Frequently Asked Questions about Spinal Muscular Atrophy Type I (Werdnig Hoffman)
I've added this page to the web site to relate the questions of other SMA families with the answers that we found for our son. Please do not rely on this advise as an alternative for medical advice from your doctor. Please be inspired to seek your own solutions under the protection of your doctor’s care. Everyone is unique, and what worked for our son may not work for everyone with SMA.
(Picture is my Shane at age 5 (1993) in his tilt-in-space wheelchair, Rifton standing frame and sidelyer.)
Q: Since finding out about your child's SMA, what have been the "good things" to come about or happen to you and your family?
A: I've learned that life is precious. We are all much stronger than we think we are. Never say impossible or allow yourself to wallow in the worst case senario. Someone with zero muscle strength can be the strongest person that you ever met in your life. I was blessed to have twenty two years with my favorite person to be around, and that was my greatest treasure. Oh and I got cat like reflexes!
Q: How do you help a non-verbal child communicate?
A: When my son was only 11 months old, I taught him to answer yes/no questions. He could frown for no and blink for yes. I thought a frown is a universal answer for no, and an eye blink could pass for a head nod. That gave us the ability to ask yes/no questions to find out how he was doing. When he was about 2 years old, we taught him to make a noise to get our attention and look at what he wanted. Using eye-gaze he was able to directly communicate “I want to go out” by looking at the window. “I want a TV show” by looking at the TV. “I need to be suctioned” by looking at his suction machine. We also taught him to count by "hooting" as we said the numbers. Eventually we could ask him how he felt on a scale of 1 - 10. At 3 years of age, we got a communication device that he controlled using a single switch on his finger. Starting with only 2 choices, by age ten, he advanced to having multiple pages of info that he could select using a slight muscle movement switch on his eyebrow. This system can be a Dynavox™ or a laptop with Speaking Dynamically™ and Boardmaker™. A laptop requires an interface that allows the switch to be the mouse click. Using this system, my son was able to take classes adapted so he used his computer to answer test questions. He took normal classes from preschool to 12th grade, and graduated with honors. Non-verbal doesn’t mean not thinking or not intelligent. There are wonderful adaptive devices that give a voice to the voiceless, and you’ll be so surprised to hear what they have to say.
It is also important to work out a list of twenty questions that can be used to allow communiation without the use of a computer. We used yes and no responses to allow Shane to develop his own list of twenty questions. The list allowed him to communication non-verbally with anyone.
Q: What is CPT?
A: SMA requires extra care to ensure good respiratory function. The treatments we gave our son followed the protocol for children with cerebral palsy or cystic fibrosis. I got the following information from “The Living with Cerebral Palsy” website at I added comments in parentheses.
Chest Physiotherapy Definition
Chest physiotherapy or CPT is the term for a group of treatments designed to improve respiratory efficiency, promote expansion of the lungs, strengthen respiratory muscles, and eliminate secretions from the respiratory system.
The purpose of chest physiotherapy, also called CPT, is to help patients breathe more freely and to get more oxygen into the body. CPT includes postural drainage, chest percussion, and chest vibration, turning, deep breathing exercises, and coughing. It is usually done in conjunction with other treatments to rid the airways of secretions. These other treatments include suctioning, nebulizer treatments, and the administration of expectorant drugs.
CPT can be used with newborns, infants, children, and adults. People who benefit from chest physiotherapy exhibit a wide range of problems that make it difficult to clear secretions from their lungs. Some people who may receive chest physiotherapy include people with cystic fibrosis or neuromuscular diseases like Guillain-Barré syndrome, progressive muscle weakness (myasthenia gravis), or tetanus. People with lung diseases such as bronchitis, pneumonia, or chronic obstructive pulmonary disease (COPD) also benefit from chest physiotherapy. People who are likely to aspirate their mucous secretions because of diseases such as cerebral palsy or muscular dystrophy also receive chest physiotherapy, as do some people who are bedridden, confined to a wheelchair, or who cannot breathe deeply because of postoperative pain.
Chest physiotherapy should not be performed on people with bleeding from the lungs, neck or head injuries, fractured ribs, collapsed lungs, damaged chest walls, tuberculosis, acute asthma, recent heart attack, pulmonary embolism, lung abscess, active hemorrhage some spine injuries, recent surgery, open wounds, or burns.
