FEEDING TUBES ARE NOTHING TO FEAR, SAYS MDA CLINIC DIRECTOR

Robert McMichael

Q: What is a feeding tube? Why does it have so many confusing names?

A: A feeding tube is a small, flexible tube, about the diameter of a pencil, used to allow liquid nourishment to enter the stomach directly, bypassing the mouth, throat and esophagus. The feeding tube is often called a PEG tube, which stands for percutaneous (through the skin) endoscopic (into the GI tract) gastrostomy (hole in the stomach) tube. It's also simply called a gastrostomy tube, or g tube.

Q: Why consider a feeding tube in ALS?

A: Many people who have ALS will develop dysphagia (difficulty swallowing) at some point. Swallowing can go from adequate to significantly impaired in months. This can lead to malnutrition and weight loss from inadequate protein and calorie intake. When this occurs, the body uses muscle as a source of energy and protein. This accelerates the progression of weakness.

A feeding, or PEG, tube can restore adequate protein and calorie intake.

One of the risks of dysphagia is that food or liquid taken through the mouth may go down the trachea (windpipe), rather than down the esophagus to the stomach. This is called aspiration. Aspiration causes a high risk of infection in the lungs (pneumonia). Thin liquids and foods that crumble are especially likely to be aspirated.

Q: What's the right time to put in a feeding tube?

A: Because of the nutritional deficiencies and the risk of aspiration, I prefer placing a PEG relatively soon after dysphagia symptoms begin. Another reason for placing the PEG early is that progression of respiratory weakness often parallels the dysphagia. When respiratory function is better, the procedure can be done with less difficulty and less risk of respiratory problems immediately following tube placement.

Q: Can you still eat and drink if you have a PEG tube?

A: The gastrostomy tube creates an alternate route of entry into the stomach but doesn't affect oral intake. In the early stages of dysphagia, it can be used to supplement oral intake of food and fluids. Later on, when it's dangerous or impossible to consume any food or drink orally, it can be used as the sole method of nutrition. Many readily available liquid pre- parations designed to provide a complete and balanced diet can be used as the sole source of nutrition.

Q: Can medication be given through a PEG?

A: When a person has trouble swallowing, he may be unable to swallow pills. Many medications can be crushed, dissolved and given through the PEG. Some capsules can be opened and sprinkled into water or liquid food. Medication taken this way should be well mixed with water, so that it doesn't remain in the tube, where it might get hard and block it.

If there is a liquid preparation of a medication, it can easily be administered through the tube.

Some medications come in patches and are absorbed through the skin. These include certain hormone supplements, some heart medications (nitroglycerin) and one blood pressure medication.

Check with your doctor or pharmacist about alternative preparations of medications.

Q: What about heartburn?

A: When gastroesophageal reflux (movement of food or liquids up from the stomach into the esophagus) is a problem, drugs to reduce acid release are useful.

Drugs to reduce acid release are useful if there is any problem with gastritis (stomach inflammation) or reflux. Gastritis and reflux aren't caused by ALS, but they're very common and can result in stomach pain or heartburn when using a PEG tube.

Q: What's involved in placing a PEG tube?

A: Placement of a gastrostomy tube generally takes about half an hour. The procedure can be done with heavy sedation and local anesthesia, or under general anesthesia. Most people experience moderate pain for up to several days after tube placement. This pain can generally be relieved well with pain medication.

I prefer to admit patients to the hospital a day in advance of placing the PEG. This permits me to use intravenous fluids to correct dehydration and to give respiratory treatments to maximize lung function. Generally, I don't begin tube feeding until the day after placement of the PEG tube. Keeping the patient in the hospital after placing the PEG also permits teaching the patient and family how to use the PEG.

The insertion procedure is generally performed by a gastroenterologist (stomach specialist).

Q: Is the PEG tube noticeable?

A: The PEG tube is cosmetically good. Most people use it several times a day, the same as taking meals. When not being used, the PEG tube can be placed into a loop and taped flat against the abdomen. It's easily concealed by clothing.

Q: What happens if the PEG tube accidentally comes out?

