CPT2 Online Resources -- an incomplete list

Blogs:
http://cpt2kids.blogspot.com/

https://www.youtube.com/channel/UCi0QDhIWGD8sWYyuh6_Mdpw (video blog by a ten year old with LCHAD about low fat cooking).


***
Medical:

http://www.newbornscreening.info/Parents/fattyaciddisorders/CPT2.html

National Institutes for Health: http://ghr.nlm.nih.gov/condition/carnitine-palmitoyltransferase-ii-deficiency

GeneReviews: http://www.ncbi.nlm.nih.gov/books/NBK1253/

Does genetic testing for CPT2: http://www.genedx.com/test-catalog/disorders/carnitine-palmitoyltransferase-ii-cpt2-deficiency/

Article about dietary supplement and CPT2: http://hmg.oxfordjournals.org/content/early/2011/03/04/hmg.ddr089.full.pdf

Very long list of CPT2 research and resources:  https://www.idph.state.ia.us/genetics/common/pdf/cpt2_ref.pdf


List of Resources I found regarding triggers (also contain a lot of good information about CPT2):

Sources:

“NIH” = http://ghr.nlm.nih.gov/condition/carnitine-palmitoyltransferase-ii-deficiency

“GeneReviews” = http://www.ncbi.nlm.nih.gov/books/NBK1253/

“Cincinnati Childrens” =http://www.cincinnatichildrens.org/WorkArea/DownloadAsset.aspx?id=100656

“FOD” (Fatty Acid Oxidation Disorders support group) =https://www.fodsupport.org/cpt2.htm

“CPTDeficiency” (the CTP2 Deficiency Association) =http://www.cptdeficiency.org/inhoud/attack_triggers_inhoud.htm

“Counsyl” = https://www.counsyl.com/services/family-prep-screen/diseases/carnitine-palmitoyltransferase-ii-deficiency/

“Connecticut” (State of Connecticut Genetics Newborn Screening Program):http://www.ct.gov/dph/lib/dph/family_health/newborn_screening/pdf/hpcptii.pdf

“Wikipedia” (Wikipedia page retrieved 9/2/2014): http://en.wikipedia.org/wiki/Carnitine_palmitoyltransferase_II_deficiency

“Nature” (the journal Nature):http://www.nature.com/labinvest/journal/v83/n11/full/3780745a.html

“NMD” (Neuromuscular Disorders Journal): http://www.nmd-journal.com/article/S0960-8966(01)00228-0/abstract

“CER” (Clin. Exp. Rheumatol.): http://www.ncbi.nlm.nih.gov/pubmed/11579721

“Annals” (Annals of Internal Medicine): http://annals.org/article.aspx?articleid=691984

“Recombine”: https://recombine.com/diseases/carnitine-palmitoyltransferase-ii-deficiency/pdf

“NextProt”: http://www.nextprot.org/db/entry/NX_P23786/medical

“Atlas” (Atlas of Neuromuscular Diseases: A Practical Guideline, by Feldman, Grisold, Russell, and Zifko, page 417): http://books.google.com/books?id=ibJ37j100jAC&pg=PA417&lpg=PA417&dq=cpt2+triggers&source=bl&ots=JdFYIf2zvw&sig=9SjhEbCl0T5_vG7Ia_yMnpHalnw&hl=en&sa=X&ei=n1QGVJHJFsq1ogSwi4H4Aw&ved=0CFgQ6AEwBzgK#v=onepage&q=cpt2%20triggers&f=false

“Myopathies” (Myopathies, An Issue of Neurologic Clinics by Mazen Dimachkie, page 788):http://books.google.com/books?id=IiA4BAAAQBAJ&pg=PA788&dq=cpt2+triggers&hl=en&sa=X&ei=t10GVNjkMte4ogSBkIL4Ag&ved=0CCwQ6AEwAg (note that this source also identifies MCT oil and Bezafibrate as improving CPT2 symptoms)