Chest physiotherapy can be performed in a variety of settings including critical care units, hospitals, nursing homes, outpatient clinics, and at the patient's home. Depending on the circumstances, chest physiotherapy may be performed by anyone from a respiratory care therapist to a trained member of the patient's family. Different patient conditions warrant different levels of training. Chest physiotherapy consists of a variety of procedures that are applied depending on the patient's health and condition. Hospitalized patients are revaluated frequently to establish which procedures are most effective and best tolerated. Patients receiving long term chest physiotherapy are revaluated about every three months.
Turning from side to side permits lung expansion. Patients may turn themselves or be turned by a caregiver. The head of the bed is also elevated to promote drainage if the patient can tolerate this position. Critically ill patients and those dependent on mechanical respiration are turned once every one to two hours around the clock. (Upper segments of the lung drain down so do them first with head of bed up or patient in a sitting position. Lower segments drain up, so it helps to do them in a head down side laying position if possible; suction or cough before returning to an upright position.)
Coughing helps break up secretions in the lungs so that the mucus can be suctioned out or expectorated. Patients sit upright and inhale deeply through the nose. They then exhale in short puffs or coughs. Coughing is repeated several times a day. (A cough assist machine is utilized by many kids with SMA to help them move their secretions if their own cough is too weak to be effective.)
Deep breathing helps expand the lungs and forces better distribution of the air into all sections of the lung. The patient either sits in a chair or sits upright in bed and inhales, pushing the abdomen out to force maximum amounts of air into the lung. The abdomen is then contracted, and the patient exhales. Deep breathing exercises are done several times each day for short periods. (If your child can blow a musical instrument like a kazoo with or without assistance; also blowing bubbles works good to get deep breathing going.)
Postural drainage uses the force of gravity to assist in effectively draining secretions from the lungs and into the central airway where they can either be coughed up or suctioned out. The patient is placed in a head or chest down position and is kept in this position for up to 15 minutes. Critical care patients and those depending on mechanical ventilation receive postural drainage therapy four to six times daily. Percussion and vibration may be performed in conjunction with postural drainage. (You can use your cupped hand, a manual percussor, a mechanical percussor like a General Physiotherapy Vibracare G5, or a vest. The important thing is that the method you choose is effective to clear your child’s lungs.)
Percussion is rhythmically striking the chest wall with cupped hands or a mechanical percussor. It is also called cupping, clapping, or tapotement. The purpose of percussion is to break up thick secretions in the lungs so that they can be more easily removed. Percussion is performed on each lung segment for one to two minutes at a time. (This is as simple as using your cupped hand to clap on the chest wall. Percuss the rib cage area only! Avoiding the breast bone, area under ribs, kidneys, etc.)
As with percussion, the purpose of vibration is to help break up lung secretions. Vibration can be either mechanical or manual. It is performed as the patient breathes deeply. When done manually, the person performing the vibration places his or her hands against the patient's chest and creates vibrations by quickly contracting and relaxing arm and shoulder muscles while the patient exhales. The procedure is repeated several times each day for about five exhalations. (You can do vibration with your hand also by gently vibrating your hand on the chest wall.)
The only preparation needed for chest physiotherapy is an evaluation of the patient's condition and determination of which chest physiotherapy techniques would be most beneficial. (Using a stethoscope to check for lung areas with diminished sounds or audible mucous sounds will help you know where to concentrate your CPT - ALWAYS percuss the worst side first - laying on the worst side can cause a quick oxygen saturation drop.)
Patients practice oral hygiene procedures to lessen the bad taste or odor of the secretions they spit out. (We did tooth brushing twice a day from age 1 and up - see a speech pathologist for help with oral care and oral stimulation exercises customized to your child.)
Risks and complications associated with chest physiotherapy depend on the health of the patient. Although chest physiotherapy usually poses few problems, in some patients it may cause
1. Oxygen deficiency if the head is kept lowered for drainage
2. Increased intracranial pressure
3. Temporary low blood pressure
4. Bleeding in the lungs
5. Pain or injury to the ribs, muscles, or spine
7. Inhaling secretions into the lungs
8. Heart irregularities
The patient is considered to be responding positively to chest physiotherapy if some, but not necessarily all, of these changes occur:
1. Clearing of sputum secretions
2. Changes in breath sounds
3. Improved vital signs
4. Improved chest x ray
5. Increased oxygen in the blood as measured by arterial blood gas values or pulse oximeter O2 Sat increases
6. Patient reports of eased breathing
How do I get updates concerning spinal muscular atrophy research?
From the Muscular Dystrophy Association
From Families of SMA
If specific corporations, universities, or research groups are mentioned, see if they maintain an email update feature on their web pages and sign up with your email address.
