was now necessary for Montanna to maintain her oxygen levels. We had a small portable one for traveling, and a large bottle set up for home.

Montanna's cot had to be modified so that her mattress was on a ten degree angle to reduce her aspiration risk, as reflux can become a real issue. By this time she was more or less permanently in a lying position.

Montanna's had numerous medications that were to benefit her in some way. We were constantly at the chemist getting another drug for her to take.

In the end she was taking Epilum, Carnitine, Losec, Panadol, Codine, Augmentin and Morphine. The morphine was on stand-by in case of respiratory distress.

The equipment used was a suction machine, with 8-10 gauge catheters, oxygen with nasal canellas and pediatric face mask. IV pole for food delivery. Feeding pump for continuous feeds. Pulse oxy-meter for O2 sats and heart rate. Bi-Pap machine for assistance in breathing and to use in conjunction with physiotherapy.

Television to keep her inquisitive mind in constant over drive. We also invested in a stethoscope and an digital thermometer.

Chest Physiotherapy was so important to maintain Montanna's respiratory health. This was also aided later on by the use of a Bi-Pap machine. After seven months of age Montanna now literally was restricted to a lying position, and had to be repositioned every 3 hours and even more when unwell to both help her lungs and to reduce bed sores etc.

In the last month of Montanna's life her care was more or less 24 hours a day. She often came within near death on numerous occasions within a day.

At this point she had lost all capacity to swallow and her cough was nearly non existent, upper airway secretions were now her main hurdle. We were now coming to the realization that it would probably be due to implications that arose from this that Montanna would leave this world.

We now had Montanna hooked up to a pulse oxy-meter 24 hours a day. It became our tell tale babysitter as such to alert us when her O2 sats were down.

At times she could drop in seconds as low as 50% due to upper airway obstruction. There must have been some days where we would have had to suction Montanna up to 60 times.

Till the last week of her life she tolerated it well and would always give us a smile after its use. Montanna never complained with what we did to deal with her symptoms as they arose, as Ross and I were very focused on the importance of quality of life not quantity.

This was our choice. Now with Montanna being gone we are both at peace in a sense that we didn't cross that invisible line. The one of putting our needs above hers. Montanna called all the shots.

People often asked us how could we maintain this level of care and our answer was always the same for the little bit we did, Montanna gave us back far more.

This is only a very brief summary of what we did to care for and support our little angel before GOD called her back home. Her job here was done. If anyone would like any more information about anything on these pages please don't hesitate to contact us through the Contact us page.