“Muscle Disease” (Muscle Disease: Pathology and Genetics by Goebel, Sewry and Wells, page 266): http://books.google.com/books?id=hyC5UvC5cAAC&pg=PA266&dq=cpt2+triggers&hl=en&sa=X&ei=t10GVNjkMte4ogSBkIL4Ag&ved=0CDIQ6AEwAw (note that this source also identifies Bezafibrate as “shown to restore the capacity for normal fatty acid oxidation in muscle cells”, and recommends MCT oil)

“Lipobiology” (Lipobiology by G.J. van der Vusse, page 306):http://books.google.com/books?id=hyC5UvC5cAAC&pg=PA266&dq=cpt2+triggers&hl=en&sa=X&ei=t10GVNjkMte4ogSBkIL4Ag&ved=0CDIQ6AEwAw
"Washington Neuromuscular" (Washington University, St. Louis, MO Neuromuscular Disease Center): http://neuromuscular.wustl.edu/msys/cardiac.html#cpt2 (note: Provides a detailed list of information about CPT2)

"Orphanet" (Orphanet, portal for rare diseases and orphan drugs): http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=157
"Iowa DPH" (Iowa State Department of Public Health): https://idph.state.ia.us/genetics/common/pdf/cpt2.pdf
"Bonnefont" (Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects by Bonnefont et al.): http://www.carnevalijunior.com.br/wp-content/uploads/2010/03/cpt2001.pdf

Medlink (Medlink Neurology): http://www.medlink.com/medlinkcontent.asp
"Biomed Research" (Journal of Biomedicine and Biotechnology, Vol. 2010, Article ID 340849): http://www.hindawi.com/journals/bmri/2010/340849/ (also provides an easy to understand description of fatty acid metabolism during exercise)
"New England Journal" (New England Journal of Medicine, Feb. 19, 2009 article by Bonnefont, et al.): http://www.nejm.org/doi/full/10.1056/NEJMc0806334
"Genetic Neuromuscular Disorders" (chapter 64):http://link.springer.com/chapter/10.1007/978-3-319-07500-6_64
"OMIM" (Online Mendelian Inheritance in Man online gatalog of human genes and genetic disorders): http://omim.org/entry/255110
"Hippokratia" ("Rhabdomyolysis updated" article): http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2658796/

Symptomatic but no CPK elevation....

I had a CPK test scheduled for yesterday to see if my numbers had come down to normal.  I was above the 20,000 maximum test limit on August 20.  By August 23, I was at 16,078.  I remained high seven days after my rhabdo, measuring 423 on August 27.

Yesterday morning I awoke with muscle pain.  It was in my lower legs and lower back, and it felt dead-on like the beginning of a CPT2 episode.  I had MCT oil, corn starch, Gatorade, carbs -- you know, the usual stuff I do when I feel a CPT2 episode coming on.  My muscles remained sore -- and I continued to feel like I was having an episode -- when I had my blood drawn at 1:38 p.m.  I tested at the high end of normal, 138.

This is a very interesting piece of information.  It means that there is a significant time window in between feeling markedly symptomatic and incurring enough rhabdomyolysis to elevate my CPK.  I doubt that intervention will stop the rhabdomyolysis every time.  In fact, I'm pretty sure it won't.  But knowing that an episode can start and end without triggering seriously elevated CPK feels like an important data point.

While I was hospitalized, I couldn't find a source for how fast CPK numbers come down.  I'm sure everybody's numbers drop at different rates, but here is my recent CPK history, in case somebody finds it helpful.

