QOL Responds to Sucraid Shortage – Time Sensitive!

*Please note this is time sensitive information. Updates will be posted as they are available.

Sucraid Shortage and QOL Response Summary

SUCRAID® SHORTAGE UPDATE

Conference Call with QOL Medical CEO Derick Cooper on April 13, 2016 at 2 p.m. (EST)

For current information on Sucraid and QOL updates, visit the following websites:

The following are highlights and follow-up on Q&A from the Conference Call

The listed questions were provided to Mr. Cooper in advance. Some were answered in conjunction with others during the conference call so not every question is answered precisely, but is addressed at one point or another. I’ve noted a reference to the answer for another question when it applies.

(Note: I’ve done my best to present this information in an objective manner and to state the information provided by Mr. Cooper as accurately as possible for informational purposes only.)

  1. Could QOL Medical clarify the reason for the shortage? Is it related to a combination of factors or primarily one?
    Information known prior to the conference:
    a) Ingredient related: The FDA website statement mentions “shortage related to unavailable approved active pharmaceutical ingredient (API) from a supplier due to requirements related to complying with good manufacturing process.”
    b) New facility and production related: Letter by QOL Medical CEO March 2016 mentions the construction of an entire new facility and transfer of production to entirely new building.
    ANSWER: Mr. Cooper further explained the change in facilities was to transfer the production of the primary ingredient in Sucraid – sacrosidase – from a food-grade manufacturing facility to a pharmaceutical-grade facility per FDA request.
  2. Reason behind the decision to change production which resulted in the shortage: Sucraid has been produced for years and used by patients for 17+ years. If the formulation hasn’t changed at all, what happened now, to get us to where we are now?
    ANSWER: Sacrosidase originally made in a food-grade facility that no longer met pharmaceutical standards. QOL has rebuilt a new facility to meet all current standards. They are working on details for final approval to begin manufacturing with FDA. Although the production of the ingredient met FDA requirements prior to this change, QOL is now  “migrating manufacturing to meet modern standards”. Mr. Cooper mentioned that there are “items” to finalize with FDA within the week,  but did not mention what those details were.
    According to Mr. Cooper, the  requirements to manufacture pharmaceutical-grade products involve two steps: 1) Tests of ingredient itself after processing to ensure that the product still effective, and no contaminants were introduced  during the process. 2) Manufacturing process itself is very complicated. Mr. Cooper did his best to describe the general steps involved as an example to demonstrate the details and steps involved in the migration. They start with a lot of yeast, then extract the sacrosidase from the yeast and filter multiple times to ensure only sacrosidase is left in a pure form. The filter must be checked by outside vendor who has valid methods for checking filter. Each part of this process needs to be documented and submitted to the FDA. QOL has made valiant efforts to follow these procedures and paperwork requirements the entire time. FDA regulations are ever-changing and do not always apply to each specific product produced. These guidelines are subject to interpretation, and QOL has done their best to interpret the guidelines according to the specific process and manufacturing of sacrosidase (Sucraid). The FDA and QOL are working together to expedite the process as much as possible.
  3. What does QOL Medical see as the current status of the shortage? Can you expand on the below information?
    Information known prior to the conference:
    a) Letter by QOL Medical CEO March 2016 mentions “we have built this new facility, transferred the manufacturing process, begun production, and filed the necessary regulatory application with the FDA for approval. We have produced Sucraid® in the new facility and are awaiting final regulatory approvals for distribution.”
    b) Calls to Sucraid Assist / One Patient Services mentioned new facility has already produced Sucraid but that those lots have not been FDA approved.
    c) Accredo representative has stated the shortage prolongation is due to the FDA not approving an ingredient in Sucraid.
    To clarify, it was the production of the main ingredient, sacrosidase within a food-grade facility the FDA decided needed to take place in a pharmaceutical-grade facility which promoted the changes. QOL did not anticipate the building and transfer to new facilities taking so long, which is the assumed reason they did not manufacture enough Sucraid prior to the transfer to meet the demand. This is my interpretation of what seemed to be implied by Mr. Cooper.
  4. FDA states they are awaiting information from QOL in order to approve Sucraid lots. What are they waiting for? As of today, all paperwork has been turned in and inspection of facility is complete. There are a few minor details (not specified by Derek) that will be address tomorrow (April 14, 2016)  in a meeting between QOL and FDA for final resolution.
    ANSWER: At the time of the phone conference, FDA had completed a full inspection of the new facility and QOL had completed and submitted all required paperwork.
  5. Does QOL anticipate a foreseeable end date to shortage?
    ANSWER: Though this could not be answered precisely, Mr. Cooper said it could take up to 2-3 months before Sucraid is available again, but he was hopeful it would be sooner.
  6. Can QOL Medical provide an estimated timeline for a resolution of the shortage in terms of weeks, months or longer? Patients would like to know if they should plan to be without Sucraid for a short, medium or long period of time. They are cognizant any specific timeline is unlikely as it will invariably change.
    See ANSWER TO #5.
  7. How can the most vulnerable patients be prioritized to receive any available, or first available, Sucraid? For the families who have quit using Sucraid to make it more available to more at risk patients, how will it be communicated to us that there is enough to begin using it again?
    There are some units left they are saving for these patients according to dire need, however they will not use this supply if they are able to begin manufacturing a new batch soon.
  8. Does QOL have any data about other medications that experienced a similar facility approval related shortage situation? If so, what was the timeframe for those similar cases to be resolved? When it is released, it will be available within a week of FDA approval. NA
  9. Can QOL confirm that all Sucraid that has been currently released is only done with full FDA approval?
    Information known prior to the conference:
    a) That there are NO unapproved lots in circulation.
    b) That Sucraid lots requiring an informed consent listed additional possible risks but were still approved as safe to be released by the FDA.
    c) That current Sucraid is safe to use and that all possible risks have been fully disclosed in writing to patients. That neither QOL nor the FDA has hidden any important information from them.
    d) FDA stated this clearly in the teleconference, but the point seems to have still been confused by other external statements on the call and noise distraction.
    Details related to this question were not addressed specifically enough for me to confirm or deny the details.
  10. Even though it was FDA approved as safe to use with informed consent, What were the specific contaminants that were identified or posed as possible risks?
    ANSWER: I asked Mr. Cooper to clarify this once the call was opened to questions. If I understood him correctly, it was the “manufacturing of sacrosidase” that no longer met FDA requirements. There was NOT any contamination of the ingredient.

