Pulling into the parking garage at Nationwide Children’s hospital, I noticed a beautiful little girl clutching a bear as she waited near the elevators with her family. The presence of the stuffed toy and absence of hair made it evident to me that she was sick. In that moment I was sad, thinking how unfair it was for kids to be sick, how unfair It is that families had to fight to keep their little ones alive. I felt sorry for the families that had to come here seeking help for their very sick little ones. Then it hit me. It hit me hard, the staunch realization that I was one of those people. I am here with my medically fragile child. I am not an outsider looking in with pity; I am a member of this group of people with the constant, immense awareness of my child’s mortality.
Sometimes I forget that Alex isn’t a healthy normal kid. For a little while, he feels like he is just as any other child. I guess because he is so small, it’s easy to think of him like a baby. I often hold him and carry him. As he is getting bigger, heavier, and more dependent than his peers, I get reminded of just how different his life is going to be. As he is getting older I am seeing more instances of challenges we will need to find solutions for, many more obstacles we will have to overcome, and mountains that will need moving.
As we enter the MDA clinic, we are surrounded by families of children in wheelchairs. Alex sits in his little Panthera wheelchair, wheels adorned with Batman emblems. We are here seeking counsel with specialists to help us keep Alex as healthy as SMA will allow. The SMA clinic visit is a very long one, but it is one that we don’t dread. We come with questions and requests, and we always leave with answers and referrals. We make the 4 hour trip every six months for these follow up visits. While here, we see the gamut of specialists, a total of 9 specialists this visit which includes: a social worker, dietitian, geneticist, neuromuscular doctor, pulmonary doctor, physical therapist, occupational therapist, Muscular Dystrophy Association rep, and complex care doctor.
Prior to Alex’s diagnosis, I was not aware of all the things social workers do. You typically only think of social workers like CPS, sweeping in to help kids who are in abusive situations. The social worker at Nationwide has been an enormous help for us. Up to this point, we receive no government benefits/help for Alexander. We applied for SSI (disability income) as well as medicaid for Alex and were denied for both due to our income. Our biggest hurdle in getting any assistance for Alex has been (aside from our income excluding us) a lack of knowledge of what programs to apply for, where to apply for them and who to contact. The social worker asks us our current needs and gives us a list of resources that could potentially benefit us and who to contact for the help. Basically, she has been a compass pointing us in the right direction towards finding financial avenues to help care for Alex.
There are so many things that insurance does not cover. We are now getting to the point of exhausting his raised and saved funds from the numerous purchases he requires that insurance won’t cover. It is a bit daunting to look at the list of expenses we will be undertaking. This week we bought new wheelchair cushions for better spinal support ($200) and had an estimate for a wheelchair ramp (over $2000) that is required by Alexander’s insurance to even consider a powerchair. We expressed our concerns to the social worker for the many things we are needing to pay for Alex to live as comfortably as possible, and she give us ideas for different avenues to pursue.
We met with a dietitian to discuss Alex’s size and dietary needs. She offered ideas to increase caloric intake and recommended supplements, including a daily multivitamin, to keep Alex healthy considering his newfound pickiness with foods. Alex is now approximately in the 10th percentile for weight, which is an improvement, and is now up to 27lbs. This is quite small for a 3 year old but reasonable considering his low muscle tone. At least now we are on the growth curve!
A geneticist did a family history at this visit. I expressed my concerns about our older boys and asked about the probability that they may have later onset SMA (Type III or IV). My fears were eased when she mentioned that it was highly unlikely since Alexander has a deletion of SMN1 and Type III and IV are usually mutations of SMN1/2. She nevertheless recommended carrier screening for all siblings and cousins when it comes time for their family planning.
The neuromuscular doctor was surprised to hear Alex had started Spinraza since he wasn’t receiving it at Nationwide. When we told her we were on the Biogen drug program, she recommended PT do a physical test to measure ability and improvement (I believe it was called Hammersmith test). She was impressed by his current ability and stability and discussed with us a little more about the mechanics of SMA and motor neurons. She talked about how Alex is lacking the gene that makes the protein that protects the nerves that his brain uses to send signals to his muscles. Those nerves are the ones that tell his muscles to move (the nerves that are used for “feeling” like touch are not affected). These nerves become sick and die due to the lack of protecting protein that he cannot make. However, with Alex on Spinraza, the drug helps his body make some of this protein and cells that aren’t too sick or already dead may come back and function (which explains the regain of some movements that we have seen). We aren’t sure what we can expect long term, but progress thus far has been promising.
Pulmonary doctor was a quick visit, as all of the kids were getting restless and loud at this point. She recommended that we keep using cough assist and rattle vest once per day in fall and winter at least. She recommend Pneumonia vaccine, and went over the sleep study results. The results were all good, which means Alex does not yet need to use a bipap at night. We were recommended to repeat the test in a couple years. She did mention that we should see an ENT doctor due to some snoring they noticed during the test.
Physical therapist briefly took Alex to do the Hammersmith test. We weren’t given his score, but she did say he was cooperative. She gave us ideas for things to work on in physical therapy, which starts in two weeks for Alex. She set us up with a prescription for AFOs (Ankle Foot Orthotics) for Alex to wear while in the stander. They are basically thin plastic braces that are molded to his foot to keep them rigid and offers support. I had noticed Alex’s ankles leaning inward recently. With SMA, when you notice an issue, you take action to correct it ASAP.
The occupational therapist spent a lot of time with us, especially going over lots of information for school. She offered advice on equipment for him to use at school. We also discussed potty training, and what equipment he would need for toileting at home and school. She also sent home a list of power chair feature recommendations.
A representative for the Muscular Dystrophy Association made sure we were getting their mail. We talked about Alex going to MDA camp in a few years (3), and she encouraged us to join them for the MDA muscle walk in Charleston in September.
The complex care pediatrician, Dr, Noretz, covers pretty much any other aspect not covered by the other specialists. He is very knowledgeable but easily approachable and makes you feel very comfortable. I wish more doctors had his bedside manner and were as likable. He referred us to urology for Alex to have surgery in Columbus instead of WV. I had mentioned that I was nervous about Alex having surgery in WV (with special regard to anesthesia), and he reassured me that my fears made sense. He said that normally general anesthesia isn’t a big deal, but with SMA kids, it can get dicey. He said to just have it done up there where they would be more than prepared.
We have been trying to make an effort to mix the many medical trips with fun for our kids. I don’t want Alex and the older boys to only remember the hard and upsetting times, all of the constant trips to doctors, all of the blood draws, spinal injections and examinations that Alex has to go through. We try to give them something good to associate with the bleak times, so we have been pairing trips to the doctor with something fun or exciting. It is either an experience like a stop at the zoo or a treat like a trip to the toy store. Our visits to the MDA clinic mean a 4.5 hour car ride immediately followed by sitting in the exam room at the hospital for 4-5 hours meeting with each specialist. That is a lot for kids to sit through.
As a reward for this trip, we took them to the Columbus Zoo and Aquarium. The Columbus Zoo is home to one of my childhood heroes: Jack Hanna. They also offer free admission to the companion of a disabled person (the person who is pushing the wheelchair). So because I was pushing Alex through the zoo, I received free admission. It rained a misty rain for most of our visit, but it was actually a pleasant day. The temperature was very warm, so the rain wasn’t cold. The rain also kept the crowds home, so we had the zoo to ourselves mostly. It also meant a lot of the animals were right near the viewing glass for us! It was a very nice zoo, that I hope to return to someday.
We are scheduled to return to Nationwide Children’s in 6 months. We are expecting a lot of changes in equipment and care before then and are excited to hopefully surprise his team again with his improvements.