• 138 IU/L

  Date

  Sept. 3, 2014 01:38 p.m. PDT

• 423 IU/L

  Date

  Aug. 27, 2014 12:24 p.m. PDT

  High

• 2561 IU/L

  Date

  Aug. 25, 2014 06:43 a.m. PDT

  High

• 2705 IU/L

  Date

  Aug. 25, 2014 06:43 a.m. PDT

  High

• 4925 IU/L

  Date

  Aug. 24, 2014 03:15 p.m. PDT

  High

• 3476 IU/L

  Date

  Aug. 24, 2014 04:17 a.m. PDT

  High

• 16078 IU/L

  Date

  Aug. 23, 2014 04:20 a.m. PDT

  High

• > >20000 IU/L

  Date

  Aug. 22, 2014 05:51 a.m. PDT

  High

• > >20000 IU/L

  Date

  Aug. 21, 2014 05:40 a.m. PDT

  High

• > >20000 IU/L

  Date

  Aug. 20, 2014 04:30 p.m. PDT

  High

CPT2 Triggers

The literature is confusing as to what triggers CPT2 episodes.  This article is an attempt to list everything identified as a possible trigger, and to identify the sources that identify it.  Obviously, triggers identified by many different sources are the most likely to be strong triggers.

Additions/Corrections to this list are ongoing.

CPT2 Triggers

Inadequate food consumption	NIH, Cincinnati Childrens, CPTDeficiency, Counsyl, Connecticut, FOD, Wikipedia, Nature, Annals, Recombine, NextProt, Atlas, Myopathies, Lipobiology,Washington Neuromuscular, Orphanet, Iowa DPH, Bonnefont, Medlink, Genetic Neuromuscular Disorders, OMIM
High fat diet	CPTDeficiency, Wikipedia, Nature, Annals, Atlas,Washington Neuromuscular, Bonnefont, Genetic Neuromuscular Disorders
Dehydration	Frequently listed as important in recovery; not recognized in the literature as a trigger. Note: There is some anecdotal support for dehydration being a trigger.
Exercise	NIH, Counsyl, Connecticut, FOD, Wikipedia, Nature, Recombine, NextProt, Atlas, Myopathies, Muscle Disease, Lipobiology,Washington Neuromuscular, Orphanet, Iowa DPH, Bonnefont, Medlink, Biomed Research, Genetic Neuromuscular Disorders, GeneReviews, Cincinnati Childrens, CPTDeficiency, OMIM
Failure to follow ER Protocol letter (including malpractice in handling a CPT2 patient in the ER)	https://www.cpt2.me/2019/08/my-er-visit-triggered-cpt2-and-landed.html
Psychological stress/anxiety	NIH, GeneReviews, CPTDeficiency, Counsyl, Connecticut, Nature, CER, Bonnefont, Medlink, Genetic Neuromuscular Disorders
Lack of sleep/inadequate sleep	CPTDeficiency, Counsyl, Connecticut, FOD,
Fever	Wikipedia, Myopathies, Bonnefont, Biomed Research, Genetic Neuromuscular Disorders, OMIM
Infection	NIH, Cincinnati Childrens, CPTDeficiency, Connecticut, Wikipedia, Nature, Atlas, Lipobiology,Washington Neuromuscular, Iowa DPH, Bonnefont, Medlink, Genetic Neuromuscular Disorders
--Viral Infection	CPTDeficiency, FOD, NextProt,, Orphanet
Extremes in temperature	NIH, Connecticut, FOD, Orphanet, Iowa DPH, Genetic Neuromuscular Disorders
-- Cold temperature	GeneReviews, CPTDeficiency, Counsyl, Wikipedia, Nature, Annals, Atlas, Lipobiology,Washington Neuromuscular,  Iowa DPH, Bonnefont, Medlink, Genetic Neuromuscular Disorders
Medications	--
--Valproic acid	GeneReviews, NMD, Washington Neuromuscular, Bonnefont, Medlink
--General anesthesia	GeneReviews, CPTDeficiency, Counsyl, Connecticut, FOD, Atlas,Washington Neuromuscular, Bonnefont, Medlink
--Ibuprofen (Advil/Motrin)	GeneReviews, CPTDeficiency, Bonnefont, Medlink
--Diazapam in high doses	GeneReviews, CPTDeficiency, Bonnefont, Medlink
--Statins	Molecular Mechanisms of Statin Intolerance
--Ezetimibe	Ezetimibe-associated myopathy in monotherapy and in combination with a 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitor; Monotherapy with Ezetimibe Causing Myopathy; Myopathy induced by statin-ezetimibe combination: Evaluation of potential risk factors; Product Monograph for Sandoz Ezetimibe; Secondary evidence of interference with fatty acid oxidation found in the article "Worsening myopathy associated with ezetimibe in a patient with McArdle disease".
Trauma	CPTDeficiency
Surgery	CPTDeficiency
Alcohol	CPTDeficiency