It’s possible the FDA was concerned the food-grade facility could lead to a risk which is why they prompted the move to new pharmaceutical grade facilities, but this inference was not made by Mr. Cooper himself.

ADDITIONAL DETAILS SHARED DURING THE CALL

To clarify the frustration of one parent on why they were not informed of the possible shortage in advance, Mr. Cooper explained that QOL doesn’t have the ability to directly contact patients due to HIPPA.

Possible Solutions to Communicating with Parents and Patients in the future  Not Explored during the call: Communicating in general with pharmacies, doctors, or insurance companies OR placing a notice in the packaging of Sucraid when this process started, letting patients know that a shortage was possible.

In answer to another caller’s question, Mr. Cooper clarified that this transfer to new facilities would not impact the cost of Sucraid or change any current programs designed to assist families in need.

Mr. Cooper ended the call by stating that QOL would love to hear stories about how Sucraid has made a difference. Again, they are doing all they can to expedite this approval process as soon as possible, and they are deeply sorry for the issues this shortage has caused to families.

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CSID and High Cholesterol?

This is the last thing I expected. My son, Parker, has struggled with gaining weight his whole life because of CSID. For years, the doctors and dietitians recommended high fat foods such as sour cream, butter, etc. to help him gain weight. When he wasn’t able to eat any form of starch, fatty dairy products were his main source of calories and carbohydrates. I have never worried about fat, since he wasn’t overweight. But I never realized how all those fatty foods could affect other areas of his health.

Over the past couple of months, I have actually started buying whole organic milk to increase his calories since he seems to be hungry all the time. However, I have also compromised in areas I would normally discourage. I have given in to allowing him to eat regular peanut butter (with Sucraid)– as much as I hate the fact it has sugar and hydrogenated oils. We have also had fast food quite frequently due to our busy schedules. This is why I discourage compromising our children’s diets, and generally don’t publicize it when I give in and allow unhealthy alternatives. We just don’t know the consequences of poor eating in an already compromised system.