Q: Are there nutritional studies for SMA?
A: I can relate our experience with my son’s diet. The only dietician I consulted tended to treat SMA as though increasing calories and the amount of formula was the answer. I found that my son was like many kids with SMA, and he had to eat slowly to avoid reflux. Many kids cannot tolerate high-calorie high-fat formulas. In the beginning, all my son’s feeds were by food pump into a g-tube starting with Pediasure formula due to its high caloric value. The pump was set with a slow rate and ran continuously. It was only off for respiratory treatments. As an infant and toddler he had poor digestion with failure to thrive. Later we found that he had undiagnosed food allergies that were severe enough to keep him from digesting properly. The pediatrician finally let me take him off the Pediasure that he was not digesting and begin to puree real food for him. Luckily the Kangaroo food pump will push pureed food. In doing this, I started with simple broth and chicken puree then added vegetables and fruits one by one eliminating any foods that caused digestion problems. I immediately noticed that milk was not digested, and switched to rice milk or soy milk. Vegetables with chicken broth and rice milk were digested quickly – especially carrots. Fruit was not digested. This allowed us to pinpoint that my son had a severe allergy to fruit and milk. Once we determined this, he began to digest well. The doctor suggested Pediatric Vivonex which is for kids with allergies, and for once my son began to thrive. Vivonex is part of the Amino Acid diet used by many children with SMA. My son took supplements for vitamin E, vitamin B complex, biotin, potassium, a liquid cal-mag-zinc and the herbal supplements Nuplus regular and Liquifive.
I am a Sunrider distributor and can help you get these foods. Just contact me.
See the following website for additional information on the Amino Acid diet for SMA https://www.aadietinfo.com.
Q: What can we do to help an SMA kid before and after intubation?
A: Intubation is a medical procedure that puts a breathing tube down the throat into the bronchial tube of the lungs. It is done to enable temporary use of a ventilator to assist or replace breathing. Keeping lungs clear is the goal, and doing as many respiratory treatments as it takes to keep them clear will get you there. Usually intubation due to illness will result in a successful extubation (removal of the breathing tube) after the illness has run its course. If a patient fights the tube and ventilator, the medical team may give drugs to make them sleep until the crisis is over.
Q: How do you help your child deal with anxiety?
A: I know my son had sweats when he was anxious. We had a stuffed animal that he could hug to comfort him during anxiety. For little kids, distraction is the best way to help them cope. Have a special friend visit, sing songs, play music, watch videos like “Big Bird Goes to the Hospital.” It was one of the best videos we found, and is available on youtube. Reassure him that everyone is there to help him and that you’ll be there. If you have to step out leave a friend or family member to watch over him and let him know you’ll be right back. Having you there is the best medicine. Older children can be taught stress management techniques and positive thinking.
Q: Are there Medicaid Waivers that are based on my child’s income instead of my own?
A: There are waivers to cover children with complex medical needs, and they vary by state. Contact your states department of human services as soon as possible after your child is diagnosed. The medicaid waiver will pay for covered services that your insurance company will not pay. For example, our TEFRA waiver in Oklahoma paid for 16 hours per day of skilled nursing at our home, because our insurance had a cap of $10,000 per year for nursing. Our insurance paid for hospitalizations, all therapy services, DME and medical supplies.
The Katie Beckett Waiver, also known as TEFRA, the Deeming Waiver or the 2176 Model Waiver, enables severely disabled children and adults to be cared for at home and be eligible for Medicaid based on the individual’s income and assets alone. Without the waiver, the income of legally liable relatives is counted when the individual is cared for at home. Some states have a long waiting list for this program, but families of severely disabled individuals who want to care for their family member at home are encouraged to apply. If you have life threatening needs your state may have the ability to move you to the top of the waiting list, so you'll get services immediately after application. Some states have income based eligibility requirements. For more information call the Medical Utilization Review Unit at your State Department of Social Services. Rules change often.
Q: What is reflux?
A: Reflux occurs when stomach contents come up into the esophagus. It is commonly called heart burn. A reflux can result in aspiration pneumonia if the stomach contents are breathed into the lung. Children with SMA may be prone to reflux, and keeping them in a head up position when their stomach is full will help lessen reflux.
Q: Can children with SMA go to school?