CPT2 Triggers based on anecdotal evidence:

Menstrual Period and/or ovulation	"increased pain and stiffness every month prior to period"; "My body gets so stiff before menstruation and I have total weakness during ovulation".
Prednisone	"I had to take it 5 years ago for pneumonia and found that I couldn’t as it increased my metabolism."
Gan Mao Ling	"I took it because it is supposed to reduce illness when taken at onset.  It caused severe cramping in my large leg muscles.  I thought it was a coincidence, and took it again a month later when I thought I was getting sick.  It again caused severe cramping.  I had my CK tested and it was elevated.  I do note that in both cases, my oncoming illness completely stopped right after taking Gan Mao Ling."
High Altitude/Low Oxygen	As CPT2 is a fatty acid oxidation disorder, availability of oxygen may be a factor.  Caution should be used in lower oxygen environments (aircraft, mountains, etc).

Air Travel

From a post on https://www.facebook.com/groups/CptType2 CPT2 episodes appear to be triggered by air travel. As flights are pressurized to around 2500 meters/8000 feet, slight hypoxia may be involved. The stress of air travel might also be a trigger.

Rhabdomyolysis Triggers (Since a primary risk of CPT2 deficiency is rhabdomyolysis, anything that triggers rhabdomyolysis in non-CPT2 patients is something CPT2 patients might want to avoid):

Alcohol  Abuse	Hippokratia
Excessive physical exertion, especially in previously untrained individuals ("white-collar rhabdomyolysis")	Hippokratia
High Temperature, temperature extremes	Hippokratia
Humidity	Hippokratia
Conditions of severe agitation, such as tonicclonic seizures	Hippokratia
Direct muscle injury (particularly "crush injury syndrome"	Hippokratia
Muscle ischemia (shock, CO poisoning, asthma, arterial thrombosis, vascular occlusion, air emboli, severe sickle cell crisis, prolonged immobilization)	Hippokratia
Abuse drugs (cocaine, heroin, other opiates, amphetamines, club drugs like ecstasy, benzodiazepines)	Hippokratia
Statins	Hippokratia
"other drugs/toxins/venoms": Various drugs, such as corticosteroids, immunosuppressants, salicylates, fibrates, antibiotics, chemotherapeutic agents, antidepressants, antipsychotics and anesthetics have been associated with rhabdomyolysis, not only in toxic, but also in therapeutics doses.	Hippokratia
Metabolic Endorine Disorders	Hippokratia
Infection	Hippokratia
There are many other events that can infrequently result in rhabdomyolysis	Hippokratia

Sources:

“NIH” = http://ghr.nlm.nih.gov/condition/carnitine-palmitoyltransferase-ii-deficiency

“GeneReviews” = http://www.ncbi.nlm.nih.gov/books/NBK1253/

“Cincinnati Childrens” = http://www.cincinnatichildrens.org/WorkArea/DownloadAsset.aspx?id=100656

“FOD” (Fatty Acid Oxidation Disorders support group) = https://www.fodsupport.org/cpt2.htm

“CPTDeficiency” (the CTP2 Deficiency Association) = http://www.cptdeficiency.org/inhoud/attack_triggers_inhoud.htm

“Counsyl” = https://www.counsyl.com/services/family-prep-screen/diseases/carnitine-palmitoyltransferase-ii-deficiency/

“Connecticut” (State of Connecticut Genetics Newborn Screening Program): http://www.ct.gov/dph/lib/dph/family_health/newborn_screening/pdf/hpcptii.pdf

“Wikipedia” (Wikipedia page retrieved 9/2/2014):  http://en.wikipedia.org/wiki/Carnitine_palmitoyltransferase_II_deficiency

