1. Pancreatic enzyme therapy reduces high triglycerides
in ketogenic diet patient
Lisa Vanatta MS RD, Beth Zupec-Kania RD, Randa Jarrar MD, Micah Olson MD, Jeffrey Buchhalter MD
Phoenix Children’s Hospital
Abstract
Background
Results
Discussion
Given that the ketogenic diet contains in excess of 80% of calories from fat, it is not surprising to have some elevation of cholesterol and lipid abnormalities. However, studies of lipid level changes on the ketogenic diet indicate that in 30% of children there may be cholesterol and triglyceride levels that exceed the current recommnedations for normal children, but that in most cases, this is a transient finding. Typically the body adjusts to the increased load of fat and lipid levels, though initially elevated, stabilize over time.
Reducing diet ratio, increasing polyunsaturated fats (omega-3), substituting medium chain triglycerides or adding carnitine are all approaches that are usually taken to control elevation of cholesterol and triglycerides. However, in this case, despite using several of these approaches, lipid levels continued to rise, causing elevation in pancreatic enzymes, and a concern for complications of pancreatitis. Though at least one study has shown that by observation alone, in 40% of patients, cholesterol reduced by 20%, we found this not to be the case with this patient.
It is well-known in other children, particularly the cystic fibrosis (CF) population, patients are supplemented with enzymes to help digest and absorb macronutrients, particularly fat. At the suggestion of a Cystic fiborisis dietitian, pancreatic enzyme replacement was implemented in a ketogenic diet patient. Since our patient had not responded to any other dietary intervention we were left with the option to reduce the ratio and risk seizures or to try pancreatic enzymes. Within 3 weeks of enzyme replacement, triglyceride levels normalized and have remained normal. He is currently seizure free, and off of all anti-seizure medications .
We present a case study of a 4 yo male with a diagnosis of myoclonic astatic epilepsy or Doose syndrome. Because of medical intractability of his seizures, he was started on the ketogenic diet. His ketogenic diet included one meal of KetoCal 4:1 powder mixed with water (240 mL) through a gastric feeding tube and two 4:1 ketogenic meals consumed orally. After 6 weeks on this therapy, his fasting triglycerides (TGY) were 1415 mg/dL and were subsequently 1988 mg/dL when drawn 1 month later. In an attempt to remedy this lipid abnormality, his diet was augmented with MCT oil (18 gms daily), KetoCal was changed to 4:1 Liquid (better fat profile) and he was treated with a pharmaceutical grade omega-3 fatty acid supplement (Lovaza). A repeat fasting TGY on this regimen was 2770 mg/dL. A pancreatic enzyme was initiated (Pancreatic Enzyme Formula) providing 1 capsule with each feeding. After three weeks, a repeat fasting blood analysis revealed a TGY of 105 mg/dL. On routine follow ups, his TGY levels have been maintained in the normal range. Although he did not exhibit any signs of malabsorption of fat such as flatulence or abnormal stooling prior to initiating the enzyme, we conclude that this enzyme is a harmless therapy that could potentially prevent discontinuation of the ketogenic diet and return of uncontrolled seizures.
Pt. started having seizures in March His types of seizures included myoclonic, generalized tonic-clonic, absence and drop seizures. The following medications were tried to control his seizures with their outcomes: Keppra – behavioral side effects; Depakote - improved seizure control but resulted in pancytopenia; Lamictal – developed a rash; and Banzel – ineffective in controlling seizures. He is currently on Zonisamide, but since initiating the ketogenic diet in December of 2011, he has been seizure free and Zonisamide is being weaned off. Due to difficulty taking his seizure medications, a gastrostomy feeding tube was placed and has since been used to supplement his ketogenic diet. Initial EEGs were abnormal showing generalized polyspike and wave discharges and slow spike and wave discharges. When he was admitted to the epilepsy monitoring unit in June 2012, his EEG was normal, without any clinical or subclinical seizures. A previous EEG in February 2012 was also normal. An MRI performed in December 2011 was also normal.
DATE
Pre diet
11/22/2011
2/13/2012
3/05/2012
3/19/2012
Glucose (mg/dL) 74 61 83 51
Cholesterol (mg/dL)
162
360
435
403
Triglycerides (mg/dL)
128
1415
934
1988
HDL (mg/dL) 38 23 28
LDL (mg/dL) 98
337
407
380
Free Carnitine (umol/L) 15 7
Amylase
(IU/L) 91 90
111
Lipase 26 32 55
DATE
4/02/2012
4/23/2012
5/07/2012
8/04/2012
Glucose (mg/dL) 58 65 66 64
Cholesterol (mg/dL)
336
317
255
254
Triglycerides (mg/dL)
2770
105
123
140
HDL (mg/dL) 20 38
LDL (mg/dL)
316
238
188
Free Carnitine
(umol/L) 23
Amylase (IU/L) 97 99 74 89
Lipase
(IU/L) 26 31 30 22
Bibliography
Kossoff EH, Freeman JM, Turner Z Rubenstein JE. Ketogenic Diets Treatments for Epilepsy and Other Disorders. Demos Medical Publishing, 2011.