Question

Dillon is a 4-year-old boy with spinal muscular atrophy who you follow in your clinic. He was discharged from the hospital yesterday after presumed aspiration pneumonia. You are seeing him for a follow-up clinic visit tomorrow and have just reviewed his discharge summary. Dillon required supplemental oxygen and increased frequency of his nebulized medications while in hospital, but at discharge, he was back to his baseline respiratory status. He is completing a 10-day course of amoxicillin-clavulanate. The Pulmonary team was consulted during the admission and recommended that he use a cough assist machine at home to help with airway clearance, which was arranged by case management. During the admission, Dillon was also found to have iron-deficiency anemia and an iron supplement was started. He required an increased dose of polyethylene glycol 3350 while in the hospital due to worsening constipation after starting the iron supplement.

List 4 potential issues that may arise for Dillon post-discharge that you should inquire about.

2)List 3 risk factors for issues post-discharge for CMC.

Answer 1

Introduction:

Spinal Muscular Atrophy is aautosomal recessive genetic disorder impacting the Musculoskeletal system leading to pregressive muscle weakness.

Part 1

List 4 potential issues that may arise for Dillon post-discharge that you should inquire about

Issue 1- Oxygen saturation Dillon required supplemental oxygen and increased frequency of his nebulized medications while in hospital. Ask and inquire about signs of decreased oxygen saturation:-

• Changes in the skin colour
• Confusion
• Cough
• Fast heart rate
• Rapid breathing
• Shortness of breath

Issue 2- Acid refux (Heart Burn) As the child is on Cough assist machine along with Iron supplements. Both of which can aggravate the Acid reflux in Dillon and can put his at more risk of aspiration pneumonia.

Issue 3 - Dehydration - As Dillon required an increased dose of polyethylene glycol 3350 while in the hospital due to worsening constipation after starting the iron supplement, he should be checked for chances of dehydration as polyethylene glycol 3350 causes water to be retained in stool and incresased dose of the same medicine if not met with increased oral intake of fluid can cause dehydration.

Issue 4- Signs of reinfection- As Dillon is completing a 10-day course of amoxicillin-clavulanate check and inquire about sign and symptoms for persistance or reoccurance of pneumonia.

Part 2

CMC stands for children with medical complexity or children with medically complex conditions. they require the highest level of services and support for their health condition to stabalize.

List 3 risk factors for issues post-discharge for CMC

Risk 1- Risk of reinfection related to decreased muscle strength.

As SMA is a progressive disease the chances of breathing problem to increase is more. The muscle weakness associated with SMA makes it difficult to cough and clear lung secretions and make the child more susceptible to respiratory infections. Repeated infections can permanently damage lung tissue causing lung fibrosis hence complicatinh the situation.

Risk 2- Risk of imbalanced nutrition related to swallowing difficulty

Family should be adviced to monitor and chart Dillon's growth and weight graph to check for any deviation from normal Growth and development. Also if the child suffers from  constipation, diarrhoea. acid reflux or swallowing difficulty they shoul immediately seek medical help.

Risk 3- Risk of redcuced movement related to progressive muscle weakness.

In SMA progressive muscle weakness may be evident therefore family should be educated about people they can reach out for help like Physical therapists, occupational therapists, speech therapists etc. Recommend exercises and tools that may help child maintain the best posture for lung function and eating, and Movement and Daily Activities