General Description
The ALS Clinic at Penn State Milton S. Hershey Medical Center is a multidisciplinary center for the evaluation and treatment of amyotrophic lateral sclerosis (ALS) and related disorders. Professional personnel representing a variety of interrelated disciplines care for the needs of each patient.
The purposes of the ALS Clinic are:
1) To establish a definitive diagnosis in patients referred for possible ALS;
2) Once a diagnosis is established, to provide comprehensive care in a supportive and understanding environment which will enable the patient to attain the best possible level of neurological functioning and the best possible quality of life.
Our ALS clinic is the only service of its kind in Central Pennsylvania, and was designated an ALS Association Certified Center of Excellence on October 19, 1998. We were re-certified on August 8, 2006 after passing a rigorous re-evaluation. About 70-80 new patients with ALS are diagnosed annually, and about 160 are followed long-term at any given point in time.
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What to Expect
First visit:
Patients being seen for the first time interact primarily with a neurologist who is an expert in neuromuscular diseases. The goal is to establish with as much certainty as possible the specific diagnosis. Most of our new patients have been evaluated previously by one or more neurologists, and often have been told that they may have ALS, or that they have a neuromuscular disease. At our clinic, they are given a comprehensive neurological history and examination, and all of their records of past tests are reviewed. Using that information, our neurologist orders various tests as needed. This may include studies of blood and urine, and radiologic studies (x-rays, CT scans, and MRI scans). Most patients are scheduled to undergo electrodiagnostic testing (EMG and nerve conduction studies) in our EMG laboratory. More involved testing, such as a lumbar puncture (spinal tap), muscle biopsy, or nerve biopsy may be scheduled. After review of these tests by the neurologist, patients are then seen back, usually accompanied by family and other caregivers, for a detailed discussion of the diagnosis with the neurologist. If ALS is confirmed, information is provided and treatment options are reviewed including established and experimental treatments. The ALS nurse is often present at that visit as well. If not, a follow-up evaluation with her is then scheduled. The neurologist and nurse work together with the patient and family to establish a plan for treatment of the disease and management of symptoms.
Follow-up visits:
Patients with ALS are seen, on average, once every 3 months in our multidisciplinary clinic. During these appointments, a patient and his or her family remains in one examining room for about 3-4 hours while various members of our team come in to perform evaluations. Precisely which team members see a patient on any given day depends on the type of problems the patient is having, and is determined by the neurologist and nurse, in consultation with the patient and family. All patients receive measurements of their weight and breathing capacity, as well as evaluations by the neurologist and nurse. They are then seen by some or all of the following team members: physical therapist, occupational therapist, speech therapist, nutritionist, respiratory therapist, mental health specialist, social worker, and pastoral care counselor.
Interaction with Your Primary Care Physician
Patients must continue to see their own primary care physicians while utilizing the ALS Clinic's specialized services. The primary care physician receives a written update from the neurologist summarizing the ALS team's recommendations after each clinic visit.
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Goals of the Clinic
- Serve as a center for the diagnosis, treatment, and follow-up care of patients with amyotrophic lateral sclerosis (ALS)
- Coordinate care with primary care physicians.
- Provide specialized services for patients with ALS and related disorders
- Maximize patient function by utilizing a multidisciplinary approach
- Provide each patient with information concerning the disease process
- Offer therapeutic drug trials
- Keep patients and families abreast of research, including research in our own Motor Neuron Disease Research Center
Click Here for Individual Photos and Bios!