“Nature” (the journal Nature): http://www.nature.com/labinvest/journal/v83/n11/full/3780745a.html

“NMD” (Neuromuscular Disorders Journal): http://www.nmd-journal.com/article/S0960-8966(01)00228-0/abstract

“CER” (Clin. Exp. Rheumatol.): http://www.ncbi.nlm.nih.gov/pubmed/11579721

“Annals” (Annals of Internal Medicine): http://annals.org/article.aspx?articleid=691984

“Recombine”: https://recombine.com/diseases/carnitine-palmitoyltransferase-ii-deficiency/pdf

“NextProt”: http://www.nextprot.org/db/entry/NX_P23786/medical

“Atlas” (Atlas of Neuromuscular Diseases: A Practical Guideline, by Feldman, Grisold, Russell, and Zifko, page 417): http://books.google.com/books?id=ibJ37j100jAC&pg=PA417&lpg=PA417&dq=cpt2+triggers&source=bl&ots=JdFYIf2zvw&sig=9SjhEbCl0T5_vG7Ia_yMnpHalnw&hl=en&sa=X&ei=n1QGVJHJFsq1ogSwi4H4Aw&ved=0CFgQ6AEwBzgK#v=onepage&q=cpt2%20triggers&f=false

“Myopathies” (Myopathies, An Issue of Neurologic Clinics by Mazen Dimachkie, page 788): http://books.google.com/books?id=IiA4BAAAQBAJ&pg=PA788&dq=cpt2+triggers&hl=en&sa=X&ei=t10GVNjkMte4ogSBkIL4Ag&ved=0CCwQ6AEwAg (note that this source also identifies MCT oil and Bezafibrate as improving CPT2 symptoms)

“Muscle Disease” (Muscle Disease: Pathology and Genetics by Goebel, Sewry and Wells, page 266): http://books.google.com/books?id=hyC5UvC5cAAC&pg=PA266&dq=cpt2+triggers&hl=en&sa=X&ei=t10GVNjkMte4ogSBkIL4Ag&ved=0CDIQ6AEwAw (note that this source also identifies Bezafibrate as “shown to restore the capacity for normal fatty acid oxidation in muscle cells”, and recommends MCT oil)

“Lipobiology” (Lipobiology by G.J. van der Vusse, page 306): http://books.google.com/books?id=hyC5UvC5cAAC&pg=PA266&dq=cpt2+triggers&hl=en&sa=X&ei=t10GVNjkMte4ogSBkIL4Ag&ved=0CDIQ6AEwAw
"Washington Neuromuscular" (Washington University, St. Louis, MO Neuromuscular Disease Center): http://neuromuscular.wustl.edu/msys/cardiac.html#cpt2 (note: Provides a detailed list of information about CPT2)

"Orphanet" (Orphanet, portal for rare diseases and orphan drugs): http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=157
"Iowa DPH" (Iowa State Department of Public Health): https://idph.state.ia.us/genetics/common/pdf/cpt2.pdf
"Bonnefont" (Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects by Bonnefont et al.): http://www.carnevalijunior.com.br/wp-content/uploads/2010/03/cpt2001.pdf