I did a quick search on foods that can lower cholesterol. Almonds, fish, and yogurt are among the top ones. This doesn’t surprise me since every time we face a health crisis, the staple foods in my version of the CSID diet always pop up. Other foods on my regular CSID approved list include:

  • coconut milk
  • coconut oil
  • almond flour
  • lemons, berries, and other fruit
  • low-starch veggies 
  • sprouted grain breads and tortilla wraps (if taking enzymes to assist with starch digestion)

However, some of the foods that need to be avoided include butter, a staple ingredient in many of my recipes. The good news is, another oil I typically use is grapeseed oil, which can lower bad cholesterol. If you are also trying to lower cholesterol and would like to enjoy my CSID recipes, simply replace butter with the same amount of grapeseed oil. If needed, add a dash of sea salt to improve the flavor.

My whole family will be having a discussion about what foods will not be allowed in our house once again. I will have to give it some thought and more research, but I think if I can find healthy fat alternatives, increase fiber with vegetables and fruit, and get Parker exercising more regularly, we can get his cholesterol back to normal in no time.

Don’t think I am taking this lightly. It is very frustrating having to adjust our diets constantly. When I was diagnosed with fibromyalgia, I learned that in addition to avoiding flour and sugar, I also needed to avoid red meat and green bell peppers. I love eating healthy fresh foods, but so many of those are also off the table. Even a trip to Whole Foods or Trader Joe’s presents limitations. But I have learned to take it one day at a time. Our bodies are quick to tell us if we have swayed too far off the path of proper food choices, so we don’t stray for long. Even as a write this, I have a headache, probably due to my own giving in of a pretzel earlier this week.

By the way, the original purpose of the blood test he had was to determine if he was suffering from hypoglycemia. Those results were normal. I still think he needs to eat frequently just because of the limited amount of carbohydrates per meal (we stay below 25 grams even with enzymes).

New Article on CSID in November JPGN!

I have just retrieved the latest article published by Mary H.  Slawson (of www.csidinfo.com) regarding CSID. Here is the link for your reference. I recommend bringing a copy of it to your next doctor and dietitian appointments to clear up any misconceptions about the types of sugars and starches that are tolerated by those with CSID.

Click the article link below to read and/or download the article.

Phenotypic Observations by the CSID Dietary and Medical Support Group by Mary H. Slawson

The link to this article will also be placed on the CSID Support page.

Managing CSID after Antiobiotics

How to Bring a Healthy Balance Back to Your Gut

This past week, my daughter without CSID got strep throat. For most families, this would not be a concern. Simply go to the doctor, get antibiotics and within a few days all should be back to normal. However, in our home, using antibiotics can result in weeks or months of digestive problems. My biggest concern was that Parker would catch strep from his sister and require antibiotics. This would put him in a highly sensitive state, and reverse much of the progress he has made in recent months. Worse, his diet would become highly limited on the cusp of school starting again, and he could potentially lose weight.

So far, he is not showing any signs of illness. Upon his sister’s diagnosis, I had him on a preventative dose of 1000mg Vitamin C (Emergen-C packs sweetened with fructose), plus gargling with Echinacea and salt water three times per day. Salt water may wash away bacteria, while vitamin C and Echinacea build immune-system strength.

However, with this current threat to Parker’s digestive health, I thought I would also list the additional steps to take if antibiotics are needed in the future.

For a normal, healthy person without digestive issues, it can take up to 5 years for good bacteria to rebuild in the digestive tract! You can imagine how difficult it is for someone with digestive problems to recover.

However, I have good news. If you are prepared and take precautions, a course of antibiotics does not have to take months or years to get over.

First, it is important to have plenty of digestive-friendly foods on hand. These include eggs, green vegetables, homemade chicken broth, plain yogurt, liquid chlorophyll, lemons and berries. Fruit and yogurt smoothies and scrambled eggs with mixed and softened non-starchy vegetables are among the easiest, nutritional options while recovering from an illness.