ALS Center Director and Neurologist
ALS Clinic Nurse Specialist, Regional Nurse Coordinator
ALS Clinic Nurse
Clinical Dietitian
- Annette Morrison, RD, LDN
Clinical Neuropsychologist
Licensed Professional Counselor
Occupational Therapist
- Pamela Hudson, OTR/L
- Zahira Paul, MS, OTR/L
Pastoral Care Counselor
- Barbara Brunk Gascho, RN, CPN, Chaplain
Physical Therapists
- Christine Brussock, PT, DPT, MS
- Marty McKeon, MPT
Social Worker
Speech-Language Pathologist
- Erica Luckenbill, MS, CCC/SLP
Neurologists
Neuromuscular Nurse
Pulmonary Medicine Specialist
Research Coordinators
Walk to D'Feet ALS
This year's Walk to D'Feet ALS will be held on Saturday July 17, 2010 at Harrisburg's City Island. This event is the ALS Association's largest fund raiser of the year. Organize a team, or grab a yellow shirt and join the Hershey ALS Clinic and Research Team! Register online at www.alsphiladelphia.org.
Click here to see event photos from 2009.
ALS Resource Groups
Individuals with ALS and their family members are invited to attend this monthly support group, sponsored by the ALS Association, and facilitated by nurse counselor Judy Lyter. The groups include information related to ALS, as well as a social community. For more information, please call (717) 566-1700.
- Harrisburg Resource Group: Meetings are held on the first Thursday of the month from 6:30 - 8:00 PM at the Hershey Medical Center in the reception area of the Neurology Clinic at 30 Hope Drive, Entrance B, Suite 1300 (see Directions).
- Lancaster Resource Group: Meetings are held on the third Saturday of the month from 10:30 - 12:00 noon at the Ephrata Public Library.
Upcoming Events
The Greater Philadelphia Chapter of the ALS Association sponsors many events for patients, families, and healthcare providers. Click here for the calendar of events.
Pasta for PALS Fundraiser
Thanks to all who supported the seventh annual Pasta for PALS fundraiser! The all-you-can-eat spaghetti dinner and silent auction event was held on Sunday, April 5, 2009 at Dafno's Italian Grill in Hummelstown, PA. Proceeds go to support ALS research at the Penn State Milton S. Hershey Medical Center. We are sincerely grateful to JoAn Zimmerman and her family, the owners and employees of Dafno’s, and the many other volunteers for all their time and effort in making this event a success.
Read Dr. Simmons' Recent Columns in the Newsletter from ALS Association Greater Philadelphia Chapter
Hershey-China ALS Collaboration
The collaboration between clinicians and researchers in Hershey and Tianjin, China continues to strengthen. Two physicians and one administrator from the First Center Hospital in Tianjin, China visited from January through March of 2009. One physician worked with Dr. Connor in his laboratory, while the other worked in the ALS Clinic and the neuromuscular clinic with Dr. Simmons, and with the other neurodegenerative physicians here at the HMC.
This ongoing collaboration began with an invitation from the physicians in Tianjin to ALS Neurologist Dr. Zachary Simmons and ALS basic scientist researcher Dr. James Connor. Drs. Simmons and Connor visited Tianjin in the summer of 2008 to help the institution with their goal of establishing a center for neurodegenerative disorders. The visit in early 2009 aimed to further this goal, as the Tianjin physicians wish to use our ALS center as a model for their own. ALS in China has not been well-studied. We are hoping that similarities and differences between patients in China compared to those in the USA will strengthen our research into causes of, and treatments for, ALS, and help us to better understand the disorder.
CURRENTLY ENROLLING STUDIES
Ceftriaxone
Research subjects for the ‘Clinical Trial of Ceftriaxone in Subjects with ALS’ are currently being recruited in the United States. Six hundred research participants with Amyotrophic Lateral Sclerosis (ALS) are being recruited for this double-blind study of Ceftriaxone. Ceftriaxone is a cephalosporin antibiotic that is being tested for its safety, tolerability, and effect on the symptoms of ALS. Interested subjects should contact Beth Stephens, study coordinator, at (717) 531-0003, extension 283395 or email hstephens1@psu.edu
Biomarkers for ALS
We are currently seeking individuals to participate in a specimen collection study under the direction of Dr. Zachary Simmons.
