Worst Birthday Ever

Cheryl Lewis with her toy poodle Leo, seated in her power wheelchair

Normally my blog post answers a community member's question(s).  In this post, I'm taking a departure from that.  - Andrea Klein

​Everyone has had an awful birthday, right? Maybe you had a party with poor turn out or were sick that day. December 10, 2007 was my worst birthday ever and is seared in my memory because my middle sister Cheryl died.  

Instead of unwrapping gifts or eating cake, I was standing in a hospital hallway hearing, "I'm so sorry; she just passed. She fought hard." It was a day of many tears and shock; my birthday was the last thing on my mind. Cheryl had been hospitalized since the evening of December 1 for a respiratory crisis related to what was then known to us as Limb Girdle Muscular Dystrophy. (Now there are more than 30 identified subtypes of this disease.)
 
In hindsight and with new understanding gained from the books of Dr. John R. Bach, I realized hospital ER and ICU staff hastened Cheryl's death. They failed to check her carbon dioxide level and focused on her low oxygen saturation;  that only told part of the story. They ignored her obvious symptoms of carbon dioxide retention or hypercapnia. She had explained she was nodding off and seemed to be losing consciousness for seconds to a few minutes at a time, even while talking or eating. She showed them her newly swollen ankles, an issue that started towards the end of November.  She said she had been having hand tremors for months. She also told them she could not breathe while lying flat, but she wasn’t sure if that was anxiety since she physically could not get up from a reclined position unaided. 

Prior to her ER visit, when her primary care physician was stumped and could only help move her neurology appointment up by a week or two, we searched the MDA USA website. The information about breathing issues was specific to ALS and Duchenne.  Knowing she had neither, this was of no help to us as we expected the treatment would be different.  I turned to Google and found an article on respiratory acidosis. It covered some of the symptoms she had been experiencing. The hospitalist in the ER agreed to take the article, but he seemed uninterested.  In his defense, the article didn't mention Muscular Dystrophy as a potential cause. 

In the ER, clinicians started Cheryl on supplemental oxygen without mechanical ventilation. It was the catalyst for a firestorm of medical errors.  Around 10 p.m., my Mom called to let me know that she had perked up and felt much better.  The plan was to observe Cheryl overnight and discharge her with an order for oxygen and the directive to see a pulmonary specialist.  That did not happen.  Settled in her hospital room, she began to speak to family members in an uncharacteristic way.  Then she had a verbal outburst with a clinician who wanted to do an arterial blood gas, (something that should have occurred when she arrived in the ER).  

Soon after, she went into respiratory arrest.  The oxygen she was getting via nasal cannula created more "waste product" of that inhaled air, and this carbon dioxide backed up in her lungs that were too weak to expel it at an efficient rate.  She had to be intubated which took multiple, difficult attempts. It was a pediatric tube that finally fit her narrow airway. Up until the tube went in, an ambu bag (manual resuscitator) was used, and an eardrum ruptured and bled. She was quickly taken to ICU, and that was when my Mom called again. Our family rushed to the ICU waiting room where we were sleep deprived, crying, and praying.

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​“Throughout the entire ICU stay, one thing was constant:  uncontrolled, high levels of carbon dioxide.”

Cheryl Lewis, seated in her power chair with her dog Leo, next to her Christmas tree in 2006.

Over the next few days, Cheryl was in ICU on mechanical ventilation with supplemental oxygen and sedatives. A few days into the stay, she had an emergency tracheotomy after another close call with respiratory arrest and no improvement in her high carbon dioxide level. She developed blood clots in the surgical site and had a bronchoscopy to remove clots from her lungs. She had a suspected allergic reaction to a drug used to reduce retained fluid and then developed pneumonia. A culture was taken from a line placed in her femoral artery and was found to be a severe infection known as sepsis. Next she suffered from lung collapse, first one and then a second lung. Throughout the entire ICU stay, one thing was constant:  uncontrolled, high levels of carbon dioxide. It was multiple organ system failure that delivered the final blow to her body. Around 7 a.m. on December 10, she died.    

It's so painful that we did not recognize incomplete or wrong care was being provided prior to her respiratory crisis and during it.  You know what they say about hindsight being 20/20. We were at the mercy of our small town hospital. They didn't know much about Muscular Dystrophy and apparently nothing about the associated respiratory involvement. That's what I want to stress to you. The burden, as if you needed anymore, is on you and your loved ones to understand how neuromuscular disease can weaken the muscles we use to breathe and cough; what makes this better; and what can worsen it like unventilated supplemental oxygen. (Learn more about the dangers of unventilated supplemental oxygen at https://breathewithmd.org/oxygen-caution.html.) The less you know about respiratory muscle weakness, the higher the risk for poor outcomes, unless the clinicians you see have experience with neuromuscular pulmonology.  

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Cheryl Lewis with her parents and two sisters on Father's Day in June, 2006.

Some are blessed to receive amazing medical care by clinicians who do in fact understand breathing/respiratory muscle weakness. However, throughout the years, I’ve come to understand that's uncommon, particularly in rural US communities.  

Awareness has increased in the 13 years since Cheryl’s death, in part due to my commitment to share her story and educate my peers. Some nonprofits for specific neuromuscular conditions are including breathing weakness information on their websites, and the quality and detail of this varies. The bottom line is that death from respiratory failure is preventable when proactive measures are taken and continued appropriate care is received. 

I frequently meet families through Breathe with MD, Inc. that are on the verge of or living through a respiratory crisis like Cheryl’s. It takes me back to those difficult days with Cheryl's hospitalization and saddens me that with all of the advances we've made in research and treatment, this is still occurring. That is why Cheryl’s story must continue to be told. Her life was cut short at age 38 because the medical community failed her. It’s failed many others like her, young and old, before her death and after it.  Her death on my birthday devastated my family, but we rejoice that some good has come from it. Her death led me to create the mission and programs of Breathe with MD, Inc.  

In memory of Cheryl today and to make this my "best birthday ever," I encourage you to get involved with my 501(c)(3) nonprofit public charity. Below are some ways you can do that.

• Make a donation to Breathe with MD, Inc. in Cheryl's memory, in the memory of a loved one, or in honor of someone. (My Birthday Facebook fundraiser will be open through December 20 at https://www.facebook.com/donate/1156009338265690/​.)
• Share posts from our social media (Facebook, Twitter, Instagram and/or YouTube).​
• Share your own breathing muscle weakness story through our website. E-mail us at Info@breathewithmd.org to discuss this.
• Join our support group, if you live with a neuromuscular disease or have a loved one who does.
• Participate in the webinars we offer, and visit our booth when we attend events and conferences.  
• Commit your time and/or talent to be part of our board of directors or to assist with a project or program.  

Cheryl's life ended on this day 13 years ago, but her memory and story lives on in the mission of Breathe with MD, Inc.