A: In the US, the school system can provide home based instruction for a child who is in danger of getting life threatening respiratory infections at school. For our son the school provided services at our home including a teacher daily for an hour each day, a speech therapist twice a week, a physical therapist once a week, and an occupational therapist once a week. Many children with SMA attend school with attendants or nurses provided by the school. Contact the special services offices of your local school system. Some school systems provide early intervention with services starting at birth or at 3 years of age. Using an Individualized Education Plan, the parents can work with the doctor and the school system to determine the least restrictive environment. This includes time at school and time at home for home based instruction. Remember that your IEP can be modified at any time. Parents or guardians have the right to call a meeting to modify the IEP at their descretion.
To keep the scheduling of all these therapist and teachers I put a calendar up in Shane's room with an AM appointment and two PM appointments. On a first-come-first-serve basis I had them fill in the times and arrange swaps amongst themselves. This simplified the scheduling for me. It also kept them from coming during respiratory treatment times.
Q: What are the pros and cons of home schooling?
A: The main benefit of home schooling for us was removing the stress and anxiety of going to school. Staying at home kept germs away, and from age 5 and on, Shane was rarely sick. Keeping your child out of school when cold and flu season is in full swing will help them stay as healthy as possible.
With home schooling the main drawbacks are lack of socializing and missing some educational opportunities. You can make up for that by working with the moms and kids in your neighborhood. Explain to them that you have to stay away from anyone with a cold or sickness to keep your child well. We let kids come over to watch TV and get to know our child. We also had kids over to play on the computer and to play board games. We had about 22 kids who lived on our block and all were friends with Shane. They all knew they had to wash hands when they came to visit and to stay away if they had a cold. There are other ways to socialize. Here in our town there is a Challenger Baseball team where kids in wheelchairs team up with kids who will help them hit a baseball and push them around the bases. Kids with power chairs can round the bases via their chairs after someone helps them hit the ball. Your child will miss a lot of educational opportunities, so make plans to take them into school for special assemblies, photo day, and any other extra activities they want to participate in.
Q: Is hip socket surgery beneficial to SMA kids?
A: Rather than surgery, a good standing program can help deepen hip sockets or even prevent problems from ever occurring. See Rifton.com for some excellent standing frames. Shane used a supine stander that laid flat for us to transfer him to the stander. It had straps and supports that kept him in good standing position. It allowed us to hand crank it into a standing position at a comfortable angle. Please make standing a time to play board games, computer games, finger painting, or other craft activities. That will help your child view standing as fun instead of as therapy.
Q: What are considerations when getting a power wheelchair?
A: Power wheel chairs are very heavy, and work best on level ground. In my opinion, here are some considerations. Be sure that your chair includes a good seat belt system and anti-tip wheels on the rear. A good anti tip system will keep the chair from falling over. An unstable chair is very dangerous. A power wheel chair user must always use the seat belts to keep from falling out of the chair. Our son used a seat belt that also crisscrossed the chest and that worked best for him. That kept him from falling forward when stopping suddenly. Tilt in space is a must for SMA, and some of the children are kept in a reclining position with head slightly elevated. The position of the wheelchair user needs to be adjusted every 15 to 30 minutes to keep from getting pressure sores. We used an ROHO low profile seat cushion. My son tried several cushions and this was his favorite. It uses pockets of air to keep from sitting on a hard surface. Try sitting in a hard chair without moving - how long before you have to reposition? With less muscle tone, our kids have to use the soft cushioning to be comfortable.
* Do an internet search for portable wheelchair ramps that may be helpful. The two ramp system that telescopes to 10 feet was our choice, it worked for multiple wheelchairs and can be used to jump stairs or get into a van. NEVER have your child in the chair for the first load and unload of the chair into a van. ALWAYS use a stuffed animal stunt double, so you can make sure head clearance is good and the chair loads and unloads safely. Make sure your DME supplier knows that the chair will not work for you if it won't load and unload into your van. * Consider upgrading to a rear entry van that allows the child to load on a more level surface and ride in the chair which saves time and energy.
* Hardwood floors are easier to run the chair on than carpet.
* Be sure that rooms allow for the full 360 degree turns for the chair. Most manufactures include the space needed for a 360 circle for the chair.
* Use cut swimming noodles to pad corners with foam if possible, and move any unnecessary furniture to allow the wheel chair user to move freely.
* You can set up cones in your garage for chair-training time. You and your child have to learn to drive the chair.
* ALWAYS have a stop switch installed on the back of the chair, so you can hit it if your child's chair is about to run into something.
* If your child tires easily, there is an attendant control that allows the parent to run the chair from behind. This is a must. It keeps you from killing your back leaning over to drive from the armrest. MDA helped us get our attendant control.