The purpose of the research study is to collect blood samples from healthy subjects, and both blood and cerebrospinal fluid (CSF) samples from people with amyotrophic lateral sclerosis (ALS, or Lou Gehrig’s disease), motor neuron diseases which are related to ALS, and other neurodegenerative diseases that may mimic ALS. Individuals with one of these disorders who participate in the study may choose to donate only blood or to have a lumbar puncture (spinal tap) performed to donate CSF, or to donate both. Through comparison of these samples, researchers hope to learn more about the underlying causes of ALS, as well as find unique biological markers which could be used to more efficiently diagnose ALS and to develop new therapies.
Participants must be at least 40 years of age and be able to answer brief questions about their medical and family history, as well as be willing to have blood and/or CSF drawn for the study. If interested, or for more information, please contact our coordinator, Beth Stephens, at (717) 531-0003, ext. 283395 or e-mail us at
hstephens@psu.edu. You may also contact the Coordination Center via the Northeast ALS Consortium (NEALS) for more information about this study at
mghneuroclinicaltrialsunit@partners.org or toll free at 1-877-458-0631.
'This research study has been approved by the Institutional Review Board, under federal regulations, at Penn State College of Medicine, Penn State Hershey Medical Center.’
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There are a variety of studies being undertaken in our ALS clinic which directly involve our patients, and are designed to better understand a number of factors which directly impact on their care.
KNS-760704
The Phase II trial of KNS-760704 trial is currently underway at our site, and enrollment is now closed for this study.
Quality of Life in Patients With ALS and their Caregivers
We are proud to report that our collaborative effort on the development of an ALS-specific questionnaire used to assess quality of life (QOL) was presented at the International ALS/MND meetings in Philadelphia in December, and then published in the journal Neurology in 2006. The instrument is called the ALS-specific quality of life questionnaire, or ALSSQOL.
We are now undertaking a multi-center study at 12 centers around the country to validate a shortened version of instrument (the ALSSQOL-R), in which we plan to enroll 280 participants. Once this study has been completed, we plan to make the instrument available to physicians and other health care professionals for their clinical use. We have also looked at the issue of QOL in caregivers of individuals with ALS, and are now trying to integrate this with our knowledge of QOL in ALS.
These studies represent a collaborative effort between two psychologists (Dr. Stephanie Felgoise at Philadelphia College of Osteopathic Medicine and Dr. Barbara Bremer at Penn State Harrisburg), our nurse Sue Walsh, our research coordinators Beth Stephens and Ally Brothers, and a number of students.
ALS-Specific Quality of Life (ALSSQOL) Questionnaire
Multidisciplinary ALS clinics are increasing in number, and have been demonstrated in Europe to result in higher quality of life (QOL) and prolonged lifespan for those who attend such clinics vs. those who do not. No such comparisons exist in the United States. During 2008, we conducted a research study that surveyed a cross-section of ALS patients in the Unites States in order to assess and compare QOL, and treatments and services received, in multidisciplinary clinic participants vs. non-participants. This research was recently presented at the International Motor Neuron Disease Association annual conference in Birmingham, England.
QOL in multidisciplinary ALS clinic patients in the United States is similar to that of non-clinic patients. However, ALS clinic patients used a far broader variety of treatments and services, including life extending treatments like NIPPV and riluzole. This raises a number of questions: Is more intensive care “better” care? In order to maintain QOL, is more intensive care needed by some patients than by others? Do patients gravitate toward a care experience which is most suitable for them? Can a more intensive ALS care experience extend lifespan? We are currently planning further studies to answer these questions, but it seems clear that the relationship between QOL and multidisciplinary care is a complex one.
Changes in Thinking in ALS
For many years, it has been widely believed that thinking and language are spared in ALS. It is now becoming increasingly more accepted that a significant number of individuals with ALS develop changes in their thinking. If severe enough, this is classified as a frontotemporal dementia, or FTD. This has profound implications with regard to decision-making by these individuals. We have published research about such changes (see publications and abstracts).