A: If the PEG tube is accidentally pulled out, it can usually be replaced easily at an emergency room with a Foley catheter, the commonly used bladder catheter. Because of the way the PEG tube is designed, it's not possible to push it back in from the outside. The Foley catheter has a bulb at the tip that is inflated after it's inserted to hold it in, so it can be inserted from the outside. This is easy to do, and no anesthesia or sedation is needed. However, it should be done within hours of a PEG or Foley coming out; if you wait until the next day, the small hole from the skin to the stomach may already be sealing up and it may not be possible to insert a Foley from the outside. If the hole does seal up, a new PEG can be put in by the same method as the original PEG.

Q: How do most people like the PEG tube?

A: My experience has been that after receiving a PEG, most people decide that it was a good decision.

Q: Are there alternatives to the PEG tube, such as dilating the esophagus?

A: You may have heard about esophageal dilation. Some neuromuscular diseases affect the muscles of the esophagus enough to cause the food bolus (chunk) not to travel down the esophagus well. Myotonic dystrophy and polymyositis are examples of conditions that can do this. Also, some muscle diseases can affect the smooth (involuntary) muscle of the lower esophagus. Sometimes, in these conditions, dilating the esophagus helps. Dilating the esophagus is not likely to help people who have ALS, as their swallowing problems are in the mouth and the throat, rather than the esophagus. 

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TAKING A CLOSER LOOK AT BDNF

BDNF (brain-derived neurotrophic factor) is a naturally occurring central nervous system protein that's being given not one, but two, "second chances" in large clinical trials to test its safety and effectiveness in ALS. Both trials are supported by Amgen-Regeneron Partners.

The first trial, which involves direct delivery of BDNF into the spinal fluid (intrathecal delivery), is being conducted by Amgen. Enrollment is complete.

The second, being conducted by Regeneron Pharmaceuticals, will deliver the same substance via injection into the skin (subcutaneous delivery). That trial has just started and is actively seeking participants.

Amgen-Regeneron Partners sponsored a trial of subcutaneously injected BDNF in 1995 and 1996 that failed to show effectiveness. The study involved 1,135 ALS patients.

Jesse Cedarbaum, a physician who serves as vice president of clinical affairs at Regeneron, said the decision to revisit subcutaneous BDNF is based on analysis of data from the earlier trial. The statistics suggested that some patients might benefit from higher doses than those used in the previous study, he said.

The new trial will use "much higher" doses, he said. He added that BDNF has been well tolerated by a small number of ALS patients during a pilot study using the higher dose.

"We also hope to strengthen the trial design," Cedarbaum said, "by using more refined entry criteria to generate a more homogeneous population this time." The new population will include those whose ALS "has progressed beyond a certain point."

Cedarbaum said the new trial will involve more advanced ALS patients because the effects of the drug might be more obvious in that group, "for purposes of conducting a clinical trial to show that benefit in the shortest possible time, using the smallest possible number of patients." If this trial is successful, the company will "acquire more information about BDNF in patients in other stages of the disease in the future," he said.

The subcutaneous delivery study requires that patients meet entry criteria related to the severity and duration of the disease. Cedarbaum declined to be more specific about the type of ALS patient being sought for the trial. He said criteria will be reviewed with individuals at the time of the application.

Applicants also must have no complicating medical conditions other than ALS and have had no previous treatment with BDNF.

To apply for participation in the new subcutaneous BDNF trial, contact one of the centers listed below. Regeneron asks that you not contact the company directly.

More information may be available on the Internet at www.regeneron.com, www.amgen.com and www.mda.org.

Sites for BDNF Subcutaneous Trial:

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AIDS VIRUS MAY HELP CROSS BLOOD-BRAIN BARRIER

A team of researchers may have found a way to get proteins through the blood-brain barrier by borrowing a tip from the AIDS virus. By unfolding a large test protein and attaching it to a small piece of the human immunodeficiency virus (HIV), they were able to deliver it to various tissues in the body, including the brain.

In ALS, investigators have been trying to figure out how to get proteins such as brain-derived neurotrophic factor (BDNF) to go to the cells where they're needed -- namely, the brain and spinal cord. If drugs containing these proteins could reach the nervous system, they might be able to stop or repair the damage caused to nerve cells by ALS.

