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Daily Care

When our girls were diagnosed with the severest form of Spinal Muscular Atrophy we decided to be very aggressive in their care. We talked to many families with children with SMA as well as doctors and therapists. We listened to everyone and then decided on a plan of action that we were comfortable with. One of the most difficult problems in dealing with SMA is realizing that there is no proven protocol in the care of children with SMA. The medical community is very diversified in their approach, especially the care for type 1 children.

Every child is different, what works for one family may not work for another. Educate yourself, talk with your doctors and decide on what course is right for you and your family.

We were told type 1 children typically do not survive past the age of two because of their weak respiratory systems. They usually have a very weak swallow and cough. A cold can be lethal to these children. They can not handle the added secretions that a cold brings and pneumonia can develop. We addressed their respiratory care as our top priority.

Below is a summary of the girls daily care:

Respiratory Care –The girls use a noninvasive respiratory protocol under the guidance of Dr. John Bach of Newark, New Jersey. The girls have respiratory treatments at least 3 times a day which consists of percussions, suction, and breaths with the in-exsuffalator.

Skylar Bi-Pap.jpg (52631 bytes)

Bi-pap Ventilatory Support - They began using Bi-pap when they were 1 year old. We knew they needed it because they had trouble with night sweats and were very restless during the night. Their oxygen saturations would also fall below 95% during sleep before they started Bi-pap. The Bi-pap assists breathing and allows the respiratory muscles rest so they are stronger the next day.

Pulse Oximeter – We use this to measure the girl’s oxygen saturation levels. Their levels should be 95% or higher. We know when their levels stay below 95% that there is a respiratory problem.

In-exsuffalator – This is used to give an assisted cough and bring up secretions from their lungs. A suction machine is then used to clear the secretions. Due to their respiratory weakness, they are unable to produce an effective cough. This piece of equipment helps not only to cough, but also to expand their lungs to keep them healthy. It is used as range of motion for the lungs.

Percussions and postural drainage – We do percussions and postural drainage to help clear the girls lungs. We also use a nebulizer with albuetrol at least once a day. We have learned to listen for breath sounds with a stethoscope to determine if the girl’s lungs are clear.

Illnesses and Respiratory Distress

Times of illness are always very trying for the girls. Sometimes they have to be hospitalized to help them through a respiratory infection. Increased secretions, trouble breathing, congested airways, and oxygen saturations staying below 95% are indicators of an illness for them. The girls have been intubated due to pneumonias. When this occurs we follow a strict protocol for extubation which requires intense respiratory treatments for a least 48 hours.

We use oxygen at times of Respiratory Distress, but are very cautious with its use. The use of oxygen can be very deceiving. When saturations are going up with the use of oxygen, it could be believed that the girls are getting better. However, it could be masking the problem and secretions need to be cleared from their lungs with good cough techniques to improve ventilation. Also CO2 levels should be considered. If breathing muscles are weak, the amount of CO2 may not be properly eliminated and levels could build up.

Diet

The girls are fed through gastrointestinal tubes. They were breast feed until they were 10 months old. They then started developing swallowing problems. They had the g-tube and the Nissen Fundolplication when they were 13 months old. They did not do well on formulas like Pediasure, so we switched them to an amino acid formula, Pediatric Vivonex. They do very well on this elemental formula. We mix it with juice, baby food, and some other things that we have found helpful. We are very specific with their protein and calorie intake and adjust it to their weight.

We have also found that extra water is also important. It helps to keep secretions thin and helps with constipation.

Therapy

Physical therapy – The girls have a physical therapist work with them once a week to help with stretching, range of motion, seating and positioning issues. Our family does physical therapy with the girls everyday. We do stretching in the morning and range of motion. But all during the day as we play with them we are bending their legs, stretching their little fingers and moving their bodies. They love the hands on and hate to lay still any length of time. They also use a stander to help with weight bearing and an opportunity to see the world upright.

Occupational therapy - The girls have two occupational therapists that each come to our house once a week. They help them use their hands for daily living activities as well as activities with the computer and artwork and so forth. To help give them more arm movement we use arm slings attached to a small A frame (picture of them using their arm slings under Pictures).

Speech Pathology

Speech pathologist - This person helps with speech and language. We try to do mouth stretching exercises everyday. The girls are three and have just started to talk. Other families have told us that their children talked much earlier.

Water therapy- This type of therapy is a lot of fun for the girls and it has been a tremendous help for them. They have a small heated pool that they swim in three to four times a week. The buoyancy of the water allows them to move their bodies freely in the water. It is great exercise for them.

Preschool teachers – Our local school system has been very helpful and supportive with the girls. Teachers come to our house twice a week for school. They do various activities with the girls and really enhance their cognitive skills.

Seating – The girls have a kidkart that they use for seating as well as new powerchairs. They activate the chairs by infra red and proximity switches. It allows them a freedom of independence that is so important. They also enjoy moving around in their motorized scooterboard that they are able to activate with a switch.

Lastly, we feel that it is very important for the girls to experience many different things just like other children their age. They enjoy the outdoors and love riding in their wagons and swinging in the hammock. They are just beginning to enjoy some independence working on their computer and activating switches to make things work. Also, we try very hard to help them overcome their limitations by assisting them in task. They love playing games like Candyland and matching cards. They love country music and Disney movies. They are very intelligent little girls and learn very quickly. They learned from the very beginning they must fight to survive. They have become true fighters. They are also very happy children who love all of the simple pleasures in life. They have taught us so much.

Disclaimer: Please realize that this is not medical advice. All information contained in this web site is intended for informational purposes only and is not intended to replace and should not be interpreted or relied upon as professional advise.  It is strongly recommended that you consult a physician to assess individual conditions and needs.

This website is dedicated to all of the special angels who are living with and have died of Spinal Muscular Atrophy (SMA)

Disclaimer: All information contained in this web site is intended for informational purposes only
and is not intended to replace and should not be interpreted or relied upon as professional advice.
It is strongly recommended that you consult a physician to assess individual conditions and needs.