Compassion Corner: One Knowledgeable Doctor Helped a Porphyria Patient Get Outpatient Treatment

Compassion [kuhmpash-uhn] noun
A feeling of deep sympathy and sorrow for another who is stricken by misfortune, accompanied by a strong desire to alleviate the suffering.

Compassion Corner is a series from Patient Worthy that will focus on the subject of compassion in the healthcare and rare disease space. In this series, we explore the role of compassion in this field and what it means for caregivers, patients, and others.

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Claire Richmond is a columnist at the Porphyria News. Claire tells her story about the difficulty she had experienced in getting approval to receive her medication as an outpatient.

Claire shares her journey with porphyria and expresses her gratitude to a doctor who showed compassion. He helped Claire to finally receive her medication as an outpatient.

One particular holiday season was especially memorable to Claire. She recalled being in the hospital over the holidays. Claire had to comply with the rules of receiving Panhematin infusions in the hospital while she was having a porphyria attack or in anticipation of an attack.

During that hospital stay, Claire was introduced to a port that was surgically implanted into her chest just below her right collarbone.

A port is a plastic disc the size of a U.S. quarter. It is used to feed medication into the patient’s large vein which goes to the heart. Blood can also be withdrawn using a port.

Although it was not a pleasant thought, by then she considered herself to be a hospital regular, as she seemed to be in the hospital on a monthly basis.

Within a month after her holiday stay at the hospital,  Claire was back again for three days to ward off another attack. This time it was on her thirty-fourth birthday.

About Porphyria

Acute hepatic porphyria (AHP) refers to a group of diseases that are set off by the buildup of porphyrins which are natural chemicals in the body. Porphyrins, found in the liver or other areas, are made when the body creates heme.

By definition, heme is a deep red porphyrin found in hemoglobin which distributes oxygen from the lungs throughout the body.

Signs and Symptoms

AHP may cause damage to nerves. Other symptoms may be:

  • Abdominal pain (most common)
  • Nausea or swollen abdomen
  • Muscle aches
  • Constipation or diarrhea.
  • Tingling or numbness
  • Paralysis or weakness

Since there is currently no cure, treatment is the only option.

Claire is No Longer a Hospital Regular

Patients receive Panhematin via intravenous infusion while they are experiencing an attack. The treatment generally requires hospitalization for several days.

Claire’s first year of treatment consisted of continuous infusions of Panhematin. Claire’s partner, Michael, accompanied her on these visits. Michael slept on the vinyl couch in her room and joined Claire in dinners she describes as “rubber pasta.”

The medication seemed to be effective, which gave Claire the impression that the hospital visits would be short-lived. On the contrary, the couple eventually learned the infusions would be ongoing.

Claire tried to settle in and accept the continued hospital stays. But as she connected with other porphyria patients she learned that many were not required to remain in the hospital in order to receive their infusions. Claire learned that Panhematin has been approved for administration in an outpatient setting.

Claire volunteered at the Porphyria Foundation and subsequently learned that many administrators were not comfortable working with an unknown disease as well as treating the disease with a little-known medication.

Although she appreciated the administrators’ concern, she sincerely felt that she was occupying a hospital bed when there were very ill patients who were in need of that bed. It just did not make sense that she would occupy a hospital room for several days instead of a chemo recliner in a local clinic for several hours.

A Doctor with Compassion

Claire gives some credit for the change in her treatment policy to being exposed to new information. However, she says that a doctor who was familiar with porphyria also helped advocate for her position and get her into outpatient administration of the treatment.

 

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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