But some of these proteins aren't normally found in the bloodstream and have no mechanisms for being transported out of it.

Proteins targeted to the nervous system also encounter an even bigger obstacle: the blood-brain barrier. This barrier, made up of a layer of tightly joined epithelial cells, is designed to protect the sensitive nervous system from harmful things that may be circulating in the bloodstream. It allows only specific molecules to pass out of the blood and into the nervous system, and these molecules use exclusive transporters on the surfaces of the epithelial cells.

Many of the proteins that may be helpful to brain cells, such as BDNF, are normally produced in the brain. There they protect nerve cells, and they never circulate in the bloodstream. Scientists attempting to inject extra BDNF into the bloodstream of people with ALS find there's no natural mechanism for it to get across the barrier into the brain.

To get around this, Steven F. Dowdy of the Washington University School of Medicine in St. Louis borrowed a tiny piece of the AIDS virus (HIV) that allows it to invade cells and fused it to various proteins that can't normally get into cells. He reasoned that, since HIV is good at getting into cells, maybe he could use some of the same tricks to get proteins into cells and through the blood-brain barrier.

No one knows exactly how the piece of the AIDS virus they used, called a protein transduction domain (PTD), is able to make its way into cells. But it has been demonstrated that the PTD can take other proteins with it when it invades a cell.

The researchers carefully unfolded a large test protein to make it less bulky and attached it to a PTD from the HIV. They then injected the PTD/test protein hybrid into the abdominal cavities of mice.

The test protein turned up in the mice's nervous systems, indicating that it was able to cross the blood-brain barrier. The test protein also turned up in every other tissue studied.

This technique may ultimately prove useful for designing treatments for neurodegenerative disorders. For instance, if BDNF were fused to a PTD, perhaps it could be administered as an injection into the bloodstream (see "Taking a Closer Look at BDNF").

The work was published in the Sept. 3 issue of Science. 

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KEEPING BUSY, MAKING PLANS BEST THERAPY FOR COOPER

For Marilyn J. Cooper, a marriage and family counselor in Northridge, Calif., focusing on the future and staying active are what keeps her going.

"Make plans and set goals for yourself," Cooper said.

Cooper, 54, has been affected by ALS since 1975. She was a busy mother and homemaker when she received her diagnosis.

Marilyn J. Cooper

"I had three babies and a husband who could not boil water. It was overpowering," she said. "They told me I would be on life support in three years and dead in five."

But Cooper said she simply wouldn't accept that prognosis.

"I said, I have a job to do, I have babies. I can't give up," Cooper recalls telling her doctors. "They all looked at me like I was crazy.

"It took a while for me to figure out what I needed to do. I just figure one has a choice: You can easily lay down and let the truck run over you, or you can get up and walk away, and that's what I did," Cooper said.

Cooper is a member of the California Association of Marriage and Family Therapists. She graduated from the University of California-Los Angeles in 1966 with a bachelor's degree in history and elementary school teaching. She received her credentials for counseling from the University of Judaism and her master's degree in counseling from California State University-Northridge in 1991.

Currently, Cooper conducts a CAPS support group -- Children of Aging Parents -- in the Santa Monica area.

"It's quite a challenge," she said of the group. "There are many, many issues."

Group members are usually middle-aged adults caring for elderly parents. They cover a wide range of topics, such as grappling with whether to put a parent in a nursing home or provide care at home, Cooper said.

Cooper also recommends support groups for coping with the challenges ALS brings. "I'm a great believer in the power and the process of a group," she said.

Family is a key element of her own support network. She attributes her energy and positive attitude to her husband, Richard, her three children and friends. Her first grandson was due in mid-October, and anticipating his birth had Cooper and her husband "over the moon" with excitement, she said.

Focusing on the future is crucial, Cooper said.

"The whole key is planning projects, looking forward to things," she said.

"I wanted to be at my oldest son's bar mitzvah, and I made that. And I planned for my daughter's and I planned for my younger son's. And then all of their high school graduations, and then all their college graduations. And now I'm planning on a grandson," Cooper said.

Cooper schedules her many activities during the mornings so she can relax at home in the afternoons. She also makes time for hobbies.

