Peutz-Jehgers Syndrome – Cannabis Treatment

Cannabis Treatment—Peutz-Jehgers Syndrome

PJS is an autosomal dominant inherited disorder in which the person develops intestinal polyps and is at higher risk for developing certain cancers.
The NIH (national institute of health) says it affects one in 25,000-3000, 000 births.

There are two types of PJS:

  • Familial PJS is due to a mutation in a gene called STK11.  The genetic defect is passed down (inherited) through families as an autosomal dominant trait.  That means if one of your parents has this type of PJS, you have a 50:50 chance of inheriting the bad gene and having the disease.
  • Sporadic PJS is not passed down through families and appears unrelated to the STK11 gene mutation.

Signs and tests

The polyps develop mainly in the small intestine, but also in the colon.  A colonoscopy will show colon polyps.  Either the small intestine is evaluated with a barium x-ray (small bowel series) or a small camera that is swallowed and then takes multiple pictures as it travels through the small bowel (capsule endoscopy).

Additional exams may show:

  • Intussusception (part of the intestine folded in on itself)
  • Noncancerous tumors in the ear (exostoses)

Treatment

Surgery may be needed to remove polyps that cause long-term problems.  Iron supplements help counteract blood loss.
Persons with this condition should be monitored by a health care provider and be checked periodically for cancerous polyp changes

Expectations (prognosis)

There may be a significant risk of these polyps becoming cancerous.  Some studies link PJS and cancers of the gastrointestinal tract, lung, breast, uterus, and ovaries.

Genetic counseling is recommended if you are planning to have children and have a family history of this condition.

The main criteria for clinical diagnosis are:
Mucocutaneous lesions causing patches of hyperpigmentation in the mouth and on the hands and feet.  The oral pigmentations are the first on the body to appear, and thus play an important part in early diagnosis.  Intraorally, they are most frequently seen on the gingiva, hard palate and inside of the cheek.  The mucosa of the lower lip is almost invariably involved as well.
In 1998, a gene was found to be associated with the mutation.  On chromosome 19, the gene known as STK11 (LKB1) is a possible tumor suppressor gene.  It is inherited in an autosomal-dominant pattern (see Mendelian inheritance) which means that anyone who has PJS has a 50% chance of passing it onto their children.


Cancer screening
Some suggestions for surveillance for cancer include the following:

  • Small intestine with small bowel radiography every 2 years,
  • Esophagogastroduodenoscopy and colonoscopy every 2 years,
  • CT scan or MRI of the pancreas yearly,
  • Ultrasound of the pelvis (women) and testes (men) yearly,
  • Mammography (women) from age 25 annually livelong, and Papanicolaou (Pap) test every year

A physician familiar with Peutz–Jeghers syndrome should supervise follow-up care.  Genetic consultation and counseling as well as urological and gynecological consultations should be done.

PJS is a chronic disorder

Cannabis is well cited in relieving abdominal pain, inflammatory polyps pain, and all other chronic pain (cancer or not).

Cannabinoids act directly on injured tissue by alleviating inflammation.  CBD and THC work together to stop pain.  Oral THC dose 5-10 mg. was found to be as effective as 60 mg. of codeine for pain relief.

Like opiates, cannabinoids block pain pathways in the central nervous system, but through a different neurochemical signaling system.

Cannabis and opium give different degrees of relief for different conditions.  Patients find that they can either replace opiates or significantly reduce them by taking cannabis.

A study found 20 mg. of cannabis to be stronger than 120 mg. of codeine but some patients did not like the psychoactive effects while others found the psychoactive effects to be analgesic(reducing pain).

References



1. Goldman L, Ausiello D. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007.
2.  James, William; Berger, Timothy; Elston, Dirk (2005).  Andrews' Diseases of the Skin: Clinical       Dermatology.  (10th ed.).  Saunders.  ISBN 0721629210.
3. Jerry E. Bouquot; Neville, Brad W.; Damm, Douglas D.; Allen, Carl P. (2008).  Oral and Maxillofacial Pathology.  Philadelphia: Saunders.  pp. 16.11.  ISBN 1-4160-3435-8.
4.  Peutz-Jeghers Syndrome.  Amos CI, Frazier ML, McGarrity TJ.  In: Pagon RA, Bird TC, Dolan CR, Stephens K, editors.  Gene Reviews [Internet].  Seattle (WA): University of Washington, Seattle; 1993-2001 Feb 23 [updated 2007 May 15]
5. "UniProtKB/Swiss-Prot entry Q15831 [STK11_HUMAN Serine/threonine-protein kinase 11"].  Retrieved 2007-07-21.
6 "Peutz-Jeghers Syndrome: In Familial Cancer Syndromes.  DL Riegert-Johnson and others.  NCBI 2009".  Retrieved 2009-07-21.
7 Boardman LA, Thibodeau SN, Schaid DJ, et al. (1998).  "Increased risk for cancer in patients with the Peutz-Jeghers syndrome".  Ann. Intern.  Med. 128 (11): 896–9.  PMID 9634427.
8 GeneReviews .Pagon RA, Bird TD, Dolan CR, et al., editors Seattle (WA): University of Washington, Seattle; 1993-.PMID: 20301443
9. Marijuana Medical Handbook:  Practical Guide to Therapeutic use of Marijuana, Dale Gieringer, Ed Rosenthal, Gregory Carter.