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Keiko Ueda is a dietitian with the Division of Genetics/Metabolism at the Boston Floating Hospital for Children at the New England Medical Center in Massachusetts. She spoke to the MSUD families on Practical Nutritional Considerations in MSUD at the National Coalition For PKU and Allied Disorders Metabolic Conference in May 2001. In this article, Keiko focuses on the support systems needed by most families in order to provide adequate nutrition for those with MSUD.

Keiko Ueda and Shayla Brubacher at the National Coalition Conference, May 2001

Introduction

Decisions regarding the medical/nutritional care plan for a child or adult diagnosed with MSUD is a team effort - with parents and caregivers often expected to take on the role of team leaders. Input from the patient, parents, and caregivers is required, most of all, in order to meet the daily medical and nutritional needs of a MSUD patient. Therefore, ongoing communication with your primary care physician, metabolic physician, and metabolic clinic staff is key. Keeping the lines of communication open with your metabolic clinic will best assist them in helping you tailor the MSUD diet to your child's needs and adjust the diet as medically necessary to optimize metabolic control, growth and development, and maintain and support good health. If there are multiple daily caregivers (parents, relatives, teachers, nurses, sitters, etc.), it is important for everyone to be consistent and aware of the most current MSUD nutritional plan in order to avoid errors or mis- communication.

I have great respect and admiration for the dedication and daily efforts of the patients, parents, and caregivers of individuals living with MSUD and other metabolic disorders. As we all know, coordinating the medical nutrition therapy for an individual diagnosed with a metabolic disorder such as MSUD is a challenge. As a metabolic dietitian, whenever I am planning the metabolic nutrition diet orders for an individual patient, I try to take into consideration individual, age-specific nutritional needs, as well as the practical issues that might influence or hinder the actual achievement of the goal dietary intake. I always try to consider each individual's short and long term dietary requirements and goals. I try to target metabolic education nutrition counseling to the needs and abilities of each individual living with MSUD. It is important to strive for realistic and achievable goals to avoid unrealistic expectations that can result in frustration and disappointment. Obviously, MSUD patients and their nutritional needs and goals vary greatly from one individual to the other. In my experience, I have found that MSUD patients have distinct nutrition and feeding issues and needs at different times and stages in their lives. Most of the MSUD patients I have known do well meeting their daily nutritional needs via oral feedings when in good health. During times of illness, they often require the support of tube feedings (TF) and/or intravenous (IV) therapy to assist in illness recovery and regain metabolic control.

MSUD dietary goals and restrictions can be very different between individual MSUD patients, and for each MSUD patient in different medical situations at different times of his or her life. An individual's MSUD diet plan depends upon his or her diet tolerance; medical and nutritional needs for growth, development, and/or health maintenance; biochemical lab results; and medical/illness status. I would urge you to continue to discuss your individual questions and concerns with your metabolic clinic physician, dietitian and staff.

Overview

There are certain practical considerations that must be addressed in order to optimize a MSUD medical nutrition therapy plan to ensure the best possible outcome.

MSUD Nutrition Practical Considerations - Availability ofResources/Tools

  • State Department of Health Newborn Screening Program for MSUD
  • Metabolic physician services and staff
  • Health insurance coverage/state legislation
  • State Department of Health assistance programs
  • MSUD medical foods (formula) & low protein specialty foods
  • Supplies: digital gram scales, books (leu/protein food counts, low pro cookbooks)
  • L-amino acid medication supplements (l-valine, l-isoleucine)
  • Local or home outpatient lab draws and home monitoring (blood amino acid levels and/or urine DNPH)
  • Emergency illness management protocols
  • Support for MSUD diet at school (and/or work)

The ongoing availability of these medical services and nutritional supplies is important - these are the tools that will assist you in implementing the MSUD diet on a daily basis. I have found that patients who do not have continuing access or have only limited access to these resources often have difficulty adhering to MSUD diet goals.

Calling Your Health Insurance Company

Medical health insurance coverage of MSUD Medical Foods (formulas) and low protein specialty foods are often key issues. If you haven't yet had difficulties with your health insurance regarding coverage of formula and/or low protein specialty foods, unfortunately chances are high that you may encounter some problems in the future. It is very important to understand your health insurance coverage plan benefits, policies, and limitations.