"My passion is reading. I love to read," she said, listing novels and best sellers as her favorites.

Cooper has also been a sports enthusiast most of her life. She follows basketball and football, both college and pro teams, among other sports.

"Anything I can get to," said Cooper, who uses a power wheelchair to get around. "I'm very active. I go everywhere."

Recently, Cooper was appointed to serve a one-year term on MDA's National Task Force on Public Awareness. The task force is composed of 12 adults, each affected by one of the 40 neuromuscular disorders in MDA's program, who help educate the community about MDA and advise the Association on issues affecting people with disabilities. The group also communicates its position on national issues to government and policy leaders.

A member of the executive committee of MDA's Los Angeles Chapter, Cooper also earned the Association's 1998 Personal Achievement Award for California. In addition, she serves on the board of the Jewish National Fund, which raises money to support projects in Israel.

Cooper acknowledges that she's often called an inspiration for others with ALS, a label she isn't entirely comfortable with.

"People tell me I am, but I don't know why. I kind of feel like I live my life, day by day," Cooper said.

But she does have this advice to others with ALS:

"Make sure you accomplish what you need to. Don't waste time on negatives, or negative people -- especially doctors," she said. "Find a doctor who will give you support and not tear you down."

And, keep your outlook positive.

"I kind of refer to myself as disabled and not ill. Maybe that's denial, but a little denial never hurt anybody," she said with a chuckle.

"It's all the way you look at things."  

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CAREGIVERS CORNER: GETTING EFFECTIVE VOLUNTEER HELP

Family members of people with ALS probably weren't surprised by the results of a study of caregiving published in the New England Journal of Medicine on Sept. 23.

News stories reported the primary finding from the study conducted by the National Institutes of Health and other institutions: That when someone is terminally ill, whether the patient is male or female, it's the women in the family who do the nonmedical caregiving. In 72 percent of the nearly 1,000 cases studied, the primary caregiver was a wife, daughter, sister or other female relative.

The study reported that the chief areas in which the dying patients needed help were transportation, nursing, homemaking and personal care such as bathing and dressing. The primary caregivers who were interviewed relied more on paid assistance than on volunteers for the support they needed.

Similar data undoubtedly apply to families in which a member has ALS. Of course, caregiving has traditionally been a family responsibility, falling largely on female relatives. Even today, when gender roles are less precisely defined and most women have jobs outside the home, many people prefer that caregiving remain a family affair.

Nonetheless, when caregiving for someone with ALS becomes a round-the-clock responsibility for several years, even the most loving spouse or adult child needs support. Friends and other relatives may be willing to help but are uncertain of just how to do so. The National Family Caregivers Association published the "Share the Caring" checklist (below) to help willing volunteers offer useful help.  

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HOW SERIOUS IS Y2K?

Almost everyone has heard of the Y2K problem. It refers to the potential for computer systems and devices controlled by computers to fail when the year changes from 1999 to 2000.

The problem, unfortunately, was built in to many standard computer systems years ago. In order to save on scarce computer memory, programmers employed two digits to designate the year, instead of four. As a result, after Dec. 31, 1999, computer systems may interpret the digits "00" to mean "1900," instead of "2000." System failure may result.

Some Y2K problems may not manifest themselves until later in 2000. For instance, because 2000 is a leap year, some failures may occur on Feb. 29. Y2K is a potential problem not only for computers but also for devices and appliances that have computer microchips embedded in them.

How real is this problem, and what consequences does it hold for people with disabilities?

GENERAL GUIDELINES

Being prepared for Y2K in some ways is similar to being prepared for any emergency, such as a flood or an earthquake, that can cause a loss of power and communications. See "A Call for Safety" in Quest (vol. 3, no. 4), or in the Publications area of MDA's Web site.

Loss of power needed to operate equipment such as ventilators is a major concern. Loss of heat could be an equally vital problem for those experiencing harsh winter conditions. If, because of ALS or another disabling condition, you have special considerations, including mobility issues or need for a ventilator, share that information beforehand with your fire department or other emergency services personnel. They'll take note of the information in order to respond more effectively in the event of an emergency.