Medlink (Medlink Neurology): http://www.medlink.com/medlinkcontent.asp
"Biomed Research" (Journal of Biomedicine and Biotechnology, Vol. 2010, Article ID 340849): http://www.hindawi.com/journals/bmri/2010/340849/ (also provides an easy to understand description of fatty acid metabolism during exercise)
"New England Journal" (New England Journal of Medicine, Feb. 19, 2009 article by Bonnefont, et al.): http://www.nejm.org/doi/full/10.1056/NEJMc0806334
"Genetic Neuromuscular Disorders" (chapter 64): http://link.springer.com/chapter/10.1007/978-3-319-07500-6_64
"OMIM" (Online Mendelian Inheritance in Man online gatalog of human genes and genetic disorders): http://omim.org/entry/255110
"Hippokratia" ("Rhabdomyolysis updated" article): http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2658796/
"Molecular mechanisms of statin intolerance": https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4889699/
"Ezetimibe-associated myopathy in monotherapy and in combination with a 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitor": https://www.onlinecjc.ca/article/S0828-282X(06)70253-7/fulltext
"Monotherapy with Ezetimibe Causing Myopathy": https://www.amjmed.com/article/S0002-9343(05)00557-7/fulltext
"Worsening mypoathy associated with ezetimibe in a patient with McArdle Disease": https://academic.oup.com/qjmed/article/98/6/461/1548198
"Myopathy induced by statin-ezetimibe combination: Evaluation of potential risk factors": https://academic.oup.com/qjmed/article/98/6/461/1548198
"Product Monograph Sandoz Ezetimibe": https://www.sandoz.ca/sites/www.sandoz.ca/files/Ezetimibe%20Product%20Monograph.pdf

Other notes:

Treatment Protocol: “Glucose remains the mainstay therapy in the management of CPT II deficiency. Intravenous glucose infusions have been shown to be beneficial in improving exercise tolerance, whereas oral glucose has not” (Nature)

Interesting:  “Exercise tolerance is markedly improved by a glucose infusion in patients with CPT II deficiency, but because of lower glucose availability and higher insulin levels that inhibit muscle glycogenolysis, the patients cannot achieve this effect themselves by oral glucose ingestion.” http://www.ncbi.nlm.nih.gov/pubmed/12370460?dopt=Abstract&holding=npg

CPT2 Emergency Room Treatment Protocol -- A non-physician's first draft

When I was recently seen in the emergency room, I realized that it would be very helpful to have a quick list to provide the triage nurse and others.  This list is in no way medical advice, and does not substitute for a discussion with your own physician.

This is a draft/guess/starting point for what such a letter might look like.  Please leave any feedback in the comments, or just email me.  I'd love to come up with a template that can be used generically for those with CPT2.  I'll be adding to and correcting this post over time.


Dear Emergency Room Staff:

Your patient, Gary Shuster, has been diagnosed with Carnitine palmitoyltransferase II Deficiency, or "CPT2 Deficiency".

Diagnostic Data:

The diagnosis was based on a muscle biopsy performed on 12/8/1992 and a genetic test conducted on 1/14/2010.

The muscle biopsy revealed that the patient has Carnitine palmityl transferase levels of 38 nmol/min/100 mg NCP (normal range is 96 - 120).  The muscle biopsy further revealed free carnitine of 14 nmol/mg NCP (normal is 15-21 nmol/mg NCP) and total carnitine of 17 nmol/mg NCP (normal is 19-27 nmol/mg NCP).  The diagnostic conclusion reached on that test was that the "muscle biopsy reveals significant CPT deficiency likely accounting for the clinical syndrome."

The genetic test results show the following:

(1) Acylcarnitine Ratio (C16+C18/C2): 0.24
Normal Controls: 0.13 +/- 0.02 [range: 0.11 - 0.15]
Carriers for 1 CPT2 mutation: 0.15 +/- 0.02 [range: 0.13 -0.19]
Affected individuals with 2 CPT2 gene mutations: 0.36 +- 0.08 [range: 0.20 - 0.52]

(2) Mutation results:
(a) Number of mutations found: 1
(b) Nucleotide changes or mutations: c.[338C>T]+[=],
(c) Amino acid change: p.[Ser113Leu]+[=]

The interpretation given those results was as follows:  Only one mutation was found, but the acylcarnitine ratio is in the range expected for CPT2 deficiency-affected individuals.    Accordingly, the patient has a second mutation in a non-coding region of the gene or in a different gene in the fatty acid oxidation pathway that has impact on CPT2 gene function.