Health Insurance Coverage - When Calling Your Insurance Company

  • Try to be patient and courteous, inform and educate, try to gain an advocate
  • Review your health insurance plan coverage benefits and limits
  • Document each call and contact person, info, department, phone numbers (extension), date of call
  • Request information in writing for future reference
  • Request a copy of your Explanation of Benefits (EOB)
  • Request case management services if available to assist in care coordination
  • Refer to MSUD formulas as "MSUD Medical Food," prescribed by your metabolic MD
  • Know your state law and its limits (if available) and have a copy in your files

Your need to be diligent does not end even when you receive approval for health insurance coverage. Some health insurance providers require periodic reassessments and regular prior authorizations for coverage of MSUD Medical Foods, low protein specialty foods, and certain medications. Ask if your authorization has an expiration date in order to be prepared to renew it in the future - and then keep track in your calendar to allow for enough time (4 weeks or more) for the renewal process. Ask if your MSUD Medical Foods are covered under your health benefits, prescription benefits or durable medical equipment benefits. Your policy coverage may vary, have limitations, or have different co-pays or deductibles depending upon the type of coverage under which your MSUD Medical foods, low protein specialty foods, and amino acid supplements are placed by your health insurance company.

Who Will Supply Your MSUD Medical Foods and Low Protein Specialty Foods?

MSUD Medical Foods

You may find it best to order your MSUD Medical Food from a local pharmacy and make the monthly trips to pick it up. This is convenient (as long as the pharmacy can do direct insurance billing), sparing you the need to pay out of pocket, submit insurance claims with your receipt, and wait for reimbursement. Depending upon your insurance policy and state laws (if applicable), you may have out of pocket co-pays or deductibles for your MSUD Medical Food prescriptions.

Some families fill their MSUD Medical Food prescriptions via home health infusion (HHI) or durable medical equipment (DME) companies contracted with their insurance and have delivery services to their homes. This option depends upon your health insurance benefits and coverage. Insurance companies often contract with different home health companies, therefore you may want to ask for a list of providers contracted with your insurance company. In most cases, your metabolic clinic staff must contact the HHI/DME to provide prescriptions and refill authorizations and to set up the initial deliveries to your home. Often the HHI or DME companies have limitations in terms of what they can provide. Most contracted HHI or DME companies can order the metabolic medical foods and bill your insurance. Some companies are limited by company policies or state license to provide only "enteral feeding supplies" (meaning supplies necessary to support a patient getting their metabolic medical food by nasogastric or gastric tube feedings). Home health infusion companies are licensed pharmacies, and are able to assist patients who require IV medications or nutrition support. Some HHI or DME companies can not assist patients who are taking all of their formula by mouth because of insurance or company policy limitations. Your metabolic clinic staff should be able to help you sort out the best options for your needs and family.

Health Insurance Coverage Issues - Denials

  • Find out WHY? Request it in writing.
  • Find out how to appeal: how long it takes, how/who will get response
  • Request your metabolic clinic to provide a Letter of Medical Necessity
  • Find out if you are eligible for your state Medicaid insurance coverage (or as a secondary provider)
  • Find out if you are eligible for your state Department of Health assistance programs
  • Contact your state Department of Insurance to check if your health insurance is in compliance with state law
  • Ask your benefits office if your insurance is a "self funded plan" exempting them from following state insurance mandates (ERISA) if you have a state law
  • Contact your local and state legislators for assistance
  • Consider obtaining assistance from an advocate
  • Consider changing your health insurance provider (make sure coverage is better, for example, watch for pre-existing condition exemptions)

Low Protein Specialty Foods

In terms of the low protein specialty foods, some state laws specify limitations of coverage per year. For example, the Massachusetts state law sets a maximum of $2500 per year per patient for low protein specialty food health insurance coverage. Most families direct order and pay the low protein food companies, then submit claims to their health insurance and wait for reimbursement. It is very rare to find a pharmacy, HHI, or DME company willing to order and successfully bill health insurance companies directly for the low protein specialty foods. In some cases, depending upon their insurance policies, families are able to arrange for the low protein food companies to submit bills directly to health insurance companies. But not all low protein food companies provide this service. This is a very convenient service, if available, but typically requires many calls and a lot of time spent filling out forms to put this in place. However, caregivers often find it worth the effort.