* Follow the manufacturer’s directions on hooking the chair up to a charger. For us this was a nightly task so the chair was always charged and ready to go. Following this also keeps the chair battery system from wearing out. You'll need to set aside a parking place by an electrical outlet for the chair to charge when not in use.
Q: How do you help your child manage pain?
A: Most pain related to SMA is due to muscle contractures or scoliosis. For pain we consulted a chiropractor and did daily electro muscle stimulation with our own LSI machine. I think a TENS unit would work as well. Insurance purchased the machine, and the chiropractor instructed us on what settings to use and how long to run it. It took about 30 minutes a day and kept my son, Shane pain free. There are range of motion exercises that you get from a physical therapist, and we did those in the morning and before bedtime to keep muscles from becoming deformed or contracted. In addition there are AFOs to keep the foot and ankle from contracting. There is a TLSO that keeps the spine from slumping and starting a scoliosis. They also help keep a scoliosis from getting worse, and using air balloons in the brace can move the spine back to correct position. We rarely used any pain medication, and only occasionally would use children’s unflavored motrin. In my opinion, I’d avoid any pain killers that are processed by the liver, neuromuscular conditions already have a compromised liver. Of course your doctor should be consulted if pain becomes an issue.
Q: How do I prevent contractures?
A: Contractures can be prevented incorporating range of motion (ROM) exercises into the daily routine, using orthotic braces and splints, and using good positioning. This involves moving every joint to stretch every muscle 20 times twice a day. It takes about 30 minutes. When one is immobilized, this muscle activity keeps muscles and tendons flexible and normal. Muscle will atrophy if inactive, and tendons will shorten which results in deforming contractures. Contractures are totally avoidable by investing 30 minutes twice a day to do the simple exercises required to keep them from happening.
Splinting using hand splints, ankle foot orthotics (AFO), thoratic lumbar spine orthotic (TLSO) – these all put limbs and back into a straight neutral position and are worn when sitting or standing, and sometimes while sleeping. They are paid by most insurance plans when ordered by a physician and filled by an orthotist.
Correct positioning. Using pillows or supports like a Whitmeyer headrest to support the body in neutral positions. Some examples of bad position are letting the hand drop with a limp wrist, letting the foot bend or stay in a dropped position, and letting the head fall forward or to the side. Positioning should be varied so legs are straight sometimes or knees bent sometimes. Side lying with a pillow at the back keeps the spine straight. Use of a pillow between the knees keeps the hip in alignment.
Our son was 22 years old, and he had no contractures. Good range of motion exercises, use of plastic braces for hands, feet, and back, and good positioning kept his contracture free.
Q: What is palliative care?
A: Palliative care is a specialized area of healthcare that focuses on relieving and preventing the suffering of patients. It is care given to a terminally ill patient which focuses on the individuals comfort and quality of life. Life expectancy is usually six months or less. NOTE: Sometimes this is an evil insurance company trick to drop therapy, nursing, and other rehab type services. Try to keep your child from being classified as requiring palliative care, and keep care on a preventative, skilled or rehabilitation care level.
If your child has been classified as such, and you are doing the respiratory care needed for them to live (trach / vent / cpap / bipap) appeal this decision with your insurance carrier and have them reclassified. Use case management options to obtain the therapy services needed. The choice of switching from rehabilitation or skilled nursing to palliative care should be made by a child’s parents or guardian after prayerful consideration. If you feel that your child’s life is ebbing, palliative or hospice care will be available to you. Never go this route without the consultation of a doctor who is familiar with SMA.
Q: What are plugs?
A: Plugs are thick mucus from the nose or lung. Buggers or bogies if you’re in England. We also called them catfish, because we’d suction them and pull them out like a fish! They are prevalent after a respiratory infection, and are the body’s way of encapsulating and eliminating foreign bodies from the lung and airway. Some remedies can be using a mucus thinning drug like mucomist or using a suction catheter / machine to physically suction them out. If you use a trach, the trach tube itself can accumulate mucus and simply changing the trach tube will get rid of the plugs attached to the tube. In small babies and children, a mucus plug can shut down a lobe of their lungs. Sometimes running a nebulizer with just normal saline will keep mucus from becoming thick. Due to these plugs it is helpful to have a suction machine and sterile catheters available to remove them.
Q: What is a Respiratory Treatment?
