Kids with Vents Facebook Group

We started out as a support group on Yahoo Groups, and we recently added a Kids with Vents Facebook group as a convenience to our families.  The Facebook group is very popular and we already have over 350 members!  If your child uses a ventilator or Bipap/AVAPS, you are welcome to join us!  Teens and young adults on a vent (or Bipap/AVAPS) from a childhood condition or injury are also welcome!  To join the Kids with Vents Yahoo group, just click on the link above the slide show.  To be added to the Kids with Vents Facebook Group, go to: https://www.facebook.com/groups/kids.w.vents/ and click on "Join Group."  Hope to see you there!

Carmen's Story

My name is Liz, and my daughter Carmen is four years old.  Carmen was diagnosed with late-onset CCHS (Congenital Central Hypoventilation Syndrome) when she was 16 months old.

We live in Tennessee, but traveled to San Fransisco in December 2007 to visit family.  Carmen had a cold and was very congested.  In the early morning, after Christmas Day, I woke Carmen to try to get her to cough up her secretions.  She aspirated and went limp in my arms.  My brother-in-law is an ER physician, and he was resuscitating Carmen while my sister called 911.

Carmen was admitted to UCSF Children's Hospital, diagnosed with RSV, and was put on a ventilator.  She failed two extubations, and the doctors were telling us that something else was wrong.  Carmen had every test imaginable (X-rays, MRI, EEG, echocardiogram, and a sleep study, etc.) to rule things out.  The sleep study showed that she was retaining CO2 only while she was asleep.  The doctors began to prepare us for the possibility of Carmen having CCHS, which is a very rare genetic disorder.  She was trached on January 17, 2008, and a week later we learned that Carmen does have a PHOX2B mutation of 20/25.

After 33 days in the PICU at UCSF, we took a medical flight to Vanderbilt Medical Center.  We were there for about a week to learn about trach care and the ventilator, before returning home.  We have never had home nursing care for Carmen, because we have not qualified for any assistance, and my husband's insurance (BCBS) does not cover private nursing services.  I quit my job so I could care for Carmen full time.

Carmen is an only child.  She attends a community based inclusion preschool so she can interact with her peers.  She does not have any developmental delays, so our public school system does not think she need to be in school yet.  They will only begin providing nursing services at school once she begins Kindergarten.  So, I go to school with her to provide her trach care needs and to hook up her ventilator when she naps.  Carmen is strong-willed, bright, sassy, and fun!  Despite her trach, she can be extremely loud, even without her speaking valve.  We look forward to the day she can be decannulated, and either be ventilated via Bipap or with a diaphragmatic pacemaker.

Thanks for reading our story!

Michael's Story

Hello, my name is Michael.  I was born with a genetic disease called Duchenne Muscular Dystrophy.  DMD is a disease that causes the weakening of skeletal muscles, respiratory system, and cardiomyopathy (weakening of the cardiac muscle).  There is no known cure for DMD and the prognosis is death.  When I was a young boy, somewhere around six or seven years of age, I was told that I probably would not live past the age of 19.  I praise the Lord every day, thanking him that I surpassed the doctor’s projection.  Today, I’m blessed; see I just turned 33 this past spring which is a milestone for a person having DMD.

Through the years, DMD has taken its toll on my body.  From the age of seven, when I could still walk, my muscles gradually began to weaken. As a result, I fell a lot because my legs were weak.  It seemed that when I walked on hard surfaces like concrete, pavement, and gravel I would fall.  I continued to stumble and fall till the age of 12 years old, when I became dependent on a wheelchair.  I lived with my parents until I was 21 years old. At that time I was more determined than ever to make something out of my life despite having DMD. I moved to Woodrow Wilson Rehabilitation Center and began studing Mental Health/Human Services. I graduated there in 2001 with an Associates Degree and Certificate in Peer Mediation through Blue Ridge Community College. I was also married to my wonderful wife Robin by then. In that same year I graduated Robin and I rented an Apartment in Stuarts Draft, Virginia. During that time, I lost the ability to feed myself, and I lost a great deal of weight. I also began having breathing difficulties along with severe Central Sleep Apnea. I began using a BiPap Machine at night and eventually a Pneumobelt in 2003. In 2005 I was placed on a Trach and Ventilator after a serious infection. The doctors told Robin that she had to make a choice.  She could either have me trached or they could administer comfort measures and let me die.  They asked me if I wanted to be trached, and I said yes.  I was facing death, but I heard God tell me “It’s not your time, your work is not done; I am not done with you.”  So, I was trached and vented a few hours later.  I started improving immediately, and by the end of that week, I was well enough to go home.

Once I got home, I was scared but glad to be home.  At first, I was not able to get out of bed or eat.  This lasted about three weeks, sleeping and drinking Ensure four times a day.  Finally, I got well enough to get out of bed however still drinking ensure.  A few days later, I said to heck with this stinking Ensure.  I got my attendant to go to McDonalds and get me a cheeseburger and French fries.  Man that was the best burger I ever ate!  Eventually, things got better and I was gaining weight and breathing great, Hallelujah!