The federal government offers some general guidelines for Y2K preparedness, including suggestions to have on hand batteries for flashlights and radios, a three-day supply of water and nonperishable foods, and at least half a tank of gas in your car.

The Federal Emergency Management Association (FEMA) offers a publication called "Y2K & You -- A New Horizon," which is available at www.fema.gov or by calling (800) 480-2520.

The publication, which maintains a determinedly cheerful tone about the government's Y2K preparedness efforts, has this to say about potential power disruptions: "There is no indication at this time that the Y2K problem will cause national disruptions in basic infrastructures such as electric power, telecommunications, banking and transportation.

"Massive national disruptions in electric power are unlikely. The industry has an aggressive program for monitoring Y2K progress coordinated by the North American Electric Reliability Council. The possibility of minor local outages can never be completely ruled out. However, the electric power industry reports that it is ready to meet that challenge each day."

About the possibility that failures in billing systems could wipe out your payment records: "If Y2K failures do occur, it is not likely that your records will be erased. But it is always a good idea to keep copies of billing and other records."

About the advisability of stockpiling cash: "There is no need. The banking industry has been working on the problem for several years. At the end of 1998, 96 percent of all financial institutions were rated as being on track to be Y2K ready by June 30, 1999.

"While banks are confident that their record keeping systems will be ready for 2000, it's always a good idea to keep copies of your bank statements."

MEDICAL EQUIPMENT

If you depend upon devices such as a power wheelchair or ventilator, your first step would be to gather your serial numbers and call the manufacturers. You may be told that your equipment is Y2K compliant, meaning that it will not experience problems on Jan. 1.

If the equipment isn't compliant, it may be possible to receive a Y2K upgrade before Jan. 1, possibly at no charge if the product is still under warranty.

However, even if the device itself is guaranteed not to fail, unless it's battery run it will need electrical power. Despite the government's assurances, there is no guarantee that you will not lose power on Jan. 1, possibly for several days or longer. Power outages occur for many reasons and not just due to Y2K, and now may be a good time to formulate a long-term plan for having access to backup power.

BACKUP POWER

A generator intended to operate medical equipment should produce 800 watts or more. Some generators have a recoil starter that requires you to pull a cord like that on a lawn mower. Other more expensive ones operate at a push of a button. Since many generators run on gasoline, fuel storage is a problem. A three-day power outage could require as much as 60 gallons of fuel, which would be very hazardous to store.

Some companies that sell generators are Generac (www.generac-portables.com), Onan (www.onan.com) and Honda (www.honda.com).

An alternative to purchasing a generator would be to check into a care facility. It would be wise to establish a relationship with the facility well before year's end.

If this option seems untenable, do some research in your community to see if there will be any facilities such as neighborhood schools that may have large generators and which could possibly serve as havens in an emergency.

Other suggestions for being prepared for Y2K include having an extra allotment of medical supplies and obtaining a cellular phone.

The President's Council on Year 2000 Conversion offers information on Y2K preparedness through its toll-free hotline, (888) USA-4-Y2K, and its Web site at www.y2k.gov.  

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PHASE 3 STUDY FINDS GABAPENTIN INEFFECTIVE
AGAINST ALS

Gabapentin (Neurontin) was found ineffective against ALS in a phase 3 placebo-controlled trial. The trial results were announced on Oct. 13 by Robert G. Miller, director of the Forbes Norris MDA/ALS Research Center at California Pacific Medical Center in San Francisco, at the annual meeting of the American Neurological Association. The announcement is particularly disappointing after gabapentin showed early promise in a phase 2 trial.

Gabapentin is a Food and Drug Administration-approved drug for the treatment of seizures. Like riluzole (Rilutek), it's thought to work by blocking the release of the neurotransmitter glutamate in the central nervous system. Glutamate in excessive amounts is toxic to the nerve cells that control muscles, and there's evidence that people with ALS can't regulate their glutamate levels properly.

Early preclinical and clinical studies indicated that, at a dose of 2,400 mg/day, gabapentin showed a modest benefit in ALS, but more studies were needed to evaluate the drug.