- New Focus for Comprehensive Cognitive Assessment
In addition to completing the brief (20-minute) cognitive screen exam in ALS Clinic, many of our patients in the past four years have also undergone a comprehensive (2 hour) examination of problem-solving. This comprehensive set of problem-solving tests has been instrumental in identifying the sometimes subtle cognitive and behavioral changes that can occur with ALS. It has also been helpful in assessing the utility of the shorter screen exam. - The focus of the comprehensive examination has recently changed. The new set of assessments has been selected for its utility in differentiating between the three subtypes of ALS-related cognitive-behavioral change. It will allow for detection of subtle language changes and/or behavioral changes, as well as associated changes in executive functioning.
- Multicenter Cognitive Study
The brief 20-minute screen of cognitive functioning designed by our neuropsychologist, Dr. Flaherty-Craig, has become a vital tool in our clinic for detecting the subtle changes that sometimes occur in ALS. It is now being used at ALS clinics around the country as part of a multi-center research study.
Turning Research into Practice - Evidence Based Practice
Evidence-Based Practice is a method of providing clinical care to patients, by systematically incorporating solid research evidence with clinician expertise. This process has inspired two collaborative teams, made up of clinicians and researchers alike from the Hershey ALS Clinic, The Pennsylvania Hospital ALS Clinic, Penn State Harrisburg, and The Philadelphia College of Osteopathic Medicine. Each group focuses on a particular aspect of patient care, in order to better meet the needs of our patients. A brief look at each group’s progress to date:
- The EBP Caregiver Group: What can we do to support caregivers of patients with ALS?
Formed in 2007, the EBP Caregiver Group has made steady progress in their efforts to develop ways of supporting caregivers of ALS patients. The team has developed a caregiver assessment which has recently been added to the clinic routine. The caregiver assessment was created by culminating information from multiple avenues, including an extensive literature review, insight from caregivers themselves, and expertise from ALS treatment team members. This assessment has received an overwhelmingly positive response so far, and is expected to help to connect caregivers with the support that they need. We look forward to the many ways in which it will improve the care provided by the Clinic team. - The EBP Cognitive/Behavioral Group: What do we recommend to patients and caregivers for the various concerns associated with ALS and cognitive-behavioral change?
Formed in 2008, the EBP Cognitive/Behavioral Group is currently in the final stages of the information-gathering component of the project. Efforts are underway to gather direct caregiver experience in a structured focus group setting. The themes culled from this interview will complete our “chart of evidence,” which also includes the results from an extensive review of the literature, in addition to the expert opinions of our ALS clinicians. Once the information-gathering piece is complete, the group will move on to begin developing interventions for ALS patients and their caregivers.
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There continues to be close collaboration between our clinical program and the research laboratory of Dr. James Connor. Under his leadership, a potential genetic risk factor for ALS has been identified. Termed the H63D HFE genetic variant, this is a variation of the hemochromatosis gene, a gene involved in iron metabolism but also involved in the immune system function and inflammatory responses. He and his co-workers are further exploring the association of this genetic variant and others with ALS (funded by the ALS Association). This work has developed into an extended project with investigators at the NIH. We are also exploring the functional effects of HFE gene variants (funded for a second straight year by Judith & Jean Pape Adams Charitable Foundation). A cell model has been developed by Dr. Sang Lee to understand the functional consequences of the mutants of the protein in cells. Ryan Mitchell, an MD, PhD student worked on these cells for his doctoral dissertation. Mitchell found that the cells with the H63D mutation secrete more glutamate, a neurotransmitter that in normal amounts stimulates a nerve cell to pass along information to the next cell. Too much of this neurotransmitter can cause cells to become over-stimulated and die. Currently the only FDA approved drug for ALS, Riluzole, targets the over stimulation as a potential mechanism of neuronal cell death in ALS. This research from Connor’s group suggests a mechanism by which carrying the H63D mutation can be a risk factor for ALS. The work has recently been accepted for publication in Neurobiology of Aging. Mitchell has also shown that an inflammatory protein, known as MCP1, is increased in cells carrying the H63D mutation and this protein is also elevated in the CSF of patients with ALS. This study has recently been accepted in the Journal of Neuroinflammation. Therefore it appears that the H63D mutation may also exacerbate inflammatory reactions. Both of these results support the hypothesis that H63D mutations in the HFE gene are as risk factor for ALS by creating a permissive cellular environment for the event (biological or genetic) that triggers the onset of ALS.
