Ventilator-Assisted Living©

Spring 2003, Vol. 17, No. 1


Luke's Progress

Mary Garrett

Our son Luke has Pompe's disease, a glycogen storage disease, in which the acid maltase enzyme necessary to break down glycogen and convert it to fuel is lacking. The glycogen is stored in the lysosomes of the cells, causing muscle damage and death on the cellular level. Luke's respiratory and skeletal muscles are affected. His profile appeared in IVUN News, Fall 1996.

With Luke's discharge from the PICU at Albany Medical Center in Albany, New York, in May 1995, my husband Rick and I believed that there would be a viable treatment for our son's disease within six months to a year. We thought we might be able to care for him at home for as long as two years.

Now, almost eight years later, we are still surviving, learning, struggling, and glad that we decided to bring Luke home. We have had many ups and downs, yet are happy that Luke has managed to avoid hospitalization since his initial discharge. We were told that Luke would probably die before age 2; he will be 10 years old in July.

Luke left the hospital with an LP10 and a CPAP unit to give him continuous flow. After meeting with John Bach, MD, in 1997, we changed his ventilator settings and eliminated the CPAP unit, enabling Luke to be more "portable." Two years ago, Luke obtained the LTV™ 950 ventilator (volume control) and that made him yet more "portable." With a recent growth spurt, Luke needed his ventilator settings adjusted. Luke is very helpful in managing his own care and works the controls on a manual CoughAssist™.

Photo of LukeLuke has a busy life. He is home-schooled for 2½ hours each weekday morning and is hooked up via computer to his regular fourth-grade classroom. (He is an A student.) He uses a Passy-Muir speaking valve and vocalizes very well.

It takes two people to get Luke into a rigid body jacket to help maintain his posture and minimize his scoliosis. He is strapped onto a standing frame for 1-1½ hours each day and receives nebulizer treatments, chest percussion, and nutrition through gastrostomy tube feeds every four hours. Daily physical and speech therapies round out Luke's schedule, but I think that it is precisely this schedule that has kept him well for so long.

Luke's siblings, now 16, 14, and 12, are loving, patient, and tolerant companions and mentors and bring the world to him.

So, we live from day to day, fight our battles when we have to, enjoy our family, and hang onto our hope that Luke will soon receive enzyme replacement therapy that shows promise in halting the progression and may-be even reversing his disease.

Phrenic Nerve Pacing

Christopher Reeve, a ventilator user due to a horseback riding accident in 1995, recently underwent experimental surgery by a new procedure (IVUN News, Summer 2001, Vol. 15, No. 2), developed at Case Western Reserve University and MetroHealth Medical Center. The procedure involves implanting phrenic nerve pacers using laparascopic (minimally invasive) surgery, instead of a thoracotomy, a major chest operation that is riskier and more costly.

In phrenic nerve pacing, electrodes implanted near the phrenic nerve connect to a control box worn outside the body to electrically stimulate the diaphragm, the primary muscle needed for breathing, and control breathing.

The main advantage of phrenic nerve pacing is the elimination of the need for mechanical ventilation. Other advantages include improved vocalization and sense of smell, although a tracheostomy is usually still necessary because of possible upper airway collapse during sleep and sudden operational failure.

The system works best in people with high spinal cord injuries who have intact phrenic nerves and normal chest walls. It is not effective for people with neuromuscular disease such as post-polio syndrome because of chest wall abnormalities and because tidal volumes cannot be routinely modified or as precisely controlled.

IVUN News Now 12 Pages

The Board of Directors of Gazette International Networking Institute (GINI), the governing organization of IVUN, decided to discontinue publication of its bi-annual Rehabilitation Gazette in 2003 and to expand the quarterly IVUN News to 12 pages, and to rename the publication Ventilator-Assisted Living. Subscribers to the Rehabilitation Gazette will receive this expanded newsletter.

The inaugural issue of the biannual IVUN News in 1987 introduced the world to the International Ventilator Users Network (IVUN). IVUN is a worldwide network of ventilator users, health care professionals, and ventilator equipment manufacturers and dealers experienced in home mechanical ventilation. Polio survivors form the nucleus of IVUN because of their use of mechanical ventilation, such as the iron lung, during the polio epidemics. Their experiences with home ventilation are invaluable for persons with high level spinal cord injuries, muscular dystrophy, ALS, and other neuromuscular diseases, and for the growing number of ventilator-assisted infants and children. Holding fast to the network's mission, IVUN News expanded to quarterly issues in 1998.

Articles from IVUN News are online to provide an immediate and reliable source of information for the home mechanical ventilation community. The comprehensive IVUN Resource Directory (now Resource Directory for Ventilator-Assisted Living) is also online and is updated continually.

Watch for the first issue of Ventilator-Assisted Living … coming in the summer of 2003.

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