If your health insurance offers case management with an assigned caseworker (usually a nurse) or if you can find a helpful member services contact person to assist you with your insurance coverage issues, this can save you some time and effort in the future.

Finally, some states have department of health sponsored programs to assist families with obtaining metabolic medical foods and/or low protein specialty foods, for example: Massachusetts - PKU and Other Metabolic Disorders Special Dietary program; Maine - Children with Special Health Needs program; Illinois - Division of Specialized Care for Children program; your own state - Women, Infants and Children (WIC) program. Most programs have eligibility requirements: state residency, diagnosis, financial need, written insurance denials for coverage, and/or patient's age. Discuss with your metabolic clinic staff whether there is a program in your state to assist you.

What to Do If Your Insurance Company Denies Coverage

Should you get insurance denials for coverage, you can research the appeals process for your insurance company. You can request assistance from your metabolic clinic staff; their phone calls and letters of medical necessity can support the need for a more in-depth insurance medical review. This can be a frustrating process that may take some time for review and decisions. It is important to continue to be as patient and courteous as you can in your interactions with your insurance company. Try to consider your correspondence with your insurance company as an opportunity to educate and inform insurance workers about MSUD and the strict and difficult medical nutrition therapy requirements for patients. By outlining just what you and your family have to go through (rather than taking a belligerent approach), you might just gain an ally at your insurance company who is willing to assist you in finding a solution to these (and other) problems and concerns.

Appendix I is a sample medical necessity letter that may assist you in your insurance appeals process. You might need the assistance of a legal advocate to support your appeals if you continue to get illegal denials for coverage in spite of all your best efforts. If your employer offers a "self funded insurance plan," the ERISA (Employee Retirement Income Security Act) exemption poses an obstacle to getting coverage; this apparently exempts the employer/health insurance from having to follow certain state insurance mandates. You must then appeal directly to your employer (through the benefits office) and/or insurance company on the basis of medical necessity for assistance with coverage. A few families have had success with the support of local or state legislators in reversing insurance coverage denials, even without a state law. But if your state doesn't currently have a state law mandating health insurance coverage for medical foods or low protein specialty foods, contact your local state and national parent support groups to assist in organizing grassroots efforts to get a law passed. (This has been successful in several states.) Providing your legislators inarguable information on medical necessity and financial burden of medical costs without insurance assistance will often win political support and sponsorship of a bill. If all else fails, it may be necessary to look for a different insurance provider.

If changing your insurance carrier is a consideration, make sure to do your homework to check the benefits of the new plan. Watch out for pre-existing condition exemptions, and ask how they would provide for your MSUD medical needs before finalizing the change. The federal law, Health Insurance Portability and Accountability Act (HIPAA) of 1996 (sometimes called "Kennedy-Kassebaum") might be helpful to continue to obtain coverage and avoid pre-existing conditions clauses (Reference 1). It is also important to know if your health insurance plan sets age limits (e.g., 18 yrs.) for coverage of dependent children. "Given the wide variation among state laws and individual policies, it is recommended to contact your insurance company or plan sponsor at least several months before the child turns age 19 to tell them that they have a dependent adult child with a disability. . . [it is critical to obtain state-specific advice]."1

Getting Organized

It takes much of your time and effort to have all of your various medical services organized, insurance coverage verified, and in place. I would recommend a methodical and step-wise approach to finding solutions to challenges that you may face in order to ensure that your child obtains the medical and nutritional support and services needed to promote good MSUD metabolic control.

