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Porphyria Educational Services

Vol. 1 No. 31                                    August 1, 1999

FOCUS:  Nursing Considerations Regarding Porphyria Patients

Often porphyria patients spend countless hours in emergency roomsunable to receive timely and adequate treatment. Quite often patients
have been "sent home" or have been dismissed as "a mental problem" because no definable diagnosis can be made "on-the-spot".

Because of the extreme pain, skin abrasision, and disabling neurological problems associated with porphyria, quite often porphyria patients have
lost employment or find themselves no longer able to work. This not only makes for heavy financial loss, patients often loose their medical
insurance and suffer heavy medical bills due to repeated testing for a diagnosis because many tests are inadequate, compromised in handling or assaying.

Stress on porphyria families is quite hard as well. Many families also have a second or third member who is also porphyric because roughly 50%
of offspring inherited the genetic defect.

Nursing considerations for porphyric patients: *Be willing to listen to the patient.

This is important because often porphyric patients are disbelieved by family and friends and not to mention the medical community. Therefore many porphyrics do not talk about their disease and fears and concerns because of being looked at as weird, or deemed "psychological" or having it {"all in the head").

Having someone listen to them with and interest in them really makes a difference.

*Begin IV intervention therapy immediately!

Secure the ordered labs immediately!     Hemoglobin, CBC, Electrolyte, Glucose, and UA.

Lab tests are very important to determine possible triggers, especially when the porphyria attack has been triggered by an other illness which can be verified by the lab tests... infection in the blood chemical tests, UTIs or bladder in the UA's.    Imbalance in the electrolytes.  The glucose is important in safeguarding the patient   if they are becoming diabetic from the high regiment of daily carbohydrate ingestion. Very important to check this.

IV glucose is what stops the overproduction of porphyrins and when this happens the pain is reduced, the nausea and vomiting begin to subside and the electrolyte balance will stop from further imbalance which is the cause of the mental changes in the porphyric patient.  Timing is everything in porphyria.

Doing the personal assessments of skin abrasions and integrity can be done after the glucose is running.

A personal belonging inventory of the patient's clothing, jewelry and personal possessions can also wait until after the glucose is running.

So again timing is everything!

*Begin pain control right away after initial assessment.

Pain control at the beginning speeds things along and usually can be withdrawn after the glucose begins to stop the porphyrin production.

*Realize that exacerbations that present at the onset of an acute attack can readily change a patient's personality.

A passive quiet person during the onset of an acute attack quite often can and does change to being disoriented, loud, pushy or very agressive through no fault of their own.

*Realize that seizure activity can occur with a porphyria patient without the patient even knowing that they have a probem.

ANS [altered neurologic state] is a basic sign/symptom of of acute attack.

0ften such ANS episodes will include partial clonic seizures. For this reason be sure the patient's bed rails are covered or that there are pillows on both sides of the patient's head so they do not get a concussion from head their head on their hospital bed rails.

*Realize it is very important for a porphyric patient to be place in a room by themselves.

Because of the extreme personality changes and the basic lack of understanding of the disease the presence of others in a patient's room makes for a more difficult time for the patient.  Visitors to other roommates can and often make comments about the other patient like the patient is deaf or unable to comprehend what they are saying. This is really a negative when a porphyric is having a bad enough time with people not understanding their disease to have to put up with more lack of understanding by roommates and their visitors.

A room should be left relativedly darkened in order to reduce light sensitivity, blurry vision, and to encourage sleep.

Likewise the door to a porphyric patient's room should rmain closed at all times to allow for quiet and privacy for the same reasons as above.

Vomiting is a common occurence with acute attacks. Place a moisten washcloth and a stacks of towels within the patient's reach.

Refrain from remarks such as "at it again" "What a mess", "If you didn't drink orange juice you wouldn't have the acid in your stomach to cause you to vomit"  or    "Stop eating  so you stop puking".

Remember that the porphyric patient is highly encouraged to ingest as much carbohydrate intake as possible since they must have between 400-500mg carbo within 24 hours to bring an acute attack under control. Remember  that remarks that are made will be long remembered.  When a porphyric is in attack and         feeling most vulberable any remarks can cut and further hurt the patient.

Furthermore orange juice is encouraged because of its high carbo content as well as the potassium which is very necessary with the loss of electrolytes during an attack.

A basin place on top of a garbage pan is the best, since the smal emenis trays rarely can contain/control sudden vomiting episodes.  Have the basins in place on both sides of the bed and both patient and nurse have a lot less to deal with.

*Do not wake the patient during the night. All vitals and labs can be maintained before 10:30 p.m. and after 5 :30 a.m.  It is vital that the patient be left undisturb.  If nursing care is needed the patient can push their buzzer.

*Be sure that the porphyric patient has available high carbo food/feeding at least every two hours during what would be their normal waking hours.

Continuous feedings are necessary to reach the 400-500 mg needed.  And it also encourages the porphyric the maintenance regiment that they should have at home from waking to going to bed.  Carbo intake every 2 to 3 hours max.  Fasting should never exceed 6 1/2 hours. [Normal sleep range].

*Do not appear starled by red colored urine.

Also do not try to make the patient believe that the urine color is due to their menses!

*Do not  continually repeat blood pressure readings on a porphyric's arm with an automated cuff.

Hypotension is common at the beginning of acute attack. Just because a BP reads 54/42 with a pulse of 43 do not repeat more than once.  A second reading enough for validation of the low statistics.

*It is important to remember that many porphyric patients begin to experience peripheral neuropathy and with use of the automatic cuff extreme bruising and possible nerve damage can be done from the repeated constriction of the cuff.

It is also common for the blood pressure to rise quite rapidly as the pain index rises during the acute attack.

Statistically most porphyrics do not have regular high blood pressure, and are not treated for high BP on a regular basis.

The most prescribed medication to control the majority of exarcebations during an acute attack is propranolol.
        Covers ANS, seizures, mental changes, anxiety, pulse, BP and induces drowsiness.

*Warm tub baths rather than showers are encouraged for porphyric patients to help abort PN problems, and also for reducing possible UTIs that are common due to the urinary retention problems ofan acute attack.

*Please do not be offended if a porphyric patient tells you how to treat their disease.  A nurs has many other patients with many other more common diseases.  Porphyria is rarely understood by the majority of emdical personnel. The average porphyric is well versed in their disease.

*Most porphyric are chemically sensitive.  Avoid the use of all adhesives.  USE PAPER TAPE ONLY!

*Do not discuss a porphyric patient with other staff just outside their door.  Conference in the room in their presence  or  in a conference removed from the hall way.

*Bring in the patient's chart and invite them to discuss their progress and allow them to  know what is being charted.

*Include the porphyric patient in their discharge planning. A porph knows their home situation better than anyone at the hospital does.

*If a porphyric patient wants to visit with their doctor during rounds, allow the patient the privacy to talk with their doctor without the nurse's presence.  Confidentiality and the trust relationship between doctor and patient does not automatically extend to nurses or others who are only temperorarily involved in a patient's long term care.

*Read thorughly all materials on porphyria brought to the hospital by the patient. It is there for you as their nurse to learn from in order to better help the patient.

Do not be offended by the patient's offer to help you.

Porphyric patients are not handicapped.  They have dealt with the disease on an ongoing basis and want to be independent and in control of their lives. This sense of control is especially important for porphyrics because of the great loss of control of their day to to day lives because of the disease.

*Greater Grand Forks Area Porphyria Support Group
Forumulated June 14, 1998.