Removing dairy products except for plain yogurt may also be needed. Avoid the normal “B.R.A.T.” diet if antibiotic use results in diarrhea. For those of us with carbohydrate intolerance, Bananas, Rice, Applesauce, and Toast can exacerbate symptoms. Ideally, implement the  Induction Diet I describe for the first phase of controlling CSID symptoms. It may take three weeks or longer to recover from a course of antibiotics, but it is important to avoid sugars and starches until normal bowel movements occur. In addition, probiotic supplements and digestive support supplements are crucial.

Finally, if starches are normally tolerated, reducing or eliminating starchy foods for ten days or longer may be needed. Antibiotics kill all the good and bad bacteria in your gut. Unfortunately, harmful bacteria grow quickly and thrive on sugars and starches. Your goal is to increase good bacteria first, which will result in a healthier balance and few digestive problems.

Although I am an advocate for natural remedies, our family has had too many serious strains of strep to consider avoiding antibiotics. However, I believe we have avoided spreading strep throat by taking precautions once we are exposed and before symptoms occur. In addition, being aware of the steps to take to bring our bellies back into balance after illness helps us to recover quickly with little long-term consequences.

Idaho Medicaid Denies Appeal to Cover Sucraid

Idaho Medicaid has officially denied our appeal to cover Sucraid for my daughter. Fortunately, back in January I began the process of working with Curascript to get 3 months of Sucraid for free. NORD will now be covering her medication until we can find another way to pay for it.

I also spoke with a representative from the Idaho Congress who will be letting me know if there is anything I can do from here on out to help bring awareness about the importance of Sucraid for those with CSID. My argument is that although it is difficult but possible to remove all sucrose from the diet, that type of diet does not provide an ample amount of carbohydrates to a growing child’s body or to an adult bordering on being underweight such as my daughter.

I still believe getting my book out there and using the power of social media are two very effective ways to bring about CSID awareness. If you have a story or struggle related to receiving Sucraid or being denied insurance coverage for Sucraid, please let me know. Every personal account we can document will help us to win this fight for our children!

The last shipment we received for Parker’s Sucraid in California listed the current cost of Sucraid at a whopping

$6116.93 for two bottles (a 28-day supply)!!

You can also contact the Partnership for Patient Assistance and search for your state for help.

I understand at this time those in Australia are not able to get Sucraid at all. If you can send me information documenting what you have tried to do up to this point to get Sucraid, I can gather every one’s stories and see if we cannot begin to pursue worldwide distribution.

In the meantime, there are enzymes you can purchase directly for low cost to help with digesting all carbohydrates. As always, there are no guarantees, and always begin with small servings to determine tolerance levels.

Enzymes my children and I have used to assist with mostly starches (but should work with natural sugars such as those found in fruit) include Nature’s Sunshine Food Enzymes and Kirkman Enzymes.

School Healthcare Plan Worksheets

A few months ago I posted a picture of the school health care plan my son’s nurse provided to manage his diet and Sucraid intake at school. I have finally created a fill-in worksheet for you to download and print for use at your child’s school.

Below you will see links to each of 4 worksheets.

The heading for Group A is LIGHT RED. The worksheets for Group A should be used for children not able to tolerate starch at this time.

The heading for Group C is TEAL. The worksheets for Group C should be used for children who are able to tolerate some starch at this time.

If you are able to store and have your child take Sucraid at school, choose the worksheet labeled WITH SUCRAID.

If you are unable to store or have your child use Sucraid at school, choose the worksheet labeled
NO SUCRAID.

School Healthcare Plan Group A WITH SUCRAID

School Healthcare Plan Group A NO SUCRAID

School Healthcare Plan Group C NO SUCRAID

School Healthcare Plan Group C WITH SUCRAID

For more information about Sucraid, dosing and its benefits for those with CSID, click HERE.

All of these worksheets will be included in the print version of my CSID book, A Place to Start once it is published.

If there is anything you would like me to change on any of these worksheets, please let me know right away. This includes any spelling or grammatical errors you may notice.

Thanks!