Parents and caregivers of individuals with special health care needs are now expected to take on increased responsibility for coordinating their child's care. Caregivers often must work with multiple institutions and agencies: primary care physicians and staff, specialty physicians and staff, hospitals, outpatient labs, state department of health programs and case workers, health insurance company employees, home health supply companies, durable medical supply companies, pharmacists and staff, visiting nurses, etc. It can be overwhelming for many caregivers and patients to organize all of the contacts and information. Therefore a MSUD Individual Health Plan (IHP)2 should be included in your records, along with your MSUD emergency illness protocol (see Appendix II). The IHP may help both caregivers and medical and specialty physicians and staff in organizing and recording important contacts and medical/nutrition issues. However, the IHP requires regular updating.

Summary

Staying in touch with your metabolic clinic physician, dietitian, and staff provides the best assistance as you adjust MSUD medical nutrition therapy goals for your child and his or her changing needs over time. Obtaining and maintaining health insurance coverage of MSUD Medical Foods, low protein specialty foods, and L-amino acid medication supplements can be very complicated. It is important to know the benefits and limitations of your health insurance policy, what your state law allows, and the availability of other state program resources. Ask your primary care physician and your metabolic clinic physician and staff for information to help you decide which resources are most helpful for your situation. Coordination of your child's MSUD medical and nutritional care can be one of your biggest challenges; organizing your information and contacts can greatly assist you and save your time.

Note from Keiko Ueda : I would like to thank Dr. Mark Korson for reviewing this article, and most of all, my thanks to the parents and individuals living with MSUD with whom I have been privileged to work and from whom I have learned the most.

*References:

1. Schulzinger, R. 2000. Youth with Disabilities in Transition: Health Insurance Options and Obstacles. An occasional policy brief of the Institute for Child Health Policy, Gainesville, FL (ISBN: 0-9700909-4-3) or (www.mchbhrtw.org/materials)

2. Silva TJ, Sofis LA, and Palfrey JS. 2000. Practicing Comprehensive Care: A physician's operations manual for Implementing a Medical Home for Children with Special Health Care Needs. Boston, MA: Institute for Community inclusion/UAP, Boston.


Appendix I: Health Insurance Letter of Medical Necessity for MSUD
(Sample letter - change all underlined sections to fit your individual case.)

 

January 3, 2002

Attn: Medical Appeals Review
Health Insurance Company
Phone # and Fax #
Re: A.B. DOB: 12-20-98 (A.B.: replace with name of individual; DOB: insert date of birth)
Insurance ID #123456789

To Whom It May Concern:

This is a letter of medical necessity for A.B. to receive coverage for MSUD Medical Food manufactured by MSUD Medical Food (formula) company, low protein specialty foods from low protein specialty foods company(ies), and L-valine and L-isoleucine amino acid powders by the amino acid manufacturer company. A.B. has been diagnosed with a metabolic disorder called Maple Syrup Urine Disease (MSUD).

Medical treatment of MSUD patients requires a strict dietary restriction of the three branched chain amino acids - L-leucine, L-isoleucine and L-valine - because of the metabolic enzyme impairment in the branched-chain alpha ketoacid dehydrogenase (BCKAD) complex. A.B.'s daily intake of these three essential amino acids is restricted to the minimum amount he needs to promote continued growth and development, while also preventing any metabolic imbalance. It is also very important to try to avoid catabolism by ensuring daily intake of adequate total calories and providing enough of the other essential amino acids. The MSUD Medical Food is specially formulated to provide trace amounts of the three amino acids (L-leucine, L-isoleucine and L-valine), adequate amounts of other essential amino acids, as well as carbohydrates, fat and vitamins and minerals.

To elaborate, A.B. requires a diet leucine (therefore protein) food restriction of 530 mg leucine per day in order to control his blood levels to treat his MSUD. This means that he is only allowed a total of about 7 grams of protein from foods per day. This protein restriction would be equivalent to about 3 slices of regular bread, which obviously as a total diet intake of food per day would not be sufficient to meet his daily nutritional needs. This means that in order to continue meeting his daily protein and calorie needs, while lowering his dietary leucine intake, he needs to daily consume a prescribed amount of the MSUD Medical Food and also consume low protein (low leucine) specialty food products. Some examples of low protein specialty food products A.B. needs are: low protein pastas (e.g. 1/4 cup of low protein pastas contain about 0.1 grams of protein vs 1/4 cup regular pasta contain about 3.0 grams of protein); and his parents make low protein breads (1 slice low protein bread contains about 0.2 grams of protein vs. 1 slice of regular bread which contains about 2.5 grams of protein). These low protein specialty food products are not widely available in local supermarkets and must often be obtained from mail order specialty food companies.