A: The purpose of a respiratory treatment is to open up all the lobes of the lung. Some treatments can be just breathing in a medication to open up the lung fields. In addition there is CPT (chest physiotherapy) which is vibrating or percussing the lung lobes to physically open them up. Then there is also PD (postural drainage) which is moving the patient so the lung can drain mucus. The upper lung field is above the nipple below the collar bone and it drains down. The lower lungs fields drain up, so being in a head down position can help them drain. Doing the aerosol treatment, CPT, and PD can open up lungs and make breathing a lot easier. The doctor will set up the schedule for the treatments, and should include standing orders for extra treatments if needed. See the related question "What is CPT?" for more info.
Q: What is a nebulizer?
A: A nebulizer is a small compressor that forces air through tubing converting a liquid respiratory medication in a neb cup into a fine mist that can be breathed into the lung. They are used with a mouth piece or there is an adapter that will allow them to attach to trach tubing or bi-pap tubing for inhalation. These are prescribed by a physician.
Q: Are there any medications that help with excessive oral secretions?
A: This may be controversial, but as the mom of a very severe type 1 child, I do not think drugs should be an answer. We made sure oral secretions could drain out of the mouth onto a cotton cloth diaper. We would change this regularly. Our son gradually learned to manage his secretions himself. Any medication has side effects that can cause more problems than they are worth. I think that drying up secretions will also dry out the eye. A bad eye infection can result. Teaching your child to force air out his mouth and to spit is much preferred. That’s right, we’re the only parents who encourage our children to spit. A speech language pathologist can do wonders in building skills for secretion management. I would consult one who specializes in oral motor exercises before just throwing drugs at the problem.
Q: When my child is sick how will we know when we should go to the hospital?
A: This is best answered by your pediatrician. Have an open discussion on what you are capable of handling at home, and what to watch for to know that it’s time to head to the hospital. For example, our pediatrician knew that we could have lab drawn at home and x-rays done at home. She knew we had a pharmacy that delivered. She knew that we could do extra respiratory treatments. This saved us from being hospitalized for respiratory infections. Our criteria for hospitalizing our son was fever that lasted longer than 24 hours, a change in heart rate or an irregular heart rate, prolonged seizure activity, low oxygen saturations that did not improve with treatments, or low body temperature that could not be warmed with warm blankets from the dryer. Always have a list like ours and a hospital admission plan in place before you need it. Keep a close watch on your child, and learn to do your own assessments. Your doctor or nurse should teach you to use a stethoscope and train you on what to watch for.
Q: Are there any camps for children with SMA?
A: Check with your local MDA office. They usually do a summer camp that includes all children with muscular dystrophy including SMA. Here in Oklahoma each child has a counselor or parents can come to camp, too. If your child has specialized medical care and/or equipment, make certain that qualified caregivers are with them at all times. My son did not tolerate hot temperatures in the summer, and was never able to go to Summer camp.
Q: Why should children with SMA eat at least every 4 hours?
A: Watch for a severe blood sugar drop if the kids fast for more than 4 hours. Without the muscle strength, the kids do not have the ability to metabolize without food. For our son, we'd switch to pedialyte plus two tablespoons of sugar for each liter of pedialyte, and give this via pump at his prescribed rate. This will prevent the drop in sugar and other electrolytes. Discuss with your doctor so you have standing orders to deal with nausea or stomach flu. The pedialyte with sugar worked best for us.
Q: Do kids with SMA need spinal fusion surgery?
A: There are children who get spinal fusion to correct their scoliosis. This has to be evaluated by a physician familiar with SMA and the Orthopedic Surgeon. Our son was too weak to tolerate the surgery. Bracing is another option. For my son, we started with bracing when he was 6 years old. He had the most severe form of SMA, and when we started bracing he had a 90 degree S curve. Using air bladders in the brace and changing braces every year his scoliosis improved to a 30 degree C curve that was only noticeable when he lay on his stomach. This correction held and he never needed fusion surgery. He used braces from age 6 to 22. The brace was a custom TLSO with a neck piece attachment. The neck piece was customized to support his head, and this allowed Shane to sit upright.
Q: Is there a durable medical equipment (DME) provider who specializes in equipment SMA kids need?
A: My favorite is http://www.smasupply.com. SMA Supply is run by the parent of a child with SMA, and they donate towards SMA research as well. We also used Allegro Medical and Spin Life.
Q: Should I rent or own the medical equipment?