Clinical Presentation:

The patient will likely present with as many as five primary symptoms:

(1) Severe muscle pain due to rhabdomyolysis secondary to CPT2 deficiency;
(2) Hypoglycemia with possible minor cognitive impairment due to hypoglycemia;
(3) Myoglobinuria secondary to rhabdomyolysis;
(4) Elevated CPK levels, potentially as high as 100,000 in the case of a severe episode.
(5) Potential renal impairment or failure secondary to rhabdomyolysis.

Please note that any one of these symptoms indicates that the patient is highly likely to be experiencing a CPT2 episode.

Urgent/Stat Medical Intervention:

The following must happen immediately in order to stabilize the patient and prevent further renal damage:

CPK levels should be checked immediately.
IV fluids should be started immediately.  While awaiting CPK test results, a rate of 1,000 cc of saline per hour is appropriate.
Sodium Bicarbonate may be appropriate depending on clinical presentation.
Dextrose (D-10) should be provided immediately.

Triggers for Relapse; Patient Care to Avoid Relapse

CPT2-induced muscle damage and rhabdomyolysis can be triggered within a hospital setting.  This patient was hospitalized on August 2014 for elevated CPK secondary to CPT2 deficiency.  While hospitalized in a two-patient room, the patient experienced a relapse (as measured by a significant increase in CPK levels several days after CPK levels began to drop) as a result of lack of sleep and stress.  The patient must be placed in a single room to maximize sleep and minimize stress.  Placement of the patient in a multiple-patient environment must be avoided as it creates a substantial risk of relapse.  As viral infections in the herpes family are strongly associated with CPT2 episodes, the patient must be maintained on Aclyclovir 400 mg bid.

CPT2 episodes are triggered by the following:

* Insufficient carbohydrate intake;
* Extended exercise;
* Stress;
* Lack of sleep;
* Infection, with heightened risk associated with viral infection;
* Cold temperatures;
* High fat diet.

The literature is in conflict about certain other potential triggers, so the patient should be monitored for signs of relapse if the following are present:

* General anesthesia;
* High dose diazapam;
* Exposure to extreme heat

Malignant Hyperthermia Secondary to General Anesthetic

CPT2 patients are known to have an increased risk of malignant hyperthermia when provided with general anesthetic.  This patient has had general anesthetic on three occasions without complication, but should still be carefully monitored for signs of malignant hyperthermia if given general anesthetic.

MCT Oil Cookies

UPDATE 9/16/2014:  I have been told that cooking corn starch (or even mixing it with orange juice) makes it much more quickly metabolized, thereby reducing its efficacy in preventing the onset of CPT2 symptoms.  I'm not a food scientist (a food fan, yes, food scientist, no) so I cannot say whether this is accurate or not, but I'm proceeding as if it is accurate.  So MCT oil cookies, yes, but the corn starch is back to being mixed with water for a "fun" drink.

I'm taking this whole "preventing muscle breakdown" very seriously.  I don't want to be back in the hospital due to my not paying attention.

There is plenty of anecdotal evidence that MCT oil and corn starch can help prevent CPT2 attacks.  I'm not sure what the deal is with corn starch, but with MCT oil my understanding is that it is one of the few fats that CPT2 patients can metabolize normally.  My new approach is to substitute MCT oil for other fats when possible.

I am also trying figure out the best way to get MCT oil and corn starch into a quick and easy-to-consume form.  I wanted to make some kind of bar, but cookies seemed easier to experiment with (since I know how to make them well without MCT oil and corn starch).

I took the normal "toll house cookies" recipe and modified it as follows:

Substitute 1 cup of MCT oil for the 1 cup of butter
Add 4 tablespoons of corn starch to the flour
Reduce the number of chocolate chips by half (I wanted to make the cookies slightly less unhealthy)

The results were great.  I vacuum sealed a bunch of "4 packs" of the cookies and put them in the freezer.  They're easy to grab and much more manageable than trying to eat a spoonful of MCT oil and a bunch of corn starch.

I'm sure I'll tire of MCT cookies soon, so I'm going to keep trying to develop new recipes.  In the meantime, I have the cookies.

The end result -- cookies that look and taste great.

The uncooked dough.  It sure looked like it was going to be a disaster.