The MSUD Medical Food, low protein specialty foods, and amino acid powder currently provides about 80% of A.B.'s daily nutritional needs for treatment of his disorder. The L-valine and L-isoleucine amino acid powder is prescribed when it is necessary to help meet A.B.'s daily minimum requirement of L-valine and L-isoleucine without concomitantly increasing his intake of L-leucine. Treatment for classic MSUD patients without the use of the MSUD Medical Foods, low protein specialty foods, and L-amino acids would result in protein-calorie malnutrition, metabolic imbalance, delayed growth, delayed development, neurologic impairment and possibly even death.

We appreciate your assistance in the coverage of the MSUD Medical Food, low protein specialty foods, and L-valine and L-isoleucine amino acid powders that are essential to A.B.'s continued medical treatment of MSUD. As you know, coverage of MSUD Medical Food, and low protein specialty foods are now mandated in the Commonwealth of Massachusetts by the passage of MA House Bill 5622 signed into law by Governor William Weld on January 4, 1994. With appropriate treatment, we are confident that A.B. will achieve his growth and development potential within the limits of MSUD. We request your approval for coverage of his MSUD Medical food, low protein specialty foods, and L-valine and L-isoleucine amino acid powder, which are essential to continuing his MSUD treatment and promoting continued growth and survival.

If you have any questions regarding any of this information, please do not hesitate to contact us. We would appreciate it if you would inform us of your decision on this matter as soon as possible. Thank you for your time and consideration.

Sincerely,

Metabolic Physician, M.D. Metabolic Dietitian, R.D.
Division of Metabolism Division of Metabolism
cc: A.B.'s caregivers (list by full name)


Appendix II: MSUD Individual Health Plan
[Form adapted from Silva, et al, 2000. Practicing Comprehensive Care.]

(Sample form - insert your information, add lines and adjust to your needs - examples are in italics.)

 

Date of Last Revision: 1/3/02
Name of Patient:
Date of Birth:
Caregivers Name(s):
Address/Phone #:
Emergency Contact: name and phone #:
Primary Care MD:
Clinic Address:
Phone #/Fax #/ After hours contact #:
Metabolic MD:
Hospital/Clinic Address:
Phone #/Fax #/ After hours contact #:
Principal Diagnosis: MSUD
Insurance Company and ID #:
Insurance Member Services Phone #:
Insurance Caseworker Name & Phone #:
Concerns for Upcoming Visits/Follow up Issues List:
1.
2.
3.

Consultants/Hospital/Phone #/ Last Seen:
1. Dr Jones, Metabolism, Hospital, #, 12-2-01
2. Dr Smith, Neurology, Hospital, #, 11-5-01
3.

Admissions History (last 12 months):
2/1/01 for a virus

Medications: Name/How Much/Schedule:
1. L-valine/ 10mg/ml solution/4 cc twice a day
2.
3.

Allergies:
Immunizations/Dates:
Home Medical Supplies/Where Obtained/Last Refill Date:
1. MSUD Medical Food/home health care company/12/2/01
2. L-valine solution/pharmacy/12/2/01
3. Low protein specialty food order/company/12/2/01

Contacts: Names and #s:
Home Health Company/DME:
Visiting Home Nurse:
Department of Public Health Program(s):
Early Intervention Caseworker:
School/Address:
School RN:
EIP/School Contact:
Pharmacy:
Respite Care Contact:
MSUD Nutrition Goals:
MSUD Formula Recipe:
MSUD 24 hr Formula Goals:
MSUD Formula Feeding Schedule:
MSUD Leucine (mg) or Protein (g) Food Restriction Goals:
Lab Testing Schedule/Date for Next Test(s):
1. Blood amino acid levels in 2 weeks: 1-16-02
2. Home urine DNPH as needed

Caregiver/Patient Goals for the Next 12 Months:
1.
2.
3.

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