A: Work with your insurance company and/ or medicaid case manager to purchase the equipment you need and insist on having back up machines for times equipment goes in for preventative maintenance or if it fails and needs to be repaired. Owning the equipment allows better stability in the event that you must change DME suppliers. I have seen cases oif kids loosing all their equipment when a DME decided to drop their case. That caused undue stress when the mom had to rush to have a new supplier bring over replacement machines on short notice.
Q: My child’s feet are starting to turn in, are there stretches or something I should be doing to help?
A: Consult a physical therapist for exercises. This turning in is a muscle contracture forming, so Range of Motion exercises will help reverse the contracture. The doctor can order a custom AFO (ankle foot orthotic). The AFO can be made so that a shoe will fit over it, so it can be worn day and / or night. Usually there is a schedule of 2 or 3 hours on and 1 hour off to follow. Always watch for skin redness and report any redness that lasts more than 20 mins to the orthotist so he can adjust the AFO fit so it won’t pinch or put too much pressure on one area of skin. We also used Silipos® Universal Toe Crests when Shane was sleeping to keep his toes from curling.
Q: Do you have helpful hints on how to handle a hospital stay?
A: I compiled this list over the 22 years that I cared for my son, Shane. SMA type I can put you in the hospital a lot. It’s surprising how many times people told me, “now you can rest.” Ha – they had no idea! Being in the hospital was always stressful and the exact opposite of vacationing. At the beginning, I was a complete basket case. I’m just hoping that my experiences will help you and that you can benefit from what I learned over the years. Things evolve to the point that you go into the hospital, get things managed and taken care of, and get back home as soon as possible!
My hospital survival kit in a rolling backpack:
1. Bible and light reading material both for me and also books for you to read aloud to pass the time.
2. Spiral notebook to keep notes on everything that is done to your loved one by date and time. More on this item later.
3. Change of clothes, toothbrush, etc. Just essentials.
4. Estimate at least a three or four day hospital stay, and put fruit, protein meal replacement bars, and bottled water into your bag. Hydrate, hydrate, hydrate. You are worthless if you get sick, too. HOSPITAL VISITORS please note that these items are so much better than flowers!
5. One of those travel pillows, so you can sleep sitting up.
6. A written exercise plan for you, caregiver. You’ll need to work out an exercise plan for keeping your body in working order. Look up the Canadian 5BX for the five basic exercises for stretching, sit-up, back extension, push-up, and running in place. A quick jog up and down the staircases at the hospital works really well, too.
7. A phone and address book just in case your smart phone has to be turned off. Today before anything happens, in advance contact neighbors, family, and friends to see if they will help you in the event your loved one has to be hospitalized. You’ll need someone to assume your home duties. Perhaps a neighbor who likes your dog will take over his care, another neighbor can pick up your mail and call you if there’s anything important. Use that phone list to divide and conquer. The people who say, “If you ever need anything call me,” will not do anything unless you call them!
8. A list of your child’s home medications and interventions. You can request to keep their home routine going while they are in the hospital. The doctor will go over your list and write orders to keep things going if possible. Without an order, you will not be allowed to do anything.
9. A running list of doctors and specialists that you’ve visited. I kept this info in another spiral notebook, so I had the history of everything in one place. Each doctor had a section, so I could jot down questions that came up. I could never remember the questions when we were in the doctor’s office, and I could not remember the answers when we got home! Journaling both kept me from forgetting things, and provided an excellent medical history record.
10. When you’re loved one is a little baby or child, you have to bring things that feel like home. That cradle music box, the night light, a favorite pillow or blanket; something that feels like home. Pick things that you can disinfect later when you get home, and that won’t disrupt the hospital. You’ll want to leave the fire helmet with working siren at home! Try to keep your bedtime routine, so days and nights do not get mixed up. Dim lights for nighttime; bright lights for daytime.
11. If your loved one uses specialty medical items, bring them with you. The hospital has a hard time ordering specialty items. If your loved one uses a medical formula for example; you want to bring a 3 to 4 day supply with you. If your stay is longer, the hospital will receive their order by then. Common items like syringes, catheters, suction equipment are not necessary to bring with you.
12. Your own canister of Clorox wipes and hand sanitizer. Wipe down hospital surfaces yourself. The cleaning crew will come through, but a good OCD caregiver will relish the activity of disinfecting!
13. If your loved one is non-verbal and you have a communication method worked out or a list of the top 10 questions to ask, put this in writing on a poster to hang at the head of the bed. For example: My son worked out his own poster.
Hello my name is Shane.
I have a normal IQ, and I am a people person.
I like to joke around a lot, so I may tease you.