Additionally, the Connor laboratory, in conjunction with our ALS clinic, are using leftover muscle biopsy tissue, leftover cerebrospinal fluid, and blood samples collected from ALS patients seen in our clinic and other volunteers to identify biomarkers to aid in the diagnosis and understanding of ALS. This work could not be done without the support of our patients, their families, and their caregivers. The results of this study have recently been published in Neurology one of the premier journals in our field. The study found a profile of proteins in the CSF that was consistent with the clinical diagnosis of ALS. The advantage of having a supportive diagnostic biomarker profile will enable physicians to start treatment options earlier for individuals diagnosed with ALS or suspected ALS. The editors of this journal chose to highlight our article and wrote an accompanying editorial that addressed the importance of this work and the search for a biomarker for ALS in general. Recently Mitchell has completed a study of serum, much easier to obtain than CSF, and the paper has been submitted for publication.
Mitchell has recently completed his doctoral thesis and has returned to medical school to complete his work for his medical degree. We wish him much success. He expresses his gratitude to the patients for providing tissue and fluid samples and inspiration and to our donors who supported him financially so that he was able to pursue a Ph.D.
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To Make an Appointment
Please call 1-800-292-3332 or 717-531-4191
To Speak Directly to the Medical Director (Physicians Only)
Please call the MD Network at (800) 233-4082 or (717)-531-5880 to speak with Dr. Simmons
All Other Contacts
Please call 717-652-2800 or e-mail sue@alsphiladelphia.org. Our nurse Susan Walsh will assist you.
Click here for a map
Penn State Milton S. Hershey Medical Center is located in Hershey, within Derry Township, Dauphin County, 12 miles east of Harrisburg on Route 322. The ALS Clinic is located within the Neurology Department in the new East Campus outpatient building. There are signs for Entrance A and Entrance B. Enter via Entrance B and follow directions to the Neurology clinic.
From the Pennsylvania Turnpike: Use exit 266 to route 72 north to route 322 west.
From Route 81, follow signs for Hershey/Route 322
If you are traveling west on Route 283, take the Hershey/Elizabethtown Exit to Route 743 to Route 322 west.
From Route 83, follow signs to Hershey/Route 322 east.
We are grateful for the support that we receive from a variety of sources, without which the ALS Clinic could not accomplish its missions.
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We also receive support from The ALS Association national office for our clinical activities and have been the recipients of several ALSA Clinical Management Grants for support of our Quality of Life research.
The Zimmerman Family Love Fund has generously provided support for our clinical and research missions through the tireless efforts of the Zimmerman family.
The Paul and Harriett Campbell Fund for ALS Research provides essential and substantial financial support for ALS research at Penn State College of Medicine and the Penn State Hershey Medical Center.
The support of the Penn State General Clinical Research Center at the M.S. Hershey Medical Center is appreciated.
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We gratefully acknowledge the many private donations we have received from our patients and their families and friends.
Would you like to support our efforts?
Those wishing to make a donation to support our clinical or research work may send a check, payable to the Pennsylvania State University, ALS Clinic, to:
Zachary Simmons, MD
Director, ALS Center
Penn State Milton S. Hershey Medical Center
Department of Neurology, EC 037
30 Hope Drive
Hershey, PA 17033
Large gifts can be coordinated through Penn State.
Please contact Dr. Simmons directly to discuss this.