The latest study, supported by MDA and Warner-Lambert Co. (the manufacturer of gabapentin), involved 204 people with ALS, half receiving gabapentin (3,600 mg/day), and half a placebo. The nine-month study was completed by 128 people, while 76 dropped out (37 on gabapentin and 39 on placebo).

The group found that the mean rate of decline of arm strength, the primary outcome measure for the study, wasn't significantly different for patients receiving gabapentin compared with those who received the placebo. Three analytic approaches all yielded similar results with no significant difference. Gabapentin was generally well-tolerated.

A host of secondary outcomes were also measured, including rate of strength decline in those completing the study; rate of decline of strength expressed as a percent of normal; rate of decline of strength in more mildly affected patients at baseline; rate of decline of breathing muscle function; rate of decline of function on scales; decline in quality of life; and symptomatic benefit. No significant difference in any of these secondary outcomes was found between those taking gabapentin and those taking placebo.

The researchers refer to the phase 3 study as "definitive" and conclude that "although the preliminary and encouraging findings from the phase 2 controlled trial of gabapentin in ALS provided some basis for optimism, the results of this longer, larger, higher-dose study of gabapentin in ALS provide no evidence of effectiveness of this drug for patients with this disease."  

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SOCIAL SECURITY INFO ON THE WEB

Want to review where you stand with the Social Security Administration? Need to check the regulations on Social Security disability benefits? It's easy through the SSA's Web site at www.ssa.gov.

Viewing some documents requires the Adobe Acrobat program. If you don't have Adobe Acrobat, you can download it for free at www.adobe.com/products/acrobat/readstep.html. 

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HELP WITH HIGH COST OF MEDICATIONS

The Pharmaceutical Research and Manufacturers of America (PhRMA) has published a directory of drug companies willing to help people with the high costs of medications such as riluzole (Rilutek, manufactured by Rhone-Poulenc Rorer).

To obtain a copy, go the association's Web site at www.phrma.org/patients/. Each company runs its own patient assistance program, and nearly all require a doctor's referral. 

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FLU SHOTS IMPORTANT FOR ALS PATIENTS

The arrival of fall and winter also means the arrival of flu season.

Influenza is particularly hazardous to people with neuromuscular diseases, including those affected by ALS. With this in mind, annual flu inoculations are included among the services provided by MDA.

Many public and private health insurance programs, including Medicare, will also authorize flu inoculations as part of their preventive care measures.

Contact your doctor or local MDA office for more information.  

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HELP FIGHT ALS TODAY AND TOMORROW

Many people who know the devastating effects of ALS are providing lasting support for MDA's battle against the disease. Through your will, you can designate a gift to MDA earmarked to support ALS research or services.

Bequests to MDA can be made with cash, securities, real estate, or other property. You can bequeath a percentage of the entire estate to MDA or make a bequest of the residue, donating property remaining after all bequests to family and others have been satisfied. You may also name a memorial gift in honor of a family or individual.

To give what remains of your estate after other bequests have been satisfied, just include the following language in your will:

"I give, devise and bequeath all (or a specified fraction of) the rest, residue and remainder of my estate, whether real or personal, of every kind and description, and wherever situated, to Muscular Dystrophy Association Inc., a New York not-for-profit corporation having its principal office at 3300 East Sunrise Drive, Tucson, Arizona, 85718-3208, for its program of research and services related to amyotrophic lateral sclerosis."

To give a dollar amount or percentage of your estate:

"I give, devise and bequeath the sum of $________ (or ________ percent of my estate) to Muscular Dystrophy Association Inc., a New York not-for-profit corporation having its principal office at 3300 East Sunrise Drive, Tucson, Arizona, 85718-3208, for its program of research and services related to amyotrophic lateral sclerosis."

Your attorney or financial adviser can help you work out the details of a bequest to MDA's ALS program. For more information, call MDA's Planned Giving Department at (800) 572-1717.  

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The Association welcomes gifts for ALS research honoring significant occasions of achievement. These gifts may be made in tribute to special people or to mark such events as anniversaries, birthdays, weddings, graduations or retirements.

THE ALS NEWSLETTER
Muscular Dystrophy Association
National Headquarters
3300 East Sunrise Drive
Tucson, Arizona 85718-3208

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