I would love it if you would tease me, too.
I am non-verbal.
I answer “yes” by rolling my eyes and “no” by frowning my eyebrows.
If I seem disturbed, it’s probably that I don’t like the TV show, so ask about that first!
I only like to have my trach tube suctioned 2 inches. NO DEEP SUCTIONING.
Additional notes on that spiral notebook. Some nurses are not going to like that you’re writing down everything. You’ll have to develop a balance of being a friend to the staff and guarding your loved one. You do not want anything to develop into a drama filled event. They do have the authority to kick you out of the hospital, and that will not do your loved one any good. Justify your need to know by making it clear that you are primary caregiver at home, and you need this information. You need to be there with your loved one for his/her comfort. The hospital staff is geared toward life-saving. They will not have time to keep your loved one comfortable, entertained, and as happy as can be. Only super exceptional nurses are able to do everything; in my experience most are just doing what they have to do to keep the hospital floor running.
Explain to the staff that you are learning. Explain that you are in this for the long haul. Explain that you have to take your loved one home and you’re using all this information you’re collecting for future use. It will serve as a record for the future, so you’ll know what interventions work and which ones do not. Most nurses will melt; most of them will be moved that you’re there to learn and that you’re planning for the future.
Your record will not only be a learning tool for you, it keeps everyone honest and it serves as an additional record. Think of yourself as quality control. Ask nurses what they are giving in advance of them giving it and write it down. Later, if the nurse forgets to enter this into the computer, you will catch that double dose before it’s given. If you know when that next dose is due, you can keep it from being late when the staff gets busy. You can offer to do some of the care and most nurses will be happy to have your help. When you’re helping, you are valuable, and when you’re valuable they will not kick you out of the room.
Watch for any negative side effects after drugs are administered. If the staff doesn’t give you a list of side effects, you can look up drugs on the internet if the smart phone is working. Just bring it to the attention of the staff if you notice anything important; for example, 5 minutes after administration of the new drug, a rash started to develop or your loved one’s heart rate went up or went down. Be vigilant and informative.
Talk to your pulmonologist, and have him instruct the hospital staff to show you how to do treatments for your child. Always use the nurses and therapists to learn how to care for your child better. Your child will have less anxiety with you providing the treatments. Sometimes hospital equipment is slightly different, and from my experience the staff is always happy to show me how to use it. You'll realize that sometimes the respiratory therapist will have an emergency or they will be short staffed, you can be in control so that doesn't affect your child's care and needs. (Per Misty)
Take a deep breath. Always remember to breathe; I think I held my breath for a couple of years. That is not good for you. Remember, this too shall pass. As the British say, “Keep calm and carry on.” If you have to cry, try not to cry on your loved one, it tends to scare them. I always cried while watching commercials, and told my son that those Kodak commercial always get to me! Be creative; those surgical masks can mask tears as well. A prayer for healing, wisdom and the favor of God always helped. I learned to keep the prayers for patience and strength to a minimum. The only way to get patience is to go through things that try your patience, and the only way to get strength is to go through things that make you stronger!
Q: What are your top tips for coping with a chronic medical condition?
A: I have ten tips for you:
1. Do not take no for an answer. Avoid negative answers and take positive actions instead.
2. Seek information and take charge to use that information to improve your health. One can be healthy with a chronic condition.
3. Seek out survivors and find out how they did it. Incorporate their ideas into your routine, and see if it helps.
4. Form constructive partnerships with health professionals. You need doctors, nurses and therapists who are on your side, and who will work with you. Keep looking until you build an A-team.
5. Make your needs clear to your family and friends. They probably will not help you until you spell things out to them and ask for their help.
6. Set aside time to be quiet and meditate for at least 15 minutes every day. I call it a 15 minute vacation.
7. Evaluate your diet and eat only foods that improve your health. If you have diet issues start with chicken broth and pureed chicken, add one food at a time.
8. Work daily exercise into your routine for a total of one hour per day. It’s ok to do several short exercise activities to make up that hour. For people with SMA sitting up or standing may make up that physical activity, and always do range-of-motion exercises.
9. Do not hesitate to make major changes to your life. For example, you may have to relocate to a new city to get the care and support network you need just like we did.
10. Cultivate acceptance, accept where you are to get where you want to be. Don’t dwell on the circumstances, instead cherish each moment. So I have SMA, so what, I am still me.
The goal is to look back at the time of illness as a gift that makes you value life and strengthens your relationship with God.
As my friend JD always says, Dance in the Rain.