Food: I nursed Amanda four months exclusively. I was neurotic about it and she did not have any formula. Very quickly I figured out that she was allergic to milk. It seemed whenever I ingested milk that she would have a stuffy nose for days. The more milk I had the more congested she was. By the time she was diagnosed, I was beginning to wean her which was at about 5 and a half months. I am not sure which formula I was using at this time, probably Lactofree. I stopped nursing when she was diagnosed because I noticed that the nursing position was not the best for her. She seemed to better take a bottle while sitting up in her bouncee seat. We then began experimenting with formulas. We found that a soy-based formula was the best for her. It seems that many of these babies are sensitive to milk products. After our first barium swallow, we began to thicken her formula with cereal to decrease the risk for aspiration.
* 5/20/00: We have placed Amanda on an elemental formula. We are supplementing the necessary vitamins and minerals. What a difference! She poops without any help from laxatives!! Her resting heart rate has dropped by about 25 beats per minute! We have also noticed so many behavioral changes!
G tube: This was the first big decision we were faced with. Right away, our doctors began to encourage us to get a PEG tube. We were terrified to have her put to sleep and the thought of not being able to feed her by mouth was overwhelmingly sad. We were advised that we wouldn't have to use the g-tube until it was necessary. But we were told that with SMA type 1, she would eventually lose her ability to swallow and without a PEG tube, she would starve. We consented, and a month after her diagnosis it was surgically inserted. She was definitely having trouble eating by this time. She would get so tired while eating that she would not be able to eat enough and even with frequent feedings, I always thought she was hungry. Now that we have the G tube, Amanda is fed 3 ounces 4 times per day and 13 ounces at night by way of a continuous feed. The G tube has really been a blessing. It is very comforting to know that I can give her the nutrition she needs and it is very easy to use. Although this has become second nature now, it was definitely an adjustment.
Constipation: I feel that this subject deserves it's own category. Due to decreased muscle strength and movement her digestive system is effected. Which can indirectly labor breathing because of pressure on the diaphragm from the intestines. Thus, we became very focused on poop. There was such a change in her behavior once she had a poop, we knew that we would have to regulate her. We tried dark Karo syrup, which did not give good results. We next tried Laculose, which caused stomach cramps. We are currently using Miralax, which has worked great! She is no longer lethargic due to elimination problems.
Reflux: This became an issue for us right after Amanda had her G-tube placed. We at first used Zantac and Propulsid. But because of the bad press on Propulsid, we switched to Reglan. Both Reglan and Propulsid cause irritability in Amanda, so we are unable to give her the maximum dosage for her age. But some is better than none. Reflux can make her sound junky and increase her risk for aspiration. This medicine cuts down on the reflux and increases the motility of her intestines.
Aerosols: We use albuterol and atrovent for her aerosols. Atrovent is used in the morning and at bedtime. Albuterol is used during the day. Amanda gets treatments 4 times per day while she is awake and only if needed at night. This is how we start her treatment.
Chest PT: This is also done 4 times per day. This is done with a cupped hand. In general, we do it for four minutes on her front getting both lobes at once. We do each side for two minutes and we do her back for four minutes both lobes at the same time. If we know that she has some atelectysis on a particular side we increase her side PT to four minutes.
Inexsufflator: This machine is such a help. We use this at least four times a day. This acts as a glorified plunger. It blows air into Amanda's lungs and then sucks it out and by doing so it brings up mucous that is in Amanda's lungs. We set the pressure between 30 and 40. After 5 breaths we suction her mouth with a yonker and then continue. We perform 5 rounds suctioning after each round.
Suction machine: We use this quite a bit. We either use a catheter or the yonker just depending on what is more appropriate.
Bi-Pap: This is a form of non-invasive ventilation. Amanda uses this whenever she is sleeping! I am sure that this piece of equipment has saved her life! We have the I-Pap set between 16-18 and the E-Pap set on 2. The settings are adjusted when she is sick. This machine allows her to rest without having to work to breathe. It also allows for deeper inspiration and assists in opening her airways.
Contractures: So far this has not been much of an issue for us. At 10 months her feet are a little affected and we do have AFO's for that. I stretch her knees, hands and hips a little bit but don't tend to concentrate on it.
Deltoid aids: An OT made Amanda some deltoid aids to enable her to use her hands better. The concept is to use an aid to life her arms so that she can position and use her hands. I do think that this enables Amanda better use of her hands. I have enclosed a picture.