Then, about two years ago, I developed Congestive Heart Failure from my ever weakening heart.  I’ve been a Christian for several years, and my relationship with Jesus has enabled me to get through many trials.  I have salvation through him.  This was reality slapping me in the face; it was time to get serious about being a Christian and stop playing Christian.  The news broke my heart; my heart is literally broken which is how the Lord wants it!  I was given a 70 percent chance of dying from sudden death from a heart attack.  This is the second year of that prognosis and my Ejection Fraction is dangerously low at 17 percent, but who cares about numbers.  By the way this ventilator and oxygen are just accessories, and not my life support.  Let me say this, Jesus Christ is my life support!  Amen!

I began to see the annointing God had on my life. I began taking Online Classes in persuit of a BA in Pastoral Ministries. The same year I came up with a Concept Christian Ministry for the Disabled. Today, we are called Spirit Roller Ministries and we are 501c3 Non Profit; we are also an Affiliate with Joni and Friends Ministries. We have been running a very successful program called MedEvange. We take used/donated Medical Equipment then we give it away free of charge to the needy. We also have a Mentoring Service, Couseling, Advocacy and Empowering them with the Word of God.

The latest bout with my health came this past March, when I started having heart rates in the lower 30's. This condition is called bradycardia which means my heart is too weak to keep a regular rhythm.  It was time for device intervention, and the doctors suggested a pacemaker. They said that the Pacemaker would prevent my heart rhythm going below 60 beats per minute; several doctors were skeptical while my Cardiologist remained optimistic, so I had this major decision to make. I chose the Pacemaker to have more time on this earth to serve Jesus and to be there for my wife and mother.  I was scheduled for April 30. They rolled me down to the Heart Cath Lab, slapped the monitors on me and transferred my onto the operating table.  I heard the doctor come in as the meds were administered; I only remember 2 things as I went out, someone turning this crazy music on and the doctor say, “someone bring me my knives.”  I was in surgery for 4 hours before waking up and saying, “Are ya’ll done yet?”  “Closing you up now Mr. Keen,” someone replied.”  The following evening I was so peaceful.  I stayed at the hospital with my mother and we watched the heart monitor and cried for joy; the surgery was a success and my rhythms were slow and steady.  “Mom,” I said “now I have more time to spend with you and Robin.”  “I know,” she said as she rubbed back tears in her eyes.  I woke later that night to my mother whispering, “Thank you Lord for lending me my son a little while longer.”

Kimi's Story

My name is Kimi, and I'm a 31 year old with autosomal dominant Emery Dreifuss Muscular Dystrophy.  EDMD is one of the more rare muscular dystrophies.  I was actually first diagnosed with Spinal Muscular Atrophy, but was recently re-diagnosed with EDMD (long story).  I've experienced a faster progression of EDMD than is typical.  I was able to walk as a young child, but received my "driver's licence" at the age of 5.  I was completely wheelchair dependent by the age of 8.  I started experiencing respiratory issues at the age of 11.  From the age of 11 to 13, I was in and out of the hospital for pneumonia.  My saving grace was the Bipap.  Once I started using Bipap at night at the age of 13, I felt much more rested and the occurrence of respiratory illness decreased tremendously.  During this time I also started experiencing cardiac issues, which at the time were thought to be unrelated to my neuromuscular disease.  It later turned out they were part of the EDMD progression.  

I was pretty stable until my Junior year of college.  I got very sick during the 2nd semester of my Junior year, and ended up being hospitalized for 6 months.  This is when I was trached and vented.  I also received my G/J tube during this hospitalization.  I use the LTV 950 and have a 5.5 pediatric Bivona cuffed trach.  At first I was on the vent 24/7, but after 4 years I was able to sprint off the vent during the day.  I now still sprint off the vent on a Passy Muir valve during the day, but must take vent breaks.  In addition to the vent/trach, I have a pacemaker, severe scoliosis, motility issues, GERD, and chronic abdominal, neck, and back pain. 

Now to the fun stuff... I enjoy hanging out with my great friends, shopping, mini scrap booking, card making (visit my Etsy site www.craftykfox.etsy.com), music, and Dunkin Donuts Iced Mocha Lattes.  I graduated Summa Cum Laude with a BS in Bio/Chem from Immaculata Univ, and pursued my Masters in Bioethics at UPENN.  I run a world wide Yahoo group for people affected by SMA called SMAFRIENDS.  I also run the Yahoo group for my college sorority's alums.  I volunteer for the state of PA by serving on a committee that advises the Governor and the Secretary of Public of Welfare on the Medicaid Waivers which allow disabled citizens to live independently in the community by providing them with nurses and/or attendants.  I mainly advocate for those on vents.  Another highlight of my life was being engaged to a wonderful guy named Eric, who unfortunately passed away before we could get married. 

My number one goal in life is to inspire others to do their best.  I personally need to learn how to be thankful for what I have, and hopefully in the process teach others to do the same.  Oh, and I don't want to grow up and be just ordinary!